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it has been about 30 years since endoscopic ultrasound ( eus ) was first introduced into clinical medicine . combination of endoscopy with ultrasound probe was such a simple idea but it exerted notable power with advancement in technology of eus . now eus is an indispensable procedure in the field of gastrointestinal endoscopy . in 2002 , michael v. sivak jr . wrote in his editorial that eus represents the first great advance in diagnostic ( not therapeutic ) gastrointestinal ( gi ) endoscopy in the last 200 years.1 however , morphologic examination with eus is not sufficient enough for definite diagnosis . so eus - guided fine needle aspiration ( fna ) was adopted with development of linear echoendoscope . with eus - fna , in addition , therapeutic utilization of eus - fna is now performed in various fields.2 if the chance is given , michael v. sivak jr . perhaps may rewrite his statement saying , ' eus represents the great advance in therapeutic gi endoscopy as well as in diagnostic endoscopy due to development of eus - fna . ' eus - fna has become an essential diagnostic tool and almost routinely performed to evaluate submucosal lesions and stage esophageal , gastric , rectal , and pancreaticobiliary malignancies in many hospitals . however the reported accuracies of eus - fna vary according to the locations and characteristics of the lesions . reported sensitivities of eus - fna differ vastly based on performers ' skill and equipments used . eus - fna is a difficult technique to become proficient with a prolonged learning curve.3 therefore , one of the drawbacks of eus - fna is that the results are operator - dependent and endosonographer 's experience is a crucial factor . every endosonographer who wants to improve their diagnostic yield should be diligent on getting useful technical tips from experts . in addition , to maximize clinical utilization and get better results of eus - fna , one should know definite indications where eus - fna plays an essential role in the management of patients . at present , eus - fna has become standard practice on obtaining tissues for histological diagnosis . therapeutic procedures with linear echoendoscope and eus - fna needle are also widely used.4 further development of new echoendoscope and accessories will bring expanded application of eus - fna and interventional eus in the future.5,6 currently , eus is considered to be the most accurate and safe procedure obtaining tissue samples from gut wall and structures of its vicinity.7 the purpose of this review series is to help the readers to get condensed knowledge of eus - fna from basic to advanced , especially for the commonly encountered diseases in daily practice . first , the title of jeong seop moon 's paper is ' eus - fna in submucosal lesion . ' he wrote about eus - fna , eus - guided trucut biopsy , and eus - guided fine needle biopsy in submucosal lesion together with brief mention of basic eus - fna techniques . then , i focused on additional value of eus - fna in the staging of hollow viscus cancer for the optimal choice of the treatment options according to the 7th edition of american joint committee on cancer tnm staging system . in the third paper , dr . robert h. hawes kindly shared his traditional and new ideas on eus - fna in pancreatic cystic lesions . in the last but not least paper , shyam varadarajulu generously presented their tips for getting the best results with eus - fna .
introduction of endoscopic ultrasound ( eus ) to medical practice has brought a huge change in diagnostic algorithm of many gastrointestinal diseases . addition of eus - guided fine needle aspiration ( fna ) upgraded diagnostic power of eus . in this focused review series , value of eus - fna in the diagnosis of various diseases and tips for getting the best results with eus - fna are described by four invited authors including myself . first , dr . jeong seop moon discussed about eus - fna in submucosal lesion . he also touched on basic techniques and needles of eus - fna in his article . next , i focused on additional value of eus - fna in the staging of hollow viscus cancer to optimize the treatment strategy . world 's well - known endosonographer , dr . robert h. hawes kindly presented his profound thoughts on eus - fna in pancreatic cystic lesions . dr . jayapal ramesh and dr . shyam varadarajulu shared their valuable tips for getting the best results when using eus - fna . nobody doubts now eus - fna is an indispensable procedure in gastrointestinal endoscopy . therefore , this focused review series will provide the readers with the concentrated knowledge of " what should we know about eus - fna . "
the pandemic of obesity is one of the greatest public health risks in industrialized countries . in australia , for instance , the prevalence of obesity ( body mass index > 30 ) has more than doubled in the past 25 years . bariatric surgery is now proven to be the most effective means of achieving sustainable weight loss from a condition that has been highly resistant to dietary , lifestyle and pharmacological interventions . the laparoscopic adjustable gastric band ( lagb ) even though decreasing in its popularity has in the past been among the most frequent preferred bariatric procedures in many western countries due to its success in achieving weight loss with the lowest mortality rate among all bariatric procedures . the authors report a late and rare complication of a small bowel obstruction ( sbo ) caused by the connection tube placed in an lagb 2 years earlier , interestingly with initial negative clinical and radiological findings on the patient s first presentation . this case examines a 52-year - old woman who initially presented to the emergency department with a 2-week history of vague abdominal pain . she had been privately investigated 1 week earlier for this pain with a negative computed tomography scan and gastroscopy . the patient s history did not suggest any of the usual alarm symptoms associated with lagb patients and neither did the clinical examination reveal significant findings . the impression was that of a port site infection , and she was discharged home with a script for cephalexin and follow - up in the outpatient clinic . two weeks later , she re - presented to the emergency department with a 24-hour history of severe central abdominal pain associated with vomiting and multiple episodes of loose stools . on examination , she was found to be hypotensive , tachycardic and peritonitic . the suspicion of an sbo was established by plain film radiograph appearances with multiple air fluid levels and distended bowel loops ( fig . 1 ) . computed tomography scan of the abdomen and pelvis showed the transitional zone to be in mid abdomen ( fig . interestingly , the laparoscopy identified the transition point of the sbo to be an adhesion formed between the lagb tube , the mid jejunum and the stomach forming a classic closed - loop obstruction ( fig . the rest of the small bowel was run along its entire length with no other transition point found . the adjustable gastric band was removed at the same time along with the associated port . postoperatively , she had an uncomplicated recovery and was discharged on day 4 post - admission without further sequelae . figure 1:sbo established on plain abdominal radiograph , with gastric band tubing and port also evident . figure 2:computed tomography reconstruction showing the sbo with transition zone in the mid ileum , later discovered to be caused by gastric band tubing ( t ) . figure 3:the gastric band connection tube had formed a dense adhesion to the jejunum resulting in a tight obstructive band . sbo established on plain abdominal radiograph , with gastric band tubing and port also evident . computed tomography reconstruction showing the sbo with transition zone in the mid ileum , later discovered to be caused by gastric band tubing ( t ) . the gastric band connection tube had formed a dense adhesion to the jejunum resulting in a tight obstructive band . while lagb is considered one of the safest due to its almost complete reversibility and preservation of gastrointestinal tract continuity , there are potential complications that could be life threatening that require early diagnosis and prompt surgical intervention . interestingly , most long - term follow - up studies have not highlighted sbos related to connection tubes as frequent complications or at all [ 4 , 5 ] . as more patients with lagbs present to our emergency department , it will be important to create a diagnostic algorithm to alert the various treating medical specialists to their potential complications , keeping in mind the connection tube as a causative factor . traditionally , the length of the connecting tube is preserved for two reasons : ( i ) to facilitate revision surgery in cases of infection and ( ii ) to prevent tension on the band from the anterior abdominal wall . mills et al . eluded to the long length of the tube being a causative factor for these sbo . in the course of great weight loss , the distance traversed by the tube from the stomach to the abdominal wall becomes significantly altered , thus leaving much of the length of the original tube redundant . others have addressed the redundant tube by electing to suture the tube to the anterior abdominal wall above the liver in an attempt to prevent future obstructions [ 9 , 10 ] . whether this should become a routine procedure intraoperatively or post - significant weight loss remains to be explored . the current treatment of managing patients with sbo who present after undergoing surgery is to observe with a trial of conservative management . this , however , should not be the default position for patients who present with an sbo and an adjustable gastric band in - situ . this creates a closed - loop obstruction and if not recognized and dealt with urgently can result in serious consequences . the increased specialization of surgeons in advanced laparoscopy has made a laparoscopic approach the preferred method of primary management . the presence of dilated loops of small bowel makes this technically challenging but very much one that would be within the capability of this generation of surgeons . the success of performing this operation with minimal invasive surgery would be partly dependent on the patient s systemic factors as well as the familiarity of the treating surgeon with managing complications from bariatric surgery . nonetheless , in an acute setting of paramount importance would be to relieve the obstruction regardless of being with laparoscopic or open surgery . most complications related to lagb in obesity surgery are non - life threatening , but importantly they often have a nonspecific clinical history and examination , as was demonstrated in our case report . this is only the fifth reported case of a connection tube causing an sbo in australia : as bariatric surgery continues to rise , these patients may present unannounced to any emergency department . although not a common complication , one that could easily see the safety record of lagb patients tarnished if this small subgroup of patients is not acted upon promptly by emergency departments unfamiliar lagb surgery . as such , we place importance on familiarizing oneself with single - case studies or series in bariatric complications to be able to recognize those surgical emergencies when they present often years after their primary procedure .
the laparoscopic adjustable gastric band ( lagb ) is a widely performed procedure for the morbid obesity epidemic . despite its low mortality compared with other mainstream bariatric surgeries , it is not without its complications . the authors report a late and rare complication of a small bowel obstruction in a 52-year - old woman from an lagb placed for 2 years . she was diagnosed clinically and radiologically with a small bowel obstruction . however , in the setting of an lagb , this became a closed - loop obstruction . she proceeded to an emergency laparoscopy , which revealed that the port connection tube had formed dense adhesions to the jejunum causing an obstructive band . this is only the fifth reported case in australia ; as bariatric surgery continues to rise , these patients may present unannounced to any emergency department and as such should be treated as a closed - loop obstruction with immediate resuscitative and surgical management instituted .
chorioangiomas are the most common benign tumors of the placenta originating from primitive angioblastic tissues . the newborn are rare and usually associated with tumors greater than 5 cm in diameter and consist of polyhydramnios , fetal anemia , massive edema with pleural effusion , ascites and intrauterine growth retardation . we present a case with large chorioangioma as the cause of non - immunologic hydrops fetalis with a successful outcome . the patient was a female newborn with history of polyhydramnios , symptoms of congestive heart failure and associated anemia , thrombocytopenia and coagulopathy . chorioangioma of the placenta has potentially serious perinatal risks and so the pregnancy needs to have regular surveillance . the term non - immunologic hydrops fetalis ( nihf ) defins an edematous fetus with fluid collections in some or all serous cavities that does not have erythroblastosis fetalis from isoimmunization . in 1943 dr edith potter published the first description of nihf ; now over 80 conditions are known to be associated with hydrops with very high perinatal mortality ranging from structural heart disease , fetal arrhythmias , chromosomal anomalies , intrauterine infections and larger chorioangiomas of the placenta[25 ] . in most countries with low rhesus - negative rates in the population , nonimmune causes are more prevalent ; the incidence depends on the region and also varies seasonally in relation to parvovirus b19 epidemics . we describe a newborn in whom cardiac enlargement and congestive heart failure were caused by a vascular shunt through a large placental chorioangioma , emphasizing the importance of the anatomical study of the placenta for the correct diagnosis , managing the neonatal care and evolution of the newborn . a 2.120 g preterm female newborn , product of a non consanguineous marriage was born from a primigravida by emergency cesarean section at 32 weeks of gestation because ultrasound examination revealed cardiomegaly with abnormal umbilical flow and signs of vascular fetal insufficiency . physical examination at birth revealed 1 minute apgar score 8 , anasarca , poor respiratory effort with bilateral wet rales , hepatosplenomegaly with ascites and no reflexes . the initial chest radiograph obtained 2 hours after birth showed soft tissue edema , cardiomegaly and pleural effusion compatible with congestive heart failure . laboratory data included hypoproteinemia and hypoalbuminemia , anemia ( hematocrit 31% ; hemoglobin 10.3mg / dl ) and hyponatremia ( na 125 meq / l ) , thrombocytopenia ( platelets 4110/l ) and deranged coagulation profile ( prothrombin time 19 seconds , activated partial thromboplastin time 120 seconds ) . the infant 's cardiorespiratory status gradually improved with water restriction , conventional respiratory assistance and inotropic drugs which resulted in 29 percent decrease in weight compared to her birth weight . pathology of the placenta showed edema without signs of placental infarction and a big tumoral mass measuring 1043 cm ( fig . microscopic examination revealed large fusiform vessels in a fibrotic stroma with focal edema and clusters of myxoid cells ( fig . 2 and 3 ) . placental chorioangioma : tumoral mass measuring 1043 cm placental chorioangioma : large fusiform vessels in a fibrotic stroma with focal edema and clusters of myxoid cells placental chorioangioma is a benign vascular tumor detected in 1 percent of placentas after systematic examination . most of these tumors are small and discovered only by microscopic examination and have no adverse impact on the fetus . larger tumors are rare and when above 5 cm in diameter , they are associated with serious complications . these tumors are found accidentally by ultrasound examination . in our case , the placental tumor measured more than 10 cm and led to cardiomegaly with abnormalities in the umbilical flow and signs of vascular fetal insufficiency . where the tumor is avascular , no specific complications should be expected . when the tumor is vascularized , and in particular if it contains numerous large vessels , serial ultrasound and doppler examinations are warranted to detect early features of fetal congestive heart failure . two hypotheses are proposed for formation of congestive heart failure in this condition : the left to right shunt as a result of intra tumoral arteriovenous shunting and chronic fetal hypoxia secondary to insufficient placental function or anemia and thrombocytopenia[8 , 9 ] . the detection of ultrasound findings of heart failure or suggestive signs of anemia as cardiomegaly , enlargement of the liver and abnormal umbilical vein . large placental chorioangiomas are rare and the prognosis is bad when a big tumor causes fetal hemodynamic changes with nihf , but treatment of heart failure may be promising in these newborns and complete recovery is achieved in some cases . chorioangioma of the placenta has potentially serious perinatal risks and so the pregnancy needs to have regular surveillance .
backgroundchorioangiomas are the most common benign tumors of the placenta originating from primitive angioblastic tissues . it comprises near 1 percent of placental tumors . clinical manifestations in the newborn are rare and usually associated with tumors greater than 5 cm in diameter and consist of polyhydramnios , fetal anemia , massive edema with pleural effusion , ascites and intrauterine growth retardation . we present a case with large chorioangioma as the cause of non - immunologic hydrops fetalis with a successful outcome.case presentationthe patient was a female newborn with history of polyhydramnios , symptoms of congestive heart failure and associated anemia , thrombocytopenia and coagulopathy . the pathophysiology and management of the complications of hydrops fetalis with chorioangioma are discussedconclusionchorioangioma of the placenta has potentially serious perinatal risks and so the pregnancy needs to have regular surveillance . the chance of developing complications is directly related with the tumor size .
. potential complications of anterior hip dislocations are a neurovascular injury to femoral vessels or acetabular fractures . we report a rare late complication of morel - lavallee syndrome occurring 3 weeks after an anterior dislocation of the hip in a 43-year - old male . the authors recommend surgeons to have a high index of suspicion for this syndrome and a stringent follow - up examination of the patient . they are relatively rare and constitute 10 - 15% of all dislocations [ 1 , 2 ] . the strong anterior capsule and ligament of bigelow anterior dislocations usually lead to fractures of the anterior acetabular wall or femoral head fractures or neurovascular injuries of the femoral vessels . we report a rare case of morel - lavallee lesion complicating an anterior dislocation of the hip . a 43-year - old presented to the emergency department following a history of road traffic accident . he was apparently standing on the road when a four - wheeler hit him from behind . the patient felt a snap following which he was unable to stand up on his affected limb . the patient was laid down in a supine position ; the pelvis was fixed by an orthopedic surgeon while another orthopedic surgeon performed a continuous axial extension of the right leg till the femoral head was located distal the acetabulum , which was checked by fluoroscopy . at that moment , the hip was flexed and internal rotated . the patient presented to us after 3 weeks with severe pain over the anteromedial aspect of thigh . there was a tense cystic swelling with ecchymosis over the medial side of thigh ( fig . aspiration using an 18 gauge needle yielded around 120 ml of blood mixed with clots ( fig . the patient was taught pelvic bridging exercises to avoid bed sores , isometric adductor , and gluteal , and hamstring strengthening exercises initially . he was then made to perform quadriceps strengthening and range of movement exercises of the knee joint . after 4 weeks , at the end of 6 weeks , the patient was made to weight bear . ( a and b ) ultrasound photograph showing 60 - 70 ml collection in the thigh . almost 35% of patients who suffer from anterior dislocations are in their third decade of life [ 1 , 2 ] . on the contrary , the ratio of anterior to posterior dislocations varies from 1:10 to 1:19 [ 1 , 3 , 4 ] . the rarity of these injuries is due to the strong anterior capsule and the ligament of bigelow . anterior dislocations are of two types , the superior iliac or pubic type and the inferior obturator type . in our patient , it was of the obturator variety of anterior dislocation . the mechanism of injury in anterior dislocation of the hip is usually one of the three types . finally blow to the back , when the patient is squatting seems to be a relatively more common mode of injury [ 1 , 3 , 4 ] . we suspect that the limb probably was forced into external rotation , abduction , and hyperextension at the hip joint at the time of impact . the sequence of events that occur following trauma are the neck of the femur or the greater trochanter impinges on the rim of the acetabulum , which in turn levers out the femoral head out of the acetabulum socket . the femoral head usually pushes itself out through a tear in the anterior capsule [ 1 , 3 ] . the incidence of avascular necrosis of the femoral head in an unreduced hip after 6 h varies from 0 to 22% [ 1 , 3 ] . aware of these statistics , a successful attempt was made to reduce the hip joint within 2 h of trauma under general anesthesia . the authors do not recommend a reduction of the hip joint under the effect of muscle relaxants , as more often than not the relaxation is not adequate to successfully reduce the joint . moreover , excessive manipulation is required which may lead to trauma of the retinacular or circumflex vessels . the usual complications after an anterior dislocation of the hip include neurovascular injury of the femoral vessels [ 1 , 5 ] , femoral head fracture [ 1 , 6 ] , and acetabulum fractures . morel - lavelle lesion refers to a cystic lesion secondary to blunt injury and especially in degloving injuries . the mechanism behind these injuries is when a tangential shearing force acts on the relatively mobile tissue , it gets torn away from the underlying firm muscle fascia . this creates a dead space between the two tissue planes which is filled with blood and/or lymph from the disrupted perforating vessels or capillaries [ 7 , 8 ] . morel - lavelle lesions are more often encountered over the trochanteric region or the proximal thigh ; however , it has been reported in other locations such as the lower lumbar region or the calf . furthermore , they have been associated with pelvicor acetabular fractures [ 7 , 9 ] . in our patient , during the course of the dislocation of the femoral head anteriorly , it induces a shearing force on the subcutaneous tissues which get dragged along with it . the key steps of the process have been identified ; after the initial formation of the potential blood filled space , there is evolution of this hematoma with absorption of the blood , which is replaced by serosanguineous fluid [ 7 , 10 ] . the last step in this chain of events is the formation of a peripheral fibrous capsule secondary to an anti - inflammatory reaction . the entrapment of fluid within the cyst may maintain a degree of chronic inflammation that could cause the gradual enlargement of the lesion over a long period . in our patient , we aspirated hematoma clots from the site of injury , i.e. , along the anteromedial aspect of thigh . the diagnosis was made clinically by palpating an extremely tender area on the anteromedial aspect of thigh . most authors , however , agree that the imaging modality of choice is magnetic resonance imaging ( mri ) . however , the findings of an mri may vary significantly , based on the chronicity and internal contents of the lesion . despite the rarity of this lesion , mri imaging has been used for the only so far available classification system [ 7 , 11 ] . the available treatment modalities depend on the timing of identification of a morel - lavelle lesion . if it is detected during the acute phase , percutaneous drainage , and compression may be sufficient . however in later stages , surgical aspiration or excision of the fibrous capsule may be warranted [ 7 , 12 , 13 ] . after surgery , it would be necessary to place suction drain to drain out any remnant fluid from the cyst . the differential diagnosis of a morel - lavelle lesion includes posttraumatic fat necrosis , coagulopathy - related hematoma , and posttraumatic early stage myositis ossificans [ 7 , 12 ] . in conclusion , we presented a rare case of a morel - lavelle lesion following anterior dislocation of the hip . although rare , this particular entity should be looked for as it may present as a late complication such as our case . the results are usually good . however , surgical excision is the preferable treatment modality when the lesion is advanced . atypical complications like morel - lavallee syndrome after hip dislocations present an unusual challenge during the treatment of the patient a diagnosis of morel - lavallee syndrome should be thought of as a potential late complication .
introduction : hip dislocations are serious injuries as hip joint is an extremely stable joint . it requires a significant amount of force to produce such an injury . anterior dislocations are uncommon . potential complications of anterior hip dislocations are a neurovascular injury to femoral vessels or acetabular fractures.case report : we report a rare late complication of morel - lavallee syndrome occurring 3 weeks after an anterior dislocation of the hip in a 43-year - old male . the patient presented to us with history.conclusion:morel-lavallee syndrome is a rare complication . however if diagnosed early can be successfully treated with minimal burden to the patient . the authors recommend surgeons to have a high index of suspicion for this syndrome and a stringent follow - up examination of the patient .
kleine - levine syndrome is a rare syndrome , possibly affecting one in a million and is characterized by recurrent episodes of hypersomnia , hyperphagia , behavioral and cognitive disturbances . the literature search done by arnulf et al . between 1962 and 2004 found only 186 cases in literature . it mainly affects adolescent males between ages of 10 and 25 years but cases have also been reported in females and tends to be of longer duration . each episode can last from days to weeks and the individuals are asymptomatic in between the episodes . recurrent episodes of hypersomnia were first described by willi kleine in 1925 and later on max levine described the association between hypersomnia and hyperphagia in 1929 and 1936 . according to american academy of sleep medicine the pathophysiology remains unclear but many etiological factors have been described . in approximately 50% of cases , the syndrome occurs following a specific factor such as viral upper respiratory tract infection , epstein barr virus , herpes zoster infection , encephalitis , head trauma and ingestion of drugs . recurrent episodes occur every few weeks to months and the condition can last for a decade or even more before spontaneous recovery . a previously well 14-year - old boy presented acutely 10 days following a viral throat infection with excessive sleepiness , tiredness , abnormal behavior and hypersexuality . he was sleeping for almost 22 hours per day and only waking up for eating / drinking and toilet needs . on examination his glasgow coma scale ( 15/15 ) and rest of the neurological examination including fundoscopy and other systemic examination were completely unremarkable . detailed investigations including electrolytes , liver function tests , copper , ceruloplasmin , csf ( cerebro spinal fluid ) , lactates ( blood and csf ) , ammonia , virology screen ( blood and csf ) and autoimmune screening including anti - n - methyl - d - aspartate receptor , antivoltage - gated potassium channel ( vgkc ) and anti - glutamic acid decarboxylase antibodies were negative his excessive sleepiness gradually improved together with his altered behavior in about 2 weeks after presentation . hypersexuality became more overt during this phase manifesting as fiddling with his genitalia , putting his pant down in front of parents and even inappropriate behavior towards female staff . all these symptoms completely disappeared 3 weeks after his presentation and he started attending , initially part - time and later full time as before . parents reported that he is back to his usual self and did nt have any decline in academic performance . however , 6 weeks later he was readmitted with exactly similar presentation and again only positive result being diffuse non - specific slowing of eeg . rest of investigations including csf , virology screen and autoimmune antibody screen were again normal . interestingly his recovery was also similar and he was completely back to his normal self in about 3 weeks time . on second admission after ruling out infectious , metabolic and auto - immune we gave him a trial of modafinil ( cns stimulant ) , however due to gastrointestinal side effects he could not tolerate that . at his 6-month follow up , he hasnt reported any further episodes and is completely normal neurologically . his behavior at school and home kls has a benign course of action with spontaneous resolution of symptoms though patient can have recurrent symptoms over period of a decade . the median duration of disease is 8 years and the attacks tend to be of less intensity toward the end of the disease course . diagnosis of kls can be very difficult as there is no single symptom that can positively make the diagnosis . rather kls is a diagnosis of exclusion and the patient needs a long list of investigations to eliminate other conditions that mimics the symptoms of kls . to diagnose kls , a patient can experience other symptoms like : ( 1 ) hypersexual behavior ; ( 2 ) hyperphagia with compulsive eating behavior ; ( 3 ) cognitive and behavior disturbances including confusion , irritability , aggressiveness , hallucination and delusion . most of the patients are misdiagnosed as having encephalitis , narcolepsy , epilepsy , psychosis and mood disorders . in a systemic review of 186 cases in the literature nearly half of the patients experienced hypersexual behavior and only few of the patients had decline in academic performance secondary to school absences . our patient had hyperphagia and hypersexuality but did nt have any decline in school performance . various treatment strategies including stimulants like methylphenidate , modafinil , d - amphetamine , ephedrine , methamphetamine , amphetamine , etc . , have been used to treat sleepiness in the literature . kls should also be differentiated from narcolepsy which is a chronic sleep disorder characterized by excessive sleepiness and sleep attacks at inappropriate times and has certain symptoms which can help physician in the diagnosis . the other differential which is worth considering is kluver bucy syndrome which mainly presents with hyperphagia and hypersexuality but hypersomnia is not its characterized feature . early diagnosis helps in starting medical treatment and patient education that helps in alleviating the anxiety of family members , school teachers and work colleagues . kls is a clinical diagnosis and should be considered in a patient with episodes of hypersomnia .
to describe the clinical presentation of kleine - levin ( sleeping beauty ) syndrome in a child , who presented with recurrent episodes consistent with encephalopathy , associated with excessive sleepiness , cognitive and behavioural disturbance and hyper sexuality . 14 years old boy presented acutely with excessive tiredness , sleeping excessively , abnormal behaviour and hypersexuality following a viral throat infection . on examination he was sleepy but easily arousable . his gcs ( 15/15 ) and rest of the neurological examination including fundoscopy and other systemic examination was completely unremarkable . all his initial investigations including electrolytes , lfts , csf , virology screen and mri brain scan were normal . detailed autoimmune screening was also negative . eeg showed non - specific diffuse slowing consistent with encephalopathy . his excessive sleepiness gradually improved together with his altered behaviour in about two weeks after presentation . hyper sexuality became more overt during this phase . all these symptoms completely disappeared three weeks after his presentation and he attended school as before . he was readmitted six weeks later with exactly similar presentation and again only positive result being diffuse non - specific slowing of eeg . his recovery was also similar and he was completely back to his normal self in three weeks time . his recurrent symptoms were consistent with kleine - levin syndrome ( kls) or sleeping beauty syndrome. kls is a rare disorder which mainly affects adolescent males . common symptoms include hypersomnia ( 100% ) , cognitive changes ( 96% ) , eating disturbances ( 80% ) , hypersexuality , compulsions , and depressed mood . the syndrome usually lasts for 8 years , with on an average seven episode of 10 days each recurring every 3.5 months . it is most frequently precipitated by infections and somnolence decreases using stimulants in nearly 40% of cases .
a 74-year - old female patient visited our department due to pain in the left lower limbs for 6 months . the patient complained of a continuous dull pain along the left 5 lumbar vertebral ( l5 ) and 1 sacral vertebral ( s1 ) dermatomes . the pain level was 9 on the numeric rating scale ( nrs ) from 0 ( no pain ) to 10 ( worst pain imaginable ) . the pain was exacerbated by walking and was alleviated by sitting or lying in bed . physical examination revealed sensory loss of 50% along the left l5 and s1 dermatomes without weakness . on deep tendon reflex ( dtr ) the straight leg raise test showed a positive result at 45 in the left lower limb . magnetic resonance imaging ( mri ) of the lumbar spine showed a large cyst in the ventrolateral epidural space of the l5 level that communicated with the adjacent 4 lumbar and 5 lumbar intervertebral disc ( fig . the cyst showed high signal intensity on t2-weighted images and low signal intensity on t1-weighted images ( fig . 2 ) . after addition of contrast , the cystic mass had a thick wall with rim enhancement ( fig . the patient was diagnosed with radiculopathy due to a discal cyst . left l5 and s1 selective transforaminal epidural blocks were performed using 20 mg triamcinolone and 2 ml of 0.3% mepivacaine for the left lower limb radiating pain . pregabalin 150 mg , tramadol 50 mg , and a muscle relaxant were administered daily for 2 weeks . two weeks later , the patient 's left lower limb radiating pain had been somewhat alleviated , as judged by the decrease in the pain score on the numeric rating scale ( nrs ) to 6 following the treatment . l5 and s1 transforaminal epidural blocks were then repeated using 40 mg triamcinolone and 2 ml of 0.3% mepivacaine . one month after the 2 procedure , the left lower limb radiating pain was rated a 5 by the patient on the nrs . therefore , we once again repeated the l5 and s1 transforaminal epidural blocks using 40 mg triamcinolone and 2 ml of 0.3% mepivacaine , and the same medications were prescribed . two months after the 3 transforaminal epidural blocks , the patient 's leg pain was rated a 2 on the nrs , although the hypesthesia persisted . however , it has been suggested that discal cyst results from focal degeneration of the intervertebral disc with fluid production , and that discal cyst develops through deficits in hematoma resorption . the clinical presentation of discal cyst is unilateral radiculopathy similar to that caused by intervertebral disc herniation . it is difficult to differentiate between discal cyst and intervertebral disc herniation through history taking and physical examination of patients . the typical finding for a discal cyst is a round to oval extradural mass lesion with high signal intensity on t2-weighted images and low signal intensity on t1-weighted images . after addition of gadolinium medium , the surrounding rim of the cyst is enhanced . in the present case , a connection between the cyst and the corresponding disc is the key to differentiate discal cysts from intervertebral disc herniation . in the present case , the cyst was observed to communicate with the adjacent 4 lumbar and 5 lumbar intervertebral disc . surgical removal of the cyst [ 1 - 4 ] and ct - guided aspiration of the cyst with steroid injection are believed to be effective treatment options for discal cyst . , the natural history of discal cysts is still unknown , and further evaluation is needed . i felt that radiculopathy due to discal cyst could be treated similarly to hnp ( herniated nucleus pulposus ) , and conservative treatment such as transforaminal epidural block should be the first choice . if transforaminal epidural block is unsuccessful in reducing radiating pain , surgery or ct - guided aspiration of the cyst with steroid injection should be considered . therefore , i performed several left l5 and s1 transforaminal epidural blocks to relieve the radiating pain . the patient refused to undergo follow - up mri due to her symptomatic improvement and the financial burden . therefore , i were unable to determine whether the discal cyst regressed spontaneously as in previous cases [ 8 - 11 ] , or whether the discal cyst persisted but the radiating pain was relieved by the transforaminal epidural blocks . in conclusion , if patients complain of radiating pain , the possibility of a discal cyst can not be excluded . for the treatment of discal cyst
discal cyst is an intraspinal cyst with a distinct communication with the corresponding intervertebral disc . it is a rare condition and could present with radiculopathy similar to that caused by lumbar disc herniation . we present a patient with a large discal cyst in the ventrolateral epidural space of the 5th lumbar vertebral ( l5 ) level that communicated with the adjacent 4th lumbar and 5th lumbar intervertebral disc , causing l5 radiculopathy . we alleviated the radiating pain with selective transforaminal epidural blocks .
takayasu arteritis ( ta ) is a chronic inflammatory disease involving large vessels like aorta and its major branches . it presents to clinician with features of arm claudication , stroke , asymmetrical pulse , or asymmetrical blood pressure in both limbs . digital gangrene may be occurred secondary to various systemic diseases like diabetes , thrombophilic states , vascular embolism , and medium and small vessel vasculitis or infections . through evaluation to diagnose the exact etiology and timely intervention is needed to prevent progression of the gangrene which at the time can be life - threatening . we are reporting a case of a 26-year - old female patient presented with right little finger and index finger gangrene , ultimately diagnosed as ta and responded dramatically to treatment . though rare , ta should be taken in the differential diagnosis for work up in a case of digital gangrene and it necessitates more studies in this field to uncover the exact pathophysiology for such presentation . takayasu arteritis ( ta ) is inflammatory vasculitis of unknown origin involving large vessels and its major branches , most frequently in young women . it occurs more commonly in japan , china , india , and southeast asia ; the disease is also prevalent in mexico . vascular insufficiency from stenosis , occlusion , or aneurysm is the common mode of presentation . these are arm claudication , absent pulse , carotid bruit , hypertension , asymmetric arm blood pressure , carotidynia , lightheadedness , stroke , and aortic regurgitation . constitutional symptoms like fever , malaise , weight loss , and night sweats are also commonly present . our patient who is suffering from takayasu 's arteritis according to american college of rheumatology criteria has digital gangrene of both hands , a rare manifestation of ta . a 26-year - old female patient came to our hospital with history of pain and black discoloration of index and little fingers of right hand for last 1 month with history of intermittent claudication of right arm for which she had difficulty in doing her household work . there was no history of fever , joint pain , oral ulcer , photosensitive rashes , raynaud 's phenomenon , chest pain , palpitation , trauma , pain abdomen , pedal swelling , headache , visual abnormalities , chronic drug intake , or addiction . pulse rate was 88 beats / min , regular with brachial and radial pulse on right side found to be feeble compared to left side . blood pressure was 94/68 mmhg in the right arm , 124/78 mmhg on left arm , and 138/84 and 144/80 mmhg in right and left leg , respectively . there was a bruit audible over infraclavicular region on the right side without any renal or abdominal bruit . local examination of left hand showed , there was a dry gangrene with a clear cut line of demarcation between normal and gangrenous tissue [ figure 1 ] . dry gangrene of left little and ring finger of left hand further investigational workup revealed patient 's hemoglobin was 10.4 g / dl , total leukocyte count of 8,600/mm with normal differential count and platelet count . erythrocyte sedimentation rate ( esr ) was 104 mm / h withhigh sensitive c - reactive protein ( hscrp ) was 8 mg / l . patient had fasting and postprandial blood glucose of 98 and 116 mg / dl , respectively . liver , renal function , and coagulation profile were normal with lipid profile study showed total cholesterol of 98 mg / dl , triglyceride of 123 mg / dl , and low density lipoprotein ( ldl ) of 45 mg / dl . antinuclear antibody ( ana ) , perinuclear antineutrophil cytoplasmic antibody ( p - anca ) , cytoplasmic antineutrophil cytoplasmic antibody ( c - anca ) , anti-2 gp1antibody , anticardiolipin antibody , lupus anticoagulant , hepatitis b surface antigen , hepatitis c virus , and human immunodeficiency virus ( hiv ) status were negative . cardiac evaluation with electrocardiogram and two - dimesnional ( 2d ) echocardiography was absolutely normal . carotid doppler study showed narrowing of lumen of bilateral internal carotid artery with normal carotid intima - media thickness ( cimt ) without any evidence of plaque . invasive peripheral conventional angiography revealed stenosis in the left subclavian artery proximal to the origin of the vertebral artery and right common carotid artery [ figure 2c e ] . according to angiographic classification , patient had type i ta . bilateral renal , common iliac artery showed normal pattern [ figure 2a and b ] . ( b ) digital subtraction peripheral angiography showing normal bilateral renal artery . ( c ) digital subtraction peripheral angiography showing total occlusion of right subclavian artery.(d ) digital subtraction peripheral angiography showing narrowing of left subclavian artery . ( e ) digital subtraction peripheral angiography showing narrowing of right common carotid artery and normal vertebral artery patient was put on prednisolone 1mg / kg body weight and wound debridement done under surgical department . the common causes of digital gangrene are atherosclerosis ; connective tissue diseases like systemic lupus erythematosus and scleroderma ; antiphospholipid antibody syndrome ; small to medium vessel vasculitis like polyarteritis nodosa ( 28 - 58% ) , microscopic angitis ( 15 - 20% ) , granulomatosis with polyangitis ( 30 - 40% ) , and buerger 's disease . dislodgement of vegetations in case of infective endocarditis and mural thrombus during episodes of atrial fibrillation or otherarrhythmiasare also responsible . for diagnosis of ta american college of rheumatology criteria for diagnosing takayasu 's arteritis the patient had gangrene involving digits of the left hand . till now , few cases of digital gangrene involving lower limbs and only one case of digital gangrene involving hand were reported worldwide in ta . there was no history of raynaud 's phenomenon , rash , arthritis , oral ulceration , and hemoptysis . antiphospholipids antibody syndrome was excluded as anticardiolipin antibody , lupus anticoagulant , and beta-2 glycoprotein - i antibody assay were negative . angiographic classification of takayasu 's arteritis digital gangrene in upper limb is very uncommon as there is abundance of collaterals and rarity of atherosclerosis . so , this patient who was suffering from ta with digital gangrene is a very rare manifestation .
context : takayasu arteritis ( ta ) is a chronic inflammatory disease involving large vessels like aorta and its major branches . it presents to clinician with features of arm claudication , stroke , asymmetrical pulse , or asymmetrical blood pressure in both limbs . digital gangrene may be occurred secondary to various systemic diseases like diabetes , thrombophilic states , vascular embolism , and medium and small vessel vasculitis or infections . through evaluation to diagnose the exact etiology and timely intervention is needed to prevent progression of the gangrene which at the time can be life - threatening . digital gangrene as an initial presentation in ta is very rare . in medical literature , there are only few case reports have been reported.case report : we are reporting a case of a 26-year - old female patient presented with right little finger and index finger gangrene , ultimately diagnosed as ta and responded dramatically to treatment.conclusion:though rare , ta should be taken in the differential diagnosis for work up in a case of digital gangrene and it necessitates more studies in this field to uncover the exact pathophysiology for such presentation .
central granular cell odontogenic tumor ) cgcot ) of the jaw , which was previously known as central granular cell odontogenic fibroma or granular cell ameloblastic fibroma , is an exceedingly rare benign odontogenic neoplasm [ 1 , 2 ] with 35 reported cases in the literature . some of these lesions have very few clinical features so they are often found accidentally in the routine radiographs . the extension of some types of this lesion and the absence of capsule around them has made resection the necessary treatment for these cgcots ; therefore , the precise and early diagnosis of these lesions could refrain their extreme extension . cgcot is usually seen in the posterior areas of the female mandible in the fifth decade of life . in most reported cases , they have a well - defined unilocular radiolucent pattern . as very few studies have focused on the cone - beam ct features of cgcot , here we report a case of an asymptomatic cgcot in a 16-year - old girl with descriptions on cbct findings . in october 2010 , a 16-year - old girl was referred to our maxillofacial radiology clinic for radiographic examination for orthodontic treatment . accidentally , in the panoramic radiograph , a unilocular mixed lesion was detected in the left mandibular angle . the posterior borders of the lesion were poorly - defined ; whereas , the anterior borders were ill - defined . in the panoramic view , intra - lesion calcifications were evident ( figure 1 ) . in the panoramic radiography and lateral cephalography , a mild expansion was observed in the posterior and inferior borders of the mandibular angle ( figure 2 ) . she did not experience pain in the region , and the lesion was non - tender on palpation . on clinical examination , a slight swelling was observed in the left mandibular angle . on intra - oral examination , the overlying mucosa of the region was smooth and of normal color . to localize the intraosseous lesion and perform further investigations on the effects of the lesion on the surrounding structures , we performed cone beam computed tomography . on cbct examination , a multilocular lesion with coarse septa , without sclerotic borders was observed . the cortical borders of the mandible and follicular space of the unerupted left third molar were normal . no displacement and root resorption was found on the teeth adjacent to the lesion ( figure 3 ) . moreover , no displacement in the inferior alveolar nerve canal position was noted , but the cortical borders of the canal were thinned ( figure 4 ) . based on age , clinical behavior , and radiographic findings , the differential diagnoses of fibrous dysplasia and desmoplastic ameloblastoma were made . histologic examination of the lesion revealed sheets and islands of large eosinophilic cells with abundant granular cytoplasm with small islands of odontogenic epithelium ( figure 5a c ) . foci of dystrophic and cementum - like calcifications were detectable in the lesion ( figure 5d ) . the fibrous stroma contained fibroblasts , and in the decalcified sections , bone trabecules were evident ( figure 5a and b ) . resection of the lesion was carried out to treat the patient . for mandibular reconstruction , allogen graft from the fibula the patient is on follow - up periodic check and there has not been any radiographic evidence of recurrence on the follow - up radiograph after 2 years of operation ( figure 7 ) . cgcot of the jaw is an exceedingly rare , benign odontogenic neoplasm with 35 reported cases in the literature until 2012 . a historical controversy regarding its nomenclature and histogenesis is evident in previous literature [ 1 , 4 ] . [ 1 , 58 ] . this lesion was later termed as granular cell ameloblastic fibroma by couch et al . . most authors , including gardners and brannon [ 1 , 11 ] introduced cgcot as a separate entity from central odontogenic fibroma ( cof ) . cgcot was not considered as an entity in the recent who classification of head and neck tumors published in 2005 . this evolution in nomenclature and the concurrent debate on the histogenesis of this lesion was due to the uncertain histogenesis of the granular cells , i.e. , it is not clear whether these cells are neoplastic , reactive , or metabolic in nature . on electron microscopy , white et al . found numerous lysosome - like particles that were similar to those previously described for granular cell tumor . , the granules found in this lesion are not specific to a particular cell line . although cgcot is more often reported in patients older than 40 years at the time of diagnosis , there has only been one case of cgcot reported by regezi ( 1978 ) in a 16-year - old boy similar to the age range of our case . cgcot is usually seen in the mandible and most often in the premolar and molar regions in previously reported cases [ 1 , 3 , 7 , 13 , 14 ] . in the current case , however , the lesion was located posteriorly in the mandibular angle region rather than in the premolar - molar areas . cgcot is usually presented as a painless swelling according to the report of gesek et al . . however , cortical expansion , teeth displacement , and locally aggressive behavior have been reported in few cases . in the present case , mild expansion of the mandibular angle was not obvious due to coverage of the masseter muscle in the region , which led to accidental detection of the lesion . however , the mild expansion was detectable on radiographic examination . from a radiographic point of view , in previous cases , most lesions were reported as radiolucent with sclerotic borders . the cases reported by gesek et al . , machado de sousa et al . , reichart et al . and brannon et al . the multilocularity of this lesion might be related to the young age of the patient or the posterior location of the lesion , although this hypothesis warrants further investigation . according to the report by ardekian et al . they reported that the intralesional calcifications were microscopically observed in 50% of the cases , but they were not detectable in the radiographic view because of the scarceness of these calcifications . in the present case , the mixed pattern and calcifications were evident in the panoramic radiograph , but in the further evaluations by means of cbct , otherwise specified . it may be used to determine the extent and condition of the internal structure of odontogenic lesions . cbct can allow the three - dimensional evaluation and determination of the amount of expansion and extension of the odontogenic lesions . compared with ct - scan , the relatively low dose and high speed image acquisition of this technique results in the widespread application in dentistry . despite the mentioned benefits , low contrast resolution prevents appropriate detection of soft tissue , which can be detected in ct scan images as well . in the present case , the cbct evaluation of the lesion revealed that this mixed pattern is actually due to the multilocular pattern and very coarse septa of the lesion resembling calcifications in the panoramic view . this concept highlights the importance of using new techniques such as cbct or ct - scan , which are superior in better description of the lesions . . different behaviors of this lesion could be related to the age of the patients . it seems that use of three - dimensional techniques could be useful in better perception of the radiographic views and behavior of the lesion .
central granular cell odontogenic tumor ) cgcot ) of the jaw is an exceedingly rare benign odontogenic neoplasm with 35 reported cases in the literature . among these , very few studies have focused on the cone - beam ct features of cgcot . here , we report a case of an asymptomatic cgcot in a 16-year - old girl and focus on the cone - beam ct features . only 36 cases of this lesion , including this one , have been reported so far . the case presented is of special importance due to the young age of the patient , the posterior location of the lesion and the multilocular pattern in the cone beam ct images
researchers within the lipid droplet field have debated nomenclature confusion regarding the multiple names of the various pat proteins . lipid droplets : metabolic consequences of the storage of neutral lipids , with encouragement by the mouse genomic nomenclature committee ( mgnc ) and consultation with the human genome organization genomic nomenclature committee ( hgnc ) . the nomenclature recommendations were agreed upon without dissent ( table 1 ) . nomenclature based upon variations of the most obvious terms , pat , lsd , etc . for example , lsd also designates lysine specific demethylase ; these lsd complexes regulate histone methylation and dynamic aspects of chromatin structure and transcriptional control ( 25 ) . we , thus , selected perilipin as the founding root term based on its precision and elegance , with the gene symbols plin and plin , for the murine and human genomes , respectively . the mgnc and hgnc have approved this nomenclature . following the plin / plin gene symbol , each family member is numbered sequentially in the order plin1 for perilipin , plin2 for adfp , plin3 for tip47 , plin4 for s3 - 12 , and plin5 for lsdp5 . both new and old terminologies ( e.g. , plin2/adfp ) may be of use in the short - term , but we strongly discourage continuous reference to additional alternatives , such as adipophilin , pp17 , pat1 , oxpat , mldp , and lsd . as standard for human and rodent nomenclature , human gene symbols are fully capitalized , whereas for mouse , only the first letter is upper case . for both systems , gene symbols are italicized , whereas full - length gene names are nonitalics , lower case . the murine plin1 gene organization is the most fully characterized of the plin gene family ( 1 ) . the mrna splice variants are predicted to encode four distinct proteins , previously termed perilipin a , b , c , and d ; three of these proteins have been confirmed ( 5 , 6 ) . lower case letters will now denote alternative protein forms plin1a , plin1b , plin1c , and plin1d , with plin1a , plin1b , plin1c , and plin1d as their respective mrnas . sequence similarity argues strongly for orthologs of plin1 , plin2 ( adfp ) , and plin3 ( tip47 ) in osteichthyes and amphibia . multiple plin family members are present in insecta , and one is found in dictyostelium ( 1 , 19 ) . these nonvertebrate proteins clearly associate with lipid storage droplets , even when expressed in mammalian cells ( 19 ) , but their current nomenclatures derive from an lsd protein root ( e.g. , lsd or lsdp ) . nonetheless , the common exon / intron gene organizations among the murine plin1 , murine plin2/adfp , and drosophila lsdp-1 genes indicate an ancient evolutionary origin for the entire plin gene family ( 1 ) . in addition , the insect proteins exhibit lipolytic regulation of lsds that are comparable to that of mammalian plin family members ( 2628 ) . the plin - based nomenclature is sufficiently flexible and unifying to allow inclusion of all members of this evolutionarily diverse gene family and we encourage all genome annotating organizations to consider the use of plin in their current and future nomenclatures . hence , the single dictyostelium plin member lsd1/ddlsd ( ddb_g0279791 ) would now be cross - referenced as plin . as with other orthologous genes , particularly in distantly related species , identical nomenclature ( e.g. , plin1 ) would not imply a common function . the defining characteristics of plin proteins include a conserved pat - domain ( 1 ) and an 11-mer repeating helical organization ( 29 ) . although , the extreme n - terminal 100 amino acids are the most conserved in plin1 , 2 , 3 , and 5 proteins , similarity extends through 250 amino acids . plin4/s3 - 12 is somewhat distinct , with a highly expanded ( > 75n ) 11-mer repeat region . although structurally similar 11-mer motifs ( 29 ) are found in other lipid - associated proteins ( e.g. , apolipoproteins and -synuclein ) , their sequences are unrelated to the plin family and are not classified as pat domains . although the lipid droplet - binding protein cidec / fsp27 ( and related family members ) shares some limited sequence similarity to plin1 , it has an unrelated genomic organization ( 30 , 31 ) . additional lipid storage droplet proteins , such as the plant oleosins , hepatitis c virus core protein , caveolins , and mettl7a / b , which possess other domains for lipid interaction ( 3235 ) , are also not members of the plin family . thus , the plin nomenclature should not be applied to proteins within a functional category defined by constitutive or transient localization to lipid droplets , but reserved for proteins with an evolutionary relationship that yields conservation of the primary amino acid sequence . we also recognize that plin - family member functions may not be solely restricted to their lipid - binding character . in summary , we recommend adoption of plin nomenclature for lipid droplet binding proteins within the perilipin gene family . this nomenclature will reduce confusion over the multiplicity of names for the individual members of the family .
the pat family of proteins has been identified in eukaryotic species as diverse as vertebrates , insects , and amebazoa . these proteins share a highly conserved sequence organization and avidity for the surfaces of intracellular , neutral lipid storage droplets . the current nomenclature of the various members lacks consistency and precision , deriving more from historic context than from recognition of evolutionary relationship and shared function . in consultation with the mouse genomic nomenclature committee , the human genome organization genomic nomenclature committee , and conferees at the 2007 faseb conference on lipid droplets : metabolic consequences of the storage of neutral lipids , we have established a unifying nomenclature for the gene and protein family members . each gene member will incorporate the root term perilipin ( plin ) , the founding gene of the pat family , with the different genes / proteins numbered sequentially .
inflammatory myofibroblastic tumor ( imt ) is a distinctive pseudosarcomatous lesion that occurs in the viscera and soft tissue of children and young adults . it was first described in the lung , however , extrapulmonary imts have also been described in the mesentery , liver , head and neck , and heart . the pathogenesis of imt remains unclear , although various allergic , immunologic and infectious mechanisms have been postulated . recently , new imaging modalities for diagnosis have been improved and intestinal tumor is becoming detectable . especially , abdominal ultrasonography was described as a useful and non - invasive technique to demonstrate intestinal tumors . the previously described cases of primary intestinal imts were in the form of case reports or small series [ 1 , 3 ] . here we present the case of a primary intestinal imt in an elderly man and specially focus on the diagnostic modalities for this disease . a 79-year - old man presented to our hospital with a 3-month history of nausea , constipation and abdominal distention . abdominal ultrasonography revealed a 5-cm mobile , well - defined , elastic hard mass in the left upper quadrant of the abdomen . multiple parallel lines provided the so - called sandwich appearance , and a 3.5-cm solid mass was observed in the presenting part ( fig . in addition , color doppler ultrasonography showed abundant blood flow surrounding the mass . computed tomography ( ct ) revealed an enhanced homogeneous mass and slight dilatation of the oral side of the intestine ( fig . 2 ) . based on these findings , we diagnosed the patient with intussusception caused by an intestinal tumor . we performed elective laparotomy that revealed an ileal intussusception located 80 cm proximal to the end of the ileum . the intussusception was found to be caused by an ileal tumor located 70 cm from bauhin 's valve . we partially resected the ileum maintaining a 10-cm margin from the tumor , and ileo - ileal anastomosis was performed in an end - to - end manner ( fig . the tumor was a homogeneous solid mass , 3.5 2.5 cm in diameter , and was well - circumscribed and grayish - white in color ( fig . histopathological examination of the tumor revealed proliferation of spindle - shaped cells with infiltration of plasma cells and lymphocytes ( fig . imt is a tumor composed of differentiated myofibroblastic spindle cells usually accompanied by numerous plasma cells and/or lymphocytes . it was originally described in the lungs ; however , extrapulmonary imt is known to occur in any anatomic location regardless of age . . showed that imt developed at a mean age of 9.7 years , and in 36 of 84 cases ( 43% ) , imts arose from the mesentery and omentum . on the other hand , only 1 case ( 1.2% ) exhibited an imt of ileal origin . to our knowledge , our patient is the first reported case aged > 70 years with imt . recent studies showed that approximately 30% of all small bowel intussusceptions are caused by malignancy , whereas the remainder are caused by benign lesions ( 60% ) or are idiopathic ( 10% ) [ 5 , 6 ] . intussusception is not easily diagnosed in adults because patients usually present with non - specific , vague symptoms such as abdominal pain , nausea and vomiting . ct is the most commonly used imaging technique , but sonography is also appropriate and useful in the diagnosis of bowel intussusception . ultrasonography facilitates examination in all planes in a non - invasive manner and in real time . these advantages of ultrasonography prove to be beneficial in emergencies . in our case , ultrasonography enabled an accurate and rapid diagnosis at the first examination . the classic appearance of an intussuscepted bowel on an ultrasonographic image in a transverse plane is called the the longitudinal appearance of intussusception is usually viewed as multiple parallel lines , which is termed as the sandwich appearance or pseudo - kidney sign. target or sausage shapes are observed both on ct as well as on ultrasonographic images . diagnosis of intussusceptions by ct enables the differentiation between non - pathologic and pathologic intussusceptions . the presence of a mass imaged as a lead on ct is indicative of a neoplasm and the need for operative treatment . in conclusion , the possibility of imt should be considered during differential diagnosis in elderly patients with a small intestinal tumor .
inflammatory myofibroblastic tumor ( imt ) , which usually affects young adults and children , is a solid neoplastic mesenchymal proliferation composed of myofibroblastic spindle cells admixed with inflammatory infiltrates . numerous extrapulmonary sites of these tumors have been found , but intestinal imt is rare , especially in elderly patient . its diagnosis is recognized as difficult because the patients usually do not have a specific symptom . here , we present the case of a 79-year - old man with an imt that caused small intestinal intussusception , which was diagnosed by abdominal ultrasonography . we review the literature on imt and specially focus on the diagnostic modalities for this disease .
to describe a case of choroidal neovascularization ( cnv ) following photorefractive keratectomy ( prk ) to correct myopia . we performed prk in both eyes of a 20-year - old girl to correct myopia . refractive error was 4.75 2.25 5 in the right eye and 5.00 1.25 180 in the left eye . metamorphopsia was noticed by the patient in the right eye one month after the surgical procedure . fluorescein angiography and optical coherence tomography ( oct ) were performed which were compatible with cnv . after three monthly intravitreal bevacizumab injections , sub - retinal hemorrhage and intraretinal fluid resolved , but subretinal scar remained without any visual acuity improvement . also , refractive surgeons should consider cnv development in cases with visual compliant or metamorphopsia following prk . there are some reports of cnv after laser in situ keratomileusis ( lasik ) surgery , but to our knowledge , only one case of cnv following prk has been reported . here prk was performed in both eyes of a 20-year - old girl to correct myopia . the procedure was performed at refractive surgery center of baqiyatallah hospital , tehran , iran . a preoperative examination including refraction and best corrected visual acuity ( bcva ) measurements , slit - lamp examination , intraocular pressure ( iop ) measurement , and fundoscopy with dilated pupil was performed by the surgeon and reported as normal . preoperative refractive error was 4.75 2.25 5 and 5.00 1.25 180 in the right and left eye , respectively . alcohol - assisted corneal epithelium debridement was done , and mitomycin c was used at the end of the procedure . one month after surgical procedure , metamorphopsia developed in the right eye , and visual acuity reduction happened 3 months later . the patient was referred to retina clinic of the baqiyatallah hospital , tehran , iran 4 months after prk . on fundus examination there was an area of subretinal hemorrhage in the para foveal area associated with macular edema of the right eye . fluorescein angiography showed para foveal leakage ( fig . 1 ) , and optical coherence tomography ( oct ) revealed a hyper - reflective sub - retinal material associated with intraretinal fluid and pigment epithelial detachment ( fig . although the effect of intravitreal anti vascular endothelial growth factor ( anti - vegfs ) in the cases of cnv following excimer laser refractive surgery has not been completely understood , we used 1.25 mg intravitreal bevacizumab ( avastin ; genetech inc , south san francisco , california , usa ) for treatment . after 3 monthly intravitreal injections , sub - retinal hemorrhage resolved and intraretinal fluid improved , but sub - retinal scar developed ( fig . 3a and b ) . one month after the 3rd intravitreal injection , the bcva was 20/400 , and no visual acuity improvement was observed . high myopia has been reported as a major cause of cnv formation ( 62% ) in young patients . in the present report neo et al reported 3 cases of unilateral cnv after lasik for high myopia . among their patients , the mean spherical equivalent was 11.42 d ( range from 6.75 to 20.00 d ) . they used a combination of intravitreal ranibizumab and photodynamic therapy ( pdt ) with verteporfin for the treatment . the mean bcva was 0.44 logmar and 0.17 logmar at presentation and after treatment , respectively . saeed et al described a case of cnv after lasik for correction of low myopia . the refractive error in their patient was 2.75 d in both eyes , and cnv developed in one eye 3 months after surgery . although loewenstein et al described macular hemorrhage in three patients with high myopia ( 13.00 to 20 d ) after prk , to date , there has only been one report of cnv after prk . ruiz - mareno and colleagues evaluated the incidence of cnv in 5963 eyes undergoing prk for the correction of myopia . cnv developed in one eye after correction of 12.00 d of myopia 26 months after prk . the same researchers reported one case of cnv after the same number of prk surgeries . progressive elongation of the axial length and degenerative changes of the choroid in highly myopic eyes may cause the linear breaks in bruch 's membrane which are called lacquer cracks . lacquer cracks may be related to development of cnv.9 , 10 myopic changes and lacquer cracks were not observed in the fundus examination of our case . iop elevation induced during lasik surgery may result in posterior segment pathologies and may be a factor for cnv formation . iop elevation does not happen during prk , and this may be the cause of lower reports of cnv formation following prk compared with lasik . acoustic shock waves produced by the excimer laser are another factor proposed to play a role in the cnv formation after both prk and lasik . cnv is not common in a 20-year - old patient without obvious myopic chorioretinal changes and lacquer cracks . also , our patients visual symptoms developed only 1 month after prk surgery . therefore , prk may play a role in the development of cnv in our case . since idiopathic and myopic cnv can not be ruled out in our case , the cause - and - effect relationship between the surgical procedure and cnv formation is not established . in conclusion also , refractive surgeons should consider cnv development in cases with visual compliant or metamorphopsia following prk .
purposeto describe a case of choroidal neovascularization ( cnv ) following photorefractive keratectomy ( prk ) to correct myopia.methodswe performed prk in both eyes of a 20-year - old girl to correct myopia . refractive error was 4.75 2.25 5 in the right eye and 5.00 1.25 180 in the left eye.metamorphopsia was noticed by the patient in the right eye one month after the surgical procedure . the patient was referred 3 months later when visual loss happened.resultsfluorescein angiography and optical coherence tomography ( oct ) were performed which were compatible with cnv.after three monthly intravitreal bevacizumab injections , sub - retinal hemorrhage and intraretinal fluid resolved , but subretinal scar remained without any visual acuity improvement.conclusionsit seems that cnv may occur after prk in myopic eyes . also , refractive surgeons should consider cnv development in cases with visual compliant or metamorphopsia following prk .
primary hyperparathyroidism ( phpt ) syndrome is an endocrine disorder , characterized by excessive secretion of parathyroid hormone from one or more parathyroid glands . the elevation of pth usually leads to hypercalcemia and hypophosphatemia ; patients may present with classic skeletal disease , recurrent nephrolithiasis , or be asymptomatic , detected on routine biochemical screening . a dramatic increase in the incidence of phpt occurred in the late 1960s , due primarily to the introduction of the multichannel autoanalyzer . the clinical profile of phpt in the western countries had shifted from a symptomatic disorder , toward a more asymptomatic state . however , phpt has a variable clinical expression and symptomatic phpt is still the predominant form of disease in many developing countries , with skeletal manifestation ( osteitis fibrosis cystica ) being very common . earlier studies from india have attributed the greater severity of phpt to delayed diagnosis and widely prevalent vitamin d deficiency . hypercalcemia is considered to be a rare cause of pancreatitis but the true cause and effect relationship between phpt and pancreatitis remains controversial . phpt has been associated with different types of pancreatitis . despite its rarity , a cause and effect relationship some patients suffer from 2 or more attacks of pancreatitis before a diagnosis of phpt is made . we present a patient who had repeated admissions for acute pancreatitis , 4 episodes over a period of 15 months . a 32-year - old man was first admitted to a district hospital with an episode of acute pancreatitis . he had elevated serum amylase and computed tomography ( ct ) abdomen revealed diffusely enlarged pancreas with irregular outline with no calcification or dilatation of main pancreatic duct [ figure 1 ] . , he suffered 3 more episodes of acute pancreatitis . during the second episode , ct abdomen revealed focal necrosis in the body ( less than 30% ) . ct abdomen showing diffusely enlarged pancreas with irregular outline for the fourth episode of acute pancreatitis , he was admitted in our institute . he denied any history of alcohol abuse and there was no family history of pancreatitis . lipid profile was normal ( total cholesterol 157 mg / dl , triglyceride 158 mg / dl , ldl cholesterol 85 mg / dl , and hdl cholesterol 40 mg / dl ) . the serum calcium was 10.4 mg / dl ( normal range 8.8 - 10.5 mg / dl ) and serum albumin was 4.6 g / dl . during the first 3 episodes of pancreatitis , the serum calcium at the upper limit of normal gave us clue that he may be having underlying hypercalcemic disorder . repeat serum calcium was 13.1 mg / dl , phosphate 2.0 mg / dl , albumin 4.8 g / dl , and serum alkaline phosphatase was 284 serum 25-hydroxyvitamin d was 10.31 ng / ml and serum ipth was 610.7 pg / ml . ultrasonography neck revealed a hypoechoic lesion 2.1 1.2 cm at the lower pole of left lobe of thyroid . left inferior parathyroid adenoma was localized on a radionuclide parathyroid scan ( 99mtc mibi ) [ figure 2 ] . he denied any history of bone pains , bone fractures , neuropsychiatric symptoms , or muscle weakness . radionuclide parathyroid scan ( 99mtc mibi ) showing left inferior parathyroid adenoma parathyroid adenoma was removed . eighteen months after successful parathyroid surgery , there has been no recurrence of abdominal pain and his serum calcium is within the normal range . his current biochemical parameters are serum calcium 8.2 mg / dl ; serum phosphorus 3.9 mg / dl ; ipth 32 pg / ml , and 25(oh ) d3 29.33 pg / ml . the mayo clinic experience between 1950 and 1975 found that out of 1153 patients with phpt , only 17 ( 1.5% ) had coexisting pancreatitis , and alternative explanations for pancreatitis were found for several patients . but several studies have suggested a causal association between pancreatitis and phpt . the prevalence of acute pancreatitis in phpt has been estimated to be between 1.5% and 13% . phpt has been associated with different types of pancreatitis , such as acute , subacute , or chronic calcifying pancreatitis . a study involving 83 cases of pancreatitis combined with phpt found that about 70% of the patients suffered from acute relapsing or chronic pancreatitis . some patients suffer from 2 or more attacks of pancreatitis before a diagnosis of phpt is made . in a study from india , pancreatitis was associated in 6 of 87 patients ( 6.8% ) with phpt . all patients with a past history of pancreatitis had suffered two or more attacks . in our patient serum calcium estimation after the first episode of pancreatitis would have eliminated this delay .. it is important to estimate serum calcium after an episode of unexplained pancreatitis . this will minimize the delay before the diagnosis of phpt is made . despite its rarity , a cause and effect relationship is still suggested by the fact that parathyroidectomy seems to prevent recurrence of pancreatitis . nearly 100% improvement in pancreatitis symptoms after the cure of phpt has been reported . hypercalcemia can lead to de novo activation of trypsinogen to trypsin , resulting in autodigestion of the pancreas and subsequent pancreatitis . another explanation is that hypercalcemia leads to the formation of pancreatic calculi , ductal obstruction , and subsequent attacks of acute or chronic pancreatitis . the mean calcium values among patients with phpt and pancreatic disease have been reported to be significantly higher than those in patients with phpt without pancreatic involvement .
the association between pancreatitis and primary hyperparathyroidism ( phpt ) is controversial . we report a 32-year - old man who presented with recurrent episodes of acute pancreatitis . primary hyperparathyroidism was diagnosed after the fourth episode of pancreatitis . he had no additional risk factors for pancreatitis . eighteen months after successful parathyroid surgery , there has been no recurrence of abdominal pain and his serum calcium is within the normal range .
does evidence exist supporting the effectiveness of sms and ch in improving health outcomes ? in this brief commentary , we will suggest that such evidence does exist , in particular for diabetes , obesity , asthma , and tobacco use . diabetes a meta - analysis of 31 studies found that self - management support , which involves not only patient education but also the teaching of disease - specific skills , is effective in improving glycemic control.10 however , sms has not been shown to be effective in preventing diabetes . in contrast , a number of studies focusing on community - based interventions directed at preventing diabetes through healthy eating and exercise have shown improvements in not only hba1c , but cholesterol and weight in patients at risk for diabetes . central to these efforts have been strategies derived from the diabetes prevention program which place interventions outside medical facilities.1114 obesity a cochrane review of interventions to change the behavior of professionals and the organization of care to assist overweight or obese adults lose weight , i.e. introducing self - management support in the clinical setting , found inconclusive evidence of success.15 community and school - based interventions have shown some success in preventing obesity.1617 planet health , a randomized controlled trial for ethnically diverse school children grades 6 through 8 , integrated a curriculum delivered by classroom teachers that focused on decreasing television viewing , improving diet , and increasing physical activity . the prevalence of obesity amongst african american girls in intervention schools was significantly reduced.18 the shape up somerville intervention , a non - randomized controlled trial , included three communities studied over a 3-year period and targeted children grades 1 to 3 and their families . the intervention , targeting the before- , during- , and after - school environments included a health education curriculum , physical activity classes , modifications to the school food menu , parent outreach and education , partnering with restaurants to provide healthy food options , and a multimedia outreach campaign . results after one year showed reduced bmi scores in 1 - 3 grade children.19 asthma a meta - analysis of 32 controlled trials of self - management support for children and adolescents with asthma found that these programs significantly reduce school absenteeism , days of restricted activity , nights disturbed by asthma , and emergency department visits . self - management support involves not only information - giving , but also skills training , self - monitoring , and follow - up.20 community - based interventions may be even more successful if they include both home - based education and elimination of all environmental triggers.21 tobacco use while a cochrane review of physicians , nurses and counselors providing self - management support to smokers found a modest increase in quit rates,22 ch interventions appear more effective than clinical interventions in preventing and reducing tobacco use . a large meta - analysis investigating the effect of public smoking bans showed a 17% risk reduction in incident acute myocardial infarctions.23 a systematic review looking at smoke - free policies in workplaces and indoor public areas found clear evidence that smoke - free policies reduce tobacco use and increase the number of smokers who successfully quit.24 additionally , several studies have noted increases in quit rates when smoking bans are implemented.2526 an emerging literature suggests that evidence - based health , which includes self - management support and community health interventions , can improve outcomes beyond what is possible with evidence - based medicine alone . implementing evidence - based health requires a re - imagining of the interface between clinical care and community - level interventions . the medical care system , largely focused on individual patients , has not paid sufficient attention to the physical and social environment in which its patients live their lives . as the rates of chronic diseases increase , inattention to these factors will lead to a disease burden that outpaces the scope and resources of the medical care system . evidence - based health requires embracing a broad definition of the causes of health and illness that extends beyond clinic doors . an example worth remembering is the mound bayou mississippi community health center founded in 1967 , which offered not only primary care but also implemented sanitary water supplies , fair loan practices by local banks allowing residents to build new homes , a bus system for transportation , a nutritional program for pre - school children , and a farming cooperative.27 these up - stream interventions termed community - oriented primary carewere integrated with clinical care in a way that addressed the multiple causes of poor health , wholly acknowledging the role of environmental factors . the patient - centered medical home concept hopes to invigorate primary care in the us . it includes self - management support as one of its functions but does not emphasize community health.28 several organizations are advocating for a health home rather than a medical home , mirroring the distinction between ebh and ebm.29 some authors have pleaded that primary care practices tear down the silos and integrate with the public health community . such entreaties fail to grasp the difficulties in doing so . a paradigm shift from evidence - based medicine to evidence - based health requires a transformation even more profound than what is needed to move traditional primary care practice into a patient - centered medical home . to implement evidence - based health , not only would practices need to create population - oriented , team - based , prompt - access , cost - controlled , and physician - satisfied care to become medical homes ; they would need to do all these things plus extend their purview to community - based prevention . to do these things would require major changes in payment such that primary care practices are financially rewarded for engaging in both self - management support and community health . this journey can begin with small steps , starting with the more actionable individual - level interventions placed outside the clinic walls and moving later to structural interventions that modify the community environment . the job description of one person in a primary care practice perhaps a medical assistant could be changed to require the development of a referral process to community - based healthy eating and exercise programs for all patients with obesity , diabetes , pre - diabetes , and hyperlipidemia . primary care training programs and board exams could require detailed understanding of the diabetes prevention program and include patient education on diabetes prevention as an integral part of health care maintenance counseling . medicare could add a reimbursement code for care coordination with community - based prevention services . practices could train patients with diabetes as peer educators who also serve as community advocates to improve the health - related environment in the neighborhoods served by the practice . ncqa accreditation could be broadened to require primary care practice involvement in modifying structural risk factors in the neighborhood surrounding the practice ; for example , advocating for stronger tobacco - free area policies or regulating asthma - associated industrial emissions . all of these activities would require payment reform that rewards team - based care ; practices can not be expected to engage in these activities without reimbursement . while the implementation of evidence based health poses substantial challenges , the first step needs to be the medical profession s acceptance of the evidence - based health paradigm . without such agreement ,
while evidence - based medicine ( ebm ) has advanced medical practice , the health care system has been inconsistent in translating ebm into improvements in health . disparities in health and health care play out through patients limited ability to incorporate the advances of ebm into their daily lives . assisting patients to self - manage their chronic conditions and paying attention to unhealthy community factors could be added to ebm to create a broader paradigm of evidence - based health . a perspective of evidence - based health may encourage physicians to consider their role in upstream efforts to combat socially patterned chronic disease .
multiple eruptive dermatofibromas ( medf ) are defined by the presence of more than 15 dermatofibromas all over the body and was first reported in 1970 . a 34-year - old male presented with multiple tense brown to black nodules over the lower limbs measuring 2 cm 2 cm to 1 cm 1.5 cm , gradually increasing in size and number since the past 5 years . lesions were nonitchy and nontender . on examination , the dimple sign was not present . a provisional diagnosis of dermatofibroma was made . histopathology on hematoxylin and eosin section showed spindle cells arranged in whorls along with interlacing fascicles and storiform pattern with many cells containing hemosiderin pigment and surrounded by a few hyalinized blood vessels . multiple dermatofibromas are rare ; however , the occurrence of the multiple lesions of the hemosiderotic variant of dermatofibroma has not been reported yet . a 34-year - old male presented with multiple bluish black nodules , the largest measuring 2 cm 2 cm and smallest about 1 cm 1.5 cm . patient 's lesions initially started over the posterior aspect of the left thigh 5 years back as a pea - sized papule and gradually grew in size over time . the dark - colored nodules also increased in number at the time of presentation and were present predominantly over both the lower limbs . there was no relevant past medical history and no history or clinical findings of immune - mediated diseases , insect bites , or concomitant infections . the patient was not on any specific treatment for the same . on examination , the nodules on the lower limbs were discrete , dome - shaped , nonmobile , brown to black in color , margins being well defined , firm to hard in consistency with the largest measuring 2 cm 2 cm and smallest 1 cm 1 cm [ figure 1 ] . however , owing to the hemorrhagic appearance , we also wanted to rule out a b- or t - cell lymphoma . the dermis showed a circumscribed neoplasm composed of plump spindle cells arranged in whorls , interlacing fascicles in storiform pattern [ figure 2 ] . the neoplastic cells exhibited mild nuclear polymorphism , oval to plump vesicular nucleus , some with prominent and moderate cytoplasm . ( a ) spindle cells arranged in fascicles and storiform pattern ( h and e , 100 ) . ( b ) plump spindle - shaped cells with bland nuclei and abundant siderophages ( 200 ) perls stain showing hemosiderin deposits ihc was positive for vimentin and negative for cd34 , melan a , hmb45 , ki-67 , and s-100 . as the patient was negative for cd34 , dermatofibrosarcoma protuberans was ruled out as well . the diagnosis of multiple hemosiderotic histiocytomas was confirmed , and the patient was advised surgical excision for all his lesions . multiple eruptive dermatofibromas ( medfs ) were first reported by baraf and shapiro in 1970 and in this case report , they defined multiple as the presence of more than 15 dermatofibromas all over the body , the relevance of which is still in question . dermatofibromas can result from something as trivial as an insect bite or in association with an immune - mediated disease as part of an abortive process mediated by dermal dendritic cells . there is also mention of a case report of medfs in association with hiv infection . these are persistent lesions and undergo very minimal change along the course of many years and this is the reason why fully evolved dermatofibromas are rarely excised unless asked for by the patient . there are few case reports of singular giant hemosiderotic dermatofibroma measuring larger than 5 cm but never has there been reported the existence of multiple dermatofibromas of the hemosiderotic variant in a single case subject to the best of our knowledge . histopathologically , hemosiderotic variant of dermatofibroma present with numerous small vessels , extravasated erythrocytes , and intra- and extra - cellular hemosiderin deposits . since dermatofibroma is a superficial form of benign fibrous histiocytoma , it is composed of a mixture of fibroblastic and histiocytic cells and on ihc , they have a profound positivity for vimentin and proliferating cell nuclear antigen in addition to other macrophage markers such as ham56 and cd68 . in our case , the patient 's sample showed positivity for vimentin but was negative for cd34 , ki-67 , hmb-45 , and s-100 . dermoscopically , hemosiderotic and aneurysmal dermatofibromas usually show a multicomponent pattern with a central bluish or reddish homogenous area in combination with white structures and a peripheral delicate pigment network along with vascular structures . treatment for medfs differs from person to person depending on the causality . in immune - mediated disease - related patients , a combination of steroids and a steroid - sparing agent is usually found to be helpful . in other cases of medfs , the use of cryotherapy has been advocated over surgical excision to avoid cosmetically unacceptable wide atrophic scars . in our case , the patient was given an option of surgical excision of the larger tumors ; however , he preferred to wait and watch . this case report was intended with the sole aim of enlightening us about the various morphological manifestations that can present in a patient with dermatofibroma , including the current variant mentioned in this article which closely mimicked a cutaneous lymphoma or a cutaneous lymphoid hyperplasia . dermatofibromas have been known to occur in different clinical and histopathological variations . in our case report we document the presence of multiple dermatofibromas of the hemosiderotic histopathological variant , with an uncanny similarity to cutaneous lymphomas or a glomangioma . dermatofibromas have been known to occur in different clinical and histopathological variations . in our case report we document the presence of multiple dermatofibromas of the hemosiderotic histopathological variant , with an uncanny similarity to cutaneous lymphomas or a glomangioma . dermatofibromas have been known to occur in different clinical and histopathological variations . in our case report we document the presence of multiple dermatofibromas of the hemosiderotic histopathological variant , with an uncanny similarity to cutaneous lymphomas or a glomangioma .
a 34-year - old male came with complaints of multiple firms to hard nontender , nonitchy nodules , measuring 2 cm 2 cm to 1 cm 1.5 cm , present predominantly over the lower limbs . this condition started 5 years back as a single nodule over the thigh but gradually increased in number and size with time to involve both lower limbs . a differential diagnosis of cutaneous lymphoma and dermatofibroma was considered . he underwent a biopsy and immunohistochemistry for the same which were consistent with hemosiderotic histiocytoma and positive for vimentin . the patient was advised surgical excision for the same . the occurrence of multiple dermatofibromas although rare has been reported in a few case reports ; however , the occurrence of multiple dermatofibromas of the hemosiderotic variant has not been documented yet .
dystrophic calcinosis is calcification associated with infection , inflammatory processes , cutaneous neoplasm or connective tissue diseases . metastatic calcification results from elevated serum levels of calcium or phosphorus . iatrogenic and traumatic calcinosis excised lesion measured 0.4 - 1 cm on gross examination , and ( h and e stain , 10 ) revealed multiple small cysts with basophilic deposits in the dermis [ figure 1 ] . scrotal calcinosis is a rare benign process , characterized by multiple , painless , hard scrotal nodules in the absence of systemic metabolic disorder . inflammation and rupture of epidermoid cysts is the pathogenetic mechanism of the disease.[35 ] calcification and basophilic deposits in the dermis with multiple small cysts in the sebaceous cyst of scrotum in case 1 of the study 40 year old male presented with swelling around the knee . the lesion measured 4 2 1 cm , and upon excision was gritty to cut , and showed chalky white areas . ( h and e stain , 10 ) showed basophilic masses in the subcutaneous tissue . the excised lesion measured 5 3 1.5 cm , with chalky white areas . ( h and e stain , 10 ) revealed basophilic masses in fibrocollagenous tissue with foreign body giant cell reaction [ figure 2 ] . tumoral calcinosis with foreign body giant cell reaction in case 3 of the study tumoral calcinosis is a special form of idiopathic calcinosis cutis characterized by large periarticular deposits of calcium and found mainly around the hip , knee , shoulder and elbow joints . the basic defect is in the proximal renal tubular cell with an elevated renal phosphate reabsorption threshold and increased production of 1 , 25-dihydroxyvitamin d. it is classified into- primary normo - phosphatemic tumoral calcinosis ( nptc ) , primary hyper - phosphatemic tumoral calcinosis and secondary tumoral calcinosis . the prognosis of nptc is better than that of other subtypes.[68 ] case-2 and case-3 fit into the nptc subtype characterized by lack of familial occurrence , solitary calcification , no history of antecedent trauma , no biochemical abnormalities and no recurrence after surgical removal . 35 year old female presented with swelling in the right arm since 4 months , which was clinically diagnosed as pyogenic granuloma . the lesion measured 3 2 1 cm , and ( h and e stain , 10 ) revealed basophilic masses in the dermis [ figure 3 ] . calcinosis in the upper dermis in subepidermal calcified nodule in case 4 of the study subepidermal calcified nodule , an uncommon but distinct type of idiopathic calcinosis is not associated with biochemical abnormalities or systemic illness . it occurs more commonly on the face and ear of children , and is asymptomatic . excised lesion measured 0.4 - 1 cm on gross examination , and ( h and e stain , 10 ) revealed multiple small cysts with basophilic deposits in the dermis [ figure 1 ] . scrotal calcinosis is a rare benign process , characterized by multiple , painless , hard scrotal nodules in the absence of systemic metabolic disorder . inflammation and rupture of epidermoid cysts is the pathogenetic mechanism of the disease.[35 ] calcification and basophilic deposits in the dermis with multiple small cysts in the sebaceous cyst of scrotum in case 1 of the study the lesion measured 4 2 1 cm , and upon excision was gritty to cut , and showed chalky white areas . ( h and e stain , 10 ) showed basophilic masses in the subcutaneous tissue . the excised lesion measured 5 3 1.5 cm , with chalky white areas . ( h and e stain , 10 ) revealed basophilic masses in fibrocollagenous tissue with foreign body giant cell reaction [ figure 2 ] . tumoral calcinosis with foreign body giant cell reaction in case 3 of the study tumoral calcinosis is a special form of idiopathic calcinosis cutis characterized by large periarticular deposits of calcium and found mainly around the hip , knee , shoulder and elbow joints . the basic defect is in the proximal renal tubular cell with an elevated renal phosphate reabsorption threshold and increased production of 1 , 25-dihydroxyvitamin d. it is classified into- primary normo - phosphatemic tumoral calcinosis ( nptc ) , primary hyper - phosphatemic tumoral calcinosis and secondary tumoral calcinosis . the prognosis of nptc is better than that of other subtypes.[68 ] case-2 and case-3 fit into the nptc subtype characterized by lack of familial occurrence , solitary calcification , no history of antecedent trauma , no biochemical abnormalities and no recurrence after surgical removal . 35 year old female presented with swelling in the right arm since 4 months , which was clinically diagnosed as pyogenic granuloma . the lesion measured 3 2 1 cm , and ( h and e stain , 10 ) revealed basophilic masses in the dermis [ figure 3 ] . calcinosis in the upper dermis in subepidermal calcified nodule in case 4 of the study subepidermal calcified nodule , an uncommon but distinct type of idiopathic calcinosis is not associated with biochemical abnormalities or systemic illness . it occurs more commonly on the face and ear of children , and is asymptomatic . after clinical diagnosis of calcinosis cutis , a laboratory workup to rule out abnormalities of calcium and phosphorus metabolism , malignant processes , collagen vascular diseases , renal insufficiency , excessive milk ingestion , vitamin d poisoning must be carried out to detect the underlying cause of the disease .
calcinosis cutis is a condition of accumulation of calcium salts within the dermis . we are presenting four cases of calcinosis cutis , with different clinical presentations , occurring in healthy individuals , with normal serum calcium and phosphorus levels . histologically , all cases showed similar morphology , the lesions were composed of large and small deposits of calcium . foreign - body giant cell reaction was seen in one case . another case had intact and ruptured epidermal cysts and calcification within the cyst .
different therapy strategies are used to reduce spasticity in patients with traumatic spinal cord lesions . these options consist of conservative management , including physiotherapeutic and pharmacological approaches , as well as surgical techniques . botulinum toxin plays an important role in the treatment of spasticity of different causes . by locally injecting botulinum toxin , thus , botulinum toxin injection is a treatment option of particular interest in cases of localized spasticity , , . the purpose of this study is to evaluate the effect of botulinum toxin injections in patients with spasticity caused by traumatic spinal cord injuries and to determine favourable patients characteristic . the study included 9 male patients ( mean age 40 y 16 y ) , suffering from a none - acute traumatic lesion of the spinal cord and chronic spastic paraplegia ( table 1 ( tab . 1 ) ) . eight patients had a complete spinal cord lesion , defined as american spinal injury association ( asia ) impairment scale type a . one patient showed an incomplete neurologic deficit , defined as asia type c. all of them offered an increased spasticity state of 3 to 4 on the ashworth scale modified by bohannon and smith , . all patients underwent a combination of physiotherapeutic and oral antispastic therapy for at least three months without satisfying success . all patients underwent botulinum toxin a injections in the affected muscle groups with a dilution of 100 u / ml under ultrasonographic control . the maximum general injection dose was limited to 2,000 u. after the injection , all patients underwent intensive physiotherapy of the affected muscles for three days to improve the botox distribution . all patients underwent clinical control examinations after 3 2 weeks and 34 3 months after botulinum toxin injections . six of the nine patients were satisfied or very satisfied after botulinum toxin injection and offered a clear reduction of spasticity in the treated muscle groups to a state of two or less on the ashworth scale modified by bohannon and smith , with an average reduction of 1.9 states after two weeks ( figure 1 ( fig . 1 ) ) . five of them reported reduced difficulties during mobilisation including transfers to the wheel chair as well as getting dressed or undressed . the patient with incomplete motor function deficit showed a relevant reduction of the adductor spasticity . however , he had increasing problems in walking due to additional muscle weakness for three months and regaining muscle strength over a period of another three months afterwards . another patient reported a temporary general weakness after injection , which fully recovered after three days . the effect of the botulinum toxin injections started after two to five days and reached a peak after an average of two weeks . no signs of infection or allergic reaction were recordable , particularly at the side of injection . at the final evaluation , three patients had persistent low spasticity at the affected muscle groups of state two or less of the ashworth scale modified by bohannon and smith . three patients regained spasticity to a slightly lower level ( 3 ) compared to the initial level of spasticity ( figure 1 ( fig . the beneficial effect lasted for at least seven months and decreased subsequently in all of them . several studies showed the beneficial effect of botulinum toxin to adults and children in cases of spasticity and demonstrated it to be safe even after repeated injections , . the side - effects consisted of focal or generalized muscle weakness and urinary incontinence in about 1% of patients . all other reported adverse events were less common and included pain at the site of the injection , fatigue , somnolence , influenza - like symptoms , fever , and purpuric skin rash . similarly , one of our patients ( 11% ) complained of mild systemic weakness after injection , lasting for three days . adverse effects were recorded after administering doses of more than 1,000 u in children and more than 2,000 u in adults . the encouraging results of our study are consistent with other studies that showed beneficial effects of botulinum toxin injections in the therapy of spasticity , , , . similarly , two case reports showed a positive effect of botulinum toxin injections in patients with spinal cord injury and ulcers caused by spasticity , . in our study population , 6 of 9 of the patients presented with a significant reduction in their spasticity , reported a better quality of life , and were satisfied or very satisfied . one of these patients showed a diffuse pattern of spasticity that affected six functional muscle groups of the upper and lower leg . thus , this patient seemed to be a poor candidate for this treatment strategy based on the restricted maximal dose of botulinum toxin . furthermore , this therapy has to be seen very critically in incomplete paretic patients ( asia c ) , particularly in those who are able to walk . these patients are commonly dependent on maximum strength of all functional muscle groups at their lower limb . thus , botulinum toxin injection causes weakness of certain muscle groups , which might lead to temporary immobilisation as seen in one of our cases . the strong limitation of this study is its limited patient group , including only nine patients and only one patient with incomplete motor function deficit ( asia c ) . thus , certain patients characteristics which might cause limited effectiveness of botulinum toxin injections , such as diffuse spasticity pattern or incomplete motor function deficit are based on single cases only . additionally , the level of spasticity was based on the ashworth scale modified by bohannon and smith that is purely observer dependent , which might lead to bias . nonetheless , this study includes a very selective patient collective with beneficial effects in the majority of the cases . future studies are warranted to further evaluate the effects of botulinum toxin and the safety profile of it in the treatment of patients with spasticity after traumatic spinal cord injury . botulinum toxin injection for the treatment of spasticity after traumatic spinal cord lesion has the potential of a very promising treatment strategy . it seems to be particularly effective in patients with complete lower limb motor function deficit and spasticity pattern limited to few muscle groups . caution has to be recommended in incomplete paretic patients , who are still able to walk , in order to avoid immobilizing effects.further studies are necessary to find parameters , which might affect the antispastic therapy with botulinum toxin . botulinum toxin injection for the treatment of spasticity after traumatic spinal cord lesion has the potential of a very promising treatment strategy . it seems to be particularly effective in patients with complete lower limb motor function deficit and spasticity pattern limited to few muscle groups . caution has to be recommended in incomplete paretic patients , who are still able to walk , in order to avoid immobilizing effects . further studies are necessary to find parameters , which might affect the antispastic therapy with botulinum toxin .
objectives : the purpose of this study was to determine the effect of botulinum toxin injections for the treatment of spasticity after traumatic spinal cord injury . methods : 9 patients were included in this prospective designed study , with a follow - up of at least 2 years . all patients suffered from a massive spasticity after traumatic spinal cord lesion . conservative treatment options did not show satisfying results . all patients were injected a maximal dose of 2,000 units of botulinum toxin a in no more than 6 skeletal muscle groups . clinical control examinations were performed after 2 weeks and after at least 2 years . results : 6 patients reported a good or very good result . one patient offered increasing difficulty in walking for a short time after injection . 2 patients showed no beneficial effects . one patient experienced a modest temporary general weakness for 3 days . after 2 years , 3 patients showed improved function with persistent reduction of spasticity . in the other cases , the beneficial effect lasted for an average of 9 months . conclusion : botulinum toxin a injection seems to be an effective complementary therapy option in the treatment of spasticity of paraplegic patients with complete deficit of their motor function ( asia a and b ) and a spastic distribution pattern , effecting only a limited number of muscle groups . caution has to be recommended for incomplete paretic patients , who are able to walk .
agranulocytosis is defined as an absolute neutrophil count ( anc ) less than 100/mm in association with infectious disease . the risk of agranulocytosis is 0.38% of all clozapine treated cases and there is a relatively lesser incidence in indian population . the risk of clozapine - induced agranulocytosis and neutropenia is highest in the first 6 months and higher in the initial 18 months after the onset of treatment . the reports of clozapine induced agranulocytosis in co - morbid multiple sclerosis and delayed onset neutropenia and 2 reports of early treatment phase clozapine induced agranulocytosis have been reported from india , so far no reports of late onset clozapine induced agranulocytosis has been reported from india . a 44-year - old female patient with a diagnosis of paranoid schizophrenia , on clozapine treatment and regular follow - up presented in psychiatry outpatient department with fever , rigor , swelling of the right hand and sore throat . she has a 22 year history of fear suspicion and hearing voices with history of treatment with typical and atypical antipsychotics and poor treatment response . she was started on clozapine around 5 years back and subsequently given risperidone and trihexyphenidyl . at the time of presentation , she was on 150 mg of clozapine , 6 mg of risperidone and 4 mg of trihexyphenidyl . a total count done 1 week back showed 6,700/mm with 70% neutrophils , 26% lymphocytes and 4% eosinophils . she was on examination , conscious , oriented , febrile , had a pulse rate of 96/min , her blood pressure was 110/60 mm of hg , diffuse swelling of right upper limb , her systemic examination was normal and had on mental status examination persecutory delusions and third person auditory hallucinations . she was admitted under the department of internal medicine and worked up . on admission , her hemoglobin level was 8.1 g / dl , total count was 700 , her differential count was n4p95e1 , packed cell volume ( pcv ) was 25.3% , erythrocyte sedimentation rate - 140 mm/1 h , platelet count was 6.8 lakhs / dl , random blood sugar was 100 mg / dl , urea - 14 mg / dl , creatinine - 1.1 mg / dl , total bilirubin - 0.8 mg / dl , serum glutamic oxaloacetic transaminase - 103 iu / l , serum glutamic - pyruvic transaminase - 102 iu / l , serum alkaline phosphatase ( sap ) - 125iu / l , total protein - 8 g / dl , a : g ratio was 1:2 , human immunodeficiency virus and hepatitis b surface antigens were negative , chest x - ray was normal , electrocardiogram was normal , computed tomography head was normal , ultrasonography abdomen was normal , but doppler of right upper limb showed dilatation of cephalic vein ( 4.2 mm ) with echogenic content and thrombus extending up to axillary vein with non - compressibility and no internal flow . she was diagnosed as having - drug induced agranulocytosis and deep vein thrombosis of right upper limb . she was started on antibiotics ( cefoperazone + sulbactum ) , warfarin , analgesics , proton pump inhibitors , supportive measures , barrier nursing and was given granulocyte - colony stimulating factor 300 g for 7 days . the bone marrow examination and biopsy carried out post - treatment showed lymphocytosis with 6% blasts . she however was at request referred to a specialized hematology unit and was treated there with antibiotics ( amoxicillin + clavulanic acid ) warfarin , analgesics , proton pump inhibitors , supportive measures and barrier nursing . improvement of blood picture patient in the meantime had an exacerbation of psychotic symptoms and was given risperidone , chlorpromazine and trihexiphenidyl . the patient on improvement of blood picture was discharged on oral antibiotics , warfarin , mupirocin ointment , glycerine mag sulfate thrombocytophobe dressing , risperidone 3 mg , chlorpromazine 150 mg and trihexyphenidyl 2 mg . the patient had an asymptomatic course and was reviewed by both medicine and psychiatry . at 6 months post event follow - up she is currently on 8 mg of risperidone , 50 mg of chlorpromazine and 4 mg of trihexiphenidyl and is in partial symptom remission and not on any other medication . the mechanism of clozapine - induced of agranulocytosis is unclear especially as neither clozapine nor its major stable metabolites , n - desmethylclozapine at therapeutic drug concentrations ( 1 - 3 m ) , have been found to be directly cytotoxic to neutrophils or interfere with the turnover of bone marrow precursor cells . a strong possibility is for an immune - mediated mechanism or at least an immunological background for clozapine - induced agranulocytosis , as on re - exposure to the drug , the course is more severe and the time interval to recurrence of toxicity is shorter . of the known risk factors of agranulocytosis such as female sex and increased age or age less than 21 of which this case had one risk factor . there is also an association between eosinophilia and neutropenia , which was not seen in this case . the report of delayed onset agranulocytosis is very rare and this is the first reported from india on a patient without co - morbidity . it is hypothesized modest growth - inhibiting effects of clozapine on bone marrow may be amplified in patients undergoing long - term therapy with clozapine . dose and serum levels of clozapine may not be directly associated with clozapine - induced agranulocytosis , although some reports suggest a dose - related aspect of this phenomenon . the phenomenon of late onset agranulocytosis should therefore be considered with care and all clinicians should insist to regular guideline based monitoring of white cell count , even in patients who have been on clozapine for years .
agranulocytosis is defined as an absolute neutrophil count less than 100/mm3 in association with infectious disease . the risk of agranulocytosis is 0.38% of all clozapine treated cases and there is a relatively lesser incidence in indian population . the risk of clozapine - induced agranulocytosis and neutropenia is highest in the first 6 months and higher in the initial 18 months after the onset of treatment . there have been very few reports of neutropenia and agranulocytosis after this period . there have so far been no reports of late onset clozapine induced agranulocytosis has been reported from india . a case of late onset clozapine induced agranulocytosis with possible mechanism of the same is reported .
the introduction of ribavirin to hepatitis c combination therapy with pegylated interferon ( peg - ifn)-2a has improved sustained clinical response rates , but it has been accompanied by an increased incidence of cutaneous side effects . there has been 1 prospective study on the de novo onset of skin symptoms associated with peg - ifn-2a and ribavirin combination treatment , and there have been some reports of drug eruption caused by ribavirin and peg - ifn-2b [ 2 , 3 ] or ifn- combination therapy [ 4 , 5 ] . most cases were not severe and improved only with a topical steroid and oral anti - allergic drug , without the need for discontinuation of the antiviral treatment . severe cases required a discontinuation of the antiviral treatment [ 3 , 4 , 5 ] ; a steroid was administered in very severe cases [ 2 , 3 ] . we describe a 59-year - old japanese woman with a chronic hepatitis c infection who developed erythema during peg - ifn-2a and ribavirin therapy . there was a faint erythema on her trunk and her arms after 8 weeks of treatment ( week 8) . administration of bepotastine besilate had no effect . however , the erythema improved with the application of crotamiton cream and alclometasone dipropionate ointment . the hcv rna virus had decreased from 6.6 logiu / ml to negative at week 16 . subcutaneous injection of peg - ifn-2a was postponed due to recurrent erythema at week 20 . eruption at the injection site of ifn occurred after each injection , and then , eruption on her head , face and neck ( exposed skin ) was observed ( fig . after peg - ifn-2a subcutaneous injection in her right arm , the eruptions became worse and she had a consultation in our department . at that time , she had infiltrative erythema without any mucosal symptoms on her whole body ( fig . 1 ) . it seemed to be an erythema multiforme type drug eruption of peg - ifn-2a , ribavirin and/or fluvastatin sodium from the clinical course . although administration of all drugs was stopped , the eruption expanded after administering 40 mg prednisolone and a topical steroid . since prednisolone was suspected to have been the cause of the drug eruption , the eruption improved after it was changed to 6 mg of betamethasone . the lymphocyte transformation test ( ltt ) was carried out for peg - ifn-2a , ribavirin and fluvastatin sodium . this is the first case of a positive result after performing the ltt for ribavirin . activation of drug - specific t - cells is generally thought to play a central role in mediating adverse drug reactions . the ltt is a safety test to assess the activation of drug - specific t - cells in vitro and is a reliable method to define the causative agent . the ltt measures the proliferation of t - cells to a drug in vitro from which 1 concludes to a previous in vivo reaction due to a sensitization . this concept of the ltt has been confirmed by the generation of drug - specific t - cell clones and the finding that drugs can directly interact with the t - cell receptor , without any previous metabolism or the need to bind to proteins . the main advantage of this test is its applicability with many different drugs in different immune reactions , as drug - specific t - cells are almost always involved in drug hypersensitivity reactions . in our case , then eruption on her head , face and neck ( exposed skin ) was seen . we should not only consider ifn , but also ribavirin in case of generalized eruption , especially on an exposed area , with combination therapy for hcv .
the introduction of ribavirin to hepatitis c combination therapy with pegylated interferon ( peg - ifn)-2a has improved sustained responses , but it has been accompanied by an increased incidence of cutaneous side effects . most cases of drug eruption caused by ribavirin and peg - ifn-2 or ifn- combination therapy were not severe and we progressed without discontinuation of the antiviral treatment . we describe a 59-year - old japanese woman with a chronic hepatitis c infection who developed erythema during peg - ifn-2a and ribavirin combination therapy . the eruption at the injection site of ifn occurred after each injection , and then , eruption on her exposed skin was observed . twenty milligrams of prednisolone was administered . the eruption recurred after each administration of prednisolone and ribavirin . she finally had infiltrative erythema without any mucosal symptoms on her body . it seemed to be an erythema multiforme type drug eruption of peg - ifn-2a , ribavirin and/or fluvastatin sodium from the clinical course . the lymphocyte transformation test ( ltt ) of ribavirin was positive . this is the first case of a positive result of an ltt for ribavirin . a photosensitive type drug eruption with ribavirin treatment has been reported . we should not only consider ifn , but also ribavirin in case of a generalized eruption , especially on an exposed area with combination therapy for hcv .
neuropathy in diabetic patients is heterogenous , so can present with many sensory and motor symptom . diverse neuropathic symptoms are important in the early recognition and diagnosis of diabetic neuropathy ( dn ) . if the neurological symptoms are atypical and are not resolved , especially when motor neurons were involved , other causes should be considered because dn is an exclusive diagnosis and nondiabetic neuropathy may be present in diabetic patients . among these , guillain - barre syndrome ( gbs ) is a disease that should be considered when acute flaccid weakness occurs . we report here a case of diabetic neuropathy combined with gbs , which was diagnosed with some delay due to the underlying diabetes . a 59-year - old man complained of pain and numbness in both lower legs of several months duration . his medical history was diabetes of 15 years and he recently had caught a common cold . laboratory findings showed : proteinuria ( + ) , glucosuria ( + + ) , hemoglobin 10.1 g / dl , aspartate aminotransferase ( ast)/alanine aminotransferase ( alt ) 24/30 u / l , glucose 288 mg / dl , na / k / cl 130/4.1/98 mmol / l , blood urae nitrogen ( bun)/creatinine ( cr ) 28/1.2 mg / dl , hba1c 8.8% , c - peptide 1.2 ng / ml , and total cholesterol 248 mg / dl . on the neurological examination after admission , the patient was treated with insulin for glucose control and alpha - lipoic acid ( ala ) , gabapentin , and amytryptilin for diabetic peripheral neuropathy based on symptoms ( total symptom score , 9.65 ) , 10 g monofilament test , and quantitative sensory test ( table 1 ) . after two days , however , the sensory symptoms in both legs were more aggravated and there was additional motor weakness in the lower legs . , he could not walk and had difficulty sensing movement of upper extremities , although he had not had any discomfort in ambulation at the visiting hospital . cervical and brain mri were performed but resulted in near normal findings except for mild herniation of an intervertebral disc . on day 5 , aggravated weakness of the lower legs had spread to the upper extremities , so we consulted with the neurology department . deep tendon reflex was absent and so cerebrospinal fluid ( csf ) examination and a neurophysiological study were performed . the csf showed albumino - cytologic dissociation ( table 2 ) and the electrodiagnostic finding was acute motor and sensory axonal neuropathy compatible with gbs ( table 3 ) . immunoglobulin was the administered intravenously and the neurological deficit of motor weakness improved gradually over the next week , allowing the patient to be discharged . the sensory discomforts however , such as pain , numbness , and tingling sense , which were thought to be caused by dn , were little relieved with continuous medication of ala and gabapentin . many diabetic patients suffer from neuropathic symptoms involving sensory and motor nerves . however , motor neuron involvement including paralysis or weakness of extremities is not common in dn . generally confirmation of dn can be established by neuropathic symptoms , sensory and autonomic function test , and quantitative electrophysiology . nevertheless , other causes should be excluded . cerebrovascular accident , peripheral vascular insufficiency , spinal disease , periodic paralysis , myasthenia , transverse myelitis , toxic neuropathy , diabetic peripheral neuropathy , and gbs are all possible causes . since the prognosis of the neuropathic patient sometimes is dependent on early recognition and prompt treatment of the cause , an exact diagnosis is needed in the early period of the disease . to avoid misdiagnosis when the patient suffers from neuropathic symptoms combined with acute weakness or paralysis of limbs , the physician must be aware of the possibility of gbs , even if the patient is being treated for another disease . although there are no reports show of a high risk of gbs in diabetic patients , a few cases of gbs associated with diabetic ketoacidosis have been described . gbs underlying dn is uncommon but could possibly be a serious cause of paralysis or weakness of limbs , although this condition can be overlooked on presentation . prompt and sufficient evaluation and management should be done in the early stages when patients are suffering from neuropathic symptoms which are not explained by previously diagnosed underlying disease . gbs is the commonest peripheral neuropathy affecting children but it can occur at any age and condition . it is characterized by rapidly progressing symmetric limb weakness , loss of tendon reflexes and sensory signs , and autonomic dysfunction . acute peripheral neuropathy in gbs includes acute inflammatory demyelinating polyradiculoneuropathy , acute motor axonal neuropathy , and acute motor and sensory axonal neuropathy . the onset of neurological symptoms , such as flaccid weakness of lower limbs , is usually sudden , but is possible anytime between 1 to 28 days after the prodromal event . weakness of the limbs is usually present bilaterally with symmetrical involvement spreading upwards to the arms , but asymmetrical presentation and only distal involvement of the legs are also possible . although the exact pathogenic mechanisms are uncertain , there is considerable evidence that an acute demyelinating process occurs by an immunemediated reaction with neural antigen after various viral illnesses including herpes , mumps , mycoplasma , cytomegalovirus and campylobacter or after immunization . for the diagnosis of gbs , in addition , neurophysiological examinations including nerve conduction studies are essential for the confirmation of gbs . marked slowing of motor conduction velocity , prolonged distal latency , and conduction block can be shown in nerve conduction studies . early symptomatic and supportive treatments including careful monitoring of vital signs are the mainstay of the management . until now several factors such as occurrence in adulthood , rapid development of paralysis , delay in the diagnosis , marked distal involvement , a low amplitude or fibrillation pattern in the electromyographic potential have been suggested as predictors of poor prognosis . in our case , due to diagnostic delay , rapid progression and electromyografic findings , a poor prognosis was predicted . we report a case of dn combined with overlooked gbs that was detected with delay to encourage prudence in caring for neuropathic diabetic patients .
a 59-year - old man was admitted with numbness , pain , and a tingling sensation in both lower legs . he was initially diagnosed with diabetic peripheral neuropathy based on a symptom questionnaire and a quantitative sensory test . despite symptomatic treatment of diabetic neuropathy , he complained of worsening sensory symptoms and additional motor weakness in both lower extremities . as the motor weakness of both extremities became more aggravated over time , brain and spine imaging tests and a nerve conduction test were performed . the nerve conduction study revealed motor and sensory axonal neuropathy . in his cerebrospinal analysis , albumino - cytologic dissociation , which is compatible to the gillian - barre syndrome , was found . cerebrospinal fluid analysis showed albumino - cytologic dissociation . he was treated with intravenous immunoglobulin and his neurologic deficits were gradually improved .
in spite of precautions , a foreign body retained in the abdominal cavity after surgery may lead to persistent problems such as adhesions , perforation , and abscess or fistula formation ( 123 ) . endoloop ligature ( ethicon , somerville , nj , usa ) is surgical instrument to facilitate the ligation of pedicles in laparoscopic procedures . vicryl ( polyglactin 910 ) and polydioxanone ( ethicon ) endoloop ligature can be used . it consists of a 45 cm long ligature inserted into a plastic tube ( narrow at one end and scored at the other ) . the plastic tube should be removed after the suture is formed in a ligature with a knot . retained plastic tube of the endoloop ligature in the abdominal cavity is difficult to detect in the absence of clinical suspicion and familiarity with the appearance , since it is very narrow and thin . there has been no report on ct findings of retained endoloop ligature plastic tube . with the approval of the institutional review board of our hospital , we describe ct findings of foreign body reaction related to retained endoloop ligature plastic tube , which was misinterpreted as acute appendicitis . she has undergone ct scan due to intermittent abdominal pain one month after laparoscopic cholecystectomy . operative findings and pathologic results revealed localized peritonitis related to retained endoloop ligature plastic tube ( fig . 1e ) that was used in laparoscopic cholecystectomy and appendix was normal . on retrospective review of ct images the imaging appearances of retained surgical instruments in the postoperative abdomen and pelvis can be confusing and difficult to identify . unintentionally retained surgical instruments lead to substantial morbidity , and mortality rates with the range of 1135% ( 4 ) . to our best knowledge , the presented case is the first report of ct findings of foreign body reaction related to retained endoloop ligature plastic tube , which had been misdiagnosed as acute appendicitis because of the location and morphologic characteristics . among retained surgical instruments , gossypiboma in the abdomen counts for 50% of malpractice claims for retained foreign body ( 2 ) . gossypiboma is the term sometimes used to describe the foreign body reaction to a surgical sponge retained within the body for a long period . the radiologists consider a gossypiboma to be specifically indicated by a ct finding of a low - density heterogeneous mass with an external high - density wall that is further highlighted on contrast - enhanced imaging and that has a spongiform pattern containing air bubble ( 5 ) . the spongiform pattern with gas bubbles is the most characteristic ct finding for gossypibomas ( 5 ) . the former usually occurs early in the postoperative period and may involve secondary bacterial contamination , which results in various fistulas and abscess ( 5 ) . ct findings of the presented case were localized peritonitis around air - containing tubular structure in right lower quadrant of the abdomen . the underlying mechanism of localized peritonitis can be explained by the nidus formation of the retained plastic tube as foreign body reaction and surrounding inflammation around the retained plastic tube nidus . however , in hindsight , there were some differences between the ct findings of acute appendicitis and those of presented case ; 1 ) tubular structure was artificially straight , 2 ) relatively long and evenly outlined , and 3 ) air was contained inside the tubular structure . we failed to identify the retained endoloop ligature plastic tube without surrounding inflammation on ct scans performed one month after laparoscopic cholecystectomy . on the retrospective review , ct images at that time showed bizarre tubular structure in upper abdomen . we neglected this finding as an unexplained artifact because we did not have any knowledge on a retained surgical item in the patient . for the radiologist , correct interpretation of the findings is even more challenging because 1 ) no surgical item is suspected , 2 ) the radiologist may not recognize the item as a foreign body , and 3 ) retained items often elicit a reaction creating a fluid collection that mimics a postoperative abscess or even tumor recurrence ( 6 ) . the confidence in the ability to identify or exclude suspected and incidentally encountered retained surgical items is critical . the radiologist 's failure to identify a retained surgical item if there is any suspicion related symptom from the retained surgical item , radiologist should recommend further study such as ultrasonography or mri and image - based retained surgical item detection may be enhanced . therefore , radiologists should remember that unintentionally retained surgical instruments are often clinically unsuspected and may be first recognized on imaging . a higher index of suspicion should be maintained when bizarre findings can not be explained in any other way . in summary , we identified that ct findings of foreign body reaction related to retained endoloop ligature plastic tube might mimic acute appendicitis . the retained endoloop ligature plastic tube was artificially straight , relatively long and evenly outlined , and air - containing tubular structure in contrast of inflammatory appendix . the possibility of a retained foreign body should be considered and a higher index of suspicion should be maintained when unfamiliar findings can not be explained in any other way . with the increased complexity and more frequent use of surgical instruments , the radiologists need to be familiar with the imaging findings of a variety of unintentionally retained surgical instruments .
many hospitals experience one or more retained surgical instrument events with risk of patient morbidity and medicolegal problems . identification of retained surgical instrument is important . the radiologists should be familiar with imaging finding of retained surgical instrument . in a 62-year - old female with a retained plastic tube , localized peritoneal infiltration around air - containing tubular structure mimicked acute appendicitis on abdomen computed tomography ( ct ) , one year after laparoscopic cholecystectomy . we reported ct findings of foreign body reaction related to retained endoloop ligature plastic tube mimicking acute appendicitis .
infantis resulted from a general increase in different biotypes or a successful spread of 1 clone . infantis identified in israel during 20072009 ( 21 human sources , 28 poultry sources , and 22 food sources ) and 16 historical strains isolated during 19702005 ( 12 human sources , 2 poultry sources , and 2 food sources ) were subjected to pulsed - field gel electrophoresis ( pfge ) . macrorestriction with the xbai enzyme discriminated the isolates into 23 distinct profiles ( pulsotypes ) , designated i1i23 . although the historical isolates showed high diversity in their pfge patterns , most ( 58/71 , 82% ) recent ( 20072009 ) isolates were homogeneous and showed an indistinguishable pfge profile ( pulsotype i1 ) , which was not found among the historical isolates ( figure 2 ; table a1 ) . these results indicate that most of the emerging isolates belong to 1 genetic clone that probably started to spread in israel sometime during 20052007 . furthermore , comparison of the i1 pulsotype with other pfge profiles through pulsenet ( www.cdc.gov/pulsenet/ ) and pulsenet europe ( www.pulsenetinternational.org/networks/europe.asp ) indicated a pattern not reported elsewhere , suggesting the emerging clone is endemic to israel . pulsed - field gel electrophoresis ( pfge ) patterns of salmonella enterica serovar infantis isolates from clinical , food , and poultry sources isolated in israel , 19702009 , showing a high degree of clonality . macrodigestion performed using xbai restriction enzyme and genetic similarity ( in % ) was based on dice coefficients . pfge was conducted according to the standardized salmonella protocol centers for disease prevention and control pulsenet as described ( 4 ) by using s. enterica ser . a complete list is provided in table a1 . to further characterize the isolates , we performed susceptibility tests to 16 antimicrobial compounds . overall , resistance to 11 antimicrobial agents was detected ( table ; table a1 ) . first , although 6/16 ( 38% ) of the historical strains were sensitive to all tested antimicrobial agents and 5/16 ( 31% ) were resistant to only 1 ( nitrofurantoin ) , none of the 20072009 isolates were sensitive to all of the tested antimicrobial agents . most ( 68/71 , 96% ) of the recent isolates were resistant to > 3 antimicrobial agents , which suggests a process of resistance acquisition over time . second , whereas isolates from 19702005 did not share any obvious resistance pattern , most ( 66/71 , 93% ) of the 20072009 strains showed a combined resistance pattern to nalidixic acid , nitrofurantoin , and tetracycline with or without additional resistance to trimethoprim / sulfamethoxazole ( table ) . the convergence of the recent serovar infantis clones to a dominant resistance pattern is consistent with their common pfge profile and shows that they share high similarity on phenotypic and genotypic levels . * pfge , pulsed - field gel electrophoresis ; i1 , emerging pfge pattern ; d , different from the emerging pattern . next , we characterized the molecular mechanisms responsible for the common antimicrobial drug resistance phenotype . in bacteria , an efficient means of acquisition and dissemination of resistance genes is through mobile genetic elements such as plasmids , transposons , or integrons ( 5 ) . plasmid analysis for 15 emerging ( 20072009 ) and 7 historical ( 19702005 ) randomly selected isolates demonstrated that all possessed 1 large plasmid of 100 kb . to identify antimicrobial drug resistance genes that are possibly encoded on this plasmid , mating experiments were conducted with a plasmid - free , rifampin - resistant escherichia coli j53 strain and recent s. enterica ser . conjugation experiments showed the obtained e. coli transconjugants received the large ( 100-kb ) plasmid and acquired the tetracycline resistance phenotype but remained susceptible to nalidixic acid and nitrofurantoin . molecular analysis by pcr showed the teta gene encoded within the tn1721 transposon in 6 of 6 randomly selected emerging isolates but in only 1 of 5 older historical strains . we examined class 1 integrons using pcr primers designed to amplify the variable region of class 1 integrons . sulfamethoxazole followed by the orfc gene of unknown function . in contrast , 3/5 historical isolates did not possess any integron , and 2/5 contained a disparate integron with a variable region of 1.3 kb . sequencing analysis indicated a different cassette encoded by the aminoglycoside adenyltransferase aada1 gene conferring resistance to spectinomycin and streptomycin . resistance to quinolones is often associated with point mutations in the quinolone - resistance determining region of the gyra gene ( 6 ) . to examine this possibility , we determined the gyra sequence from 6 recent naladixic acid resistant and 4 naladixic acid sensitive isolates . all resistant clones showed the same nucleotide substitution from guanine to thymine at position 259 ( g259 t ) in the gyra gene , resulting in the exchange of asparagine in position 87 to tyrosine ( asp87tyr ) in the quinolone resistance determining region domain . no mutations were found in the gyra sequence of the naladixic acid sensitive isolates , suggesting that the asp87tyr point mutation is responsible for the observed naladixic acid resistance phenotype . it is likely that environmental selective pressure caused by use of antimicrobial drugs has led to the distribution of appropriate resistant genes . nitrofurans and sulfonamides , for example , have been widely used to treat infections and promote growth of livestock ( 7 ) . because the emerging clone was dominant in all levels of the food chain , including broiler chickens , it is possible that the emerging clone was originally introduced from a poultry source . recent studies from other countries identified healthy poultry as a potential reservoir of s. enterica ser . infantis isolates from different sources and regions in israel showed high homogeneity of emerging isolates that differ genetically and phenotypically from previously isolated strains . we showed that the emerging clone is multidrug resistant and is characterized by a large conjugative plasmid harboring the tn1721 transposone and teta gene , which provides reduced susceptibility to tetracyclines . additional characteristics include a class 1 integron containing the dfra1 cassette , a gyra mutation that mediates nalidixic acid resistance and furthers resistance to nitrofurantoin . our results suggest the recent emergence of serovar infantis is an outcome of a clonal expansion and establishment of a specific biotype that took place during a relatively short period .
to determine whether rapid emergence of salmonella enterica serovar infantis in israel resulted from an increase in different biotypes or spread of 1 clone , we characterized 87 serovar infantis isolates on the genotypic and phenotypic levels . the emerging strain comprised 1 genetic clone with a distinct pulsed - field gel electrophoresis profile and a common antimicrobial drug resistance pattern .
transient osteoporosis of the hip ( toh ) is an uncommon disease that has very characteristic clinical features ( 1 ) . radiologically , osteopenia of the proximal femur is the only one abnormal finding and it also disappears in parallel with symptoms . the osteopenic area shows increased radionuclide uptake in bone scintigram and bone marrow edema pattern on mr images . although toh affects middle - aged men more frequently , the first reported cases were in women during the last trimester of pregnancy . the etiology of toh remains unknown , but it was recently proposed that subchondral fracture of the femoral head ( sffh ) causes toh ( 2 - 4 ) . recently , the authors treated a woman with bilateral toh that occurred sequentially during the peripartum period of her first delivery . serial computerized tomograms ( cts ) of the femoral heads showed evidences of trabecular injury . the patient , a previously healthy housewife , had delivered her first baby about 3 weeks previously . pain had first developed in the left hip some 3 months previously , at gestation week 32 , and in the right hip 3 weeks previously ( immediately after delivery ) . she had to use crutches because of disabling hip pain on weight bearing . at the time of presentation ( postpartum 3 weeks , 3 months after left hip pain onset ) , the left hip pain was improving , but the right hip pain was worsening . plain radiographs taken at 1 week postpartum showed diffuse osteopenia in both proximal femora , which was more marked in the left side ( fig . 1 ) . magnetic resonance ( mr ) images taken at 2 weeks postpartum showed a diffuse bone marrow edema pattern in the femoral head , neck and intertrochanteric areas of both femurs . symptoms improved gradually and disappeared completely at 7 weeks postpartum ( 4 months after left hip pain onset ) in the left hip and at 11 weeks postpartum in the right hip . follow - up mr images taken at 7 weeks postpartum showed markedly reduced bone marrow edema in the left and increased bone marrow edema in the right . 4 ) . coronal multiplanar reformation ( mpr ) ct images were taken serially to evaluate the condition of trabecular bone of the femoral heads ( fig . the first ct images taken at 4 weeks postpartum ( 4 weeks after right hip pain onset , and 12 weeks after left hip pain onset ) showed marked decreases in the sclerotic densities of primary compression trabeculae with irregular discontinuation , more so in the right . in addition , a focal area showing irregular thickening of trabeculae was observed in the left femoral head , and in the right femoral head , breakages of subchondral bone with a linear dense sclerotic line crossing vertical trabeculae ( suggestive a fracture line ) were observed . on the second ct images taken at 11 weeks postpartum ( 11 weeks after right hip pain onset , 19 weeks after left hip pain onset ) , sclerotic densities of primary compression trabeculae had recovered markedly in both femoral heads . a focal area showing irregular thickening of trabeculae third ct images taken at 20 weeks postpartum ( 20 weeks after right hip pain onset , 28 weeks after left hip pain onset ) demonstrated increased trabecular density in both femoral heads , and the focal area of irregular trabecular thickening disappeared in both heads . the fourth set of ct images taken at 1 yr postpartum showed almost complete recovery of primary compression trabeculae in both femoral heads . the etiology of toh remains unknown , but several hypotheses have been proposed ( 1 , 6 ) . these include intermittent compression of the obturator nerve , a non - traumatic form of reflex sympathetic dystrophy , viral infection , transient ischemic insult to bone , hormonal imbalance and trauma . recently , sffh had been proposed as a possible etiology ( 2 - 4 , 6 , 7 ) . ( 8) have described the histological findings of biopsy specimens of the femoral head in toh . both observed irregular new bone formation in apposition to trabeculae . ( 6 ) considered that microfracture might play a role in the early pathophysiology in toh , whereas plenk et al . ( 8) described new bone formation as microcallus - like , but considered that it was not associated with microfractures . in our case , irregular trabecular bone thickening was observed in both femoral heads in late stage of toh , and this disappeared gradually . in view of our ct findings , which suggested subchondral fracture in the right femoral head , we consider that irregular trabecular thickening is due to callus formation around injured trabeculae which is gradually remodeled . todd et al . ( 9 ) observed lesions of irregular bone accretion around trabeculae of the femoral head in cadavers and patients suffering from femoral neck fractures , osteoarthrosis , and rheumatoid arthritis . this suggests that some degree of fatigue type microfracture and repair occur continuously in normal healthy adults , but that when microfracture occurs massively , it might become symptomatic like toh . a sudden increase in body weight during the third trimester of pregnancy could exacerbate any microfracture process . the clinical course and image findings of toh closely resemble those of sffh ( 2 - 4 , 6 , 7 ) . the only difference between them is the presence of the subchondral fracture line on mr images in sffh . in cases with sffh , the presence of a fracture line has been reported to be detectable only on some slices of mr images made with various techniques including t1-weighted , t2-weighted , and gadolinium enhanced ( 1 , 10 ) . it is expected that it is more difficult or impossible to detect subchondral fracture line when fractures are incomplete . in the present case , we were unable to detect any definite evidence of subchondral fracture of the femoral head on mr images . however , on mpr ct images , we observed a linear dense sclerotic line suggestive of a fracture line in the right femoral head , and a definite area of irregular trabecular thickening in both femoral heads , which suggested a subchondral trabecular injury and subsequent repair . the presented case study confirmed the evidence of subchondral trabecular injury in toh which might be the milder form of injury than sffh . the authors recommend that ct be used to evaluate trabeculae of the femoral head of toh cases diagnosed by mri .
a 28-yr - old woman presented with both hip pain that started sequentially during the peripartum period . diagnosis of transient osteoporosis of the hip ( toh ) was made based on typical findings of plain radiographs and magnetic resonance images . the subchondral trabeculae of the femoral head were evaluated on serially taken coronal multiplanar reformation computerized tomogram images . at 4 weeks after pain onset , marked decrease in the sclerotic density with irregular discontinuation was observed in the primary compression trabeculae . at 12 weeks , a focal area of irregular thickening of trabeculae was observed . at 20 weeks , sclerotic density of trabeculae recovered markedly and the focal area of irregular trabecular thickening disappeared . at 1 yr , subchondral trabeculae recovered almost completely . the evidence of subchondral trabecular injury was observed in the femoral heads of toh .
the relationship between weight and blood pressure ( bp ) has been demonstrated by studies . in one study , a 10-kg increase in weight has caused a 3/2.3 mm hg increase in blood pressure . this increase has resulted in a 12% increase in the risk of coronary heart disease and a 24% increase in the risk of stroke . until the ideal body mass index is reached , for every10 kg of weight that is lost , the bp decreases 520 mm hg . as the visceral adipose tissue increases , so does bp . with an increase in visceral adipose tissue , there is increased intake of protein , calorie and carbohydrate , and increase in plasma catecholamines , sympathetic nerve system activity , and insulin secretion . angiotensin aldosterone system ( raas ) activation results in renal sodium retention and increased cardiac output . the increase in sympathetic activation causes insulin resistance and endothelial dysfunction , which consequently lead to an increase in peripheral vascular resistance . leptin is a mediator released from adipose tissue that has an appetite - reducing effect . in obese humans , leptin resistance and a positive association between leptin and bp have been demonstrated . the levels of angiotensinogen , renin , aldosterone , and angiotensin - converting enzyme have been shown to be significantly higher in obese women than in lean women . there is a linear relationship between weight loss and decreases in bp and this is related to the decrease in the visceral fat mass . when dietary treatment , medical treatment , and invasive or surgical interventions were compared for achieving weight loss , dietary treatment was found to be the most effective way to decrease bp . especially in the case of weight loss with sibutramine , no changes in bp were observed . approximately 10 kg of weight loss can decrease systolic bp by 520 mm hg . it causes a 4 mm hg decrease in average systolic bp and a 3 mm hg decrease in diastolic bp . studies have shown that regular aerobic exercise changes the vascular structure by improving endothelium - dependent vasorelaxation and increasing central artery compliance . exercise training also decreases the stimulation of the sympathetic nervous system and reduces the heart rate and cardiac output . therefore , aerobic exercise ( e.g. , brisk walking ) is recommended for a minimum of 30 min on most days of the week . excessive alcohol consumption causes an increase in bp . with alcohol consumption , the amount , duration , and the form of intake is important . in hypertensive patients , for example , in japan , the intake is 25 ml . in comparison , the intake is 17.7 ml in the usa and 10 ml in england . in a study with heavy drinkers , the relationship between alcohol intake and hypertension , stroke , and cardiomyopathy has been demonstrated . for this reason , mind and body exercises ( qigong , thai chi , transcendental meditation , yoga , breath exercises , and so on ) can be performed to lower hypertension . speaking therapy and stress management may also be helpful . particularly in traditional chinese medicine , there are several publications demonstrating that mind and body exercises can also be helpful in metabolic syndrome . one hundred and fifty ml of coffee includes 60120 mg of caffeine and 150 ml of tea includes 2040 mg of caffeine . this can increase bp by 2.4/1.2 mm hg . the effects on bp are eliminated with the long - term use of caffeine and nicotine . dark chocolate and cacao reduce blood pressure and this effect is due to flavonoids . a daily 1030 g intake of dark chocolate can significantly decrease bp . melatonin is secreted in the absence of light , leading to lower bp during sleep . these findings have led researchers to study melatonin . in hypertension studies , it has been demonstrated that melatonin moderately reduces bp . 25-hydroxyvitamin d levels should be maintained above 75 nmol / l ( 30 ng / ml ) . lifestyle modification measures are safe and effective . they can delay the occurrence of hypertension . although these non - pharmacologic interventions may be difficult for patients and physicians , they should be pursued .
hypertension is an important health problem . informative counseling is required for patients to completely understand the importance of non - pharmacologic treatments . lifestyle changes such as restriction of salt intake , exercise , restriction of alcohol intake , diet , and weight loss are included in all hypertension treatment guidelines . however , serious motivation is required from the patient and the physician to succeed in this . although the decrease in blood pressure may be limited with these measures , lifestyle modifications should be continued .
pulmonary tuberculosis is divided into primary and post - primary types according to pathogenic and radiological features . pleural involvement is generally seen in the form of a pleural effusion , which can be a component of both primary and post - primary tuberculosis . empyema , residual pleural thickening and calcification are other forms of pleural tuberculosis involvement and are generally seen in association with parenchymal disease . in approximately two - thirds of cases , there is no associated parenchymal lesion on the chest radiograph ; yet tubercle bacilli are frequently found in biopsy specimens of pleural tissue . we report an unusual form of pleural tuberculosis demonstrated by pleuroscopy and computed tomography ( ct ) presenting with multiple pleural nodules and masses with large hemorrhagic pleural effusion and without any evidence of either parenchymal or lymphatic disease . a 68-year - old male was admitted with complaints of gradually progressive exertional dyspnea and dry cough of three months duration . he had pleuritic chest pain , low grade fever and weight loss for one month . his routine hemogram , urine examination , renal and liver function tests and coagulation profile were normal except raised erythrocyte sedimentation rate . chest x - ray was suggestive of large right - sided pleural effusion . at thoracentesis , it was a lymphocytic exudate ( total protein 3.4 g / dl , sugar 76 mg / dl , cholesterol 67 mg / dl , lactate dehydrogenase 253 iu / l , adenosine deaminase 9 polymorphs : 5% , lymphocytes : 92% , cytology negative for malignant cells , gram stain and afb smear negative ) . post - aspiration chest x - ray showed multiple irregular pleural masses [ figure 1 ] . ct of the chest revealed multiple different size masses involving the parietal as well as visceral pleura [ figure 2 ] . post taping chest x - ray shows multiple irregular pleural masses computed tomography ( ct ) chest shows multiple different size masses on parietal pleura as well as on visceral pleura a medical pleuroscopy was performed using a flexible bronchoscope placed through the chest tube . multiple pleural - based ( parietal as well as visceral ) masses of varying sizes were observed . histopathological examination of the pleural biopsy from a parietal pleural mass revealed epitheloid cell granulomas indicative of tuberculosis [ figure 3 ] . the major differential diagnostic considerations in a patient with multiple pleural nodules and masses are malignant mesothelioma , pleural lymphoma , hematogenous pleural metastases , malignant thymoma and on rare occasion sarcoidosis.[38 ] although pleural tb infection is thought to result from the rupture of a subpleural caseous focus within the lung into the pleural space , it may occasionally be as a result of hematogenous dissemination or contamination from adjacent infected lymph nodes . pleural infection in tuberculosis is usually characterized by an effusion in early stages , or by diffuse pleural thickening and adhesions often with calcification in long standing cases . pleural thickening may also result from chronic inflammatory conditions , for example , hemothorax , empyema , tubercular effusion , or recurrent pneumothorax , often with calcification . in our case the provisional diagnosis was a pleural neoplasm ( primary or secondary ) based on clinical , radiological and pleuroscopic findings . to our knowledge , pleural tuberculosis presenting with multiple pleural nodules and masses without parenchymal involvement or lymphadenopathy have been reported rarely in the standard english medical literature,[1113 ] but pleural tuberculosis presenting as multiple pleural masses with large hemorrhagic pleural effusion has not been reported . it should be considered in the differential diagnosis of pleural masses without radiographically visible parenchymal lung disease even when there is large hemorrhagic effusion . confirmation of the diagnosis with sputum smears , culture , or histology is mandatory since treatment is highly successful .
tuberculous involvement of the pleura usually presents as pleural effusion , empyema or pleural thickening . pleural tuberculosis presenting with multiple masses without parenchymal involvement or lymphadenopathy has been reported rarely in the english literature . we report a case of a 68-year - old male with unilateral pleural tuberculosis presenting as multiple different sized nodular masses evident on computed tomography ( ct ) of the chest with a large hemorrhagic pleural effusion , which initially raised a possibility of mesothelioma . pleuroscopy also revealed multiple pleural masses . pathological examination of the biopsy specimen showed multiple epitheloid cell granulomas confirming the diagnosis .
a 73-year - old indian female patient presented with rapidly progressive painless swelling of face , mainly localized in the parotid and submandibular region bilaterally for the duration of one year . the patient had a history of fever for the most recent two months , with unexplained weight loss ( weight at the time of presentation was 35 kg ) . physical examination revealed the patient to be moderately built and poorly nourished with signs of anemia . the spleen was also palpable 10 cm below the costal margin , whereas the liver was just barely palpable . further systemic examination including that of the respiratory , cardiac , and central nervous systems was normal . extraorally , she had a diffuse firm swelling of both the parotid and submandibular glands ( fig . 1a ) , and a rubbery consistency of the cervical lymph nodes along with those of the submental , submandibular , and jugulodiagastric groups . intraorally , two soft , non - tender swellings of about 23 cm were seen in the buccal vestibule in the region of the left upper and lower second molar . a routine hemogram showed pancytopenia with a hemoglobin level of 8.8 gm / dl , a total leukocyte count of 2.510/mm and platelet count of 1.0310/l . a lactate dehydrogenase level of 621 iu / l was noted and tests for human immunodeficiency virus ( hiv ) i and ii were found to be non - reactive . high resolution ultrasonography and color doppler sonography studies confirmed the enlargement of the bilateral parotid and submandibular glands , and also the buccal and lingual lymph nodes ( figs . the cervical group of lymph nodes revealed similar enlargement of multiple nodes belonging to ia , ib , iia , and iib levels along with the pre- and post - auricular and supraclavicular group of lymph nodes . ultrasonography of the abdomen confirmed the enlargement of the spleen , which measured 17 cm . contrast - enhanced computed tomography confirmed the diffuse symmetrical enlargement of the parotid and submandibular glands ( figs . 3a and b ) , while asymmetric thickening of the subglottic larynx and right aryepiglottic fold were evident with narrowing of the subglottic lumen . the ocular adnexa of the left orbit were involved , presenting as a bulky lacrimal gland . fine needle aspiration cytology ( fnac ) from the right parotid and submandibular glands , buccal vestibule , and tongue nodule showed a monomorphic population of lymphoid cells with the absence of reed - sternberg cells , which was suggestive of non - hodgkin 's lymphoma . incisional biopsy of the right submandibular lymph node was performed , which revealed diffuse infiltration by sheets of a monomorphic population of moderately large cells , with moderate cytoplasm and large round vesicular nuclei having prominent nucleoli . 4b ) and were immunonegative for cd10 , cd3 , cd5 , cd23 , and cyclin d1 . the mib-1 labeling index ( for proliferation marker ki-67 ) was approximately 10 - 15% . diagnosis of nhl - diffuse large b - cell type was concluded based on fnac and biopsies of the submandibular glands , parotid glands , and lymph nodes , supported by immunohistochemical positivity for cd20 marker . non - hodgkin 's lymphoma arises from a lymphocyte progenitor and comprises a heterogeneous group of highly diverse malignancies.2 25 - 40% of nhls are extranodal in origin4 and usually manifest in the gastrointestinal tract , followed by the head and neck region.5 nhl mostly occurs in the pediatric age group in the head and neck region;6 however , it may be seen in an older age group , such as in the present case of a 73-year - old female . the etiological factor for primary lymphoma of the salivary gland region is unclear.3 oral lymphomas are frequently seen with acquired immune deficiency syndrome ( aids ) . in certain individuals , it might serve as the first presentation of aids.7 the present case was seronegative for hiv . the most common presentation of primary oral and paraoral lymphoma is a painless local mass with superficial ulceration.7 in the present case , patient presented with progressive painless swelling of the face involving mainly the parotid and submandibular glands . lymphoma of the ocular adenexa is the most common in those aged over 60 years . orbital lymphoma can involve the lacrimal gland , extraocular muscles , orbital fat , eyelids , and conjunctiva.8 in the present case , involvement of the larynx , orbit , and oropharynx were suspected based on the history and later confirmed by imaging . based on the morphology , cell lineage , and immunohistochemical findings , the present case was categorized as aggressive nhl of diffuse large b - cell type . to determine the prognosis and to guide therapy for nhl the ann arbor staging system9 is one of the most widely used systems , and includes physical examination , hematological tests , imaging studies , and selective biopsies.10 according to the ann arbor system , stage iveb ( stage iv : diffuse or disseminated foci of involvement of one or more extralymphatic organs or tissues ; e : extranodal organ involvement ; b : presence of systemic symptoms like fever and loss of weight ) was ascribed . all ipi parameters were found to be positive in this case , thus categorizing our patient as a high risk case . staging of salivary gland lymphoma plays a major role in management and enables more favorable prognosis.11 nhl is associated with significant morbidity . early stages of nhl ( minimal lymph node involvement ) are more manageable with a greater prospect of long - term disease - free survival , whereas advanced stages ( widespread in the lymph nodes ) of the disease have a lower prognostic index . such patients are also vulnerable to infectious diseases that may involve multiple organ systems ( e.g. central nervous system , liver).12 a diagnostic dilemma often occurs for oral physicians when these lymphomas occur at extranodal sites.13 the present case is disseminated nhl with predominant involvement of the salivary glands . though the involvement of both nodal and extranodal sites was present , the patient had complained mainly of enlargement of the salivary glands . since nhl constitutes only 1.7% of salivary gland malignancies,14 a clinical diagnosis of lymphoma is rarely suspected at initial presentation . in conclusion , the present case emphasizes the importance of imaging and prognostic markers for staging , which is essential for management .
non - hodgkin 's lymphoma ( nhl ) constitutes a group of malignancies those arises from cellular components of lymphoid or extranodal tissues . the head and neck is the most common area for the presentation of these lymphoproliferative disorders . primary involvement of salivary glands is uncommon . this report described a case of a 73-year - old female patient who presented with involvement of both nodal and extranodal sites , with predominant involvement of salivary glands . the tumor staging worked up along with imaging , histopathological , and immunohistochemical findings were discussed . computed tomographic images showed the involvement of waldeyer 's ring , larynx , orbit , and spleen . this report described imaging and prognostic tumor markers in diagnosing , treatment planning , and prognosis .
variant or prinzmetal 's angina , characterized by transient resting st - segment elevation on electrocardiogram ( ecg ) , was first described by prinzmetal et al.1 ) in 1959 . he emphasized the occurrence of resting angina and the observation of reversible st - segment elevation during angina . maseri et al . reported that coronary artery vasospasm occurred spontaneously during coronary angiography in only 17 of 107 variant angina patients.2 ) in 1949 , stein3 ) proposed that ergonovine may be clinically useful in the diagnosis of patients with suspected coronary artery vasospasm . currently , ergonovine provocation test is a mainstay of clinical practice for diagnosing variant angina . here , the case report of a male patient who presented with atypical chest pain and underwent coronary angiography and ergonovine provocation test is described . coronary angiography did not show any significant stenosis in coronary arteries and ergonovine provocation test was negative . after 5 years , he visited the emergency room ( er ) with an acute myocardial infarction due to coronary artery vasospasm , drawing attention to the false negative results of the ergonovine provocation test . a 49-year - old - man visited our outpatient clinic on october 1 , 2007 with atypical chest pain that had started 2 months before . echocardiography also showed normal left ventricular systolic function and there were no regional wall motion abnormalities . he was not taking any medication except for a budesonide inhaler and mometasone ( topical corticosteroid ) for treatment of asthma . coronary angiography showed no significant lesions in the coronary arteries and the intravenous ergonovine provocation test was negative ( fig . 1 ) . there was no chest pain , st - segment changes , or coronary artery vasospasm noted during the ergonovine provocation test . we did not prescribe any antianginal medication such as nitroglycerine before the ergonovine provocation test , which would have affected the results of the test . the gastrointestinal tract was thought to be the source of the pain and he was prescribed a proton pump inhibitor and prokinetics for pain relief . after 5 years , on january 15 , 2012 , he visited the er with sudden onset of chest pain while sleeping . the characteristics of the chest pain were very similar to the pain that occurred in 2007 . in the er , the chest pain was relieved by sublingual nitroglycerin and he was admitted to the coronary care unit . after several hours , the chest pain recurred and the patient complained of dizziness , nausea , and vomiting . his blood pressure dropped to 76/41 mm hg , and he had symptomatic sinus bradycardia . 2 ) , but troponin i was elevated to 90.3 ng / ml . suspicious of non st - segment elevation myocardial infarction combined with cardiogenic shock , we performed coronary angiography . coronary angiography showed no significant stenosis of the left main , left anterior descending , or left circumflex coronary arteries ( fig . 3 ) , but total occlusion with distal thrombolysis in myocardial infarction flow grade 2 in the distal right coronary artery , posterior descending artery , and posterolateral branch bifurcation segment was observed ( fig . 4 ) . after intra - coronary nitroglycerin injection , the occluded segment was dilated ( fig . 5 ) and the incidence of variant angina is 2 - 20% in western populations4 ) and 4.4% in the korean population.5 ) the incidence appears to have declined over the past few decades , which may be attributable to smoking cessation and more frequent use of calcium channel blockers.6 ) the mechanism of variant angina is coronary artery vasospasm with or without atherosclerotic coronary artery disease.7 ) calcium channel blockers , which are extremely potent in preventing episodes of coronary vasomotor tone abnormalities , are especially effective in preventing episodes of myocardial ischemia in variant angina patients . -adrenergic blocking agents and therapies directed against platelet aggregation are ineffective in preventing episodes of ischemia in these patients.8 ) variant angina can lead to ventricular fibrillation and even sudden cardiac death . the key to diagnosing variant angina is to detect episodic st - segment elevation often accompanied by chest pain , usually occurring at rest . however , as in our case as well as the studies by feldman et al.,9 ) there are cases of variant angina without st - segment changes . ergonovine is thought to act directly on vascular smooth muscle to increase arterial and central venous pressure , and to decrease venous compliance . intravenous injection of ergonovine is most commonly used to provoke coronary artery vasospasm.11)12 ) according to waters et al.13 ) the sensitivity of intravenous ergonovine provocation test was 100% using anginal chest pain as the diagnostic criterion and 94% using st - segment elevation . after a negative ergonovine provocation test , our patient presented with acute myocardial infarction accompanied by cardiogenic shock and symptomatic bradycardia due to coronary artery vasospasm . it is possible that the patient had suffered from non - anginal chest pain 5 years ago and then developed coronary vasospasm 5 years later . however , the characteristics of chest pain in 2007 and 2012 were very similar in nature ( crushing ) , location ( substernum ) and onset time ( in the early morning ) , indicating that the two events shared a common etiology . the negative result on the ergonovine provocation test in our patient could be related to the sensitivity of the test . in 2004 , sueda et al.12 ) reported the incidence of coronary artery vasospasm after intracoronary ergonovine injection in 596 consecutive japanese patients to be 2.2 - 2.6 times higher than previous reports of intravenous ergonovine provocation . the clinical impact of intracoronary ergonovine provocation test in diagnosing variant angina needs further study . our case exhorts that ergonovine provocation test is far from perfect , and one should always consider the possibility of false negative results , and intracoronary ergonovine provocation tests may be helpful where appropriate . vasodilators such as calcium channel blockers or nitrates , as well as discontinuation of smoking and alcohol consumption should be considered in patients with a clinical manifestation suggestive of variant angina , even after a negative ergonovine provocation test .
ergonovine provocation test is known to be very sensitive for diagnosing variant angina . the patient described in this study initially presented with atypical chest pain and underwent coronary angiography and ergonovine provocation tests , which were negative . the patient was subsequently prescribed a proton pump inhibitor and prokinetics for pain relief , but then presented with acute myocardial infarction and cardiogenic shock due to coronary artery vasospasm 5 years later . this case suggests that ergonovine provocation test generates false negative results , which can lead to unwanted outcomes . even with a negative ergonovine provocation test , prescription of calcium channel blockers or nitrates should be considered in patients with a clinical history suggestive of variant angina .
it is not uncommon to encounter patients in critical care unit who develop sudden problem with ventilation while on mechanical ventilation . causes may vary from patient related to ventilator related notably poor pulmonary compliance , increased airway resistance , and problem with ventilator , circuit or its settings . to manage the patient in this scenario where the ventilator is unable to deliver predetermined tidal volume failing to appropriately recognize the underlying issue may lead to inappropriate management and its consequences . a 40-year - old female admitted with complaint of shortness of breath for last 2 - 3 day , associated with cough and sputum streaking with blood for last around 15 days . she had pulmonary tuberculosis in the past and had destroyed lung on the right side with mediastinal shift [ figure 1 ] . on the left side , she was having pulmonary infiltrates , for which she was receiving antibiotics . her condition further deteriorated to the point that the patient was intubated with an endotracheal tube ( ett ) number eight and mechanically ventilated with sedation and paralysis . mediastinal shift with tracheal deviation after a few hours of mechanical ventilation , her saturation began to drop , and blood pressure increased to more than 200 mmhg systolic with tachycardia . on realizing the problem that tidal volume however , it was found that the suction catheter could not be passed as an obstruction was encountered . on removing the tube , there was no blockage in the tube but now patient was reintubated with a seven number ett . this was followed by delivery of set tidal volume and normalization of oxygen saturation and blood pressure . further into the patient 's management , after a few hours , patient developed the same problem of worsening oxygen saturation with rise in blood pressure and tachycardia . again , the delivered tidal volume was not adequate and less than the predetermined value . on looking specifically at airway pressure alarm limit nothing relevant found which could be the reason for failure to deliver predetermined tidal volume . now , it was suspected that there could be impingement of ett bevel on the tracheal wall after intubation due to the anatomical distortion of trachea . gradual withdrawal of the ett tip up to just below the vocal cords did not restore the ventilation . in neonates , the distal orifice of an uncuffed ett abuts the tracheal wall and causes frequent obstruction . but for the adults , a cuffed ett does not allow free movements of the ett in the trachea and obstruction does not occur as often . head movement shifts the secured ett within the trachea . yet for adults , anatomical deviations from normal seem to cause malposition or difficulty in ventilation . they also did several manipulation to overcome the problem including withdrawing of tube as we did but to no avail . the most likely reason of persisting problem in our patient was right - sided mediastinal shift which was creating an angle in tracheal anatomy in such a way that left - sided bevel was abutting tracheal wall . we suspected this on the basis of not able to negotiate the catheter and left - sided bevel in a patient with right mediastinal shift . furthermore , there was no other apparent cause and problem was overcome by cutting bevel of tube . reported a case of tube orifice abutting on the tracheal wall in a patient with forestier 's disease - diffuse idiopathic skeletal hyperostosis . tracheal deviation due to destroyed lung may cause this problem due to tube tip abutting tracheal wall .
a 40-year - old female presented with respiratory difficulty , cough and sputum with blood streaking . her right lung was destroyed , and trachea was shifted to the same side . on mechanical ventilation , she developed hypoxia and rise in blood pressure . ventilator was not delivering set tidal volume . after looking into the cause , it was decided to reintubate the patient with new endotracheal tube after cutting bevel . thereafter , there was successful ventilation .
catabacter hongkongensis is a strictly anaerobic , catalase - positive , motile and nonsporulating gram - positive coccobacillus . it was first isolated in 2007 in hong kong and canada in blood cultures from samples taken from patients with sepsis ; it has since been identified in fewer than 15 patients worldwide , . in europe , the only report of c. hongkongensis in humans was published in 2007 in france , and its genome has been reported in water samples in spain . five more c. hongkongensis infections were recently described in hong kong . to our knowledge , this microorganism has never been described in italy . a 55-year - old man without any previous comorbidity was admitted to the intensive care unit in ravenna after a motorcycle accident , in which he experienced sternal and humeral lesions and d4 and d9 vertebral fractures . a hypertensive pneumothorax and a glasgow coma score of 14/15 were also present , accompanied by intracapsular spleen haematoma with multiple fractures of the pelvis . five days later , empirical therapy with ceftazidime and gentamicin was administered for 18 days . four days after that , an abrupt onset of fever ( temperature > 38.5c ) accompanied by clinical features of septic shock appeared ( with severe leucocytosis and low platelet count ) , and the patient was provided vasoactive amines with mechanical ventilation . a monolateral purulent pleural suffusion was also present on the right , as demonstrated by tomography , and thoracic drainage was applied , with copious purulent leakage . two sets of blood samples were taken for culture via a central arterial catheter as a result of the poor condition of peripheral veins . after 82 hours of incubation ( virtuo system ; biomrieux , marcy ltoile , france ) , one bottle ( anaerobic ) showed growth of pleomorphic gram - positive microorganisms . identification by matrix - assisted laser desorption / ionization time - of - flight mass spectrometry ( maldi - tof ) , performed following standard and rapid procedures , failed several times because no spectra matched with the isolate . blast analysis ( http://blast.ncbi.nlm.nih.gov ) showed 100% identity with c. hongkongensis ( strain hku16 , accession no . the metabolic profile was evaluated by api20a ( biomrieux ) , showing a similar pattern to already published data . the antimicrobial susceptibility testing was performed by etest and interpreted following european committee on antimicrobial susceptibility testing criteria ( http://www.eucast.org/clinical_breakpoints/ ) . this was hampered by the slow and difficult growth of the isolate , which took over 10 days . after 2 weeks , empirical treatment with vancomycin and meropenem was discontinued , and the patient recovered from sepsis . c. hongkongensis is an environmental microorganism that may occasionally be present in intestinal flora , . most of the reported cases of blood infection caused by this microorganism are connected with malignancies ( either metastatic or involving peritoneal organs ) and with acute intestinal and biliary tract inflammation . in our case , none of the above - reported favouring conditions was present before the accident , but the patient had extensive trauma involving the area of the lower diaphragm . despite the wide capability of maldi - tof to identify anaerobic bacteria , it is noteworthy that this technique was unable to identify c. hongkongensis . we suggest that the use of sequencing be considered when anaerobic microorganisms that can not be identified by routine technologies are isolated from patients with abdominal sepsis .
the first isolation of catabacter hongkongensis in italy is reported . pleomorphic gram - positive rods were grown in blood cultures from samples obtained from a 55-year - old patient admitted to the intensive care unit with sepsis after he experienced massive thoracic and abdominal trauma . the identification was obtained by amplification and sequencing of the 16s rrna gene .
optic nerve head drusen ( onhd ) are hyaline material calcificated deposits due to an axoplasmic transport alteration in the presence of a small scleral canal.1,2 they are a casual fundus finding but are rarely complicated with a peripapillary choroidal neovascularization , an anterior ischemic optic neuropathy , or a central serous papillopathy ( csp).3 herein , we report a patient with bilateral peripapillary and right macular serous detachment as first manifestation of onhd . a 38-year - old man presented with complaint of progressive loss of vision for five days in his right eye . past systemic and ocular history was unremarkable , but flu was reported three weeks before . pupils were of equal size and constricted briskly without a relative afferent pupil defect when exposed to direct light . the right eye showed a serous detachment extending from optic nerve to macula ( figures 1 and 3 ) . fluorescein angiography showed autofluorescence of the disk drusen ( figure 4 ) and abnormal hyperfluorescence from temporal optic discs in early phases ( figure 5 ) . our patient also presented one hyperfluorescent focus in lower part of right optic nerve head ( figure 5 ) and multiple pinpoint foci of hyperfluorescence within temporal inferior veins in late frames ( figure 5 ) . a cranial tomography scan showed calcium density bodies at both head optic nerves ( figure 6 ) . after two months most of subretinal fluid disappeared ( figure 7 ) with no treatment and visual acuity improved to 20/20 in both eyes . evidence suggests that axoplasmic transport alteration and axonal degeneration are involved in disc drusen formation . most patients are asymptomatic4 and visual acuity is normally well preserved.5 when a rapid decrease of visual acuity appear in these patients , the differential diagnosis should include anterior ischemic optic neuropathy , central retinal artery or vein occlusion , peripapillary choroidal neovascularization,6,7 and csp.8,9 central serous chorioretinopathy ( csc ) is a condition characterized by accumulation of transparent fluid under the neurosensory retina , retinal pigment epithelium ( rpe ) , or both , causing a circumscribed macular detachment or rpe detachments . the pathophysiology of csc remains poorly understood , but focal choroidal hyperpermeability has been recently implicated.10 an exceptional form of csc is csp that may occur in normal optic disc or in those with a pit or coloboma . in our patient , spontaneous reabsorption of fluid and benign evolution ruled out a peripapillary choroidal neovascularization , suggesting csp as a final diagnosis . moisseiev et al described a case of optic disc drusen and csp.11 we report the second case in literature describing simultaneous occurrence of optic nerve drusen and csp , but the first one in which both bilateral peripapillary and macular detachment are observed . optic nerve drusen seems to alter the normal barrier between the prelaminar optic nerve and the peripapillary retina . this anomalous peripapillary area joined to focal hyperpermeability seen in csc may act together in the subretinal fluid production in patients with onhd . it is critical to correctly diagnose patients with csp to avoid unnecessary work - up and overlooking potential serious conditions such as true papilledema . csp should be considered as a potential cause for acute loss of vision in patients with optic nerve head drusen .
we report a 38-year - old man with a complaint of blurred vision in his right eye for the previous 5 days . he had bilateral optic disc drusen . fluorescein angiography revealed multiple hyperfluorescent foci within temporal optic discs and temporal inferior arcade in late phase . optical coherence tomography showed bilateral peripapillary serous detachment as well as right macular detachment . this is the first reported case of a concurrent peripapillary and macular detachment in a patient with central serous papillopathy by optic disc drusen . central serous papillopathy is an atypical form of central serous chorioretinopathy that should be considered as a potential cause of acute loss of vision in patients with optic nerve head drusen .
kaposi 's sarcoma ( ks ) is a spindle - cell malignant low - grade vascular tumor associated with human herpesvirus 8 ( hhv-8 ) . it was first published by the dermatologist moritz kaposi in the journal archiv fr dermatologie und syphilis , in 1872 . in the current literature , four main types of ks are described : ( 1 ) epidemic or aids - related ks , ( 2 ) ks in immunocompromised patients , ( 3 ) classic ks , and ( 4 ) endemic or african ks . chronic lymphocytic leukemia ( cll ) is presented with an increasing number of functionally incompetent mature lymphocytes which are of monoclonal origin , mainly in the peripheral blood , bone marrow , and all throughout the reticuloendothelial system . herein we report an interesting case of ks which was diagnosed during the course of cll as a secondary malignancy . a 64-year - old female case presented with complaints of bluish and indurate lesions all over both lower extremities ( fig . after initial evaluation , biopsy was done from the lesions and ks was diagnosed ( fig . the patient has had a history of cll since the year of 1999 but she has not needed any active drug treatment since 1999 . she did not have any complication such as bacterial infection or hemolytic anemia throughout the disease course . there was no risk factor in her personal history that disposed the patient to aids . quantitative immunoglobulin m and a levels were decreased , whereas immunoglobulin g level was normal . although all tests were negative for systemic involvement of ks , ks lesions have an allover pattern in both extremities , so we planned to administer systemic chemotherapy which consisted of anthracycline , vincristine , and bleomycine . after three cycles of systemic chemotherapy , the lesions had completely disappeared and there was no toxicity of chemotherapeutics . although in the literature , there is a strong relationship between immunosuppression after solid organ transplantation and ks , there is no strong data that shows a relation between cll and ks . patients with cll are prone to the viral , bacterial , and opportunistic infection even without taking any chemotherapeutics . although immunosuppression in cll can dispose the patient to the infection , can it cause tending to the formation of secondary malignancy ? reported that ks is 5 times more common in the cll population than the normal population . in this surveillance analyzed 16,367 patients with cll , so we can not just say that the increased incidence of ks in cll patients is an incidental finding . median latency time between diagnosis of cll and preceding ks was 75 months in this unique study . it is true for all types of ks including aids - related ks , ks in immunocompromised patients , classic ks and endemic or african ks . control of hhv-8 infection is mediated by an antiviral t - cell response to hhv-8 proteins . latent hhv-8 infection of endothelial cells can cause subsequent conversion of endothelial cells into the spindle cells . then the proliferative phase started , and vegf secretion increased from the infected endothelial cells . this activation plays a pivotal role in the development of the final tumor . is this a mechanism that causes ks in cll patient ? we know that there is an imbalance in circulating t - cell subpopulations in cll . so theoretically , latent hhv-8 infection can be reactivated in cll patients and can trigger ks lesions . in conclusion , cll can dispose to the development of ks but the exact mechanism is not explained as much as ks preceding solid organ transplantation . so physicians should be very careful about the probability of ks in newly and progressively developing skin lesions in cll .
cutaneous manifestations can occur in the wide range of internal malignancy . they can occur by metastases or local spread , direct infiltration , or a site of primary malignancy itself . sometimes these manifestations are related with an underlying malignancy but they do not contain malignant cells as paraneoplastic dermatological syndromes . chronic lymphocytic leukemia ( cll ) is the most common leukemia all over the world . cutaneous lesions occur in up to 25% of patients . most commonly seen cutaneous lesions in cll are those of infectious or hemorrhagic origin . skin cancer risk was also increased eightfold in cll when compared with normal population , so cutaneous lesions in cll can be the first manifestation of secondary skin malignancy . herein , we report an interesting case of kaposi 's sarcoma which was diagnosed during the course of cll .
there are three conditions of intersex associated with persistence of derivatives of mullerian duct : persistent mullerian duct syndrome ( pmds ) , mixed gonadal dysgenesis , and dysenteric male pseudo - hermaphroditism . pmds is typically characterized by undescended testes with presence of small underdeveloped uterus in genetically male infant or adult with normal external genitals and virilization . the condition is usually detected during evaluation for undescended testes / infertility or during surgery for other abdominal condition . treatment is controversial , and various treatment options include gonadal biopsy , orchidopexy , orchidectomy , excision of derivatives of mullerian duct , etc . laparoscopy is very useful in such cases as it is diagnostic and therapeutic at the same time and also offers advantages of minimally invasive approach . we hereby report a rare case of pmds with left inguinal hernia along with seminoma of right testes managed by laparoscopic approach . a 38-year - old male was evaluated in an emergency department for a painful swelling in left inguinal region for 5 days . detailed history , revealed associated infertility , investigated elsewhere having 14 years of married life . he reported normal sexual activity and had well developed secondary sexual characters with male pattern voice . per abdominal examination was unremarkable . on genital examination , he had male genitals with normal appearing penis along with bilaterally undescended testes and underdeveloped scrotum . he also had left sided complete inguinal hernia , which was partially reducible without any signs of strangulation . on reviewing old medical records , it was found that he had bilateral intra - abdominal testes with presence of rudimentary uterus . cross - sectional imaging confirmed presence of the uterus with bilateral intra - abdominal testes located along the iliac vessels [ figure 1 ] . multi - detector computed tomography pelvis : oblique coronal section showing presence of bilateral intra - abdominal testes with uterus . right iliac vessels , liv left iliac vessels patient planned for diagnostic laparoscopy with bilateral orchidectomy and possible hysterectomy with hernia repair . diagnostic laparoscopy revealed presence of small underdeveloped uterus with bilateral adenexa and undescended testes [ figure 2a ] . there was the presence of left indirect inguinal hernia with omentum herniating into the sac [ figure 2b ] . subsequently intra - operative picture : ( a ) presence of underdeveloped uterus with bilateral adenexa and bilateral undescended testes . surprisingly , section from right testes had showed presence of seminoma , stage i , which was confirmed by immunohistochemistry . based on clinical examination , intra - operative findings and histological features , we propose a diagnosis of pmds . persistent mullerian duct syndrome is an uncommon variety of pseudo - hermaphroditism seen in phenotypically normal males with chromosome 46 , xy . this condition occurs due failure of fetal anti - mullerian hormone ( amh ) to induce regression of mullerian ducts either due to mutation in gene coding for amh ( chromosome 19 ) or amh receptors ( chromosome 12 ) . these individuals develop testes and have normal androgenic function with the development of near normal wolffian duct derivatives and normal external genitals . interestingly , all of them have either unilateral or bilateral undescended testes , probably due to close structural relationship between the gonad and the retained mullerian duct structures and sometimes it is associated with inguinal hernia as in this case . almost always there is presence either of derivatives of mullerian duct , that is , uterus , cervix and fallopian tubes , sometimes pulled inside ipsilateral or contralateral inguinal canal , condition referred to as hernia uteri inguinalis . few cases of preserved fertility in pmds have been reported , however , infertility is common due to chronic cryptorchidism and obstruction of vas deference or other parts of male excretory ducts . there is a definite risk of malignant transformation in undescended testes as seen in cases of cryptorchidism . malignant transformation of mullerian duct derivatives is rare with only two cases reported in the literature . surgical management of this condition is controversial and depends on the status of the gonads , degree of development of the mullerian derivatives , fertility , and age of the patient . derivatives of mullerian ducts are carefully excised to preserve vas and to improve the chances of fertility . orchidectomy is advised for testes that can not be mobilized out of the inguinal canal so also for an adult where the risk of malignant transformation of the undescended testis is high . in this patient , decision for orchidectomy was taken in view of high risk of malignancy which was subsequently proved to be present on histopathological analysis , while excision of the mullerian derivatives was based on the facts that apart from it being a rudimentary and nonfunctional organ , there is a small but definite risk of malignant change . to conclude , in given scenario laparoscopy has offered a unique advantage in terms of better visualization , less blood loss , combining multiple procedures along with early return to work and excellent cosmetic outcome .
persistent mullerian duct syndrome ( pmds ) is one of the three rare intersex disorders caused by defective anti - mullerian hormone or its receptor , characterized by undescended testes with presence of underdeveloped derivatives of mullerian duct in genetically male infant or adult with normal external genitals and virilization . this population will essentially have normal , 46(xy ) , phenotype . we hereby present a case of pmds , presented with incarcerated left inguinal hernia associated with cryptorchidism and seminoma of right testes . patient underwent laparoscopic hernia repair with bilateral orchidectomy and hysterectomy with uneventful postoperative recovery . here we highlight the importance of minimal access approach for this scenario in terms of better visualization , less blood loss , combining multiple procedures along with early return to work and excellent cosmetic outcome .
a 17-year - old male patient visited our institution with a complaint of a painful movable mass on the lateral distal aspect of the right femur that was not caused by trauma . the mass had been slowly growing for 6 months and started to cause increasing pain from one month ago . a firm and movable mass with a size of a quail egg was palpated in the lateral distal aspect of the right femur . neither muscle weakness and numbness nor abnormal sensation was observed in the lower leg . in the absence of abnormal findings on radiography , ultrasound was performed , in which a cystic tumor approximately 4.12.5 cm in size was identified in the quadriceps femoris ( fig . was considered unnecessary because it had a relatively distinct border and was well - encapsulated . exploration and excision of the mass were performed under the assumption of a vascular lesion or a lipoma within the vastus lateralis of the quadriceps femoris . noninvasive methods such as aspiration and steroid injection could be an option , but surgical excision was our treatment of choice in order to reduce the possibility of recurrence . intraoperatively , spindle - shaped swelling was observed and a firm mass was palpated in the vastus lateralis of the quadriceps femoris . the mass could be dissected with ease from the surrounding tissue and had a stalk as other ganglion cysts . when the muscle sheath was removed , the mass was found to originate from the muscle , not from the muscle sheath . in the naked eye , the intramuscular cystic mass was 3.42.21.4 cm in size , weighed 4.7 g , and had some muscle tissue on its smooth surface ( fig . 2 ) . when dissected upon excision , clear jelly - like fluid was noted in the sac . the biopsy results showed that the cystic wall was composed of fibrous tissue , lacked a synovial lining , and had muscular tissue on the surface , which led us to the diagnosis of an intramuscular ganglion cyst ( fig . there has been neither recurrence nor complications for the 1 year and 6 months of follow - up period . ganglion cysts are the most common soft tissue lesions of the hand that are composed of a fluid - filled sac connected to a synovial sheath , a tendon sheath , or a tendon . some studies associate them with mucoid degeneration of soft tissues , outpouchings of tissues through defects in the joint capsule or tendon sheath , or a trauma3 ) . the most common area of occurrence is the radial side of the extensor digiti communis tendon on the dorsum , followed by the area between the flexor carpi radialis and the abductor pollicis longus tendons on the volar side of the wrist4 ) . ganglions cysts arise from joints in most cases and rarely from the tendon sheath , cartilage , cruciate ligaments , nerves , and muscles5 - 7 ) . although most of the ganglia around the knee joint are located in the tendon sheath or joint capsule , they can form in the meniscus , anterior cruciate ligament , posterior cruciate ligament , and common peroneal nerve9 ) . intraosseous ganglion cysts that are characteristically located in the epiphysis of long bones are mostly solitary and involve the distal end of the tibia . currently , there are only 2 reported cases of ganglion cysts in the quadriceps femoris that were diagnosed using plain radiography , ultrasound , and computed tomography . in our patient , the majority of ganglion cysts are palpable tender lumps without any accompanying symptoms . however , when a ganglion cyst exerts pressure on the nerves or blood vessels , pain , numbness , and muscle weakness may occur or tenderness and discomfort during joint motion may present from the initial stage . although ultrasound is a valuable diagnostic tool , it is limited in its ability to evaluate the relationship between a cyst and adjacent soft tissues . mri is essential for the differential diagnosis from other masses such as vascular lesions and lipomas . in our patient , mri was considered unnecessary because the mass was seen well - encapsulated with a distinct border on ultrasound . it is important to identify during surgery whether a lesion is a mass , has a connection with a joint , or is associated with intraarticular disease and to perform thorough excision in order to reduce the likelihood of recurrence . ganglia rarely recur with proper curative measures , but inappropriate treatment methods can result in a recurrence rate of 50%3 ) . the available treatment options range from non - surgical methods such as rupture by direct pressure , aspiration with or without a steroid injection , and draining using threads to surgical excision with or without arthroscopy . the cure rate of ganglion cysts has no association with gender , age , and size and location . nelson et al.4 ) reported that the cure rate was 94% after excision under general anesthesia or axillary nerve anesthesia , 84% after excision under local anesthesia with a tourniquet in place , and 65% after rupture by direct pressure or aspiration with a steroid injection . in the study by aydin et al.10 ) on 40 cases of excision of ganglion cysts of the hand , the recurrence rate was 22% , damage to the median palmar cutaneous nerve was observed in 10% , radial nerve damage was present in 5% , and joint stiffness occurred in 5% . they cautioned that the possibility of recurrence and other complications should be taken into consideration in determining a surgical option for the treatment of a ganglion cyst . intramuscular ganglion cysts are rare with only a few cases involving the hand present in the literature . , we described a case of an intramuscular ganglion cyst in the vastus lateralis of the quadriceps femoris that was successfully treated with excision without complications .
ganglion cysts are common lesions that are most often found around the joints of the hands and feet . ganglia around the distal femur usually occur within the synovial membrane or tendon sheath , but rarely within muscles . several cases of intramuscular ganglions in the hand and wrist have been reported , but a ganglion cyst in the quadriceps muscle has rarely been addressed in studies . in this report , we present a 17-year - old patient with a painful movable mass in the intramuscular area of the quadriceps femoris that was diagnosed by ultrasound and treated by excision and biopsy .
a 28-year - old woman in the 13th week of pregnancy was referred to the emergency department of our hospital for sudden abdominal pain . she had neither a medical nor surgical history and had thus far experienced a normal pregnancy . upon arrival , her blood pressure was 114/56 mmhg , pulse rate was 96/min , and body temperature was 35.8c . laboratory data showed a white blood cell count of 15,100/mm , hemoglobin level of 13.4 g / dl , and c - reactive protein level of 0.3 mg / dl ( normal , < 0.3 mg / dl ) . transvaginal ultrasonography ( us ) revealed a dilated bowel without ascitic fluid and no abnormal findings in her infant . she was admitted to our hospital for intensive observation and because of the possibility of miscarriage or enteritis . transabdominal us was performed after admission because of rapidly worsening abdominal pain with diffuse and rebound tenderness . an x - ray of the abdomen showed dilatation of the small intestine with air fluid levels and the presence of a sigmoid colon loop in the mid - abdomen ( fig . was performed to reach an accurate diagnosis after obtaining detailed informed consent regarding the risk of radiation exposure . the ct scan revealed 2 abnormal findings : ( 1 ) whirling of the sigmoid mesocolon and mesenteric root , suggestive of strangulation , and ( 2 ) a dilated loop of the sigmoid colon in the right lower abdomen , suggestive of a sigmoid volvulus ( figs . she underwent emergency exploratory laparotomy , and 2 l hemorrhagic ascitic fluid with gangrenous change in the ileum and sigmoid colon was identified ( fig . the gangrenous ileum and sigmoid colon were treated by resection with primary anastomosis , respectively . an abdominal x - ray reveals a sigmoid colon loop ( white arrows ) in the mid - abdomen . ( a ) contrast - enhanced ct scan ( axial view ) shows a dilated loop of intestine with a surrounding whirl ( small white arrows ) . ( b ) contrast - enhanced ct scan ( coronal view ) shows distended sigmoid colon ( white arrowhead ) . ( a ) intraoperative photograph shows gangrenous change in the ileum and sigmoid colon ( black arrows ) . ( b ) ileosigmoid knot ( white arrows ) with gangrenous ileum and sigmoid colon . isk is a rare form of bowel obstruction in which the ileum wraps around the base of the sigmoid colon . isk is predominately seen in male patients ( 80.2% ) with mean age of 40 years ( range , 490 years ) . several studies have reported the relationship between dietary habits and isk in that ingestion of a single daily meal was associated with a higher incidence of isk . when a semiliquid , bulky meal progresses into the proximal jejunum , it increases the mobility of the intestine . the empty loops of the ileum and distal jejunum twist and rotate around the base of the narrow sigmoid colon . subsequent peristalsis results in the formation of an isk with an obstruction involving 2 closed loops : one in the small intestine and the other in the sigmoid colon . anatomic factors are also known to be responsible for isk , including a long small bowel mesentery and freely mobile small bowel , and a long sigmoid colon on a narrow pedicle . a predisposing risk factor for isk is late pregnancy because of the obvious displacement of the bowel . in the present case , the patient was in early pregnancy ; thus , formation of the isk in this case was not related to displacement of the bowel resulting from an enlarged uterus . one possible risk factor for isk in the present case was an unbalanced oral intake owing to morning sickness during the pregnancy , together with the anatomic bowel features mentioned above . twelve cases of isk in the perinatal period were identified in pubmed using the keywords ileosigmoid knot , ileal knotting and pregnancy , labor ( table 1 ) . moreover , isk was not diagnosed preoperatively in any case other than the present case . the accuracy of preoperative diagnosis of isk is generally reported to be as low as 0% to 28% because of its infrequency and atypical radiographic findings . summary of data of patients with ileosigmoid knot during pregnancy the gold standard radiographic imaging modality for the evaluation of acute abdomen , including isk , is ct with high spatial resolution . radiation exposure to pregnant women undergoing medical imaging procedures and the management of such patients are difficult topics to address . there are radiation - related risks throughout pregnancy , and these risks are associated with the stage of pregnancy and absorbed dose . to diminish these prenatal risks , the radiation dose should be minimized . the first - line examination of a pregnant woman with abdominal pain should be us , which is free from radiation exposure , less expensive , and comparatively safer than ct . moreover , lazarus et al found that ct findings provided important diagnostic information in 30% of pregnant women with abdominal pain who were determined to have normal us findings . on the other hand , the efficacy of magnetic resonance imaging ( mri ) has been reported for the diagnosis of acute abdomen in adults . however , the national radiological protection board in the united kingdom advises that mri be avoided in the first trimester because there is limited experience assessing its safety during organogenesis . mri for acute abdomen in pregnancy should be further evaluated in clinical studies . according to the international commission on radiological protection , radiation risks are most significant during organogenesis and in the early fetal period , less in the second trimester , and least in the third trimester . malformations have a threshold of 100 to 200 mgy or higher and are typically associated with central nervous system problems . however , 3 pelvic ct scans or 20 conventional diagnostic x - ray examinations do not reach the fetal threshold of 100 mgy . therefore , prenatal doses of most properly performed procedures for the diagnosis of acute abdomen present no measurably increased risk of prenatal death , malformation , or mental impairment . efforts to avoid radiation exposure in women with acute abdomen during pregnancy may lead to a diagnostic delay , especially in early pregnancy . a missed or delayed diagnosis can pose a greater risk to the woman and fetus than any hazard associated with ionizing radiation . radiation exposure may be responsible for the difficulty in obtaining an accurate diagnosis of isk in the perinatal period , which may explain the high mortality and morbidity rates in previous reports ( table 1 ) . in the present case , enhanced abdominal ct was performed for evaluation of acute abdomen in early pregnancy , and an emergency laparotomy saved both the mother and child . this is the first case in which an isk during pregnancy was preoperatively diagnosed and successfully treated with surgery . in conclusion , ct should be performed for a prompt diagnosis and optimal management of acute abdomen , including isk and even in early pregnancy .
the ileosigmoid knot ( isk ) is a rare cause of intestinal obstruction . isk is a condition in which the ileum wraps around the base of the sigmoid colon and forms a knot , leading to high mortality with rapid progression to bowel gangrene . we herein report a rare case of isk at week 13 of pregnancy . the isk was diagnosed by computed tomography , and the patient underwent emergency surgery for acute abdomen . laparotomy showed segmental gangrenous change in the sigmoid colon , which was twisted around the distal ileal loop . the gangrenous bowel was resected , and primary anastomosis was performed . to our knowledge , the present case involves the first and earliest pregnancy in which a preoperative diagnosis of isk was made and successful treatment was performed with surgery . a radiologic approach should be undertaken for prompt diagnosis and optimal management , even in early pregnancy .
hemangiopericytoma is an unusual highly vascular soft tissue sarcoma of pericytic origin that commonly affects adults in the fifth or sixth decade of life . complete surgical removal is the treatment of choice for primary tumour , local recurrence and solitary metastasis . computed tomography ( ct ) , magnetic resonance imaging ( mri ) and angiography are classically considered the diagnostic modalities of choice for preoperative evaluation and surgical planning . recently , contrast - enhanced mri has emerged as a valuable non - invasive technique which provides a rich analysis of the regional vascular properties of a tumour . ultrasonography ( us ) with the use of second - generation contrast agent we describe the ct , contrast - enhanced mri , us with second - generation contrast agent , and angiography findings in a patient with hepatic metastases of hemangiopericytoma . a 57-year - old man presented with a 1 month history of slowly progressive mild right upper abdominal pain and weakness . the patient had been treated with radical surgery and adjuvant chemotherapy for a retroperitoneal hemangiopericytoma 3 years earlier . six months before his current physician visit , a pelvic disease recurrence with one liver metastasis occurred . the radical excision of these two tumour lesions was performed without evidence of disease in the follow - up . the patient underwent multiphase helical ct with a bolus of 120 ml of low - osmolar iodinated contrast medium ( iomeron 400 [ iomeprol ] , bracco , milan , italy ) revealing a single mass in the left lobe ( fig . dynamic contrast - enhanced mri was performed using gadobenate dimeglumine ( gd - bopta ) ( multihance , bracco , milan , italy ) . all images were obtained from 1.5 tesla mri system ( achieva 1.5 t , philips medical systems , eindhoven , netherlands ) . the three lesions were optimally visualized during delayed phase gd - bopta - enhanced t1-weighted mri ( fig . mri was also performed with ferumoxides ( endorem , guerbet , aulnay - sous - bois , france ) . all metastases were hyperintense on the t2-weighted mri images obtained before and after ferumoxides administration ( fig . hepatic angiography revealed an increase in the number and calibre of feeding arteries and draining veins in the three hepatic metastases , which were readily identified on angiograms obtained after intra - aortic injection of contrast material . 1(g ) ) . us was performed with br1 ( sonovue , bracco , milan , italy ) . 1(i ) ) , and appeared progressively hypoechoic with regards to the surrounding parenchyma during the portal ( fig . 1(k ) ) . us fine - needle aspiration biopsy confirmed the diagnosis of a round - cell sarcoma compatible with hemangiopericytoma . the consensus of the literature is to treat all resectable tumour lesions , including local recurrences and a solitary metastasis , with wide excision . imaging plays an extremely important role in the appropriate treatment planning by demonstrating the vascular nature of the tumour , revealing the exact source of its blood supply , and , finally , demonstrating its size . increased sensitivity of new diagnostic imaging modalities in the detection and characterization of liver metastases has created a need for detailed understanding of the pathological - radiological relationship of each type of tumour . angiography added further information about the number and calibre of arteries feeding and veins draining the liver metastases . us with br1 showed detection results similar to those of gd - bopta and ferumoxides - enhanced mri and angiography . to the best of our knowledge , mri with gd - bopta and ferumoxides , and us with br1 findings of hepatic metastases of hemangiopericytoma have not previously been reported . hemangiopericytomas occur most frequently in the extremities , and the retroperitoneum is the second most common site . patients with hemangiopericytoma can experience either early or late local recurrences and distal haematogenous metastases . the prognosis for these patients is very variable , and less than 50% are disease - free at 5 years . recurrences can develop following an extended disease - free interval with nearly 50% of recurrences occurring at 3 or more years after diagnosis and reported cases relapsing after 20 years . therefore , the early detection of locoregional and distant recurrence can play an effective role in the prognosis of these patients . multidetector computed tomography ( mdct ) is the modality of choice for follow - up imaging , in particular with regard to lung metastases , the lung being a frequent site of metastases of hemangiopericytoma . contrast - enhanced mri with gd - bopta or ferumoxides and us with br1 can be used for supplementary liver imaging . us with br1 is an upcoming way to illustrate liver lesions in various vascular phases , and has some advantages over other modalities for imaging of liver metastases , such as convenience , the probability of fewer side - effects , no exposure to radiation , and no risk of missing the optimal time for observation . a 57-year - old man with hepatic metastases of retroperitoneal hemangiopericytoma . ( a ) contrast - enhanced ct reveals a mass in the left lobe . ( b ) ( c ) gd - bopta - enhanced axial t1-weighted mr image in the dynamic phase shows homogeneous enhancement of the left lobe liver lesion with multiple signal flow voids representing large tumour vessels . ( d ) the corresponding image of gd - bopta in the delayed phase shows the wash - out of contrast agent from the metastasis with delineation . ( f ) ferumoxides - enhanced axial spin - echo t2-weighted mr image reveals lesion to have homogeneously increased signal . ( g ) angiography shows three tumour lesions ( arrows ) and demonstrates increase in the size and number of feeding arteries and a profusion of draining veins . ( h ) unenhanced us scan of the liver with detection of multiple hypoechoic lesions ( arrow indicates the one highlighted in this image ) . ( i ) following br1 administration , in the same scan of ( h ) , the metastasis is clearly depicted and appears hyperechoic in the arterial phase ( arrow ) . ( j ) in the portal venous phase , the lesion shows a central hypoechoic area with peripheral hyperechoic ring ( arrow ) . ( k ) in the delayed phase , the lesion appears hypoechoic ( arrow ) .
hemangiopericytoma is a rare and characteristically hypervascular tumour . we report a case of hepatic metastases of hemangiopericytoma for which there was correlative imaging by ultrasonography , ultrasonography with second - generation contrast agent ( br1 ) , computed tomography , gadolinium - enhanced , gd - bopta - enhanced and ferumoxides - enhanced magnetic resonance , and angiography . to our knowledge , this is the first reported case in which all these modalities were used in the diagnostic evaluation .
the anterolateral thigh ( alt ) flap is one of the most widely used reconstructive options in modern surgery , chosen because its vascularization is well known and extensively studied ; it is very versatile , easily dissected , and is associated with a low rate of donor site morbidity . the anteromedial thigh ( amt ) flap , meanwhile , is equally versatile , with similar low donor site morbidity , can be pedicled [ 1 , 2 , 3 ] or used as a microvascular free - tissue transfer [ 1 , 4 , 5 , 6 , 7 ] , but comparatively little is known of its vascularization . we report a case of soft tissue loss in the inguinal region with exposure of a femorofemoral crossover bypass and occlusion of the superficial femoral artery in a 48-years - old patient treated successfully with an amt pedicled flap to cover the inguinal region . this report shows that the amt flap is a valid option for covering the inguinal region in cases involving lower extremity revascularization , despite the superficial femoral artery being unavailable . we believe that this is a good example of association of the amt flap with the branches from the deep femoral artery . a 48-years - old man had underlying chronic lower extremity ischemia , fontaine grade iib , with occlusion of the superficial femoral artery ( figure 1 ) and extensive collateral circulation through the deep femoral artery . coronary revascularization was performed , inserting an intra - aortic balloon pump via the common femoral artery . acute ischemia and compartment syndrome of the lower left extremity followed as a complication of the procedure . thrombectomy and iliofemoral endarterectomy were performed , followed by a second procedure to perform a femorofemoral crossover bypass with prosthesis . the patient presented infection at the bypass site associated with torpid evolution of the inguinal wound . a third procedure was performed involving a new deep femorofemoral crossover bypass using the right internal saphenous vein . one week after the third procedure , the patient presented a 10 7 cm inguinal defect with exposure of the bypass . it was decided to cover the defect by transposition of sartorial fascia and an amt pedicled flap . arteriogram showing the lower right extremity ( healthy extremity ) and the lower left extremity ( revascularized extremity ) . the arrow indicates the superficial right femoral artery ( left picture ) and the left deep femoral artery ( right picture ) . during surgery , a preliminary incision measuring 5 cm was made in the proximal third of the line connecting the anterior superior iliac spine and the superolateral corner of the patella . musculocutaneous perforators were then located ( figure 2 ) ; these could only come from the rectus femoris branch of the descending branch of the lateral circumflex femoral artery , as the superficial femoral artery was occluded ( figure 1 ) . the presence of this perforator facilitated the procedure , and a skin paddle was designed to coincide with the perforator , which was transposed to cover the inguinal defect ( figure 2 ) . donor site was primarily closed after the insetting of amt perforator flap into the groin defect ( figure 3 ) . final result was good coverage of the bypass and closure of the wound after 2 months ( figure 4 ) . illustration of amt perforator flap based on the musculocutaneous rectus femoris branch perforator ( x ) of the descending branch of the lateral circumflex femoral artery , which is surrounded by a vessel loop . abbreviation : amt = anteromedial thigh . illustration of the final result 2 months after surgery : coverage of the bypass with the amt perforator flap and closure of the wound . the amt flap can be a good alternative to the alt flap option : the scar is more discreet , fewer pilous hair follicles are present , and the fascia is more elastic . nevertheless , because vascularization of this area is more variable , the amt flap has to a large extent been considered a second - line option . alt flap perforators are present in > 95% of cases , unlike amt flaps , where perforators are present in between 100% and 50% of cases , considering only rectus femoris branch perforators as the true pedicle of this particular flap . the amt flap was first described by baek in 1983 , and a year later , in 1984 , by song et al . . baek describes the flap as being vascularized by an unnamed artery from the superficial femoral artery . an innominate branch of the lateral circumflex femoral artery which arises near its base and descends medially on the medial aspect of the rectus femoris muscle and between the sartorius and vastus medialis muscle . following song et al.s lead , serafin and buncke describes the vascularization of the amt flap based on the innominate branch of the lateral femoral circumflex artery . several subsequent cadaver and in vivo studies yu and selber s article is probably the most successful at unifying criteria in regard to the question what is the pedicle of the true anteromedial thigh flap ? in their prospective in vivo study of 100 thigh muscles , yu and selber found that 21% had no perforators , 28% had superficial femoral perforators , and 51% had rectus femoris branch perforators of the descending branch of the lateral circumflex femoral artery , these latter being what could be considered the true amt flap . we present the first case , to our knowledge , in which a pedicled amt flap is used the cover the inguinal region in a femorofemoral crossover bypass following deep femoral artery revascularization . in addition , the superficial femoral artery was occluded , so vascularization was only possible via rectus femoris branch perforators . moreover , we hypothesize that in this situation where only the deep femoral system is permeable , larger collateral and amt perforators can be found ( figure 2 ) . donor site was closed primarily without any tension . still a small wound ( 2 1 cm ) with granulation tissue can be observed on the proximal part of the flap in the 2-month follow - up picture ( figure 4 ) . these complex patients have a slow healing process due to their comorbidities . in the patient here we still think that alt flap would be the standard and first approach for a broad spectrum of defects . however with this case we tried to illustrate that , when superficial femoral artery occlusion is found , amt flap could still be a potential first option in some situations ( i.e. , no alt perforators ) . some degree of confusion has been found in literature about the amt perforators ( superficial vs deep femoral artery ) . we think this patient is a good illustration of amt perforator anatomy and potential clinical use . in summary , our report shows : i ) the amt flap is a viable option to cover the inguinal region in cases of lower extremity revascularization or as an alternative to the alt flap ; ii ) the association of the amt flap with the vascular system of the deep femoral artery , instead of the superficial femoral artery as some papers had described .
abstractwe report the case of a 48-year - old male with an exposition of a femorofemoral crossover bypass in the inguinal region and superficial femoral occlusion . this was successfully treated using an anteromedial thigh ( amt ) pedicled flap based on the perforator vessel of the descending branch of the lateral circumflex femoral artery . our report focuses on : i ) considering the amt flap as a safe and easy option to cover the inguinal region in cases of bypass exposure ; ii ) describing the attachment of this flap to the deep femoral artery in a patient with superficial femoral occlusion , in spite of some literature controversy .
the current approach to the surgical management of patients with univentricular hearts is staged repair , which includes neonatal surgery to establish a source of controlled pulmonary blood flow and eliminate systemic outflow obstruction , followed successively by bidirectional superior cavopulmonary shunt ( bscps ) and a fontan completion . respiratory compromise is an important cause of desaturation following a bscps and is usually due to consolidation or collapse of the lung parenchyma and/or collections of fluid or air in the pleural space . we present a patient with a functionally univentricular heart who had a normal airway . following a bscps , she developed desaturation with inability to wean from ventilator . brochoscopy and ct angiography revealed compression of left main bronchus by pulmonary artery anteriorly and descending aorta posteriorly . the site and cause of obstruction was clearly defined by ct - based 3d - modelling of the trachea , bronchi and great vessels . the patient improved with conservative management and was extubated and discharged home without any residual airway obstruction . a full - term baby was diagnosed with double inlet left ventricle ( dilv ) , levo - transposition of great arteries ( l - tga ) , large unrestrictive ventricular spetal defect ( vsd ) , and an atrial septal defect ( asd ) . aorta originated from the non - dominant anterior ventricle and pulmonary artery came from the dominant posterior ventricle . follow up echocardiography showed pulmonary artery band gradient of 71 mm hg with no sub - aortic obstruction . at 5 months of age the main pulmonary artery was disconnected from the ventricular mass and the pulmonary valve was oversewn . the child was extubated soon after surgery , but had respiratory distress , requiring reintubation . auscultation of the chest showed diminished air entry into the left lung , which was attributed the position of the endotracheal tube . echocardiography showed unobstructed shunt , unobstructed branch pulmonary arteries , good ventricular function and no atrioventricular valve regurgitation . echo - imaging injection of agitated saline in the right upper limb vein was not suggestive of pulmonary arteriovenous malformations . ventilator strategy to maintain relative hypercarbia to improve superior venacaval return did not improve saturations . femoral arterial saturation was 56% , and the left and right pulmonary artery saturations 37% superior venacaval , right and left pulmonary artery pressures were 17 mm hg . mean left and right atrial pressures were 4 mm hg and the left ventricular end diastolic pressure was 5 mm hg . during cardiac catheterization it was observed from chest screening that left lung expansion was poor . the position of the tube was optimized but there was no improvement in the left lung expansion . the endotracheal tube was maneuvered into the left main bronchus and hand ventilation attempted , but it was too difficult to inflate the left lung , and this was clearly observed on screening . ct angiography confirmed impingement of the left main bronchus between pulmonary artery anteriorly and descending aorta posteriorly ( figure 1 ) . the site and cause of obstruction was clearly defined by ct - based 3d - modelling of the airways and great vessels . the patient was managed conservatively with ventilator support , selective bronchial suctioning and mucolytic installation under bronchoscopic guidance and systemic steroid were given for one week , the child was successfully extubated to nasal cpap and was subsequent discharged home with oxygen saturation in 80s . ct scans were obtained via a siemens sensation 64 with a slice thickness of 1.0 mm and a slice increment of 0.8 mm . dicom were imported into mimics ( materialise , leuven , belgium ) for 3d reconstruction of the blood volumes in the single ventricle , aorta and pulmonary artery . the processed files were exported as stl files into 3-matic ( materialise , leuven , belgium ) to create the various images of interest . causes of desaturation flowing bidirectional superior cavopulmonary shunt include anastamotic obstruction , presence of decompressing vein from the cavopulmonary circuit to the inferior vena cava territory or to the atrium , high pulmonary vascular resistance , ventricular dysfunction , and , in rare cases , acute pulmonary arteriovenous malformations . bronchial obstruction has been commonly described following aortic arch repair and conduit reconstruction of the pulmonary outflow tract but it is uncommon following bscps . compression of the left main bronchus between the left pulmonary artery anteriorly and the descending aorta posteriorly has been described in a 3-month - old child following patch augmentation of aortic arch and closure of vsd . augmented aortic arch was the main culprit , which was surgically elongated to relieve the obstruction . a 13-year - old patient in their series was the only case of a functionally univentricular heart , who , after an extra cardiac fontan operation , developed left bronchial compression by pincer effect between the posterior side of the ascending aorta and the anterior side of the descending aorta . this patient needed aortopexy and placement of a stent in the left main bronchus to relieve the obstructions . we believe that in our patient disconnection of the main pulmonary artery from the ventricular mass caused the branch pulmonary arteries to fall back into the posterior mediastinum where the left pulmonary artery caught the left main bronchus between itself and the posteriorly placed descending aorta ( figure 2 ) . in combination with inflammatory edema that follows surgery , the left bronchus was trapped between two big vessels was obstructed . with time , conservative management , and steroids , the edema subsided and the compression on the bronchus was relieved . 3d reconstruction based on medical imaging is an effective method of defining the cause of respiratory obstruction . computed tomography scanning is particularly useful for demonstration changes in airway caliber , in addition to the location , degree and extent of the airway narrowing . ct angiography was successful in confirming the mechanism of airway obstruction and planning therapeutic intervention in 17 patients who developed airway obstruction following operations that involved reconstruction of the aortic arch or the right ventricular outflow tract . the use of 3d modelling described here represents a major refinement for accurately determining the site and cause of the obstruction , and can be repeated using mri if necessary to determine the response to therapy .
extrinsic compression of airways is one the most important causes of respiratory insufficiency in the perioperative period in children with congenital heart disease . this is especially true of pathologies that involve surgery of the aortic arch or conduit replacement of the right ventricular outflow tract . however bronchial obstruction is uncommon in the setting of bidirectional cavopulmonary shunt alone.we report the case of an infant with a functionally univentricular heart who had a bidirectional superior cavopulmonary shunt and disconnection of the main pulmonary artery from the ventricular mass with oversewing of pulmonary valve . post - operatively the patient desaturated due to compression of left main bronchus by the left pulmonary artery anteriorly and the descending aorta posteriorly . this was clearly defined by ct based on 3d - modelling of the airways and great vessels . the child was managed conservatively by ventilator support , selective bronchial suctioning and systemic steroids with a successful outcome .
the mesenchymal metaplasias are a rare phenomenon ; varying from reactive , degenerative lesions to those able to associate with malignancy or those having a preneoplastic potential . the main significance is that they should not be mistaken for more serious processes , particularly the heterologous elements of a carcinosarcoma . hormonal or irritative stimuli are the main inducing factors , although some metaplasias have a mutational origin . it should be noted that in some instances the presence of cartilage or bone is the result of some retained fetal parts . clinically , the symptoms range from a casual finding in an otherwise asymptomatic woman to menstrual irregularities , pelvic pain , dyspareunia , and vaginal discharge . the aim of this review is to update current issues and provide a practical clinicopathological approach . a 38-year - old multiparous female , with obstetric score of gravida 4 , para 4 , live birth 4 , abortion 0 , presented with third degree uterovaginal prolapse ( recto , cysto , and enterocele ) for which she underwent transvaginal hysterectomy . also , there was no history of previous surgical intervention like dilatation and curettage after the last pregnancy or between pregnancies . hysterectomy specimen received for histopathological examination revealed the uterus with cervix measuring 8 3 2 cm . the cut section showed a tiny gray - white smooth , glistening rice grain like lesion measuring approximately 0.6 0.2 cm was embedded in the substance of uterus . an intramural fibroid measuring 2 cm in diameter , firm in consistency , showing whorled appearance was noted [ figures 1 and 2 ] . cut section of uterus showing tiny gray white lesion in the endometrium and an intramural fibroid glistening , gray - white lesion in the endometrium microscopic examination revealed unremarkable squamocolumnar junction of cervix with squamous metaplasia of few cervical glands . a section from the endometrium incidentally showed a solitary focus of well - formed hyaline cartilage in the endometrial stroma . transition between stromal and cartilaginous cells was evident , which helped us to identify it as metaplastic . photomicrograph showing island of cartilage surrounded by endometrial tissue ( h and e , 10 ) a histological diagnosis of endometrial cartilaginous metaplasia with adenomyosis and leiomyoma - uterus was made . endometrial metaplasias have been classified by hendrickson and kempson into three groups - epithelial , mesenchymal , and glial . the newer world health organization ( who ) classification divides them into those with epithelial and non - epithelial changes . who classification of endometrial metaplasias and related changes : epithelial metaplasias and related changesnon - epithelial metaplastic and related changes smooth muscle metaplasiaosseous metaplasiacartilaginous metaplasiafatty changeglial tissuefoam cell change epithelial metaplasias and related changes non - epithelial metaplastic and related changes smooth muscle metaplasiaosseous metaplasiacartilaginous metaplasiafatty changeglial tissuefoam cell change smooth muscle metaplasia cartilaginous metaplasia heterotopia is the occurrence of a mature tissue at an abnormal location . heterotopic uterine cartilage can be of metaplastic origin or it can result from the displacement and retention of fetal tissues in the uterine wall . the multipotent cells present in normal endometrial stroma can show metaplastic transformation into various elements , including the cartilage . heterotopic uterine cartilage of metaplastic origin may occur as solitary or multiple foci , and may be located in the endometrium or even in the stroma of endometrial polyp . genital tuberculosis , unspecific chronic endometritis , or pyometra are other sources of chronic inflammation such as that occurring after retained embryonic tissue , and this inflammation acts as a promoter of secondary changes . in india , endometrial tuberculosis should be ruled out as it can cause infertility as well as chondrogenesis . chronic endometritis also stimulates the proliferation of mesenchymal cells that have the inherent property of metaplasia and can differentiate into chondroblasts or osteoblasts . another interesting mechanism is the iatrogenic implantation of the fetal tissue , including the cartilage , into the uterine wall following dilatation and curettage . it is important to recognize the non - neoplastic nature of this condition to differentiate it from malignant mixed mullerian tumor of the uterus . hence , a careful clinical and histopathological examination is required to avoid the misinterpretation of non - tumor cartilaginous foci as a component of malignant neoplasm .
endometrial metaplasias are of two types , epithelial metaplasia which is commonly encountered and stromal metaplasia , unusually seen . this includes formation within endometrial stroma , islands of smooth muscle , cartilage , and bone . endometrial stromal ( cartilaginous ) metaplasias are conditions frequently overlooked and misdiagnosed . hence , a careful clinical and histopathological examination is required to avoid the misinterpretation of non - tumor cartilaginous foci as a component of malignant neoplasm . herein , we report a rare case of endometrial cartilaginous metaplasia which was an incidental finding in a 38-year - old female with third degree uterovaginal prolapse .
multiple myeloma ( mm ) is thrombogenic as a consequence of multiple hemostatic effects , including elevated interleukin-6 levels , procoagulant antibody formation , paraprotein interference with fibrin structure , activated protein c resistance and endothelial damage . thalidomide is an effective treatment both in advanced mm and as first - line therapy in combination with dexamethasone or other cytotoxic chemotherapy ; it has been associated with an increased risk of thromboembolic events including pulmonary hypertension ( ph ) [ 3 , 4 ] . ph in the absence of thromboembolic events has also been described in 3 patients with mm during thalidomide treatment [ 5 , 6 , 7 ] and in a pilot study . in september 2010 , a 79-year - old woman was diagnosed with igg lambda mm evolved from monoclonal gammopathy of unknown significance ( mgus ) . in april 2011 , about a month later , she presented with asthenia , palpitation and dyspnoea on exertion and was hospitalized . ecg showed tachyarrhythmia with atrial fibrillation ( 140 beats / min ) . within 24 h , a blood sample analysis yielded the following results : nt - pro - bnp 3,021 ng / l ( normal value < 450 ) , d - dimer 519 ng / ml ( normal value < 300 ) , and t - troponin 37.2 ng / l ( normal value < 14 ) . blood arterial gas analysis parameters were : ph 7.49 , po2 70.2 mm hg , pco2 25.1 mm hg , hco3 19.2 mmol / l , sato2 95.7% , po2/fio2 336 mm hg . an echocardiography performed a few days later showed severe ph ( systolic pulmonary artery pressure 75 mm hg ) with mild tricuspid insufficiency without paradoxic movement of ventricular septum or right ventricular dysfunction signs , and normal left ventricular function . connective tissue disease was also excluded by further examination . based on the hypothesis of a pharmacological pathogenesis , the patient was sent to the center for pulmonary hypertension ( policlinico san matteo , pavia , italy ) for further investigations . in june 2011 ( about a month later ) , she was hospitalized . sinus rhythm on ecg and echocardiography showed normal right ventricular function and normal pulmonary artery pressure . a clinical control in july 2011 confirmed absence of symptoms , and showed a peripheral sato2 of 97% and a pulmonary artery pressure of 26 mm hg . as in the 3 cases reported in the literature [ 5 , 6 , 7 ] , we suggest a possible direct correlation between thalidomide and ph , since in all cases a rapid decrease of pulmonary artery pressure after thalidomide discontinuation was observed . furthermore , in the pilot study of lafaras et al . , 4 of the 82 treated patients had ph and improved after discontinuation of therapy . our patient showed clinical and echocardiographic signs of ph after a few days of thalidomide therapy . in the described case reports , symptoms occurred after a few months , but in the cohort described by lafaras et al . thalidomide possibly causes a vasodilator and vasoconstriction imbalance , which may cause abnormal pulmonary vascular response leading to a vicious circle perpetuating ph [ 8 , 9 ] . a direct effect of thalidomide on the pulmonary endothelial cells could be postulated . in the case described by hattori et al . , autopsy revealed an extensive formation of plexogenic pulmonary arteries accompanied by thickening of intimae and media arteries . indicate that the subgroup of mm patients with unknown heart disease ( coronary artery disease , valvular heart disease and hypertension ) receiving thalidomide should be thoroughly examined by echocardiography for the early detection of clinical and/or subclinical ph . thus , echocardiographic evaluation might be necessary to all patients receiving thalidomide ; it not only provides an estimate of pulmonary pressure but also may help to exclude any secondary causes of ph , predict the prognosis , monitor the efficacy of specific therapeutic interventions and detect the preclinical stage of the disease .
multiple myeloma ( mm ) is thrombogenic as a consequence of multiple hemostatic effects . thalidomide is an effective treatment ; however , it has been associated with an increased risk of thromboembolic events including pulmonary hypertension ( ph ) . ph in the absence of thromboembolic events has also been described in some patients with mm during thalidomide treatment . we experienced occurrence of ph in a mm patient during treatment with thalidomide . a 79-year - old woman was diagnosed with igg lambda mm and was started on thalidomide treatment . about a month later , she presented with asthenia , palpitation and dyspnoea on exertion and was hospitalized . an echocardiography revealed severe ph ( systolic pulmonary artery pressure 75 mm hg ) without paradoxic movement of ventricular septum or right ventricular dysfunction signs ; a previous echocardiography was normal . pulmonary computed tomography and perfusion scan were negative for pulmonary embolism . based on the hypothesis of a pharmacological pathogenesis , thalidomide was promptly interrupted . about a month later , she was hospitalized for further investigations . physical examination documented absence of dyspnoea or other respiratory signs , and echocardiography showed normal right ventricular function and normal pulmonary artery pressure . as in the cases reported in the literature , we suggest a possible direct correlation between thalidomide and ph , since in all cases a rapid decrease of pulmonary artery pressure after thalidomide discontinuation was observed .
bragadottir and colleagues found utilization of glomerular filtration rate ( gfr ) estimating equations of limited value , no value or perhaps even negative value in intensive care unit ( icu ) acute kidney injury ( aki ) patients . they confirmed the limited , inaccurate and highly variable data obtained from urinary creatinine collections to quantify the gfr . they acknowledge this had been shown before and point to inaccurate urine collections and variable release of creatinine from muscle as problems [ 2 - 4 ] . unfortunately , the available tests to quantify the gfr utilize a single - compartment model that requires several hours for equilibration between the plasma volume and extracellular fluid space . in addition , they require laboratory determinations that are complex , sensitive to user error , time consuming and expensive . the use of serum creatinine , and the accompanying equations to estimate the gfr in patients with stable kidney function , has been beneficial in longitudinal population studies where trends are followed and the known wide variation between calculated and determined gfrs is acceptable . the necessity of a plasma equilibrium state for creatinine , based on the stable release of creatinine from muscle and stable removal by the kidney via the gfr , is well known in these situations . in states of aki , however , these necessary parameters do not hold and thus it is predictably impossible to use serum creatinine or equations based on serum creatinine . further complicating the use of these parameters in the acute situation is the availability of renal reserve . the renal reserve represents the ability of the normal kidney to increase the gfr following specific challenges such as a high - protein meal , early diabetes , unilateral nephrectomy or progressive loss of kidney function . renal reserve can be > 50% of baseline unstimulated kidney function , thus increasing the gfr from 100 ml / minute/1.73 m to > 150 ml / minute/1.73 m. renal reserve may therefore be one reason why the gfr can fall to 50% of normal values prior to detection based on a rise in serum creatinine . this observation has made the use of serum creatinine insensitive as a marker of development of chronic kidney disease and is in part why the estimating equations for the gfr are not useful above a gfr of 50 to 60 ml / minute/1.73 m. in theory , therefore , a patient could have lost up to 67% of their baseline total gfr , going from a potential gfr of 150 ml / minute/1.73 m to one of 50 ml / minute/1.73 m prior to a change in serum creatinine ( figure 1 ) . we know very little about renal reserve in aki . an additional problem with serum creatinine as a marker of kidney function in cases of aki is the slow rate of rise to an equilibrium state . in a noncatabolic 70 kg patient , if you were to remove both kidneys the serum creatinine would rise by only 1 mg / dl / day . thus , 1 day post bilateral nephrectomy the patient s serum creatinine would be 2 mg / dl , on day 2 it would be 3 mg / dl , and so on , even though the patient has zero gfr . patients with severe aki therefore sit in the icu waiting for blood chemistries , or their volume status , to rise to renal replacement levels , all the while being in a state we believe requires renal replacement therapy for appropriate support . add to this the other factors known to confound serum creatinine as a marker of the gfr and is there any wonder why its use in the icu , where accurate quantitative data are a must , is resulting in less than maximal care of the patient [ 6 - 10 ] . with loss of the glomerular filtration rate ( gfr ) the renal reserve may be first lost , reducing the maximally stimulated gfr . thereafter , as the gfr is lost the serum creatinine ( scr ) does not rise until the gfr is reduced to around 50 to 60 ml / minute/1.73 m. therefore , up to two - thirds of the patient s original gfr has been lost before changes in scr occur . on a brighter note , commercial attempts have been initiated to develop a rapid , sensitive , reproducible , affordable and user - friendly gfr technique . clearance of a freely filterable water - soluble molecule is being used with either noninvasive or minimally invasive approaches . both one - compartment and two - compartment models are being developed , with two - compartment approaches offering a more rapid result because equilibration with the extracellular fluid is not needed . fast biomedical ( indianapolis , in , usa ) has recently completed a phase i , single - blind , dose escalation study in normal patients with satisfactory results ( unpublished observation ; see competing interests statement ) . perhaps a more intriguing question for the clinician to now consider is , when available , how will a gfr test be used to advance care in the hospitalized patient ? to commercialize such a product there must be defined clinical pathways resulting in meaningful , cost - effective and patient - beneficial outcomes that can be standardized for general use . in aki , functional confirmation and quantification of the severity of injury would allow the physician to determine the immediate need for renal replacement therapy . additionally , the response to hydration or pressor therapy could be quantified with a second test post - therapy . weaning from continuous veno - venous hemofiltration therapy or movement of the patient to the ward , based on the return of kidney function that is now quantifiable , may minimize valuable days in the icu . knowing the actual gfr for drug dosing of renally cleared drugs or nephrotoxins may provide more reliable serum levels . accurate gfrs would also accurately identify patients with low gfrs requiring preventative therapies prior to use of agents such as radiocontrast or gadolinium . . however , it now seems time to turn attention to the care of the aki and chronic kidney disease patient in an effort to minimize , or at least forestall , the development of end - stage renal disease and requirement for chronic renal replacement therapy . the cost of such development and utilization can be easily rationalized based on the patient and societal costs of end - stage renal care of chronic dialysis patients . aki : acute kidney injury ; gfr : glomerular filtration rate ; icu : intensive care unit . bam is a co - founder , part owner , and medical director of fast biomedical ( indianapolis , in , usa ) .
acute kidney injury ( aki ) , due to its increasing incidence , associated morbidity and mortality , and potential for development of chronic kidney disease with acceleration to end - stage renal disease , has become of major interest to nephrologists and critical care physicians . the development of biomarkers to diagnose aki , quantify risk and predict prognosis is receiving considerable attention . yet techniques to accurately assess functional changes within patients still rely on the use of an insensitive marker ( creatinine ) , creatinine - based estimating equations and unreliable urinary tests . therefore , it is critical that functional tests be developed and used in combination with biomarkers , thus allowing improved care in aki and chronic kidney disease patient populations .
a 10-year - old boy , 147 cm tall and 40 kg weight , presented to our hospital with complaint of sustained bilateral hip pain for 4 weeks . he showed positive sign of patrick 's test in bilateral hip and a slight limping gait and limited range of motion ( rom ) on a physical examinations . blood test results revealed no abnormalities , such as systemic disease or evidence of pyogenic coxitis . radiographs showed a sclerotic line along the left femoral neck and evidence of callus formation . a minute disruption of the medial cortex at the trochanteric area was detected on the right side ( fig . was taken to help with the diagnosis , and t1-weighted images showed decreased signal intensity along the left femoral neck and on the medial cortex of the right femur . after 2 weeks , which was 6 weeks from the start of pain , he felt diminished bilateral hip pain and radiographs showed more sclerosis along the bilateral femoral neck , since then , partial weight - bearing ambulation with two crutches was permitted ( fig . ten weeks from the start of pain , he did not complain of any discomfort and showed full rom bilaterally . radiographs obtained 4 and 12 months after the initial pain developed showed complete healing of the stress fracture ( fig . the compression type , which account for most of stress fractures in children , typically show inferomedial cortical disruption initially , whereas a transverse type fracture , also known as tension type , tend to appear at the superolateral cortex5 ) . up to now , only one case of a bilateral femoral stress fracture has been reported in a child6 ) . some cases had a history of excessive sports activity and problems affecting general health condition , such as anorexia nervosa27 ) , whereas others had no clear contributory factors48 ) . radiographs are initially used to diagnose disease . however , in early stage of injury , it is difficult to detect a femoral neck stress fracture because of its nondescript features on radiographs . a few weeks after injury after two to eight weeks from the onset of symptoms , periosteal reaction or callus formation are generally seen as a result of bone remodeling9 ) . however , mri makes early diagnosis possible for patients who have clinical suspicion for stress fracture but radiographs are negative . early resorption of bone can be manifested as local hyperemia and edema along the endosteal surface on mri . several weeks after injury , a mri shows bone marrow edema on both t1- and t2-weighted images and signal changes in the cortical bone . the common pattern of stress fracture on mri is a low signal intensity line , surrounded by ill - defined large edema . the diagnosis of stress fracture was based on the presence of a focal , cortical and medullary zone of increased radionuclide uptake in all phases of bone scintigraphy . although false - negative cases have been reported , bone scintigraphy has been considered as gold standard for early detection of stress fracture910 ) . the cortical interrupted region differs in compression ( inferomedial ) and tension types ( superolateral ) of femoral neck stress fractures . according to this classification , most fractures are the compression type and the remainder are tension type5 ) . given past experience , a compression type fracture usually does not require internal fixation and conservative treatment with limited weight - bearing is sufficient for healing6 ) . however , an increased risk of displacement and a potential increase in osteonecrosis of the femoral head can occur in tension type fractures . differential diagnoses include hip joint strain , synovitis , osteoid osteoma , legg - calv - perthes disease , slipped capital femoral epiphysis , and malignant conditions such as osteosarcoma . because a femoral neck stress fracture is rare , it is easy to overlook in the differential diagnosis or may be misdiagnosed . thus , a femoral neck stress fracture should be considered in the differential diagnosis in children who present with sustained hip or groin pain , and mri , close follow - up with serial radiographs should be performed .
a femoral neck stress fracture in child is rare , particularly in bilateral case . it is easy to miss initially or may be misdiagnosed . the authors experienced a case of bilateral femoral neck stress fracture in a 10-year - old boy with bilateral hip . the patient was successfully healed by conservative treatment . we report this rare case with a review of the literature . a femoral neck stress fracture should be included in the differential diagnosis in children who present with sustained hip or groin pain .
apremilast is an oral phosphodiesterase iv ( pde4 ) inhibitor recently registered for the treatment of psoriasis and psoriatic arthritis . apremilast is frequently used in private practice , as it showed no relevant safety signals in clinical trials and currently , no laboratory tests are recommended during treatment . here we report the case of a man in his 40ies with plaque - type psoriasis for 3 years . he presented with a psoriasis area and severity index ( pasi ) of 10.3 and a dermatology life quality index of 8 . previous treatments included topical combined calcipotriol / betamethasone ( daivobet ) and uvb narrowband therapy . his medical history included hypercholesterinemia and gilbert - meulengracht disease with slightly elevated bilirubin of 23 a differential blood count showed a mild lymphopenia of 1.05 10/l ( reference 1.54.0 10/l ) of unknown etiology . one month later , the lymphopenia had worsened to 0.64 10/l without any clues suggestive of an underlying event such as a viral infection , for example . psoriasis plaques had worsened at 1 month of therapy , resulting in a higher pasi of 16.6 . apremilast treatment was interrupted , and 2 days later , lymphocyte counts increased to 0.78 10/l and 3 weeks later to 1.14 10/l ( fig 1 ) . leukocyte and especially neutrophil counts had almost doubled during apremilast therapy , remaining , however , within normal range , and returned to baseline levels after cessation of apremilast . a topical treatment with clobetasol propionate 0.05% ( clarelux foam ) was initiated and pasi improved to a value of 12 within 4 weeks . the negative temporal correlation of the lymphocyte counts with apremilast led us to the clinical conclusion that the drug might have been causally related to the lymphopenia . lymphopenia during apremilast treatment in humans has not been reported before and we expect that our observation is a rare event . clinical trials showed consistently good laboratory safety data , leading to some expert recommendations that blood sample monitoring can be omitted . newer guidelines , such as the swiss s1 , recommend several obligatory and optional laboratory safety checks before treatment initiation . in monkey studies with apremilast , lymphopenia and neutrophilia were reported but evaluated as negligible in magnitude and/or in the range of pretest values . in mice and rat studies , lymphopenia and neutrophilia and also crp elevation and albumin decrease were associated with arteritis and perivascular inflammation in various tissues . in general , with pde4 , inhibition by apremilast through accumulation of camp leads to inhibition of pro - inflammatory cytokines like tnf- , il-17 , ifn- , and il-23 , whereas il-10 is increased . t lymphocytes ( cd4 and cd8 ) contain mostly the pde4b and to a lesser extent the pde4a subtype as well as pde3 and pde7 . in copd patients , treatment with cilomilast , a pde4 inhibitor selective for pde4d , or roflumilast , a pde4 inhibitor almost nonselective on pde4 isoforms besides being less selective for pde4c , airway inflammation by lymphocytes and other cells like neutrophils was reduced . there is inconsistent evidence that pbmcs of atopic patients have a higher susceptibility to pde inhibitors . interestingly it was shown that structural and molecular differences in the m - loop region of pde4 can enhance the potency of its ligands . in summary , it could be speculated that a structural variance in pde4 or an overexpression of a certain isotype of pde4 , like pde4b , lead to reduced cell numbers . from our single case , as the clinical use of apremilast increases , it will be interesting to see whether our observation will also be made by others . such safety signals would not necessarily be seen in clinical trials , because clinical trials include only highly selected patient populations that may not represent the general population . hence , reporting of rare clinical observations in the post - marketing phase and ideally long - term registry data are crucial for developing a solid basis for recommendations on whether laboratory investigations for new drugs should be routinely performed or not . this study was funded by hsm-2 of kanton of zurich ( switzerland ) , promedica , and bruno bloch foundation , all to a.a.n .
apremilast is an oral phosphodiesterase iv inhibitor recently registered for the treatment of psoriasis and psoriatic arthritis in switzerland and other countries . even though it offers only moderate efficacy compared to biologics , many patients prefer drugs given by the oral route . apremilast is frequently used in private practice , as it showed no relevant safety signals in clinical trials and often , laboratory tests are omitted completely . here we report a patient who developed acute lymphopenia and worsening of psoriasis during apremilast treatment , which resolved with discontinuation of apremilast . we suggest that at least in prospective registries , that regular monitoring of laboratory surrogate markers should be performed on a long - term basis to detect rare but potentially important safety signals .
almost half of the patients with rhabdomyolysis develop acute renal failure and , in a large survey , the short - term mortality was 3.4% . the etiology of rhabdomyolysis is multifactorial , but the most common risk factors are medical and illicit drugs , alcohol , muscle trauma and diseases . in this paper , we describe a highly unusual cause of rhabdomyolysis . this case was reported to the adverse drug event manager of the capital region of denmark . ; a 65-year - old man with type ii diabetes , mild hypertension and a previous history of gout was admitted to the bispebjerg hospital , university of copenhagen , denmark . the patient 's daily prescriptions were indapamide 1.5 mg , allopurinol 300 mg , metformin 2000 mg and simvastatin 40 mg . fourteen days prior to hospitalization , he experienced muscle fatigue and soreness in the lower extremities . his blood pressure was 153/88 mm hg , his pulse was 87 bpm and his temperature was 37c . the blood samples revealed rhabdomyolysis , severe hypokalemia and normal renal function [ table 1 ] . furthermore , the anamnesis uncovered a newly acquired habit of a relatively high consumption of licorice ( approximately 100 g / day ) . there were no signs or history of trauma , diarrhea , polydipsia , infection , intoxication or alcohol intake . treatment with iv fluids , including potassium substitution , returned the patient 's potassium level to normal within 2 days and creatine kinase gradually decreased [ figure 1 ] . the patient was discharged after 9 days of hospitalization with no signs of kidney injury and an estimated glomerular filtration rate above 90 ml / min . the patient was treated intensively with iv - fluid and had a daily diuresis of 3 to 5 l the patient presented in this case report was on daily treatment with simvastatin , which has been associated with rhabdomyolysis . we can not rule out this causal relationship ; however , this usually implies hyperkalemia instead of hypokalemia . forty years ago , knochel and schlein showed that muscle blood flow was severely impaired in potassium - depleted dogs . thus , one possible explanation of the association between hypokalemia and rhabdomyolysis could be ischemia . indapamide inhibits the na+/cl- cotransporter in the distal convoluted tubule , and its primary side - effects are electrolyte disturbances and alkalosis , to a large extent mediated through the aldosterone system . the proposed mechanism is an increased conductance of the epithelial sodium channel and enhanced activity of the renal outer medullary potassium channel and h / k atpase . the main component of licorice is glycyrrhizin and its metabolites strongly inhibit the 11--hydroxysteroid dehydrogenase , which transforms cortisol to cortisone and 5--reductase , which is involved in the metabolism of aldosterone . we conclude that this patient developed rhabdomyolysis due to severe hypokalemia , possibly induced by a pharmacodynamic interaction between licorice and indapamide .
the adverse drug event manager of the capital region of denmark received a report of a 65-year - old male with type ii diabetes and long - lasting treatment with indapamide . in addition , he had a history of a high consumption of licorice . for 2 weeks , the patient suffered from myalgia , which the general practitioner suspected to be polymyalgia rheumatica and referred him to the hospital . initial blood samples revealed a reduced potassium concentration of 1.5 mmol / l ( reference value : 6.6 - 4.6 mmol / l ) and an elevated creatine kinase of 18,400 iu / l ( reference value : 40 - 280 iu / l ) . we believe that the patient developed rhabdomyolysis due to severe hypokalemia , possibly induced by a pharmacodynamic interaction between licorice and indapamide .
a 76-year - old female patient presented at the emergency department with dyspnea , a productive cough , and a fever for several days . she had experienced exacerbations of congestive heart failure for the previous 20 years and had a permanent cardiac pacemaker that had also been initially inserted about 20 years earlier due to sick sinus syndrome with atrial fibrillation . she also had dementia , and a history of multiple incidents of cerebral infarction and its complications . at the emergency department , her blood pressure was 112/72 mmhg , pulse rate was 101/min with an irregular rhythm , and body temperature was 36.5. on examination , she appeared tachypneic ( respiratory rate , 28/min ) and her breathing sounds were reduced over the left lung field . chest radiography revealed opacification of the whole left lung field , thought by a physician of the emergency department to be unilateral pleural effusion ( fig . after local anesthesia and under sterile conditions , a 16f catheter ( prime - s ; sungwon medical , cheongju , korea ) was inserted into the 6th left intercostal space in the anterior axillary line without image guidance . shortly after insertion , blood was draining in a pulsatile way from the catheter . however , she was hemodynamically stable with a blood pressure of 132/78 mmhg and a heart rate of 98/min . after clamping the catheter , contrast - enhanced computed tomography ( ct ) of the chest was performed ( fig . 2 ) and showed that severe cardiomegaly with displacement of the heart to the left with atelectasis ( originally thought to be massive pleural effusion ) . the catheter had been inserted anterolaterally into the sixth intercostal space on the left and was within the left ventricle , exiting the ventricle through the aortic valve with the tip of the catheter located in the ascending aorta . transthoracic echocardiography revealed mild aortic regurgitation with a small amount of pericardial effusion without hemodynamic significance . open mini - thoracotomy with chest tube insertion was performed under general anesthesia the next day . although the removal of the catheter was successful and well tolerated by the patient , the hospital course was complicated by pneumonia and long - term mechanical ventilation . about a month after the complication , the patient died from pneumonia with septic shock . chest drain insertion is a commonly used invasive procedure for managing pneumothorax , and draining the pleural cavity is also performed for patients with pleural effusion and empyema . it may be associated with certain complications such as infection , bleeding , and injury to the adjacent organs . however , cardiac injury during chest drain placement is a very rare but catastrophic complication , ten cases of which have been reported to date . although diverse types of heart injury have been reported , to our knowledge , this particular case describes for the first time cardiac injury with the catheter tip placed in the ascending aorta through the left ventricular cavity . most cardiac injuries during the procedure were related to anatomic distortion such as post - pneumonectomy , extreme kyphoscoliosis , and cardiomegaly [ 3 - 6 ] . similarly to these previous reports , our patient had marked cardiomegaly with atelectasis , which manifested as a dense opacity of the left hemithorax on chest x - ray . a chest drain was inserted in the chest cavity , eventually resulting in perforation of the left ventricle . the chest x - ray is the most important , widely used , and time saving means to evaluate pleural effusion . however , it is not specific , and many other conditions can mimic pleural effusion . furthermore , interpretation of a chest x - ray in cases of suspected pleural effusion is relatively complicated , and it has been reported that the discordance rate between radiologist and emergency department physicians is relatively high and can influence clinical outcomes . therefore , emergency department physicians should be aware of the possibility of misinterpretation . especially in cases in which the chest x - ray is used to make clinical decisions , such as chest tube insertion as in our case , meticulous attention is needed . this report emphasizes that delineating the locations of vital organs in the thorax must be confirmed prior to drain insertion . if anatomical orientation is hampered , ultrasound or ct should be used during or prior to chest drain placement . although chest drainage generally has a low complication rate , the procedure can be guided with fluoroscopy , ultrasound , or ct to place the catheter accurately and to reduce the complication rate . therefore , it is recommended that image guidance be used if the effusion is very small or initial aspiration fails . however , it should also be taken into consideration that imaging guidance can raise medical costs and is limited for everyday use . in conclusion , considering the possibility of underreporting , cardiac injury from chest drain insertion might not be very infrequent with certain conditions , and if the anatomical orientation is not established before the drain insertion , more complex imaging than a chest x - ray is needed to prevent life - threatening complications and image - guided chest tube insertion can make the procedure safer .
chest draining is a common procedure for treating pleural effusion . perforation of the heart is a rare often fatal complication of chest drain insertion . we report a case of a 76-year - old female patient suffering from congestive heart failure . at presentation , unilateral opacity of the left chest observed on a chest x - ray was interpreted as massive pleural effusion , so an attempt was made to drain the left pleural space . malposition of the chest drain was suspected because blood was draining in a pulsatile way from the catheter . computed tomography revealed perforation of the left ventricle . mini - thoracotomy was performed and the drain extracted successfully .
this community - based cross - sectional study was undertaken in cascavel municipality , a typical semirural municipality 70 km south of fortaleza , the capital of cear state in northeastern brazil . in 2003 , cascavel had a population of 57,000 . the main sources of income are fishing , agriculture , tourism , and the cashew nut industry . most ( 95% ) of the pregnant women in cascavel are registered in the family health program and receive prenatal care from the public health system . all pregnant women at < 26 weeks of gestation registered in this program from may one female investigator interviewed the women with respect to demographic , socioeconomic status , and behavioral characteristics by using pretested structured questionnaires . emphasis was given to the presence or ownership of animals , eating habits , soil contact and drinking water sources . serum samples were tested for igm and igg antibodies to toxoplasma by elisa ( vidas , biomrieux , nrtingen , germany ) . women with positive igg titers but negative igm titers were considered latently infected . women with positive titers both for igg and for igm were considered to possibly have recent infections and were further tested for avidity of igg antibodies ( vidas , biomrieux ) . sensitivity and specificity of the vidas test are 97.3% and 99.8 , respectively ( 7 ) . we were unable to rule out recent infections in women with low or intermediate igg avidity . ethical approval for the study was obtained from the ethical review board of cascavel municipality . before the study data were entered twice into a database by using epiinfo version 6.04d software ( centers for disease control and prevention , atlanta , ga , usa ) and checked for errors . multivariate logistic regression with backward elimination was used to calculate adjusted odds ratios for the independent association between toxoplasma infection ( defined as the presence of specific igg antibodies ) and possible risk factors . for logistic regression analysis , stata version 7 software ( stata corporation , college station , tx , usa ) was used . a total of 231 pregnant women were identified during the study period , and all agreed to participate ( median age 23 years , range 1443 years ) . of these women , 161 ( 69.7% , 95% confidence interval [ ci ] 63.375.6 ) had igg antibodies against t. gondi . prevalence was not significantly higher in older women than in women < 25 years of age ( p = 0.5 ) . five women ( 2.2% , 95% ci 0.75.0 ) had igm antibodies ; of these women , 3 ( 60% ) had low - avidity igg antibodies . specific igg showed that none of the demographic or socioeconomic variables were associated with infection . other risk factors previously described , such as contact with cats or consumption of raw meat , were not associated with igg seropositivity . in the logistic regression model , the only variable associated with igg antibodies to toxoplasma was regular consumption of homemade ice ( table ) . four ( 80% ) of the 5 igm - positive women regularly consumed these ices . this ice is made by people at home , is sold locally , and consists of tap water , artificial flavor , and sugar , frozen in small plastic bags . * according to an ordinal socioeconomic score from 0 to 10 . using a commercial extraction kit ( qiagen , valencia , ca , usa ) , we extracted dna from randomly chosen aliquots ( 1.5 ml ) of > 50 homemade ice samples obtained from local vendors in cascavel and performed a standardized nested pcr assay ( t. gondii b1 gene , sensitivity 1 parasite ) . the igg prevalence of 70% found in this study is consistent with results of a study in fortaleza in which 72% of pregnant and postpartum women were seropositive for igg to toxoplasmosis ( 8) . in our study population , prevalence did not increase with age , which indicated that in this setting most infections occur in childhood or adolescence . risk factors identified in other studies were not associated with toxoplasmosis in the typical semirural community in our study . a previous study from brazil reported an outbreak of toxoplasmosis associated with the consumption of raw mutton ( 9 ) . other studies from south brazil suggested that consumption of undercooked beef and working in a garden were risk factors ( 3,10 ) . we did not find an association between consumption of raw meat and seropositivity , which may be because people in northeastern brazil ( unlike those in southern brazil ) do not eat undercooked or raw meat . ownership of free - ranging chickens and consumption of cheese were negatively associated with toxoplasmosis . these 2 variables are associated with higher socioeconomic status in rural communities in northeastern brazil . we can not rule out that our results were confounded , even when we used a score to quantify socioeconomic status in multivariate regression analysis . we found that homemade ice , which is stored in small plastic bags , was a possible risk for infection . this finding does not rule out that the water was contaminated because identification of parasites in water requires large volumes . drinking water ( which is used for the preparation of this type of ice ) was not a risk factor for infection . we can not rule the possibility that the outer surface of the plastic bags in which the ice was packed was contaminated by oocysts from soil . these plastic bags are often opened by ripping them with the teeth , which may result in infection . ( 3 ) , who identified a marginal association between consumption of homemade ice stored in plastic bags and t. gondii infection . because our study was community based and included virtually all women who were pregnant during the study period , . however , our study has limitations . because of the cross - sectional design , causal and temporal relationships are difficult to establish . additionally , because few women were igm positive , igg positivity was used as a marker for toxoplasma infection . however , because igg antibodies to toxoplama persist for years , many infections had probably been acquired some years ago , the environment and behavior patterns may have changed , and risks that are no longer present would not have been included . in conclusion , the study indicates that the pattern of risk factors for infection is different from that found in other studies . future studies should show if these results are caused by chance or unknown confounders , or if the consumption of homemade ice has a direct association with infection with t. gondii .
two waterborne outbreaks of toxoplasmosis have been described recently in southern brazil . we present data from a community - based study of pregnant women in northeastern brazil . consumption of homemade ice was the only variable associated with seropositivity ( adjusted odds ratio , 3.1 , 95% confidence interval , 1.536.24 ) . our results suggest water as a source of infection with toxoplasma gondii .
toxoplasmosis , a zoonosis caused by a protozoan , toxoplasma gondii , is probably the most widespread human parasitosis . asexual phase occur in warm blooded animals , and human acquire the parasite in this phase ( 1 ) . most pregnant women with acute acquired infection do not experience obvious symptoms or signs ( 2 ) . acute and latent t.gondii infections during pregnancy are most commonly diagnosed by detecting the immunoglobulin igg and igm antibodies in the serum samples of the patients using enzyme - linked immunosorbent assay ( elisa ) ( fig . 1 ) ( 3 ) . the circulation of toxoplasma gondii transmission form animals to pregnant women . chemokines are a group of chemotactic polypeptides that are key mediators of leukocyte activation and chemotaxis ( 1 , 4 ) . they are divided into groups of related families based on the arrangement of cysteine residues in their amino - terminal domain ( 5 ) . the c - x - c or b - chemokines , of which il-8 is a prototype , are primarily involved in the recruitment and activation of neutrophils , although they may attract other leukocyte populations ( 4 ) . interleukin-8 ( il-8 ) is produced by macrophages and other cell types such as epithelial cells and endothelial cells . primary function of il-8 is the induction of chemotaxis in its target cells like neutrophil and granulocytes ( 3 ) . it has been cited as a pro - inflammatory mediator in toxoplasmosis ( 1 ) . it is well recognized that t cell - mediated immunity plays a central role in the host response to intracellular pathogens ( 2 ) . t cell- mediated immunity and activated macrophages have been shown to play important roles in resistance to t cell - mediated immunity t. gondii infection ( 68 ) . in the current study we evaluated the serum levels of il8 in three groups of pregnant women serums ; group i women with igg positive , t. gondii serology , group ii igm positive t. gondii serology and group iii , a control group , with negative t. gondii serology . the pregnant women who participate in this study were attending rural and urban health care centers . during the period of january 2007 to july 2009 , two hundred and thirty five ( 43.9% ) and 333 ( 56.1% ) samples were collected from rural and urban areas respectively . after obtaining consent , demographic data , clinical data ( including obstetric history , gestational age ) and behavioral data ( including animal contacts , exposure to cat faeces ) il8 level was measured by commercial enzyme linked immunosorbant assay kit ( human il8/nap-1 elisa , bender medsystems , austria ) in all recruited pregnant women according to the manufacturer 's instruction . for data analysis and statistics , percentages were compared by rates and proportion ; 95% confidence intervals are reported and the differences were considered to be statistically significant when the p value obtained was less than 0.05 . the igg antibodies were found in 121 of 333 ( 36.3% ) urban and 81 of 235 ( 34.5% ) rural pregnant women , whereas igm antibodies to t.gondii were found in 40 of 333 ( 12% ) urban pregnant and 26 of 235 ( 11% ) rural pregnant women ( table 1 ) . the mean serum concentration of il8 was not statistically significant different when urban and rural subjects were compared ( table 2 ) . seroprevalence of toxoplasmosis in different groups of pregnant women according to different variables figures in the parentheses represent percent . the mean serum concentration of il-8 in chronic and acute phase of t.gondii infection in pregnant women with positive t.gondii igg serology was 116.1 pg / ml and 134.8 pg / ml in pregnant women with positive t.gondii igm serology . however , in healthy subjects , the mean il-8 serum concentration was ( mean 68.9 pg / ml ) ( p < 0.001 ) significantly lower than the mean il-8 serum concentration in pregnant women with positive t.gondii igm serology . the mean il-8 serum concentration in pregnant women with limited educational achievements was statistically higher than the mean il-8 serum concentration educated women ( p < 0.006 ) . eighty seven of 568 patients had been contact with cats . of these 87 subjects , there was no significant difference in the mean il-8 serum concentration between the groups ( table 2 ) . acute toxoplasmosis causes host cell lysis and an inflammatory infiltrate consisting of lymphocytes , macrophages , and neutrophils . one signal for the observed cellular infiltrate after t. gondii infection is the release of pro - inflammatory chemokines from infected cells . infection of primary fibroblasts , as well as transformed epithelial cell lines , with t.gondii stimulates secretion of the pro - inflammatory chemokines like il-8 . the chemokine response is dependent on invasion by live tachyzoites and subsequent host cell lysis . furthermore , supernatants or lysates from t.gondii infected fibroblasts could elicit significant il-8 secretion ( 10 ) . increased level of il-8 correlates with early acute inflammation or with a reactive form of toxoplasmosis . il-8 is responsible for activation and recirculation of neutrophils and neutrophils can phagocytose and kill or inhibit tachyzoites of toxoplasma and showed that human intestinal epithelial cells infected with t.gondii elicit rapid secretion of il-8 ( 7 ) , so it has an important role in innate immunity in response to toxoplasma . in our study , the concentration of il-8 level in patients with serological evidence of acute infection with t.gondii was statistically significantly higher than in healthy subjects . there was no significant difference in the mean il-8 serum concentration when urban and rural subjects were compared . furthermore a history of previous miscarriage or exposure to cats was not associated with a significant difference in the mean il-8 serum concentration . in this report , we demonstrate that mean level of il-8 in pregnant women with serological evidence of t.gondii infection was higher than other groups .
aimwe investigated whether the level of il8 was different in patients with chronic and acute toxoplasma gondii infection during the pregnancy compared with control group.backgroundit is well established that t.gondii infection induces a strong cell - mediated immune response.patients and methodselisa was used to determine the level of il8 in sera of 568 pregnant women . patients were divided into three groups according to a t.gondii serology . the first group included 202 women with positive igg titres , the second group was 66 women with igm and negative igg t.gondii serology ; and the third group comprised the sera of 300 healthy pregnant women with negative t.gondii serology and served as controls.resultsthe level of il8 in group i was within normal range similar to control group . however , the level of il8 was increased in those pregnant patients with positive igm t.gondii serology.conclusionthe serum levels of pro - inflammatory cytokines such as il8 seem to be increased in patients with serological evidence of acute t.gondii infection .
cutaneous polyarteritis nodosa ( cpan ) is an uncommon and rare form of cutaneous vasculitis . it should be differentiated from systemic polyarteritis nodosa ( pan ) due to the different clinical course and management of the two conditions . the diagnosis is based on skin biopsy , as there are no specific serological tests . the treatment is with steroids , cyclophosphamide or other immunosuppressant though there is no effective definitive therapy . a fourteen - year - old girl presented with history of fever , painful subcutaneous nodules with ulcerations in both the lower limbs for two months , and digital gangrene of the right index finger for one - month duration . there was no history of purpura , raynaud 's phenomenon , recurrent oral ulcers , hair loss or malar rash . the general physical examination showed normal vitals with multiple subcutaneous ulcers predominantly distributed over both the lower limbs [ figures 1a and b ] and bluish black discoloration of distal phalanx of the index finger . the peripheral smear showed elevated total count and anti - streptolysin o ( aslo ) titer was elevated . the deep incisional skin biopsy taken from the subcutaneous nodule revealed leukocytoclastic vasculitis of the dermal vessels [ figures 2a and b ] . the patient was treated with methyl prednisolone ( three pulses doses of 750 mg / day ) followed by oral prednisolone of 1 mg / kg / day . the skin lesions completely healed over a period of six months with scarring [ figures 3a and b ] , and patient was on regular follow up for one and half year after which she lost to follow up and treatment . photograph shows healing subcutaneous ulcer over thigh ( a ) and leg ( b ) immunological profile and other laboratory investigations done for the patient in the study [ h and e , 400 ] shows normal epidermis and the dermis with inflammatory infiltrate , eosinophils and neutrophils ( a ) superficial dermis with destruction of the vessel walls by inflammatory infiltrate ( b ) ( arrow ) photograph shows healed subcutaneous ulcer over thigh with scarring ( a ) and healed gangrene of finger ( b ) ( i.e. , autoamputated distal phalanx ) she presented to us again in july 2010 ( three years after the initial episode in december 2007 ) with similar complaints of bluish black discoloration of left middle finger and subcutaneous nodules . the skin biopsy was repeated which showed leukocytoclastic vasculitis of the dermal vessels suggestive of cpan . the patient was treated with injection cyclophosphamide 750 mg / meter square ( 6 pulse doses every month ) and advised to continue steroids ( prednisolone 30 mg / day ) and methotrexate 7.5 mg / week . the patient is on regular follow up in our immunology clinic and there is no further episode of relapse till date . the precise etiology of cpan remains unknown , but immune complex mediated disease plays a role in etiopathogenesis . there is high prevalence of igm antiphosphatidylserine prothrombin complex among patients with cpan . these immunoglobulins are presumed to activate the classical complement pathway to cause cpan . several infectious and noninfectious conditions have been associated both with initiation and relapse of the disease . others infectious agents implicated are parvovirus b19 , mycobacterium , hepatitis viruses b and c. the noninfectious conditions associated with cpan are connective tissue diseases ( systemic lupus erythematosus , rheumatoid arthritis ) , wegener 's granulomatosis and churg - strauss syndrome . in cpan , the characteristic manifestations are tender subcutaneous nodule , livedo reticularis , and ulceration , mostly localized on the lower extremity . the subcutaneous tender , erythematous nodules ( usually 0.5 - 3 cm in diameter ) may disappear spontaneously or undergo ulceration . other findings include petechiae , purpura , cutaneous necrosis , auto amputations , and local extracutaneous manifestations like arthralgia , myalgia , constitutional symptoms ( such as fever , malaise ) and peripheral neuropathy ( mononeuropathy and mononeuritis multiplex ) . the diagnosis is based on clinical features of isolated skin involvement and confirmed by histopathological findings . a deep incisional biopsy , including subcutaneous tissue , is necessary for the accurate diagnosis of the disease . the characteristic pathologic feature is a leukocytoclastic vasculitis in the small to medium sized arterioles of the deep dermis or hypodermis with or without associated fibrinoid necrosis . both cpan and systemic pan share the same histopathologic features of necrotizing arteritis of small and medium sized vessels . immunological testing does not appear helpful in confirming the diagnosis of cpan , however , negative results help to exclude other systemic vasculitis . corticosteroids remain the mainstay of treatment for cpan and are used in most severe cases . immunosuppressive agents such as cyclophosphamide , azathioprine , or methotrexate , can been used in cases unresponsive to steroid therapy . the non - steroidal anti - inflammatory ( nsaids ) drugs are used only in the milder form of cpan , and antibiotics are used in cases of antecedent streptococcal throat infections . in cases resistant to systemic steroids and immunosuppressive therapy , there are some reports that vasodilators and antithrombotic agents are effective , especially for ulcers and gangrene associated with cpan . though cpan has chronic , relapsing benign course there are reports of it evolving into systemic pan . initial episode was treated with steroids alone and following relapse she received six pulse doses of cyclophosphamide and presently she is on regular follow up with drugs , prednisolone ( 30 mg / day ) and methotrexate ( 7.5 mg / week ) . in conclusion , cpan is a rare and benign cutaneous vasculitis of unknown etiology with chronic and relapsing course distinct from systemic pan . however , these patients should be kept on close regular follow up for its evolution into systemic pan .
cutaneous polyarteritis nodosa ( cpan ) is a rare form of cutaneous vasculitis that involves small and medium sized arteries of the dermis and subcutaneous tissue without systemic involvement . it presents with tender subcutaneous nodules , digital gangrene , livedo reticularis and subcutaneous ulcerations . the diagnosis is by skin biopsy and characteristic pathologic feature is a leukocytoclastic vasculitis in the small to medium - sized arterioles of the dermis . we report a rare case of benign cutaneous pan in a 14-year - old girl who presented with history of fever , subcutaneous nodules with cutaneous ulcer and digital gangrene . the skin biopsy showed leukocytoclastic vasculitis with fibrinoid necrosis in the dermal vessels . she received treatment with steroids and lesions resolved completely over a period of month .
the syndrome of severe , acute , intensive care unit ( icu)-acquired neuromuscular weakness poses a common and serious diagnostic , prognostic , and therefore management issue . it goes by various names , some of which presuppose a mechanism : acute necrotizing myopathy of intensive care , acute quadriplegic myopathy , critical care myopathy , critical illness myopathy ( cim ) , critical illness neuromuscular disease , critical illness neuromyopathy , critical illness polyneuromyopathy , critical illness polyneuropathy ( cip ) , icu - acquired paresis , quadriplegic and areflexic icu illness , rapidly evolving myopathy with myosin - deficient fibres and thick filament myopathy [ 1 - 6 ] . the problem affects at least 1.7% of children in paediatric icus , more than half of adult patients admitted to general icus for more than 1 week and more than 70% of those with sepsis and multiorgan failure [ 7 - 9 ] . neuromuscular weakness typically becomes apparent when an attempt is made to wean the patient from the ventilator , although there are earlier clues , which include grimacing without movement with noxious stimuli before recovery of consciousness , relative lack of movement after regaining consciousness , and ( not inevitably ) loss of deep tendon reflexes that had been present earlier . although most cases will turn out to be critical illness polyneuromyopathy a term that embraces cip , cim , or a combination of the two other potential causes should not be overlooked . 1 . the algorithm illustrates the early ruling in or out of spinal cord disease ( e.g. in cases of trauma , coagulation disturbance , west nile virus infection , acute disseminated encephalomyelitis , etc . ) , and then moving on to a clinical biochemical electromyographic assessment . a neuromuscular transmission defect ( e.g. slow inactivation of neuromuscular blocking agents , unrecognized myasthenia gravis , or myasthenic [ lambert eaton ] syndrome ) is easily detected with repetitive nerve stimulation , revealing either a decremental or incremental response . neuropathies other than cip that may manifest after icu admission include guillain barr syndrome / acute immune - mediated demyelinating polyneuropathy ( and its various subtypes ) , porphyria and recurrent chronic inflammatory demyelinating polyneuropathy . demyelinating inflammatory neuropathies usually cause slowing of conduction velocity and conduction block on electromyographic studies and produce increased protein in the cerebrospinal fluid . other conditions , such as inflammatory myopathies or unrecognized motor neurone disease , are not discussed here because they are usually diagnosed before admission to icu , although they sometimes present with respiratory weakness that requires icu admission . the last step , differentiating the most common causes of icu - acquired generalized weakness ( myopathy , neuropathy , or a combination of the two ) , is practical because their prognoses differ considerably . some cases of myopathy appear to be merely ' membranopathies ' , with normal histology and rapid recovery . presumably , the muscle membrane becomes dysfunctional , inexcitable and leaky , allowing myoglobin to leave the muscle . others , especially in those treated with corticosteroids and neuromuscular blocking agents , exhibit a relative loss of thick myosin filaments . many cases show variable panfascicular necrosis , which can sometimes be widespread and severe . in general , the prognosis for recovery from myopathies is favourable , with most patients recovering fully within 13 months . this takes many months and recovery is often incomplete , leaving patients with significant weakness , sensory loss , and absent reflexes distally in the lower limbs and variably more proximally . patients with cip who fail to show significant recovery by 4 weeks are often disabled with diminished quality of life . some older patients may fail to survive or wean from the ventilator because their recovery is so protracted and other complications ensue . electromyography is helpful in differentiating cip from cim but it has limitations . reduced or absent sensory nerve action potentials favours a neuropathy , but sensory potentials may be difficult to record if there is considerable oedema or a pre - existing polyneuropathy ( e.g. from diabetes mellitus ) may have clouded the issue . direct muscle stimulation ( not commonly done ) may reveal no or absent response in cim but normal responses in cip . unfortunately , needle electrode studies of muscle can show similar features in cip and cim ; both may exhibit spontaneous activity ( fibrillations and positive sharp waves ) . often cip and cim coexist and their relative contributions to the weakness may vary considerably when this occurs . elevated serum creatine kinase may help to identify cim , but the peak may be missed in the membranopathy / necrotizing varieties or creatine kinase may not be significantly elevated in cases with loss of myosin filaments . to determine the relative contributions of nerve versus muscle disease in explaining weakness , muscle or both nerve and muscle biopsies have been utilized and recommended , most recently in this issue by kerbaul and colleagues . in most cases muscle biopsy will address the relative contribution of myopathy to the picture because the neuropathy can be adequately assessed electrophysiologically . percutaneous muscle biopsy , although providing limited tissue , has a number of advantages over operative biopsies : greater spatial sampling , minimal bleeding , negligible infection rate ( we have had none in over 1000 biopsies ) , portability ( done at the bedside ) , no general anaesthetic , speed of performance and ease of arrangement . nerve and muscle biopsy is seldom necessary in the icu , unless a vasculitis is suspected . being aware of the incidence and signs of icu - acquired weakness with ventilatory failure and having an approach to such disorders will prove valuable in management . further insights into the mechanisms of cip and cim may provide some preventive strategies that will ameliorate their severity , shorten the duration of icu stays and improve long - term outcomes . cim = critical illness myopathy ; cip = critical illness polyneuropathy ; icu = intensive care unit . a flow chart giving an approach to generalized weakness and/or ventilatory failure in the intensive care unit . ck , creatine kinase ; csf , cerebrospinal fluid ; gbs , guillain barr syndrome ; emg , electromyography ; mri , magnetic resonance imaging ; lems , lambert eaton ( myasthenic ) syndrome ; mg , myasthenia gravis ; ncv , nerve conduction velocity studies ; n - m , neuromuscular transmission ; snaps , sensory nerve action potentials . modified from bolton and young .
icu - acquired limb and respiratory muscle weakness is a common , serious icu syndrome , increasing in frequency with prolonged icu stay and sepsis . a systematic approach facilitates precise localization of the problem within central or peripheral nervous system . most cases relate to critical illness polyneuropathy or myopathy or a combination of both ( critical illness neuromyopathy ) . within the latter entity , the relative contribution of neuropathy versus myopathy varies considerably among affected patients . muscle enzyme testing , electromyography - nerve conduction and muscle biopsy are valuable investigative tests . nerve biopsy is less commonly needed , but is useful when vascultis is suspected .
peritoneal dialysis ( pd ) catheter rupture is rare , but can occur traumatically or spontaneously . when the rupture occurs close or proximal to the exit site however , this requires an interruption in pd and subsequent hemodialysis ( hd ) catheter insertion for temporary transfer to hd . therefore , partial replantation of the catheter instead of removal of the whole catheter has been suggested , , . in previous reports of partial replantation , medical adhesive glue or a specific endoluminal connector was used to connect the remaining part of the old catheter to the extraperitoneal segment of a new catheter . in this report , partial replantation using two pd adaptors and part of a transfer set as a new connecting technique in a patient with a spontaneously ruptured catheter is introduced . a 36-year - old man on continuous ambulatory peritoneal dialysis ( capd ) visited an outpatient clinic because of dialysate leakage from the exit site . he began capd after surgical implantation of a double - cuff standard swan neck catheter through a paramedian incision in 2005 . the rupture site was 2 cm distal to the exit site , and a new pd adaptor was connected to the catheter after cutting it at the level of the rupture site . although the adaptor was located very close to the exit site , capd was maintained with the catheter ( fig . 1 ) . at the time of his visit , a small rupture was discovered posteriorly near the outer cuff when the catheter was pulled away from the exit site ( fig . 2 ) . peritoneal fluid leakage was easily discernible from the rupture site without pericatheter leakage , edema , redness , or tenderness at the exit site . the catheter tip was appropriately positioned inside the pelvic cavity and the catheter function was excellent . because the catheter rupture site was located between the exit site and the outer cuff , it was decided to rescue the inner conduit and perform a salvage procedure rather than the radical removal and exchange of the whole catheter . a longitudinal skin incision from the exit site along the catheter and dissection were carried out to expose the outer cuff and tunnel ( fig . then , the catheter was cut proximal to the rupture site and the outer cuff ( fig . we used two pd adaptors to connect the end of the remaining old catheter to a new extraperitoneal segment of a new catheter ( catheter adaptor luer lock ; fresenius medical care deutschland gmbh , bad homburg , germany ) . because we could not directly connect the two pd adaptors , a piece of a transfer set ( stay safe catheter extension luer lock 25 cm ; fresenius medical care deutschland gmbh ) was placed between the pd adaptors ( figs . after connecting the old and new catheters , the skin incision was extended downward from the old exit site in order to implant the two pd adaptors into the tunnel to leave 1 cm of tunnel between the exit site and the end of the distal pd adaptor ( fig . the patient promptly restarted capd after surgery and was discharged on the 2 postoperative day . over 6 months of follow up , the catheter function was excellent and there was no evidence of peritonitis , exit site infection , or dialysate leakage . all cases with a rupture near the exit site were managed by radical removal of the old catheter and reinsertion of a new catheter , . this catheter exchange may be complicated by hemorrhage , peritoneal leakage , or malposition of the catheter associated with the surgery , and interruption of pd and hd catheter insertion are required for temporary transfer to hd . therefore , we sought to address the application of partial replantation ( replacement of the extraperitoneal segment of the catheter ) of a pd catheter , which has been performed in patients with intractable exit - site / tunnel infection to salvage the still - functioning conduit . in previous reports of partial replantation , a medical adhesive glue or a specific endoluminal connector was used to connect the remaining part of the old catheter to the extraperitoneal segment of a new catheter . in early reports , , a stenting tube ( a segment of siliconized tube taken from an infusion extension set ) was inserted into the inner lumen of both catheters , and an adhesive silicone type a glue was used to seal the interfaces between the catheters and the stenting tube . the authors indicated that the length of the stenting tube is very important , because a tube that is too short increases the risk of leakage or disconnection , and a tube that is too long protrudes and causes the patient discomfort . furthermore , the adhesive material may induce a chemical reaction with some types of catheters . dialysate leakage due to a defective connection occurred in one out of seven patients , and that connector is no longer commercially available . in our case , we used two pd adaptors and a part of a transfer set , which were inexpensive and easily accessible because they are kept in the pd unit at all times . the part of the transfer set was detachable and could be connected to a pd adaptor using its luer lock design , enabling the connection of the two catheters between two pd adaptors . this new connecting technique was easy to do and there was no dialysate leakage or disconnection . these devices have been demonstrated to be safe by standardized tests , including intradermal reactivity , cytotoxicity , hypersensitivity , systemic toxicity , hemocompatibility , hemolysis , and sterility studies . all of these biologic evaluations were executed by the korea testing laboratory under the official request of the korean food and drug administration . however , long - term follow up of the patient is needed to evaluate the biological safety of this procedure . furthermore , this procedure should not be performed in the same subcutaneous tunnel in a case with concomitant tunnel infection . therefore , we would recommend this salvage technique in a case of ruptured pd catheter without tunnel infection . in conclusion , partial pd catheter replantation is a simple , safe , and effective salvage procedure for patients with catheter injury . our new connecting technique using pd adaptors and part of a transfer set is a novel means of partial pd catheter replantation .
peritoneal dialysis catheter ruptures have been managed by immediate removal and subsequent reinsertion of the catheter which inevitably entails interruption in peritoneal dialysis and a need for vascular access . a 36-year - old man on continuous ambulatory peritoneal dialysis complaining of dialysate leakage was found to have a small rupture near the outer cuff of the peritoneal dialysis catheter . rather than employing the traditional method of exchanging the whole catheter , a partial replantation procedure to salvage the still - functioning conduit was performed . two peritoneal dialysis adaptors were used to connect the end of the remaining old catheter to a new extraperitoneal segment of a new catheter and a piece of a transfer set to connect the adaptors . a novel , yet simple and safe , means of partial peritoneal dialysis catheter replantation when managing catheter injuries is suggested .
a congenital midline cleft of the sternum is a rare developmental anomaly , usually occurring as a part of a defined syndrome , e.g. , cantrell 's pentalogy . it results from failed ventral midline fusion of the sternal bars , which normally occurs during the first 3 months of embryonic life . isolated sternal cleft without any associated anomaly is very uncommon and presents in early infancy because of the obvious cosmetic deformity . we report an extremely rare case of isolated upper sternal cleft in an adult male patient , found incidentally on technetium-99 m ( tc-99 m ) methylene diphosphonate ( mdp ) bone scan . a 45-years old male patient , who was a recently diagnosed case of moderately differentiated adenocarcinoma of prostate with gleason 's score of 3 + 4 = 7 , was referred for tc-99 m mdp bone scan to rule out skeletal metastases . he complained of upper backache for 2 months and had a history of trauma to the left lower leg 1 week prior to performing the bone scan . the serum prostate specific antigen level was > 100ng / ml . on clinical examination , there was a midline bony defect in the region of the manubrium sterni with no apparent bulge or paradoxical respiratory movements . a whole body planar tc-99 m mdp bone scan and single - photon emission computed tomography - computed tomography ( spect - ct ) of the thoracic region was performed 3 h after intravenous injection of 20 mci of tc-99 m mdp on a dual head gamma camera ( infinia : single slice spect - ct ; ge , usa ) . the planar images of the bone scan revealed focal mdp uptake in the d4 vertebra [ figure 1 ( red arrow ) ] , focal uptake in the mid shaft of the left tibia [ figure 1 ( blue arrow ) ] and a u - shaped photopenia in the region of the sternum [ figure 1 ( black arrow ) ] . corresponding spect - ct images of the thorax revealed localization of the focal tracer uptake in the sclerotic metastasis in the body of the d4 vertebra [ figure 2 ( red arrows ) ] . the u - shaped photopenia in the sternum corresponded to a bony cleft involving the manubrium and upper half of the sternum [ figure 2 ( blue arrow ) ] . the anterior ( a ) and posterior ( b ) planar technetium-99 m methylene diphosphonate bone scan images show a u - shaped photopenic defect involving the manubrium and upper half of the sternum ( black arrow ) . focal tracer uptake in the mid shaft of the left tibia corresponds to known traumatic injury at the same site ( blue arrow ) . focal tracer uptake is seen at a metastatic site in the d4 vertebra ( red arrow ) coronal , sagittal , and transaxial images of the single - photon emission computed tomography - computed tomography of the thorax demonstrates photopenia in the region of superior sternal cleft ( blue arrow ) . the computed tomogram demonstrated bilateral paired sternal cartilaginous centers on either side of the wide gap in the anterior chest wall . the inferior most sternal segment and xiphoid were positioned at the midline and were covered by normal appearing cutaneous tissues . a sclerotic lesion is noted in the body of the d4 vertebra ( red arrow ) the tc-99 m mdp bone scan thus helped in diagnosing a congenital superior sternal cleft , metastasis in the body of the d4 vertebra and post - traumatic changes in the mid shaft of the left tibia . a cleft sternum , also known as a bifid sternum or sternal fissure , is a partial or a complete separation of the two lateral sternal bars . sternal cleft is a rare anomaly with reported incidence < 1% of all chest wall malformations . most isolated sternal defects are due to failure of the mesenchymal lateral processes to fuse during the 8 week of gestation . although the embryologic development of the sternum has been studied in detail , the etiology of the cleft sternum is unknown . congenital sternal clefts can be divided into three separate varieties : superior , inferior and complete . the most frequently encountered form is the partial superior type , accounting for 67% of all patients , followed by the complete form ( 19.5% ) , the partial inferior form ( 11% ) and the sternal foramen ( 2.5% ) . the superior clefts have been described as u - shaped if the cleft extends to the last sternal body segment or v - shaped if it extends to the xiphoid process . complete absence of the sternum as opposed to a sternal cleft , is usually seen in other fetal anomalies such as ectopia cordis or the pentalogy of cantrell and phaces ( posterior fossa malformations , hemangiomas , arterial malformations , coarctation of the aorta / cardiac defects , eye abnormalities , and sternal defects ) syndrome . our patient had an isolated superior sternal cleft with a u - shaped photopenic defect in the manubrium sterni on the tc-99 m mdp bone scan . the other differential diagnoses of photopenia in the sternum include sternal foramen , metal attenuation artefact and metastasis in a known case of malignancy . to conclude , our case demonstrates that not only areas of increased tracer uptake , but areas of photopenia on a bone scan should also be carefully evaluated . awareness about the sternal cleft and other differential diagnosis is necessary when reporting a photopenic defect in the sternum .
a sternal cleft is an extremely rare developmental anomaly , which results from failure of fusion of sternal bars which contribute to the formation of the sternum . most cases are diagnosed in early childhood , where it is associated with serious other midline defects . a sternal cleft is seen as a photopenic area on technetium-99 m methylene diphosphonate bone scan and can be confused with other conditions . we report an extremely rare case of isolated upper sternal cleft in a 45-years - old male , found incidentally on tc-99 m mdp bone scan .
verrucous hemangioma is a rare variety of angiomatous nevi usually involving the , lower extremities . it has been reported under a diversity of names such as angiokeratoma circum - scriptum neviforme , unilateral verrucous heman - gioma , keratotic hemangioma etc . they are best treated by excision while still small , as they enlarge with body growth and do not regress spontaneously . in contrast to angiokeratomas , which respond to various means of therapy ( e.g. , cryotherapy , electrocautery , and argon laser ) , verrucous hemangiomas require a large , deep excision as a superficial therapeutic approach would be unsuccessful because of the deep angiomatous proliferation . a 14-year - old girl presented with an exuberant warty brownish growth on her right great toe . history revealed that when she was 4 years old , she had a small similar lesion , a part of which was biopsied by a general surgeon and was reported as angiokeratoma . , the lesion recurred and continued to grow in a slow but progressive manner to the present size without any treatment . examination revealed a solitary hyperpigmented , hyperkeratotic plaque consisting of closely set warty papules measuring 20 25 mm involving the pulp space of the right great toe . surface of the lesion was firm and verrucous with no ulceration , bleeding , or atrophy . we considered a clinical differential diagnosis of tuberculosis verrucosa cutis and angiokeratoma [ figure 1 ] . the entire growth was excised by a wide excision resulting in a defect that involved one - third of the pulp space of the great toe extending till the bone . a plantar digital artery flap was designed in a v - y fashion adjacent and just proximal to the excision wound . skeletonizing this pedicle permitted the elevated flap to advance and cover the excision defect completely with sensate , glabrous plantar skin without any contour defect . hyperpigmented verrucous plaque over the pulp space of the great toe the excised material was subjected to histological examination . h and e stained sections of the specimen showed marked hyperkeratosis , parakeratosis , papillomatosis , and elongation of the rete ridges . extending from the dermo - epidermal junction into the deeper dermis , there were numerous small to large vascular channels lined by flattened endothelial cells and filled with blood . lesions are mostly seen at birth or appear during childhood . initially , they are soft , bluish - red vascular lesions , but with time they enlarge and develop keratotic and verrucous features . secondary bleeding and infection are frequent complications , which often result in reactive papillomatosis and hyperkeratosis and thus the older lesions acquire a warty surface . unlike other angiomatous nevi , they do not involute spontaneously . clinically , verrucous hemangioma mimicks angiokeratoma , but histopathologically they can be clearly differentiated as the former involves the dermis and subcutaneous fat and the latter involves only the papillary dermis . hence , deep excisional surgery is the proper treatment for verrucous hemangioma as a superficial therapeutic approach would be unsuccessful because of the deep angiomatous proliferation . superficial ablative procedures such as electrocautery , cryosurgery , and laser ablation almost invariably lead to recurrence of the lesion . a combined approach using surgery and erbium laser have been reported with good results . in our case , as the large defect size would not permit a direct primary closure , either a skin graft or a flap was needed . a skin graft would have had disadvantages such as ; ( a ) uncertain acceptance on the bare bone with risk of delayed healing , ( b ) a sunken appearance resulting in poor aesthesis causing social anxiety , and ( c ) poor tolerance to wear and tear . although conventional local flaps would heal the wound reliably , loss of the toe contour and the skin graft needed on the flap donor area would leave obvious cosmetic deformity on the young girl 's toes . therefore , we performed a wide local excision of the growth and closed the defect with a plantar digital artery flap designed in a v - y fashion just proximal to the excision wound . excellent color , contour match as well as preservation of sensation in 2 weeks ensured a good aesthetic reconstruction . the digit shape was also preserved to the satisfaction of the patient [ figures 2a d ] ( a ) verrucous growth excised by a wide local excision . ( b ) a plantar digital artery flap was designed in a v - y fashion adjacent and just proximal to the excision wound . ( c ) the pedicle was skeletonized permitting the flap to advance by about 1 cm to cover the entire excision defect . skin lesions needing excisions very often can not be closed primarily or tension of primary closure results in unacceptable distortion of adjacent anatomic landmarks . while the former problem may present in any area of the body , the latter usually presents on vital areas such as face , hand , foot , or genitals . when wound healing alone is the concern , simple skin grafts - either partial / split ( stsg ) or full thickness ( ftsg ) may suffice . however , these are usually associated with problems of function ( unable to tolerate wear and tear , inadequate sensation or contraction of graft ) or aesthesis ( color or texture mismatch , contour defect ) . in this case , there was the additional uncertainty of poor acceptance of the graft on bare bone with the risk of delayed healing . although conventional local flaps would heal the wound reliably , loss of the toe contour and the skin graft needed on the flap donor area would leave obvious stigmas on the young girl 's toes . this final problem can also be avoided by careful design and dissection of the flap to permit direct closure of flap donor areas , as demonstrated in our case [ figures 3a and b ] . ( b ) postoperative picture with acceptable contour and good aesthetic reconstruction of the great toe yang and ohara reported 14 patients presenting with small , localized lesions that were cured by one session of surgery without recurrence , while the 9 patients with wider and more extensive lesions required combination therapy in several stages for optimal results . reported a case of verrucous hemangioma over the lateral aspect of left leg of a 19-year - old girl , treated with multiple sessions of electrocautery , which healed with scarring and hyperpigmentation . used percutaneous injection of n - butyl cyanoacrylate ( nbca ) prior to excision of the lesion which on contact with tissue fluid , causes inter compartmental tissue adhesion , thereby causing cessation of blood flow into the vascular malformation tissue . after few injections , the whole mass forms a solid polymer , which is then excised . during excision , there is remarkably minimum bleeding . when dimensions permit , local flaps are far better than distant flaps in vital areas , on grounds of better function and aesthesis . the limit for local flap dimensions in critical areas is reached when the donor area can not be closed primarily and is likely to need a skin graft . in areas with axial pattern blood supply , flaps can be islanded and creatively designed to permit larger donor areas to be closed primarily . this ensures the best of wound healing , function , and aesthesis with minimal scars , which is called aesthetic reconstruction . ability to restore the toe virtually back to normal permits a fearless and wide excision needed for definitive cure of the disease .
a 14-year - girl presented with solitary exuberant warty plaque over her right great toe since the age of 4 years . ten years ago , an excisional biopsy was performed by a surgeon , and a histopathological diagnosis of angiokeratoma was made . a wide local excision of the lesion followed by a plantar digital artery flap in a v - y fashion adjacent and just proximal to the excision wound was performed . this procedure has not only led to complete growth removal , but also a well - preserved digit with an excellent color and contour match .
the brachial plexus is a major and complicated plexus at the root of the neck . it begins in the lateral cervical region ( posterior triangle ) and extends into the axilla in the angle between the clavicle and the lower posterior border of the sternocleidomastoid . this allows the nerve fibers derived from different segments of the spinal cord to be arranged and distributed efficiently in different nerve trunks to the various parts of the upper limb . it emerges between the scaleni anterior and medius , superior to the third part of the subclavian artery , and is covered by platysma , deep fascia , and skin , through which it is palpable . the brachial plexus is formed by the ventral rami of c5 , 6 , 7 , 8 , and t1;[135 ] and small twigs from the ventral rami of c4 and t2 . these constitute the roots of the plexus , which lie in the lower part of the neck , between the scalenus anterior and scalenus medius muscles . the ventral rami of the c5 and c6 unite to form the superior trunk , those of the c7 continue as the middle trunk , and the rami of c8 and t1 unite to form the inferior trunk.[135 ] a short distance above the clavicle , each of these trunks split into anterior and posterior divisions . it may be damaged in open , closed or obstetrical injuries , be pressed upon by a cervical rib or be involved in a tumor . it is encountered in operations upon the root of the neck , and hence it is in danger . knowledge of the variations of the brachial plexus in the anatomy is important to anatomists , radiologists , anesthesiologists , and surgeons . also knowledge of the variation of the brachial plexus may be useful for surgeons for improved guidance during supraclavicular block procedures , and for surgical approaches for brachial plexus . here , we are reporting a case , in which the superior trunk of the brachial plexus was absent unilaterally on the right side . during a study on the formalin - fixed , adult cadaver - neck specimens in the department of anatomy of the medical college , anatomical variations in the formation of the brachial plexus were recorded . in one of the specimens the ventral rami of c5 and c6 were not joined to form the upper trunk [ figure 1 ] . the variation was unilateral and was found only on the right side of the specimen . no other anatomical variation was found on the right side . dissected lower neck and axilla showing absence of the upper trunk the brachial plexus is formed by the ventral rami of c5 , 6 , 7 , 8 , and t1 . in some individuals , trunk divisions or cord formations may be absent in one or the other part of the plexus ; however , the makeup of the terminal branches was unchanged . fazan , et al . , reported a variation on the superior trunk found in two cases , both on the right side . in both cases , the c5 and c6 roots were split in the anterior and posterior divisions . both anterior divisions joined to give origin to an anterior superior trunk and both posterior divisions joined to give origin to a posterior superior trunk. these trunks joined to give origin to the superior trunk . prakash et al . , reported a case in which the middle trunk of brachial plexus was found missing . the c5 , c6 , c7 roots of brachial plexus united to form the upper trunk ; and the c8 and t1 roots formed its lower trunk . after giving two branches , that is , the suprascapular nerve and the nerve to the subclavius , the upper trunk divided into two divisions . the anterior division of the upper trunk continued as a lateral cord , while the posterior division combined with the posterior division of the lower trunk to form a posterior cord of brachial plexus . although variations of brachial plexus are common , the absence of the upper trunk of the brachial plexus is sparesly reported . this may be the result of a lack of connection between c5 and c6 during the early stages of inra uterine life .
the brachial plexus is a complicated plexus supplying the upper limb . the brachial plexus is of great practical importance to the surgeon . it is encountered during operations upon the root of the neck , and hence it is in danger . variations in the formation of the brachial plexus are common ; and knowledge of the variation of the brachial plexus may be useful for surgeons , for improved guidance during supraclavicular block procedures , and for surgical approaches for brachial plexus . here we report a case in which the superior trunk of the brachial plexus was found to be absent on the right side during a study on the cadaver - neck specimens in the department of anatomy .
a three - year - old child presented with complaints of pain , watering and severe photophobia in both eyes since one month . there was history of absence of hair all over her body including eyebrows and eyelashes since birth . there was dryness , itching and scaling of skin since birth , and recurrent self - resolving angular cheilosis . ocular examination revealed bilateral severe meibomianitis , multiple superficial punctate keratitides in the right eye and a large corneal epithelial defect ( 7 mm 7 mm ) in the left eye [ fig . fundus in right eye was within normal limits and in the left eye was not visible . female child with ifap syndrome showing angular cheilosis figure showing corneal epithelial defect in the left eye severe meibomian gland dysfunction showing total absence of hair over the scalp and eyebrows absence of hair in the limbs she was given systemic erythromycin for two weeks . hot fomentation of eyelids , massage of lids with tetracycline eye ointment , liberal lubrication and low - dose steroid fluorometholone bid was advised for two weeks . the epithelial defect in the left eye had healed with minimal scarring and the punctate keratitis resolved over 4 weeks . visual acuity at final follow - up was 20/20 in right eye and 20/60 in left eye . the father had all the systemic features as the daughter [ fig . 3 ] . these are ablepharon ichthyosis , alopecia ichthyosis and pseudo - hermaphrodites , alopecia skeletal abnormalities mental retardation , deafness ichthyosis , dermotrichic syndrome , ectodermal dysplasia alopecia mental retardation syndrome , hayden syndrome , hereditary mucoepithelial syndrome , ifap syndrome , ichthyosis deafness , hirschsprung disease , ichthyosis hypertrichosis hyperhidrosis syndrome , kids syndrome , keratosis follicularis spinosa decalvans , sjogren larson syndrome , triculodermovertebral syndrome and woodhouse syndrome . out of these conditions , ocular involvement is primarily seen in ablepharon ichthyosis syndrome , kids syndrome , ifap syndrome and keratosis follicularis spinulosa decalvans syndrome . in patients with kid syndrome , nails are often dystrophic , teeth may be small or malformed and ocular changes are usually observed during the second or third decade . in addition , there is a congenital hearing loss , palmoplantar hyperkeratosis with leather grain - like keratoderma . she had very severe photophobia , non - scarring ichthyosis , total alopecia , high forehead , large ears and angular stomatitis all features that has been found in cases of ifap syndromes reported till date . an interesting feature of this syndrome is that patients bear a striking resemblance to each other probably due to the loss of hair and photophobia . the ocular features that have been reported up till now are punctate keratopathy , erosion , corneal scarring , atopic keratoconjunctival inflammation , horizontal nystagmus , myopia and absence of eyebrows and eyelashes . the presence of mgd as the cause of tear film instability has not been noted in the previously reported cases . embryologically , the meibomian glands are formed by the ingrowth of the basal epithelial cells at the inner margin and the sweat glands of moll that arise from the wall of the hair sacs . photophobia and punctate keratitis may be due to secondary to tear film instability caused by mgd . have highlighted the pilosebaceous anomaly in a three - year - old boy with ifap syndrome . in our patient , both photophobia and punctate erosions resolved with treatment of mgd . adequate treatment of mgd helped in preventing the recurrent attacks of inflammation and scarring in our patient . most of the reported patients were males , and in some families milder effects were noted in possible transmitting female . recently , a mother and daughter , and two unrelated female patients , with an ifap syndrome have been reported . however , there are no reports till now , which shows a father to daughter transmission like in our case , which may throw some light to the pattern of inheritance in this disease . the daughter had more severe disease as compared to the father indicating the possibility of variable penetration of the genetic abnormality . in our case it seemed likely that the inheritance was x - linked dominant with variable penetrance . in conclusion ,
we are reporting an interesting case of ichthyosis follicularis with alopecia and photophobia syndrome in a daughter and father from the indian subcontinent associated with meibomian gland dysfunction . a three - year - old female child presented with pain , redness and severe photophobia in both eyes since one month . cutaneous examination revealed ichthyosis , absence of hair all over the body including eyebrows , scalp and angular cheilosis . ocular examination revealed bilateral severe meibomianitis , multiple superficial punctate keratitides in right eye and corneal epithelial defect in the left eye . other systemic features were prominent high forehead and large ears . her father had similar symptoms . her symptoms improved after adequate treatment of meibomian gland dysfunction . she is asymptomatic at present .
endobronchial ultrasound ( ebus ) is a well - established technique for visualizing structures around the major airways . enlarged mediastinal nodes and masses can be seen and sampled with the help of an ultrasound probe at the tip of the bronchoscope . while looking for these lesions the major vessels in the chest namely the aorta , the main pulmonary artery and its branches , superior vena cava and azygous vein are also seen and can be confirmed with the help of a color doppler mode on the ultrasound processor . we report one such case where it was seen which is an incidental but an interesting finding . a 21-year - old man had been running fever and cough with chest pain for a fortnight and was being treated at another hospital . clinical examination was unremarkable and initial investigations showed a slightly raised total leucocyte count-11400/cmm and a normal x - ray chest . a contrast - enhanced ct scan of the chest showed patchy infiltrates in the lung periphery and left hilar lymphadenopathy [ figures 1 and 2 ] . he was referred to our unit for a bronchoscopy and ebus evaluation of the left hilar lymph node . the bronchoscopy did not reveal any abnormality and we proceeded to the ebus examination . during the routine initial examination of all the lymph nodal stations it was found that besides a left hilar node there was a large hyperechoeic shadow occupying the lumen of the main pulmonary artery and another similar opacity though smaller shadow , in the right pulmonary artery [ figures 3 and 4 ] . the procedure was completed , by taking transbronchial needle aspirations from the left hilar node . it was assumed that the intravascular opacities were due to thrombi and this was immediately confirmed by performing a ct pulmonary angiography where filling defects were seen in the pulmonary artery and its branches [ figures 5 - 7 ] . ct showing precarinal and left hilar node patchy infiltrate in lung periphery ultrasound picture of thrombus in right pulmonary artery thrombus in left pulmonary artery ct pulmonary angiography ( ctpa ) with thrombus in main pulmonary artery ctpa withe thrombus in right pulmonary artery ctpa with thrombus in left pulmonary artery the patient was put on anticoagulants and made an uneventful recovery . the aspiration from the lymph node showed only lymphoid cells and the bronchial washings did not reveal acid - fast bacilli or any other significant infection . thus , the ebus procedure was very helpful in making an important diagnosis , which was otherwise unsuspected . endobronchial ultrasound has become an invaluable tool in evaluating mediastinal lesions . since the operator also looks at all the major vessels , which are in close apposition to the central airways , any abnormality like an intraluminal thrombus is also likely to be seen during the ebus scan . the presence of a color doppler mode helps in confirming the presence of a vessel . the diagnosis of pulmonary embolism by ebus has been reported by several authors . in a pilot study aumiller et al . reported the feasibility and safety of ebus in detecting pulmonary emboli in the central pulmonary arteries . in a series of 32 cases it was found to be equally accurate as ct pulmonary angiography , which is considered the gold standard for the diagnosis of pulmonary embolism . they concluded that in situations where ct pulmonary angiography was not possible due to contraindications to contrast agents , hemodynamic instability preventing transport , and radiation exposure , ebus proved to be a feasible alternative . santaolalla et al . have reported a case where ebus was able to detect a pulmonary thrombus not picked up on ct angiography . in another case report . le rouzic et al . have reported the incidental detection of a thrombus in the right pulmonary artery by ebus , in a patient who was being investigated for lung cancer . have reported a similar case as ours where a young male who was being investigated for fever and lung opacities was found on ebus to have a definite intravascular thrombus . we believe that our report , probably the first of its kind from the indian subcontinent , again shows that the detection of pulmonary emboli in the central arteries is possible by ebus . with more experience it may find regular usage in situations where conventional ct angiography is contraindicated or not possible .
this is a case report of a young 20-year - old male who was referred for an endobronchial ultrasound ( ebus ) procedure to investigate a fever and a left hilar lymph node and patchy pleural - based opacities seen on ct of the chest . during the ebus procedure besides the left hilar node , a large thrombus was noticed in the main pulmonary artery and another one in the right pulmonary artery . ebus proved invaluable in making a diagnosis in an unsuspected case of pulmonary embolism .
it is now recognized that both acute aerobic and anaerobic exercise can cause to production of free radicals that lead to acute oxidative stress , which can result in oxidative damage and induce inflammation . recent studies have demonstrated that there is a link between plasma concentration of inflammatory mediators and pathogenesis of insulin resistance , hypertension , obesity , and complications of diabetes such as retinopathy . furthermore , there are some evidences about the association of low - grade inflammation and cardiovascular risk . resveratrol is a natural anti - oxidant polyphenol that is present in red wines , grapes , and roots of polygonum cuspidatum that has received noticeable attention in recent years . this strong polyphenolic compound has shown several biological functions such as anti - inflammatory and anti - oxidant . it has been revealed to exert some health - enhancing properties like protection against cardiovascular disease and inhibition of cancer . so far , most studies about resveratrol have focused on animal models ; however , there are some works shown the beneficial effects of this compound on human , however , its strong anti - inflammatory properties have not been investigated in professional athletes . thus , we have hypothesized that the extracts of polygonum cuspidatum containing resveratrol can reduce inflammation levels in male professional basketball players . the aim of this study was to investigate the effects of polygonum cuspidatum extract ( pce ) containing resveratrol on inflammation in male professional basketball players . twenty healthy professional basketball players ( aged 17 - 35 years ) were randomized into two groups ( 10 each ) . for 6 weeks , they received either 200 mg of pce standardized to contain 20% trans - resveratrol equivalent to 40 mg trans - resveratrol ( pure encapsulations inc . the subjects were instructed not to take any anti - oxidant supplements , and anti - inflammatory drugs during , and 2 weeks before the study . venous blood samples were collected after and 2 h of intensive endurance exercise between 6 and 7 pm at the baseline and after 6 weeks of treatment with resveratrol . ethical approval from the medical ethics committee of tehran university of medical sciences was obtained and participants signed informed consent . the serum levels of tumor necrosis factor- ( tnf- ) , and interleukin 6 ( il-6 ) were measured by enzyme immunometric assay kits using the kits of assay designs ( ann arbor , mi ) . nutritionist 4 ( first data bank , san bruno , ca , usa ) was used to perform nutrient calculations for 3d dietary records that obtained before and after the intervention . the statistical tests were conducted using spss ( version 16 ; spss , inc . , twenty - four athletes were recruited but 20 of them completed the intervention for 6 weeks . incidence of diseases and personal reasons were the main reasons of their withdrawal of the study . mean ( sd ) plasma levels of tnf- and il-6 are seen in table 2 . baseline characteristics and some nutrients intake throughout the study tumor necrosis factor- and interleukin-6 levels of participants during the study as table 1 shows , there were no significant differences between the groups with regard to weight , body mass index and dietary intake . il-6 and tnf- decreased significantly in resveratrol group and there were significant differences between the two groups after intervention [ table 2 ] . the present study , investigated the effect of pce containing resveratrol on plasma level of tnf- , and il-6 in male professional basketball players . in this randomized double - blind placebo - controlled clinical trial study , intake of pce containing resveratrol for 6 weeks was demonstrated to reduce the plasma concentration of tnf- and il-6 significantly ( p < 0.05 ) . , ghanim et al . in rats and healthy humans . in 2008 , bujanda et al . demonstrated that production of tnf- decreased in rats treated with resveratrol . furthermore , this author proposed that anti - tnf- effect of resveratrol could be related to decreasing liver damage in a model of liver steatosis . ghanim et al . in 2010 found that intake of pce containing resveratrol suppressed plasma concentration of tnf- , il-6 , and c - reactive protein after 6 weeks in healthy humans while they did not observe any changes in these indices in the control group . on the other hand , ghanim et al . in 2011 have shown the anti - inflammatory effects of resveratrol and polyphenol preparation supplement in healthy humans . it is now recognized that both acute aerobic and anaerobic exercise can cause production of free radicals that lead to acute oxidative stress , which can result in oxidative damage and induces inflammation . in addition , strenuous exercise can lead to sequential release of tnf- and il-6 in the blood that is comparable to that observed in relation to bacterial diseases . one of the suggestive mechanisms for this effect includes down - regulation of inflammatory response via inhibition of production and release of pro - inflammatory markers by its suppressive effect on nuclear factor-b or the activator protein.[1315 ] to our knowledge , this is the first study to investigate the effects of this supplement in male professional basketball players ; however , there were some limitations . the major limitation is that it has been conducted in a small number of professional athletes because of the limitation in accessing to them . in conclusion , this study indicates that pce containing resveratrol has suppressive effects on some of immune system factors , including tnf- and il-6 plasma levels .
background : exercise can lead to acute oxidative stress , which can result in oxidative damage and induce inflammation . resveratrol may reduce the levels of inflammatory cytokines . thus , we investigated the effects of this compound on the plasma levels of tumor necrosis factor- ( tnf- ) and interleukin 6 ( il-6 ) in male professional basketball players.methods:twenty healthy male professional basketball players were randomized into two groups ( 10 each ) . for 6 weeks , they received daily either 200 mg of polygonum cuspidatum extract ( pce ) standardized to contain 20% trans - resveratrol equivalent to 40 mg trans - resveratrol or placebo . indices of inflammation were measured before and after 6 weeks of supplementation.results:there was a significant reduction in plasma levels of tnf - a and il-6 after 6 weeks of supplementation ; while no change was observed in these markers in the control group.conclusions:present study shows that 6 weeks of pce containing resveratrol supplementation reduces the inflammation in male professional basketball players .
in spite of the advances that have been made , the management of chronic wounds is still a challenge for the clinician . sushruta was quite aware of the importance of wound management and has described shashthi upakramas ( sixty measures ) for vrana ropana ( wound healing ) , of which the application of madhu is one . many research studies have been carried out to examine the value of topical application of honey for wound healing.[25 ] madhu is commonly used as anupana ( i.e. , given along with active medication to enhance activity ) and also , sometimes , for its primary medical property systemically as well as locally , either alone or in combination with other drugs . it has been described to have properties like lekhana ( scraping ) , sandhana ( union ) , shodhana ( purification ) , ropana ( healing ) , and tridoshaghna ( pacifying all three doshas : vata , pitta , and kapha ) . it is used as an external application in vrana ( wound ) , either alone or in combination with sarpi ( goghrita , i.e. , ghee made from cow 's milk ) . honey is hygroscopic in nature , with a ph of 3.24.5.[911 ] it prevents colonization and bacterial growth in tissues due to this acidic nature . most microorganisms do not grow in pure honey because of its low water activity ( aw ) of 0.6.[1213 ] honey also has antibacterial properties[1418 ] the presence of hydrogen peroxide and a high osmotic pressure also contribute to the antibacterial effect of honey . these natural properties of madhu are said to make it suitable for use in wound management . a 70-year - old female patient of vata - kaphaja prakriti presented to us for treatment of a chronic infected wound that involved entire anterior tibial aspect of the right lower limb . she complained of burning pain in the wound as a result of a drug reaction , foul - smelling pus discharge , difficulty in walking , and occasional fever . on examination a large ( 22 4.5 cm ) ugly - looking ulcer was present on the anterior aspect of the right leg , extending from the knee to the ankle joint . dushta vrana on the anterior aspect of the right leg before treatment at the first consultation on 19 - 09 - 2009 . the characteristics of dushta vrana are ativivrita ( broad base ) , bhairav ( ugly look ) , putipuyamansa ( pus discharge ) , gandha ( foul smell ) , vedana ( pain ) , dirghakalanubandhi ( chronicity).[38 every morning the wound was first treated with freshly prepared lukewarm neem bark decoction , which was poured on the wound while it was simultaneously cleaned with sterile swabs . after cleaning , madhu ( dabur india ltd . , india ) was applied in adequate quantity with the help of a spatula and the wound was covered with sterile gauze and loosely bandaged . along with the local wound treatment , the following drugs were given orally in powdered form 12 hourly : yashtimadhu ( glycyrrhiza glabra linn . ) 2 g , shatavari ( asparagus racemosus willd . ) 2 g , gokshura ( tribulus terrestris ) 2 g , and guduchi [ tinispora cardifolia willd ( miers ) ] 2 g. the drugs were administered along with lukewarm water . at the end of the 5 week ( the 35 day ) the wound had healed completely , leaving only a minimal scar [ figure 2 ] . healed scar on the anterior aspect of the right leg after treatment ( on 23 - 10 - 2009 ) . madhu has vranaropak properties as per the principles of the sixty upakramas of vrana management described in the sushruta samhita . vata , pitta , and kapha by multiple actions attributable to its madhura ( sweet ) rasa , kashaya ( astringent ) uparasa , ruksha ( dry ) guna , sheeta ( cold ) virya , madhura vipaka , and sukshma marga anusari ( ability to permeate in microchannels ) prabhava . madhura rasa gives nutrition to the tissue , which helps in granulation tissue formation , while kashaya rasa provides lekhana ( scraping ) that helps in desloughing , preparing the wound for healing . thus , madhu has excellent properties to heal the wound by virtue of its sodhana ( purification ) , ropana ( healing ) , and sandhana ( union ) actions . at the time of presentation , the patient had pain , discharge , discoloration , etc . , with predominating vata dosha . the madhura rasa of the honey reduced the vitiated vata dosha , leading to reduced pain and enhanced healing . madhu has been described as having the ability to promote phagocytosis , detoxification , and proteolyses , all of which assist in cleaning the wound . further , madhu pacifies pitta dosha by virtue of its madhura rasa and sheeta guna . kapha is taken care of with kashaya rasa and ruksha guna , which accelerate healing . daily dressing of the wound with neem bark decoction helped to inhibit the growth of microorganisms.[2730 ] honey is a hyperosmolar medium , preventing bacterial growth . because of its high viscosity it forms a physical barrier , and the presence of the enzyme catalase gives honey antioxidant properties . honey has been shown to be useful in the prevention of hypertrophic scarring and post - burn contractures . honey is a very effective agent for dressing of split - thickness skin graft . in our patient , the drugs that were administered internally in powdered form ( yashtimadhu , shatavari , gokshura , and guduchi ) have antioxidant , immunomodulating , and adaptogenic properties . as the patient was elderly , we gave rasayana drugs to improve rasa , rakta , and mamsa dhatus ( tissues ) . this might have contributed to the wound healing and helped the patient to attain and maintain good health . the mode of treatment was found to be cost - effective , safe , and easy to implement .
application of madhu ( honey ) is one among the shashthi upakrama ( sixty treatment modalities ) described by sushruta . clinical observation has shown its effectiveness in treatment of dushta vrana ( chronic wounds ) . we report a case of dushta vrana on the anterior aspect of the right leg that was treated successfully with local application of madhu and neem ( azadirachata indica ) bark decoction .
the anal canal is a very unusual site of foreign body impaction by ingestion . if an ingested foreign body has passed beyond the cricopharynx it will generally pass through the entire alimentary canal uneventfully . there are few cases reported in the literature of mediastinitis , peritonitis or intra - abdominal abscesses secondary to perforation by ingested foreign body . common sites of impaction and perforation include the appendix , caecum and meckel s diverticulum ( 1 ) . the incidence of bezoars or impacted foreign body is higher in patients with previous operative manipulation of the gastrointestinal tract ( 2 ) . anal stenosis and spastic anal sphincter are known risk factors predisposing to foreign body impaction in the anal canal . careful inspection and digital examination can establish the diagnosis of the abscess and an associated fistula , but will not necessarily demonstrate the presence of the impacted foreign body ( 3 ) . early drainage and adequate exploration of the abscess cavity can help remove the offending cause and provide cure . this report illustrates a rare case of a man with a pig bone fragment impacted in the anal canal presenting with anal pain and perianal fistula . a 36-year - old afro - caribbean man presented to colorectal clinic with 2 weeks history of anal pain and occasional perianal discharge . digital examination showed induration at the same position but with only minimal discomfort . during subsequent examination under anaesthesia a superficial subcutaneous fistula was demonstrated through passage onto the internal opening in a straight line . the fistula was de - roofed by careful diathermy , which also revealed a small abscess cavity adjacent to the external opening . within the fistula , a piece of 3 cm pig bone fragment with sharp ends was discovered ( see figure 1 and 2 ) . the abscess cavity and fistula were curetted and packed to allow healing by secondary intention . on further questioning , the patient recalled eating pork spare ribs in combination with alcohol several days prior to onset of anal pain . impaction of ingested foreign body in the anal canal is an extremely rare cause of perianal abscess or fistula formation ( 4 ) . these bone fragments are often sharp and therefore complications such as obstruction and perforation of the alimentary tract may arise . in our patient a short segment of bone with sharp ends did reach the anal canal and penetrated through the wall to lodge in the perianal tissues . risk factors predisposing to impacted foreign body by ingestion include the presence of dentures , previous anal surgery complicated by anal stenosis and alcohol intoxication ( 5 ) . our patient did not have any relevant risk factors except consumption of alcohol at the time he enjoyed the spare ribs . perianal fistulae often require examination under anaesthesia to determine the anatomical relationship with the sphincters . due to the force exerted by rectum during defecation , a sharp object is more likely to pierce the perianal space below the sphincter complex as it relaxes . digital examination may reveal the presence of an abscess but often fails to detect the presence of any foreign body in the abscess cavity ( 4 ) . occasionally , plain x - ray is diagnostic and helpful in preventing sharp penetrating injury to the physician s examining finger . however , x - rays are only indicated when per - anal insertion of a foreign body is suspected . although magnetic resonance imaging of the rectum and anus is useful in the assessment of complex perianal sepsis ( 6 ) , it is unlikely to be used in a setting such as occurred in this case and its role in the detection of unintentional foreign body is unknown . generally , management includes adequate incision and drainage of abscess cavity in combination with removal of foreign body to allow immediate pain relief and cure . antibiotics are not usually required unless there is evidence of cellulitis , systemic sepsis or in patients with pre - existing immunosuppression ( 7 ) . once treated , emphasis must be placed upon prevention of recurrence , which includes patient education and meticulous mastication . careful examination under anaesthesia and adequate drainage of abscess are the key to successful outcome .
anal pain is a common presentation in colorectal clinic . accurate diagnosis often requires examination under anaesthesia as pain prevents careful assessment . this report intends to highlight a rare cause of a superficial perianal fistula caused by an ingested foreign body.a 36-year - old afro - caribbean gentleman underwent examination under anaesthesia of rectum . he had a 2-week history of perianal pain and discharge . intra - operatively , a piece of bone from a pork spare rib was found embedded within the superficial subcutaneous fistula . there was an associated abscess cavity adjacent to the fistula , which was curetted . the fistula was laid open and the bone was removed . the cavity was successfully left to heal by secondary intention.ingested foreign body is a very unusual cause of perianal sepsis and subsequent fistula formation . incision and drainage of the abscess along with removal of foreign body is the key to immediate pain relief and cure .
psammomatoid ossifying fibroma ( pof ) is a rare , slowly progressive tumor of the extragnathic craniofacial bones , with a tendency toward locally aggressive behavior . the pathognomonic histologic feature is the presence of spherical ossicles , which are similar to psammoma bodies . fibro - osseous lesions composed of pof and aneurysmal bone cyst ( abc ) of the frontal sinus in a 12-year - old boy is reported , followed by a literature review . to our knowledge , this is the second case of a fibro - osseous lesion composed of ossifying fibroma with reactive abc to be reported in the literature . psammomatoid ossifying fibroma ( pof ) , also known as juvenile ossifying fibroma , is a rare tumor of the extragnathic craniofacial bones , particularly the periorbital , frontal , and ethmoid bones . it is a slowly progressive lesion with a tendency toward locally aggressive behavior , including invasion and destruction of surrounding tissue , bony erosion , and recurrence after surgical excision . the pathognomonic histologic feature is the presence of spherical ossicles , which are similar to psammoma bodies . here , we report pof of the frontal sinus in a 12-year - old boy associated with an aneurysmal bone cyst ( abc ) . although abc can occur as a secondary change in association with a number of benign and malignant bone lesions , to our knowledge , this is the second case of a fibro - osseous lesion composed of ossifying fibroma and abc to be reported in the literature . a 12-year - old boy presented with progressive left eye proptosis for the last 3 years . the condition was diagnosed initially by an ear , nose , and throat surgeon as a mucocele of frontal sinus , but after endoscopic biopsy of the lesion , the pathology result was suggestive of a meningioma . computed tomography ( ct ; fig . 1 ) and magnetic resonance imaging ( mri ) , plain and contrast ( fig . 2a , b , ) , of the skull revealed a huge cystic mass involving left frontal sinus with expansion of the lesion into the ethmoid and orbital cavities and extensive enhancement of the lesion . b preoperative contrasted mri t1 axial view preoperative ct scan brain axial views a preoperative ( plain ) mri brain t1 wi axial view . b preoperative contrasted mri t1 axial view the lesion was partly cystic and completely extradural . the anterior wall of the frontal sinus and the supraorbital part was destroyed by the tumor ( fig . 4 ) , and reconstruction of the supraorbital area was performed by acrylic cement that was fixed in place with screws . 4post operative ct scan brain 3d ct scan of skull showing destroyed left supraorbital bone post operative ct scan brain the material sent for histopathological study consisted of multiple grayish - white and grayish - brown tissue fragments and bone measuring 4.5 4 1.5 cm in aggregate . microscopic examination revealed proliferated oval to spindly cells that often formed small whorls centered by calcified osteoid masses ( ossicles ) reminiscent of psammoma bodies ( fig . however , there was no significant nuclear pleomorphism , and mitoses could not be demonstrated . immunohistochemically , the lesional cells showed strong and diffuse reactivity for vimentin ( fig . fig.5microscopic view shows calcified ossicles resemble psammoma bodiesfig.6immunohistochemically , cells showed strong and diffuse reactivity for vimentinfig.7no immunoreactivity for ema microscopic view shows calcified ossicles resemble psammoma bodies immunohistochemically , cells showed strong and diffuse reactivity for vimentin no immunoreactivity for ema the lesion was interspersed by large blood vascular spaces in areas . osteoclastic giant cells and osteoid could be focally demonstrated in the lining of these spaces establishing the presence of an associated abc ( fig . the final diagnosis was psammomatoid juvenile ossifying fibroma associated with an abc . pof was initially described by gogl in 1949 as psammomatoid fibroma of the nose and paranasal sinuses . in 1985 , margo et al . described pof as a distinctive solitary fibro - osseous lesion of young persons that affects the orbit and shows characteristic histologic features . psammous desmo - osteoblastoma by makek , juvenile active ossifying fibroma by johnson et al . , and juvenile ossifying fibroma with psammoma - like ossicles by slootweg et al . . it probably arises from overproduction of the myxofibrous cellular stroma normally involved in the development of the septa in the paranasal sinuses as they enlarge and pneumatize . these stromal cells secrete hyaline material that ossifies and connective tissue mucin that initiates the cystic areas . the myxoid tissue , cystic degeneration , and aneurysmal - bone - cyst - like areas characteristic of some juvenile pof may simply be reactive changes and may not be specific for this particular lesion . the majority of cases have been reported in children and young adults between 5 and 25 years of age with slight male predominance ( 1.2:1 ) . clinical manifestations of the pof include proptosis , visual disturbances , progressive blindness , airway obstruction , headache , and progressive craniofacial deformities . local expansion of the tumor may extend into adjacent structures , such as the paranasal sinuses , nasal cavity , nasopharynx , palate , and cranial cavity . our patient was a 12-year - old boy and the lesion was located in the frontal sinus . clinically , he was presented with left eye proptosis , visual disturbances , ptosis , and disturbances in ocular mobility . radiographic examination reveals an expansile well - circumscribed radiolucent or mixed radiolucent / radiopaque lesion surrounded by a thick bony wall . ct and mri of the skull revealed a huge cystic mass involving left frontal sinus with expansion of the lesion into the ethmoid and orbital cavities . the cystic nature of the present lesion , which was also verified intraoperatively , may be attributed to the presence of an associated abc . the most characteristic histologic feature of pof is the presence of numerous small , round ossicles or the ossicles are mineralized collagenous foci that vary from small , smoothly contoured round - to - oval patterns to larger , irregularly shaped patterns , with concentric layering similar to that of psammoma bodies . the completely extradural location and the partly cystic nature of the present lesion , the young age of the patient , and the ema negativity of the tumor cells favor the diagnosis of pof over meningioma . they have distinctive histomorphologic features and a tendency toward locally aggressive behavior including invasion and destruction of adjacent anatomic structures .
introductionpsammomatoid ossifying fibroma ( pof ) is a rare , slowly progressive tumor of the extragnathic craniofacial bones , with a tendency toward locally aggressive behavior.case reportthe pathognomonic histologic feature is the presence of spherical ossicles , which are similar to psammoma bodies.discussionfibro-osseous lesions composed of pof and aneurysmal bone cyst ( abc ) of the frontal sinus in a 12-year - old boy is reported , followed by a literature review.conclusionto our knowledge , this is the second case of a fibro - osseous lesion composed of ossifying fibroma with reactive abc to be reported in the literature .
treatment involves excision of the accessory urethra usually through a suprapubic open approach . we report a novel use of extra peritoneal pelvic laparoscopic approach to disconnect accessory urethra from normal urethra in complete urethral duplication . a 10-year - old boy presented with stress incontinence , splaying of urinary stream , and dorsal curvature of the penis . he had an intact foreskin , which , when retracted , revealed a dorsally grooved glans with two meati : one at the tip and the other smaller one at the corona . the prepuce itself was reverse aligned with the frenulum being dorsal , as in epispadias . first surgery : cystoscopy , correction of chordee , repair of glans , rearrangement of foreskin and partial excision of the dorsal urethra . on cystoscopy , the ventral urethra , bladder neck and posterior urethra were normal . a tube passed through the dorsal urethra was seen entering the ventral urethra at the posterior urethra just opposite the veru . the dorsal accessory urethra was partially excised in the distal shaft and glans repaired to achieve a conical glans with normal coronal collar . the skin was re - arranged to give a circumcised appearance [ figure 1 ] . post - operatively he passed urine through both the meati but finger occlusion of the dorsal meatus produced one stream through the normal meatus . chordee has been corrected second surgery performed after 1 year : through a suprapubic 10 mm port the prevesical space was developed with finger dissection and co2 insufflation . two 5 mm ports were inserted as shown in figure 2a ( inset ) . in the space of retzius the accessory urethra was identified as it entered the normal urethra at the prostate . the accessory urethra was severed from the normal urethra using bipolar cautery and scissors [ figure 2a ] . the distal stump of the accessory urethra was obliterated by cautery ablation of the mucosa with monopolar electrode passed through the meatus [ figure 2b ] . inset shows port placement schematic diagram showing mucosal excision of the distal portion of the accessory urethra first surgery : cystoscopy , correction of chordee , repair of glans , rearrangement of foreskin and partial excision of the dorsal urethra . on cystoscopy , the ventral urethra , bladder neck and posterior urethra were normal . a tube passed through the dorsal urethra was seen entering the ventral urethra at the posterior urethra just opposite the veru . the dorsal accessory urethra was partially excised in the distal shaft and glans repaired to achieve a conical glans with normal coronal collar . the skin was re - arranged to give a circumcised appearance [ figure 1 ] . post - operatively he passed urine through both the meati but finger occlusion of the dorsal meatus produced one stream through the normal meatus . chordee has been corrected second surgery performed after 1 year : through a suprapubic 10 mm port the prevesical space was developed with finger dissection and co2 insufflation . two 5 mm ports were inserted as shown in figure 2a ( inset ) . in the space of retzius the accessory urethra was identified as it entered the normal urethra at the prostate . the accessory urethra was severed from the normal urethra using bipolar cautery and scissors [ figure 2a ] . the distal stump of the accessory urethra was obliterated by cautery ablation of the mucosa with monopolar electrode passed through the meatus [ figure 2b ] . inset shows port placement schematic diagram showing mucosal excision of the distal portion of the accessory urethra urethral duplications usually occur as an isolated deformity in males , the two urethra lying one behind the other in a sagittal plane . usually the normally placed ventral urethra is more functional and contains the sphincter mechanism and verumontanum . our case had an incomplete epispadic sagittal duplication as per william 's classification . however , as per effmann classification it falls into complete patent duplication type ii ( a2 ) . surgery to excise the accessory urethra has traditionally involved a combined suprapubic and penile approach to deal with the proximal end and the distal end , respectively . the key step is suprapubic disconnection of the accessory urethra from the good ventral urethra through dissection in the retropubic space . this is technically difficult due to limited space , and risk of venous bleeding from a rich prostatic venous plexus , and carries a risk of damage to the normal urethra , sphincter mechanism and the nerves . however , it would have been a major undertaking considering the length of the tract and its course between the corpora cavernosa . it would have been technically difficult to reach under the pubic bone to completely excise the distal portion . the laparoscopic pre - peritoneal approach has been used in adults for excision of benign prostatic enlargement and in partial cystectomy for bladder pheochromocytoma . the reported advantages are minimal bleeding , a reduced transfusion rate , shorter hospitalization , reduced morbidity and faster recovery . . however , the pre - peritoneal laparoscopic approach for disconnection of accessory urethra has not been described in children to the best of our knowledge . insufflations of gas then result in pressure dissection which creates more space to insert working ports . the visualization of accessory and the main urethra is easy as the view is magnified , free from bleeding and well retracted . since this is probably the first case report of such a procedure in urethral duplication , there is no available data in the literature about dealing with the stump . however , there is now sizable experience with laparoscopic pull thru for anorectal malformations with several authors leaving the stump of rectourethral fistula open with no ill effects . transfixation , suture closure and closure by an endo - loop are other options . identification of the good urethra was a key safety step . in case of difficulty
we report an extraperitoneal pelvic laparoscopic approach to disconnect accessory urethra from normal urethra in complete urethral duplication . first stage consisted of chordee correction , partial excision of the accessory urethra and glansplasty . in the second stage the remaining accessory urethra was disconnected from the normal urethra through a pre - peritoneal minimal access approach to the retropubic space . the remaining distal mucosa was ablated using monopolar cautery .
primary spontaneous coronary artery dissection ( scad ) as a cause of acute coronary syndrome or sudden death is a rare entity with complex pathophysiology . it appears mostly in young women without traditional risk factors for coronary artery disease and a significant proportion of them present during the peripartum period . a 33-year - old african - american woman ( para = 2 , gravida = 0 ) was discharged home after an uneventful full - term vaginal delivery of a healthy baby . two weeks postpartum , she presented to a regional hospital with severe chest pain of 24-hours duration radiating to both arms . she did not have a history of hypertension , hypercholesterolemia , or diabetes mellitus or connective tissue disorder . she denied any history of medication that could have caused vasoconstriction or recreational drugs use , and she had had no intense physical activity prior to the onset of chest pain . , she had a pulse rate of 52 beats per min and a blood pressure of 150/85 mmhg . there was no clinical evidence of the marfanoid feature or connective tissue disease , and her cardiovascular examination was essentially normal . her initial electrocardiogram ( ecg ) showed st segment elevation throughout the antero - lateral leads ( figure 1 ) . a bedside echocardiogram revealed severe hypokinesia of the antero - apical walls and lateral walls with left ventricular ejection fraction < 40% . cardiac enzymes were elevated with peak total creatine kinase ( ck ) 500 iu / l ( normal < 145 iu / l ) , peak ck - mb 235 iu / l ( normal < 16 erythrocyte sedimentation rate ( esr ) was 3 and c - reactive protein ( crp ) < 1 . thrombolytic therapy was contraindicated in her case ; therefore , within one hour , the patient was transferred to the cardiac center for urgent coronary angiography and revascularization . the invasive coronary angiography showed mild ectasia with abnormal flow and hang - up of contrast in the proximal part of the left anterior descending artery ( lad ) , which suggested a dissection flap . also , there was a significant caliber reduction with thrombolysis in myocardial infarction ( timi ) grade 2 flow in the distal part , and the rest of the study was unremarkable ( figure 2 ) . an intravascular ultrasound ( ivus ) was preformed to assess the size of the vessel and confirm the diagnosis ( figure 3 ) . thereafter , the patient underwent coronary artery angioplasty to the area of dissection , whereby two bare metal stents ( 4.0 x 16 mm , and 4.0 x 12 mm ) were deployed at 16 atmospheres across the lesion in the proximal and mid lad . post - stent ivus showed good result with no further residual dissection detected and good stent wall opposition and expansion ( figure 4 ) . after revascularization , an intra - aortic balloon pump ( iabp ) was placed for hemodynamic support , and the patient was started on intravenous heparin . in addition , after consultation with her obstetrics / gynecologist , we initiated an eptifibatide infusion in order to reduce the risk of thrombosis and then she was transferred to the coronary care unit . the following day , the patient was asymptomatic , with stable vital signs and improved myocardial ischemia blood works , so the intravenous medication and iabp were discontinued and she was started on oral medications which included aspirin , clopidogrel , ramipril , metoprolol , and simvastatin . spontaneous coronary artery dissection ( scad ) is a rare well recognized cause of acute coronary syndrome . seventy - five percent of cases occur in females with a mean age of 40 years and 33% are in the peripartum period.1 it leads to sudden cardiac death in 50% of cases and a further 20% die within hours of symptom onset . clearly , the mortality rate depends upon the extent and location of the dissection . scad is a result of a hematoma formation within the outer third of the tunica media , with subsequent expansion leading to compression of the true lumen and resultant myocardial ischemia.2 a combination of hemodynamic factors and changes in the integrity of the coronary vessel lead to scad , which is often seen in the absence of atherosclerotic disease . scad is difficult to identify by conventional cardiac catheterization and may be an under recognized phenomenon . dissection may be missed in the absence of an intimal tear or if the true lumen is severely narrowed . the classic angiographic appearance includes contrast media seen in two lumens separated by a radiolucent intimal flap , with persistence of contrast in the false lumen after washout from the remainder of the vessel . consequently , the diagnosis of scad increasingly relies on ivus , which can identify the presence and extent of medial hematoma . the rarity and poor survival of scad limits data on the best available treatment options . medical therapy of scad is appropriate for patients with resolution of symptoms and limited disease . nitrates , beta blockers , and calcium channel blockers are thought to reduce coronary spasm and extent dissection.3 aspirin , clopidogrel , and low - molecular weight heparin may limit hematoma formation and subsequent luminal compression.4 the role of glycoprotein iib / iiia inhibitors is unclear.5 thrombolytic therapy may lyse the compressing hematoma and allow the native vessel to re - open . however , there have been several deaths following thrombolytic therapy in middle - aged females presenting with acute coronary syndrome ( acs ) in the absence of atherosclerotic risk factors.6 clearly , lysis might allow expansion of the intramural hematoma and further myocardial ischemia from the native lumen compression . surgical revascularization is best reserved for the left main , multi - vessel disease , or refractory ischemia despite aggressive medial therapy.7 in the case of a well - localized symptomatic single coronary dissection not involving the left main , percutaneous coronary intervention with stenting is possible.8 patients who have experienced peripartum dissection should be counseled against future pregnancies as the risk of dissection increases with multiparity and increasing age.9 patients should be followed clinically for symptoms of recurrent ischemia . stress testing with nuclear perfusion imaging is reasonable for surveillance , especially in patients with dissections of large vessels with large areas of myocardium at risk . we conclude that the diagnosis of scad should be considered in the differential diagnosis of chest pain , especially in younger patients , peripartum women , and patients with underlying connective tissue disease . if the diagnosis of scad is suspected , then patients should be referred for primary percutaneous coronary intervention , where the diagnosis of coronary dissection can be made by coronary angiography and an ivus may help in stent sizing and implant .
spontaneous coronary artery dissection ( scad ) is a rare condition that can result in unstable angina , acute myocardial infarction , and sudden death . this condition may occur particularly in women during late pregnancy and in the postpartum period . we present the case of a 33-year - old african american woman , who had spontaneous left anterior descending coronary artery ( lad ) dissection two weeks postpartum , resulting in acute st - segment elevation myocardial infarction with severe left ventricular dysfunction . the use of the intravascular ultrasound ( ivus ) in our case confirmed the diagnosis and helped with stent sizing and implantation . on subsequent follow - up , there was marked left ventricular function recovery and clinical improvement .
enteropathy - type t - cell lymphoma ( etl ) is primary extranodal t - cell lymphoma arising in the intestine originating from intraepitheial t - cells.1 etl is very rare and accounts for fewer than 5% of all gastrointestinal tract lymphomas . five - year survival rate is 20% to 25%.2 - 6 the treatment options are chemotherapy with or without radiotherapy and surgery . currently , there are no definite treatment guidelines for etl . diagnosis of etl was very difficult and usually made by surgery because of complications of etl at presenting time , such as intestinal perforation or obstruction , or lack of endoscopic experiences . there were few articles about endoscopic findings of etl with only white light and capsule endoscopy.7 - 12 relatively long segmental wall thickening of jejunum with mild dilatation , fluid collection and suspicious mesenteric fat infiltration was noted on abdominal computed tomography . increased fluorodeoxyglucose ( fdg ) uptake ( suv , 6.0 ) was also noted on 18-fdg positron emission tomography ( fig . 1 ) . to evaluate for obscure gastrointestinal bleeding and small intestinal mass , double - balloon enteroscopy ( en-450t5 ; fujinon , saitama , japan ) was performed via oral route and showed multiple small shallow ulcerations , edematous villi and fusion of villi ( fig . 2 ) . push enteroscopy with conventional colonoscopy via oral route ( cf - h260al ; olympus , tokyo , japan ) showed mosaic and fissured like villi and more prominent nodularity mucosal pattern with narrow band imaging ( nbi ) and indigocarmin spray ( fig . immunohistochemical staining showed cd3 ( + ) , cd8 ( + ) , cd56 ( + ) , tla-1 ( + ) , cd4 ( - ) , cd5 ( - ) , cd20 ( - ) , and cd30 ( - ) ( fig . the patient was treated with rituximab , cyclophosphamide , adriamycin , vincristine , and prednisone ( r - chop ) chemotherapy and radiotherapy . six months after the initial diagnosis , the patient underwent an operation because of intestinal obstruction with perforation and died from sepsis . mean age of diagnosed etl was 60 years old and sex ratio of etl was about 1:1 ( male : female ) . but , less frequently , etl can arise anywhere in the small intestine , stomach and colon . about 40% of etl patients have acute clinical manifestations such as acute peritonitis due to perforation and intestinal obstruction which needs emergent operation . other clinical manifestations are abdominal pain , weight loss and protein losing enteropathy . in early disease , etl is localized in gastrointestinal tract or mesenteric lymph node . as progression of disease , it can spread to liver , spleen , skin , or other organs.5,14 prognosis of etl is very poor . five - year survival rate is 20% to 25%.2 - 6 few endoscopic findings of etl were reported by enteroscopy and capsule endoscopy.7 - 12 typical endoscopic findings of etl are multiple shallow ulcerations and diffuse thickening of mucosa with innumerable coarse or fine granular elevations ( mosaic mucosal pattern ) . no articles have been published about nbi of etl . in our case of etl , more prominent mucosal nodularity and scalloing was observed with nbi and indigocarmin spray . the specimen of etl endoscopic mucosal biopsy shows that mucosal edema and villous distortion by tumor cell infiltration ( fig . the surgery for etl has poor outcomes and its role is limited to debulking and/or treatment of etl complications such as perforation and obstruction . chemotherapy such as vincristine , doxorubicin , methotrexate , and prednisolone ( vamp ) ; vincristine , doxorubicin , and prednisolone ( vap ) ; chop ; procarbazine , etoposide , prednisolone , and doxorubicin ( pepa ) ; cyclophosphamide , etoposide , vincristine , and prednisone ( ceop ) ; prednisolone , cytarabine , lomustine , etoposide , and thioguanine - bleomycin , vincrisitine and methotrexate ( peace - bom ) ; and surgery are options for treatment of etl.5 but , more than half were unable to complete their planned chemotherapy courses and early treatment failure is frequently occurred because of malnourished state and complications of disease or chemotherapy recently , some clinical trials for feasibility and activity of high - dose chemotherapy supported by autologous stem cells transplantation ( asct ) as therapeutic option for etl in small series15 - 17 and retrospective studies.18 - 20 in these clinical trials , asct could increase overall survival rate , even up to 60% . there are two reports of chemotherapy with asct about prevention of etl21 and reduction in aberrant t - cells in patients with refractory celiac disease.22 alemtuzumab , humanized anti - cd52 monoclonal antibody currently used in treatment of chronic lymphocytic leukemia or t - cell lymphoma , can be another option for etl.23,24 alemtuzumab can treat etl successfully with chemotherapy . there is the report about increased risk for etl with using this monoclonal antibody for treatment of refractory celiac disease.25 so , more clinical studies are needed for alemtuzumab . in conclusion , etl is a very rare intestinal tumor . the endoscopic characteristics are edematous vili , fusion of vili , and multiple shallow ulcerations . in chromoendoscopy , indigocarmin and nbi , prominent mucosal noduarity , fissured or mosaic patterns are the characteristics of etl . but , recently , molecular target agent such as alemtuzumab and asct can be the treatment options for etl instead of conventional chemotherapy with poor outcomes .
enteropathy - type t - cell lymphoma ( etl ) or enteropathy - associated t - cell lymphoma is a very rare malignant intestinal tumor . etl is usually diagnosed by surgery . endoscopic findings of etl are not well known , and there are few reports of findings from endoscopy that has been performed only using white light . additionally , there are no definite treatment guidelines for etl . therefore , we report a case of etl diagnosed by enteroscopy with imaging - enhanced endoscopy and also review recently developed treatment options .
about 50% of patients with a diagnosis of non - small cell lung carcinoma exhibit metastatic disease at the time of diagnosis . the preferential sites of extrapulmonary spread are the lymph nodes , liver , brain , adrenal glands , and bones . a 51-year - old smoker was diagnosed with a poorly differentiated non - small cell lung carcinoma of the right lower lobe in stage ct2a he received front - line therapy with cisplatin - pemetrexed . a ct scan after 4 cycles revealed a partial response in the lung and a complete response in the brain . after 2 cycles , locoregional progression was observed , with the extrapulmonary disease still controlled . after 2 cycles , the patient complained of abdominal pain and reported melena , yet without signs of intestinal obstruction . biological data revealed severe aregenerative anaemia ( haemoglobin concentration at 6.9 g / dl , reticulocyte count at 120,000/mm ) and iron deficiency signs consisting of low serum ferritin , iron , and transferrin saturation . the stool examination for occult blood was strongly positive , while gastric fiberscopy and colonoscopy results were normal . abdominal ct with contrast medium enhancement showed an intussusception of the small bowel ( fig 1 ) . we then decided to explore the small bowel by means of capsule endoscopy , which revealed several secondary lesions , including one stenosing the first jejunal loop and another one ulcerated and bleeding ( fig 2 ; online suppl . videos 1 and 2 ; for all online suppl . a double - balloon enteroscopy was performed thereafter for lesion biopsy , confirming the metastatic localisation of a poorly differentiated lung carcinoma . on account of the patient 's poor performance status , surgical treatment was not considered , with only symptomatic treatment performed using several blood transfusions and iron supplementation . at last , the patient died 20 months following the cancer diagnosis and 5 months after discovering the intestinal metastasis . improvement in metastatic lung cancer management has led to extending survival , and we are thus faced with an increasing number of uncommon metastatic sites . gastrointestinal metastases are considered to be rare , usually occurring in advanced disease stages , and associated with poor prognosis . due to their mostly asymptomatic presentation , were observed in 12% of patients , with the small bowel as the most common site of metastatic involvement . gastric and duodenal metastases likely cause abdominal pain , haematemesis , as well as chronic bleeding resulting in anaemia . small bowel involvement often leads to acute abdomen , and may be associated with various clinical complications such as perforation , obstruction , or haemorrhage . clinical cases of gastrointestinal metastases from lung cancer have been reported , with diagnosis systematically delayed . as illustrated in our case report , although conventional imaging revealed jejunal intussusception , the correct diagnosis of metastatic intestinal involvement was only brought upon by capsule endoscopy . this non - invasive procedure thus represents a valuable method contributing to a rapid and detailed diagnosis while reducing underdiagnosis . this strategy allows for obtaining a therapeutic effect in patients with solitary gastrointestinal metastases , and a favourable palliative effect on survival in those with preoperatively diagnosed metastases . we reported a case of small bowel metastases from primary lung cancer revealed by abdominal pain and severe recurrent anaemia . capsule endoscopy allowed for an aetiologic diagnosis to be obtained , and should thus be considered in lung cancer patients complaining of abdominal symptoms , which may indeed be related to gastrointestinal metastases . written informed consent was obtained from the patient for publication of this case report and any accompanying images .
gastrointestinal metastases from lung cancer are rare and usually asymptomatic . we report a case of small bowel metastases from primary lung cancer revealed by abdominal pain and severe recurrent anaemia . the diagnosis was obtained with capsule endoscopy . this non - invasive procedure thus represents a valuable method contributing to a rapid and detailed diagnosis while reducing underdiagnosis , and it should thus be considered for lung cancer patients complaining of abdominal symptoms , which may indeed be related to gastrointestinal metastases .
at 35 weeks of gestation , a 37-year - old ( height 162 cm and weight 85.1 kg ) primigravida visited the emergency department with complaints of unceasing palpitations , without associated symptoms such as dizziness , syncope or dyspnea . upon questioning , she described a history of occasional palpitations over the past few years , which went away spontaneously in minutes . ekg showed the presence of a svt at a rate over 160 beats / min with stable blood pressure ( fig . three boluses of iv adenosine ( 6 mg 12 mg 12 mg ) were injected after unsuccessful carotid sinus massage . the svt reverted to a normal sinus rhythm for only several minutes with adenosine , and svt recurred . iv verapamil ( 5 mg ) and two trials of synchronized electric cardioversion ( 150 j 200 j ) were also unsuccessful . emergency cesarean delivery was then performed because of uncertainty about the maternal health and concern for the fetus . a spinal anesthesia was performed using 10 mg of bupivacaine and 20 g of fentanyl after 500 ml of 6% he s 130/0.4 infusion . phenylephrine ( 0.2 g / min / kg ) was given to prevent spinal anesthesia related hypotension and 50 g of phenylephrine was occasionally administered when systolic blood pressure was lower than 100 mmhg . total infused dose of phenylephrine before delivery of a baby was 350 g with bolus and 60 g with continuous infusion . a healthy baby was delivered uneventfully , and 100 g of carbetocin was infused slowly without any complications . her heart rate was around 150 beats / min throughout the operation , which suddenly dropped to 80 - 90 beats / min at the end of surgery . 2 ) . a 24-hour holter monitor and a cardiac echography were performed the next day , but no abnormalities were found . she was discharged after one week of postnatal care in the hospital without any episodes of palpitations . it has been reported that newly onset of paroxysmal svt was rare but symptoms of paroxysmal svt were shown to be exacerbated in some patients during pregnancy . hemodynamic , hormonal , autonomic , and emotional changes during pregnancy can be the precipitating factors for arrhythmia [ 3 - 5 ] . depending on the gestational age , maternal and fetal condition , emergency delivery should always be an option . in our case , thus , considering her gestational age and the possibility of heart failure , emergency delivery was the best course of action for the mother and the baby . anesthetic management of parturients with paroxysmal svt for cesarean delivery is not a straightforward decision . anesthetic management should focus on avoiding factors known to produce cardiac ectopy , such as increased sympathetic tone , electrolyte imbalances and acid - base disturbance . volatile agents may reduce the incidence of perioperative tachyarrhythmias because they increased refractoriness within the accessory and atrioventricular pathways . but , it is almost impossible to avoid sympathetic stimulation during intubation in general anesthesia for cesarean delivery . however , we decided to do spinal anesthesia because we had more experience in spinal anesthesia than epidural anesthesia and hypotension during spinal anesthesia can be managed safely with appropriate treatment . there are a few points that should be considered for spinal anesthesia during cesarean delivery in this case . the reduced atrial filling associated with spinal anesthesia has been identified as a risk factor for arrhythmia . fluid pre - loading could prevent a reduction in atrial filling and also reduce sympathomimetic requirements , which may trigger svt . sympathomimetics , which are frequently used to treat hypotension during spinal anesthesia , may also initiate tachycardia . there was a previous case of svt , which was induced by ephedrine during cesarean delivery . the combined use of ephedrine and phenylephrine in treating hypotension after spinal anesthesia for cesarean delivery abolishes the increase in heart rate and effectively maintains the blood pressure . we used phenylephrine alone throughout the surgery and weaned the patient off this drug after svt terminated . phenylephrine increases the vagal tone by indirectly stimulating baroreceptor reflexes , which ultimately reduces svt occurrence , and may have advantages over ephedrine . in general , drug therapy strategy used in parturients is similar to that in non - pregnant patients . however , the effects of antiarrhythmics on the fetus and uterine blood flow and contractility must be considered . for this reason , non - pharmacological treatment such as vagal maneuvers , valsalva maneuver and facial ice immersion should be tried first to terminate tachycardia . when the vagal maneuver fails , intravenous adenosine is the first - choice drug because of its rapid onset , high efficacy and short half - life . the adverse side effect of these drugs is hypotension , which is more common with the use of diltiazem than verapamil . these changes may not be physiologically important to the fetus , but may be consequential in patients with cardiovascular dysfunction . synchronized electrical cardioversion may become necessary for svt that are resistant to pharmacological therapy , like our case . direct current electrical shock has been used in all stages in pregnancy without significant complications . the amount of current that reaches the fetus is thought to be negligible . however , fetal heart rate monitoring during cardioversion is advisable . non - pharmacological treatments , antiarrythmics , and even electrical cardioversion were unsuccessful in our case and emergency cesarean delivery was performed . every treatment provided to the mother should be considered carefully , weighing the risk of their use against the risk of continuing svt . if cesarean delivery is performed , conditions that could exacerbate the symptoms must be avoided .
paroxysmal supraventricular tachycardia ( svt ) is a common arrhythmia in the parturient and can occur with or without an underlying organic heart disease . a woman of 35 weeks ' gestation , who had a paroxysmal svt that was resistant to antiarrhythmic drugs and electric cardioversion , required emergency cesarean delivery . the cesarean delivery was performed under spinal anesthesia and a healthy baby was delivered uneventfully . svt spontaneously converted to normal sinus rhythm right after delivery of the baby .
spontaneous central vein thrombosis is a rare but potentially serious condition as it can present with a fatal pulmonary embolism . activated protein c resistance ( apc - r ) due to homozygous factor v leiden mutation is an exceptional cause of spontaneous central venous thrombosis . we describe here a right - sided internal jugular vein ( ijv ) and subclavian vein ( scv ) thrombosis in a patient due to apc - r and homozygous factor v leiden mutation . the relationship with dengue viral infection and apc - r with central vein thrombus has been explored . a 21-year - old non - obese female patient with no co - morbid illness was referred to our intensive care unit ( icu ) from a private hospital with history of high grade fever with chills 8 days back with respiratory distress for 6 days and altered sensorium for 2 days . she was intubated for altered sensorium and had already received mechanical ventilation for 48 hours . she was referred to us in a state of shock with vasopressors infusions on flow ( dopamine through peripheral line ) . additionally , she had edematous peripheries ( right upper limb swelling was more as compared to the left ) . she had leukocytopenia ( 2,400/cmm ) , thrombocytopenia ( 20,000/cmm ) , and normal prothrombin time , activated partial thromboplastin , and thrombin times and fibrinogen levels . neck ultrasonography ( usg ) prior to an attempt of right ijv cannulation revealed a non - compressible right ijv along with absence of venous flow in color doppler strongly suggestive of right ijv thrombus . her left ijv and scv were compressible ( checked by using linear ultrasound probe supraclavicularly ) and patent , hence we inserted the central line in the left scv for hemodynamic monitoring , fluid resuscitation and vasopressor use . echocardiography showed mild tricuspid regurgitation with ejection fraction < 55% with mild dilatation of main pulmonary arteries . urgent thoracic computed tomography ( ct ) angiography revealed left distal pulmonary artery thrombosis [ figure 1 ] . we immediately started unfractionated heparin ( loading dose : 80 u / kg followed by continuous infusion @ 20 u / kg / hr . ) to achieve a target activated partial thromboplastin time ( aptt ) of 2 - 2.5 times the normal . further investigations demonstrated apc - r using the test kit sta -staclot apc - r ( diagnostica stago , asnieres , france ) and genetic markers for thrombosis using polymerase chain reaction showed homozygous to factor v leiden mutation . after achieving the target aptt , oral vitamin k anticoagulants ( ovka ) in the form of warfarin 5 mg was started along with the heparin to aim international normalized ratio ( inr ) of 2 - 2.5 . her other supportive management included fluids , sedation , vasopressors , broad spectrum antibiotics , enteral nutrition , and mechanical ventilation . in the meantime , dengue igm enzyme - linked immunosorbent assay ( elisa ) ( dengue igm capture elisa , panbio , australia ) was also found to be positive . within the next week after the shock recovered , we did thrombophilia testing of this patient [ table 1 ] . but , her right ijv and scv thrombus persisted with partial recanalization in follow up usg [ figure 2 ] . importantly , her father , mother , and younger sister underwent factor 5 leiden mutation test which was tested negative . computer tomography angiography scan of thorax coronal view showing pulmonary artery thrombus ( arrowed ) usg scan of right ijv ( 1 ) showing presence of partial thickness thrombus ( 2 ) and right carotid artery ( 3 ) ( follow up pictures ) thrombosis screening in our patient apc - r is an autosomal dominant abnormality leading to thrombophilia due to point mutation ( position 1691 ) in the factor v gene . with this mutation , factor v becomes unresponsive to proteolytic action of activated protein c. thus protein c resistance occurs and resulting in uninhibited coagulation and intravascular thrombosis . the clinical picture is of spontaneous intravascular thrombosis at an early age ( > 45 years ) in an unusual venous site or pulmonary embolism or recurrence . hence after radiological confirmation of right ijv thrombus and thoracic ct angiography , thrombophilia testing was initiated . age of the patient and unusual venous site thrombus with distal pulmonary artery thrombus prompted us to send thrombophilia testing for secondary prevention .. additionally , we did testing for homocysteine levels which were very low in our patient . epidemiological studies have proved that increased homocysteine level is a risk for venous thrombosis , but the recent evidence has also challenged this concept . antibodies against dengue antigen nonstructural protein 1 ( ns1 ) can cross - react with endothelial cells , leading to endothelial activation and expression of more cytokine , chemokines , and adhesion molecules , resulting in widespread endothelial cell damage . dengue virus infected endothelial cells can inhibit prostacyclin release and intensify von willebrand factor release from endothelial cells . similar intravascular thrombosis is also known with sepsis of any cause . in our case , we believe that central venous thrombosis was due to dengue infection mediated endothelial injury or dengue virus sepsis syndrome induced . venous thromboembolism in patients with factor v leiden mutation related heritable thrombophilias is managed with anticoagulants . ovka , like warfarin , are preferred with a target inr of 2.5 ( range 2.0 - 3.0 ) . the duration of anticoagulant therapy ( 3 - 6 months ) is based on the risk factors , no of events , recurrence , and area of involvement . after a first episode , like in this case , the british committee recommends anticoagulation for 6 months . this case report elaborates about a rare incidence of spontaneous central vein thrombosis in a patient of acp - r . the relation between dengue infections and dengue viral sepsis with spontaneous thrombosis in a predisposed patient was also explored . this case report also emphasizes the significance of usg as fortuitous detection of central vein thrombosis during usg for venous access .
spontaneous central vein thrombosis is a rare and potentially fatal condition in critical care setting . activated protein c resistance due to homozygous factor v leiden mutation is an exceptional cause of central venous thrombosis . we recently treated a healthy female student who presented with acute febrile illness , septic shock , and encephalopathy . neck ultrasonography ( usg ) prior to an attempt of right internal jugular vein ( ijv ) cannulation revealed non compressibility of the vein along with absence of venous blood flow . right ijv and subclavian vein thrombus was confirmed subsequently in usg doppler by radiologist . radiological evidence of distal pulmonary artery embolism in pulmonary angiography was also evident . further investigations demonstrated homozygous factor v leiden mutation and activated factor c resistance and dengue igm positivity in our patient . intravenous heparin followed by oral vitamin k anticoagulants ( ovka ) aided in her recovery . spontaneous intravascular thrombosis with activated protein c resistance and the relationship of acute dengue infection were explored in our report .
oral myiasis was however , first described by laurence ( 1909 ) and is reported mainly in the tropics and is associated with , inadequate public and personal hygiene . the term myiasis refers to infestation of living tissues of animals or humans by diptera larvae . the species of flies that cause myiasis are cochiliomyia hominivorax , known as the screw worm fly , dermatobia hominis or human botfly , sacrophagidae species , alouttamyia baeri and anastrepha species family . myiasis can be classified clinically as primary ( larvae feed on the living tissue ) and secondary ( larvae feed on dead tissue ) . depending on the condition of the involved tissue it is classified into accidental myiasis ( larvae ingested along with food ) , semi - specific myiasis ( larvae laid on necrotic tissue in wounds ) and obligatory myiasis ( larvae affecting undamaged skin ) . further classification can be based on the site as cutaneous , external orifice , internal organs and generalized . the most common anatomic sites for myiasis are the nose , eye , lung , ear , anus , vagina and more rarely , the mouth . incidence of oral myiasis as compared to that of cutaneous myiasis is less as the oral tissues are not permanently exposed to the external environment . reported cases of oral myiasis are most commonly seen in patients with special health care needs like mental and physical challenging conditions , neurodegenerative disorders , cerebral palsy , learning difficulties , and in bedridden and medically compromised patients . the reason for the high occurrence in such patients relates to the many common predisposing factors of myiasis in these patients like poor oral hygiene , incompetent lips , halitosis , seizure disorders and trauma to oral tissues with wounds exposed to environment and hyper - salivation . the species is widely distributed throughout south - east asia , china , the indian subcontinent , tropical africa , and papua new guinea . human myiasis due to c. bezziana is very rare and first was reported in hong kong in july 2003 . the unusualness of c. bezziana infestation is that it can cause extensive tissue invasion of intact skin or mucosal surfaces and leads to burrowing and extensive damage to soft tissues and bone . the affected locations in oral myiasis reported are upper lip , anterior palate , gingiva , buccal mucosa , extraction sites and the lower jaw . traditionally , treatment consists of manual removal of maggots after local application of asphyxiating agents like oil of turpentine , ether , ethyl or mercuric chloride , chloroform , calomel , iodoform , olive oil , phenol , gentian violet and hydrogen peroxide . systemic therapy of ivermectin , a macrolide semi - synthetic antibiotic isolated from streptomyces avermitilis has improved the management by decreasing the course of infection , faster recovery and complete resolution of lesions . it acts by blocking the nerve impulses on nerve endings through the release of gamma amino butyric acid ( gaba ) leading to palsy and death . most recently , nitrofurazone topical application with flushing of the wounds in gingiva has shown promising results without any surgical intervention . this is due to the prion rods found in flies ( hypoderma bovis and oestrus ovis ) and being implicated in at least five human diseases , including sporadic creutzfeldt - jakob disease ( cjd ) which is responsible for 85% of all cases of chronic wasting diseases . thus myiasis is considered as a risk factor for prion diseases in humans . as oral myiasis is not a common occurrence , many clinicians and diagnosticians are unaware of its clinical presentation and progression leading to delay in treatment and morbidity in some cases . prevention of oral myiasis includes good community sanitation and maintenance of good individual and environmental hygiene . wounds should not be left open and oral hygiene care of medically compromised patients and those with special health care needs should be monitored by an attendant / guardian or parent . to conclude , oral myiasis is an uncommon infestation in the general urban population but is seen more often among rural and urban patients with special health care needs and requires immediate attention to prevent debilitation and disfigurement of oral tissues .
myiasis is a rare disease caused by invasion of tissue by larvae of certain dipteran flies . it is more common in countries with tropical climate . oral myiasis is not a very common condition and many clinicians are unaware of its diagnosis . common predisposing factors are poor oral hygiene , halitosis , trauma , senility , learning disabilities , physically and mentally challenged conditions . oral myiasis can lead to rapid tissue destruction and disfigurement and requires immediate treatment . treatment consists of manual removal of maggots from oral cavity after application of chemical agents . use of antibiotics reduces the duration of infection and hastens the recovery period . good sanitation , personal and environmental hygiene and cleanliness and special care for debilitated persons are the best methods to prevent oral myiasis .
erosive adenomatosis of the nipple ( ean ) was first defined by jones in 1955 who referred to it as florid papillomatosis or papillary adenoma of the nipple . in 1959 legal , et al . coined the term erosive adenomatosis because of the clinical and pathologic findings . adenoma of the nipple , sub areolar papillomatosis , papillary adenoma of the nipple , and florid sub areolar duct papillomatosis are some of the other terms to describe ean . ean , a benign neoplasm , is common after the fifth decade but has been reported in children . ean is often suspected as paget 's disease clinically and misinterpreted as syringadenoma papilliferum , hidradenoma papilliferum , or low - grade adenocarcinoma histologically , but it has received almost no notice by the dermatologists . a 23-year - old unmarried woman presented with history of oozing of serous and very rarely sanguineous discharge from right nipple for more than a year . there was no definite history of exacerbation in relation with menstrual cycles , which were normal . there was no family history of similar illness or carcinoma of breast . on examination , the skin over the nipple was rough and thickened with erosion and superficial fissuring [ figure 1 ] . she was diagnosed with ean and treated with antibiotics and short - term mild topical steroids with remission but was not sustained . when she saw discharge of frank blood , the alarmed patient sought opinion from various specialists and was advised mastectomy by some of them . a biopsy of the nipple was performed , which showed features of ean [ figures 2 and 3 ] . thick , rough skin of nipple with erosion and fissuring low power - perpendicular tubular structures showing connection with the overlying eroded epidermis ; dermis showing round , oval , few large or irregular shaped ducts lined by columnar epithelium [ h & e , 40 ] high power of a duct showing apocrine snouts [ h & e , 100 ] ean , a benign tumor of the nipple , is neoplasm of lactiferous duct . the pathogenesis is not very clear and there are two schools of thought regarding the origin of this proliferation of ducts . one concept being that the origin is considered to be a precursor of breast adenocarcinoma and the other , a marker for fibrocystic breast changes . a careful review and the available data indicate that the evidence for an association between ean and breast carcinoma is not convincing . two patterns have been observed : an adenomatous pattern consisting of a proliferation of round , oval or irregular - shaped ducts within a stroma , which may be normal , fibrotic or hyalinized . the second papillomatous pattern shows ducts that are larger , oval or round with increased cellularity . these 2 patterns seem to represent a spectrum , with most cases having some degree of overlap . differentiating the histological pattern from carcinoma is very important . in our case , the absence of cytological atypia , the 2 cell types lining the ducts , and intraductal papillomatosis differentiates it from carcinoma . the organized pattern of the ducts and absence of prominent inflammatory reaction rules out syringocystadenoma papilliferum . the connection of tubular structure with the overlying eroded epidermis rules out possibility of hidradenoma papilliferum . partial or complete nipple excision is the recommended treatment of choice , which is not without chance of recurrence . it was postulated that cryosurgery , as used in the treatment of other benign conditions , could eradicate the tumor through local destruction . deeper necrosis , as required for treatment of a malignancy , would not be needed . although a single treatment may suffice , re - treatment can be safely carried out . being an unmarried woman , absence of other clinical findings in the breasts and with a normal ultrasonogram , the authors hope and wish that the second school of thought , that ean be a reaction pattern to fibrocystic breast changes , as suggested by perzin , et al . , is the cause for the origin in this patient . cryosurgery is planned for the patient and she will be followed up regularly . the patient and the family are counseled enough and adequately re - assured of the benign nature of the disease .
erosive adenomatosis of the nipple ( ean ) is a rare , benign neoplasm of breast lactiferous ducts . peak incidence is in the fifth decade in women . clinically , it is mistaken for eczema during early stages and paget 's disease at later stages . we present a young lady with ean who was advised mastectomy . it is important for dermatologists to be familiar with this entity to avoid topical steroid abuse and unwarranted breast removal . this case is presented for awareness and importance of correct and early diagnosis .
convulsions and loss of consciousness in patients can be caused by arrhythmias , conduction disorders or epilepsy . when syncope is misinterpreted as epilepsy , this may not only lead to the unnecessary use of anticonvulsant drugs but , more importantly , life - threatening arrhythmias can be missed which may lead to sudden unexpected death . the aim of this case report is to present a patient with previously unknown noncompaction cardiomyopathy ( nccm ) in whom syncope induced by ventricular tachycardia was misinterpreted as epilepsy , in order to underline the necessity for cardiological assessment in patients with assumed mild epilepsy or syncope of unknown origin . a 57-year - old man without any history of cardiovascular disease was initially referred to the neurologist for analysis of collapse . an electroencephalogram ( eeg ) revealed an evident paroxysmal disturbance of function in the right frontotemporal lobe . he was diagnosed with mild focal epilepsy and was treated with levetiracetam without any result . a second eeg showed no eeg abnormalities ; however , a simultaneous electrocardiogram ( ecg ) showed a sudden onset of a wide complex tachycardia when the patient collapsed ( fig . 1a fragment of a 24-h eeg which shows a sudden onset of a wide complex tachycardia on a simultaneously recorded ecg , while there is no electrical disturbance in the eeg leads ( only one of the eeg is shown , o1-av ) a fragment of a 24-h eeg which shows a sudden onset of a wide complex tachycardia on a simultaneously recorded ecg , while there is no electrical disturbance in the eeg leads ( only one of the eeg is shown , o1-av ) one year before admission the patient started to suffer from dizziness and collapse several times a day . he did not complain of palpitations or chest pain . in the last 2 months ordinary physical activity resulted in fatigue and dyspnoea ( nyha class ii ) . besides smoking 10 ecg showed a sinus rhythm with normal conduction times and no st - t deviations . transthoracic echocardiography demonstrated an impaired systolic left ventricular function with an ejection fraction of 45 % . magnetic resonance imaging ( mri ) of the heart confirmed the diagnosis of nccm ( fig . 2 ) . telemetry monitoring showed monomorphic non - sustained ventricular tachycardias ( nsvt ) with a right bundle branch block configuration , a superior axis in the frontal plane and a frequency of 150170 beats / min ( fig . 3 ) . during these vts the patient noticed dizziness without loss of consciousness . 2mri of the heart confirmed the diagnosis of noncompaction cardiomyopathy , excessive trabeculation ( arrow ) lv = left ventricle , rv = right ventriclefig . the vt originated from the infero - apical left ventricle mri of the heart confirmed the diagnosis of noncompaction cardiomyopathy , excessive trabeculation ( arrow ) lv = left ventricle , rv = right ventricle telemetric rhythm observation : non - sustained vt , monomorphic , 170 complexes/ min . the vt originated from the infero - apical left ventricle the patient was treated with a beta - blocker and an ace - inhibitor which reduced the number and duration of non - sustained vts and the patient became asymptomatic . considering the nccm with poor systolic lv function and symptomatic vts without a treatable arrhythmogenic substrate , there was a class i ( evidence level a ) indication for icd implantation according to the guidelines for the management of patients with ventricular arrhythmia and the prevention of sudden cardiac death ( scd ) . transient loss of consciousness ( t - loc ) is defined as an abrupt , self - limiting complete loss of consciousness . cardiac syncope is caused by reduced global cerebral perfusion due to a sudden decrease in cardiac output . arrhythmia and structural heart disease such as aortic stenosis and cardiomyopathy are the most important cardiac causes of syncope . recognising cardiac syncope is of utmost importance because of all patients with t - loc , the ones with cardiac syncope have the highest likelihood of subsequent sudden death . kapoor et al . showed in 1983 that in the 12 months following presentation with cardiac syncope , sudden death was found to be six times more common than in patients with noncardiac syncope . the patient of this case report suffered from syncope induced by a ventricular tachycardia as a consequence of noncompaction cardiomyopathy ( nccm ) . even with the help of modern imaging modalities , such as contrast echocardiography and mri , diagnosing nccm can be difficult . the clinical spectrum of nccm shows a highly variable clinical presentation , ranging from asymptomatic to severely symptomatic with end - stage heart failure , lethal arrhythmias , and/or thromboembolic events . there is a high prevalence of , mostly asymptomatic , cardiac disease ( 26 % ) among first- and second - degree family members of patients with nccm . this warrants genetic counselling , dna diagnostics , and cardiological family screening and offers an opportunity for early intervention [ 12 , 13 ] . . showed in a relatively large group of nccm patients that icd therapy for primary or secondary prevention of scd was appropriate in both groups supporting the application of icd in these patients . however , although icd seems to be an effective option for preventing scd , data on the long - term follow - up of nccm patients remain scarce . a substantial number of the patients with t - loc have cardiac syncope and these patients have a substantial risk of subsequent sudden death . sudden death may be preventable in these patients , which underlines the necessity for a cardiological assessment in patients with syncope .
convulsions and loss of consciousness can be caused by , among other things , arrhythmias , conduction disorders or epilepsy . in clinical practice it can be difficult to distinguish between these causes of syncope , even for well - trained specialists . patients with cardiac syncope have a substantial risk of subsequent sudden death . we present a patient with previously unknown noncompaction cardiomyopathy in whom syncope induced by ventricular tachycardia was misinterpreted as epilepsy . we present this case report in order to underline the necessity for cardiological assessment in patients with assumed mild epilepsy or syncope of unknown origin .
anogenital involvement is most common in lsa in both the sexes.blister formation in the lesion of lsa occurs due to basal cell degeneration resulting in dermal - epidermal separation and marked edema in upper dermis . blister formation in the lesion of lsa occurs due to basal cell degeneration resulting in dermal - epidermal separation and marked edema in upper dermis . lichen sclerosus et atrophicus ( lsa ) is a rare inflammatory disease of unknown etiology . it mainly affects post - menopausal women in the genital area . in 15 - 20% of cases extragenital involvement is associated with classic genital lsa and only in 2.5% cases it is found exclusively at an extragenital site . we describe a rare case of isolated nongenital bullous lsa in a male . a 38 year old male patient , engineer by an occupation , presented with minimally pruritic white colored lesions over anterior aspect of neck since 3 months . there was associated mild stinging sensation and minimal watery discharge from the pre - existing lesions since last two weeks . closer examination revealed few lesions having a superficial wrinkled appearance which on palpation appeared to be due to the presence of atrophic epidermis overlying a collection of fluid which was confirmed after puncturing the lesion with needle [ figure 2 ] . at certain areas atrophic papules were coalescing to form plaques and few papules hematological investigations including the complete blood count , liver , renal and thyroid profiles were within normal limits . biopsy findings from a representative lesion revealed presence of an atrophic epidermis with hydropic degeneration of basal cell layer forming a subepidermal split with pronounced dermal edema and homogenization of collagen in upper dermis and dense lymphocytic periappendageal infiltrate in the mid dermis [ figure 3 ] . considering bullous nature of lesions a short course of oral and then topical superpotent steroids ( clobetasol dipropionate 0.05% cream ) with tacrolimus ointment ( 0.1% ) was administered . there was clearance of lesions without any recurrence till 6 months follow up . lesions healed with post - inflammatory hypopigmentation and scarring [ figure 4 ] . multiple atrophic papules superficial wrinkled appearance of atrophic epidermis overlying a collection of fluid which was confirmed after puncturing the lesion h and e , 10-atrophic epidermis with hydropic degeneration of basal cell layer forming a subepidermal split with pronounced dermal edema and homogenization of collagen in upper dermis and dense lymphocytic periappendageal infiltrate in the mid dermis healing of lesions with post - inflammatory scarring and hypopigmentation 6 months post - treatment hallopeau is credited with the first clinical description of lsa in 1887 and darrier first reported histopathological changes in 1892 . however , the maximum incidence occurs between the 5 and 6 decade of life , perimenopausal group , and another peak in girls between the ages 8 and 13 years , prepubertal group . in both the sexes extragenital involvement is associated with classic genital lsa in 15 - 20% of cases and only 2.5% cases show exclusive involvement of extragenital site as in our case . the underlying cause is unknown , but there seems to be genetic susceptibility and a link with autoimmune mechanism . extragenital lsa is most common on upper trunk involving neck and shoulders , axilla , upper arms , flexor aspects of wrists , around the umbilicus and on forehead and very rarely on scalp , palms , soles and in the oral cavity . subclinical trauma caused by friction with the collar could be responsible for the lesions on the shoulder , possibly attributable to the isomorphic phenomenon of koebner . the lesions are small , ivory or porcelain - white , shiny , round macules or papules , coalescing to form sclerotic plaques . individual lesions are usually slightly raised , or level with the surface of the skin and their surface shows prominent dilated pilosebaceous or sweat duct orifices , which often contain yellow or brown horny plugs . occasionally hemorrhagic transformation or blister formation occurs due to basal cell degeneration resulting in dermal - epidermal separation and marked edema in upper dermis . bullous lsa has been described in a localized and generalized distribution , and often the vesicles and bullae have a hemorrhagic component . telangiectases and purpura may occur . in the later stages , atrophy occurs , and the surface of the lesions becomes wrinkled , and may actually be depressed . bullous lesions may sometimes heal with milia formation . in a similar case described by hallel , widespread hemorraghic bullae were described in a patient with extragenital lsa , our case in contrast shows presence of localized involvement and non - hemorrhagic lesions . similar localized bullous lesions of lsa over back have been described by ballester irene et al . and lesions resolved completely with topical potent steroid cream . histologically well - developed lesion of lsa is characterized by marked orthohyperkeratosis , keratotic plugging of the hair follicles and dermal appendages , atrophy of stratum malpighii with flattening and loss of rete ridges . there is broad zone of pronounced edema and homogenization of the collagen . within this zone collagen fibers the blood and lymphatic vessels are dilated , and there may be areas of hemorrhage . edema in the upper part of the dermis may be enough to result in clinically apparent vesiculation . the lower dermis is characterized by the presence of dense lymphocytic infiltrate in the mid dermis which drifts from its sub epidermal position to mid dermis as the lesion develops on account of the edema and homogenization of collagen in the upper dermis . as opposed to case of genital lesions , no increased risk of malignant transformation has been found in extragenital lsa . there is no definitive treatment for lsa , therapy for extragenital lsa is warranted only for extensive lesions , bullous and haemorrhagic lesions , symptomatic and cosmetically disfiguring lesions . numerous therapies have been used including topical and systemic corticosteroids , topical estrogen and testosterone containing ointments , retinoids , tacrolimus and psoralen ultraviolet a therapy ( puva ) therapy . marked improvement in generalized bullous and haemorrhagic lsa has been reported with adrenocorticotropic hormone in one patient . the bullous lesions are usually transient and heal before typical plaques of the disease , which are more resistant to treatment . it is important to bear in mind this form of presentation of lsa in order to facilitate clinical diagnosis and prompt treatment . appearance of bullous lesions of lsa over an extragenital area in the absence of lesions elsewhere on the body is a rare presentation .
lichen sclerosus et atrophicus ( lsa ) is a rare , chronic , mucocutaneous disease of unknown cause . onset can occur in subjects of any age but more prevalent in adult females around the time of menopause . in both the sexes anogenital involvement is more common . extragenital cases are rare , and common localizations are neck and shoulders , axilla , upper arms , flexor aspects of wrists and around the umbilicus . bullous lsa is an unusual manifestation of the disease . isolated extragenital bullous lsa is a distinctly rare event with very few cases reported till date .
ashy dermatosis , also known as erythema dyschromicum perstans , is an idiopathic dermal melanosis of unknown etiology , but probably with an immunologic basis . it is characterized by asymptomatic blue - grey pigment patches with or without erythematous borders most commonly located on the face , neck , trunk and upper limbs , but can affect any region of the body . ashy dermatosis typically occurs in the second decade of life and generally affects subjects with type iv skin ; in fact , it has been described mainly in patients from tropical areas of central and south america . a 63-year - old caucasian male with fototype iii was admitted to our department of dermatology because of irregular , oval or round dark - brown macules with central resolution symmetrically appeared in both armpits . the skin around the armpit was red by initial exposure to the sun ( figure 1 ) . moreover classic gray - blue macules were present on the lumbar region with a size of 0.62 cm ( figure 2 ) . dark - brown macules with central resolution in both armpits . figure 2typical gray - blue lesions of the ashy dermatosis on the lumbar region . concomitant diseases were type 2 diabetes , dyslipidemia , hypertension , coronary ischemic disease with two angioplasties , supra - ventricular extrasystoles , obstructive arteriopathy with intermittent claudication . the patient was in treatment with bisoprolol fumarate ( 1,25 mg / day ) , acetylsalicylic acid ( 100 mg / day ) , ticlopidine ( 250 mg / day ) , fenofibrate ( 145 mg / day ) , glibenclamide + metformin ( 5 mg + 500 mg / day ) , dipeptidylpeptidase inhibitor ( 50 mg2/day ) , irbesartan ( 150 mg / day ) , and pantoprazole(20 mg / day ) . laboratory examinations , except glycemia , glycosylated hemoglobin , cholesterol and triglycerides , were within normal values . a skin biopsy of a lesion from right armpit was performed and histology was suggestive for ashy dermatosis ( figure 3 ) . melanophages are intermingled with lymphocytes in middle - deep dermis ( hematoxylin and eosin 200 ) . melanophages are intermingled with lymphocytes in middle - deep dermis ( hematoxylin and eosin 200 ) . the basic treatment for internal disorders had not been changed . at a control , three months later , all lesions had cleared up ( figures 4 and 5 ) . figure 5almost complete clearing up of the macules on the lumbar region three months later . the patient recovered without discontinuation of exposure to the sun , also the following summer after the sun exposure has not been resubmitted to the skin disease . ashy dermatosis is a relatively rare skin disease , included in the group of acquired idiopathic hypermelanosis . the etiology of the disease is not known though it has been associated with the ingestion of ammonium nitrate , exposure to environmental contaminants , pollutants , worm infestation , endocrinopathy ( hypothyroidism , diabetes mellitus ) , atopy , dyslipidemia . a number of immunopathological studies of active lesions have shown that ashy dermatosis may involve immune mediation . it has been postulated that damage to melanocytes and basal layer keratinocytes results from an abnormal immune response to antigens . the drugs taken by our patient for the underlying conditions have never been suspended or changed until healed , so can not be responsible for the ashy dermatosis . the active lesions show vacuolar degeneration of the basal cells , and pigmentary incontinence with many melanophages in the upper dermis . dermal blood vessels are surrounded by an infiltrate of lymphocytes and histiocytes . in the residual macules , the incontinence of pigment predominates , whereas the cellular infiltrate and vacuolar degeneration of the basal cell layer may range from minimal to intense . differential diagnosis includes lichen planus pigmentosus , the pigmented macules of the late pinta , post - inflammatory pigmentation , figurate erythemas , pityriasis rosea , multiple fixed drug eruption , hemochromatosis , addison s disease , melasma , leprosy , idiopathic eruptive macular pigmentation , macular amiloidosis , confluent and reticulated papillomatosis of gougerot and carteaud . the most frequent cause of confusion and controversy is with lichen planus pigmentosus , which is characterized by hyperpigmented dark - brown macules with a non - characteristic distribution over predominates in exposed areas and flexural folds . its course is characterized by exacerbations and remissions , occasionally accompanied by pruritus . in both lichen planus and ashy dermatosis max - joseph spaces , although not observed in all cases of lichen planus , are absent in ashy dermatosis . in ashy dermatosis a perivascular infiltrate is often present , in contrast with the typically band - like infiltrate in lichen planus . the immunopathology of ashy dermatosis and lichen planus are similar including populations of helper / inducer ( cd4 + ) , cytotoxic ( cd8 + ) t cells and epidermal keratinocytes expressing hla - dr+ . opinions vary about whether ashy dermatosis is an abortive form of lichen planus or a distinct entity . the our case showed axillary lesions with typical clinical appearance of lichen planus pigmentosum but with ashy dermatosis suggestive histological examination , because it lacks the typical band - like infiltrate and the max joseph - spaces . the diagnosis is further confirmed by the coexistence of lesions in the lumbar region with a typical clinical appearance of ashy dermatosis . this case is of interest because it prove the existence of ashy dermatosis with clinical aspect evoking lichen planus pigmentosus . this is the first case in the literature of lichen planus pigmentosus - like ashy dermatosis confirming the view that ashy dermatosis is a variant of lichen planus without the typically band - like infiltrate and max joseph spaces .
ashy dermatosis , also known as erythema dyschromicum perstans , is an idiopathic dermal melanosis of unknown etiology . we here describe an unusual case of 63-year - old caucasian male with ashy dermatosis and skin lesion of lichen pigmentosus - like . no treatment was tried because the lesions were totally asymptomatic . after a control , three months later , all lesions had cleared up . this case is of interest because it proves the existence of ashy dermatosis with clinical aspect lichen planus pigmentosus - like . this is the first case in the literature of lichen planus pigmentosus - like ashy dermatosis confirming the view that ashy dermatosis is a variant of lichen planus without the typically band - like infiltrate and max joseph spaces .
although lobular type breast carcinoma is less common compared with ductal type carcinoma , it has a higher propensity to metastasize to the git . with improved longevity of breast cancer patients owing to gallstone ileus , on the other hand , accounts for < 1% of all cases of mechanical bowel obstruction and mainly affects the elderly population ( > 70% ) . we report a case of small bowel obstruction caused by an obstructing gallstone in small bowel strictured by metastatic breast cancer . a 76-year - old caucasian woman was admitted with a 2-week history of intermittent colicky lower abdominal pain associated with bilious vomiting and mild abdominal distension . she was previously diagnosed with left breast cancer 18 years ago for which she had undergone an oncological wide local excision , focused external beam radiotherapy and adjuvant treatment with tamoxifen for 5 years . unfortunately , she developed regional recurrence with bony metastases 17 years following her original diagnosis . the abdomen was mildly distended with tenderness and guarding noted in the peri - umbilical region . laboratory investigations , including haematological , biochemical , inflammatory and coagulation profiles , were all within normal limits . she proceeded to have computerized tomography ( ct ) scan of her abdomen and pelvis , which showed dilated small bowel loops consistent with small bowel obstruction . there were two filling defects noted , one in her proximal ileum suspicious for a non - calcified gallstone and the second distally in her terminal ileum ( figs 1 and 2 ) . appearances were suggestive of gallstone ileus . she was adequately resuscitated and underwent a laparotomy . the small bowel was narrowed in two segments : proximally secondary to an impacted gallstone and an area of stricturing noted distally . both segments were resected with end - to - end anastomosis performed in a two - layer standard hand - sewn fashion . the clinical impression was one of dual pathology including crohn 's disease and gallstone ileus . she made an uneventful post - operative recovery and was discharged home well on day 7 . figure 1:axial ct image demonstrating gallstone in proximal ileum ( block arrow ) and distal ileal luminal narrowing ( thin arrow ) . figure 2:coronal ct image demonstrating gallstone ileus ( block arrow ) and stricture in distal ileum ( thin arrow ) . axial ct image demonstrating gallstone in proximal ileum ( block arrow ) and distal ileal luminal narrowing ( thin arrow ) . coronal ct image demonstrating gallstone ileus ( block arrow ) and stricture in distal ileum ( thin arrow ) . histopathological examination of the resected specimens showed a 3-cm intraluminal gallstone within the proximal ileum , with focal metastatic carcinoma consistent with breast primary present in the adjacent mesenteric adipose tissue ( fig . 3 ) , while the more distal , strictured ileal segment revealed mucosal and transmural involvement by metastatic breast carcinoma ( fig . 4 ) . the tumour cells were strongly positive for cytokeratin , oestrogen receptor and focal progesterone receptor ( < 5% ) , consistent with metastatic breast carcinoma . figure 3:haematoxylin and eosin ( h&e ) stain of proximal ileum showing focal metastatic breast carcinoma in mesenteric tissue ( medium power field ) . figure 4:immunohistochemical stain of distal ileal resection showing an extensive transmural involvement by metastatic carcinoma ( 200 ) . haematoxylin and eosin ( h&e ) stain of proximal ileum showing focal metastatic breast carcinoma in mesenteric tissue ( medium power field ) . immunohistochemical stain of distal ileal resection showing an extensive transmural involvement by metastatic carcinoma ( 200 ) . a minority ( 10% ) of patients with breast cancer develops distant metastases to the liver , lungs , bone and brain despite undergoing surgical excision and medical oncologic therapies . metastases to the bowel in general are relatively rare ; however , when present classically originate in the setting of malignant melanoma , colon cancer , ovarian or cervical cancer [ 5 , 6 ] . metastatic small bowel disease can present with a variety of non - specific symptoms , and often masquerades as other commoner gastrointestinal disorders [ 6 , 7 ] . the terminal ileum is the commonest site involved in gastrointestinal spread from breast cancer , followed by the proximal small bowel . clinical findings may mimic crohn 's disease , as reflected in our case . despite invasive ductal breast carcinoma being the more prevalent subtype in women ( 90% ) , invasive lobular carcinoma has a higher propensity to spread to the small bowel , accounting for 54% of all gastrointestinal metastasis in one series . the most frequent sites for impaction include the terminal ileum and ileocaecal valve ( 5075% ) , followed by the proximal ileum and jejunum ( 2040% ) , stomach and duodenum ( 10% ) . diagnosis may be delayed as only 10% of gallstones are adequately calcified to enable radiological detection . while a one - stage procedure consisting of enterolithotomy , cholecystectomy and fistula repair is the mainstay in non - high - risk patients , it carries a high incidence of complications in the severely comorbid . . other surgical options include an enterolithotomy alone , or an enterolithotomy followed by an interval cholecystectomy ( two - stage procedure ) . it is also prudent to note that an entero - biliary fistula can be successfully managed conservatively and spontaneous closure has been reported in more than 50% of cases . our case is different from the reported literature in that small bowel obstruction was triggered by the dual pathologies and synchronous presentations of metastatic breast carcinoma causing a gallstone ileus . to the best of our knowledge , this is the first case report describing metastatic breast carcinoma to the small bowel presenting as a gallstone ileus . metastatic breast cancer involving the small bowel can mimic other more common gastrointestinal pathologies and it is vital to bear this in mind when considering the differential diagnosis in women with a history of breast cancer who present with new onset of gastrointestinal symptoms . written informed consent was obtained from the patient for the publication of this case report and accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal .
metastatic breast cancer to the small bowel ( sb ) presenting as gallstone ileus and resulting in sb obstruction has not been described previously . a 76-year - old woman with previous metastatic breast cancer to the axial spine and hips presented with abdominal pain and bilious vomiting . ct scanning revealed sb obstruction consistent with gallstone ileus . the patient underwent two segmental sb resections for distal ileal strictures mimicking what appeared to be macroscopic crohn 's disease . the entero - biliary fistula was undisturbed . pathological analysis revealed the dual pathologies of gallstone ileus and metastatic carcinoma from a breast primary causing luminal sb obstruction . improvements in staging and treatment modalities have contributed to the increased overall long - term survival for breast cancer , compelling clinicians to consider metastatic breast cancer as a differential diagnosis in women presenting with new onset of gastrointestinal symptoms in order that appropriate treatment be administered in a timely fashion .
our aim was to describe a case of a 25-years - old male diagnosed with anterior megalophthalmos in both eyes and present the usefulness of pentacam hr in such a case . at birth , the lengths of both corneas of the proband were found specifically enlarged . at examination under anesthesia , buphthalmus was excluded with intraocular pressure measurement and optic disc examination . he had a visual acuity of 15/20 and 14/20 with myopic correction at the age of 25 . anterior and posterior keratometry map was done with pentacam hr ( oculus , wetzlar , germany ) in both eyes . anterior keratometry values were 40.4/41.4 d on the right eye , 40.2/41.9 d on the left eye . posterior keratometric data were -5.5/-5.7 d on the right eye , -5.8/-5.3 d on the left eye [ fig the total rms ( root mean square ) was 132.71 m in the right eye and 134.26 m in the left eye , the spherical aberration value was 4.51 m and 5.16 m . image was taken at the age of 25 at slit lamp examination , an optically clear cornea was detected . 24 ] at the age of 1 , 9 , and at the age of 25 , too . corneal pachymetry maps were obtained with pentacam hr using the scheimpflug imaging method , which showed a central pachymetry value of 390 m and a corneal thickness between 450 - 500 m in the periphery [ fig . 5 ] in both eyes . specular microscopy ( em-1000 , tomey ) measured endothelial counts of 2450 cell / mm in both eyes . clear cornea and the significantly deep anterior chamber can be seen scheimpflug picture of anterior megalophthalmos . the extreme depths of the anterior chamber on both eyes are clearly shown the anterior chamber was found deep measured with ultrasound , 7.24 mm in the right eye and 7.47 mm in the left eye . ultrasound biomicroscopy ( ht 35 - 50 ubm system , ophthalmic technologies inc . ) ( which was done at the age of 15 ) showed anterior chamber depth of 7.95 mm and 7.9 mm [ fig . 6 ] . with pentacam hr , 8.04 mm anterior chamber depth was measured in the right eye , and 8.31 mm in the left eye , the volume of the chamber was 721 mm in the right eye and 742 mm in the left eye . the angle of the chamber measured with pentacam hr is an average of 50.7 and 54.9 ( between 34.5 s 58.7 degree in both eyes ) in the right and left eye [ fig . ultrasound biomicroscopy picture in case of anterior megalophthalmos : the similar images taken of both eyes show the really deep anterior hall and central lens opacities significant iridodonesis was observed , but phacodonesis was not detected . central opacity was observed in the lens in both eyes , of which ultrasound biomicroscopy images were taken [ fig . the thicknesses of the lens were 3.61 and 3.43 mm in the right and left eye , measured with ultrasound biomicroscopy . indirect gonioscopy detected wide ciliary band , dense pigmentation was observed on the trabecular meshwork in both eyes [ fig . wide ciliary ring can be observed on both eyes with indirect gonioscopy the axial length was 28.4 mm in the right eye and 29.06 mm in the left , the vitreous index ( vitreous length / axial length x100 ) was 61.8% in the right eye and 62.5% in the left eye . only few readings have been published on anterior megalophthalmos in literature . among the image scanning methods , only ultrasound biomicroscopy examinations the lens of the device is tilted relative to the film plane , so the lens plane , film plane , and the plane of focus are not parallel to one another . anterior segment structures ( lying in the same plane of focus ) from the corneal surfaces to surface of the lens can be sharply focused . the high - resolution scheimpflug system used by us automatically corrects data of the anterior and posterior corneal surfaces for optical and geometric distortion , so all data presented by pentacam hr are valid . this device uses 475 nm monochromatic blue light for imaging , the camera captures 25 , 50 , or 100 scans in 2 seconds with 2760 measuring points . the software allows for automatic analysis of the anterior segment , anterior and posterior topography of the cornea , pachymetry , calculation of the chamber angle , volume , chamber height ( anterior chamber depth ) , and analysis of the lens . scheimpflug analysis demonstrates clearly that the cornea is uniformly thinner than the normal and aberrometric values , such as spherical aberration are high . the depth of anterior chamber and also the angle of the chamber can be observed with pentacam hr . we would like to demonstrate that all anterior segment parameters can be obtained with an easy , noncontact device . in many cases , anterior megalophthalmos involves cataract , as in our case , too . beside central lens opacities , our patient had relatively good and acceptable visual acuity , so no cataract surgery was needed . in these cases , the application of a cataract surgery and precise biometry is rather complicated.[68 ] beside cataract surgery , a few study reported photorefractive keratectomy performed in case of anterior megalophthalmos and penetrating keratoplasty conducted due to a developed bullous keratopathy . in summary , anterior megalophthalmus is a rare disease affecting the whole anterior segment , in whose detailed description scheimpflug imaging can play an important role .
we report an anterior megalophthalmos case with decreased corneal thickness and show the findings using scheimpflug imaging . a 25-year - old male was diagnosed with anterior megalophthalmos . in both eyes , enlarged corneal length was measured . beside a comparatively good visual acuity , a thin but clear cornea , a fairly deep anterior chamber , and central lens opacity were found . scheimpflug images were taken using pentacam hr . scheimpflug - based imaging can provide us new data at the examination of this syndrome affecting the whole anterior segment .
factor vii ( fvii ) is a vitamin k - dependent clotting factor that is part of the extrinsic pathway of blood coagulation . congenital fvii deficiency is a rare bleeding disorder . in patients with congenital fvii deficiency , bleeding manifestations and clinical findings vary widely , ranging from asymptomatic subjects to life threatening bleeding , , . however , severe and life - threatening hemorrhaging is rare in general ( about 5% ) and occurs most frequently during the first six months of life , . treatment has traditionally involved fvii replacement therapy using fresh frozen plasma , prothrombin complex concentrates or plasma fvii concentrates , . intravenous administration of recombinant fviia is now widely used for the treatment of fvii deficiency . as fvii deficiency is a rare disease , experience with surgery in fvii - deficient patients there have been some reports describing the use of recombinant activated fvii ( rfviia ) during surgery in patients with congenital fvii deficiency , , . human prothrombin complex ( ppsb ) contains coagulation factors ii , vii , ix , x , so we thought it could also be used as a substitute for factor vii deficiency patients . in this report , we describe a case of successful replacement treatment with ppsb , without episodes of bleeding and thrombosis . this is the first report of the patient with congenital fvii deficiency undergoing laparoscopic cholecystectomy with repeated administration of ppsb . the patient was a 48-year - old man ( 170 cm , 70 kg ) , he came to our hospital suffering abdominal discomfort . he had no significant past medical history , he had never experienced a significant bleeding episode . we discussed treatment options with the patient , and planned to perform the laparoscopic cholecystectomy . during pre - surgical preparation the prothrombin time ( pt ) was 32.1 s ( reference range , 10.016.0 s ) and the activated partial thromboplastin time ( aptt ) was 26.5 s ( reference range , 27.241.0 s ) . other blood test results , including those for liver and renal function , total protein were all within normal range , and test for hepatitis b and c was negative . as the pt was abnormal , while other tests were basically normal , the consulting hematologist recommended more tests to confirm the diagnosis . fvii activity ( fvii : c ) was 2.7% , with no fvii inhibitor activity , so he was diagnosed with congenital fvii deficiency . we worked out a plan to inject ppsb perioperatively , according to the instruction for ppsb . we first administered ppsb 1800u ( 2025u / kg ) intravenously before the surgery , and checked pt and fvii : c . at that time , pt was 11.8 s , fvii : c was 51.4% , within normal range and hematologist thought that was enough , so we started the operation . laparoscopic cholecystectomy proceeded smoothly and took 20 min with a total blood loss of 30 ml 900 u ppsb was administered intravenously every six hours postoperatively for the first 48 h , cause the half - time of factor vii is 68 h , . pt , aptt , and levels of fibrin degradation product ( fdp ) and d - dimer during the clinical course are summarized ( table 1 ) . the patient was recovering well , and discharged from the hospital two days after surgery without episodes of bleeding and thrombosis . here we described a very rare case of a fvii - deficient patient requiring ppsb for the laparoscopic cholecystectomy . unfortunately , the optimal dose and method of administration for ppsb has not been established for various surgeries due to its rarity . the accumulation of cases of this disease in various fields is needed to determine the most appropriate replacement therapy . none declared . all of the authors stated that they had no interests which might be perceived as posing a conflict or bias . a copy of the written consent is available for review by the editor - in - chief of this journal on request . approval has been given , ethics committee of luwan branch , ruijin hospital , shanghai jiaotong university school of medicine , number 252675 . a copy of the written consent is available for review by the editor - in - chief of this journal on request .
highlightswe describe a case of successful replacement treatment with ppsb , without episodes of bleeding and thrombosis.this is the first report of the patient with congenital fvii deficiency undergoing laparoscopic cholecystectomy with repeated administration of ppsb.ppsb is made in china , it also provides direction for the use of other similar drugs to solve the same problem .
current techniques in acl surgery have been associated with satisfactory long - term results in the majority of patients . however , there remains a considerable subset , up to 30% of patients , with unsatisfactory outcomes [ 2 , 7 ] . specifically , patients report problems relating to rotational instability and return to previous level of activity [ 3 , 10 ] . it has been suggested that a more anatomical approach to restore the original acl anatomy may benefit these patients [ 4 , 17 ] . some authors have advocated placing a single graft in a position closer to the oblique femoral attachment of the acl [ 14 , 15 ] . however , it is not possible to fully restore normal knee kinematics with a single graft , regardless of the position [ 13 , 16 ] . the double bundle reconstruction technique ( dbt ) for acl reconstruction aims at restoring the acl anatomy with its two bundles and is gaining popularity [ 11 , 17 ] . anatomic studies have demonstrated the presence of two functional bundles within the acl , the anteromedial ( am ) and the posterolateral ( pl ) bundle [ 1 , 8 ] . although it is somewhat of a simplification , the double bundle description of the acl is generally accepted as an anatomic model for understanding the complex structure and function of the ligament [ 6 , 9 ] . the am bundle often obscures the pl bundle , and it may appear that only one bundle is present without careful inspection . the goal of this study was to describe the presence of the double bundle structure from an arthroscopic point of view , and to evaluate the value of different portals in knee arthroscopy . we prospectively examined 60 knees during standard arthroscopy . in each knee , the double bundle acl structure was evaluated , along with the usefulnes of different portals for visualization . all knees that were included in the study had a previous x - ray and mri in order to rule out any significant changes to the bone , and to ensure that the acl was intact . all patients were less than 60 years and had no history of acl injury . surgical indications for the 60 total subjects examined included 31 cases treated for meniscal findings alone , 21 treated for articular cartilage findings alone , and 8 cases of a combined repair of meniscus and cartilage . there were 25 female and 35 male knees , 29 right knees , and 31 left knees included in the study . age distribution ranged between 16 and 60 , with an average age of 40.2 years . arthroscopy started with an anterolateral portal ( alp ) located just lateral to the patellar tendon using the inferior pole of the patella as a vertical landmark , and an anteromedial portal ( amp ) approximately 0.5 cm medial to the edge of the patella tendon , 1 cm superior to the joint line , and 1 cm inferior to the tip of the patella . for each knee , the acl anatomy and the visibility of the pl bundle through the alp and amp were evaluated with and without retraction of the am bundle according to the description of the two bundles by girgis et al . and arnoczky . gross biomechanics of the two bundles using a probe were also assessed . for statistical analysis with the arthroscope in the alp , we were able to distinguish the am and pl bundle in 17 cases ( 28.3% ) ( fig . 1 ) . in the remaining cases ( n = 43 , 71.7% ) , the pl bundle was obscured by the am bundle , and visualization was only possible with retraction of the am bundle with a probe ( fig . 2 ) . switching the arthroscope to the amp , differentiation of the am and pl bundle without using a probe to retract am was possible in 40 cases ( 66.7% ) ( fig . 3 ) . in the remaining cases ( n = 20 , 33.3% ) , visualization of the pl bundle was possible only after retraction of the am bundle ( table 1 ) . there was a statistically significant better visibility of the pl bundle using the amp ( p < 0.05).fig . 2anterolateral portal : pl bundle with retraction of am bundle , pl bundle loose with knee flexionfig . bundlepl bundleanterolateral portaln = 17 ( 28.3%)anteromedial portaln = 40 ( 66.7% ) * * p < 0.05 anterolateral portal : pl bundle without retraction of am bundle anterolateral portal : pl bundle with retraction of am bundle , pl bundle loose with knee flexion anteromedial portal : pl bundle without retraction of am bundle visualization of the pl bundle the femoral insertion site of the acl was semilunar at the inner surface of the lateral condyle . the centre of the pl bundle visualized more shallow than the centre of the am bundle with the knee held in 90 flexion , while both insertion sites were oriented horizontally ( fig . 4 ) . the pl bundle insertion was located at the posterolateral aspect of the tibial attachment with a close approximation of the pl bundle to the posterior root of the lateral meniscus . gross assessment using a probe while applying a flexion - extension motion pattern to the knee demonstrated a taut am bundle throughout the range of motion and a tightening of the pl bundle with knee - extension ( figs . 2 , 5 ) . pl bundle tight with knee extension anteromedial portal : pl bundle without retraction of am bundle anteromedial portal : pl bundle tight with knee extension the principal finding of the present study was that it is possible to distinguish the double bundle structure in acl anatomy as described by girgis et al . and arnoczky by applying a two portal knee arthroscopy technique . the visualization of the acl anatomy seems to be improved through the use of a medial portal . the anteromedial portal not only helps in visualization of the double bundle structure but also in distinguishing the am and pl portions of the femoral attachment sites . consistent with the literature , we found the pl bundle tightening when the knee is extended [ 6 , 12 ] . this study is limited by the fact that we observed only acl intact knees and did not consider cases with a torn acl , where the anatomic position of the attachment sites is sometimes obscured by the knee injury and bony changes that occur between injury and reconstruction . therefore , we stress the need for further arthroscopic evaluation of different portals in acl reconstruction . the clinical relevance of this study is that it can be beneficial to establish an additional medial working portal . the amp can be used for the arthroscope and a better visualization of the femoral insertion site of the acl at the inner surface of the lateral condyle and a precise femoral tunnel placement can be achieved . reported the use of an additional medial portal without any increase in morbidity to patients for acl surgery .
in order to describe the arthroscopic presence of the double bundle structure and to evaluate the value of different portals in knee arthroscopy , we assessed the am and pl bundle anatomy . we prospectively examined the knees of 60 patients undergoing arthroscopic surgery for pathology unrelated to the acl . arthroscopy was performed in a two portal technique using an anterolateral ( alp ) and an anteromedial ( amp ) portal . with the arthroscope in the alp , we could distinguish an am and pl bundle in 28% . switching the arthroscope to the amp , differentiation of the bundles was possible in 67% . in all remaining cases visualization of the pl bundle was possible after retraction of the am bundle . use of amp increased visualization of the pl bundle . it seems reasonable to perform arthroscopy for acl reconstruction with the arthroscope in the amp and to establish an additional medial working portal to increase the visualization of the femoral acl insertion sites for optimal femoral tunnel placement .
ventriculus terminalis ( vt ) is the name given to a congenital disorder that consists in the appearance of a small oval ependymal cystic cavity in the conus medullaris . it is formed during embryogenesis as a result of the differentiation between the canalization and regression of the spinal cord . normally , such a cavity is identifiable only histologically in children and adults and can be visualized using common neuroradiological techniques only after dilation . we describe a case of cystic dilatation of ventriculus terminalis ( cdvt ) in adult . a 66-year - old female was admitted to our hospital with hypoesthesia and dysesthesia of the lower limb , distal paresis , and disturbance while walking evolving for the past 2 years . the patient 's medical history has surgical intervention for cyst of conus medullaris 15 years ago , but the details are unknown . a magnetic resonance imaging ( mri ) image revealed the presence of a cystic lesion located in the conus medullaris , with a well - defined outline , and which was not enhanced following contrast intravenous [ figure 1 ] . postoperatively , the patient experienced marked improvement in her lower extremity strength including lower limb control and gait recovery . follow - up mri scans 3 years later revealed complete regression of the dilation without evidence of relapse . magnetic resonance images . a preoperative t2-weighted image ( a and b ) shows the cystic dilatation of the conus medullaris , and postoperative t2-weighted images ( c ) obtained 3 years after surgical treatment shows regression of the dilatation without evidence of relapse ( a ) intraoperative image of the window of thin cyst wall with a suture ( 8.0 ) . ( b ) photomicrographs of histological samples obtained from wall of cyst , showing an ependymal - lined wall without any evidence of neoplastic proliferation ( h and e , 400 ) the vt is an ependymal - lined cavity in the conus medullaris which is usually in continuity with the central canal of the spinal cord . the vt is formed during fetal development . in the normal development of the spinal cord , different stages are recognized as follows : neurulation , canalization , and retrogressive differentiation [ figure 3 ] . during neurulation , which begins at 3 weeks of embryonic development , there is a flexion and closure of the neural plate to form the neural tube . the craniocephalic closure occurs within 23 days of gestation , ending at the anterior neuropore . the caudad closure is complete after approximately 26 days , ending at the posterior neuropore . at about 45 weeks of gestation , the caudal end of the neural tube and the notochord combine to become an aggregate of undifferentiated cells , called the caudal cell mass . small vacuoles develop within this mass , canalize , and form an ependymal - lined tube that usually fuses with most rostral central canals . this cavity is the vt or fifth ventricle . finally , during retrogressive differentiation , a major portion of the distal cord involutes to become a glioependymal strand , the filum terminale . present during fetal development , this space typically regresses after birth , although it has been detected in mri scans in 2.6% of children under the age of 5 years imaged for unrelated symptoms . the cdvt represents a failure of regression of the vt ; possible etiologies include failed fusion between the cranial and caudal neural tubes secondary to congenital disorders , trauma , and ischemia . an association between cdvt in children and the full spectrum of spinal dysraphism and tethered cords has been identified , but the exact causation has not been identified . because descriptions of cdvt in adults have been limited to case reports and small series , true associations are harder to identify . only 45 cases have been reported to date , and 35 patients suffered from specific symptoms and were surgically treated . treatment for patients without symptoms or with stable nonspecific low - back pain seems to be the best conducted conservatively whereas patients with focal neurological deficits seem to be best handled surgically . while some authors performed percutaneous aspiration of cdvt using real - time mri and navigation with good early results , fenestration of the cyst and excision of a window with / without cystic - subarachnoid drainage is a safe and effective surgical technique to prevent its recurrence generally . small vacuoles ( v green ) form in the caudal cell mass ( brown ) . the small vacuoles fuse to form a dilated ependymal - lined tube , which usually merges with the more rostral central canal . it may present an abnormal dilation and we need to know of its existence . treatment for asymptomatic patients seems to be the best conducted conservatively whereas patients with focal neurological deficits seem to be best handled surgically , including fenestration of the cyst and excision of a window with / without cystic - subarachnoid drainage .
the ventriculus terminalis ( vt ) is a virtual cavity of the conus medullaris that appears during embryonic life . we presented a case with the cystic dilation of the ventriculus terminalis ( cdvt ) in a symptomatic patient . a 66-year - old female suffered from disturbance while walking evolving for the past 2 years . an mr image revealed a cystic dilatation of ventriculus terminalis . the patient experienced marked improvement of lower extremity strength by a fenestration of cyst and cyst - subarachnoid shunt . treatment for asymptomatic patients seems to be the best conducted conservatively , whereas patients with focal neurological deficits seem to be best handled surgically .
metastasis to the bone is a common complication of breast , lung , prostate , gastrointestinal , haematological , melanoma , and other tumours , , . in many patients with primary breast , prostate and lung primary tumours , bone metastases ( bom ) are signs associated with the presence of disseminated disease . these metastases to the bone will usually result in patients experiencing pain which is often palliated using single fraction radiotherapy ( sfrt ) or multiple fraction radiotherapy ( mfrt ) depending on the factors such as cancer type and presence of complication within the bone , , , . although the distribution of bom have been observed in many skeletal locations , bom that are distal to the elbow and knee joints are considered rare and therefore are infrequently reported . depending on the primary tumour histopathology , the frequency of skeletal bone metastases ranges from 10% to close to 90% and bone lesions are found more commonly within the axial skeletal versus the appendicular skeleton . although any skeletal bone may be involved with metastatic disease , the vertebral body is most commonly associated with metastatic disease ; long bone involvement or metastases distal to the elbow and knee are rare , , . this study sought to assess the incidence of distal bone metastases treated with radiotherapy in a provincial programme . all courses of rt for bone metastases from 2007 to 2011 for patients living in bc were identified in a provincial rt programme . the bc cancer agency ( bcca ) information system ( cais ) was used to identify 8008 patients who received 16,277 palliative radiotherapy courses for bone metastases from 2007 to 2011 . information retrospectively collected on these patients included : patient age , gender , primary tumour histology , fractionation schedules , and location of bone metastases . treated bom were categorized as distal if the bom was located within or distal to the elbow or knee . patients were grouped by primary tumour site as breast , lung , prostate gastrointestinal , haematological , melanoma , and other . multivariable linear regression analysis was used to determine associations between primary tumour type and incidence of distal bone metastases . p values were two - sided , and values less than.05 were considered statistically significant . we used the spss statistical software package , version 19.0 ( chicago , il ) , for data entry and statistical analysis . from 2007 to 2011 , 8008 patients in bc were treated with 16 , 277 palliative radiotherapy courses ; 425 ( 3% ) of the courses prescribed were used to treat bones located within or distal to the elbow or knee . male patients were treated more often than female patients ( 52% ) and sfrt was prescribed 49% of the time ( table 1 ) . breast and lung malignancies were observed most commonly ( 23% ) , followed by prostate ( 19% ) , other ( 15% ) , lymphoma ( 11% ) , gastrointestinal ( 8% ) and melanoma ( 1% ) ( table 1 ) . we demonstrated that melanoma malignancies resulted in the highest frequency ( 5% ) of distal bom ; followed by haematological ( 3% ) , lung ( 2% ) prostate ( 2% ) , other ( 2% ) , gastrointestinal ( 1% ) , and breast ( 1% ) . distal bom where more commonly identified in the lower extremity ( 87% , p<0.001 ) . distal bom were treated with sfrt significantly more often than non - distal bom ( p<0.001 ) ( fig . 1 ) . table 2 presents the multi - variable analysis performed to determine the associations between distal bom and patient characteristics , which demonstrated that patients with melanoma have a significant higher odds of having distal bone metastases ( p=0.004 ) . the regression analysis also showed significant correlation between having distal bone metastases treated at abbotsford centre ( odds ratio 2.37 , 95% confidence interval 1.543.64 , p<0.0001 ) ( table 2 ) . this is the first large scale study to illustrate the incidence of distal bone metastases in patients being treated with palliative radiotherapy ( rt ) . from 2007 to 2011 , 16,277 courses of palliative rt was used to treat bone metastases in 8008 patients ; 425 ( 3% ) of which were rt courses for distal bom . in addition , distal bone metastases were significantly more likely to be treated with sfrt . this population - based study demonstrates that patients with melanoma have the highest proportion of distal bom . this differs from previous reports , which only present that prostate , breast , and lung cancer have the highest absolute number of distal bom , likely because these are the most common cancers with any type of bom , , , , . here we showed that sfrt was used to treat distal bom more often than non - distal bom ( 66% versus 49% , respectively ) , likely because distal bom are located in regions that do not contain organs at risk such as the spinal cord . a weakness of this study is that untreated bom are not captured , and therefore the incidence of distal bom may be under - represented . however , it is unlikely that the proportion of distal bom is markedly different than reported . in addition , the large population - based nature of this study provides adequate power to identify correlations between distal bom and primary tumour type , while being relatively free of selection bias . this population based study identified an incidence of distal bom of 3% among patients treated with palliative rt . mb conceived of the study , participated in the data collection and statistical analysis , and designed and draughted the manuscript . xxx participated in the acquisition of data , analysis and interpretation of data and helped draughted the manuscript .
purposethis study assesses the incidence of distal bone metastases in palliative radiotherapy ( rt ) patients.material and methodsall courses of rt for bone metastases from 20072011 for patient living in british columbia ( bc ) were identified in a provincial rt programme . treated bone metastases ( bom ) were categorized as distal if the bom was located within or distal to the elbow or knee . patients were grouped by primary tumour site as breast , lung , prostate gastrointestinal , haematological , melanoma , and other . the incidence of distal bone metastases and associations with primary tumour types were determined.resultsfrom 2007 to 2011 , 8008 patients were treated with 16,277 courses of rt , of which 425 ( 3% ) were courses of rt for distal bom . the incidence of distal bom in decreasing order by primary tumour type was melanoma ( 5% ) , haematological ( 3% ) , lung ( 2% ) , other ( 2% ) , prostate ( 2% ) , breast ( 1% ) and gastrointestinal ( 1% ) . distal bom where more commonly identified in the lower extremity ( 87% , p<0.001 ) . single fraction rt was used more commonly for distal vs non - distal bom ( 66% vs. 49% ; p<0.001).conclusionthe incidence of distal bom among patients treated with palliative rt was 3% and most commonly identified in patients with melanoma and haematological malignancies .
clinical manifestations of ph are heterogeneous with respect to age clinical presentation , severity , and rate of progression of renal insufficiency . ph1 is caused due to mutations in the alanine - glyoxylate aminotransferase ( agxt ) gene that codes for the enzyme agxt . although ph 1 can present as infantile oxalosis , the most common presentation is recurrent urolithiasis with nephrocalcinosis resulting in end - stage renal disease ( esrd ) by the second decade of life . ph 2 , generally less severe than ph 1 , has a similar age of presentation . 4-hydroxy-2-oxoglutarate aldolase ( hoga1 ) which encodes the enzyme hoga is mutated in ph 3 . being least severe , it usually presents in the first decade of life with the less active stone formation and preserved renal function . genetic analysis is invaluable in deciding the appropriate management , prognostication , prenatal diagnostic testing , and sibling screening . we report a series of seven children , five of them diagnosed with ph 1 and two with ph 2 with the aid of genetic analysis . the mutations seen in our children are different from those previously reported as common both within and outside the subcontinent . among 211 newly diagnosed chronic kidney disease ( ckd ) patients over 3 years , genetic work up for hyperoxalosis was indicated in seven children . of the seven children , the median age of presentation was 4.9 years ; the youngest and the oldest child being 3 months and 13 years , respectively . two children were siblings born to a consanguineous couple , the younger one though asymptomatic was found to have bilateral renal calculi on screening . five children had bilateral nephrolithiasis , renal dysfunction being evident in two of them . the other two children presented in esrd , one among them had infantile oxalosis . the urinary oxalate levels of the three children with preserved renal function were elevated , mean value being 3.2 mmol/1.73 m / day . the blood samples of these children were sent to the mayo clinic , usa and polymerase chain reaction technique was employed for the genetic analysis . five children were homozygous for nontruncating mutations , and the other two had truncating mutations . no two children except for the siblings had the same mutation [ table 1 ] . phenotype and the corresponding genotype three children with normal renal function are on conservative measures such as hyperhydration , pyridoxine ( ph 1 ) , and crystallization inhibitors . those in esrd are on continuous ambulatory peritoneal dialysis , the median duration being 34.6 months . short of mutational analysis , other investigations such as plasma and urine oxalate levels are not confirmatory . we report a series of seven children with clinical manifestations ranging from asymptomatic nephrolithiasis to infantile oxalosis in whom genetic analysis was done . the results of the genetic analysis confirmed ph 1 in five children and ph 2 in two of them . screening of asymptomatic siblings of the index cases aided in early detection and initiation of conservative measures before the occurrence of renal dysfunction . the ph 1 mutations c. 508g > a , c. 33dupc , and c. 731 t > c have been reported as the commonest in european and african countries whereas reports from north india claim c. 302 t > c as the commonest . the ph 2 mutations c. 103delg and c. 403_404 + 2delaagt are the most common ; the latter predominates in asians . however , the different set of mutations detected in our patients substantiates that these previously reported mutational hot spots may be population specific . data from the same registry also revealed that barring p.g170r , renal survival in ph 1 did not depend on the other mir ( p.g41r , p.f152i , p.i244 t ) mutations . hence , children with renal calculi should be thoroughly investigated for ph . as urinary and serum indices are unreliable in children with ckd , genetic analysis should be attempted in every child where ph is being considered clinically . the mutations being population specific , sequencing of the entire gene rather than targeted analysis
primary hyperoxaluria ( ph ) has heterogeneous renal manifestations in infants and children . this often leads to delay in diagnosis . in the past 3 years , genetic samples were sent for seven children with a clinical diagnosis of ph . their medical records were reviewed for clinical presentation and outcomes . of the seven children , three were males . the median age of presentation was 4.9 years with the youngest presenting at 3 months of age . nephrolithiasis , the most common presentation was associated with renal dysfunction in two children . two children with no significant history presented in end - stage renal disease ( esrd ) . the sibling of one of the children in esrd , with a history of consanguinity in parents , was screened for asymptomatic nephrolithiasis . bilateral multiple renal calculi were found in majority of children followed by echogenic kidneys on ultrasound examination . genetic analysis suggested ph type 1 in five children and type 2 in two children . the mutations detected in our cohort were different from the previously reported common mutations . there was no obvious genotype - phenotype correlation noticed . three children in esrd are on maintenance dialysis . nephrolithiasis being a common presentation of ph needs prompt evaluation . mutations are generally population specific , and whole gene sequence analysis is critical in diagnosis .
the surgical options for the treatment of bone loss include bone transport , vascularized fibula graft , and induced membrane . interestingly patient sustained this injury in spite of having intramedullary nail in tibia which was inserted to stabilize previous fracture 9 months prior to trauma . the proximal half of the nail was protruding out of the wound at the time of presentation in emergency department . the bone gap and nonunion at fracture site was managed with ilizarov fixator . at the end of treatment patient got satisfactory functional outcome . ilizarov method is a biologic and comprehensive method for management of bone loss , non union and limb length discrepancy . the treatment of tibia bone loss can be challenging because of associated co - morbidities such as soft tissue problems , infection , deformities , adjacent joint contractures and socio - economic factors . the surgical options for the treatment of bone loss include bone transport 1vascularized fibula graft , and induced membrane . vascularzed fibula graft has certain limitations like highly demanding surgical technique , risk of microvascular anastomosis failure , graft fracture ; graft non - union and donor site morbidity . we are reporting this case report due to its unusual pattern of injury and complexity of treatment . it was a grade iii b compound fracture with large wound over the proximal tibia . interestingly proximal portion of previously done interlocking nail was seen protruding out of the wound ( fig 2a ) . a ) nail protruding out of wound , 2b ) wound closure with fixator , 2c ) ilizarov fixator spanning knee and ankle joint the previous surgery was done 9 months back . then he had sustained a closed fracture of tibia which was managed with closed reduction and interlocking nailing . as the patient was presented in casualty with this complex injury he was directly taken to operation theatre for debridement without performing preoperative radiographs . the bone loss is probably the result of extrusion of intramedullary nail with its proximal bolts out of the wound . then the linear external fixator was replaced by ring fixator spanning knee and ankle joint . as the proximal fragment ( proximal to bone loss ) and distal fragment ( distal to corticotomy ) were very short , frame was extended to include lower femur and foot for better stability ( fig 2c ) . corticotomy was done in distal tibial metaphysis between non - union site and ankle joint . 3b ) & 3c ) radiographs showing distraction at corticotomy site and compression at the site of bone loss with the use of ring fixator trifocal osteosynthesis gradual distraction was started at distal corticotomy site ( 0.25 mm four times a day ) . the distal non - union site was also put under compression ( fig 3b & 3c ) . finally good consolidation was achieved at proximal fracture ; distal fracture and corticotomy site -trifocal osteosynthesis . patient was followed up for 15 months after the fixator removal . at the time of latest the ankle movements were significantly restricted probably due to prolonged ring fixator application and bone transport . the outcome was assessed according to bone results and functional results as described by paley . open fractures with bone loss require special attention as not only the wound coverage but the bridging the gap is required . in cases of compound fractures the the immediate treatment is in the form of airway management , early resuscitation followed by immobilization of extremity and administration of intravenous antibiotics . urgent debridement of wound with skeletal stabilization is recommended . in certain cases repeated debridement of this case was unusual as , in spite of having previous intramedullary nail in tibia , patient sustained compound fracture with bone loss with the half of the nail protruding through the wound . interestingly the previous fracture which was stabilized with the intramedullary nail was also in nonunion . there was extensive soft tissue injury with segmental fracture with bone loss of 10 cm . the bone defect was in proximal metaphysic and diaphysis with no place for corticotomy in upper tibia . the nonunion was in lower tibial diaphysis leaving a short distal segment for corticotomy and bone transport . various options are available to manage the bone loss and nonunion like bone transport , vascularized fibula graft , induced membrane . distraction osteogenesis with aid of ring fixator has been reported to be a useful technique in the treatment of bone loss especially if associated with severe soft tissue injury . in our case paley et al reported 100% union in 22 patients with tibial bone defects treated by ilizarov method . the bony results were 74% excellent , 16% good , 5% fair and 5% poor with functional results of 63% excellent , 32% good and 5% poor . rozbruch et al reported on 38 tibial nonunions , bony results of 53% excellent , 37% good , 5%poor versus functional results of 53% excellent , 37%good , 5% fair , and 5% poor . the principle advantage of this minimally technique is that it provides sufficiently stable fixation without extensive soft tissue dissection that is necessary for open reduction and internal fixation . the corticotomy is usually advised in proximal metaphysic of tibia as it more vascular as compare to distal metaphysis . but in this case bone loss was extending to involve the proximal tibial metaphysis , so the corticotomy was performed in distal metaphysis . wound coverage , control of infection and temporary stabilization are the cornerstone of the treatment . in cases of bone loss and nonunion distraction osteogenesis helps us to address these issues effectively to have good functional outcome at the end of treatment . ilizarov is a biologic and comprehensive technique to address union , manage bone defect , equalize limb lengths , permits eradication of infection and correct any deformity at the same time maintaining static function and permitting weight bearing as tolerated .
introduction : the treatment of tibia bone loss can be challenging . the surgical options for the treatment of bone loss include bone transport , vascularized fibula graft , and induced membrane.case report : we present a case of complex compound fracture of tibia with bone loss . interestingly patient sustained this injury in spite of having intramedullary nail in tibia which was inserted to stabilize previous fracture 9 months prior to trauma . the proximal half of the nail was protruding out of the wound at the time of presentation in emergency department . the nail was removed and stabilized with external fixator after wound closure . the bone gap and nonunion at fracture site was managed with ilizarov fixator . at the end of treatment patient got satisfactory functional outcome.conclusion:ilizarov method is a biologic and comprehensive method for management of bone loss , non union and limb length discrepancy .
pigmented villonodular synovitis ( pvns ) is a proliferative condition of the synovium , which is composed of nodules and/or villi and has an abundant number of hemosiderin - laden macrophages . pigmented villonodular synovitis ( pvns ) is a proliferative condition of the synovium , which composed of nodules and/or villi and has an abundant number of hemosiderin - laden macrophages . arthroscopy revealed a vascular mass with active contact bleeding , with histology confirmed a diagnosis of pvns . the presence of hemarthrosis suggested that acute bleeding of the lesion into the joint may be the cause of such an atypical acute presentation . pvns , although rare , should be considered as one of the differential diagnoses of an acute irritable knee presenting in children . a 10-year - old boy had sudden onset of left knee pain and swelling 1-day before admission . the range of movement of the knee was markedly limited from 30 to 40. knee aspiration yielded 40 ml of blood - stained fluid . radiographs of the knee showed local soft tissue swelling without any bone lesion ( fig . arthroscopic findings showed a 4 cm x 3 cm multi - lobulated vascular mass at the intercondylar notch with active contact bleeding . the mass was so big that it essentially occupied the arthroscopic view of the intercondylar notch ( fig . 2 ) . there was no other similar lesion in any other area of the joint . histological examination of the resected mass confirmed the diagnosis of pvns with areas of necrosis ( fig . , there was no more knee pain and the patient could run and squat as usual . magnetic resonance imaging performed at 3 months post operation showed no residual lesion ( fig . the latest follow - up at 1-year post operation was normal with no sign of local recurrence . ( a ) arthroscopic view showed a multi - lobulated vascular mass at the intercondylar notch , ( b ) resection of the mass . histologic pictures of the lesion , ( a ) low power field ( xloo ) showing multinucleate giant cells , ( b ) high power field ( x200 ) showing multinucleate giant cells , ( c ) hemosiderin deposits in macrophages , ( d ) area of necrosis at the left side of the picture post - operative magnetic resonance imaging showed no residual lesion . acute , non - traumatic , irritable knee in children is not uncommon in daily practice . the presence of effusion should alert the physician to a diagnosis of infective arthritis and consideration of arthrocentesis . other differential diagnoses include reactive arthritis , transient synovitis , hemophilia , inflammatory arthritis , and malignancy , e.g. , leukemia . the common causes of inflammatory arthritis include rheumatic fever , reiters syndrome and vasculitis ( e.g. henoch - schonlein purpura ) . to the best of our knowledge , our patient is the first case in english literature in which pvns presented as an acute irritable knee . first described by jaffa et al . in 1941 , pvns is a rare inflammatory granulomatous condition of unknown etiology . it can classified into diffuse or localized / nodular type , with knee joint being the most commonly affected joint . the world health organization classification now defines localized pvns under the group of giant cell tumor of the tendon sheath , which encompasses a group of lesion arising from the synovium of joints , bursae , and tendon sheath . while diffuse pvns with diffuse intra - articular involvement is under the group of diffuse - type giant cell tumor . it is even rarer in children and has only been presented as a case report or small case series [ 6 - 10 ] . among the reported cases , the typical presentation was that of a child presenting with chronic recurrent knee pain or swelling , who had sought multiple medical workup . not until a magnetic resonance imaging mri ) or arthroscopy examination was the diagnostic opinion confirmed . delay in diagnosis has been shown to be a risk factor of future morbidity . moreover , the diffuse pvns usually presented with insidious onset of pain , swelling , and stiffness in the involved joint ; while the local pvns more commonly presented with locking , catching , and instability [ 11 - 16 ] . the patient presented with acute irritable and swollen knee , which clinically was highly suggestive of infective arthritis . in our practice , mri is not readily available in such an acute setting . under such clinical scenario , emergency arthroscopy is a justified choice for early diagnosis and treatment of the suspected infection , as a delay in treatment of septic arthritis can lead to the long - term morbidity of the patient . as a result , fortunately , a complete excision of the lesion was achieved which lead to complete recovery . to the best of our knowledge , only one similar case has been reported in an adult , in which a pregnant patient presented with acute knee arthritis mimicking infective arthritis . microscopic evaluation revealed hemorrhage and necrosis , which may suggest torsion or bleeding of the tumor as the cause of the acute symptom . since the stalk of the lesion was quite narrow , this might suggest acute torsion or strangulation of the lesion as the cause of the acute severe knee pain . together with the presence of hemarthrosis , this might suggest an episode of spontaneous hemorrhage from the lesion into the joint leading to acute massive hemarthrosis , which can mimic clinically an infective arthritis . the treatment of pediatric pnvs of the knee follows that of the adult counterparts . for the local / nodular form of pvns in adult , it is well - reported in many studies that local excision can lead to excellent or very good result with complete and persistent regression of the pathology [ 18 - 21 ] . although no comprehensive data are available for the pediatric group in view of the small case load , the similar result has been obtained from the available literature [ 6 - 9 ] . concerning the diffuse type of pvns , studies from the adult age group had shown a higher risk of recurrence and morbidity . subtotal or total synovectomy , either arthroscopic or open , can reduce the risk of recurrence . in contrary to adult , the role of radiotherapy as an adjunctive therapy is not recommended in children in view of potential damage of epiphyseal growth plate and post - radiation sarcomas . pvns , although rare , should be considered as one of the differentials in acute irritable knee in children .
introduction : pigmented villonodular synovitis ( pvns ) is a proliferative condition of the synovium , which is composed of nodules and/or villi and has an abundant number of hemosiderin - laden macrophages.case report : a 10-year - old boy presented with an acute irritable knee . emergency arthroscopy showed a nodular pvns in the intercondylar notch . the symptoms resolved after resection of the lesion.conclusion:pvns of the knee in children is a rare entity . it can be one of the causes of acute irritable knee . complete resection of the nodular pvns can cure the disease .
retinal changes and long - bone infarction are common features in sickle cell disease , orbital involvement is however , not common.123 vaso - occlusive process cause bone infarction in the orbit with subsequent acute rapidly progressive periorbital swelling , fever , marked proptosis , and subperiosteal collection which can closely mimic infectious orbital cellulitis.3456 prompt diagnosis and appropriate management is required to achieve resolution without adverse sequela following this sight threatening complication.7 conservative management is the treatment of choice with occasional surgical intervention.4589 orbital disease without prior history of vaso - occlusive crisis has been reported in a patient with beta thalassemia.9 authors are not aware of previous report of bilateral orbital infarction without prior vaso - occlusive crisis or the concomitant presence of retinal detachment . previously , undiagnosed sickle cell hemoglobinopathy ( hbss ) nigerian child presenting with severe bilateral orbital infarction and retinal detachment is presented . an 11-year - old girl presented with 4 days history of fever , headache , generalized body weakness , and 1 day history of bilateral painful proptosis associated with sudden loss of vision and purulent discharge . her genotype was not known ; she is the fourth of seven children and a 3-year - old sibling died following fever and severe anemia . she had lived all her life in a rural community with both parents who are farmers and genotypes unknown . she was conscious but lethargic , pale , afebrile , mildly dehydrated with bilateral sub - mandibular , and sub - mental tender lymph node enlargement . visual acuity was no light perception in both eyes with marked tender periorbital swelling , axial , non - pulsatile proptosis of 23.5 mm and 23 mm on the right and left respectively [ figure 1 ] . there were no extraocular muscle movements and inferior prolapsing chemosis ; the right corneal was hazy with turbid anterior chamber , round and unreactive pupil , clear lens , and a retrolental greyish reflex . the cornea was clear on the left , with clear anterior chamber , small , unreactive pupil , clear lens , and a retrolental glow . initial diagnosis of bilateral orbital cellulitis to rule out cavernous sinus thrombosis was made ; she was investigated and commenced on broad spectrum intravenous and topical antibiotics empirically as in - patient . ocular ultrasound of bilateral proptosis , bullous retinal detachment , and subretinal fluid collection with no orbital collection or mass [ figure 2 ] , cranial computed tomographyscan exclusion of space occupying lesion , orbital collections or intracranial extension [ figure 3 ] as well as hemoglobin genotype result of hbss lead to diagnosis of bilateral severe orbital infarction in a sickle cell disease . management with systemic / topical steroid , intravenous fluids , analgesics as well as cycloplegics produced sustained progressive reduction in proptosis , chemosis and improved general health although , her vision remained no perception of light in both eyes . she was discharged on oral medications and tapering steroid dose after 2 weeks to continue out - patient management . severe bilateral orbital congestion with proptosis and chemosis ocular ultrasound scan cranio - orbital computed tomography scan orbital infarction , a potentially vision threatening disease of acute onset is known to closely mimic infectious orbital cellulitis;3410 previous knowledge of hemoglobin genotype aids prompt clinical diagnosis while magnetic resonance imaging ( mri ) and technetium assist in making definitive diagnosis.10 orbital infarction requires steroid therapy and rehydration to aid resolution in addition to prophylactic antibiotics use . delay in diagnosis would have been avoided in the index case were the genotype known or from previous history of bone pain crisis . minimal body pains and mild dehydration level in comparison with the severity of orbital involvement was noted in this patient ; ganesh et al . reported the degree of severity of the orbital manifestations appeared unrelated to the severity of sickle cell disease.10 non - availability of mri facility or nuclear scintigraphy studies prevented definitive documentation of bone density changes in our patient.310 bilateral orbital infarction in a previously undiagnosed hbss patient retinal detachment is not a common finding in sickle cell induced orbital bone infarction ; severe acute infarction with attending orbito - ocular inflammation is a probable cause of the exudative retinal detachment . hemoglobinopathy induced orbital infarction should be considered in african children with acute proptosis with or without previous history of vaso - occlusive crisis .
bone infarction involving the orbit in sickle cell disease is not common . bilateral orbital infarction in a previously undiagnosed sickle cell hemoglobinopathy has not been previously reported . in this report , we present a case of an 11-year - old previously undiagnosed sickle cell disease nigerian girl with severe acute bilateral orbital infarction and retinal detachment to highlight that hemoglobinopathy induced orbital infarction should be considered in african children with acute onset proptosis with or without previous history of sickle cell hemoglobinopathy .
since the beginning of the 21st century , with evidence that effective treatment of septic shock requires early recognition supplemented by the use of lactate levels , there has been increasing interest in lactate as a diagnostic , prognostic and therapeutic guide . lactate in its own right thus deserves a brief historical review with respect to the recent article by nichol and colleagues . lactic acid was first discovered in sour milk in 1780 by the swedish chemist karl wilhelm scheele . in january 1843 , german physician - chemist johann joseph scherer found lactic acid in the blood of a woman who died from postpartum septic shock . this report was the beginning of numerous investigations into the utility of lactate as a marker of critical illness . debate also arose regarding whether lactate is a measure of oxygen debt or some combination of increased production and decreased clearance . in 1964 , , these authors stated that they ' neither support nor refute the concept that excess lactate is a valuable index of oxygen debt ' . mmol / l is a safe level ; lactate of 2 to 4 mmol / l signifies a twilight zone ; and lactate > 4 mmol / l reflects probable irreversible global tissue hypoxia with mortality approaching 100% . despite varying opinions over the last several decades regarding the etiology of lactate elevation in critically ill patients , , vincent and colleagues introduced the concept of serial lactate measurements as frequently as every 20 minutes during circulatory shock . they observed that survivors had at least a 10% decrease in lactate during the first 60 minutes of treatment . several investigators in the 1990s continued to study the time variable in lactate kinetics [ 6 - 8 ] . in patients with septic shock , the lactime - the time during which lactate levels remain > 2 mmol / l - was the best predictor of outcome in a multiple regression analysis . similarly , in trauma patients , lactate normalization within 24 hours was associated with 100% survival . at the turn of the century , with the results of early goal - directed therapy , further data showed that a decrease in lactate of at least 10% during the first 6 hours of septic shock was associated with improved outcome , and a 11% decrease in mortality was observed with each 10% increase in lactate clearance . other studies have confirmed these findings and further advocated for lactate - guided treatment protocols [ 10 - 14 ] . in the recent article by nichol and colleagues , both static and dynamic measurements of lactate elevation in critically ill patients the authors studied 36,673 lactate measurements in 5,041 heterogeneous critically ill patients , each with at least two lactate measurements over the first 24 hours in four australian university hospitals . static lactate measures included admission lactate , maximum lactate , and minimum lactate ; whereas dynamic lactate measures were time - weighted average lactate ( lactw24 ) , absolute change in lactate ( lac24 ) , and percentage change in lactate over the first 24 hours in the icu . in multivariate analyses , they found that lactw24 and lac24 were most predictive of hospital and icu mortality . for every unit increase in lactw24 or lac24 , the risk of hospital death increased by 37% or 15% , respectively . when examining prediction models that included other established risk factors such as age , gender , mechanical ventilation , and acute physiology and chronic health evaluation ii score , the addition of lactw24 and lac24 significantly increased the area under the receiver operating characteristics curve to 0.84 and 0.90 for predicting hospital death and icu death , respectively . future comparisons of the receiver operating characteristics curves of these dynamic lactate measures against known prediction models such as the acute physiology and chronic health evaluation , the simplified acute physiology score , or the mortality prediction model would also be informative . the authors acknowledged that the study was limited by its retrospective design , lack of information on confounding patient conditions or treatments that may have affected lactate levels , and assumption that lactate kinetics was linear in nature . additionally , the maximum ( range ) lactate level of 3.4 ( 2.0 to 6.2 ) mmol / l in non - survivors suggested that their study population may not represent patients with a full range of abnormal lactate levels . nichol and colleagues ' study , however , objectively confirmed what we often do at the bedside when faced with laboratory markers that have some prognostic importance . when a marker decreases after some treatment , we are relieved that we have done some good for the patient . when a marker increases significantly , we become concerned and are attentive to the patient 's progress . we are more concerned if the marker worsens over an unexpected period of time . in their study , nichol and colleagues provided a novel approach of examining the dynamic lactate changes that incorporate both the magnitude of change and the time interval of such change . their use of the time - weighted average lactate was most intriguing and makes physiologic sense , which they modeled after the work of finney and colleagues in glucose control . lactw24 is determined first by multiplying the mean lactate between two consecutive time points by its time period ; that is , a time - weighted lactate measurement . this computation is then repeated for each pair of consecutive lactate measurements within a 24-hour interval . the sum of all time - weighted lactate measurements is then divided by the total time period of lactate measurements to determine the time - weighted average lactate over the 24-hour period . importantly , this approach also will provide a framework for investigators to further study trends in lactate when the clinical setting does not provide repeated lactate measurements at absolute time points . such computation can be easily programmed in a handheld device for use at the bedside . in summary , lactate as a marker of disease severity has survived the test of time since its first discovery in 1843 after a patient had died . nichol and colleagues have taken it beyond a static measurement ; hopefully we will continue to use and study lactate , and respect the marker 's presence over time in order to prevent unnecessary patient deaths . lac24 : absolute change in lactate over the first 24 hours ; lactw24 : time - weighted average lactate over the first 24 hours .
lactic acid was first discovered in human blood in 1843 . since then it has been used as a prognosticator of outcome in critical illness . regardless of its etiology , lactate 's presence and trend over time have been shown to be independently associated with mortality . two dynamic lactate measures , the time - weighted average lactate and the absolute change in lactate over the first 24 hours in the icu , were recently shown to be better than static lactate measurements in predicting hospital and icu deaths .
we monitored trachoma prevalence in a village in western nepal for 3 years , using both clinical grading system and nucleic acid amplification tests . three annual azithromycin ( 20 mg / kg ) treatments were distributed to all children ages 110 years in the village ( figure ) . all children were examined biannually , and the conjunctivae of a stratified random sample of children were swabbed and later tested for chlamydia trachomatis dna . at the final visit , 6 months after the last treatment , every child was examined , and their conjunctivae were swabbed . before the first treatment , 39% had active infection determined by the clinical examination , and an estimated 26% ( 95% confidence interval [ ci ] 16% to 35% ) were infected with chlamydia . at the final , may 2001 visit , 7 ( 4% ) of 187 pediatric cases were clinically active . only 1 child of the 187 ( 0.5% ) had evidence of chlamydia by polymerase chain reaction . the prevalence of clinically active trachoma ( gray curve ) and ocular chlamydial infection , as determined by dna amplification tests ( black curve , with 95% confidence intervals due to stratified sampling ) in children 110 years of age in a village in western nepal over time . all children were examined at each visit , so no sampling confidence interval is indicated . likewise , conjunctivae of all children were swabbed for evidence of infection at the may 2001 visit . this study suggests that local elimination of the ocular chlamydia that causes trachoma may be possible in a village with moderate baseline disease . children are by far the most likely to harbor ocular chlamydia , and mathematical models imply that they will be the most difficult group to clear from infection ( 13,14 ) . in fact , 1 year after mass azithromycin treatment in a village in egypt , more infection was identified in children 15 years old than in the rest of the community combined ( 9,15 ) . thus , the nearly complete absence of infection in children after three treatments implies that elimination is a possibility . whether success in this village was due solely to our treatment program or due in part to a secular trend in the area , the results are encouraging . first , repeat infections are almost certainly required to cause severe conjunctival scarring ; occasional sporadic infections probably do not lead to blindness . second , some investigators hope that if ocular chlamydia is reduced to a low enough level , the disease will have difficulty repopulating the community ( population biologists call such a prevalence threshold an allee effect ) . while we see no reason for such a phenomenon a priori , if present , it would certainly establish a threshold target . finally , bacterial , viral , and allergic conjunctivitides can occasionally mimic ocular chlamydia , so eradication of clinically active trachoma programs have already distributed more than 1 million doses of oral azithromycin , and some villages have received three annual treatments . how will we know when to stop ? now is the time to discuss the most appropriate target for trachoma programs and the most appropriate definition for trachoma elimination . the common wisdom is that complete local elimination of ocular chlamydia to zero in a defined geographic area is an unattainable goal , and that programs should settle for reducing the prevalence of ocular chlamydia to a level where little if any subsequent blindness would exist . these results from nepal imply that the strict definition of elimination of ocular chlaymdia in children may be an attainable goal , at least in areas with modest to moderate disease . whether or not elimination is necessary is a separate question .
the common wisdom is that a trachoma program can not eliminate ocular chlamydia from a community , just reduce infection to a level where there would be minimal blindness . we describe the success of multiple mass antibiotic treatments , demonstrating that complete elimination of infection may be an attainable goal in an area with modest disease .
there is no doubt that , among patients with severe intestinal symptoms , resection is mandatory before starting systemic chemotherapy [ 1 , 2 , 3 ] . palliative resection of the primary tumor is also reported to improve the efficacy of systemic chemotherapy and prolong the duration of chemotherapy . a recent review article suggested that non - curative resection of asymptomatic colorectal primary tumors may prolong survival in patients with metastatic colorectal cancer . on the other hand , another article concluded that initial chemotherapy should be started , with resection of the primary tumor reserved for the small proportion of patients who develop major complications from the primary tumor , because resection of an asymptomatic primary tumor provides only minimal palliative benefit . the purpose of surgical resection of primary tumors is the prevention of hemorrhage , perforation , and bowel obstruction . in many cases , it is not possible for patients to continue chemotherapy treatments because of complications such as bleeding , perforation , and bowel obstruction after chemotherapy is started without surgical resection of the primary tumor . thus , it seems necessary to surgically remove the primary tumor in order to continue chemotherapy with few complications . generally , an interval of 4 weeks is considered necessary after an operation before beginning chemotherapy treatments such as folinic acid , fluorouracil , and oxaliplatin ( folfox ) ; folinic acid , fluorouracil , and irinotecan ( folfiri ) ; and capecitabine and oxaliplatin ( xelox ) ; however , there is no apparent evidence for this delay . a metastatic tumor can enlarge rapidly before the start of chemotherapy and possibly lead to patient death . because the significance of the postoperative 4-week delay prior to the start of chemotherapy is not clear , we performed an early chemotherapy start in a patient who had undergone a right hemicolectomy for synchronous multiple liver metastases . a 43-year - old man was referred to our hospital with upper abdominal pain and abdominal fullness . the patient underwent a right hemicolectomy ( open surgery ) with functional end - to - end anastomosis . the oral side of the tumor was accompanied by significant edema and expansion of the ileum ( fig . 2 ) . no drain was used , and the nasogastric tube was removed immediately after surgery . therefore , we started xelox therapy on postoperative day 7 [ oxaliplatin ( 130 mg / m ) on day 1 plus capecitabine ( 1,000 mg / m ) twice daily on days 114 , every 3 weeks ] . the patient was subsequently discharged in good condition on postoperative day 13 . xelox + bevacizumab [ bv ( 7.5 mg / kg ) and oxaliplatin ( 130 mg / m ) on day 1 plus capecitabine ( 1,000 mg / m ) twice daily on days 114 , every 3 weeks ] was administered on postoperative day 28 . after 6 cycles of xelox + bv , ct revealed a reduction in tumor size ( fig . laboratory studies revealed a serum carcinoembryonic antigen level of 1,499 ng / ml , which improved to 312 ng / ml after 6 cycles of chemotherapy . observed adverse events that had previously appeared included only a peripheral neuropathy in the acute stage ( grade 1 ) and hand - foot syndrome ( grade 1 ) . hematological toxicity , surgical site infection , and incisional hernia were not noted . over the past 6 months , tumor progression has not occurred ; the patient is now undergoing xelox + bv therapy and works daily as a pharmacist . the national comprehensive cancer network currently recommends that patients with metastatic colorectal cancer undergo surgical intervention if they have bowel obstruction , an impending obstruction , or metastases that are potentially resectable . complications from the primary lesion are uncommon in these circumstances , and the removal of the lesion delays initiation of systemic chemotherapy . resection of colorectal cancer in patients with severe symptoms is mandatory before starting chemotherapy . in the past , some investigators recommended routine resection of the primary tumor in order to prevent the need for urgent surgical procedures because of local complications [ 1 , 2 ] . in a study by ruo et al . , 30 ( 29% ) of the 103 patients who were initially managed without bowel resection required a subsequent operation for palliation of complications . recently , some authors have suggested elective resection of asymptomatic colorectal cancers in at least a subset of patients with less advanced stage iv disease [ 3 , 9 ] . other authors have suggested deferring resection of minimally symptomatic colorectal tumors because most of these patients succumb to progressive systemic disease instead of the complications related to the intact primary lesion [ 8 , 10 , 11 ] . currently , the precise timing for starting treatment with chemotherapeutic agents prior to and/or after surgery in order to avoid postoperative complications is not clear , but an at least 4-week interval has been suggested . in most clinical trials , patients who have undergone an operation within 4 weeks of the trial 's start are excluded . in benoist 's study , the mean interval between diagnosis and chemotherapy start was 44 days in the resection group versus 15 days in the chemotherapy group . in this case , although an operation was performed , the interval between diagnosis and chemotherapy start was 17 days . resection of the primary tumor significantly increases hospital stay and delays the initiation of chemotherapy , although there is no evidence to suggest that this delay is associated with reduced response rates leading to curative resection or reduced survival . however , there is a chance that patients may die if they are not able to start chemotherapy because of the rapid postoperative progression of a metastatic tumor [ 12 , 13 ] . enhanced recovery after surgery ( eras ) protocols aim at reducing the surgical stress response and optimizing recovery to reduce the length of hospital stay . all elements in eras have separately been shown to improve patient outcome . because eras was developed , an early start of chemotherapy after surgery has been enabled . resection of colorectal tumors with severe stenosis and bleeding is the first step in treatment in order to prevent the complications related to colorectal tumors . an early start of chemotherapy after surgery may be safe and improve the prognosis of colon cancer patients with synchronous metastases . prospective studies are needed to confirm the effect and safety of an early start of chemotherapy after surgery . we have already started a new clinical trial to examine patients receiving an early start of chemotherapy .
the start of chemotherapy treatment usually requires a delay of about 4 weeks after surgical resection in patients with primary colorectal cancer and synchronous distant metastasis . however , there is no evidence to indicate the required length of this delay interval . in addition , there is a chance that a patient may die because postoperative chemotherapy was not started soon enough and a metastatic tumor was able to develop rapidly . here , we present a case in which combination chemotherapy with capecitabine and oxaliplatin ( xelox ) was started within 1 week after a right hemicolectomy for synchronous multiple liver metastases . to our knowledge , this is the first report of the start of chemotherapy , involving treatments such as folinic acid , fluorouracil , and oxaliplatin ( folfox ) ; folinic acid , fluorouracil , and irinotecan ( folfiri ) ; and xelox , within 1 week after a colorectal cancer operation with anastomosis . the findings suggest possible changes in the start time of chemotherapy after surgery in the future .
retroperitoneal liposarcomas represent a rare subtype of soft tissue sarcoma because of their localization ( only 20% of soft tissue sarcomas are located retroperitoneally ) and histological type ( only 15% of soft tissue sarcomas are liposarcomas and , of these , only a third develop from perirenal fat ) [ 1 , 2 ] . the disease is more frequent in men in the fifth decade of life and has non - specific symptoms induced by the tumour . here , we present the cases of two patients , of different ages , diagnosed with perirenal myxoid liposarcoma . the first is the case of a 65-year - old man without significant personal history who presented to the general practitioner with a progressively distended abdomen associated with weight loss , fatigue and dyspnoea . abdominal ultrasonography and contrast - enhanced abdominal computed tomography ( ct ) found a voluminous , mixed - density mass located retroperitoneally in the right perirenal area ; the mass was approximately 39 39 38 cm in size , with clearly defined , irregular margins and a few visible vessels , and was subsequently identified macroscopically as a right renal angiomyolipoma . the mass was in contact with the pancreas ( displacing the cephalic and corporeal sides of the pancreas ) , the stomach ( the distal third ) , the aorta , the liver ( the visceral side ) , the gallbladder , the ascending and transverse colon , the inferior vena cava and the portal vein . the mass occupied the entire right hemi - abdomen , compressing the abdominal organs , displacing the liver cranially above the sixth rib and compressing the large vessels ( fig . 1 ) . 2 ) , for which we initiated specific therapy . figure 1:thoracic and abdominal ct after the haemodynamic status of the patient was optimized , surgical intervention was performed via a transperitoneal approach , and a retroperitoneal mass weighing 14 kg was excised . during surgery , no metastases were found in other abdominal organs , so no other organ resection was necessary . the operating time was 250 min , and 3 units of abo / d group - compatible blood were administered . pathological examination revealed elements of perirenal myxoid liposarcoma , fdration nationale des centres de lutte contre le cancer ( fnclcc ) grade 2 ( score 2 + 1 + 1 = 4 ) , without lymphatic or vascular invasion and with negative resection margins . adjuvant therapy was administered after surgery according to the inno-206 protocol as follows : inno-206 was administered at 350 mg / m ( 260 mg / m doxorubicin equivalent ) intravenously on day 1 every 21 days for up to six consecutive cycles . the patient was monitored by thoracic and abdominal ct at 3 , 6 and 12 months after surgery . local recurrence was identified at the 18-month check - up , and the patient was started on chemotherapy with duloxetine at 30 mg / day for the first week and 60 mg / day for the next 4 weeks . the second case was of a 70-year - old male patient who presented with abdominal pain . contrast - enhanced ct revealed , in the retroperitoneal right perirenal area , a mass measuring 20 22 27 cm in size , of mixed structure including predominantly fluid and fatty components . the mass displaced the right kidney and the ascending colon towards the midline . the surgical procedure chosen was en bloc excision of the mass together with the right kidney via a transperitoneal approach . histopathological examination revealed elements suggestive of an fnclcc grade 2 ( score 4 ) myxoid liposarcoma derived from perirenal fat ( pt2bn0m0l0v0r0 ) . the patient was monitored via ct and , 3 years after the surgery , presented a local recurrence , for which he required chemotherapy . retroperitoneal perirenal liposarcomas are rare tumoural entities that are highly aggressive and have a poor prognosis . since they develop in the retroperitoneal space , initially with asymptomatic progression , their diagnosis is frequently delayed until they are large , and they are difficult to treat . sixty percent of retroperitoneal liposarcomas measure > 10 cm at the time of diagnosis ; thus , early diagnosis and treatment of perirenal liposarcomas remain challenging . most authors report local recurrence in the first 2 years after surgery , sometimes at multiple sites , even in cases with well - differentiated histology [ 3 , 4 ] . both our reported patients had local recurrence even the patient diagnosed at the local stage ( the second ) . retroperitoneal liposarcomas contain adipocytes of various sizes , and lipoblasts , and these cells are suspended in an intensely vascularized myxoid matrix . the mitotic index in these tumours is high , thus producing areas of acute ischaemic necrosis , and focal areas which can be described as sclerosing liposarcomas . some histological types also include an osteosarcoma - like or clear renal cell component , but we have not encountered those types in our experience . high - grade liposarcomas have an aggressive evolution with systemic dissemination , especially to the lungs , so the majority of patients have a low survival rate after surgical intervention . several factors are known to correlate with recurrence or survival , such as age over 50 years , advanced tumoural stage , incomplete tumoural resection , poor tumour differentiation , infiltration of resection margins , lymph node infiltration and the presence of metastases . treatment involves resection of the tumoural mass en bloc , with adjacent organ resection when needed . the positive margin rate in one large series , either macroscopically or microscopically , was 51% , and the efficacy of radiotherapy or chemotherapy in these cases remains controversial .
liposarcomas are neoplasms of mesodermic origin , are derived from adipose tissue and represent < 1% of all malignant tumours . primary liposarcomas of the kidney are very rare . here , we present the cases of two patients diagnosed with retroperitoneal perirenal myxoid liposarcoma . the patients were diagnosed via imaging , which in both cases revealed a huge right retroperitoneal tumour mass compressing the abdominal organs and large blood vessels . surgical intervention consisting of en bloc resection of the tumour and the right kidney was performed using a transperitoneal approach . three years after the surgery , both patients presented local recurrence , for which they underwent chemotherapy . liposarcomas with renal origin are rare clinical entities with a high rate of malignancy and a poor prognosis . because the use of chemotherapy and radiotherapy in the treatment of such liposarcomas is controversial , the treatment of choice is wide surgical resection with clean margins .
eruption is the axial or occlusal movement of the tooth from its developmental position within the jaw to its functional position in the occlusal plane . the common and important reasons attributed to delayed eruption are usually insufficient space , early loss of primary teeth with eventual closure of space , crowding of arches and rotation of tooth buds . excessive fibrous tissue over an erupting tooth is also one of the most common causes , with eruption cysts being relatively rare . complete lack of eruptive force can be the reason for unerupted tooth when the normal number of teeth is present radiographically , although this is debatable , since few workers have debunked this claim . syndromes , metabolic , and hormonal disorders are also other causes of multiple impacted permanent teeth . finally crowding and rotation of tooth buds can also lead to impacted teeth . the objective of the study is to increase awareness of cases of multiple unerupted teeth in which multiple etiologies need to be discussed and investigated . a 17-year - old male patient reported with a chief complaint of multiple missing teeth and compromized esthetics . he had no relevant family history and this was his first dental visit . on further enquiry the patient revealed that he had loss of deciduous teeth with no eruption of permanent teeth . on examination , the patient had multiple retained deciduous teeth with only the permanent maxillary central and lateral incisors , mandibular central incisors , right maxillary first molar and left mandibular first molar present . the incisal length of the maxillary central incisors appeared reduced ( partial eruption ) and the patient had a high arched palate [ figure 1 ] . high arched palate along with reduced incisal length is also visible the patient was advised opg , chest x - ray and skull x - ray . opg revealed the presence of impacted , unerupted , and missing permanent teeth [ figure 2 , table 1 ] . skull and chest x - rays did not reveal any other obvious deformities [ figure 3 ] . panaromic radiograph of patient showing retained deciduous teeth and multiple impacted permanent teeth missing , unerupted , and impacted teeth skull and chest xrays which do not reveal any obvious deformities the patient did not report for investigations and was unresponsive . multiple impactions are seen rarely and are usually associated with systemic conditions . guided eruption of many teeth with the help of coordinated multidisciplinary management there are numerous eruption - regulating molecules having similar and overlapping functions , which ensures that even the absence of a single factor does not interrupt the event of eruption . but egf , egf - r , csf-1 , csf-1r , il-1 , il-1r , c - fos , nfb , mcp-1 , tgf-1 , pthrp , cbfa - l ( now called runx2 ) , opg , rank / rankl are the major tooth eruption molecules . stellate reticulum is the site for few molecules with the majority of the eruption molecules residing in the dental follicle . while most eruption defects are part of a genetic syndrome , they can also be nonfamilial , as in the case in primary failure of eruption ( pfe ) . in pfe localized failure of eruption of candidate genes for primary failure of eruption would be the molecules that function solely in the pre - eruptive phase and that are expressed in cells of the dental follicle and surrounding structures . hence , it is likely that genes like csf-1 , nfb , and c - fos are the genes responsible for the eruption defect . common reasons like lack of space , prolonged retention or early loss of deciduous tooth , abnormal position of tooth bud , presence of alveolar cleft , ankylosis , cystic or neoplastic formation , alveolar or dental trauma , and dilacerations of teeth falling into the localized category . the differential diagnoses in case of syndromes are cleidocranial dysostosis , gardners syndrome , gorlin sedano syndrome , and yunis varon syndrome . in the case of cleidocranial dysplasia the patients usually exhibit high - arched palate with prolonged retention of the deciduous teeth leading to subsequent delay in eruption of the deciduous teeth . though both the intraoral features were present in our case the abnormalities of skull and shoulder girdle along with multiple supernumerary impacted teeth were absent . gardner 's syndrome that also presents with multiple impacted teeth was ruled out as associated signs like multiple epidermoid cysts and other lesions were absent . sedano syndrome represents as short hands , foot bones with short and straight collar bone along with multiple impacted teeth . features of yunis varon syndrome are agenesis or hypoplasia of clavicle , severe micrognathia , digital anomalies , hypodontia , spinal defects , and impacted teeth . both the syndromes presented with multiple impacted teeth but were ruled out from our case as other symptoms were not present . in the case of hormonal disorders hypothyroidism , hypoparathyroidism , and pseudo hypoparathyrodism are to be considered . estimation of t3 , t4 , and tsh is required for diagnosing hypothyrodism where there is low serum t4 and elevated tsh . evaluation of total serum calcium concentration and serum parathyroid hormone is required for hypoparathyrodism and pseudohypoparathyroidism . there is decrease of serum calcium in both and elevation of parathyroid hormone in pseudo hypoparathyroidism and decrease of hormone in hypoparathyrodism . metabolic disorders like vitamin d deficiency rickets are also associated with impacted teeth . in our case , failure of eruption could also be due to crowding of supernumerary teeth but in our case that was not observed . in this case , the medical and family histories along with extraoral examination were not suggestive of any syndrome or metabolic disorder . only a few cases of nonsyndrome multiple impacted teeth were reported.[913 ] in those studies the predominant explanation was that some physical barrier led to impaction and noneruption of teeth . duration and results of treatment in less frequent cases of multiple impactions is a major concern when compared to more frequent single impaction cases . multidisciplinary approach would be the appropriate choice as treatment involves esthetics , functional , and oral health problems . in case of unerupted teeth , orthodontic extrusions may be attempted . in view of the pathological potential of the impacted teeth , surgical removal is recommended . rehabilitation of the patient with fixed dentures needs to be planned with the help of a prosthdontist and implantologist . obtaining stable results along with enhanced esthetics , oral health , and function would be the objective of the treatment . hypodontia and eruption problems are mainly focused on genes such as csf 1 , nfb , and c - fos . radiographs reveal the presence of multiple impacted teeth but additional investigations are needed to rule out systemic and metabolic conditions . a decision about the management since the patient was regrettably lost to follow - up , genetic , and other studies could not be performed . . therefore , an idiopathic case of multiple impacted teeth could be the only possible diagnosis , since the term idiopathic refers to unknown cause , which is the case in this particular scenario .
multiple impacted permanent teeth are usually related to syndromes , metabolic and hormonal disorders . however , in some cases , impaction of multiple teeth is not associated with any syndrome . in this report , we present a case of 17-year - old male patient with missing teeth . radiographs revealed multiple impacted permanent teeth , though medical and family history along with physical examination was not suggestive of any syndromes . if other investigations are negative , an idiopathic case of multiple impacted teeth is suggested to be the possible diagnosis . the objective of this report is to increase awareness of such cases especially in the absence of hereditary / genetic / metabolic factors usually inherent in such scenarios . the patient management in such cases needs to be planned specifically from a multidisciplinary standpoint .
vagal paragangliomas are rare tumours that are estimated to account for 5 - 25% of all paragangliomas of the head and neck region ( 1,2 ) . they can develop anywhere along the course of the vagus nerve and their clinical presentation depends on where it is located in the parapharyngeal space . the most common symptoms are neck masses , dysphonia , dysphagia , fullness of the pharynx , pain , and coughing . the presence of a functionally active paraganglioma that secretes catecholamies is only found in 1 - 3% of all reported cases ( 3 ) . malignant paragangliomas are also rare and are found in 10 - 19% of cases ( 4 ) . malignancy is diagnosed via the presence of metastases in lymph nodes . in this case report , we describe a man presenting with the most common symptoms of a progressively growing neck mass and dysphonia , and an eventual diagnosis of a malignant , functionally active paraganglioma . a previously healthy 61- year- old right - handed male presented to his family physician with a right neck mass and a worsening hoarse voice ( dysphonia ) . the neck mass had been present for the past few years , but had noticeably increased in size during the past 2 months . when he presented to his family physician , his dysphonia had worsened when compared to its original onset and was resulting in voice fatigue and pitch breaks . his past medical history was significant for labile hypertension , coronary artery disease , with a myocardial infarction and subsequent stent insertion , diabetes mellitus type 2 , and hypercholesteremia . his family physician ordered a ct neck and referred him to our hospital for an otolaryngology - head and neck surgery consult . during his initial assessment , his physical exam confirmed the presence of a pulsatile neck mass involving levels 2 , 3 , and 4 with extension into the tail of the parotid . the cephalad extent of the mass was located above the angle of the mandible . at the completion of the examination , a tentative diagnosis of a right glomus vagale paraganglioma with right vocal cord paralysis further lab and imaging investigations were required to confirm the diagnosis and determine the presence of any coincidental or metastatic disease . a 24h - urine test was performed and it revealed elevated norepinephrine ( 3011 nmol / d [ n : < 600nmol / d ) , creatinine ( 107 ummol / l [ n : 44 - 106 ] ) , and a norepinephrine / creatinine ratio ( 292.3 umol / mmol [ n < 70 umol / mmol ] ) , thus confirming biochemical catecholamine activity , suggesting a presumptive diagnosis of a biochemically active glomus vagale . ct angiogram ( figure 1 ) confirmed the palpable neck mass to be a glomus vagale . a 4 cm x 4 cm x 8.5 cm vascular lesion was found posterior to the carotid sheath with anterior displacement of carotid bifurcation and the internal carotid artery . the vascular lesion extended from the level of the proximal carotid bifurcation to the skull base . the right jugular bulb remained intact and hypoplastic when compared to the left jugular bulb . mass is located posterior to the carotid sheath with anterior displacement of carotid bifurcation and the internal carotid artery . this was done to devascularize the tumour as thoroughly and safely as possible in order to limit the amount of surgical blood loss . the next day , the patient underwent a right neck dissection , parotidectomy and right glomus vagale tumour resection and right injection laryngoplasty . the right vagus nerve , glomus vagale tumour , and right thyroid were sent to pathology . the pathology report also revealed the presence of metastatic tumour in a positive chromogranin - stained lymph node , thus fulfilling the criteria for malignancy . lymph node stained positive for chromogranin , confirming the presence of metastatic disease ( dark brown / black cells ) . was booked at the otolaryngology clinic for a few weeks post - hospital discharge , with radiation oncology consultation . a new neck mass is a relatively common head and neck problem . for patients presenting with adrenergic - type symptoms , a 24h urine test can be done to detect the presence of a biologically active tumour . imaging studies can help to confirm the presence of the mass , its extent , as well as to help narrow the possible diagnoses . neck masses should be carefully monitored for changes in size and for any distortions to surrounding structures and their given function . radiotherapy is associated with a lower incidence of complications and morbidity , but only provides partial local control of paragangliomas . therefore , surgery is considered the treatment of choice for both benign and malignant vagal tumours . embolization is performed pre - operatively to safely and thoroughly devascularize the paraganglioma . for patients with functionally active vagal paragangliomas , careful follow - up post treatment is necessary to monitor the patient 's well being and status .
this teaching case report represents an unusual example of a neck mass in a previously healthy individual . the presence of a new neck mass is a relatively common head and neck problem and requires a full work up including a complete history and physical examination . with respect to our patient , thorough history taking , physical examinations and specific investigations led to the diagnosis of a malignant and functionally active paraganglioma.vagal paraganglioma themselves are rare tumours and account for only 5 - 25% of all paragangliomas in the head and neck region . the presence of a malignant , functionally active , catecholamine - secreting paraganglioma is even rarer and accounts for only 1 - 3% of all reported glomus vagale tumours.this case report illustrates the need to carefully monitor all neck masses for changes in size , for any distortion to surrounding structures , and their given function .
benign tumours , such as leiomyomas account for less than 1% of all hypopharyngeal and oesophageal tumours ( 1,2 ) . leiomyoma is the most common benign oesophageal neoplasm , the reported post - mortem incidences range from 0.005% to 5.1% . however in comparison to oesophageal carcinoma they are relatively rare . usually presenting in the 3rd-5th decade with a male : female ratio of 2:1 ( 3 ) . we report on a 48-year old female ex - smoker who presented to the emergency department with a sizeable right - sided oral cavity mass associated with audible stertor , but no significant airway compromise in the upright position . the mass appeared after an episode of forceful vomiting having had a cough for two weeks . the remainder of the history was unremarkable apart from an alcohol intake of up to 27 units of wine a week . this demonstrated that the large pedunculated necrotic mass was in fact , appearing to arise from the right piriform fossa and not the tonsil confirmed by rigid pharyngolaryngo - upper oesophagoscopy . the upper oesophagoscopy was limited to 20 centimetres ( cm ) due to mucosal oedema . the mass was ligated at its base ( silk suture ) and sent for further analysis ( figure 1 ) . it is likely that the mass had acutely twisted and protruded into the upper oesophagus causing obstructive symptoms leading to the history of sudden - onset vomiting . the patient s post - operative recovery was uneventful and an outpatient review was arranged . histology confirmed a benign leiomyoma macroscopically measuring 5 x 3.5 x 3 cm , which had been shelled out of a squamous covering with no evidence of dysplastic or malignant change . further imaging [ computerised tomography ( ct ) of neck ] demonstrated a remnant of the base of the lesion low in the piriform fossa possibly going up to the upper oesophagus and a barium swallow revealed some oesophageal mucosal irregularity at approximately 21 cm . further flexible nasoendoscopy assessment in clinic was concerning and unhelpful in assessing the piriform fossa ( right side erythematous and oedematous ) , prompting a further general anaesthetic rigid laryngopharyngo - upper oesophagoscopy procedure to exclude a synchronous tumour . this revealed a normal piriform fossae and a stump in the left posterior upper oesophagus at approximately 19 cm , with the original silk suture attached to it suggesting that the mass was in fact an oesophageal leiomyoma . the patient was kept under close review jointly with the general surgeons for flexible oesophageal endoscopy and eventually underwent a flexible endoscopy whereby the stump was removed , confirming the same histology ( oesophageal mucosa ) . our case presented with leiomyoma emanating from the upper oesophagus , which is rare as they are most frequently found in the mid to lower oesophagus correlating with the predominant muscular composition found in each of the regions ; smooth muscle in the lower third and mixed ( smooth / skeletal ) muscle in the middle third . they usually appear intramurally our case presented intraluminally as a pedunculated mass with sudden onset symptoms of nausea and vomiting as opposed to the usual non - specific , longer duration symptoms of dysphagia , pain and weight loss ( 3 ) . macroscopically leiomyomas are characterised as well circumscribed , smooth or nodular masses with a whorled cut surface ( figure 2 ) and microscopically with bundles of smooth muscle cells intermingled with collagenous stroma ( 4 ) . imaging modalities may include ct scanning to assess extra - oesophageal extent , barium swallow ( filling defect ) and endoscopic ultrasonography to assess which oesophageal layer the mass is in and its size , extent and nature ( sold or cystic ) . management options are non - operative ( asymptomatic ) or operative from enucleation via endoscopic , open or thorascopic methods to oesophageal resection and reconstruction for select tumours ( greater than 8 cm or adherent to the mucosa etc . ) anecdotally , a similar case was described by simpson , 1934 of a lady coughing up a mass , which was subsequently swallowed . the patient was erroneously diagnosed as having globus hystericus and presented ten months later with a similar picture associated with sudden onset dysphagia . furthermore , direct laryngoscopy confirmed a mass suspended by a pedicle from the area of the right piriform fossa hanging into the oesophageal entrance . this appeared to have twisted causing a haemorrhagic necrosis and the acute symptoms ( 5 ) . our case describes the rare occurrence of an upper oesophageal leiomyoma presenting acutely as a large mass in the oral cavity . this diagnosis should be entertained as a differential in the circumstance of an odd - sounding referral of a patient who is reported to have coughed up their tonsil.
we report on a case of oesophageal leiomyoma presenting as a potential cause of sudden airway obstruction . the patient presented with a large mass in the oral cavity after an episode of forceful vomiting . the operative findings and histology of which confirmed this to be an oesophageal leiomyoma emanating from the upper oesophagus , a rare finding . the mass was excised and the patient s subsequent post - operative recovery and follow - up has been unremarkable.this diagnosis should be borne in mind when one is referred the odd sounding case of a patient who appears to have coughed up their tonsil.
( keller et al . , 2011 ) trans - tiliroside an a active principle of potentilla chinesis decreased blood glucose level and total cholestrol , low density lipoprotein ( ldl - c ) and triglyceride levels in alloxan - induced diabetic mice and streptozotocin - induced diabetic rats . ( qiao et al . , 2011 ) eleutherinoside a from eleutherine americana , displayed in vitro -glucosidase inhibitory activity . ( ieyama et al . , 2011 ) kaempferol-3-neohesperidoside an active antidiabetic compound showed insulin mimetic action . ( trojan - rodrigues et al . , 2011 ) bergenin , a major constituent isolated from caesalpinia digyna rottler ( leguminosae ) displayed significant antidiabetic , hypolipidemic and antioxidant activity and regenerative effect on pancreatic cells in type 2 diabetic rats . ( kumar et al . , 2012 ) two new flavones isolated from callistemon lanceolatus dc ( myrtaceae ) characterized as 5,7-dihydroxy-6,8-dimethyl- 4 -methoxy flavone and 8-(2-hydroxypropan-2-yl)-5-hydroxy-7-methoxy-6-methyl-4-methoxy flavones exhibited blood glucose lowering effect in streptozotocin induced diabetic rats . marrubiin , a constituent of leonotis leonurus , increased the insulin level and glucose transporter-2 gene expressions in ins-1 cells . ( mnonopi et al . , 2012 ) three new germacrane sesquiterpenes obtained from tithonia diversifolia , significantly increase glucose uptake without significant toxic effects in 3t3-l1 adipocytes . ( zhao et al . , 2012 ) alisol f and alisol b compound of alismatis rhizoma displayed in vitro -glucosidase inhibitory activity . ( li et al . , 2012 ) scrophuside , obtained from the roots of scrophularia ningpoensis hemsl . ( hua et al . , 2014 ) iridoid glycosides , ningposide i and ningposide ii obtained from the roots of scrophularia ningpoensis hemsl ( hua et al . , 2014 ) chalcomoracin , moracin c , moracin d and moracin n are the compounds isolated from morus alba exhibited a significant degree of -glucosidase inhibitory activity . ( yang et al . , 2012 ) malonyl ginsenosides , from the roots of panax ginseng showed significantly lower fasting blood glucose level , improvement of insulin sensitivity and improvement of lipid profile in diabetic rats . ( liu et al . , 2013 ) 6-o - galloyl-5'-hydroxy mangiferin , mangiferin , 5-hydroxy mangiferin , and methyl gallate are the compounds isolated from mangifera indica reduced the blood glucose levels in alloxan - induced diabetic rats . ( md et al . , 2013 ) 1,2,3,4,6 penta - o - galloyl--d - glucose , a bioactivity guided isolated compound from mangifera indica inhibits 11--hsd-1 and ameliorates high fat diet - induced diabetes in c57bl/6 mice . ( mohan et al . , 2013 ) ginsenoside re , exhibited antidiabetic activity by reducing insulin resistance through activation of ppar- pathway by directly increasing the expressions of ppar-2 and its responsive genes in 3t3-l1 adipocytes . ( gao et al . , 2013 ) 8-oxo - berberine isolated from berberis brevissima inhibited protein tyrosine phosphatase 1b ( ptp 1b ) . ( ali et al . , 2013 ) chicoric acid isolated from ocimum gratissimum l. reduced significantly the glycemic levels of diabetic mice . ( casanova et al . , 2014 ) asiatic acid , showed antidiabetic activity with improvement in the lipid profile in rats .
diabetes mellitus ( dm ) is one of the most prevalent metabolic disorders characterized by increased blood glucose levels and improper primary metabolism resulting from the defects in insulin secretion , insulin action , or both . it is one of the most common health problems worldwide , and the prevalence of this disease is rapidly increasing , leading to microvascular ( retinopathy , neuropathy and nephropathy ) and macrovascular ( heart attack , stroke and peripheral vascular disease ) complications ( umar et al . , 2010[18 ] ) . the number of individuals with diabetes is increasing due to population growth , aging , urbanization and increasing prevalence of obesity and physical inactivity . according to recent estimates , the greatest absolute increase in the number of patients with diabetes will be in india and the total number is projected to 79.4 million in 2030 . it is expected that about 366 million are likely to be diabetic by the year 2030 ( rahman and zaman , 1989[14 ] ) . medicinal plants are the main source of organic compounds such as polyphenols , tannins , alkaloids , carbohydrates , terpenoids , steroids and flavonoids . these organic compounds represent a source for the discovery and development of new types of antidiabetic molecules . many compounds isolated from plant sources have been reported to show antidiabetic activity . the key messages summarize some recent information in the field of antidiabetic phytochemicals .
there are few case reports of corporal abscess in males , which are usually caused after trauma , intervention or infection.12 these were managed with either incision and drainage or percutaneous aspiration . we are reporting an unusual case of spontaneous emphysematous periurethral abscess which involved the bilateral corpora cavernosa , and was managed endoscopically . a 48-year - old gentleman , with history of diabetes mellitus , presented to the emergency department with high grade fever , lower urinary tract symptoms and perineal pain for 3 weeks . local examination revealed a mildly tender swelling at the penoscrotal [ figure 1 ] and perineal region . per - rectal examination revealed bogginess over the prostate , but the trans - rectal ultrasound was inconclusive . an urgent computed tomography scan revealed a moderate sized periurethral collection around the bulbo - membranous urethra extending into both the corporal bodies , containing specs of air foci [ figure 2 ] . the patient was taken up for cystourethroscopy , which revealed the bulging area near bulbo - membanous junction with trickling of pus . the prostatic urethra and verumontanum the most bulging point in the bulbar urethra was de - roofed , which drained 75 - 80 ml of copious thick pus . his fever and toxemia resolved after drainage and broad spectrum intravenous antibiotics . a retrograde urethrogram after 3 months revealed mild narrowing at bulbo - membranous region , which was managed endoscopically . he was put on self calibration and remained asymptomatic after 6 months and did n't have erectile dysfunction . clinical photograph showing swelling in scotoperineal region , which on compression discharged the pus per urethra computed tomography scan showing abscess in the region of corporal bodies ( a ) and root of penis ( b ) with presence of air foci corporeal infection and abscess formation has been described in association with trauma , penile prosthesis , cavernosography , intracorporeal papaverine injection and gonorrhoea.123 yachia and fiedman reported a case of tuberculous cold abscess of corpus cavernosum.4 this condition had been treated with either percutaneous minimally invasive aspiration or conventional incision and drainage.156 minami et al . , have described two cases of corporal abscess , although not emphysematous ; which were treated by incision and drainage.6 this resulted in erectile dysfunction . others also found the similar outcome.7 in our case , we got the response with the endoscopic drainage and did n't encounter the problem of erectile dysfunction.67 we performed a thorough search of literature , but did n't found any report of emphysematous abscess involving the corpora . take home message : the periurethral and corporal abscess does n't always require open or percutaneous drainage , but can be managed endoscopically with good results .
we came across an interesting case which was presented with fever , dysuria and perineal pain , not responding to antibiotics . the computed tomography scan showed periurethral abscess containing multiple air specs with involvement of bilateral corpora cavernosa . we successfully treated this patient with endoscopic drainage . spontaneous periurethral and corporal abscess in male is a rare entity and emphysematous form in corpora has not been described before .
intracranial acc is even more infrequent and could be primary or secondary , occurring either by direct invasion , hematogenous spread or perineural spread . we report cytodiagnosis of lacrimal gland adenoid cystic carcinoma , metastatic to the calvaria , epidural compartment and brain parenchyma . a 61-year - old male presented with swelling and pain on right side of forehead , nasal obstruction and bleeding from right nostril . as per history given by the patient , he developed gradually progressive pain as well as swelling in the supraorbital region of right eye for which the biopsy was taken in a local hospital and after going through the histopathology report , he was referred to a tertiary care hospital for further management . the patient had lost all records and only gave us history of undergoing enucleation of right eye and taking chemotherapy after surgery . on local examination , a diffuse swelling over right side of forehead extending from supraorbital aspect to frontoparietal region of head was observed . the swelling was approximately 8 7 cm in size , tender and margins were indistinct , merging imperceptibly into the surrounding structures . computed tomography scan showed an irregular area of focal calvarial destruction in the right frontal region . this was associated with a lobulated extra axial mass lesion , with multiple peripheral areas of ring enhancement [ figure 1 ] . ct scan showing irregular area of focal calvarial destruction in the right frontal region fine - needle aspiration cytology of the forehead swelling was done . the aspirate yielded highly cellular smears with a monomorphic population of small , slightly atypical cells , arranged in multilayered groups with abundant fine intercellular metachromatic substance between cell groups [ figure 2 ] . at places , dense aggregates of monomorphic small cells with uniform round to oval hyperchromatic nuclei and scanty cytoplasm were seen . numerous hyaline globules of varying size surrounded by tumor cells were also seen [ figure 2 , inset ] cytodiagnosis of metastatic acc was rendered . on exploration , an infiltrating lobulated irregular soft - tissue mass was found involving the right frontal bone , adjacent dura and the brain . biopsy was taken and histopathological examination confirmed the diagnosis of metastatic acc . basement membrane material in finger - like structures between the tumor cells . acc is the most common primary in the major and minor salivary glands but can also arise from submucosal seromucinous glands of larynx and trachea and from lung . these tumors show slow but relentless growth . their aggressive behavior with a high - risk of local recurrence and late distant spread of the tumor even after aggressive management has been reported . the metastasis from accs is known to occur late , even many years after the removal of the primary tumor . they occasionally metastasize through hematogenous spread to lungs , brain and bone , in decreasing order of frequency . a few reports on lacrimal gland acc have described the time interval needed for presentation of metastases and duration of follow - up required . the overall frequency of intracranial invasion of acc has been reported to be between 4 and 22%.[810 ] true hematogenous metastases to the central nervous system are rare , even though hematogenous metastases to liver , lung and bone are reasonably common late in the course of this disease . perineural invasion is considered an indicator of poor prognosis , because of the inherent risks of spread to the skull base and local recurrence . acc of the lacrimal gland has a greater likelihood of invading intracranially , since more neural and vascular structures exist in the orbit and the bones of the orbit are connected directly to the intracranial cavity . these factors increase the risk that an acc of the lacrimal gland will invade intracranially through perineural , vascular , intraosseous and leptomeningeal routes and through nerve sheath . therefore , neurosurgeons need to be familiar with the tendency of acc to extend intracranially . extra - axial intracranial metastatic adenoid cystic carcinoma mimicking meningioma has been reported.[911 ] these reported lesions were , however , basal and therefore were consistent with the perineural invasion so typical of these tumors . koller et al . reported a hematogenous brain metastasis from an acc of the breast . reported a hematogenous frontal lobe metastasis of acc with an occult primary tumor . in summary , our case demonstrates a histologically confirmed metastasis of lacrimal gland acc to the meninges and brain , which is an unusual manifestation of this tumor . we could establish the diagnosis of intracranial metastasis on fnac of the mass in the frontal region . the cytomorphologic features of acc of both primary and metastatic sites are so distinctive as to permit a definite diagnosis .
adenoid cystic carcinoma ( acc ) of the lacrimal gland is a rarely encountered orbital tumor . it invades intracranially more frequently than carcinomas of other glands in the head and neck . a 61-year - old man underwent right orbital exenteration for a tumor in the supraorbital region . he had lost all records and presented to us with a diffuse swelling in the right forehead , six years later . fine - needle aspiration cytology of the right frontal swelling revealed monomorphic population of small , slightly atypical cells , arranged in multilayered groups with abundant fine intercellular metachromatic substance between cell groups . there were hyaline globules of varying size with attached tumor cells . cytological diagnosis of metastatic intracranial acc with a lacrimal gland primary was suggested . biopsy of the swelling confirmed our cytological diagnosis of acc with perineural , vascular and lymphatic invasion . thus , the authors report this case of intracranial metastasis of acc of the lacrimal gland to remind neurosurgeons planning intervention that this disease shows a tendency to invade intracranially .
primary neuroendocrine carcinoma ( nec ) of the breast is a rare distinct clinicopathological entity , comprising 0.5 - 2% of breast carcinomas world - wide . in 2003 , primary nec of the breast was identified as a distinct entity by the world health organization ( who ) classification of tumors . the who classification defines primary nec of the breast as tumors that express 50% or more of ne markers there are no previous reports of f-18 fluorodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) in patients with primary nec of breast with liver and bone metastasis . here we describe a case of a 45-year - old female patient who presented with jaundice and was evaluated to have multiple liver lesions and biopsy from liver showed metastatic neuroendocrine tumor . her chromogranin level was 886 ng / ml ( normal < 78 ng / ml ) . she was referred for the whole body pet / ct for detection of the primary site . pet / ct showed an intense uptake in the soft- tissue necrotic lesion in the inner quadrant of the left breast [ figure 1a and b ] with the same charecteristic feature of multiple hypodense liver lesions [ figure 1c ] . furthermore uptake noted in a lytic lesion in the d4 vertebra [ figure 1d and e ] . patient underwent fine - needle aspiration of the breast lesion , which confirmed neuroendocrine origin [ figure 2a and b ] . a diagnosis of primary nec of the left breast with metastasis was made and she was treated with peptide receptor radionuclide therapy and is on follow - up now . whole body fluorodeoxyglucose - positron emission tomography / computed tomography ( pet / ct ) maximum intensity projection image ( a ) , axial fused pet / ct showed a intense uptake in the soft - tissue lesion in the left breast ( b ) , liver lesions ( c ) and bone lesion ( d ) , axial ct showing lytic bone lesion in d4 vertebra ( e ) immunohistochemistry staining showing positive for chromogranin ( a ) and synaptophysin ( b ) primary nec of the breast is extremely rare with the first reported case in 1983 . the most frequent reported age varies from 40 to 70 years , with a higher incidence in women greater than 60 years . as metastatic neuroendocrine tumors of the breast are more common than that of primary neuroendocrine tumors of the breast , it is , therefore , important to differentiate primary breast neuroendocrine tumor from metastatic disease to the breast because of the differences in treatment focus . primary nec of the breast can be diagnosed if the presence of a non - mammary primary site can be clinically ruled out or if an in situ component is histologically detected or both . however , findings of certain studies have revealed that ne - differentiated tumors of the breast present as dense round or irregular masses with spiculated or lobular margins on the mammogram . definitive diagnosis is made with core needle biopsy , allowing for the immunohistochemical evaluation of the specimen for the ne markers . although the use of pet for the evaluation of ne tumors has been limited , tumors with moderate or high proliferative activity can be identified by fdg pet . there are reports of fdg pet / ct in a case of neuroendocrine differentiated breast carcinoma with pleural metastases using indium-111 octreotide . there are case reports of synchronous metastases to the liver and pancreas from a primary nec of the breast . our case is the first demonstrates that 18f - fdg pet / ct provides the most significant additional information related to the accurate detection of primary nec of breast and bone metastasis and guiding treatment .
cases of primary neuroendocrine carcinoma ( nec ) of the breast have been reported , though rare . we report the case of a 45-year - old woman presented with jaundice and evaluated to have liver metastasis from neuroendocrine origin . she underwent whole body positron emission tomography / computed tomography , which showed left breast lesion and bone metastasis . fine - needle aspiration ( fna ) of breast revealed a nec . a diagnosis of a primary nec of the breast was rendered with hepatic and bone metastasis . she was treated with peptide receptor radionuclide therapy and is on follow - up .
antidepressants are widely used in the treatment of depression , which is the most common psychiatric disorder with an enormous burden globally . for a long time , tricyclic antidepressants ( tcas ) were the mainstay of drug treatment of depression . thromboembolic phenomena are also very common and need treatment with anticoagulants . of the oral anticoagulants , studies of drug interaction between warfarin and antidepressant drugs show that bleeding is the most common and also the most feared manifestation of the interaction . we report a patient who was started on warfarin for treatment of deep vein thrombosis ( dvt ) , who was receiving bupropion for depression . while the international normalized ratio ( inr ) had been stable at the desired level , bupropion was stopped and this resulted in an alarmingly high inr . higher values of inr than the desired levels for adequate anticoagulation indicate a high risk of bleeding . to our knowledge , there is no reported case so far , of drug interaction between bupropion and warfarin . a 55-year - old male was on treatment for dysthymic disorder ( dsm iv tr ) since 12 years . he was mostly on bupropion , in doses ranging from 150 mg/ day to 300 mg / day . he responded well to treatment , had few depressive symptoms intermittently , and his smoking had decreased to about 6 - 8 cigarettes a day . a year back , he developed dvt , at which time , the dose of bupropion was 300 mg / day . he was put on warfarin and the desired level of inr , that is , 2 - 3 , was achieved . at this time , the surgeon did not know that the patient was taking bupropion 300 mg / day . after a couple of weeks , when the patient informed him of this , bupropion was abruptly stopped . the patient was under observation , and the surgeon put him back on bupropion , 300 mg / day . it is among the 15 most prescribed drugs in the united states , with more than 1 million prescriptions / year and 75.7% of those on warfarin are elderly . considering that antidepressant drugs are so widely prescribed across all age groups need to study the drug interactions between warfarin and antidepressants is obvious . warfarin has a narrow therapeutic range , and for most patients on this drug the target inr usually is 2.0 - 3.0 . a higher target inr of 3.0 - 4.0 is recommended for patients with the mechanical prosthetic heart valves . above a level of 4.0 , the risk of bleeding is very high , especially intracranial hemorrhage . in our patient , the average dose of warfarin to achieve the desired level of inr ranges from 2 mg / day to 10 mg / day . the dose of warfarin can vary according to the individual sensitivity to it . in a sensitive individual if there is warfarin resistance , a dose in excess of 20 mg may be required . the list of drugs and other factors like diet and food supplements containing vitamin k , that affect the action of warfarin , is prodigious and expanding ; for example , acetaminophen , barbiturates , phenytoin , rifampin , phenylbutazone , sulfinpyrazone , metronidazole , disulfiram , allopurinol , cimetidine , and ingestion of large amounts of alcohol . there are four hypothetical mechanisms by which antidepressants or any other drugs can alter warfarin levels : by decreasing absorption ; by inhibiting cytochrome p2c9 ( cyp2c9 ) isoenzyme - causing an increase of inr ; by enzyme induction of cyp2c9 causing decrease in inr ; by increased protein binding of the drug , resulting in dislodging warfarin , which is highly bound to plasma albumin . while more than 40 hepatic enzymes have been identified , only six isoenzymes ( 1a2 , 3a4 , 2c9 , 2c19 , 2d6 , and 2e1 ) significantly affect the metabolic clearance of more than 90% of all drugs . for warfarin , the isoenzyme 2c9 is the most important , followed by 1a2 . in recent studies , specific serotonin reuptake inhibitors ( ssris ) have been studied the most and tcas the least in terms of interaction with warfarin . as mentioned earlier , about three - fourth of all patients on warfarin are older people . in this population , reported drug interactions between antidepressants and warfarin , of which bleeding is the commonest , are seen most with fluoxetine and fluoxamine . tcas , serotonin norepinephrine reuptake inhibitors venlafexine , duloxetine and desvenlafexine , and mirtazapine , are relatively , safe and have no clinically significant interaction with warfarin . this perhaps is due to their minimal or no inhibitory action on relevant cyp450 isoenzymes , 2c9 and 1a2 . there are no reports or empirical data suggesting that there is any risk in using the combination of bupropion and warfarin . it needs to be mentioned though that bupropion has been much less studied compared to ssris . this makes the bupropion - warfarin interaction , in this case , difficult to explain in the light of available evidence . it is possible that there is a hitherto unknown mechanism of action that causes an adverse interaction with warfarin .
depressive illness and thromboembolic disorders are both highly prevalent . warfarin is frequently combined with an antidepressant drug , the choice of which depends mainly on the risk of a hemorrhagic complication . patients requiring the warfarin are often in the older age group , where the newer antidepressants with a better safety profile are preferred over tricyclic antidepressants . we report herein , a patient who was on bupropion for depression , when he developed deep vein thrombosis high - risk . warfarin was started . while on this combination bupropion was abruptly stopped . this caused a more than two - fold elevation of international normalized ratio ( inr ) above the level , which is considered a high - risk for a hemorrhagic complication . inr reverted back to the desired level on reintroduction of bupropion . this indicates that a bupropion - warfarin combination should be used with the caution , though there has been no reported interaction so far .
carotid angioplasty and stenting ( cas ) has become an alternative option to carotid endarterectomy ( cea ) for treatment of carotid artery stenosis . the carotid revascularization endarterectomy versus stenting trial ( crest ) supported this alternative treatment option although the periprocedural stroke rate was higher in the stenting group.3 ) distal embolization of plaque fragments raises concerns regarding the safety of cas . whereas distal embolization resulting from cas usually occurs in the cerebral hemisphere , it sometimes develops as central or branch retinal artery occlusion ( crao or brao).8)11 - 13 ) however , there have been no reports of ophthalmic artery occlusion . a right internal carotid artery ( ica ) angiogram showed collateral circulation to the anterior cerebral artery ( aca ) and the middle cerebral artery ( mca ) via anterior communicating artery and leptomeningeal anastomoses ( fig . 1 ) . a left common carotid artery ( cca ) angiogram revealed left proximal cervical ica occlusion ( fig . after insertion of an 8 fr sheath , an 8 fr guiding catheter ( fas ; boston scientific , natick , ma , usa ) was introduced into the left cca . a 420 mm balloon ( ultra - soft sv ; boston scientific , natick , ma , usa ) was used for pre - dilatation followed by stent implantation using an 840 mm carotid stent ( acculink ; abbott vascular , abbott park , il , usa ) ( fig . , the thrombus in distal ica was removed using the penumbra aspiration system ( penumbra , inc . , alameda , ca , usa ) . a careful interview revealed that his visual acuity decreased soon after cas . upon examination , the patient 's visual acuity in the left eye was finger counting at a distance of 50 cm . fluorescein angiography revealed no abnormal finding except a mild delay ( 18 sec . ) in the arm to retina time . we checked the previous cerebral angiogram after cas and found ophthalmic artery occlusion ( fig . follow - up of the cerebral angiogram showed no occlusion in the left ophthalmic artery ( fig . cas is an effective alternative treatment for patients who are not good candidates for cea . however , it has several complications such as major or minor stroke , vasospasm , arterial dissection , residual stenosis and subarachnoid hemorrhage.1)2 ) distal arterial embolization is the most common complication , reported as high as 29 - 39.5%.4)6 ) most often , embolization occurs in the cerebral hemisphere . jaeger et al.4 ) reported that most embolic lesions were smaller than 5 mm and occurred in the distribution of the cortical branches of the mca and aca , in the parietal and frontal lobes , and in the upper and middle areas of the brain . these authors explained these embolic properties by the particles released during the procedure traveling in a straight path with the blood flow to the cortical branches of the mca and aca . crao is an ophthalmologic emergency , characterized by a sudden onset of devastating visual loss . crao is a rare event , encountered in less than 0.1% of the cases.7 ) its causes include carotid bifurcation disease , cardiac disease , retinal artery thrombosis , vascular spasm , and vasculitis . among these , embolism from the carotid bifurcation is the most common cause of crao and brao.7 ) unlike crao , which can be thromboembolic or embolic , the causes of brao are almost always embolic.8 ) several studies reported crao or brao after cas.8)11 - 13 ) samardzic et al.10 ) reported retinal circulation embolism after cardiac procedures . there are not many reports of the incidence of retinal artery embolization during or after cas . wilentz et al.12 ) reported that the incidence of retinal artery embolization during cas was 5% ( six out of 118 patients ) . a study using transcranial doppler measurements found a considerable number of embolic episodes during cas , with an average of 74 emboli per procedure.5 ) atherosclerosis of the ophthalmic artery can be another cause of crao.11 ) the third mechanism involves anastomosis between the ophthalmic artery and the external carotid artery.12)13 ) the ophthalmic artery has numerous extra - orbital branches , which can have extensive anastomoses with the ica . these anastomoses may cause retinal embolization when some types of embolic protection devices are used . several reports address risk factors of crao in patients with carotid stenosis . muller et al.9 ) reported that the patients with ulcerated , heterogeneous plaques have a higher risk of crao or brao . kimura et al.7 ) suspected that severe carotid stenosis , in addition to heterogenous plaques , is an important risk factor for retinal artery occlusion . we present a case of ophthalmic artery occlusion during emergency revascularization of an acutely occluded ica . during this procedure , interventionists can not detect crao if the patient does not complain about visual symptoms . unlike crao , ophthalmic artery occlusion can be easily detected and is treated simultaneously if the interventionists are aware of this condition . in our case , the ophthalmic artery occlusion was caused by a thrombus that filled the ica due to ica occlusion . therefore , we suggest that acute ica occlusion carries a higher risk of ophthalmic artery occlusion or crao than ica stenosis . ophthalmic artery occlusion or crao during cas are rare complications , but when they occur they are disastrous . endovascular neurosurgeons should closely observe ophthalmic arterial blood flow during cas , especially in the treatment of acute ica occlusion .
distal embolization resulting from carotid angioplasty and stenting ( cas ) occurs mainly in the cerebral hemisphere . we report a case of ophthalmic artery occlusion after carotid revascularization . a 75-year old man received emergency cas for cervical internal carotid artery occlusion . two months later , the patient was readmitted for decreased visual acuity . we found ophthalmic artery occlusion that was not noticed soon after cas . although ophthalmic artery occlusion after cas is rare , endovascular neurosurgeons should be aware of this potential complication .
epidemiologic data were obtained from a nationwide survey of 269 villages conducted from august through september 2005 . the country was divided into 4 ecologic zones on the basis of differences in elevation , temperature , and land cover type ( figure , panel a ) . the number of villages selected in each zone was proportional to the population in each zone . these data were combined with data from an additional 64 villages in known areas of malaria endemicity that were surveyed during 20002003 , to give data from a total of 333 villages . comparability was ensured by using the same sampling and parasitologic methods in each set of surveys . in each village , households were sampled along perpendicular transects . the survey team started from a central point and randomly selected the direction by spinning a bottle . along the transect , every household was selected and every household member invited to participate . a blood sample was collected from each person , and giemsa - stained thick and thin blood films were prepared and stored for microscopic examination for the presence of malaria parasites . a case - patient was defined as a person for whom malaria blood stage parasites were seen after examination of 100 fields . a ) prevalence of plasmodium vivax in afghanistan , according to a 2005 survey ( n = 269 ) and previous prevalence surveys conducted by healthnet - tpo , 20002003 ( n = 64 ) . lower - right inset shows ecologic zones in afghanistan according to differences in elevation , temperature , and land cover . white , high altitude rangeland ; light gray , desert ; dark gray , grassland ; black , irrigated / marshland . b ) predicted probability of p. vivax transmission ( prevalence > 0% ) in afghanistan , according to logistic regression model . the geographic locations of villages were recorded in the field by using a nondifferential global positioning system ( garmin international inc . , the village - level prevalence data were then included into the geographic information system ( gis ) ( arcview , version 3.2 , esri inc . , redlands , ca , usa ) . global satellite sensor - derived data at 88 km spatial resolution were obtained from the united states geological survey , distributed active archive center ( http://edcdaac.usgs.gov/1km/comp10d.asp ) and included the normalized difference vegetation index ( ndvi ) and land surface temperature . ndvi is an indicator of photosynthetic activity and is associated with saturation deficit and rainfall . the locations of rivers were downloaded from afghanistan information management system project 's website ( http://www.aims.org.af ) , and minimum distance between each village and rivers was calculated by using arcview . these environmental data were imported into arcview and linked by location to the parasitologic data . we used logistic regression analysis to investigate the relationship between environmental variables and the probability of transmission ( p. vivax prevalence > 0% ) . initial variables were selected by developing univariate models ; variables with wald p>0.2 were excluded from further analysis . colinearity was investigated between all possible pairs of potential predictor variables ; if any pair had a correlation coefficient > 0.9 , the member of the pair that was less likely to be biologically important was excluded . with the remaining variables , backward - stepwise logistic regression analysis was conducted by using wald p>0.1 as the exit criterion and p<0.05 as the entry criterion . entry of categorized predictor variables into the models was explored , but preliminary analysis indicated that linear forms were most significant . the final model was then cross - validated by using a jackknife procedure ( 5 ) . predicted occurrence was compared with observed occurrence by using receiver operating characteristic analysis . the statistic used for the comparison was the area under the curve , a plot of sensitivity versus 1 minus specificity ( 6 ) . the coefficients from the best - fit model were then applied to the predictor variables to generate a map of predicted probability of transmission . a total of 40,350 persons in 269 villages , ranging in age from 1 through 98 years , were examined . the overall prevalence of p. vivax was 0.49% , but infection levels varied considerably among areas of the country ( figure , panel a ) . prevalence of p. vivax was highest in faryab province in the north and in nangarhar and kunar provinces in the southeast part of the country . small foci of p. vivax were found in baghlan and badakhshan in the northeast and kandahar and hilmand in the south . no transmission occurred in villages at elevations > 2,000 m , likely because of variation in temperature . prevalence was highest in river valleys , and no transmission occurred in villages > 10 km from rivers . the odds ratios indicate that transmission probability was much higher in locations adjacent to perennial rivers . validation of the model using an observed prevalence threshold of > 0% gave an area under the curve of 0.67 ( 95% confidence interval 0.610.74 ) , which indicates a moderately good predictive performance of the model . the map of predicted probability of transmission is presented in the figure , panel b. * wald , 14.26 ; probability> , 0.0008 ; log pseudo - likelihood , 184.38873 ; pseudo r , 0.062 . spatial epidemiology aims to investigate spatial distributions of disease to identify geographic risk factors and populations at risk , which facilitates the rational implementation of control . although p. vivax malaria is a serious problem in afghanistan , only certain areas of the country are affected . our analysis shows that this distribution is determined by climatic and other geographic factors , which affect mosquito and plasmodium reproduction . the use of gis and remote sensing has enabled the first detailed description of the spatial variation of p. vivax malaria in afghanistan and will facilitate implementation of a rational strategy by allowing differential , stratified control mechanisms to be used and resource allocation to be managed more efficiently . afghanistan 's malaria control strategy consists mainly of social marketing of insecticide - treated nets , coupled with support for healthcare providers in the delivery of effective diagnosis and treatment . the national malaria strategic plan ( 2005 ) , which adopted the millennium development goals for malaria , calls for targeted interventions aimed at reducing the prevalence and effects of disease in those areas most at risk . our results demonstrate that gis and remote sensing are important tools for rapid mapping of disease patterns and for targeting limited control resources . further work is ongoing to determine areas at risk for p. falciparum transmission , the less prevalent but more dangerous parasite , and to devise a combined risk map .
plasmodium vivax is endemic to many areas of afghanistan . geographic analysis helped highlight areas of malaria risk and clarified ecologic risk factors for transmission . remote sensing enabled development of a risk map , thereby providing a valuable tool to help guide malaria control strategies .
juvenile xanthogranuloma ( jxg ) is a benign non - langerhans cell histiocytic proliferation of skin and soft tissue . it is a relatively rare cutaneous lesion with limited literature available on its cytological features . we report a case of jxg in a 12-year - old boy who presented with a chest wall nodule . ultimately , accurate cytologic diagnosis could be done and it was confirmed by subsequent histopathological examination . a 12-year - old boy presented with a gradually increasing skin nodule in the anterior chest wall [ figure 1 , inset ] . the lesion was present for more than 6 months and it was 2.5 cm in diameter , yellowish in color , soft in consistency and not fixed to the underlying structure . all routine investigations , including chest radiograph and serum lipid profile , were within normal limits . the patient was subjected to fine - needle aspiration cytology ( fnac ) , which revealed dispersed as well as clusters of foamy histiocytes admixed with touton giant cell having wreath - like nuclei surrounded by foamy cytoplasm and multinucleated foreign body - type giant cells in a background of lymphocytes , eosinophils and few polymorphs [ figure 1 ] . the cytologic differentials were jxg , cutaneous langerhans cell disease ( xanthomatous variety ) and xanthoma . location of the lesion and normal lipid profile along with typical cytological features ruled out possibility of xanthoma . meticulous examination of the histiocytes was performed and none of the cells showed folded , lobulated or kidney - shaped nucleus typically found in langerhans cell disease . fnac smears showing clusters of foamy histiocytes admixed with touton giant cells in a background of inflammatory cells ( h and e , 400 ) ; inset : shows skin nodule in the anterior chest wall the lesion was excised ; on gross examination , it showed a 2.5 2 cm grayish yellow soft mass . subsequent histopathological examination confirmed our diagnosis , which revealed thinned - out epidermis and a well - circumscribed lesion in the dermis composed of foamy histiocytes , touton giant cells , foreign body giant cells , lymphocyte , eosinophils and neutrophils . jxg is a normolipemic , non - langerhans cell histiocytosis characterized by yellowish cutaneous nodules that usually appear in early infancy and childhood , but may present even in adulthood . our case was a 12-year - old boy who presented with a non - tender nodule over chest wall . the most common site is the head and neck region with solitary lesions in 60 to 82% of the patients that usually regress spontaneously within 3 to 6 years , leaving a small atrophic scar . the clinical differential diagnosis includes spitz nevi , mastocytomas and dermatofibromas , which present more academic interest than practical diagnostic difficulty . jxg has characteristic cytologic features which include presence of numerous finely vacuolated histiocytes , eosinophils , multinucleated giant cells and scattered touton giant cells . these histiocytes are cd 68 positive and s100 and cd 1a negative , unlike those of langerhans cell disease . according to li et al . , jxg should be considered in the differential diagnosis of any histiocytic / fibrohistiocytic soft tissue lesion of childhood and this entity can be diagnosed by its fnac and immunocytochemistry findings . jxg can be differentiated from xanthoma in fnac by the distribution of lesion and absence of lipid abnormalities . although stain for cd 68 , cd1a and s100 may be necessary for differentiating two conditions , meticulous examination of cells , particularly nuclear groove , may help in proper diagnosis of the condition . as the typical histologic picture of jxg is unmistakable we conclude that jxg has characteristic diagnostic cytologic features and this case has been presented to highlight the importance of fnac in the preoperative diagnosis of this rare cutaneous lesion .
juvenile xanthogranuloma ( jxg ) is a rare cutaneous lesion with paucity of literature on its cytological features . we report one such case which on fine - needle aspiration cytology yielded a mixed population of foamy histiocytes , multinucleated giant cells and variable admixture of lymphocytes and eosinophils causing diagnostic dilemma with other differentials , especially langerhans cell histiocytosis . however , clinical correlation followed by histopathology confirmed the diagnosis . hence , jxg has characteristic and diagnostic cytologic features .
a 67-year - old female patient presented with neck pain and stiffness for the past 4 months . the neck pain radiated to both upper limbs and was associated with tingling and numbness . the symptoms had increased in intensity over the past month . the patient had constitutional symptoms , including loss of appetite , loss of weight ( 5 kg over a month ) , evening rise of temperature , and night cries . she did not have any history of trauma or coexisting medical illnesses . on physical examination x - rays of cervical spine showed a kyphotic deformity , destruction of c6 vertebral body with only a thin part of inferior end plate of c6 remaining , and the associated posterolisthesis of c5 over c6 ( fig . blood reports showed lymphocytosis with raised erythrocyte sedimentation rate and c - reactive protein values . a magnetic resonance imaging ( mri ) showed infective spondylitis of c5/c6 with a pre / paraspinal abscess from c2 to t2 which indented the pharyngeal wall ( fig . 2a ) . a computed tomography scan showed posterolisthesis of c5 over c6 with bony spinal canal stenosis , anterior wedging of c6 , erosion of the superior end plate of c5 , and prevertebral soft tissue from c3 to c6 ( fig . although the patient was intact neurologically , there was extensive bone destruction and high grade spondylolisthesis ; thus , an operative line of management was preferred . since operative management was likely and the mri was highly suggestive of tuberculous spondylodiscitis , a preoperative biopsy was not considered . the surgery was performed under general anesthesia in the supine position through the right anterior approach . c5 and c6 corpectomy using an anterior cage and cervical plating from c4 to c7 was performed ( fig . after 2 years , an mri was performed , which showed that the disease had been healed ( fig . , the patient was symptomatically better and did not have any recurrence of the disease . the thoracolumbar spine is the most commonly affected.3 ) the cervical spine is a rare site for tuberculous infections . clinically , pain is the most common symptom in cervical spine tuberculosis , with varying degrees of kyphosis and instability.1 ) constitutional symptoms are non - specific and do not lead to the diagnosis.8 ) tuberculosis of the lower cervical spine presents differently in children and adults . in children , it is of an aggressive nature with diffuse involvement and abscess formation . healing results in significant kyphosis.19 ) in adults , it is more localized and involves a single vertebral body with less pus formation . the resulting kyphosis in adults is not as severe because the articular surfaces are spared.19 ) spondylolisthesis is a rare presentation in of any cervical spine pathology . it is most commonly due to trauma and degeneration.2 ) although pathological spondylolisthesis in the cervical spine have been reported in cases of aneurysmal bone cyst , neurofibromatosis , and skeletal fluorosis , 2 ) spondylolisthesis as a presentation of tuberculous has not been reported.37 ) in our case , there was a pathological posterolisthesis subsequent to the tuberculosis . table 1 shows the reports of concomitant tuberculosis with spondylolisthesis described in the literature . in the case described by ratliff,5 ) anterolisthesis was present before the vertebral tuberculous affection . nissen - lie ( as described by ratliff5 ) ) in his report , postulated that , since the disease involved posterior elements of vertebra , it lead to the development of a stress lesion , causing anterolisthesis . chadha et al.3 ) described three cases , in which one case had preexistent anterolisthesis and other two had tuberculosis of the posterior elements causing the anterolisthesis . kirkman and sridhar7 ) described a case of grade 4 posterolisthesis due to a vertebral tuberculous affection in a 12-year - old girl . our case did not have any prior complaints of neck pain or radiculitis , so we feel that the destruction of the vertebral body due to the tuberculous process may have led to the instability and subsequent posterolisthesis . treatment of cervical spine tuberculosis should be undertaken early to prevent neurological deficits and deformities.10 ) anterior surgery remains the mainstay treatment of cervical tuberculosis with instability , allowing for the debridement and evacuation of an abscess with stabilization of the anterior collapsed segment.810 ) posterior surgery is rarely used in lower cervical tuberculosis unless there is a posterior abscess.9 ) our case showed destruction of the vertebral body with posterolisthesis and a prevertebral abscess , so we performed an anterior decompression and stabilization .
spondylolisthesis coexisting with tuberculosis is rarely reported . there is a controversy whether spondylolisthesis coexists or precedes tuberculosis . few cases of pathological spondylolisthesis secondary to tuberculous spondylodiscitis have been reported in the lumbar and lumbosacral spine . all cases in the literature presented as anterolisthesis , except one which presented as posterolisthesis of lumbar spine . spondylolisthesis in the cervical spine is mainly degenerative and traumatic . spondylolisthesis due to tuberculosis is not reported in the lower cervical spine . the exact mechanism of such an occurrence of spondylolisthesis with tuberculosis is sparsely reported in the literature and inadequately understood . we report a rare case of high grade pathological posterolisthesis of the lower cervical spine due to tubercular spondylodiscitis in a 67-year - old woman managed surgically with a three - year follow - up period . this case highlights the varied and complex presentation of tuberculosis of the lower cervical spine and gives insight into its pathogenesis , diagnosis , and management .
individuals with diabetes do not realize the consequences of poorly controlled blood sugars until injury occur [ 14 ] . the diabetes control and complications trial ( dcct ) showed that glycemic control delays the onset of microvascular and macrovascular complications . glucose monitoring allows individuals with diabetes the opportunity to establish targets they should achieve for glucose control . in the united kingdom prospective diabetes study ( ukpds ) , patients with new onset type 2 diabetes showed benefits in achieving glucose control evidenced by reductions in microvascular and neuropathic complications . self monitoring glucose is an important tool used to provide education and self management instructions to achieve optimal glucose levels . there are multiple barriers that exist between the transfer and application of theoretical knowledge to patients . for example , recent studies have focused on specific aspects of diabetes education in patients with cognitive impairment to a specific education program for insulin management and suggest that education should focus on specific needs expressed by the older population [ 1215 ] . diabetes education is a key aspect in diabetes management and is a collaborative process allowing the patient with diabetes to receive knowledge and skills needed to change behavior and successfully manage the disease . a formal diabetes education program is a comprehensive program that is designed to enrich a patient s knowledge in diabetes management skills to problem solve unanticipated situations of hyperglycemia and hypoglycemia and sick - days , reduce risks associated with diabetes , and help with the healthy coping of day to day challenges of living with the disease [ 1820 ] . goals of diabetes therapy are to achieve optimal glucose and lipid values , improve quality of life and reduce health care costs . the study looked at 67 subjects with type 2 diabetes treated with diet alone , 350 subjects treated with tablets , 155 subjects treated with insulin , and 228 subjects treated with both tablets and insulin . subjects were followed in a shared medical appointment ( sma ) as part of a diabetes self management education ( dsme ) program in the primary care setting in three intervals over 28 months . exclusion criteria include subjects with limited physical abilities who were unable to participate in group diabetes self management education . the study utilized the facility s computerized patient record system ( cprs ) upon entry and again at the third visit to collect hemoglobin hga1c and frequency of reported glucose tests . specific instructions for home glucose monitoring are given at routine office visits and at dsme visits . all subjects with a diagnosis of diabetes are given precise instructions to monitor glucose and interpret results . formal dsme is provided by interdisciplinary staff instructors and re - enforced in three group visits before discharge to the primary physician . the curriculum content used is structured according to the national standards for diabetes self management education , tailored to match individual s needs and adapted as necessary for age , type of diabetes , cultural influences , health literacy and other co - morbidities . spss version 19 is used to regress the dependent variable , drop in hemoglobin hga1c level , on the independent categorical variables through linear regression . using the enter method of linear regression the full model including medication type , medication level , gender , and family support indicates overall model significance ( p<.01 ) and r=.056 . the medication levels , insulin and tablet plus insulin produce positive significant coefficients ( p<.05 ) . the positive coefficient indicates that both variables produce a mean difference value greater than that of no medication used , lowering the hga1c level by a larger amount . under the glucose monitoring category , monitoring 47 times per week produces a positive significant coefficient ( p<.05 ) . a second model was estimated using the enter method of linear regression without gender and family support indicating overall model significance ( p<.01 ) and r=.054 , finding the same conclusions as previously stated . a third model using the enter method of linear regression was used to assess the significance of medication level . the combined model finds significance ( p<.05 ) for the computed variable combining insulin and tablet plus insulin . further analysis comparing the combined model to the reduced model indicates that taking both the tablet and insulin is more effective at lowering hga1c levels than taking insulin alone at the 0.10 level of significance . as a matter of fact , the raw data indicates that the percentage lowering of hga1c of tablet plus insulin is 15.64% as the mean hga1c at the first visit was 9.35 compared to 7.89 at the later visit whereas the percentage lowering of hga1c for insulin was 12.24% as the mean hga1c at the first visit was 9.37 compared to 8.23 at the later visit . we tested the hypothesis that achieving optimal hga1c results is directly correlated to the frequency of self monitoring in subjects with type 2 diabetes . we found that at the time of diagnosis , many patients with type 2 diabetes were motivated to test frequently as was prescribed , and problem solve towards successful outcome [ 2426 ] . the national standards for diabetes self management education guide was used to formulate the education and match individual s needs , and adapt as necessary for age , the type of diabetes , cultural influences , health literacy and other co - morbidities . utilization of print , audio , and models to deliver the education was also used . however , after 5mos , many subjects were less inclined to test glucoses , report results , and maintain focus . when this behavior was observed , our intervention was to engage each subject individually by offering 1:1 appointments with dsme staff to discuss obstacles . we found that coaching subjects towards glucose monitoring and problem solving lead to preventable hospitalizations . furthermore , the present study has shown that empowering the subject with diabetes to practice self care management is imperative to reaching hga1c targets . however , the subject with the diagnosis , ultimately decides the glucose monitoring frequency needed to successfully achieve glucose targets . tables 13 represent each of the estimated models , the un - standardized coefficients , corresponding significance levels , and the ninety five percent confidence intervals . thus , the following conclusions were made : ( 1 ) frequency of self monitoring should be based on individualized goals and willingness to participate , as seen in this study the best frequency for glucose monitoring is between 4 and 7 times per week ; ( 2 ) both insulin alone and tablet plus insulin levels of medication are effective at lowering hga1c levels in patients , however , using the tablet and insulin combined produced lower hga1c levels than using insulin alone ; ( 3 ) family support and gender have no effect on lowering hga1c levels in patients .
summarybackgroundthe primary objective of our study is to compare hga1c to self monitoring frequency in diabetes subjects . a secondary objective is to evaluate the influence of family support and gender on glucose monitoring frequency and hga1c.material/methodswe studied the glucose monitoring frequency and hga1c outcome of 67 subjects treated with diet alone , 350 subjects treated with tablets , 155 subjects treated with insulin , and 228 subjects treated with both tablets and insulin.resultseleven percent of the subjects monitoring 47 times per week produced a positive significant coefficient ( p<.05 ) . self monitoring less than 4 times per week showed no statistical significance and self monitoring more than 8 times per week showed no statistical significance . forty - eight percent of subjects treating with insulin alone and tablet plus insulin produced positive significant coefficients ( p<.05 ) . the percentage lowering of hga1c of tablet plus insulin is 15.64% as the mean hga1c at the first visit was 9.35 compared to 7.89 . the percentage lowering of hga1c for insulin alone was 12.24% as the mean hga1c at the first visit was 9.37 compared to 8.23 at the later visit.conclusionswe conclude that ( 1 ) frequency of self monitoring should be based on individualized goals and willingness to participate , ( 2 ) both insulin alone and tablet plus insulin levels of medication are effective at lowering hga1c levels ; however , using the tablet and insulin combined produced lower hga1c levels than using insulin alone ; ( 3 ) family support and gender have no effect on glucose monitoring frequency and lowering hga1c levels .
baclofen is a gamma - aminobutyric acid type b ( gaba - b ) receptor agonist used primarily in the treatment of spasticity and in recent years it has gained popularity as an anti - craving medicine for the treatment of alcohol dependence syndrome.1,2 ) the anti - craving and anti - reward effects of baclofen are attributed to its agonist effect on gaba - b receptors in the ventral tegmental area , which are reported to control the activity of mesolimbic dopamine neurons , one of the major pathways in the regulation of the reinforcing properties of drugs of addiction.3,4 ) baclofen is found to be effective and well tolerated by patients with hepatic impairment also . a provisional evidence of dose - response effect has been demosntarted by addolorato et al.5 ) with 20 mg three times a day ( tds ) dose found to be more effective than 10 mg tds in suppression of carving for alcohol . there have been individual case reports of using baclofen in a much higher dose , i.e. 270 mg / day for suppressing the craving for alcohol . however there is no systematic study on the use of higher doses.6,7 ) however recently published case reports have shown several serious safety concerns which includes drowsiness , seizure vulnerability , risk for triggering specific pharmacologic withdrawal syndrome and intoxication.811 ) baclofen has previously been reported for inducing behavioural disinhibition and manic like symptoms.1,2 ) due to its mood elevating property its abuse potentiality can not be ruled out . here we are presenting a case of baclofen abuse in a patient with alcohol dependence with comorbid major depressive disorder who reported to have used baclofen in order to overcome his depression . a 36 years old hindu , literate married male patient from urban background took treatment for alcohol dependence and recovered well . he was better but often on , he used to drink alcohol along with his medication baclofen . on enquiry he was found to be having moderate depression with sleep problem for last 1 year . so he was prescribed with quetiapine 100 mg daily along with mirtazepine 15 mg , escitalopram 20 mg , lorazepam 2 mg , and baclofen 20 mg daily . so he discontinued coming for checkup . on the next visit when he came after seven months he revealed that he was only taking mirtazepine 15 mg , lorazepam 2 mg and whenever he takes baclofen 80 mg daily along with those medication he feels much better . his family member also gave history that whenever he takes baclofen 80 mg and sometimes more than that dose his confidence increases becomes irritable and angry . but his work function remains same without deterioration . on understanding his change of behavior after abusing baclofen 80 mg they stopped baclofen but interestingly during his hypomanic phase he never took alcohol which is most probably for taking baclofen which is an anti - craving drug for alcohol . there have been many case reports depicting manic or hypomanic episode resulting from use of baclofen.1,2 ) the mood elevating property of baclofen has not been properly known . however it is postulated that mood altering property of baclofen may be due to its gaba - b receptor agonist action which results(a ) acceleration of noradrenaline turnover by changing post synaptic receptor density and ( b ) up regulation of serotonin ( 5ht2 ) receptor thereby leading to diminished liberation of serotonin.12 ) to our knowledge , abusing baclofen due to it mood elevating property has not been reported till date in literature . our patient who did not show any improvement on antidepressant medicines reported to have felt better in terms of his mood state whenever he takes baclofen . the patient may be regarded as a case of resistant depression as per the criteria . he reported that whenever he takes higher doses of baclofen his confidence increases becomes irritable and angry . hypomania / mania symptom checklist ( hcl-32 ) by angst et al.13 ) was applied and the score was found to be 12 . naranjo algorithm14 ) was also applied to ascertain the occurrence of such hypomanic symptoms in the patients and the results are shown in table 1.15 ) in our case the score was 7 which means hypomania is probably related to baclofen use and because of it he has started abusing baclofen . baclofen has in recent time gained popularity as an anti - craving agent due to its safety profile in hepatic compromised patients . however , one must be very cautious in using it because of its abuse potential due to its mood elevating property . one possible positive outcome of this report may be that it may be a potential area of research to find out whether baclofen can be used in the treatment of resistant depression .
baclofen is a gamma - aminobutyric acid type b receptor agonist used as an anti - craving agent for treatment of alcohol dependence . it has gained popularity in the recent times because it is well tolerated even in patients with hepatic impairments . herein we are summarizing the latest literature about baclofen induced hypomania and are reporting a case of baclofen abuse because of its mood elevating property in a patient of alcohol dependence with comorbid major depressive disorder . literature review and case study of a 36-year - old male with alcohol dependence with comorbid major depressive disorder was prescribed with tablet baclofen as an anti - craving agent along with antidepressant medicines . the patients who did not improve with conventional antidepressant therapy started feeling better in terms of his mood symptoms on taking tablet baclofen . owing to the mood elevating property he started abusing baclofen . despite its safety profile in hepatic impairment , one must be very cautious in prescribing baclofen because of its mood altering property which may account for its abuse potentiality .
the annual incidence of pg in the united states is approximately 1 case per 100,000 people . the diagnosis of genital pg is one of exclusion , with penile lesions having a substantial differential diagnosis including infections like syphilis , herpes simplex , mycobacterial ulceration , amoebiasis and non - infectious causes of genital ulceration like squamous cell carcinoma , cutaneous crohn 's disease , ulcerating sarcoidosis . pg should be suspected in any patient with ulcerative penile lesion(s ) who fails to respond to adequate antibacterial or antiviral therapy and who has a negative or non - confirmatory diagnostic evaluation for infectious or other inflammatory conditions . there are very few cases of penile pg reported in patients with hiv / aids . a 24- year- old , unmarried male plhiv , presented with painless genital ulcer gradually increasing in size for 1518 months . the patient had a history of paid , unprotected heterosexual exposure 6 months before the onset of the lesion . on examination , the lesion had well - defined edges , irregular border , verrucous surface and a granulomatous base with serous discharge [ figure 1 ] inguinal lymphadenopathy was absent.the patient was taking anti - tubercular therapy ( att ) since 4 months for scrofuloderma over neck . complete blood count was 7200/mm , erythrocyte sedimentation rate was 25 after 1 h and 40 after 2 h. chest radiograph was normal . tzanck smear and gram stain prepared from the ulcer were negative . local tissue culture was also negative.histopathologic examination from the ulcer edge showed nonspecific inflammation with eosinophilic infiltrate [ figure 2 ] biopsy was repeated three times to rule out sexually transmitted disease and malignancy . single , well - defined erosion with granulation tissue and elevated edges on the undersurface of shaft of penis histopathology suggestive of nonspecific chronic inflammation and ulceration of squamous mucosa , 4 low - power view the patient is s. hiv-1 reactive . patient 's cd4 progression is 204/mm3 on 13/6/2008 , 582 on 23/12/2008 , 354 on 30/6/2009 , 216 on 18/12/2009 , 257 on 14/6/2010 , 358 on 18/12/2010 . he was started on efv - based regimen as he was on concurrent att chemotherapy for scrofuloderma . doxycycline 100 mg twice a day for 4 weeks but lesion did not show any improvement . the lesion did not show any improvement and it continued to increase in the size . initial size of lesion was 21 cm in size which progressed to 610 cm in size over 10 months [ figure 3 ] . prednisolone 30 mg in the mornings with milk for 14 days and then tapered 5 mg every week . the lesion initially showed improvement but lesions soon started increasing in size on tapering the doses [ figure 4 ] . finally , diagnosis of pg was made and the patient was asked to apply imiquimod 5% cream once a day for 4 weeks . the lesion started clearing within first 2 weeks and it was completely healed after 3 weeks of application and the patient is in remission since last 6 months without any medication [ figure 5 ] . lesion increased in size upto 8 10 cm failing to respond to therapies for conventional sexually transmitted diseases partial response after therapy with tab . prednisolone complete epithelization after 4 week topical application of 5% imiquimod in this case , it is possible that repeated biopsy elicited the pathergic response . differential diagnosis of genital erosion in plhiv is enormous but possibility of pg should always be kept in mind to avoid unnecessary surgical trauma and debridement . pg is a rare , non - infectious neutrophilic dermatosis commonly associated with underlying systemic disease most often affecting the lower extremities but may involve the face , neck , scrotum or penis.- pathogenesis of pg is not fully understood , an immune - mediated process is thought to have an important role . penile pg may occur following local trauma , such as urological surgery or treatment for cancer , or complicate ulcerative colitis or chronic lymphocytic leukaemia , or it may be idiopathic . diagnosis is based on typical clinical features and exclusion of other cutaneous ulcerating diseases , as described in several reviews.[69 ] although the histopathological findings of pg are often variable and non - specific , they can be useful in excluding other possible aetiologies . the extension of lesions in response to trauma or surgical debridement , termed pathergy , is a hallmark of pg . we present a case of penile pg as a reminder that , although pg of the penis is rare , a progressive , non - healing painful penile lesion with negative laboratory and histopathological evaluations should prompt the consideration of this entity . penile pg being present in plhiv poses two important challenges:(1 ) differential diagnosis of genital ulceration in plhiv is wide , including various sexually transmitted infections , tropical ulcers , mycobacterial , amoebic , deep fungal , aphae , drug - induced and malignancy . complete diagnostic workup to exclude all the above possible causes is a must in order to establish the diagnosis . ( 2 ) management of penile pg is also difficult in the plhiv as these patients are more likely to develop potential adverse effects of the immunosuppressive agents . topical and systemic corticosteroids only gave partial control in this case and patient being plhiv long term use of corticosteroids and other immunosuppressive agents is prohibited . thus , topical imiquimod 5% cream with its immunomodulating properties could be a feasible choice for management of such patients . choice of treatment generally depends on disease severity as well as on the presence of associated disease . for early or mild lesions , topical therapy may be sufficient . for more severe disease , or for pg resistant to topical therapy ,
pyoderma gangrenosum(pg ) is a rare ulcerating inflammatory neutrophilic dermatosis . genital involvement has been rarely reported . we report such a case of 24- year- old , male patient living with hiv / aids(plhiv ) who presented with progressive genital ulceration , not responding to oral antibiotics and aciclovir , gradually increasing in size over 15 - 18 months . repeated biopsies showed acute on chronic inflammation . the lesion partially responded to oral and topical corticosteroids but soon increased in size after tapering the dosage of the steroids.then patient was given imiquimod 5% cream to be applied over the lesion once daily for 2 - 4 weeks . lesion cleared completely in 4 weeks and is in remission since last 6 months . the case report highlights the successful use of topical imiquimod 5% cream in the treatment of penile pg .
ovarian hyperstimulation syndrome ( ohss ) is a life - threatening condition consequent to ovulation induction in assisted reproductive technology ( art ) cycles . although the majority of ohss cases are iatrogenic , spontaneous ohss ( sohss ) , a very rare event , has also been reported . although sohss almost always occurs in conjunction with pregnancy , the actual incidence still remains obscure . sohss usually develops between 8 and 14 weeks of gestational age , possibly due to a mutated follicular stimulating hormone ( fsh ) receptor , which shows abnormal sensitivity to human chorionic gonadotropin ( hcg ) or due to wild type of fsh with abnormally high levels of hcg . ovarian torsion , a major complication of ohss , is estimated to occur in 12 - 25% of pregnant women . we report a very rare case of bilateral ovarian torsion complicating sohss in a singleton pregnancy , which was successfully managed by laparoscopic detorsion . the present case report is about a 26-year - old primigravida at 9 weeks gestation presented with the complaints of severe abdominal pain . she gave a history of pain during ovulation since several years and had undergone laparoscopic surgery twice in the past : for persistent left ovarian cyst in 1999 and for bilateral ovarian cysts in 2000 . she was diagnosed with recurrent hemorrhagic cysts following repeated admissions to hospital for abdominal pain and was managed conservatively . subsequently , the patient had a natural conception but developed severe abdominal pain due to sohss . ultrasound was suggestive of 7 weeks intrauterine gestation with bilateral enlarged ovaries [ figure 1a - c ] . the right ovary with retained vascularity , measured 81 mm 42 mm 64 mm and the left ovary had a corpus luteal cyst measuring 23 mm 32 mm , with increased vascularity . the ischemic - hemorrhagic appearing right ovary was enlarged and measured 7 - 8 cm with three twists [ figure 2 ] . the left ovary measured 3 - 4 cm and had undergone one twist [ figure 3 ] . bilateral detorsion was performed [ figure 4 ] along with ovarian puncture to aid size reduction . the post - operative period was uneventful and the patient was discharged on the 3 post - operative day . follow - up with an ultrasound showed successful continuation of the intrauterine pregnancy and normal ovarian blood flow . ( c ) ultrasound image showing enlarged left ovary with cysts laparoscopic view of right ischemic torsed ovary laparoscopic view of left ovary with single twist laparoscopic view of left ovary undergoing detorsion and the detorsed right ovary ovarian accidents , such as torsion and rupture , which complicate ohss are known but rare events . a search of articles from 1857 to 2013 in the pubmed database was conducted to find the number of reported cases with sohss complicated by bilateral ovarian torsion in singleton pregnancies . to the best of our knowledge , this is the first case with bilateral ovarian torsion complicating sohss in an early singleton pregnancy . so far , even though torsion of the right adnexa is more frequent , in the current case , the left ovarian torsion may be attributed to the presence of a corpus luteal cyst , the most common risk factor for torsion during the first trimester of pregnancy . an assessment of the extent of ovarian ischemia is crucial in deciding the mode of management , which is either untwisting for ischemic adnexa or adnexectomy if gangrene has developed . the macroscopic ischemic - hemorrhagic appearance of the affected ovary simulating gangrene could be attributed to venous and lymphatic stasis . in the current case , both the ovaries were twisted and enlarged with several hemorrhagic spots giving a gangrenous appearance . on performing detorsion and ovariopexy of the ovaries , significant color change was noted . this implies impeded blood supply from either uterine or ovarian arteries , but not complete cessation of the same . in addition , a study by oelsner et al . , which involved a retrospective analysis of 102 patients managed with surgical interventions for adnexal torsion , reported that 91.3% of patients with bluish - black ovary regained normal function . none of the patients developed pelvic or systemic thrombo - embolism , the risk presumed to occur following detorsion of the adnexa . oelsner et al . concluded that laparoscopic detorsion with ovarian salvage helps regain normal blood flow and at the same time retain normal ovarian function and reserve . the study reported that restoration of normal ovarian function was almost similar with laparoscopy ( 93.3% ) and laparotomy ( 90.6% ) in terms of macroscopic appearance , follicular development and fertilization of eggs retrieved from the detorsed ovary . however , laparoscopy was preferred owing to the reduced risk of adhesions , less post - operative pain , decreased use of analgesics , shorter hospital stay and early restoration of normal activity . in summary , this case highlights the importance and appropriateness of early laparoscopic detorsion of bilateral ovarian torsion complicating ohss in early pregnancy . although sohss is a very rare event , it may be a life - threatening complication if associated with adnexal torsion and therefore necessitates a clear understanding of the syndrome .
a 26-year - old primigravida with a singleton pregnancy of 9 weeks gestation presented with severe lower abdominal pain , following spontaneous hyperstimulation of the ovaries in a natural conception . emergency laparoscopy was done and bilateral ovarian torsion with retained vascularity was noted . bilateral detorsion with ovarian puncture and ovariopexy was performed . a review of international literature suggests that this is the first case reported with bilateral ovarian torsion following spontaneous ovarian hyperstimulation syndrome in a singleton pregnancy .
rheumatoid arthritis ( ra ) is a chronic , progressive , inflammatory joint disorder , the primary treatment for which consists of medical regimens aimed at controlling the synovial inflammation of the joint . its intention is to destroy the inflamed synovium , in the expectation that the regenerated synovium will be disease - free and the symptoms will thereby be alleviated . lutetium-177 ( lu-177 ) is considered to be a promising radionuclide for use in rs of joints owing to its favorable decay characteristics . hydroxyapatite particles ( ha ) are regarded as one of the most suitable carriers for applications in rs . we describe the first case of application of lu-177 ha in the treatment of the elbow joint synovitis in rheumatoid arthritis . a 35-year - old female patient was presented with a right elbow joint pain and swelling for a duration of six months . the patient 's blood investigation revealed elevated c - reactive protein ( 60 mg / ml ) and diagnosed to have rheumatoid arthritis . perfusion phase images [ figure 1a ] demonstrates increased perfusion in the right elbow joint and blood pool images [ figure 1b ] shows increased blood pooling consistent with synovitis . whole body images [ figure 1c ] showed increased uptake in the right elbow joint . the patient was then administered intraarticular injection of lu-177 ha and images of the elbow joint was acquired in a dual head gamma camera ( siemens symbia true point ) in high energy general purpose collimator with multiple windows of 113 kev and 208 kev . lu 177 ha images [ figure 2 ] showed good distribution of tracer in the elbow joint . perfusion ( a ) and anterior and posterior blood pool ( b ) images demonstrates increased perfusion and blood pool concentration in the right elbow joint consistent with synovitis . whole body images ( c ) showing increased tracer uptake in the right elbow joint ( arrow ) lu-177 ha images of the elbow joint showing good tracer distribution in the right elbow joint radiation synovectomy ( rs ) is a local intraarticular injection of radionuclides in colloidal form . rs may relieve synovitis , joint pain , knee flexibility and joint effusion in about 60%-80% of the cases . , in 1952 , the technique has been applied for over 50 years in the treatment of resistant synovitis in individual joints after the failure of long - term systemic pharmacotherapy and intra - articular steroid injections . radioisotopes with emission of radiation have the capacity to diminish the inflammatory process and ablate the inflamed synovial membrane ( pannus ) with subsequent fibrosis . three radionuclides are currently in use : yttrium-90 ( y-90 silicate / citrate ) , rhenium-186 ( re-186 sulfide ) , and erbium-169 ( er-169 citrate ) , which have been indicated for large , medium , and small joints , respectively . lu-177 has emerged as a pivotal radionuclide in the field of in vivo radionuclide therapy could be considered owing to its suitable nuclear decay characteristics [ t = 6.65 d , e ( max ) = 497 kev , e = 113 kev ( 6.4% ) , 208 kev ( 11% ) ] . the presence of gamma photons of imagable energy with low abundance provides the additional benefit of carrying out simultaneous scintigraphy relatively longer half - life of the radioisotope provides logistic advantage during its transportation to distant nuclear medicine centers . therefore , rs agents based on lu-177 will be economically more viable compared to most of the other agents so far in clinical use . an ideal agent for rsv would be one which the radionuclide is irreversibly attached to pre - formed particles of appropriate size . hydroxyapatite , one of the preferred particulates for use in rs applications , is a naturally occurring mineral form of calcium apatite , mainly found in bone and teeth . lu-177 ha can be potentially used as an ideal agent for radiosynovectomy of the joints . the choice of this radio - pharmaceutical was made based on the optimal penetration of beta radiation in the synovia . the beta radiation , because of its short penetration distance , reaches only structures in the intermediate vicinity of the joint cavity . as per the limited number of stability and animal studies performed by the scientists it has been observed that lu-177 ha is highly stable and leakage from the synovial site is negligible . leakage has been particularly difficult to quantify where the isotope used was y-90 or p-32 , radioisotopes which are both pure beta emitters with no accompanying gamma emissions that might be used to quantify biodistribution and dosimetry . rs treatment was performed with precise intra - articular injection of a typical dose of 333 46 mbq under sterile conditions . before joint puncture , local anesthesia was administered with 2% lidocaine - hydrochloride prior to the injection of lu-177 ha . depomedrol ( 40 mg in 1 ml ) was injected into the joint in order to reduce the risk of acute radiation induced synovitis and to avoid skin radiation necrosis . subsequently , 333 mbq ( range : 259 - 370 mbq ) of lu-177 ha particles dispersed in 1 ml of sterile , apyrogenic normal saline was administered intra - articularly and then the needle was flushed with 2 - 4 ml of normal saline . in case of any uncertainty about the exact location of the tip of the needle , arthrography was performed to check the correct location . imaging of activity distribution with a dual head gamma camera showed appropriate homogeneously intra - articular distribution of the radionuclide within the joint space . our case describes the first application of lu-177 ha in the elbow joint synovitis and image of lu-177 ha .
rheumatoid arthritis is a chronic disease that is mainly characterized by asymmetric erosive synovitis , particularly affecting the peripheral joints . radiation synovectomy or radiosynovectomy , also known as radiosynoviorthesis was first described in 1950 's as a adjuvant treatment for rheumatoid arthritis . radiosynovectomy is based on the irradiation of the joint synovium by the intra - articular administration of various -emitting radiopharmaceuticals . lu-177 has presence of gamma photons of imagable energy with low abundance which provides the additional benefit of carrying out simultaneous scintigraphy . we describe the first case report of use of lu-177 hydroxylapatite particulates in a 35-year - old female patient who was presented with elbow joint synovitis due to rheumatoid arthritis .
most cases occur in immunosuppressed hosts , including patients with hiv / aids , patients receiving immunosuppressing drugs , and solid organ transplant recipients [ 2 , 3 ] . primary cryptococcosis of the skin is very rare , and cryptococcal skin disease should therefore be interpreted as a sign of systemic cryptococcal infection . cryptococcal skin disease can manifest itself in a variety of ways , each of which is uncharacteristic for cryptococcus sp . [ 4 , 5 ] . we describe a case of disseminated cryptococcal infection in a liver transplantation patient with a clinical presentation of bullous erysipelas in the lower limb that rapidly culminated to a fatal outcome . a 50-year - old male underwent liver transplantation in october 2010 due to cryptogenic cirrhosis . after 13 months , he started to present clinical and laboratory signs consistent with liver rejection , such as fever , abdominal pain , jaundice and an increase in hepatic enzymes . after 7 days of hospitalization and readjustment of immunosuppressive therapy doses , he was discharged with clinical and laboratory regression of liver rejection . after 4 months , he sought the hospital with a 1-week history of severe pain in the left leg , associated with edema , local heat and redness with discrete blisters in the distal third of this limb ( fig 1 ) . amoxicillin / clavulanic acid 1,000/200 mg ev , three times a day , was prescribed due to the assumption of bullous erysipelas ; 3 days after starting antibiotic treatment , there was persistence of severe pain in the left leg and emergence of extensive ecchymosis and erosion of blisters . among the tests antigen was positive , and in both the blood culture and blister culture cryptococcus sp . even though liposomal amphotericin b 5 mg / kg / day were started on the fifth day of hospitalization , in less than 12 h , the patient had a decreased level of consciousness ( glasgow coma scale 4 ) and was transferred to the intensive care unit . a cranial computed tomography scan with contrast was normal , and a lumbar puncture was performed . the cerebrospinal fluid was clear and colorless , no cells , 75 mg / ml protein , 25 mg / ml glucose and the direct microscopy with indian ink revealed structures consistent with cryptococcus sp . the patient had onset of status epilepticus and on the 6 day of hospital admission ; 36 h after the beginning of neurological symptoms , he progressed to death . cutaneous lesions occur in 1020% of all cryptococcal infections and are often an indication of disseminated infection [ 2 , 7 ] . clinical recognition is essential for timely initiation of therapy to minimize the morbidity and mortality of this potentially lethal fungal infection [ 1 , 3 ] . there is no pathognomonic finding for cryptococcal skin disease and incorrect initial diagnosis because atypical presentations may lead to inappropriate or significantly delayed treatment , thereby having an impact on the outcomes . initially , our patient was treated for presumed bacterial erysipelas but due to the absence of response , direct examination and culture of a blister skin lesion showed the correct diagnosis . treatment of cryptococcal erysipelas has been based on recommendations for disseminated disease and central nervous system disease , as no data from clinical trials are available . current recommendations suggest liposomal amphotericin b 36 mg / kg / day or amphotericin b deoxycholate 0.51 mg / kg / day plus flucytosine 100 mg / kg / day for at least 14 days or until blood and cerebrospinal fluid cultures became sterile [ 7 , 8 ] . diagnosis of cutaneous manifestations of cryptococcosis can be challenging , as it can resemble more common bacterial infections , and a delay in appropriate diagnosis can lead to high morbidity and mortality rates . clinicians should maintain a high index of suspicion for atypical pathogens , especially fungi , among patients with skin infection not responding to antimicrobial therapy .
a 50-year - old male underwent liver transplantation due to cryptogenic cirrhosis and was admitted with severe pain in the left leg as well as phlogosis . amoxicillin / clavulanic acid was prescribed , assuming bullous erysipelas . among the tests performed , the latex agglutination test for the cryptococcus sp . antigen was positive , and in both the blood culture and blister culture cryptococcus sp . was isolated . daily fluconazole was started . even though liposomal amphotericin b has been started on the fifth day of hospitalization , the patient progressed to death .