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malignant mixed tumors are usually classified as one of three main subtypes : ( a ) carcinoma ex pleomorphic adenoma ( cxpa ) , ( b ) true malignant mixed tumor ( carcinosarcoma ) , and ( c ) metastasizing mixed tumor . cxpa is reported to represent approximately 3 - 5% of all major salivary gland neoplasms and 5 - 15% of major salivary gland malignancies . a 17-year - old female reported to us with a slowly enlarging mass in her right side of buccal mucosa [ figure 1 ] . the mass was not painful and did not trouble her , except that it had become increasingly noticeable . intraoral preoperative view bimanual palpation revealed a firm mobile , 3-cm well - circumscribed mass in the right buccal mucosa region lateral to the upper premolars . ultrasonography showed a well - defined , round ; predominantly hypo echoic mass lying underneath the right buccinator muscle . the tumor was enucleated under general anesthesia [ figure 2 ] . on gross examination , the encapsulated lesion measured 4 2.5 cm , with a whitish , faintly lobulated surface . histopathological examination , ( 20 , hande stain ) showed a neoplasm with an admixture of epithelial and stromal components . tumor epithelial cells arranged in the form of ducts containing eosinophilic material , islands and sheets [ figure 3 ] . ( hande stain , 20 ) on magnification ( 40 , hande stain ) certain areas showed tumor epithelial cells invading the fibrous capsule [ figure 4 ] . cellular details revealed moderate nuclear pleomorphism , loss of chromatin , prominent nucleoli , and moderate mitotic activity . keratin formation by epithelial cells is also seen [ figure 5 ] . due to the presence of atypia and capsular infiltration , this rare histopathological diagnosis warranted a therapeutic reconsideration as some of the margins showed infiltration . the second histopathological report was free of tumor cells , and exhibited connective scar tissue . after a 1 year follow - up period , the patient is symptomless and shows no signs of recurrence . pleomorphic adenoma is very rarely reported in the minor salivary glands and cxpa involving the buccal minor salivary gland is even rarer . cxpas are subclassified into : noninvasive , minimally invasive ( about 1.5-mm penetration of the malignant component into the extra capsular tissue ) , and invasive ( more than 1.5-mm penetration into the extra capsular tissue ) . however , due to the rarity of occurrence , it is not usually considered in the differential diagnosis , as in our case where the histopathological finding necessitated a second surgery . we should , therefore , always advise an incisional biopsy in large lesions of the buccal mucosa followed by thorough histopathological evaluation and definitive radical treatment . in smaller lesions , complete excision with wide margins should be undertaken . | carcinoma ex pleomorphic adenoma ( cxpa ) , as a group , constitutes 12% of malignant salivary gland tumors .
we present a case of cxpa of the buccal mucosa in a 17-year - old patient .
the buccal mass was of a size of 3.0 cm located in the right cheek .
pleomorphic adenoma was the provisional diagnosis .
the tumor was excised under local anesthesia .
histopathological evaluation revealed a pre - existing pleomorphic adenoma .
however , on magnification , certain areas showed islands of dysplastic epithelial cells invading the fibrous capsule and cxpa was diagnosed .
the patient was recalled and secondary surgery of the site performed .
no tumor tissue could be detected in the secondary resection specimen .
there is no sign of recurrence since 2 years . |
we report a case of an adolescent sustaining bilateral femoral neck fractures due to a first time epileptic seizure , as a result of expansion of his known syrinx . a 19-year - old patient suffering from hypophosphatasia ( hpp ) , arnold - chiari malformation , and a ventriculoperitoneal shunt sustained a trivial fall with profound pain and an inability to mobilize . radiographs demonstrated a right - sided garden-4 femoral neck and left - sided multi - fragmentary intracapsular / extracapsular fractures . the patient had previously suffered bilateral proximal femoral shaft fractures , treated with intramedullary unlocked nail fixation that was still in situ . operative treatment with an exchange to synthes adolescent lateral recon nail was performed on the right with two recon screws inserted into the femoral head . on the left , the existing pedinail was preserved with an additional single screw inserted into the femoral head . in addition , 3 months of non - mobilization was required for adequate bone healing . after 1-year from time of injury , there is no avascular necrosis on radiographs and the patient is mobilizing pain - free . we recommend surgical fixation with an intramedullary device and periods of non - mobilization until there is radiographical evidence of adequate bone healing . hypophosphatasia ( hpp ) is a rare metabolic disorder with a mutation occurring within the liver / bone / kidney alkaline phosphatase gene ( alpl ) . this leads to the production of ineffective tissue - non - specific alkaline phosphatase with resultant defective skeletal mineralization and weakened bone morphology . bilateral femoral neck fractures occurring within young adults are rare , associated with high - energy trauma and occurred more frequently in the subset of patients treated for drug - induced convulsions . we report a case of a young man suffering from childhood hpp , who presented with bilateral femoral neck fractures after experiencing a first - time epileptic seizure . a 19-year - old adolescent presented to the emergency department after being found on his bedroom floor complaining of sudden onset excruciating bilateral hip pain and an inability to mobilize . past medical history included childhood hpp , arnold - chiari malformation with syrinx , and bilateral ventriculoperitoneal shunts . the patient had previously suffered bilateral proximal femoral shaft fractures treated with intramedullary fixation in 2011 ( 8 mm orthopedic nails ) and a proximal tibial fracture , treated conservatively with a sarmiento cast in 2012 . plain radiographs on admission demonstrated a right - sided garden-4 femoral neck and left - sided multi - fragmentary intracapsular / extracapsular fractures ( fig . 1 ) . investigations ( 12-lead electrocardiogram , 24-h tape , echocardiogram , electroencephalogram , computed tomography ) confirmed a diagnosis of frontotemporal epilepsy due to an expansion of his known syrinx . the right - sided garden-4 femoral neck fracture was reduced with the lead better maneuver . the previous intramedullary pedinail was removed and exchanged for a 380 mm x 9 mm synthes adolescent lateral recon nail with two proximal locking screws within the head component . the left multi - fragmented intracapsular / extracapsular fracture the existing pedinail was preserved , its position adjusted slightly to allow a screw to be placed through it and into the femoral head . this was reinforced with 2 mm x 7 mm - cannulated screws inserted posterior to the nail ( fig 2 . ) . surgery was followed by 6 weeks of bed - rest and a further 3 months of non - mobilization . radiographs taken 1-year after initial injury demonstrate good fracture healing with no evidence of avascular necrosis ( figs . 3 and 4 . ) . right hip radiograph 1-year from time of injury left hip radiograph 1-year from time of injury fracture occurrence in hpp is documented within the literature and occurs due to impaired mineralization and calcification with altered non - mineralized osteoid bone matrix and subsequent degradation of bone quality . reports within the literature suggest fractures occurring in hpp patients should be managed operatively with internal fixation using intramedullary nails . this is because of their load sharing properties and to help reduce the risk of stress risers . in this adolescent with hpp , the goal of operative treatment was to minimize bone damage in an already softened bone and to preserve bone and blood supply to maximize chances of healing . the left pedinail was successfully preserved requiring only minor adjustment to allow the insertion of a femoral head locking screw , with further reinforcement using a cannulated screw . the right - sided garden-4 femoral neck fracture was successfully manipulated achieving good reduction allowing the femoral head to be preserved . however , it was felt that additional reinforcement was required to prevent any displacement of the femoral head . the pedinail was exchanged for a synthes adolescent lateral recon nail with two proximal locking screws inserted within the head component . six weeks of bed - rest and 3 months of non - mobilization were necessary to prevent any cut out of the prosthesis and screw displacement as the patient has proven to heal slowly from his previous fractures . there have been previous reports , which demonstrate delayed bone healing in hpp taking up to 3 months . fractures occurring in hpp patients present a challenge to the orthopedic surgeon due to soft bone quality and delayed bone healing . reports within the literature suggest intramedullary devices be used for surgical fixation due to their load - sharing properties . we recommend coupling this with periods of non - mobilization until there is radiographical evidence of bone healing . patients suffering from hpp have an increased propensity for fractures due to soft bone quality . periods of non - mobilization after fracture fixation may be required until there is radiographical evidence of bone healing . | introduction : we report a case of an adolescent sustaining bilateral femoral neck fractures due to a first time epileptic seizure , as a result of expansion of his known syrinx.case report : a 19-year - old patient suffering from hypophosphatasia ( hpp ) , arnold - chiari malformation , and a ventriculoperitoneal shunt sustained a trivial fall with profound pain and an inability to mobilize .
radiographs demonstrated a right - sided garden-4 femoral neck and left - sided multi - fragmentary intracapsular / extracapsular fractures .
the patient had previously suffered bilateral proximal femoral shaft fractures , treated with intramedullary unlocked nail fixation that was still in situ .
operative treatment with an exchange to synthes adolescent lateral recon nail was performed on the right with two recon screws inserted into the femoral head .
on the left , the existing pedinail was preserved with an additional single screw inserted into the femoral head .
in addition , 3 months of non - mobilization was required for adequate bone healing .
after 1-year from time of injury , there is no avascular necrosis on radiographs and the patient is mobilizing pain-free.conclusion:patients with hypophosphatasia have delayed bone healing .
we recommend surgical fixation with an intramedullary device and periods of non - mobilization until there is radiographical evidence of adequate bone healing . |
for this study , patients with a clinical diagnosis of sfts were those who had fever , thrombocytopenia , or leukopenia without another known acute infectious disease ; patients with laboratory - confirmed sfts had sftsv antibodies or rna detected by elisa or reverse transcription pcr ( rt - pcr ) . acute - phase ( within 2 weeks after onset of illness ) and convalescent - phase serum samples obtained during hospitalization of the patients were tested for total antibodies against sftsv by using a double - antigen sandwich elisa kit ( xinlianxin biomedical technology limited , wuxi , china ) . undiluted patient serum samples were used for elisa ; sftsv antibodies were detected with horseradish peroxidase labeled recombinant sftsv protein . serum samples were considered positive for sftsv when absorbance of the sample was > 2.1 times that of a negative control at 450 nm ( 8) . nested rt - pcr amplification of the sftsv rna large segment ( 900 bp ) and small segment ( 600 bp ) have been described previously ( 8) . pcr products were confirmed to be sftsv rna by dna sequencing . during june 26 , 2011august 26 , 2012 , a total of 46 patients were hospitalized and given a clinical diagnosis of sfts in a local hospital in yiyuan county , shandong province , china . we confirmed by elisa or rt - pcr that 33 ( 71.7% ) of these 46 patients were infected with sftsv . of the confirmed cases of sfts , 22 occurred in 2011 and were reported previously ( 8) . two ( 6.1% ) patients with confirmed sfts died . among the 31 laboratory - confirmed living patients with sfts , 25 agreed and 6 refused to donate blood samples for neutralization assay after discharge . thirteen ( 52% ) volunteers were male and 12 ( 48% ) were female ; their ages ranged from 42 to 75 years ( median age 62 years ) . blood samples were obtained from the 25 sfts volunteers 2 or 3 times during a 4-year period . serum samples were heat inactivated at 56c for 30 min and diluted in 2-fold increments from 1:5 to 1:1,280 . each dilution of serum was mixed with an equal volume of solution containing sftsv ( 1,000 pfu / ml ) at 37c for 1 hour . the titer obtained is the reciprocal of the highest serum dilution that reduces the number of plaques by 50% relative to the average number of plaques in viral control wells . at first initially , 10 sftsv is inoculated into cells in 1 well of a 6-well plate . when a cytopathic effect is visible , cells with the cytopathic effect are aspirated with a pipette tip and transferred to a new well . a single plaque is picked and used for viral stock when the plaques are clearly visible on the fifth passage . to determine the viral titer with a plaque assay infected cells are incubated at 37c in 5% co2 for 1 h ; then , viral inoculum is replaced with dulbecco s modified eagle medium containing 1.5% methylcellulose , 1% fetal bovine serum , 10 mmol / l hepes , penicillin ( 100 units / ml ) , and streptomycin ( 100 g / ml ) . plates are incubated at 37c in 5% co2 for 10 d. the monolayer is fixed with 4% paraformaldehyde and stained with crystal violet . prnt50 results showed that all 25 patients developed neutralizing antibodies against sftsv at titers from 20 to 640 ; the neutralizing antibodies lasted for the entire study period of 4 years ( table ) . we also performed prnt90 tests for all 25 patients ; these showed similar results to prnt50 , but the titers were less in extent than those of prnt50 ( data not shown ) . * convalescent - phase serum samples were obtained during the first , second , third , and fourth years after discharge of the patients from the hospital . nt , no test performed because serum was not available ; prnt50 , plaque reduction neutralization test indicating the serum titer that reduced 50% of plaque - forming units of sfts virus ; sfts , severe fever with thrombocytopenia syndrome . in general , the titer of neutralizing antibodies decreased over time in all but 2 patients ( nos . 5 and 11 ) , who had a higher prnt50 titer in the last year than in the first year ; this increase may have been caused by reinfection with sftsv . however , our previous studies indicate that , in the local area , the incidence of sftsv infection is < 5 cases/100,000 population and the seroprevalence rate of sftsv in the healthy population is < 1% ( 810 ) , suggesting that the chance of reinfection of a patient with sftsv is low . we can not exclude , however , that these 2 patients could have been infected with other phleboviruses that have not yet been isolated in china . serum samples from 2 healthy persons were also tested for neutralizing antibodies as controls ; neither of them had any neutralization activity against sftsv . we found that hospitalized patients with sfts produced long - lasting neutralizing antibodies to sftsv . we do not know the characteristics of the neutralizing antibodies against sftsv , which need to be further investigated . in general , neutralizing antigens of bunyavirus are located on the viral glycoproteins ( 1113 ) . a neutralizing monoclonal antibody to sftsv is found to bind a linear epitope in the ectodomain of glycoprotein gn of sftsv . its neutralizing activity is attributed to blockage of the interactions between the gn protein and the cellular receptor ( 14 ) . the limitation of this study is that we obtained sfts patients serum samples for only up to 4 years after diagnosis ; we do not know how long the neutralizing antibodies in patients will last . | severe fever with thrombocytopenia syndrome is an emerging hemorrhagic fever disease in eastern asia , caused by a tickborne bunyavirus . of 25 patients hospitalized with this disease in china ,
100% produced and maintained neutralizing antibodies to severe fever with thrombocytopenia syndrome virus for the study period of 4 years . |
a 60-year - old female presented with a history of serosanginous discharge and bleeding ( small amount ) per vaginum for the last 4 months . she was postmenopausal for the last 10 years with p5 + 1 + 0 + 5 . local examination revealed hard infiltrative growth destroying both cervical lips and involving the vaginal wall . the upper third of the lateral and upper half of the anterior and posterior walls of the vagina were involved . hemogram ( hb = 11.4 gm% , tlc = 7600/l ) and renal function tests ( urea = 24 mg / dl and creatinine = 0.81 mg / dl ) were normal . ultrasound of the abdomen and pelvis showed no intra - abdominal / pelvic collection or pyometra . with carcinoma cervix iii b ( figo clinical staging ) , she was planned for external radiotherapy ( 46gy in 23 fractions over 4.5 weeks ) . but after receiving 18gy/9 fractions , she presented with pain in the abdomen and vomiting . contrast - enhanced ct showed multiple pelvic and intra - abdominal collections , a dilated endometrial cavity with breach at the uterine fundus and its continuation with large pelvic collection , suggestive of perforated pyometra . sagittal and coronal reformats in multi - detector ct depicted the site and size of the uterine breach . the patient was managed with pigtail drainage of the larger pelvic and subhepatic collections , and with appropriate antibiotic cover ( cefoperozone , sulbactam and amikacin ) . she improved clinically and follow - up ultrasound after 1 week showed near complete resolution of the abscesses , and the catheter was taken out ( fig . 1 and 2 ) .
figure 1contrast - enhanced axial ct images showing dilated endometrial cavity with site of the breach at uterine fundus ( arrow ) . pelvic collections ( * ) are seen with the abdominal / mesenteric extension and resultant intra - abdominal collections ( * ) . figure 2contrast - enhanced coronal and sagittal reformatted ct image depicting dilated endometrial cavity with site and size of the breach at uterine fundus ( arrow ) . contrast - enhanced axial ct images showing dilated endometrial cavity with site of the breach at uterine fundus ( arrow ) . pelvic collections ( * ) are seen with the abdominal / mesenteric extension and resultant intra - abdominal collections ( * ) . contrast - enhanced coronal and sagittal reformatted ct image depicting dilated endometrial cavity with site and size of the breach at uterine fundus ( arrow ) . pyometra is an uncommon condition occurring mainly in elderly postmenopausal females , and results when natural drainage of the uterine cavity is compromised . the common causes of pyometra include malignant condition of the genital tract and sequele of their treatment , benign conditions such as infection and congenital cervical anomalies . the benign or malignant conditions cause accumulation of secretions and gradual enlargement of the uterus , leading to thinned uterine walls which may be sloughed with spontaneous uterine rupture and causing generalized peritonitis . spontaneous uterine perforation is rare and extensive review of the english literature has revealed only 26 such reported cases to date . furthermore , spontaneous rupture of pyometra in cervical cancer presenting as generalized peritonitis is extremely rare and only four cases have been described . we reviewed all the cases of spontaneous uterine perforation in cervical cancer and the findings are summarized in table 1 . no.agesymptomsprovisional diagnosisperforation sitehistologytreatment867ap , gbgpfundussquamous cell carcinomaaspiration and drainage234apgp , ppleft corneal regionsquamous cell carcinomadrainage and pl272apgpfundussquamous cell carcinomadrainage and pl160ap , fgpfundussquamous cell carcinomatah with bsopresent case60ap , f , vgpfundusadeno - carcinomapigtail drainageap , abdominal pain ; bso , bilateral salpingo - oophorectomy ; f , fever ; gb , genital bleeding ; gp , generalized peritonitis ; pl , peritoneal lavage ; pp , perforated pyometra ; tah , total abdominal hysterectomy ; v , vomiting . cases of spontaneous uterine perforation in cervical cancer ap , abdominal pain ; bso , bilateral salpingo - oophorectomy ; f , fever ; gb , genital bleeding ; gp , generalized peritonitis ; pl , peritoneal lavage ; pp , perforated pyometra ; tah , total abdominal hysterectomy ; v , vomiting . the most common perforation site was the uterine fundus and in only one case it was the uterine cornual region . ct features of perforated pyometra have been described in only one case in which ct revealed the diagnosis and surgical intervention was performed . in our case , the patient presented with features of generalized peritonitis , ct showed perforated pyometra at the uterine fundus with multiple pelvic and intra - abdominal collections . sagittal and coronal reformats in multi - detector ct are very helpful in depicting the site and size of uterine breach , demonstrating the resultant intra - abdominal collections and staging of cervical cancer . the site of uterine breach can be missed on axial images as it is usually seen in fundus ; hence , in elderly postmenopausal females with peritonitis sagittal and coronal reformats of uterus should be carefully evaluated . sonography plays a limited role in the diagnosis of ruptured pyometra because of its inability to demonstrate the uterine breach and the limited sonographic window available due to perforation . treatment of ruptured pyometra in cervical cancer patients varies depending on the clinical condition of the patient and the preoperative diagnosis . in most cases drainage of the collections was carried out , except for one case in which total abdominal hysterectomy and bilateral salpingoophorectomy were peformed . the index case was managed by putting percutaneous pigtail catheters in pelvic and subhepatic collections . spontaneous rupture of pyometra in cervical cancer is very rare and should be considered in elderly postmenopausal women with cervical cancer presenting with an acute abdomen . multi - detector ct with sagittal and coronal reformatted images could play an important role in the diagnosis of ruptured pyometra . | abstractpyometra is an uncommon condition with an incidence of less than 1% in gynaecologic patients .
spontaneous rupture of pyometra in cervical cancer presenting as generalized peritonitis is very rare .
only four cases have been described in the english literature to the best of our knowledge and from a pubmed search .
the index case is an elderly postmenopausal female who was diagnosed with cervical cancer , started on radiotherapy and presented with features of generalized peritonitis .
contrast - enhanced ct revealed uterine perforation at the fundus with multiple abdominal and pelvic collections .
a brief review of all the cases of ruptured pyometra in cervical cancer in the literature and a discussion of the role of imaging is presented . |
while cardiac magnetic resonance imaging ( mri ) has been used to evaluate the myocardium in terms of the myocardial function , perfusion , and viability , rather than coronary stenosis ; coronary magnetic resonance angiography ( cmra ) is currently considered a reliable imaging test for the evaluation of coronary anatomy.1 ) this paper reports a case with triple - vessel disease that could not be accurately assessed by coronary computed tomography angiography ( ccta ) because of calcification , and without significant perfusion defects or scar changes observed on a cardiac mri . however , whole - heart cmra performed as part of the cardiac mri protocol demonstrated significant triple - vessel disease with left main ( lm ) involvement , as confirmed by subsequent invasive angiography with a fractional flow reserve measurement . he had a history of hypertension , diabetes mellitus , dyslipidemia , and ischemic stroke . ccta using a 256-slice multi - detector computed tomography scanner ( brilliance ict ; philips medical systems , best , netherlands ) showed multifocal stenoses in the lm bifurcation , proximal left anterior descending artery ( lad ) , proximal left circumflex artery ( lcx ) , and mid right coronary artery ( rca ) ( fig . while significant stenosis was suspected in the lm bifurcation , it was difficult to evaluate the stenosis severity of the lad , lcx , and rca because of heavy calcification . we subsequently performed a cardiac mri using a 3-t mr system ( ingenia ; philips medical systems , best , netherlands ) to evaluate myocardial ischemia and viability.2 ) despite the multifocal stenoses of the coronary arteries observed on ccta , neither evidence of inducible myocardial ischemia on adenosine stress myocardial perfusion mri ( fig . 2 ) nor evidence of a myocardial scar on late gadolinium enhanced mri was observed . however , when we performed a quantitative analysis of stress - rest images , the myocardial perfusion reserve indices for all segments were significantly decreased ( range , 0.48 - 0.99 ) , and the myocardial blood flow reserve estimated from the coronary sinus flow was only 1.4 . moreover , a non - contrast enhanced whole - heart cmra demonstrated significant stenosis of the lm bifurcation ( fig . 1b ) . calcified plaques in the proximal lcx and mid rca were also identified as significant stenoses on cmra ( fig . 1e , h ) , while the calcified plaque in the proximal lad was indicated as an insignificant lesion ( fig . invasive coronary angiography revealed critical stenoses in the lm bifurcation , proximal lcx , and mid rca , whereas that in the proximal lad appeared insignificant ( fig . the fractional flow reserve values of the lad , lcx , and rca were 0.50 , 0.59 , and 0.81 , respectively . considering that stress perfusion mri did not demonstrate a significant perfusion defect despite the presence of hemodynamically significant stenosis and impaired perfusion reserve , taken together , the findings indicated a balanced three - vessel coronary artery disease ( cad ) . thus , a percutaneous coronary intervention to the lm bifurcation , proximal lcx , and mid rca was performed . assessment of the coronary arteries by cardiac mri is challenging , owing to the small size of the vessels and the complex motions caused by cardiac contractions and respiration.2 ) however , in the past decades , considerable technical advances have been made , and the imaging time , success rate , and diagnostic accuracy of cmra have steadily improved.2)3 ) moreover , cmra allows a noninvasive assessment of the coronary anatomy without exposing the patients to radiation , and is superior to ccta for the evaluation of luminal narrowing in heavily calcified coronary segments.1 ) in the present case , a whole - heart cmra was useful in determining the significance of coronary stenoses that can not be assessed by ccta because of coronary calcification.4)5 ) moreover , the patient 's condition might have been missed without cmra , since myocardial perfusion mri failed to detect a significant triple - vessel disease . this is an unusual case since myocardial perfusion mri is known to be less susceptible to balanced ischemia ( a false negative finding ) , owing to its high spatial resolution.6 ) quantitative myocardial perfusion analysis showed a significantly decreased myocardial perfusion reserve index in the present case . however , quantitative analysis is not routinely performed in our daily clinical practice and is largely confined to the research field , since it is still laborious and time - consuming . considering that cmra can be easily performed as part of comprehensive cardiac mri examinations , this case highlights the value of cmra for the detection and evaluation of cad . the incorporation of cmra with cine , stress perfusion , and late gadolinium - enhanced mri allows a comprehensive assessment of cad by combining functional and anatomic evaluations . | coronary magnetic resonance angiography ( cmra ) allows a noninvasive assessment of the coronary anatomy without exposing the patients to radiation .
it is also superior to coronary computed tomography angiography ( ccta ) for the evaluation of luminal narrowing in heavily calcified coronary segments .
we report a case with triple - vessel disease , but it could not be accurately assessed by ccta because of calcification and lack of a significant perfusion defect or myocardial scarring on cardiac magnetic resonance imaging ( mri ) .
however , whole - heart cmra performed as part of the cardiac mri protocol demonstrated significant triple - vessel disease with left main involvement , confirmed by subsequent invasive angiography with a fractional flow reserve measurement . |
tenotomy of the tendo - achillis is required in almost 85% of cases of congenital talipes equino varus ( ctev ) treated with ponseti technique . mini - open and percutaneous techniques are been described for doing tendo - achillis tenotomy . use of wide bore needle to perform percutaneous tenotomy of tendo - achillis was first described by minkowitz et al and has been reported by few other authors . with the global ponseti initiative aiming to reach the rural population , tendo - achillis tenotomy is planned when midfoot pirani score came to zero after serial casting by ponseti technique . knee is kept at 90 flexion and hip abducted to reach the posterior part of the foot . the tendo - achillis is palpated as a tense cord when the foot is dorsiflexed . the foot is kept dorsiflexed to tense the tendo - achillis and needle is inserted from the medial border of the tendo - achillis about one finger breadth proximal to the insertion of tendo - achillis or the posterior heel crease ( fig.1c ) . in case the child is very chubby , it is sometimes difficult to feel the complete extent of tendo - achillis . in these cases a more proximal insertion of the needle will be required and needle will be required to be inserted to a greater depth . the tip of the needle is used to cut the tense fibres of tendo - achillis and a grating sensation can be felt and heard ( refer video ) . a dorsiflexion force is continuously applied to the foot and as the tenotomy is completed a snap is felt and heard ( refer video ) with visible correction of dorsiflexion ( fig.1d ) . after this the needle is removed and dorsiflexion is rechecked and ankle movements are full and free ( fig.1e , f ) . preprocedure ankle plantar flexion and dorsiflexion where tendo - achillis tightness can be felt ( a , b ) . 16 gauge needle is inserted from the medial side of tendo - achillis about a finger breadth proximal to the insertion ( c ) . tenotomy is completed by cutting through the tendon while a constant dorsiflexion force is applied to the foot ( d ) . post - procedure ankle movements are much improved as compared to the preprocedure movements ( e , f ) tenotomy of the tendo - achillis is an essential step of ponseti treatment of ctev . conventional blade tenotomy achieves good correction , however complications like damage to neurovascular structures leading to bleeding or pseudo aneurysms are reported . in this respect needle tenotomy is a relatively simple procedure which is less invasive and probably has less morbidity . it does not require an operation theatre and can safely be done as opd procedure under sedation . again as mentioned earlier , with global clubfeet initiative , more paramedics are being involved in treatment of ctev . this percutaneous needle technique is easy to learn and is relatively free of complications and will be much useful for these initiatives too . this technique is already described in literature and we are just providing a detail technical steps and a video for easy learning and thus promote use of this safe procedure . the author has been using this technique for over 6 years now and has performed over 600 procedures with good results in all cases . in all cases adequate correction could be achieved and no open procedure was required in any case . even with such good results , authors found that the technique is poorly reported in literature with only three series . the main purpose of this technical note is to create awareness of this technique and to evoke other surgeons to use and validate it as more data is required in terms of defining the limitation of the technique , use in delayed and older cases and use in syndromic feet . available on www.jocr.co.in volume 2 | issue 1 | jan - mar 2012 percutaneous needle tenotomy for tendoachilis release in club foot is a simple and reliable procedure . | tendo - achillis tenotomy is required as last step of ponseti method of treatment of congenital talipes equino varus ( ctev ) .
this technical note describes a simple method of doing a percutaneous tenotomy of tendo - achillis using a wide bore needle .
this is a simple method and easy to learn and gives predictable results . |
it has been reported that in autopsy cases of prostate cancer patients , adrenal metastasis is observed in 17 - 20% of the cases and pleural metastasis is observed in around 20% of the cases [ 1 , 2 ] . however , there are few reports on clinical cases , and the case described here is only the fourth case of adrenal metastasis and the second case of pleural metastasis . as far as we could determine based on an extensive literature search , there have been no reports of prostate cancer that developed simultaneous metastases in both the adrenal glands and the pleura , and we herein report this case with a few bibliographical considerations . he became aware of increased urinary frequency starting in 2004 but did not undergo any specific check - ups . in 2006 , a high psa level was observed and the patient was referred to our hospital for a check - up . at the check - up , a high psa level of 515 ng / ml was observed and a transrectal prostate needle biopsy was performed . the pathological finding was moderately differentiated prostate cancer with a gleason score of 7 ( 4 + 3 ) . although bone metastasis was observed , there was no lymph node metastasis or any distant metastasis . the patient was diagnosed with having clinical stage d prostate cancer , and a maximum androgen blockade ( mab therapy ) was started . the cancer became exacerbated 1 year after starting the mab therapy . in response to the exacerbated prostate cancer , we started a therapy involving the administration of 8 mg / kg body weight of dexamethasone and 55 mg / m of docetaxel every 3 weeks . after completing 8 courses , an enlargement of the bilateral adrenal tumor was observed , and after completing 12 courses , a pleural tumor was discovered and the psa level was also increased . the patient was therefore diagnosed with having bilateral adrenal metastasis and pleural metastasis of prostate cancer through diagnostic imaging ( fig . although it has been reported that 17 - 20% of all autopsy cases of prostate cancer patients have adrenal metastasis , this case is only the fourth known clinical case , following cases reported by navarro et al . long and husband reported that out of 398 cases of prostate cancer in which an abdominal ct was performed , adrenal metastasis was observed in only 1 case . although many cases of unilateral pulmonary metastasis have been reported , there has been only 1 case of pleural metastasis in japan , reported by takagi et al . . however , it is believed that cases of pulmonary metastasis include cases that also involve pleural metastasis , and that the actual number of cases of pleural metastasis is therefore higher than reported so far . so far , there have been no reports of multiple occurrences of prostate cancer in the adrenal glands and the pleura , thus making this case the first such case . | our case was 65 years old . at check - up ,
a high psa level of 515 ng / ml was observed , the patient was diagnosed with having clinical stage d prostate cancer and a maximum androgen blockade ( mab therapy ) was started . in response to the exacerbated prostate cancer , we started a therapy involving the administration of 8 mg / kg body weight of dexamethasone and 55 mg / m2 of docetaxel every 3 weeks .
after completing 8 courses , an enlargement of the bilateral adrenal tumor was observed , and after completing 12 courses , a pleural tumor was discovered and the psa level was also increased .
the patient was therefore diagnosed with having bilateral adrenal metastasis and pleural metastasis of prostate cancer through diagnostic imaging .
so far , there have been no reports of multiple occurrences of prostate cancer in the adrenal glands and the pleura , thus making this case the first such case . |
after its composition was firstly described by billroth1 in 1856 , tcherkoff and sedlis2 reported lesions of the same type in the uterine cervix . later baggish and woodruff3 recognized it as a distinct type of cervical neoplasm from adenoid cystic carcinoma . the incidence of adenocarcinoma of the uterine cervix is reported to account for less than 1% . although the origin is debatable , it is considered derived from multipotential cells of the basal layer or reserve cells of cervical epithelium . clinically , adenoid basal carcinoma is differentiated from other types of cancer for the rare metastasis and the excellent prognosis . . twenty - two - year - old young female with adenoid basal carcinoma of the uterine cervix have been rarely reported in the literature . in korea where carcinoma of uterine cervix is one of the most common malignancy , adenoid basal carcinoma of the uterine cervix is considered relatively rare . the patient was a 22-year - old korean woman who presented with a history of abnormal genital bleeding for 3 weeks . no gross lesion was noted on the cervix and the pap smear was reported to be high - grade squamous intraepithelial lesion ( hsil ) . the serum level of the tumor marker carbohydrate antigen ( ca ) 125 was elevated ( 48 u / ml ; normal < 35 u / ml ) , whereas the serum levels of carcinoembryonic antigen ( cea ) , ca 19 - 9 , a - fetoprotein and squamous cell carcinoma antigen were within the normal ranges . concerned with the patient 's hsil results , the clinician performed a cervical loop electrosurgical excision procedure ( leep ) and observed multiple erosions of inflammation overlying the cervix . the clinician was able to discover hsil with superficial glandular extension and report adenoid basal carcinoma . the patient is being closely followed up and has shown no evidence of recurrence within 24 months after the operation . macroscopically , the tumor size of the lesion was 1.0 cm in the largest dimension . microscopically , tumor cells were arranged in small nests or cords , with focal squamous differentiation , however , cystic change was not noted . the adenoid basal carcinoma was adjacent to the hsil lesion , but no transition between the two lesions was observed . stains for p16 and ki-67 showed positive staining , whereas staining for cytokera - were negative ( fig . cervical cancer is the second most common cancer among women worldwide and is one of leading causes of death by cancer in women.4 it is generally considered that adenoid basal carcinoma of the cervix is a rare lesion which occurs mostly among postmenopausal african - american women . however , recently there have been reports that the tumors can also occur in asian women . in korea , there were four cases reported of adenoid basal carcinomas of the cervix . the rare form of mucinous adenocarcinoma of the cervix , adenoma malignum , requires differential diagnosis . especially because it is histologically and radiologically similar to the benign form and often causes confusion upon diagnosis.5 adenoid basal carcinoma is located below the epithelium . with naked eyes , it is observed as normal cervix without clear lesion . histologically , it is composed and proliferates in the form of nests of small round cells . the cells are characterized by relatively a large dense nucleus and the light cytoplasm ( fig . the most important differential diagnosis is adenoid cystic carcinoma because of the local invasion and remote metastasis . as its name suggests , the histological aspects of the two tumors include basaloid cell proliferation , squamous and granular differentiated filament . brainard and hart7 proposed the use of the term basal cell epithelioma as adenoid basal carcinoma with typical histological structure is not malignant . we summarize that both adenoid basal carcinoma and adenoid cystic carcinoma originate from the reserve cells in the uterine cervix . the tumor is often observed in salivary glands , sometimes in respiratory organ , skin , head and neck mucosa , and breast . it is found rarely in the female genital organs , if found , mostly in the cervix , bartholin 's gland , and endometrium . a common symptom of adenoid cystic carcinoma of the uterine cervix is postmenopausal menorrhagia . in many cases more than half of the patients are diagnosed with clinical stage i with unfavorable prognosis . in contrast to adenoid basal carcinoma , adenoid cystic carcinoma appears as a polyp at the cervix . histologically , it shows an increase in cell size , the number of cell colonies , the number of mitotic cells , and organic reaction.8~10 immunohistochemically , adenoid basal carcinoma of the uterine cervix typically shows positive staining for ki-67 and p16 . grayson et al.11 observed that immunohistochemical analysis of the adenoid cystic carcinoma revealed positive staining for epithelial membrane antigen ( ema ) , collagen iv and laminin , while adenoid basal carcinoma revealed positive staining for ema and negative staining for collagen iv and laminin . ferry and scully6 reported one patient , a 67-year - old female , who died in 3 months as a result of metastatic lung cancer from adenoid basal carcinoma . it is treated with hysterectomy , chemotherapy , and radiation therapy . in conclusion , for treatment and clinical management of patients , it is important to understand adenoid basal carcinoma differently from other kinds of uterine cervix cancer . it is also critical to distinguish adenoid basal carcinoma of low metastatic potential and favorable prognosis from adenoid cystic carcinoma of similar shapes and unfavorable prognosis . in this paper , we have discussed a pimipara young woman under close observation after conization . for young female cases have been rarely reported , the case provides a clinical insight into diagnosis of adenoid basal carcinoma . | adenoid basal carcinoma of the uterine cervix is uncommon neoplasia mostly occurring in postmenopausal women .
it has excellent prognosis and a favorable clinical course . in addition
, adenoid basal carcinoma is differentiated from adenoid cystic carcinoma by histologic and cellular morphologies , and immunohistochemistry . in this paper , we present the case of a 22 year old korean female .
she initially had a high - grade squamous intraepithelial lesion ( hsil ) on pap smear and a subsequent cervical loop electrosurgical excision procedure ( leep ) specimen revealing adenoid basal carcinoma .
the lesion showed the histologic characteristics of adenoid basal carcinoma .
because of the lesion 's low potential for recurrence and metastasis , the young primipara had a conization procedure performed and has been under close observation . |
dengue fever ( df ) is characterized by a spectrum of clinical features that ranges from asymptomatic infection to severe illness ( dengue hemorrhagic fever [ dhf]/dengue shock syndrome ) . spontaneous splenic rupture is however a rarity , which we encountered in the case mentioned below . a 26-year - old female presented to the emergency department with an acute abdominal pain and prostration in a background of intermittent fever and watery diarrhea for 4 - 5 days . her past medical history was unremarkable . on examination , she had severe pallor , tachycardia ( pulse 120/min ) , tachypnea and a blood pressure ( bp ) of 90/60 mm hg . there was a marked tenderness over all quadrants of the abdomen with ascites and hepatosplenomegaly . initial investigations revealed , hemoglobin ( hb)-3.4 g / dl , total leukocyte count-3.3 10/l ( n-90% , l-8% , e-2% ) , platelet count -40 10/l packed cell volume 12.2% , mean corpuscular volume 87 fl ; microscopic examination of urine was normal . liver function test , serum amylase , lipase and electrolytes were within normal limits , but values of serum urea was 88 mg / dl and serum creatinine was 2 mg / dl . an urgent straight x - ray abdomen showed no evidence of pneumoperitoneum , whereas the chest x - ray revealed bilateral pleural effusions [ figure 1 ] . she was resuscitated with intravenous normal saline and blood transfusions ( 4 units of packed cells and 6 units of platelets ) dopamine was used transiently in the first 24 h when fluids failed to raise bp adequately . after she became hemodynamically stable , ultrasonography was done and the report corroborated with clinical findings . subsequently computed tomography scan of the abdomen showed perisplenic hypoattenuating collection with the splenic border displaced by mass effect . patient denied any abdominal trauma ; hence a diagnosis of hemoperitoneum due to spontaneous splenic rupture was entertained . blood test for epstein - barr virus was negative but dengue serology was positive for non - structural protein 1 ( antigen capture elisa ) and immunoglobulin m ( ivd micro - well elisa df kit ) sent on the 2 day of admission ( 7 day after the onset of fever ) . meanwhile her hemogram improved on the 4 day onward - hb 11.4 g / dl , platelet 130 10/l and hematocrit was 47% . chest x - ray showing bilateral pleural effusion computed tomography scan of abdomen showing perisplenic hypoattenuating collection with the splenic border displaced by mass effect and the fluid around spleen ( purple arrow on the right ) , arrow on the left showed fluid around the liver ( black arrow on the left ) df is caused by all four serotypes of dengue virus carried by the vector aedes aegypti and rarely by aedes albopictus . the complicated df affects primarily children , but because of shifting pattern of immunity and infection , it is now prevalent among the adult population too . the exact pathogenesis of complicated dengue is still unknown , but increased vascular permeability , abnormal homeostasis , increased capillary fragility , thrombocytopenia , impaired platelet function and disseminated intravascular coagulopathy all have been incriminated . spontaneous splenic rupture is a rare , but the life - threatening complication of infectious diseases . the diagnosis of spontaneous splenic rupture should be entertained in young patients with acute atraumatic abdominal pain and hemoperitoneum with a history suggestive of a recent infection . splenic rupture may be secondary to abdominal trauma or due to non - traumatic causes . spontaneous splenic rupture is caused by lymphoproliferative diseases , connective tissue disorders , solid neoplasm , aneurysm , pancreatitis and various infective disorders . the spleen is frequently congested in cases of dhf and sub - capsular hematomas are found in 15% of necropsy cases . of the 11 dengue cases describing splenic rupture , eight ( out of 11 ) patients have survived . the survival observed in these cases should be attributed to timely diagnosis and management rather than the natural course of splenic rupture , which is expected to be poor if the diagnosis is missed . the pathogenesis behind this serious complication is probably congestion of spleen and thrombocytopenia or both . splenectomy is the treatment of choice for spontaneous splenic rupture with hemoperitoneum , but several reports also advocate a trial of initial conservative management . the therapeutic choice depends upon the hemodynamic status of the patient and cases not responding to conservative therapy must undergo splenectomy . we kept the patient on conservative management to which she responded with a good outcome . | spontaneous rupture of the spleen with hemoperitoneum is a very rare , but serious manifestation of dengue fever ( df ) .
we report a case of a young female who was presented with atraumatic abdominal pain , hypovolemic shock , anemia , ascites and hepatosplenomegaly with a recent history of a febrile illness .
subsequent investigations proved the presence of hemoperitoneum with spontaneous splenic rupture with seropositivity for df .
early diagnosis and conservative management in this case resulted in a favorable outcome . |
a 59-year - old man came to the emergency department with a painful left ankle after twisting his foot following an unexpected fall from a 60-cm step . , there was significant oedema and palpable tenderness over the medial aspect of the left ankle , with reduced range of movement but with neuromuscular structures intact . plain radiographs of anteroposterior , mortise and lateral views revealed a fracture of the medial malleolus with no other bony injuries ( fig . surgery was performed with the patient under general anaesthetic in a supine position with an above - knee tourniquet . a medial incision was made along the medial malleolus , inline with the tibialis posterior tendon . the periosteum of the posteromedial fragment was then elevated , and the tibialis posterior tendon was retracted with an intact tendon sheath posterolaterally . two 4.0 cannulated screws were then inserted with countersink to stabilize the fracture , and the periosteum was repaired over the top of the screw ends . the flexor retinaculum was then repaired meticulously , followed by subcutaneous and subarticular closure of the wound . figure 1:plain radiographs of anteroposterior , mortise and lateral views revealed a fracture of the medial malleolus with no other bony injuries . a ( above ) : before surgery , b ( below ) : after surgery . plain radiographs of anteroposterior , mortise and lateral views revealed a fracture of the medial malleolus with no other bony injuries . a ( above ) : before surgery , b ( below ) : after surgery . two weeks post - operatively , the patient attended a follow - up clinic , complaining of pain over the ta . the ruptured ta was managed non - surgically with a below - knee plaster for 8 weeks . at a 12-month follow - up , moritz commented that for ta ruptures associated with skiing injuries , a non - displaced medial malleolus fracture occurs occasionally , but no details were available in their reports . however , to support their assertion , lugger et al . reported on a series of injured alpine skiers collected mainly in the 1960s and found a 5.3% incidence of medial malleolar fractures in those alpine skiers also treated for a ta rupture . however , this injury is rare in the skiing population nowadays , due to modified skiing boots that stop ankle motion and prevent any excessive posterior ta stretching . from a search of pubmed / medline literature , there are five case reports describing an ankle fracture combined with a ta rupture [ 15 ] . in each case , the ta rupture was complete and the fracture was a closed oblique to vertical medial malleolus fracture , with extension into the tibia plafond in two cases . the mechanism of injury consistently implied a sudden excessive upward force applied to the forefoot followed by ankle hyperextension ( in four cases ) , by hindfoot inversion ( in three cases ) or eversion ( in one case ) . in three cases , the ta rupture was initially missed , and in another case , the fracture of the medial malleolus was missed . the combination of a closed ankle fracture and ta rupture is rare , and only five individual cases have been reported in the literature . a review of these publications , along with our report , reveals obvious similarities in the mechanism of injury , the fracture pattern and the risk of one of the injuries being missed . the initial mechanism of injury was the same in each case report and consisted of a sudden upward overloading force applied to the forefoot . this was assumed to be followed either by ankle hyperextension [ 3 , 4 ] or hindfoot inversion [ 1 , 2 ] . in our case , the patient clearly reported landing with his forefoot first , followed by lateral aspect of the foot with adduction ( supination adduction ( sad ) danis forceful loads applied to the forefoot have been experimentally and clinically reported to produce ta ruptures , and concomitant active stretching of the ta with an even lesser forefoot load causes ta rupture . our literature review revealed that when ta rupture and ankle fracture were combined , an oblique to vertical fracture of the medial malleolus was present . such a fracture was found to result either from ankle hyperextension or hindfoot inversion . in either instance , a forceful overload of the forefoot preceded and was likely to have produced the ta rupture instead of a syndesmosis injury or high fibula fracture . we therefore recommend that all patients with an sad ankle injury ( danis weber sad injury ) have a thorough examination of the ta . additionally , all patients presenting with ta rupture should have routine anteroposterior and lateral radiographs of the ankle to rule out an associated medial / posteromedial malleolar fracture . any history of significant overload trauma to the forefoot should alert physicians to consider the possibility of these combined injuries . in addition , in any case of trauma , a thorough and systematic examination should be performed before a temporary splint is applied . | a 59-year - old man fell off a 60-cm - high step , with his ankle in a twisted position , and sustained a closed fracture of the medial malleolus , with an ipsilateral complete achilles tendon ( ta ) rupture .
the ta rupture was initially missed but diagnosed by ultrasound examination , 2 weeks post - operatively .
the ankle fracture was diagnosed from routine radiographs .
such a combination of injuries has been reported infrequently in the literature , but significant similarities have been described in the mechanism of injury and fracture patterns . nevertheless , three of five reported cases with combined medial malleolus fractures were initially misdiagnosed . |
obstructive sleep apnea ( osa ) and obesity hypoventilation syndrome ( ohs ) are severe sleep - related breathing disorders that in total affect > 5% of the adult population . these disorders are associated with significant morbidities , mortality , and consequently significant societal burden.14 the danish national database for osa ( ndosa ) was initiated in 2009 for the monitoring of management of osa , diagnostic and treatment procedures , and follow - up to create a nationwide database for quality monitoring and research . more than 50,000 children and adult patients are included in the danish national patient registry . since the start of quality database in 2009 , ~8,000 new patients per year have been included in the ndosa . during its first year of existence diagnostic modalities include polysomnography , partial polygraphy ( cardio - respiratory monitoring ) , and oximetry . treatment modalities include positive airway pressure ( pap ) using all modalities : fixed pressure ; auto - adjusted , bilevel , and adaptive pap ; surgical procedures ; and other procedures . the main aim of the ndosa was to improve the quality of procedures and care for patients undergoing diagnosis and treatment procedures for osa in denmark . in addition , we included patients with a diagnosis of ohs a more severe form of sleep - disordered breathing associated with severe overweight . by describing variation in clinical practice across the country , the aim was to stimulate quality improvement initiatives to standardize excellence in practice and thus improve outcomes . the ndosa aims to monitor and support the implementation of evidence - based treatment and care for patients undergoing diagnostic and treatment procedures for sleep - disordered breathing . the ndosa includes data on all diagnostic procedures from all public and private hospital departments ( eg , pulmonary , ear nose throat , neurologic ) evaluating osa in denmark . all data including diagnostic and treatment procedures in the ndosa are extracted from the danish national patient registry . all danes hold a unique personal identification number ( central person registry number ) , allowing accurate linkage between health registers and other nationwide administrative registers on an individual level . ndosa is approved as a clinical quality database by the danish health authority and hospitals and clinics are thus required to report details of all treated patients to the database . patient consent is not required for entry of data in ndosa , because according to danish law , approved clinical quality databases are exempt from the requirement of obtaining patients consent to collect relevant data . patient consent is therefore not required for entry of data in the danish clinical registries . the variables recorded in the ndosa are basic patient characteristics , comorbidity , indication for the procedure , the diagnostic tests ( polysomnography , partial polygraphy , oximetry ) and treatments ( pap and surgical or no treatment ) , pretreatment apnea severity , treatment compliance , effect of treatment ( change in apnea severity , sleepiness ) , and waiting time . registration in the ndosa is mandatory for all procedures performed in public and private danish hospitals . as the data registration is obligatory for some of the indicators , the completeness is 100% ; however , for one indicator that is not obligatory , the completeness is 97% . the key outcome data are quality measures , morbidities , mortality , and use of health care ( table 1 ) . the reported results are critically evaluated at annual meetings with the aim of improving the database content . in the case of outlier results ( positive as well as negative ) , local audit is planned , performed , and published in the final annual report . periodic assessments of the quality indicators are published on a monthly basis in the regional information systems . each department has access to their own results , both to ensure real - time registration and the opportunity to make usage of own results for the further development and quality assurance in the units , respectively . these results are of interest to clinicians in this area and a useful guide for health care administrative workers and managers to help allocate maximum value for the limited resources available . based on the register data , it has been shown that the quality regarding diagnostic and treatment procedures as well as follow - up percentage has improved overall ( figure 1 ) . for five indicators , an improvement was observed ; for two indicators , a decrease was found ; and two indicators were unchanged from 2012 to 2014 . however , the mean increase was only 2.7% with a range from 10% to + 8% in the actual values . based on the available data from the national patient register / ndosa combined with other national databases , a number of studies have been conducted . these studies suggest that osa / ohs is associated with significant societal burden in terms of direct and indirect costs and consequently societal costs,1,2 as well as an increased morbidity and mortality in children3 and adults when compared with healthy controls.4 the ndosa was started after a pretest period from 2007 to 2009 to develop data extraction from the national patient register . the main problem experienced has been to transform an administrative database to a medical longitudinal database . the first local initiative , by a group of medical doctors , ndosa is part of the danish clinical registries a national improvement program and thus funded by the danish regions . it contains information about baseline characteristics , comorbidity , procedures conducted , complications , medical treatment , and follow - up data . due to these data , we have obtained a national uniform monitoring of the diagnostic and treatment quality in this area . | aimthe aim of the danish national database for obstructive sleep apnea ( ndosa ) was to evaluate the clinical quality ( diagnostic , treatment , and management ) for obstructive sleep apnea and obesity hypoventilation syndrome in denmark using a real - time national database reporting to the danish national patient registry.study populationall patients diagnosed with obstructive sleep apnea or obesity hypoventilation syndrome at public and private in- and out - hospital departments in denmark were included.main variablesthe ndosa contains information about baseline characteristics , comorbidity , diagnostic procedures conducted , treatment ( continuous positive airway pressure and similar treatments , surgery , mandibular advancement devices , etc ) complications , and treatment status.descriptive datayearly report with indicators for diagnostic procedures , treatment , and follow - up comparing different departments was involved in the management of sleep apnea in denmark for the purpose of quality improvement.conclusionthe ndosa has proven to be a real - time national database using diagnostic and treatment procedures reported to the danish national patient registry . |
in a recent issue of critical care , van de louw and haouzi report on the effects of lethal hemorrhage on blood and tissue levels of hydrogen sulfide ( h2s ) . the role of h2s during hemorrhage is a matter of debate : while both inhaled h2s and intravenous sodium sulfide and sodium hydrosulfide improved survival [ 2 - 4 ] , other authors reported that sodium sulfide did not exert any beneficial effects . since hypoxic conditions decrease and supplemental vitamin b12 ( hydroxocobalamin ) increases ( due to the rise in oxidative capacity ) the rate of h2s metabolism , the authors hypothesized that hemorrhage would increase plasma and tissue h2s levels , and that vitamin b12 would improve survival . rats were hemorrhaged by five times withdrawal of 5 ml / kg blood ( that is , approximately 30% of the calculated blood volume ) . the total h2s content was measured in the first and last blood samples , using the methylene blue assay . indirect calorimetry for oxygen uptake and carbon dioxide production before and at the end of the hemorrhage period allowed determination of the shock - induced oxygen deficit . the major finding was that , despite a severe cumulative oxygen debt ( 100 to 140 ml / kg ) , h2s blood and tissue concentrations did not change , rendering them useless as markers of shock severity . in line with this finding , vitamin b12 failed to exert any therapeutic effects despite an increased capacity to oxidize h2s . what do we learn from this study ? according to the authors ' standard curve for the methylene blue assay , this absorbance , however , was due to turbidity rather than the presence of the blue dye . the true h2s concentrations were most probably therefore much lower , possibly even below the detection limit of 1.5 m . there is considerable discrepancy in the literature on blood h2s concentrations . in rats , baseline values of 25 to 50 m have been reported , which increased up to 80 m after hemorrhage , endotoxin exposure and injection of sulfide donors . however , bolus ( 4 mg / kg ) or continuous intravenous ( 20 mg / kg / hour ) sodium sulfide only increased blood h2s levels from 0.4 to 0.9 m to 4.0 to 4.5 m when the monobromobamine assay was used to determine h2s concentrations . in mice , 10 mg / kg endotoxin either decreased ( from ~2.3 to ~1.8 m ) or increased ( from ~34 to ~65 m ) the blood sulfide content . finally , inhaling up to 200 ppm gaseous h2s in mice increased the sulfide content by < 1.5 m . according to the available literature , the blood h2s content may vary by three orders of magnitude - so which h2s concentrations are real ? at physiological ph , dissolved h2s gas represents 20 to 50% of the total sulfide , which can of course escape into the headspace . furthermore , blood - borne h2s is rapidly bound and/or metabolized : using a polarographic sensor with a detection limit for h2s gas corresponding to 100 nm total sulfide in blood at ph 7.4 , a 10 m sodium sulfide spike only transiently increased sulfide from undetectable levels to about 0.5 m . finally , the odor threshold of h2s is 0.01 to 0.3 ppm , and simply smelling the blood allows one to verify that plasma h2s concentrations are at , or below , 1 m : in a phosphate buffer , the human nose can detect as little as1 m h2s . the gas / water coefficient of distribution for h2s is 0.39 . assuming that only 20% of the dissolved gas ( that is , 4 to 10% of the total free sulfide ) disappears from the blood sample due to volatilization during the 2 or 3 seconds of sniffing , a 10 ml blood sample would have a total free sulfide concentration of 20 to 50 m . this is within the range reported for rat blood , but clearly rat blood does not smell like rotten eggs ! based on the results of their study , the authors conclude that ' h2s in the blood can not be used as a marker of hemorrhagic shock ' , because any h2s accumulation resulting from ' tissue hypoxia must be reconciled with the ability of tissues to oxidize h2s ' . clearly , h2s consumption via the sulfide quinone reductase system is reduced under hypoxic conditions , which in turn would cause h2s accumulation : in lung tissue homogenates and pulmonary artery smooth muscle cells , h2s consumption dropped at oxygen concentrations of 10 m ( that is , ~8 mmhg ) - a 50% drop occurring at oxygen partial pressure values of ~4 mmhg . in the present study , at the end of the hemorrhage the arterial oxygen partial pressure was still normal and , despite a 50% reduction of minute ventilation , unfortunately , the authors only reported lactatemia , and not ph , so the severity of lactic acidosis can not be definitively estimated . mild hypothermia of 34c is well established to attenuate lactic acidosis and better preserve tissue oxygenation . consequently , albeit ultimately lethal , the authors ' model might have been unable to detect the hypoxia threshold necessary to cause h2s accumulation . in this context , it is tempting to speculate whether any local h2s accumulation might decrease energy expenditure and thereby oxygen consumption due to inhibition of cytochrome c oxidase , which in turn would restore the oxygen partial pressure and thereby at least partially resume h2s oxidation . furthermore , it is noteworthy that h2s can even sustain atp synthesis during mild hypoxia , and thus presumably attenuate tissue lactic acidosis . van de louw and haouzi meticulously conducted a study in a " hot " field . moreover , lacking any observable increase in h2s levels during hemorrhage coupled with the currently available knowledge on the role of cystathione--lyase for oxygen sensing and vascular homeostasis , blocking endogenous h2s production most probably has little therapeutic benefit and may actually prove to be contra - indicated . additional studies are mandatory on the exogenous h2s supplementation during hemorrhagic shock , in order to answer the question of whether h2s is an innocent bystander or a central player under these conditions . supported by the deutsche forschungsgemeinschaft ( klinische forschergruppe 200 ' die entzndungsantwort nach muskulo - skeletalem trauma ' ) and by national science foundation grant number ios-1051627 . | the role of the gaseous mediator hydrogen sulfide ( h2s ) in hemorrhagic shock is still a matter of debate .
this debate is emphasized by the fact that available literature data on blood and tissue h2s concentrations vary by three orders of magnitude , both under physiological conditions as well as during stress states .
therefore , in a rat model of unresuscitated , lethal hemorrhagic shock , van de louw and haouzi tested the two hypotheses of whether blood and tissue h2s levels would increase due to the shock - related tissue hypoxia , and whether vitamin b12 would attenuate organ injury and improve survival as a result of enhanced h2s oxidation .
hemorrhage did not affect the blood and tissue h2s content , and , despite the increased capacity to oxidize h2s , vitamin b12 did not affect any parameter of shock severity .
the authors concluded that h2s concentrations can not be used as a marker of shock , most probably as a result of tissue 's capacity to oxidize h2s even under conditions of severe oxygen debt .
this research paper elegantly re - adjusts the currently available data on blood and tissue h2s levels , and thereby adds an important piece to the puzzle of whether h2s release should be enhanced or lowered during stress conditions associated with tissue hypoxia . |
actinomycosis is a rare chronic , granulomatous , suppurative , and progressive infectious disease , which is characterized by the development of sinuses that may secrete sulfur granules . this is a non - spore gram- positive , anaerobic bacterium that is universally distributed . this bacterium is not very virulent and frequently arises from cervicofacial mucosa ( 50% ) , abdominal ( 20% ) , and thoracic ( 15% ) areas that cause opportunistic infections . patients with immunosuppressive state such as recipients of chemotherapy or organ transplant are at risk of opportunistic infections and morbidity and mortality . nevertheless , primary bowel involvement has not been reported frequently . in this context based on previous reports , abdominal actinomycosis can imitate the presentation of other abdominal pathologies such as malignancy , abscesses , inflammatory bowel disease ( ibd ) , and diverticulitis . herein we report a case of primary sigmoid colon actinomycosis in an otherwise healthy 53-year - old lady . a 53-year - old woman presented with 6 months history of generalized vague abdominal pain , bloating , and a few episodes of bloody - mucous rectal discharge with change of bowel habit . she had never used intrauterine devices and reached menopause at the age of 48 years . physical examination was unremarkable except for mild generalized abdominal tenderness without rebound or involuntary guarding . the laboratory examinations revealed a mild normocytic anemia ( hemoglobin=11.7 gr / dl with mcv=82 fl , c - reactive protein ( crp ) was in the normal range , and erythrocyte sedimentation rate ( esr ) was 13 mm / hr . abdominal and pelvic ultrasonographic studies were also normal . colonoscopy showed a polypoid mass like lesion in the distal part of the sigmoid colon with some amount of necrotic material around the lesion ( fiures 1 , 2 ) . it was located 20 cm proximal to the anal verge , above the rectosigmoid junction . microscopic examination of the specimen revealed ulcerated and slightly inflamed large intestinal mucosa with polypoid inflammatory granulation tissue , and a small amount of fibrin purulent exudates , sometimes enclosing bacterial colonies in forms of branching filaments morphologically compatible with actinomyces ( fiures 3 , 4 ) . the polypoid mass like lesion in the distal part of the sigmoid colon the polypoid mass like lesion in the distal part of the sigmoid colon the histopathological features of the lesion the histopathological features of the lesion
after confirming the diagnosis , intravenous penicillin ( 20 million iu / day ) was prescribed . the patient received intravenous treatment for 10 days and continued with oral penicillin v ( 2 gr / day ) for next six months . she was discharged after ten days and her complaints and colonic mass resolved totall ( fiures 5 , 6 ) . the first paper ever reported on abdominal actinomycosis was published more than 80 years ago . this organism is considered as a normal flora of the digestive , respiratory , and female genital systems . as a result any processes that makes a breach in the normal mucosa such as surgery or trauma can be a trigger for pathologic proliferation of actinomycosis , although the pathophysiology of this phenomenon is not fully known . usual predisposing factors include immune suppression , surgery , diverticulitis , foreign body , and bowel perforation . abdominal actinomycosis is not a common clinical issue having a non - specific clinical presentation . it always presents as a slow growing mass that alters the bowel habits , and is accompanied by abdominal pain and cramps , and constitutional symptoms . abdominal mass detected either clinically or radiologically is a usual finding that mimics other abdominal pathologies . digestive actinomycosis seldom presents as an abdominal abscess with or without a discharging sinus into the abdominal or perianal sites . the most frequently encountered laboratory findings are anemia , leukocytosis , and positive inflammatory markers . although the involvement of the left side of the colon is rare , right side of the colon and the ileocecal involvement is also common . the efficacy of colonoscopy for the diagnosis of extramucosally originating actinomycosis is low but it is usually used to exclude inflammatory or neoplastic conditions . however , in the presence of a mass in intestinal lumen , histological evaluation of endoscopically removed specimen can confirm the diagnosis . computed tomography ( ct scan ) is an applicable modality for detection of a mass and to explore the adjacent area but for actinomycosis it is not diagnostic . nevertheless , ct - guided drainage of an abscess can give an appropriate sample for identification of the organism . usually the findings include mural invasion along with stricture formation or presence of a mass in the lumen with thickenings of the mucosa . it should be mentioned that sulfur granules can be observed in other infections caused by nocardia and some types of staphylococci . definitive diagnosis rests on the specimen culture or the use of specimen - specific antibodies but this is not easy to perform in the clinical setting .
in our presented case , actinomycosis had affected an area 20 cm above the anal verge , which is a very rare incident . in addition , the clinical and laboratory findings only revealed mild lower gastrointestinal symptoms with normocytic anemia . all of them make this case almost unique compared with previous reports . while in previous reports the abdominal actinomycosis usually tends to express itself in a more prominent clinical picture and elevated serum levels of inflammatory markers that resemble other abdominal pathologies such as malignancy , ibd , or tuberculosis . if the diagnosis can be confirmed without surgery and the patient does not experience complications , non - surgical treatment with a high dose penicillin is the first choice .
in conclusion , colonic actinomycosis should be kept in mind as a rare differential diagnosis of abdominal mass , with either tumoral or inflammatory characteristics . colonoscopy and histopathological study of the removed specimen can be considered as the first choice modality for the diagnosis of colonic actinomycosis . high index of suspicion leads to an immediate and accurate diagnosis by clinicians and can prevent unnecessary surgical intervention . | actinomycosis is a rare and chronic infectious disease caused by a non - spore gram- positive , anaerobic bacterium that rarely infects the colon , in particular the left colon.a 53-year - old woman was referred to us due to chronic abdominal pain , bloating , a few episodes of bloody - mucous rectal discharge , and change of bowel habits .
her medical history and physical examination were unremarkable .
colonoscopy revealed a polypoid mass like lesion located 20 cm proximal to the anal verge above the rectosigmoid junction .
several biopsy samples were taken .
histopathological evaluation showed actinomycosis infection .
consequently the patient was treated with intravenous and then six months oral penicillin .
her complaints and colonic mass resolved totally .
diagnosis of colonic actinomycosis is not an easy task .
it is advisable to keep this infection in mind among the differential diagnoses of unusual abdominal masses .
colonoscopy and histopathological examination can be the preferred modality for diagnosis of colonic actinomycosis infection . |
colchicine is a widely used drug for treatment of familial mediterranean fever ( fmf ) . clinical manifestations of colchicine intoxication include abdominal cramps , diarrhea , myotoxicity , hemolytic anemia and ( pan)cytopenia . a 9-years - old female patient , receiving colchicine for four months with a dose of 1 mg / day for fmf , was admitted to a hospital with gastrointestinal disturbance four days ago . she was referred to our hospital with liver and kidney dysfunction . at the initial physical examination her laboratory results revealed : hemoglobin 13.6 g / dl ; leukocyte , 9.610/l ; thrombocyte 5710/l ; fibrinogen 157 mg / dl ( 230 - 500 ) ; pt 26.1 sec ; inr 2.3 ; aptt 43.8 sec ; ldh 5329 iu / l ; ferritin 2320 g / l ( 10 - 55 ) ; triglyceride 7.1 mmol / l ( 0.32 - 1.46 ) . the patient was taken to intensive care unit ; vitamin k and fresh frozen plasma were administered . on the second day of her hospitalization hemoglobin was 9.6 g / dl ; leukocyte was 2.310/l ; neutrophil was 0.7210/l and thrombocyte was 2410/l . on peripheral blood smear , loss of lobulation in neutrophils was detected ( pelger - hut anomaly ) . laboratory tests revealed ferritin 54,632 g / l , triglyceride 7.4 mmol / l , and fibrinogen 63 mg / dl , serum creatinine 3.2 mg / dl ( 0.4 - 1.4 ) , serum blood urea nitrogen 43 mg / dl ( 6 - 21 ) , serum sodium 131 meq / l ( 134 - 148 ) , potassium 5.2 meq / l ( 3 - 4.8 ) , calcium 8.1 mg / dl ( 7.9 - 9.9 ) , and inorganic phosphate 2.1 mg / dl ( 2.4 - 4.7 ) . at the bone marrow aspiration , many pelger - hut cells were observed . soluble cd25 level was 2840 u / ml ( 220 - 710 ) , and creatine kinase level was 18,959 when the history of colchicine intoxication was detailed , during fmf attack - free period of five days prior to admission to the hospital , it is learned that the patient was upset over a quarrel between her parents , and that she had taken approximately 30 pills of her drug ( total of 15 mg ; 0.5 mg / kg body weight ) , at which vomiting and diarrhea complaints were started . the patient was administered hlh-2004 protocol and plasma exchange is applied twice . on the second day of therapy , severe hypotension , renal failure and av complete block occurred . the patient was resistant to all therapies and had died at the fourth day of her hospitalization . in the colchicine intoxication , the most affected organs are those that have a rapid cell turnover . liver damage , renal failure , arrhythmias , neuromuscular disturbances , and bone marrow depression can be detected in the later stage . the worst predicted doses were classified regarding the risk of toxicity as sub - toxic ( < 0.5 mg / kg ) , toxic ( 0.5 - 0.8 mg / kg ) , and lethal ( > 0.8 mg / kg ) doses . however , cases with cardiogenic shock and death were also reported with doses less than 0.5 mg / kg . although our patient had taken a dose of 0.5 mg / kg body weight colchicine , the patient died due to severe myelosupression , hlh and multi - organ failure . pelger - hut anomaly is a state of limitation in segmentation of lobes in neutrophils . a pelger - hut - like change in granulocyte morphology may occur as an acquired condition in several diseases . this same finding may be produced by certain drugs such as colchicine . on the peripheral blood smear of our patient , pelger - hut cells were detected in myeloid series , and as the history of colchicine use was detailed , it is learned that the patient had taken lots of colchicine pills . on the examination of concurrently taken bone marrow , pelger - hut cells were also detected in the myeloid series as well as hemophagocytosis . for this reason , we suggest that hlh was not due to her disease . to our knowledge , this is the first reported case of a patient with hlh associated with colchicine intoxication . in conclusion , if patients with colchicine intoxication present with pelger - hut anomaly in addition with unexplained fever , cytopenia , organomegaly , and biochemical changes , hlh should be investigated for prompt diagnosis and treatment . | colchicine is frequently used in the treatment of familial mediterranean fever ( fmf ) .
first symptoms of colchicine intoxication are gastrointestinal disturbances , such as abdominal cramps , diarrhea , pancytopenia and so on .
herein , we report a female fmf patient with pancytopenia and hemophagocytic lymphohitiocytosis ( hlh ) , following colchicine intoxication for committing suicide . to our knowledge
, this is the first reported case of a patient with hlh associated with colchicine intoxication . |
in november 2006 , a 23-year - old woman referred to the department of dermatology of bologna university with a two - month - history of a softy painless nodule located on the right cheek ( figure 1 ) . physical examination revealed a yellowish lesion that showed a central ulceration measuring 2 cm across and infiltrating the sub - cutis . based on macroscopic appearance and anamnesis , a cutaneous leishmaniasis was suspected : in particular , the patient had spent 3 months on the sardinia coast where leishmania is endemic . however , neither cytological nor cultural examination did confirm such hypothesis . therefore , an incisional biopsy was performed under local anaesthesia that was fixed in formalin and embedded in paraffin , according to routine procedures . light microscopy showed that the nodule consisted of spindle - cells with slight cytological atypia , a moderate amount of eosinophilic cytoplasm , oval or elongated nuclei , and small but distinct nucleoli . some mitotic figures were present ( 3/10hpf ) , more numerous in the ulcerated areas . at immunohistochemistry , neoplastic elements expressed smooth - muscle actin but were negative for cd34 , cd68/pg - m1 and protein s-100 . the ki-67/mib-1 rate was low ( < 5% on average ) with the exception of a thin area surrounding the ulceration . according to macroscopic , histological and phenotypic findings the lesion was surgically removed : its examination confirmed the diagnosis . at four - year - follow up , cutaneous leiomyomas , first described in 1854 by rudolf virchow , are benign , smooth - muscle tumours . in particular microscopically , it corresponds to a non - encapsulated lesion , consisting of bland , interwoven fusiform cells with a central small nucleus . so far , cellular atypia has been reported only by harford et al . in a case that is somewhat reminiscent of our own observation . in particular , the present case confirms that , like leiomyomas of other anatomic sites , piloleiomyoma can at times be provided with features of cellular atypia , a fact that suggests to periodically follow - up the patient because of the risk , although low , of recurrences . interestingly , our patient has so far remained in complete remission , a finding that is of interest since no information on the clinical outcome were provided by harford and co - workers . in addition , our case unusually presented with an ulcerative lesion in the absence of spontaneous or evoked pain . this along with the anamnestic data initially led to suspect cutaneous leishmaniasis . it was the biopsy which allowed the correct diagnosis that was validated by immunohistochemistry . based on our observation , we think that clinicians should consider atypical leiomyoma in case of a non - healing ulcer with bizarre clinical features . | piloleimyoma is a type of cutaneous leiomyoma arising from arrectores pilorum .
it can present as either a solitary nodule or multiple lesions .
usually it is localized on the extremities , but can also occur on trunk , neck area and face .
lesions are usually cold , ulcerated and spontaneously painful .
herein we report a case of piloleiomyoma with cytological atypia that was painless despite the presence of central ulceration . |
the advent of laparoscopic cholecystectomy in the early 1990s led to a paradigm change and a shift from open approach towards minimally invasive techniques . meanwhile , the laparoscopic cholecystectomy is the most common laparoscopic procedure in general surgery and considered to be the gold standard in the treatment of symptomatic cholelithiasis and acute / chronic cholecystitis . the laparoscopic technique results in lower postoperative pain , shorter hospital stays and a proper cosmesis . in times before the laparoscopic era the incidence of biliary injuries after conventional open cholecystectomy amounted 0.2% . however , despite of contemplated advantages , a rapid learning curve and constant improvements in methodology , the complication rates of bile duct injuries after laparoscopic cholecystectomy count from 0.4% to 0.5% , dependent on the underlying disease and remain higher than in the open approach . the most common cause of serious biliary injury is misidentification . due to abovementioned significant divergence between open and laparoscopic procedures strasberg and colleagues in 1995 first suggested a three - pronged strategy called the critical view of safety ( cvs ) , to minimize the risk of bile duct injuries in laparoscopic cholecystectomy . this technique follows three principles : ( 1 ) dissection of the triangle of calot from all fatty and fibrous tissue , ( 2 ) mobilization of the lowest part of the gallbladder from its bed and ( 3 ) the unambiguous identification of two and exclusively of two structures ( cystic duct , artery cystica ) entering the gallbladder [ fig . 1 ] . in the following case report we highlight the importance of the cvs in laparoscopic cholecystectomy and its classical pitfalls the patient suffered from coronary heart disease and benign prostatic hyperplasia , there was no history of relevant pre - existing surgical conditions . after blood analysis revealed following results : pancreatic amylase : 1214 u / l [ normal range : 1353 u / l ] , -lipase : 2619 u / l [ 1360 u / l [ 1071 u / l ] and abdominal ultrasound showed presence of gallstones in the gallbladder and dilatation of the common bile duct a magnetic resonance cholangiopancreatography ( mrcp ) was performed . following the diagnosis of choledocholithiasis a papillotomy with stone extraction via endoscopic retrograde cholangiopancreatography ( ercp ) was executed . the patient signed a patient consent form and laparoscopic cholecystectomy was carried out in a classical four - port technique . after establishment of a pneumoperitoneum and installation of all four ports , a shrunk gallbladder was found [ fig . 2 ] . first , the gallbladder was grasped in the usual way at the fundus and pulled into the right upper abdomen , followed by dissection of the triangle of calot [ fig . 3 ] . despite adequate preparation and removal of all fatty and fibrose tissue only one structure entering the gallbladder initially this formation was interpreted as the cystic duct . however , on the one hand the putative cystic duct appeared of strong calibre , on the other hand a successful lateralization of the gall bladder could not be performed . to provide clear anatomical conditions , the gall bladder was entirely dissected from its bed in a fundus first-approach [ fig . 5 ] . preparation was continued and finally the cystic duct , showing a long - segment adhesion with the common bile duct was properly identified in terms of a cvs , secured with clips and divided . following haemostasis of all bleeding sites , inspection of the dissected cystic artery and cystic duct , retrieval of the gallbladder and drainage insertion nowadays laparoscopic cholecystectomy is regarded as the gold standard in the treatment of gallbladder diseases . according to german census bureau an initial rate of bile duct injuries from 0.74% to 2.8% at the onset of the laparoscopic era , could be reduced steadily to about 0.4% nowadays . nonetheless , despite permanent increase in learning curves and new approaches , bile duct injuries still remain twice as frequent as in the conventional open approach . the introduction of strasbergs cvs could not approximate the original 0.2% rate of bile duct injuries . howbeit data show , that a consequent use of cvs technique may prevent some biliary injuries , there is no level i evidence that this technique prevents bile duct injuries in general , as there are no randomized trials published up to date . the question remains to what extent we surgeons are willing to accept complications when adopting new surgical techniques . considering minimally - invasive procedures , the assumed benefit of lesser trauma to the patient always has to be balanced against such undisputable higher complication rates compared to an open approach , no matter how low the total event rated may be . like in the case with cvs , strict adherence to such safety nodal points throughout the procedure may help narrowing the gap between complication rates of open and minimally invasive procedures into acceptable bounds . this subject , which has given rise to considerable debate , is a very crucial one , as minimally - invasive methods more and more replace or supplement traditional open surgical approaches . in the case presented , transection of the common bile duct was prevented through critical examination of the cvs . instead , we recommend to use it as a framework , which shall help the surgeon to re - evaluate each surgical step before proceeding . injuries of the common bile duct are the most frequent bile duct injuries described in literature ranging from 66% to 72% of all bile duct lesions . compliance with all three criteria of the cvs may prevent inadvertent bile duct injuries , as it indicates reliable exposure and identification of all structures in the triangle of calot . only 1/3 of all injuries can be treated by ercp and stenting . in 2/3 of all patients stephan b. archer and colleagues estimated a total annual economic damage of approximately $ 40 million in the u.s . at 600700 bile duct injuries . the cvs certainly offers not a full protection to avoid biliary lesions , but may lead to a significant risk minimization when consistently implemented . unfortunately , it can be assumed that probably misperception , rather than technical errors are the leading cause of biliary injuries , as most bile duct lesions occur to experienced surgeons ( > 200 cholecystectomies performed ) . therefore , we believe that a consistent adherence of established guidelines in surgery is of particular importance . the rate of bile duct injuries after laparoscopic cholecystectomy averages around 0.4% and can lead to far - reaching consequences for the patients in the postoperative course . the critical view of safety can be used as a safe tool to prevent bile duct injury and classical pitfalls under critical evaluation of the surgical process . an adequate mobilization of the gallbladder from its bed and unambiguous identification of the structures entering the gallbladder shall always be ensured . written informed consent was obtained from the patient for 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 on request . | highlightsmisperception of intraoperative anatomy is one of the leading causes of bile duct injuries.the critical view of safety in laparoscopic cholecystectomy serves the unequivocal identification of the cystic duct before transection.a sufficient mobilization of the gallbladder from its bed is essential in performing the
critical view of safety in laparoscopic cholecystectomy . |
preparation of solid dispersion of domperidone : domperidone inclusion complexes were prepared with -cd in different ratio ( 1:1 , 1:2 and 1:3 ) by kneading method . preparation of fast dissolving films : solid dispersion of domperidone and -cyclodextrin ( 1:3 ) was selected and dispersed in half quantity of water and to it methanol is added along with tween-80 and heated at 60c . in other half quantity of water , hpmc e15 it is dried in oven at 60c for 5 hrs to obtain the film . morphological properties : properties such as homogeneity , color , transparency and surface of the oral films were evaluated by visual inspection.film mass : the mass of three films were determined by an analytical balance and means.d was calculated.film thickness : film thicknesses were measured at five positions ( central and the four corners ) using the digital vernier caliper and the mean thickness was calculated.folding endurance study : it was measured manually by repeatedly folding a film at the same place till it broke.in vitro disintegration studies : the film as per the dimensions ( 3 2 cm ) required for dose delivery was placed on a stainless steel wire mesh placed in a petridish containing 10 ml phosphate buffer ph 6.8 . time required for the film to break was noted as in vitro disintegration time.dissolution and drug release : dissolution test of films was performed using ( 900 ml ; phosphate buffer ph 6.8 with usp dissolution apparatus ii at 50 rpm and 370.5c temperature . the drug release was analyzed spectrophotometrically at max 286.5 nm using ultraviolet ( uv ) spectrophotometer ( shimadzu model no : 1800 ) . morphological properties : properties such as homogeneity , color , transparency and surface of the oral films were evaluated by visual inspection . film mass : the mass of three films were determined by an analytical balance and means.d was calculated . film thickness : film thicknesses were measured at five positions ( central and the four corners ) using the digital vernier caliper and the mean thickness was calculated . folding endurance study : it was measured manually by repeatedly folding a film at the same place till it broke . in vitro disintegration studies : the film as per the dimensions ( 3 2 cm ) required for dose delivery was placed on a stainless steel wire mesh placed in a petridish containing 10 ml phosphate buffer ph 6.8 . dissolution and drug release : dissolution test of films was performed using ( 900 ml ; phosphate buffer ph 6.8 with usp dissolution apparatus ii at 50 rpm and 370.5c temperature . the drug release was analyzed spectrophotometrically at max 286.5 nm using ultraviolet ( uv ) spectrophotometer ( shimadzu model no : 1800 ) . morphological properties : properties such as homogeneity , color , transparency and surface of the oral films were evaluated by visual inspection.film mass : the mass of three films were determined by an analytical balance and means.d was calculated.film thickness : film thicknesses were measured at five positions ( central and the four corners ) using the digital vernier caliper and the mean thickness was calculated.folding endurance study : it was measured manually by repeatedly folding a film at the same place till it broke.in vitro disintegration studies : the film as per the dimensions ( 3 2 cm ) required for dose delivery was placed on a stainless steel wire mesh placed in a petridish containing 10 ml phosphate buffer ph 6.8 . time required for the film to break was noted as in vitro disintegration time.dissolution and drug release : dissolution test of films was performed using ( 900 ml ; phosphate buffer ph 6.8 with usp dissolution apparatus ii at 50 rpm and 370.5c temperature . the drug release was analyzed spectrophotometrically at max 286.5 nm using ultraviolet ( uv ) spectrophotometer ( shimadzu model no : 1800 ) . morphological properties : properties such as homogeneity , color , transparency and surface of the oral films were evaluated by visual inspection . film mass : the mass of three films were determined by an analytical balance and means.d was calculated . film thickness : film thicknesses were measured at five positions ( central and the four corners ) using the digital vernier caliper and the mean thickness was calculated . folding endurance study : it was measured manually by repeatedly folding a film at the same place till it broke . in vitro disintegration studies : the film as per the dimensions ( 3 2 cm ) required for dose delivery was placed on a stainless steel wire mesh placed in a petridish containing 10 ml phosphate buffer ph 6.8 . dissolution and drug release : dissolution test of films was performed using ( 900 ml ; phosphate buffer ph 6.8 with usp dissolution apparatus ii at 50 rpm and 370.5c temperature . the drug release was analyzed spectrophotometrically at max 286.5 nm using ultraviolet ( uv ) spectrophotometer ( shimadzu model no : 1800 ) . all the formulation contained varied amount of polymer and hence thickness of each film was varied between the ranges of 0.14 mm-0.36 mm . when the concentration of hpmc e15 is increased from 5%-10% , thickness of the strip increased . folding endurance was measured manually and it was found to decrease with increase in the concentration of film forming polymer hpmc e15 . the surface ph of all the formulation was found to be in the range of 6.8 - 7.2 and hence will not cause any irritation to oral mucosa . f1 formulation found to give minimum disintegration time ( 45 sec ) as compared to other formulations . the drug release rate decreases as the concentration of the film forming agent hpmc e15 increases in the formulation . after 15 min interval more than 75% of drug was released from batches as defined in the guidances such as usp30 . films were prepared of domperidone--cyclodextrin solid dispersion by the use of hpmc e15 as a film forming agent and peg-400 as a plasticizer . domperidone mouth dissolving films were prepared successfully by the use of solid dispersion of domperidone with -cyclodextrin and it can be used to treat emesis caused by various conditions in geriatric , bedridden and non - cooperative patients due to its ease of production . | the present investigation was undertaken with the objective of formulating mouth dissolving film(s ) of the antiemetic drug domperidone to enhance the convenience and compliance by the elderly and pediatric patients .
domperidone is a drug of choice in case of nausea and vomiting produced by chemotherapy , migraine headaches , food poisoning and viral infections .
it causes dopamine ( d2 and d3 ) receptor blockage both at the chemoreceptor trigger zone and at the gastric level .
it shows high first pass metabolism which results in poor bioavailability ( 10 - 15% ) . in view of high first pass metabolism and short plasma half - life
it is an ideal candidate for rapid release drug delivery system .
the solid dispersions of domperidone were prepared with the use -cyclodextrin in various ratios ( 1:1 , 1:2 , 1:3 ) and solubility study was performed to determine the ratio in which solubility of domperidone was highest ( 1:3 ) .
the selected solid dispersions were then utilized for the preparation of film by solvent casting method utilizing hpmc e15 as a film forming agent and peg-400 as plasticizer .
five formulae were prepared and were evaluated for their in vitro dissolution characteristics , in vitro disintegration time , and their physico - mechanical properties .
the promising film ( f1 ) showed the greatest drug dissolution ( more than 75% within 15 min ) , satisfactory in vitro disintegration time ( 45 sec ) and physico - mechanical properties that are suitable for mouth dissolving films . |
in this issue of critical care , shorr and colleagues tested the potential value of surrogate markers for the treatment of patients with severe sepsis with activated protein c , also known as drotrecogin alfa ( activated ; drotaa ) , using data from the prowess ( recombinant human activated protein c worldwide evaluation in severe sepsis ) and enhance ( extended evaluation of recombinant activated protein c ) clinical trials . prowess was a double - blind , randomized clinical trial of drotaa for the treatment of severe sepsis , which identified a mortality benefit of treatment with drotaa in patients at a high risk for death . enhance was a subsequent open - label trial of drotaa in severe sepsis that was designed to confirm the findings of prowess and to provide additional data on drug safety . using a biomarker as a surrogate end - point in a clinical trial is challenging but potentially innovative and valuable . whereas definitive phase iii clinical trials that are based on mortality are large and expensive , phase ii clinical trials that are powered to test differences in surrogate end - points can provide safety and efficacy data that determine whether a large phase iii trial is indicated . in general , a biomarker can be considered to be a reasonable surrogate end - point if changes in the biomarker predict changes in a clinical end - point , such as death . thus , the degree to which a surrogate end - point is influenced by a given therapy should correlate with the influence of that treatment on the disease of interest . however , caution must be used when considering surrogate end - points . in a classic example , although antiarrhythmic drugs reduce cardiac arrhythmias , these agents were subsequently associated with an increased rather than decreased risk for death in cast ( cardiac arrhythmia suppression trial ) . if the clinical outcome of interest is influenced by several different factors in addition to the surrogate end - point , then the surrogate marker may not be a valid surrogate end - point . consequently , freedman and colleagues recommended that a valid surrogate end - point should explain at least 50% of the impact that a therapy has on the outcome of interest . because plasma protein c levels are low early in the course of severe sepsis and then rise in those who recover and survive , shorr and colleagues hypothesized that protein c might be a good surrogate end - point ( type 2 biomarker ) for treatment of sepsis with drotaa . because several other biomarkers had been measured in these cohorts , those investigators also explored the potential value of interleukin-6 , antithrombin iii , prothrombin time , protein s , and d - dimers . for this analysis , they used the conceptual framework for biomarkers proposed by vasan . in this model , a type 0 biomarker is defined as ' a marker of the natural history of the disease and correlates longitudinally with known clinical indices . ' the authors tested the association of these six biomarkers with 28-day mortality and selected cut - offs for biomarker levels based on sensitivity and specificity analyses . however , even at the optimum cut - off , baseline levels of all six biomarkers , including protein c , exhibited poor discriminant function for death , with areas under the receiver operator characteristic curve ranging from 55% to 60% . the authors next examined whether any of these biomarkers could identify a subgroup of patients in whom a greater treatment benefit was observed . the cohort was divided into groups with a higher and lower risk for death , based on the baseline biomarker cut - off levels . the relative risk for death was compared in those treated with drotaa in each of these two risk groups . of the biomarkers studied , the only significant difference was in the protein c group . in patients with lower baseline plasma protein c levels ( who were at greater risk for death ) , finally , to test whether these biomarkers could serve as a potential surrogate end - point by predicting clinical benefit , the authors found that 57% of the drotaa effect was explained by the change in protein c levels . the authors also demonstrated that in drotaa - treated patients , a higher proportion of individuals had normal protein c levels ( > 80% of normal ) at the end of the drug infusion compared with control individuals , and that survival in those with normal protein c levels was higher in those patients treated with activated protein c , as compared with control individuals . to be a valid surrogate end - point , there should be some plausible mechanism by which treatment influences the surrogate end - point . the mechanism by which drotaa influences protein c levels is not entirely clear but may be due to decreased consumption or increased hepatic production of endogenous protein c ; the proposed mechanisms should be explored in future studies . based on the data and analysis presented in the report by shorr and colleagues , what is the potential value of protein c as a surrogate end - point for treatment trials using drotaa ? the authors propose several potential but untested benefits , including identification of patients with severe sepsis who are most likely to benefit from treatment with drotaa as well as to monitor patient response to therapy . the use of protein c as a surrogate end - point may allow tailoring of infusion length or drug dose to an individual patient ; rather than treating patients with a fixed dose of drotaa for a fixed period of time , it might be possible to tailor the infusion to normalize protein c levels by 96 hours . indeed , the respond ( research evaluating serial pc levels in severe sepsis patients on drotaa ) study is examining the safety of higher doses of drotaa ( up to 48 g / kg per hour ) and longer infusion times ( up to 7 days ) , and the efficacy of these infusions for normalization of protein c levels . we agree that these are clinical scenarios in which protein c may have value as a surrogate end - point . however , the true test of protein c as a surrogate end - point will depend on demonstrating that normalization of plasma protein c levels by drotaa correlates with patient benefit in future studies , including the prowess - shock trial . drotaa = drotrecogin alfa ( activated ) ; enhance = extended evaluation of recombinant activated protein c ; prowess = recombinant human activated protein c worldwide evaluation in severe sepsis . | identification of good surrogate end - points can greatly facilitate the design of clinical trials . using data from prowess and
enhance , shorr and colleagues explore the potential value of several plasma biomarkers for treatment trials of activated protein c for severe sepsis .
based on the framework proposed by vasan , they tested the utility of several factors ( protein c , interleukin-6 , antithrombin iii , prothrombin time , protein s , and d - dimers ) as type 0 , 1 and 2 biomarkers .
only protein c had acceptable performance characteristics as a type 2 biomarker , or surrogate end - point .
the utility of protein c as a surrogate end - point for studies of severe sepsis must be validated in future prospective studies . |
there are basically three variants of primary localized cutaneous amyloidosis ( plca ) : macular , papular ( lichenoid ) and nodular forms [ 1 , 2 , 3 ] . in macular and papular plca , cytokeratins serve as the amyloid precursors , and these variants are limited to the dermis [ 1 , 2 , 3 , 4 ] . in contrast , the rare type of nodular plca originates from immunoglobulin light chains ; it may extend to the subcutaneous tissue , and blood vessel infiltration can also be observed [ 1 , 2 , 3 , 5 ] . furthermore , nodular plca can originate from systemic amyloidoses or progress to systemic disease . here , we present a rare case of nodular plca , which resembled a giant tumor on the head . a 79-year - old man presented with a large tumor on the left side of his head ( fig . the waxy tumor exhibited a sulcated , mostly yellowish and reddish surface , and in some areas hemorrhages . a punch biopsy showed amorphous eosinophilic material in the dermis , so that we suspected a giant amyloid tumor [ 1 , 2 , 4 ] . histology showed that nearly the entire dermis was filled with amorphous eosinophilic material ( fig . immunohistochemistry was negative for cytokeratin , but showed that the entire dermis and parts of the subcutis were filled with amyloid consisting of both kappa and lambda immunoglobulin light chains ( fig . , we performed fine - needle abdominal fat aspiration , and the samples showed only minimal traces of amyloid , thus no signs of systemic amyloidosis . therefore , the diagnosis was primary localized cutaneous nodular light chain amyloidosis . at 1-year follow - up the diagnosis of plca requires histological analysis of a skin specimen . in order to discriminate the amyloid precursors in plca , immunohistochemistry with antibodies against cytokeratin and immunoglobulin light chains ( lambda and kappa subtypes ) it is best to also use electron microscopic work - up of a sample fixed in karnofsky 's fixative ( glutaraldehyde ) . as amyloid fibrils are quite stable , electron microscopy can also be done from formalin - fixed samples ; however , image quality will be reduced with this standard fixative . the typical amyloid fibrils with diameters of 710 nodular plca can often be successfully treated surgically , e.g. via curettage , excision or ablative laser therapy ( co2 lasers etc . ) . patients should regularly be screened for progression to systemic disease as this may occur in 550% of cases . to do so , a full history and physical examination along with an electrocardiogram , complete blood count , serum creatinine level , serum liverassociated enzymes levels , serum protein electrophoresis and urine protein electrophoresis should be preformed . it has also been suggested that an abdominal fat biopsy ( easy access for screening ) be performed to rule out systemic disease . to our knowledge , this is the most extensive case of nodular plca reported in the literature . | a 79-year - old man presented with a large tumor on the left side of his head , which had grown over 5 years .
regional lymph nodes were impalpable and computed tomography revealed no signs of bone infiltration .
histology showed that the entire dermis was filled with amorphous eosinophilic material .
immunohistochemistry was negative for cytokeratin , but showed that the dermis and parts of the subcutis were filled with amyloid consisting of immunoglobulin light chains .
there were no signs of paraproteinemia or underlying plasmocytoma . in electron microscopy , the typical amyloid fibrils were found .
the tumor was completely removed via curettage . at 1-year follow - up
, the patient was doing fine with no signs of relapse or systemic disease . |
bardet - biedl syndrome ( bbs ) is a rare autosomal recessively inherited disease with prevalence rates of 1:140,0001:160,000 . higher incidence have been found in isolated populations of newfoundland and kuwait ( 1:1700 ) . this syndrome is characterized by cardinal features , namely central obesity , mental retardation , postaxial polydactyly , pigmentary retinopathy , hypogenitalism and renal involvement . renal abnormalities include renal dysplasia , fetal lobulation , cystic tubular disease , glomerular disease ( most commonly focal segmental glomerulosclerosis ) . here we report a rare case of bbs with steroid sensitive , probably minimal change nephrotic syndrome . a 10-year - old male , born of non - consanguineous marriage presented with generalized edema . the parent also complained of short stature , obesity , poor school performance , poor night vision and passing urine from under surface of penis and poor genital development . he had delayed developmental milestones , started walking at the age of 2 years and spoke bi - syllable word at 3 years of age . weight was 45 kg ( between 90 and 97 centile for age ) and height 121 cm ( <3 centile for age ) with body mass index of 30.73 . there was brachycephaly , large ears , mild retrognathia , high - arched palate , short and broad hands , post - axial polydactyly in upper limb and left lower limb , micropenis ( < 2.5 cm ) and small testis , hypospadias [ figure 1 ] , central obesity , and anasarca . his intelligence quotient , determined by stanford binet method ( indian adaptation by s. k. kulshrestha ) , was 70 . polydactyly , micropenis , small scrotum , hypospadias and distended abdomen his complete blood count was normal . urinalysis showed nephrotic range proteinuria ( 3 on dipstick test ) , leucocytes 12/high power field , and no erythrocytes , with spot urine protein - creatinine ratio of 3.5 . the blood urea was 26 mg / dl and serum creatinine was 0.5 mg / dl . the glomerular filtration rate was 99.2 ml / min/1.73 m. his serum cholesterol was 275 mg / dl . hormonal evaluation showed serum testosterone level of 0.06 ng / dl ( normal range = 2165 ng / dl ) . ultrasonography of whole abdomen showed that both kidneys were normal in size and location with intact corticomedullary differentiations , and bilateral increased echogenicity . no endocapillary proliferation or neutrophil exudation or segmental glomerular sclerosis was noted [ figure 2 ] . the renal biopsy findings were suggestive of minimal change disease . the minimal change nature of the glomerular pathology could not be confirmed as the biopsy specimen contained only seven glomeruli and electron microscopy was not carried out . immunofluorescence staining for iga , igg , igm , c3 , and c1q was negative . hematoxylin and eosin stained biopsy specimen of glomeruli he was treated with prednisolone and responded remarkably . the patient is in regular follow up for last 8 months and there is no relapse till date . renal abnormalities , both structural and functional , are common in bbs . some authors considered it as sixth cardinal feature . abnormal renal function leading to end stage renal disease is the most common cause of death in these patients . the known renal anomalies include dysplasia , unilateral renal agenesis , cystic tubular disease , fetal renal ectopia , vesicouretric reflux , calyceal clubbing , and blunting . tubular functional defect results in abnormal urine concentrating ability leading to nephrogenic diabetes insipidus and type 1 renal tubular acidosis . renal histology has revealed mesangial proliferative glomerulosclerosis , increase in mesangial matrix , and ultrastructural change in glomerular basement membrane and chronic interstitial nephritis . very rarely , a case of bbs with structural renal anomaly ( calyceal clubbing ) and nephrotic range proteinuria has been reported . the renal biopsy of this case yielded only seven glomeruli , and electron microscopy was not performed . in view of the same , focal segmental glomerulosclerosis that is why , in the present case , we found the term steroid - sensitive nephrotic syndrome more preferable to that of minimal change nephrotic syndrome . the presence of steroid - sensitive nephrotic syndrome in a case of bbs may be a feature of the syndrome or may just be an associated finding . these genes are known to be associated with cell structures called cilia and basal bodies . the present case report suggests that there seems to be an association between ciliopathy and podocytopathy ( presence of nephrotic range proteinuria ) ; more research is needed to explore the exact association . bbs1 , researchers found that patients with mutation in bbs6 , bbs10 , and bbs12 genes had more severe renal disease . the present case is unique also in the way that the nephrotic syndrome was the presenting feature in this case . on thorough evaluation , bbs was found to be the primary entity . to the best of our knowledge , no case of bbs with documented steroid - sensitive nephrotic syndrome has been reported from india . | bardet - biedl syndrome ( bbs ) is a rare autosomal recessive disorder characterized by postaxial polydactyly , retinitis pigmentosa , central obesity , mental retardation , hypogonadism , and renal involvement .
renal involvement in various forms has been seen in bbs .
cases with nephrotic range proteinuria not responding to steroid have been described in this syndrome . here
we report a case of bbs who presented with nephrotic range proteinuria .
the biopsy findings were suggestive of minimal change disease .
the child responded well to steroid therapy and remains in remission . |
in order to isolate neuraminidase inhibitors , the fruiting bodies of p. igniarius were extracted using methanol for 2 days at room temperature . the methanolic extract was concentrated under reduced pressure , and the resultant residue was partitioned twice with ethyl acetate . the ethyl acetate - soluble layer was subjected to sephadex lh-20 column chromatography ( pharmacia , uppsala , sweden ) eluted with methanol to yield two active fractions . one active fraction was further purified by sephadex lh-20 column chromatography eluted with 70% aqueous methanol to provide compound 1 . the other fraction was subjected to sephadex lh-20 column chromatography with 70% aqueous methanol as the eluting solvent to yield compound 2 . the chemical structures of compounds 1 and 2 were determined via electrospray ionization ( esi)-mass and proton nuclear magnetic resonance ( h nmr ) spectrum measurements . the molecular weight of compound 1 was 474 on esi - mass measurement , where it exhibited a quasimolecular ion peak at m / z 475 [ m+h ] . the h nmr spectrum of 1 in dmso - d6 demonstrated seven methine protons at 8.24 , 7.85 , 7.47 , 6.97 , 6.61 , 6.15 , and 6.09 and one methyl signal at 2.50 . based on literature review , compound 1 was identified as phelligridin e ; the molecular weight and h chemical shift values were in good agreement with those previously reported for phelligridin e . the chemical structure of compound 2 was also determined via esi - mass and h nmr spectrum measurements . the molecular weight of compound 2 was 594 on esi - mass measurement , and it demonstrated a quasi - molecular ion peak at m / z 595 [ m+h ] . the h nmr spectrum of compound 2 in dmso - d6 showed three aromatic methine signals assignable to a 1,2,4-trisubstituted benzene moiety at 7.17 ( d , j = 2.1 hz ) , 7.10 ( dd , j = 8.2 , 2.1 hz ) , and 6.79 ( d , j = 8.2 hz ) , two olefinic methine peaks attributable to a trans-1,2-disubstituted double bond at 7.54 ( d , j = 16.2 hz ) and 7.10 ( d , j = 16.2 hz ) , and seven methine singlets at 8.28 , 7.86 , 7.48 , 7.01 , 6.64 , 6.25 , and 6.02 . these spectral data were consistent with those of phelligridin g previously reported in the literature . therefore , compounds 1 and 2 were identified as phelligridin e and phelligridin g , respectively , as shown in fig . 1 . the inhibitory activity of compounds 1 and 2 against neuraminidases from recombinant rvh1n1 , h3n2 , and h5n1 influenza viruses was evaluated . neuraminidase inhibition assay was performed in 96-well plates as previously described , with some modifications . briefly , the substrate , 50 l of 0.2 mm munana ( 2-(4-methylumbelliferyl)--d - n - acetylneuraminic acid sodium salt ; sigma , st . louis , mo , usa ) , was mixed with 90 l of 50 mm tris buffer ( containing 200 mm nacl , 5 mm cacl2 , ph 7.5 ) at room temperature . ten microliters of the sample and 50 l of h1n1 neuraminidase ( 50 ng / ml ) were added to each well . the mixture was then recorded at the excitation and emission wavelengths of 365 nm and 445 nm , respectively , using a polar optima ( bmg labtech , ortenberg , germany ) . in the case of h3n2 and h5n1 neuraminidases , 25 mm mes buffer ( containing 500 mm nacl , 5 mm cacl2 , ph 6.5 ) and 50 mm mes buffer ( containing 500 mm nacl , 5 mm cacl2 , ph 6.5 ) were used , respectively , and h5n1 required an activation period of 24 hr at 37 before assay . compounds 1 and 2 showed h3n2 neuraminidase inhibition activity with an ic50 value of 6.7 m , respectively , in a dose - dependent manner . inhibitory activity of compounds 1 and 2 against h1n1 and h5n1 neuramanidases were also evaluated . these compounds significantly inhibited the h1n1 and h5n1 neuramanidases with ic50 values in the range of 8.0~1.0 m in a dose - dependent manner ( table 1 ) . to study the mode of inhibition , dixon plots were used to distinguish the mechanism of neuraminidase enzyme action and to confirm the inhibition constant ( ki ) . compounds 1 and 2 displayed noncompetitive inhibitory activity , as indicated by a decrease in vmax while km remained stable at increasing inhibitor concentrations . the inhibition constant ( ki ) values of compounds 1 and 2 were 7.1 0.3 and 6.9 0.3 , respectively ( fig . | during our ongoing investigation of neuraminidase inhibitors from medicinal fungi , we found that the fruiting bodies of phellinus igniarius exhibited significant inhibitory activity against neuraminidase from recombinant h3n2 influenza viruses .
two active compounds were isolated from the methanolic extract of p. igniarius through solvent partitioning and sephadex lh-20 column chromatography .
the active compounds were identified as phelligridins e and g on proton nuclear magnetic resonance ( 1h nmr ) and electrospray ionization mass measurements .
these compounds inhibited neuraminidases from recombinant rvh1n1 , h3n2 , and h5n1 influenza viruses , with ic50 values in the range of 0.7~8.1 m . |
infantile hemangiomas ( ih ) are benign tumors in the pediatric population with an incidence of up to 10% . they are usually not present at birth but undergo a rapid proliferation within the first weeks of life . this is followed by the plateau stage and then a slow involution of the lesion such that 60% of 4-year - olds and 76% of 7-year- olds will experience complete regression of their hemangiomas . a subset of ih that could be considered high risk and might require treatment would include multiple ih , ocular involvement , oropharyngeal and airway involvement , large or disfiguring lesions , and ulcerated hemangiomas . for high risk ih , corticosteroids have traditionally been the mainstay of treatment with variable response and significant side effects including hypertension , increased risk of infection , cushingoid appearance , and growth suppression . since 2008 , propranolol has become a favored first line treatment for ih . propranolol shows good efficacy , rapid improvement in the lesion and fewer severe adverse events compared to corticosteroids . nadolol is a beta - blocker that has similar mechanism of action as propanolol , but has less central nervous system penetration and is dosed twice rather than three times per day . an 11-week - old healthy baby girl presented to the dermatologist with a well - defined red plaque over her right face that had been getting progressively more visible over the last 7 weeks [ figure 1 ] . the baby 's parents had noticed a whitish - red mark over the patient 's cheek in the hours after her birth , which they attributed to trauma from vaginal delivery . the lesion developed a bruised appearance over the next 2 weeks , and then grew more noticeably red , well defined and thickened . ( b ) patient at 21 weeks of age , after 10 weeks of treatment physical exam revealed a well - defined 7 3 cm , soft , warm red plaque following a v2 distribution on the right side of the patient 's face affecting the right temple , zygoma , malar cheek and lower eyelid , compatible with an ih . due to concern over the facial v2 distribution , phaces syndrome , which can be associated with segmental ih , ophthalmology exam , ekg , abdominal ultrasound , echocardiogram , and mri of head were all normal . phaces refers to posterior fossa malformations , hemangiomas , arterial anomalies cardiac defects , eye abnormalities , sternal cleft and supraumbilical raphe syndrome . the patient was started on nadolol at 0.5 mg per kg , adding 0.5 mg per kg each week until the 4 week where a maintenance dose of 2 mg per kg was reached . there was marked decrease in the redness , vascularity , and elevation of the hemangioma , including over the lower eyelid [ figure 1 ] . ongoing improvement in the appearance of the hemangioma on nadolol was noted at age 22 weeks , with a plan to continue therapy until age 1 and then titrate slowly off the medication . many recent studies have shown propranolol to be highly effective in treating high - risk or disfiguring ih . potential advantages of nadolol over propranolol include its inability to cross the blood brain barrier , which may lead to decreased sleep disturbance and irritability , and a longer half - life with dosing twice a day . a small cohort - blinded study showed that nadolol might be somewhat more effective as a treatment for ih than propranolol . typically , few side effects are reported in patients receiving beta - blockers for ih . blood pressure , heart rate , and serum glucose should be monitored in infants receiving beta - blockers . sweating , shakiness , tachycardia , hypotonia , and hunger are early signs of hypoglycemia in infants . beta - blockers produce an immediate vasoconstrictive effect due to inhibition of adrenaline - mediated vasodilation . this leads to decreased erythema and softening of the hemangioma within a few treatments . during the proliferative stage of ih , beta - blockers are thought to decrease the expression of vascular endothelial growth factor ( vegf ) , thereby opposing aberrant angiogenesis . beta - blockers have been also theorized to decrease inhibition of apoptotic pathways in ih , which may hasten tumor involution . | infantile hemangiomas are common benign tumours of infancy affecting up to 10% of children .
they are typically not present at birth but undergo a rapid proliferation stage and then plateau in growth before resolving spontaneously .
recently , beta - blockers have been favoured over systemic corticosteroids for treatment of disfiguring or life - threatening infantile hemangiomas .
we present a case of an 11-week - old female with a 7 week history of an evolving hemangioma along a facial v2 distribution .
physical exam revealed a well - defined bright red plaque over the right zygoma and lower eyelid .
mri , echocardiograph , and liver ultrasound were normal .
patient was treated with nadolol and had a rapid and substantial regression of the hemangioma .
nadolol is an effective treatment option for disfiguring facial infantile hemangioma .
the use of beta - blockers as treatment offers clues into the pathogenesis of infantile hemangioma , which is not yet completely understood |
acute angle - closure glaucoma ( aacg ) occurs in predisposed individuals when the pupil is mid dilated . drugs with 1 adrenergic or anticholinergic effects can precipitate attacks of aacg mainly by mydriasis . this causes paralysis of ciliary muscle tone which causes aqueous outflow resistance through trabecular meshwork . atropine , adrenaline , ephedrine are the common drugs used during general anesthesia that precipitate angle closure . these drugs are commonly used when there is airway obstruction or buckling of the endotracheal tube . postoperatively aacg was reported in patients after general anesthesia for abdominal , orthopedic , facial and endoscopic surgery . in predisposed individuals ( hypermetropia , shallow anterior chamber , thick lens ) anticholinergic drugs such as atropine , scopolamine and muscle relaxants and adrenergic drugs such as ephedrine , epinephrine can precipitate angle closure attack . moreover , the preoperative period causes the risk of psychological stress and darkness induced mydriasis might increase the risk of glaucoma attack . if anesthetists perform oblique pen torch test to assess the depth of the anterior chamber during preanesthetic evaluation , angle closure attack can be managed better in patients at risk . in the present case , the delay in referral to ophthalmologist is due to masking of pain by sedation and the redness in eyes were not noticed because the patient was keeping her eye closed due to the same reason . since symptoms of aacg may be overlooked or misinterpreted in a sedated or comatose patient , any patient who has a red eye and a subjective vision loss in the postoperative period should be examined urgently . | angle closure glaucoma is one of the ophthalmic emergencies and treatment has to be given at the earliest .
it is a rare complication of general anesthesia .
a female patient underwent hysterectomy under general anesthesia . following this , patient developed bilateral angle closure glaucoma .
this patient was treated with antiglaucoma medications followed by yag laser iridotomy and patient regained vision . |
gastrointestinal stromal tumors ( gist ) are the most common mesenchymal tumor of the human gastrointestinal tract , which arise from intestinal cells of cajal . before the imatinib ( glivec , novartis korea , seoul , korea ) era , surgery was the only therapeutic treatment for gist . even after the complete resection of gist , however , most patients with advanced disease relapsed making the prognosis of patients with metastatic and/or recurrent gist extremely poor . although a number of experiences have been reported with imatinib mesylate ( i m ) in patients with unresectable or metastatic gists without operation , there were few reports about neoadjuvant experience in those cases . we report a case of unresectable gastric gist who underwent neoadjuvant therapy followed by surgical resection . a 53-year - old female was admitted to our department of general surgery because of dyspepsia and vomiting during the previous month . for her past history , contrast - enhanced abdominal computed tomography ( ct ) and f-18 fluorodeoxyglucose positron emission tomography ( fdg - pet ) ct showed an approximately 12 cm sized heterogeneous mass located from fundus to mid body of stomach , which seemed to invade pancreas , spleen and left adrenal gland ( fig . endoscopic biopsy performed in our institution and pathologic report revealed a spindle cell tumor consistent with gist ( figs . 2 , 3 ) . results of immunohistochemical stain : the tumor cell was positive for cd34 and c - kit ( cd117 ) , weak positive for smooth muscle actin ; whereas s-100 was negative ( fig . two weeks later , we performed laparotomy , but the mass was huge and invaded the pancreas and retroperitoneum very tightly so that it could n't be resected . ten days after the operation , the patient was started on imatinib therapy , a daily dose of 400 mg , maintained for 4 months . a follow - up ct , 2 months after the start of imatinib , showed a dramatic reduction in the size of the tumor . and 4 months after follow - up ct , there was no evidence of mass lesion in the stomach ( fig . 5 ) ; on the gastroduodenoscopy , the mass lesion was invisible with only an ulcerative lesion observed in the same location ( fig . although the mass lesion was not revealed clearly at the follow - up imaging study ( fig . 5 months after the treatment with imatinib , the patient underwent total gastrectomy and splenectomy . the stomach was adhered to the spleen , tightly , and the lesion was found between stomach and spleen , firm and white , having irregular margin . pathologically , very few tumor cells were in the fibrotic tissue ; residual tumor size was 0.3 0.2 cm and 0.2 0.2 cm , respectively . the tumor consisted of spindle cells with a mitotic activity index of 1/50 hpf on microscopy . around the residual tumor , hyalinized fibrosis , inflamed granulation tissue , dystrophic calcification , and necrosis and abscess gist is the most common mesenchymal tumor of the gastrointestinal tract . up to two - thirds of gists show malignant behavior with high recurrence rates . therefore , improving the rate of complete resection is a key challenge in the treatment of gists . neoadjuvant therapy with tyrosine kinase inhibition has potential usefulness in primary gist , although not yet as a standard of care . several retrospective studies suggested that resection of residual lesions could prolong progression - free survival if it is done while the tumors are under control with imatinib . it is also emphasized that imatinib should be continued even after complete resection of all visible disease . however , the role of resection of residual tumors after imatinib therapy has not been established , and several phase iii clinical trials to investigate the role of surgical resection in this setting are ongoing or planned worldwide . there is no disagreement on the benefit of treatment with imatinib for malignant gists , but the clinical outcome of neoadjuvant treatment with imatinib is not well established . in our case , we could perform complete resection of the gist by down - sizing of the tumor after treatment with imatinib . the duration of neoadjuvant therapy with imatinib may vary according to response to the treatment , but surgery may be performed after sufficient shrinkage of tumors is observed ; typically after 4 to 6 months and within 12 months of imatinib treatment . the standard initial dose of imatinib is 400 mg per day in patients with unresectable or metastatic gists . in a randomized phase iii trial that compared 400 mg daily with 800 mg daily , the 800 mg daily was a more toxic but not more effective dose . the goal of surgery is complete resection of tumor , possibly avoiding the occurrence of tumor rupture and achieving negative margins . in our case , there was no tumor rupture during the operative procedure and pathologically negative margins were obtained . radiographic tumor response to preoperative imatinib correlated significantly with complete resection , with 91% of patients with partial responses achieving complete resection , versus 4% of patients with progressive disease . radiographic and metabolic complete response based upon fdg - pet are not always concordant with a pathologic complete response ; therefore , it should be born in mind that the pathological evaluation on the surgically resected materials obtained from patients treated with i m might be indispensable for the elucidation of the therapeutic effect of i m on gist . in our case , follow - up pet showed a hypermetabolic lesion around medial side of the spleen ; residual tumor was observed in that site on microscopy , but follow - up ct showed no evidence of mass lesion in the stomach . in the surgical findings , the lesion had extensive areas of fibrotic changes and there were severe adhesions between the lesion and spleen , so it was suspected to be invasion , thus we performed total gastrectomy and splenectomy . first , the patient 's follow - up period is short and will not be an accurate prognostic assessment . suspected to be in an inoperable state , neoadjuvant chemotherapy should take precedence over surgical procedure . in summary , we report a case of gist in an unresectable state , initially , who underwent complete surgical resection following imatinib treatment . in cases of advanced gists , neoadjuvant treatment with imatinib may provide an opportunity for surgeons to perform a complete resection . | a 53-year - old woman was diagnosed with gastrointestinal stromal tumor ( gist ) of the stomach . computed tomography ( ct ) revealed a huge mass ( 12 cm in diameter ) , likely to invade pancreas and spleen . in the operation field ,
the tumor was in an unresectable state .
the patient was then started on imatinib therapy for 4 months . on follow - up imaging studies ,
the tumor almost disappeared .
we performed total gastrectomy and splenectomy upon which two small - sized residual tumors were found on microscopy . in this paper , we describe a case of clinicopathologic change in unresectable gist after neoadjuvant imatinib mesylate . |
in december 2005 , an 84-year - old man was transported to the trauma unit at the karolinska hospital after a severe accident in which he was run over by a reversing car . at arrival his glasgow coma scale score was 15 and he had multiple costa fractures and pneumothorax and was treated at the intensive care unit for 2 days and was in hospital for additional 5 days before he could be discharged . at arrival to the emergency department it was noted that he had a scar at the left hip . he had a total hip arthroplasty at the karolinska hospital on may 25 , 1970 and the prosthesis had worked perfectly ever since . an x - ray showed no evidence of loosening or osteolysis ( figure 1 ) . the prosthesis was a mckee farrar metal - on - metal cobalt - chromium alloy hip arthroplasty ( howmedica limited , london , uk ) . since we have an interest in metal - on - metal prosthesis in relation to the metal ion concentration in blood , we decided to measure the concentrations of chromium and cobalt in his blood . the analysis was made with inductively coupled plasma mass spectrometer ( icp - ms ) ( moens et al 1995 ) . l ( 0.050.48 ) and the cobalt concentration was 22.92 g / l ( 0.030.8 ) . the hip was completely painless with good range of motion : flexion 85 , extension 0 , internal rotation 5 , external rotation 15 , abduction 30 , and adduction 10. the creatinin level was slightly increased at 129 mol / l ( ref . 6388 ) , and normal levels of s - igg , s - iga , and s - igm were found . today , the numbers of metal - on - metal implants are increasing rapidly due to the high interest in surface replacement arthroplasty . one side effect of this type of articulation is that metal ions such as cobalt , chromium , manganese , and nickel are released from such articulations and can be found in the proximity of the implant . these heavy metal ions are distributed via the blood stream to distant parenchymal organs ( urban et al 2000 ) and are subsequently excreted renally . it is well known that ions such as nickel and chromium can induce allergic reactions . however , very few reports have shown allergic reactions from metal implants ( niki et al 2005 ) . several other concerns as lymphocyte - mediated osteolysis ( willert et al 2005 ) , inhibition of osteoblast proliferation and collagen synthesis ( anissian 2002 ) , toxicological considerations ( visuri et al 1996 ; signorello et al 2001 ) and immunological changes ( hart et al 2006 ) have also been raised . several studies describe increased levels of metal ions after implantation of a metal - on - metal prosthesis ( eg , masse et al 2003 ; ladon et al 2004 ) and the increased levels have been shown to be up to 10-fold in serum . a 3-fold increase in serum cobalt 20 years after mckee there are no previous case reports or studies of ion levels 37 years after metal - on - metal implantation . this case indicates that metal ion levels might rise to extreme levels in the very long term for patients with metal - on - metal articulations . nowadays , since patients who are selected for metal - on - metal prosthesis are mostly young , this might be a future concern . metal ion concentration after implantation of a metal - on - metal prosthesis can rise to extreme levels in the long run . however , this might not be associated with illness or poor hip function . on the other hand , | this case report describes a patient who had a metal - on - metal hip prosthesis implanted 37 years ago . the hip function and x - ray
are presented .
the levels of cobalt and chromium ions in blood are analyzed and found to be about 40 times higher than normal .
consequences are discussed . |
microspordia are emerging pathogens in recalcitrant stromal keratitis.1 the disease is difficult to diagnose both clinically and by laboratory diagnosis . existing reports have shown clinical and histological evidence that the disease is restricted to the stroma , with no recurrence after penetrating keratoplasty.12 currently , there is little evidence demonstrating penetration into the anterior chamber ( ac ) . we report a case of stromal microsporidial keratitis with presence of an intact descemet membrane ( dm ) and demonstrable microsporidial spores in the ac exudates . a 26 year old healthy male presented on november 1 , 2008 with a one - year history of recurrent redness , pain and watering in the right eye . the patient was a non - diabetic , with good general health and nutritional status . the condition waxed and waned despite multiple ophthalmology consults , and therapy that included topical antibiotics , corticosteroids and oral antiviral medication . slit lamp examination showed intact , edematous epithelium , while the underlying stroma showed multiple , gray - white irregular to oval shaped flocculent infiltrates with indistinct borders ; diffusely distributed over the entire cornea ( except the limbus ) , involving the deep stroma [ figures 1a and b ] . ( a and b ) slit lamp photographs showing the dense corneal infiltrates at presentation . the smear revealed multiple oval , spore - like structures , with a waist - band which was gram positive , 1% acid fast positive and showed blue fluorescence on potassium hydroxide with calcofluor white stain , confirming a diagnosis of microsporidial stromal keratitis . medical therapy was instituted with topical 0.02% chlorhexidine gluconate ever half - hour and oral albendazole 400 mg twice daily ; however here was no response even after three weeks [ figure 1c ] . a 9.5 mm corneal graft was sutured with 16 interrupted sutures into a 10 mm recipient bed . postoperatively , the patient was prescribed topical chlorhexidine 2% eight times a day ( formulated in the hospital pharmacy ) which was discontinued after three weeks , topical prednisolone acetate 1% eight times a day tapered over the next several months , and topical atropine sulphate 1% three times a day for two weeks . however , a year later , the patient presented with severe allograft rejection ( he had stopped topical steroids ) and subsequently the graft failed . an endothelial keratoplasty was performed and the patient was doing well at the last follow - up , two years postoperatively . the histopathology of the corneal button revealed an ulcerated cornea with dense inflammatory infiltrates in the stroma composed of neutrophils , lymphocytes and plasma cells with stromal necrosis . numerous microsporidial spores were present in the deeper stroma upto the dm [ figures 2a c ] on gram stain and 1% acid - fast stain . the ac exudates noted in some sections showed the presence of microsporidial spores [ figure 2d arrow ] . ( a ) section of the cornea shows epithelial ulceration , dense stromal infiltrates and ac exudates posterior to and separate from the dm ( h and e , 10 ) . ( b ) 1% acid fast stain shows brightly stained microsporidial spores within the posterior stroma . ( c ) under higher magnification ( 100 ) , the spores show the characteristic waist - band . ( d ) 100 magnification with 1% acid fast stain showing presence of microsporidial spore ( arrow ) in the ac exudate microsporidial keratitis is an emerging , opportunistic clinical entity caused by parasites belonging to the genus microspora.1 clinically the stromal form presents with mid to deep stromal infiltrate mimicking stromal hsv keratitis . vemuganti et al , reported the largest series ( five cases ) of stromal microsporidial keratitis from this center that underwent penetrating keratoplasty , where the presentation ranged from stromal infiltrate to thinning with descemetocele formation.1 interestingly , font et al , reported a case of stromal microsporidial keratitis which had recurrence of infection following therapeutic dalk ; requiring a penetrating keratoplasty.2 das et al , recently reported a case of microsporidial keratits with the spores noted in the ac exudates.3 there have been reports on intraocular microsporidiosis causing endophthalmitis45 or sclero - uvetis with retinal detachment,6 however the mechanism of spread was presumed to be a systemic in these reports and none - of these patients had corneal involvement . with the current cases being reported , it is possible that the spread could have been through the intact ocular tissues rather than a hematologic route . our case clearly demonstrates the presence of microsporidia in the ac exudates ; which to our knowledge is the second case reported in literature . this finding may suggest a hitherto unknown , additional pathogenic mechanism of microsporidia with an ability to penetrate the intact dm ( similar to fungi , under which these organisms have been reclassified7 ) . with an increasing trend towards dalk , it would be important to note that in the presence of endothelial exudates or ac exudates , a penetrating keratoplasty is preferred over lamellar keratoplasty , as the latter may be associated with recurrence of infection . | we report a rare case of a deep stromal keratitis with a chronic indolent course , diagnosed as microsporidial keratitis from corneal scrapings .
the patient s condition worsened despite medical therapy and penetrating keratoplasty was performed .
the histopathology of the corneal tissue revealed multiple microsporidial spores in the posterior stroma and the endothelial exudates , whereas there was no clinical or histopathological breach in descemet s membrane .
this is the second report in the literature to report that micropsoridial spores can cross the intact descemet s membrane . |
with the development of preimplantation genetic diagnosis ( pgd ) using ivf technology , it is now technically possible to determine the gender of the embryo before being transfered with a high degree of certainty . there has been much discussion in the media whether the technique should be allowed . in the state of victoria gender selection and throughout australia it is against the guidelines of the national health and medical research council , thus preventing australian couples from using this technology . whilst the technique is forbidden in india and the european community , it is permitted in several countries . the ethics of clinicians referring couples for gender selection from countries where it is forbidden to other countries where it is permitted and is performed has recently been the subject of debate . opinion of ethics committees can reflect the attitudes of its membership , or they can be swayed by vocal minorities . we carried out a survey by an experienced and reliable gallup poll organization to assess the attitude of the general australian community on social gender selection . as part of the regular morgan gallup polls three questions with respect to ivf and gender selection were included with the morgan gallup telephone poll of the week of february 1st , 2011 . morgan gallup polls are carried out each fortnight as cross - sectional surveys , conducted by telephone of a random sample of 650 australians . this survey was conducted as part of a larger omnibus community survey performed by roy morgan market research ( melbourne , australia ) about voting intentions and consumer preferences . the sample was drawn from the residential phone numbers in the australian electronic white pages and stratified by geographical area with quotas controlled by gender and age to be representative of the australian population . multiple attempts were made to contact each phone number that was randomly drawn at different times on different days . the survey was carried out by asking three questions to determine the respondents ' attitudes to gender selection ; the questions in order are presented below , with an australia wide cross section of 650 respondents aged 14 years and over . these were as follows.it is now possible for people having ivf treatment to decide the baby 's sex ( gender selection ) . at present gender selection is not allowed . in your opinion should people having ivf treatment be allowed to select the gender of their baby or not?couples who are not infertile can determine the sex of their baby using ivf ( gender selection ) . in your opinion , should gender selection be allowed for anyone?should couples who already have one or more child of one sex be allowed to use the gender selection technology to select the gender of their next child ( family balancing ) ? it is now possible for people having ivf treatment to decide the baby 's sex ( gender selection ) . at present gender selection should people having ivf treatment be allowed to select the gender of their baby or not ? couples who are not infertile can determine the sex of their baby using ivf ( gender selection ) . in your opinion , should gender selection be allowed for anyone ? should couples who already have one or more child of one sex be allowed to use the gender selection technology to select the gender of their next child ( family balancing ) ? the attitudes of respondents were measured as yes - allowed , no - not allowed , or analysis of responses by several variables was carried out . as this study contains no identifying material , and it conforms to the standards established by the nhmrc for ethical quality , ethics approval was not sought . the responses were compared between residents in capital cities and rural inhabitants and shown in table 3 . however , the numbers analyzed here are small and at best are indicative but certainly not significant . interestingly , respondents who only had a primary school education ( 101 respondents ) were more liberal and had a significantly higher rate of allowed responses ( 35.4% for question 1 , 31% for questions 2 and 3 ) , whereas current university students ( 31 respondents ) were much more conservative ( 12.8% yes for question ) . it appears from our study that the majority of australians in 2011 agree with the members of the health ethics committee that social gender selection should not be permitted , as 73% of all respondents responded that it should not be allowed . the prohibition of gender selection in australia was recommended by the health ethics committee of nhmrc in 2004 . why did the nhmrc prohibit gender social selection ? in appendix 1 of the document ( nhmrc ) the first was that parental love should be unconditional acceptance and not depend on the sex of the childsex selection is incompatible with the parent - child relationship being one that involves unconditional acceptance . the second was , that sex selection may be an expression of sexual prejudice , in particular against girls . if male children are chosen in preference , this denigrates the value of females . as practiced today around the world , it generally reflects and contributes to bias and discrimination against women . the third was , that the natural sex ratios may become unbalanced if there is preference for a particular sex selected by this technique . sex selection harms men in some cultural groups ( by contributing to the shortage of women for men to marry ) . are these reasons valid and do they mirror the opinions of the community ? unconditional love is an excellent motherhood statement in theory , but does it always apply in practice ? may there be some situations when the community would condone gender selection , such as where a couple has several children of one sex already ( family balancing ) or where they have lost a child of one sex which they would hope to replace ? secondly , in some countries there is a preference for boys , but this is not the case in australia , where requests for female children are just as common . thirdly , although 1 in 30 children in australia is now conceived by ivf , a very small percentage would choose gender selection if available , and the numbers would be too small for unbalancing the sex ratio to happen . the reasons given for permitting gender selection are that it may enable parents to fulfill religious obligations or cultural expectations , and that selection of gender is properly thought of as a matter for individual autonomy . interestingly , our 2011 survey is in exact agreement with an australian mail - out survey of social attitudes carried out back in 2006 , but published recently , where it was found that 69% of respondents disapproved or strongly disapproved of the use of ivf for sex selection . it would appear that despite repeated discussion in the media , as detected by this survey , the community 's attitudes have not changed during the last five years with the overwhelming opinion in the australian community being against gender selection . whether this is due to a lack of informed discussion or because of its association of eugenics using the thin edge of the wedge liberal ethicists , who are proponents for gender selection and maintain that the technique should not be forbidden as it does not harm anybody , have not been able to get their message accepted by the community . it is noted that amongst younger respondents ( 1834 years ) up to 31% supported one or other of the options , which may mean that attitudes may change in the future . however , this is only speculating and it is clearly is not possible to measure accurately what people 's opinions may be five or ten years hence ! | this study was carried out to determine the attitudes of the australian community to ivf by a reliable community poll .
cross - sectional surveys , conducted by telephone of a random sample of 650 australians were undertaken .
the sample was drawn from the residential phone numbers in the australian electronic white pages and stratified by geographical area with quotas controlled by gender and age to be representative of the australian population .
the participants were asked to answer to three questions about gender selection , and their response was measured as yes - allowed , no - not allowed , or
undecided for each of the questions . whilst 91% of respondents supported the use of ivf to help infertile couples , only 20% supported gender selection within ivf or for family balancing .
when it came to the use of ivf only for gender selection , only 17% were in favour .
this survey shows that australian community overwhelmingly opposes gender selection for social reasons . |
a 66-year - old woman with recently diagnosed durie and salmon stage iiib igg kappa myeloma was started on cybord chemotherapy including cyclophosphamide 500 mg orally weekly , bortezomib ( given weekly after hemodialysis , at a dose of 2.5 mg subcutaneously ) , and dexamethasone 2040 mg orally weekly , having failed plasmapheresis . she was also on antiviral prophylaxis ( acyclovir 200 mg orally twice a day ) and double - strength trimethoprim sulfamethoxazole once daily on mondays , wednesdays , and fridays . however , 6 h after the initiation of her third dose , she was brought to the emergency room due to acute - onset shortness of breath that was progressive and associated with palpitations without chest pain , dizziness , or lightheadedness . she had no fever , chills , cough , sputum production , orthopnea , pain , or change in bladder or bowel habits . her medical history included renal failure attributed to myeloma , hypertension , diabetes mellitus type 2 , stage i ovarian cancer treated with surgery , as well as carboplatin and paclitaxel 5 years prior , and left bundle branch block ( lbbb ) first noted 2.5 months prior but associated with normal echocardiography ( echo ) . she had started hemodialysis the day before commencement of chemotherapy , and had not missed any scheduled dialysis sessions . , she weighed 64 kg and had tachypnea and tachycardia ; her oxygen saturation was 76% on room air . she was in distress but alert and oriented ; examination revealed increased jugular venous pressure , diffuse bilateral crackles , and normal heart sounds with no murmurs , gallops , or rubs . her troponin was 0.323 , with rise to 0.916 in 17 h ; hemoglobin was 7.5 , hematocrit 22 , sodium 128 , chloride 91 , creatinine 5.9 , and glucose 410 . electrocardiography revealed normal sinus rhythm and lbbb ; chest radiography showed increased interstitial markings suggestive of pulmonary venous congestion , which was confirmed by contrast - enhanced computerized tomography of the chest done to rule out pulmonary embolism . echo revealed dilated cardiomyopathy with ejection fraction ( ef ) of 15% , worse than 55% obtained 2 months prior ( table 1 ) . cardiac catheterization showed insignificant coronary artery disease with minimal myocardial bridging of the posterolateral branch of the right coronary artery ; this was thought not to be responsible for the heart failure . bortezomib - induced cardiotoxicity was entertained ; the naranjo adverse drug reaction probability scale ( 1 ) indicated a probable relationship ( score of 5 ) between bortezomib and acute left ventricular dysfunction . her ef improved , but did not reach her prior baseline ( table 1 ) ; n - terminal pro - b - type natriuretic peptide ( bnp ) was 11,000 three weeks after presentation . in place of cybord , she received lenalidomide and dexamethasone chemotherapy , which was expanded to bendamustine / lenalidomide / dexamethasone . she , however , developed infection of her vascular access catheter , as well as atrial fibrillation , and ultimately expired in home hospice care 3 months after presenting in heart failure . systolic ejection fraction by months before ( denoted by negative numbers ) , at ( time 0 ) and after presentation recent recommendations for treatment of multiple myeloma include induction with bortezomib and dexamethasone - based regimens in patients with myeloma kidney ( 2 ) . bortezomib is a boron - containing molecule and proteasome inhibitor ; the ubiquitin - proteasome system the major pathway for intracellular protein degradation plays a role in cell survival . pig data suggest that chronic ( over 12 weeks ) proteasome inhibition is associated with increased propensity to atherosclerosis ( 4 ) ; other animal studies have associated proteasome inhibition with control of cardiac hypertrophy ( 5 ) or reversible systolic dysfunction in rat hearts ( 6 ) . in a study comparing bortezomib to high - dose dexamethasone for treatment of relapsed myeloma , 2% in each group had congestive heart failure ( chf ) ( 7 ) . a meta - analysis of that and 24 other trials found a statistically insignificant increase in cardiotoxicity associated with bortezomib compared with control but only patients with nevertheless , acute development of heart failure has been associated with bortezomib treatment . in one instance , this was after four 3-week cycles of bortezomib on days 1 and 8 of each cycle ; pretreatment bnp was elevated to 1,389 ( 9 ) . however , cotreatment with anthracyclines which are better known for association with cardiotoxicity occurred in two of seven cases in one review ( 10 ) . lbbb predisposes to heart failure ( 11 ) ; the role of lbbb or other features of asynchrony in bortezomib cardiotoxicity is unclear . cyclophosphamide is known to induce diastolic dysfunction via direct endothelial damage , but cardiotoxicity has not been reported in association with cumulative doses less than 100 mg / kg ( 13 ) . fortunately , most patients with bortezomib - associated heart failure respond to standard therapy for heart failure and cessation of bortezomib but it has taken 6 or more months to document this ( 9 , 10 ) . in light of the above , cardiac function should be assessed at baseline with echocardiogram , bnp , and ekg , even in asymptomatic patients , as part of a routine workup before starting proteasome inhibitor therapy . we also recommend that bnp be monitored serially during therapy or at the first hint of symptoms referable to cardiac failure . further research is required to inform guidelines for cardiac assessment of patients on proteasome inhibitor therapy . the authors have not received any funding or benefits from industry or elsewhere to conduct this study . | chemotherapy - associated cardiotoxicity can present as a spectrum from arrhythmia to acute congestive heart failure . unlike anthracyclines , proteasome inhibitors for example , bortezomib are not notorious for causing cardiotoxicity in absence of pre - existing cardiac dysfunction or without concomitant use of other cardiotoxic agents .
we describe a 66-year - old woman with end - stage renal disease who developed acute dyspnea hours after a third treatment with bortezomib for igg kappa myeloma .
the naranjo adverse drug reaction probability scale indicated a probable relationship ( score of 5 ) between bortezomib and acute left ventricular dysfunction .
patients receiving proteasome inhibitors should be closely monitored for evidence of cardiac dysfunction during treatment . |
upper thigh subcutaneous emphysema manifested by swelling , crepitus and cellulitis is a common orthopaedic presentation , usually related to underlying musculoskeletal problem or penetrating wound . in a few situations , this can be related to abdominal pathology rather than limb pathology and most of these cases we present a case of subcutaneous emphysema of the thigh secondary to para - caecal abscess . this was managed by laparotomy alone for abdominal pathology without the need for surgical debridement or thigh exploration . a 75- year old female was admitted as an emergency with right iliac fossa pain . clinical examination was inconclusive . during her over - night stay , she developed tachycardia and hypotension which responded to fluids . the patient had a ct abdomen , pelvis and upper thigh that showed gas containing fluid collection in the right iliac fossa in keeping with either diverticular or appendicular abscess ( figure 1 ) . it also showed inferior extension of the gas into the right ilio - psoas muscle , groin and upper thigh ( perivascular ) raising the possibility of necrotising fasciitis ( figure 2 ) . right iliac fossa abscess subcutaneous thigh emphysema on laparotomy , an abscess was found in close proximity to a caecal mass . there was no pus tracking retroperitoneally or along the psoas muscle and the muscle looked healthy . most likely cause of the subcutaneous emphysema in thigh was thought to be gas in the abscess cavity tracking along fascial planes . no exploration of the thigh was performed and the plan was to review the thigh swelling the next day . however , this settled down completely following laparotomy alone . post operatively the patient recovered well , no further surgery was done and the patient went home after a slow convalescence . . this collection can trickle down by gravity along fascial planes posterior to the inguinal ligament and both of them can lead to subcutaneous emphysema and possibly thigh abscess ( 2,3,4,5 ) . perforated appendix is the commonest cause of bowel - related right thigh emphysema , whereas on the left side it is usually caused by diverticular disease or perforated rectal cancer ( 2,3,4,5 ) . due to the nature of the disease enteric flora are usually detected but chances of developing gas gangrene is usually remote ( 4 ) . the subcutaneous emphysema may result from several mechanisms : first many of the colonic flora are gas producing , and perhaps more important passage of intestinal gas from gastrointestinal tract in to tissues as the result of positive gradient between bowel lumen and subcutaneous space ( 5 ) . the diagnosis like in our case is often delayed or missed due to paucity of localising signs . if no necrotic tissue or dead muscle is found next to primary pathology , provided that primary pathology is treated timely , subcutaneous emphysema of the thigh may herald a benign course . on the other hand if necrotic muscles are found in the abdomen or pus tracks in to the thigh , exploration of thigh is mandatory to ensure complete debridement and to avoid missing necrotising infections of the thigh . in our case the patient was managed conservatively ( without thigh exploration ) as once the abdominal pathology has been treated , the thigh subcutaneous emphysema started to improve with antibiotics and with close monitoring of the patient . subcutaneous emphysema of the thigh , secondary to abdominal pathology when associated with healthy psoas muscle is a condition that follows a benign course . this can be treated conservatively without exploration of the thigh as long as the abdominal pathology is promptly managed . good imaging modalities including ct and mri are strongly recommended when the underlying pathology of the thigh subcutaneous emphysema is not clear . | thigh subcutaneous emphysema is an usual orthopaedic presentation normally associated with musculoskeletal problems or penetrating wounds .
but , sometimes it can be related to abdominal pathology .
we present a case of subcutaneous emphysema of the thigh secondary to para - caecal abscess . |
hydatid disease is a worldwide zoonosis caused by infection with a small tapeworm parasite called echinococcus granulosus . sheep and cattle are intermediate hosts for the pastoral form , and humans are an accidental intermediate host ( 12 ) . patients with hydatidosis usually present with hepatic ( liver ) ( 75% ) , pulmonary ( lung ) ( 15% ) , and other organ involvements ( 10% ) ( 35 ) . it is usually microscopic and just happens in only 1020% of renal hydatidosis cases ( 16 ) . hydatid cyst of the kidney is a very rare and just 15% of all hydatid disease in humans involving the kidneys ( 5 ) . opening of renal hydatid cyst into collecting system may result in acute renal colic and/or hydatiduria . however , most of the hydatiduria cases do not have renal colic ( 7 ) . we report a case of isolated renal hydatid disease presenting with right flank pain and a sensation of fullness in the abdomen and hydatiduria secondary to opening of hydatid cysts into renal pelvis and urinary excretion of daughter cysts . in 2012 a 34-year - old female referred to razi medical center in rasht , north of iran , complaining of a dull right flank pain and a fullness feeling in her abdomen since 9 months ago . the patient s medical history was unremarkable ; however , she was living in a rural area and had been in close contact with animals such as dog and sheep . laboratory tests revealed normal serum creatinine levels and blood cell count , and no abnormalities on urinalysis . ultrasonography demonstrated a large size multicystic lesion of 645 cm with mixed echogenicity containing multiple septations in right kidney ( fig . multiple cysts at sonographic appearance of rt kidney ( source : by authors ) abdominopelvic ct scan showed a lesion containing cystic compartments , originating from middle pole of right kidney , traversing to its pelvis and showing exophytic growth toward perirenal space . based on history of passing grape - like material in urine and complex cystic lesion in kidney , and absence of gross involvement in other organs , possible diagnosis of primary renal hydatid disease was made . but albendazole treatment was started as a 4-week cycle prior to operation and was continued after it . because of extensive involvement of kidney and history of hydatiduria , simple right - sided nephrectomy was performed through a subcostal extra - peritoneal flank incision and retroperitoneal cavity was irrigated with hypertonic saline solution . in renal specimen , the collecting system was filled with massive numbers of daughter cysts ( fig . 2 , 3 ) . the excised kidney and multiple daughter cysts ( source : by authors ) communication between cysts and pelvicaliceal system ( source : by authors ) the pathologist reported a multilocular hydatid cyst in the nephrectomy specimen . the postoperative period was uneventful , and albendazole 400 mg twice daily was prescribed to prevent metastatic cyst formation for 6 months . we have followed up the patient for 2 year with periodic examination including chest x - ray , retroperitoneal and abdominal cavity and ultra sonography every 3 months , without any relevant problem . the patient was taken informed consent and privacy of information was followed in this study . there are no specific clinical symptoms or signs that will reliably confirm the diagnosis of renal hydatid disease . the combination of clinical history , imaging studies , serological and urine investigations yields a reliable pretreatment diagnosis in only 50% of cases and a presumptive diagnosis in 71% ( 5 ) . imaging studies are suggestive but usually inconclusive , and the differential diagnosis between a cystic renal tumor and complicated cyst may not be made without surgery ( 5 ) . the clinical presentation of hydatid disease depends on the size and site of the lesion and the accessibility of the involved organ for clinical examination . hydatiduria , which is the presence of daughter cysts and larvae ( hydatid sand ) in urine , is easy to recognize macroscopically ( grapelike appearance of material ) . it is the only pathognomonic sign of renal echinococcosis , which presents in about 518% of cases ( 3 ) . this event results from the rupture of a cyst into the collecting system ( 3 , 8) . different serological tests are being carried out for the diagnosis , screening and post - operative follow up for recurrence . these include the hydatid immunoelectrophorsis , elisa , latex agglutination and indirect haemagglutination ( iha ) test . the casoni - weinberg test as a skin test has little efficacy and has been abandoned nowadays ( 10 ) . ring - like calcification of the cyst can be visualized by plain radiography in 1/3 of patients ( 11 ) which may suggest diagnosis of hydatid disease ( 12 ) . if hydatid cysts open to the collecting system irregular filling defects can be seen due to daughter cysts . the us and ct features of renal hydatid cysts are similar to those of cysts in other locations . however , ct is superior to us in diagnosis of renal hydatid cyst ( 13 ) . under real time us on changing the patient s posture , there will be shifting of hydatid sand , which may give rise to the falling snowflake pattern ( 4,14 ) . ct may show a single cyst with multiple daughter cysts , or a multiseptated cystic mass with or without calcifications . ct densities of the daughter cysts are significantly lower than the mother cyst ( 14 ) . in cases of communicating cyst with the collecting system , contrast may enter into the cyst , which may be seen as streaky hyperdense areas in the cyst . because of the lack of an absolutely effective systemic scolicidal agent , surgical treatment offers the only hope of cure . in general , surgery is the preferred type of treatment in renal hydatid cysts ( 4 ) . however , there are also some cases in literature that have healed by only medical treatment with albendasole ( 12 ) . the cysts should be removed without rupture to reduce the chance of seeding and recurrence . if most of the kidney is invaded by hydatid cyst , then nephrectomy is inevitable ( 11 ) . besides , nephrectomy is the preferred type of surgery in cases with hydatiduria when medical treatment has been considered , albendazole which is a benzimidazole anthelmintic , believed to be superior to mebendazole for treating hydatid disease . the usual dose is 1015 mg / kg body weight daily or 400 mg twice daily . for adults , the drug is given in cycles such as twice daily for 28 days followed by a 12 week drug - free interval , followed by another course of the drug . for most patients three cycles is sufficient , although the course can be repeated up to 12 times ( 15 ) . postoperative recurrence of intra - abdominal echinococcosis can be seen because of intra - operative rupture of cysts and intraperitoneal spillage . therefore pre - surgical chemotherapy of infection can reduce the size and number of viable protoscolices and therefore postoperative recurrence ( 16 ) . | isolated renal hydatid disease is a rare endemic infestation caused by larval form of echinococcus granulosus .
hydatiduria is an uncommon presentation of renal hydatid disease . in 2012
a 34-year - old female referred to razi hospital , rasht , iran with complaints of right flank pain and grape - like material in urine .
diagnosis was made by ultrasonography and ct scan .
the patient was treated surgically with nephrectomy in combination with perioperative chemotherapy with albendazol . |
collagenomas or collagen nevi are a type of connective tissue nevi characterized by hamartomatous proliferation of collagen fibers in the dermis with normal , decreased or increased elastic fibers . they present as asymptomatic skin colored papules , plaques , nodules or swelling of variable size in grouped , solitary , linear / segmental or irregular distribution . we report a case of isolated giant collagenoma of scalp in an adult male patient . the present case is about a 20-year - old male patient who presented with an asymptomatic swelling on the left fronto - parieto - occipital region of the scalp since last 10 years . the swelling was insidious in onset , started as single papular lesion in the parietal region and gradually progressed to reach the present size [ figure 1a ] . contrast enhanced computed tomography scan of the head showed that the swelling was outside the skull bones , without any intracranial extension . patient had gone to a plastic surgeon 5 years back and had undergone partial excision of the swelling . excised tissue was subjected to histopathological examination at that time , but the report was not available with the patient . there was no history of trauma or any manual or other manipulation at the site prior to the development of the swelling . there was no feature suggestive of tuberous sclerosis such as adenoma sebeceum , seizures , ash leaf macules or confetti like macules . on physical examination , a well - defined circumscribed swelling measuring 14 cm 11 cm , presented over scalp , involving left fronto - parieto - occipital region with some extension over right [ figure 1a ] . the skin and hairs overlying the swelling were normal except the brownish discoloration of the skin . linear scar of size 13.5 cm 0.5 cm of previous surgery was present over the swelling on the left side [ figure 1b ] . systemic examination including skin , mucosa , nail and hair did not show any abnormality . a 4 mm incisional punch biopsy at the margin of swelling was sent for histopathological examination , which showed hyperplastic epidermis with thickened reticular dermis with dense collagen bundles that were arranged haphazardly in lower reticular dermis [ figure 2a ] . special staining with masson 's trichrome stain showed green colored collagen bundles arranged haphazardly [ figure 2b ] . as patient had cosmetics concerns so he was referred to the plastic surgery for the complete excision of the lesion in steps . well defined circumscribed swelling measuring 14 cm 11 cm , presented over scalp , involving left fronto - parieto - occipital region with some extension over right linear scar of size 13.5 cm 0.5 cm of previous surgery was present over the swelling on left side hyperplastic epidermis with thickened reticular dermis with dense collagen bundles that were arranged haphazardly in lower reticular dermis ( h and e , 400 ) special stain showed green colored collagen bundles arranged haphazardly ( masson 's trichrome , 400 ) connective tissue nevi are circumscribed hamartomatous malformations of the dermal extracellular matrix , i.e. of collagen , elastic fibers or glycosaminoglycans collagen nevi are the hamartomatous malformations of the collagen and are classified into familial and non - familial types . familial include cutaneous collagenomas with autosomal dominant inheritance associated with cardiological abnormalities and shagreen patches associated with tuberous sclerosis . isolated collagenomas comprise of planter cerebriform collagenoma alone or associated with proteus syndrome , linear or zosteriform collagenoma , knuckle pads collagenoma and papulolinear type of collagenoma . reported two cases of scalp collagen nevi associated with turner syndrome and considered it as resolving stage of lymphoedema . in a study by laxmisha et al . described isolated scalp collagenoma mimicking cutis verticis gyrata in a 35-year - old female with no associated abnormalities . isolated collagenoma of scalp can be differentiated from buschke - ollendorff syndrome by absence of elastic nevi , lack of family history and normal x - rays of long bones and pelvis ( no evidence of osteopoikilosis ) . turner syndrome ( xo genotype ) is easily excluded as the case is a male patient with no associated suggestive clinical findings such as webbed neck , short stature , edema of hands , etc . , the present case is a rare type of isolated collagenoma presenting over the scalp with no associated abnormalities . | connective tissue nevi of the skin are benign hamartomatous lesions consisting predominantly of one of the components of the extracellular matrix comprising of collagen , elastin or glycosaminoglycans type
. connective tissue nevi may be solitary or multiple , sporadic or inherited .
collagenomas are asymptomatic and usually occurs over upper trunk , arms , back , thighs and soles .
we , hereby report a young boy with collagenoma over the scalp , a rare site . |
the styloid process ( sp ) , stylohyoid ligament , and the small horn of the hyoid bone from the stylohyoid apparatus , which originally derives from the reichert cartilage of the second brachial arch during embryogenesis . the sp , the thin and long osseous part of the temporal bone . eagle 's syndrome appears during or following the third decade of life . symptoms of eagle 's syndrome depends on factors such as the length , width , and angulation of the sp . it is characterized by pharyngeal pain radiating to the ear , neck , tongue , and a vegetative syndrome consisting of pallor , sweating and hypotension ; this is all due to excessively long sps . there are several different theories , which try to explain the etiopathology of eagle 's syndrome such as congenital elongation of the sp and calcification and ossification of the stylohyoid ligament . fini et al . reported that past tonsillectomy is somehow related to eagle 's syndrome . diagnosis of an eagle 's syndrome may be confused with diverse conditions which occur with orofacial pain or dysphagia , such as neuralgias of the glossopharyngeal nerve , trigeminal nerve , dental problems , chronic tonsillitis , cervical arthropathies or pharyngeal tumors . in the present cases , the pain in the ear and pharynx of the mandible was masquerading the pain due to elongated styloid . kaufman et al . reported that 30 mm is the upper limit for normal sps . performed a cadaver study on the sp and reported that the normal length is between 1.52 cm and 4.77 cm . in radiological studies , several imaging modalities have been used for the diagnosis of eagle 's syndrome thus far , including lateral head and neck radiograph , towne radiograph , panoramic radiograph , lateral - oblique mandible plain film , anteroposterior head radiograph , and computed tomography . langlais and associates proposed a radiographic classification of the elongated and mineralized stylohyoid ligamant complex as follows . type i : elongated , type ii : pseudoarticulated and type iii : segmented . based on the pattern of calcification the types are calcified outline , partially calcified , nodular complex , and completely calcified . the length , angulation , and morphology classification as shown in table 1 . classification of styloid process camarda and associates stated that , in eagle 's syndrome , surgery is the initial treatment of choice because of the severity of the rapidly occurring ossification and symptoms . if the sp is excessive or radical amounts must be removed , the extraoral approach is a direct , anatomically concise approach to the sp . conservative treatment involves injecting steroids or anesthetics into the lesser cornu of the hyoid or the inferior aspect of the tonsillar area to tone down symptoms . the surgical excision can be done by the extraoral or transcervical and the intraoral or transpharyngeal approach . to conclude , elongated sps should be considered when a patient complains of oropharyngeal or maxillary pain originating from dental caries or impacted third molars . | the name styloid process ( sp ) was derived from the greek word stylos meaning a pillar .
it is a bony , cylindrical , needle - shaped projection , which originates from the posterior - inferior side of the petrous bone , immediately in front of the stylomastoid foramen , and goes obliquely down and forward .
when elongated leads to pain and discomfort called eagle 's syndrome .
elongated sp accounts approximately to 47% of the population , 4% only are symptomatic . |
a 66-year - old male patient visited the urology clinic in another hospital because of acute urinary retention . his prostate - specific antigen ( psa ) level and prostate volume as measured at the initial visit were 1.19 mg / ml ( tandemr - r psa immunoradiometric assay ) and 53 g , respectively . the hard prostate was palpated by digital rectal examination , without any palpable masses , and there were no abnormal findings except for a white blood cell ( wbc ) count of 3 - 5/high power field ( hpf ) and a red blood cell ( rbc ) count of 3 - 5/hpf in the urine analysis . no abnormal findings were reported for the complete blood cell count , biochemistry indexes , and simple radiologic exam . in may 2009 , turp was performed on this patient , and abdominal pelvic computed tomography ( ct ) ( fig . 1 ) , chest ct , and whole - body bone scan were also performed because the pathology results indicated small cell cancer . in the abdominal pelvic ct , multiple lymphadenopathies that were assumed to be metastatic lesions were observed near the bilateral iliac chain . no metastatic lesions were found on the chest ct or whole - body bone scan . to treat the primary small cell carcinoma of the prostate , the patient was admitted for robot - assisted laparoscopic radical prostatectomy with adjuvant chemotherapy after transfer to the urology clinic in our hospital . we performed magnetic resonance imaging ( mri ) in order to check the cancer stage . in the prostate mri , the malignant lesion of the prostate 2 ) . in july 2009 , the patient underwent robot - assisted laparoscopic radical prostatectomy and extended pelvic lymph node dissection ; the bilateral nerves were not saved . the total operative time was 240 minutes , and the amount of blood loss was 250 cc . the drainage tube was removed on day 3 after surgery , and the patient was discharged without any postoperative complications on day 4 . the pathologic results showed small cell carcinoma invading the capsule of the prostate ( fig . 3 , 4 ) and also metastasis of carcinoma in the right external iliac lymph nodes and right obturator lymph nodes . the weight of the prostate was 57 g , and the intraglandular tumor volume was 21 cc . the tumor involved both lobes , but the resection margin was not involved by the tumor . on postoperative day 7 , the catheter was removed in the urology clinic . after brain mri confirmed no metastatic lesions , adjuvant chemotherapy was started with vp-16 100 the multiple lymphadenopathies that had been observed before the surgery improved and there is currently no evidence of remnant disease . extrapulmonary small cell cancers are observed in the head and neck , skin , gastrointestinal system , cervix , and so on , but they are very rare , accounting for 0.1% to 0.4% of all malignant tumors and approximately 5% of all small cell cancers . in the urological system , small cell cancer of the prostate is known to occur as the result of changes in the argyrophil cells and argentaffin cells that exist in the normal prostate . however , the hypothesis that small cell cancer of the prostate occurs with the differentiation of various protocells is now convincing , showing coexistence with prostate adenoma or past history of prostate adenoma and positive responses in prostatic acid phosphatase and psa . the proper treatment guidelines for small cell prostate cancer are unclear owing to its low prevalence and an insufficiency of studies compared with typical prostate adenocarcinoma . it has been reported that only radical prostatectomy shows therapeutic effects on early small cell cancer in the prostate ; however , adjuvant chemotherapy is generally used . although the need for adjuvant radiotherapy is controversial , it should be considered if the pathologic report shows prostatic capsular invasion or resection marginal invasion . in our patient , we performed robot - assisted laparoscopic radical prostatectomy and pelvic lymph node dissection . the patient did not undergo adjuvant radiotherapy but did receive adjuvant chemotherapy because no remaining cancer was reported by postoperative pathology , although it was reported that there had been metastasis in the right iliac lymph node and right obturator lymph node . at 9 months after surgery , the cancer had not recurred and the patient 's urinary incontinence had improved compared with right after the surgery , with the use of 1 safety liner per day . a case of robot - assisted laparoscopic radical prostatectomy together with adjuvant chemotherapy to treat primary small cell cancer of the prostate has not been reported previously . therefore , it may be valuable to study the clinical significance of this case through further follow - up monitoring . | primary small cell carcinoma of the prostate is a rare and very aggressive disease with a poor prognosis , even in its localized form .
we managed a case of primary small cell carcinoma of the prostate .
the patient was treated with robot - assisted laparoscopic radical prostatectomy and adjuvant chemotherapy .
herein we report this first case of robot - assisted laparoscopic radical prostatectomy performed in a patient with primary small cell carcinoma of the prostate . |
kienbock 's disease is a condition that leads to osteonecrosis and collapse of the lunate bone . it usually involves the dominant hands of males between 20 and 40 years of age , and presents with wrist pain and decreased wrist motion . usually , only one wrist is affected in this disease . a smaller lunate diameter and height , a more radially inclined lunate tilting angle and a flatter radial inclination are important anatomical risk factors for kienbock 's disease . it is generally agreed that the end point of the disease , which is fragmentation and collapse , is the consequence of osteonecrosis . herein we describe a case of a 11-year - old girl , who presented with avascular necrosis of lunate bone , and after a short period of casting fully recovered within one year . the case is an 11-year - old girl , who had a history of six months continuous right ( dominant ) wrist pain . radiographs showed sclerosis of lunate bone , which was graded according to lichtman and colleagues classification as the stage iia of kienbock 's disease ( figure 1 ) . isotope scan with tc 99 m revealed increased lunate bone absorption and magnetic resonance imaging ( mri ) confirmed signal change and deformity of lunate suggesting avascular necrosis ( figure 2 , 3 ) . the patient wore a long arm cast for six weeks and stopped all of her sporting activities . after one year , radiographic pattern returned to normal ( figure 4 ) , and clinical manifestations disappeared . bone scan with tc 99 m isotope revealing increased lunate bone absorption six months after the trauma . kienbock 's disease ( carpal lunate necrosis or lunatomalacia ) is a process of unknown etiology resulting in osteonecrosis of the lunate bone . the most common clinical presentation is intermittent wrist pain , decreased wrist motion , and weakness of grip in the dominant hand . magnetic resonance imaging confirming signal change and deformity of lunate six months after the trauma . usually , the pain is activity related and subsides with rest . in the early stages , when symptoms of disease are similar to wrist sprain , diagnosis of the disease is difficult . the patients may not give a history of trauma , but the trauma may often exist in the distant past . at presentation , the patient may have swelling of wrist and tenderness in palpation of radiocarpal joint . motion is decreased in normal flexion / extension , and grip strength is commonly diminished to 50% of that of the other hand . there are limited number of published cases of kienbock 's disease in childhood , but the incidence of the disease in children aging between 10 to 12 years is higher . in the early stages of the disease , the use of mri , which is more specific and sensitive than bone scanning , can help in the diagnosis . in our patient , koji shigematsu treated an 11-year - old girl with kienbock disease ( stage iiia ) by temporary scaphotrapeziotrapezoidal pin fixation . , a 13-year - old boy with symptomatic stage iii kienbck 's disease was treated successfully with a radial shortening procedure . this case showed evidence of lunate revascularization and remodeling after a radial shortening osteotomy . in the study of almquist , twelve patients with early stages of kienbock 's disease and ulnar minus variant were treated by radial shortening procedures , and were followed for five to ten years . luc de smet , reported a case of a twelve - year - old girl with grip , which is similar to the present case . the case was treated conservatively , and during one year follow up favorable outcome was achieved , and the patient was pain free . the goal of surgical procedures is to unload the lunate and to decrease the compressive forces . these will result in the prevention of additional fragmentation and collapse , and theoretically improve revascularization of the lunate . the case in the present study wore a long arm cast for six weeks , and was forbidden from all her sporting activities . clinically , wrist pain and other symptoms resolved , and after one year , radiographic pattern was normalized ( figure 4 ) . kienbock 's disease rarely occurs in children , however , early diagnosis can result in simple nonoperative treatment , which is usually associated with a good outcome . because of the disease 's progressive and destructive effects on the wrist , it is important for physicians to take the announcement of the condition , try to diagnose it , and refer the patients to expert authorities in the early stages . | kienbock 's disease , which is an idiopathic osteonecrosis and collapse of the lunate bone , is an uncommon disease in children .
this report describes a case of an 11-year - old girl , who presented with avascular necrosis of lunate bone confirmed by radiographic and magnetic resonance imaging evidence . after a short period of casting ,
the case was fully recovered within one year .
the signal change was confirmed by magnetic resonance imaging , and the deformity of lunate that was demonstrated by avascular necrosis .
the patient wore a long arm cast for six weeks , and after one year radiographic pattern was normalized . |
nineteenth and twentieth century biology was completely based on misleading ideological imposition that living entities are particular states of matter and in that era biologists have only made several attempts to deny the living organism of its veracity as an immortal soul , in favor of genes , molecules , atoms and so on . twenty first century biology realizes that living entities ( animate objects ) do things , which are intentional and purposeful ( internal teleology ) and nonliving objects ( inanimate objects ) have things done to them ( external teleology or design ) . from bacterial antibiotic resistance we can see that even the tiny bacterium displays the sign of great intelligence ( natural genetic engineering ) . unlike inanimate objects , all living cells ( and all living organisms ) create and maintain order . to create and maintain this order every cell has to work like a tiny chemical factory , performing many millions of reactions every second . thus , the proponents of creationist movement/intelligent design argue that an intelligent being is necessary for the creation / design of factory ( example of external teleology ) from the basic elements or parts . this is a more reasonable argument as compared to the imprudent materialistic view that the cell / life is a product of mere accumulation of inert chemicals . however , vednta philosophy is not based on creationist movement/intelligent design or reductionistic materialistic views . according to vednta philosophy an organic whole ( life comes from life or every cell comes from a cell biogenesis ) and an organic whole ( prna ) can not come from the mechanical and chemical additive sum of the parts ( khanda ) . caetano - anolls completely missed this main point , which the paper life and consciousness it is empirically observable that every day sun rises in the east and hence it is logical to conclude that the first sun rise was in the east. there is no problem , if someone wants to dedicate his whole life to do a rigorous scientific research to prove the opposite first sun rise was in the west. the problem arises when keeping such illogical views in mind someone wants to critique those who have the conviction on the obvious vedntic explanation that independent supreme cognizant being is the source of everything is founded on 2 scientifically verifiable axiomatic facts : ( 1 ) life comes from life , and ( 2 ) matter comes from life . consciousness arises from consciousness , or life comes from life . where there is life there is consciousness . consciousness does not originate from that which is unconscious or impersonal , and life is not a product of insentient matter . the conception that life comes from life ( biogenesis ) is the only scientific idea that has ever been verified by experiment and observation . matter comes from life are 2 scientifically observable deductions from vednta . on the other hand , materialism ( life originates from matter ) is an unverified ideological presupposition that has no scientific or observation - based evidence to support it . for a detailed critique of darwinian objective evolution of bodies and abiogenesis ( material origin of life ) , and a further elaboration on vedntic view for soul hypothesis , consciousness and subjective evolution of consciousness one can refer author 's recent paper why biology is beyond physical sciences? , the published book chapter and the article sorry , darwin : chemistry never made the transition to biology. | abstractthe author would like to thank professor gustavo caetano - anolls from department of crop sciences , university of illinois for his interest in his work .
we may sometimes observe that there is a noticeable difference between the anecdote people narrate about the implications of a scientific paper and the real conclusion of the paper .
prof .
gustavo caetano - anolls 's response1 is an ideal example of the same , where he has tried to make great hay about the implications of the article life and consciousness the vedntic view.2 the vedntic view subscribes neither to the views of creationist movement/intelligent design , nor it supports some splendid anti - science proposal .
vedntic view refutes the dominant reductionistic view of life in modern biology by proposing a viable alternative concept of organic whole and thus serves a scientific critique to the nescience ( avidy ) that is practiced on the name of science . |
the technique described provides extracorporeal control of one limb of the suture and has the advantage of continuous tension application on both ends of the thread during knot formation . it is easy to learn , fast to perform , and no new equipment is used . the needle end is advanced through the trocar , and the free end is firmly grasped extracorporeally by the assistant . the needle is passed through the tissues to be sutured and held with the right needle holder . the left - hand instrument holds the filament about 5 cm proximal to the needle , thus resulting in a triangle formation , the apex of it being presented by the tissues to be sutured and the base by the segment of the filament between the left instrument and the needle holder ( figure 1 ) . the needle is rotated around the free end of the filament , which is aligned and held in place by external traction ( figures 2 and 3 ) . as many turns as desired are thrown choosing the appropriate direction : for clockwise throws , the needle is grasped proximally and for opposite throws distally . the surgeon prepares to rotate the needle around the limb of the suture for a first time . accurate and safe tissue suturing is essential for advanced laparoscopic surgery . during the last decade , suturing devices such as clip applicators , staplers , and endo - loops have been developed . extracorporeal knots are easy to perform and are usually created by multiple throws that are advanced intracorporeally with a knot pusher . most important , tension can not be maintained while throwing turns , and the tying instruments before the next throw release the ends of the filament . on the other hand , sequential throw formation in opposite directions is technically demanding , resulting in an unsecured sliding sequence instead of a square knot . another problem is tissue exposure to unnecessary manipulation and traction from pulling long lengths of suture through the needle track and pushing the knot into position . intracorporeal knots seem to deal better with the last issue , and they have the advantage of focusing on the operative field during creation . however , a considerable degree of virtuosity is required , and these knots are not available as an option to occasional laparoscopists . on the other hand , the main problem of preserving ligature tension while forming the knot remains to be settled . even though the technique is an intracorporeal one , continuous tension is guaranteed by providing extracorporeal control of one limb of the suture , thus allowing the manipulation of the needle end with both instruments . winding sequential throws in both directions the procedure necessitates the presence of a curved needle and can be used for ligating tissues that must be securely approximated , as in gastric banding and nissen fundoplication . the greek term for intracorporeal knot is easy to learn and is secure and therefore presents an option not only to the laparoscopic surgeons but to other surgeons as well . | accurate placing of securely tied knots in laparoscopic surgery is technically demanding and time consuming .
surgeons must face difficulties arising from 2-dimensional vision , spatial limitations , and restricted movement .
issues to be taken into account include security , virtuosity , and cost effectiveness .
the authors believe that in spite of advances in instrumentation and optics , training should aim at manual skill development and application of the basic principles of general surgery . |
selective serotonin reuptake inhibitors ( ssris ) are considered to be efficacious and relatively well - tolerated . even though the neurological side effects with ssri are less common , they are presumably also less reported . ssris have been linked with the occurrence of drug - induced parkinsonism , dystonia , dyskinesia , and akathisia . sertraline is an ssri , which has been previously reported to have associated extrapyramidal adverse effects such as akathisia and dystonia . though advancing age has been reported to be a risk factor for ssri - induced extrapyramidal adverse effects , sertraline has been reported to have caused oromandibular dystonia in an adolescent patient . there is a previous report of mandibular dystonia occurring with sertraline , which was prescribed concomitantly with metoclopramide ( a dopamine antagonist ) . here , we describe a patient who developed severe mandibular dystonia with sertraline in the absence of concurrent prescription of medications which have potential action on the dopaminergic system . a 25-year - old single female with a temperamental history of mood swings and low frustration tolerance was on treatment for emotionally unstable personality disorder - impulsive sub - type . she had a past history of two impulsive self - harm attempts of low intentionality and lethality . currently , she presented with a depressive episode of 2 months duration characterized by low mood , lack of interest , ideas of hopelessness , and anhedonia . she did not have significant preexisting medical or neurological illnesses . her metabolic profile , liver , and renal function tests were within normal limits . sertraline 50 mg once daily was started while continuing oxcarbazepine 300 mg / day . within 2 days of starting treatment , she began to experience frequent episodes of sustained involuntary opening of jaw lasting for about 23 min each time suggestive of mandibular dystonia . the naranjo probability score of 6 suggested a relationship between sertraline use and this adverse event . subsequently , the patient was started on mirtazapine 15 mg / day and there was no recurrence of dystonia . in this case , the patient developed acute mandibular dystonia with sertraline , and there were no other medications with known effect on the dopaminergic system . the patient was on oxcarbazepine , which has not been associated with extrapyramidal symptoms previously . drugs which cause variations in the pharmacokinetic factors such as cytochrome p450 enzyme cyp2d6 have been known to increase vulnerability to adverse effects like dystonia . however , oxcarbazepine is metabolized with different liver enzymes and hence this is unlikely to be due to drug - interaction related factors . the etiological factors for dystonia include genetic predisposition , secondary causes such as peripheral traumatic or surgical incidents , neurological disorders and drugs mainly antipsychotics . a review of extrapyramidal manifestations demonstrates that dystonia can also occur with ssris including sertraline . it has been hypothesized that ssri - induced extrapyramidal side effects are probably due to the inhibitory effect , which the serotonergic inputs have on the dopaminergic pathways in the striatum . commonly used medications are anticholinergic agents , benzodiazepams , antiparkinsonism drugs , anticonvulsants , baclofen , carbamazepine , and lithium . anticholinergic medications have been found to be the most effective oral medication , especially when prescribed in early stages . botulinum toxin injection is the next line of treatment , especially preferred in focal dystonia . botulinum toxin blocks acetylcholine release at the presynaptic junction , producing a transient weakening of the muscle activity without systemic effects . it can be immunogenic , and there are rare reports of patients becoming immune - resistant with multiple injections . muscle afferent block , by intramuscular injection of anesthetic and alcohol , has also been used for treatment . in this case , the resolution of dystonia after discontinuation of sertraline and the naranjo scale indicates a causal relationship . it specifically highlights the need for clinicians to be aware of this alarming acute adverse effect with sertraline , which is conventionally considered to be well - tolerated and safe . | specific serotonin reuptake inhibitors have been linked with the occurrence of drug - induced parkinsonism , dystonia , dyskinesia , and akathisia . here , we describe a patient with a diagnosis of emotionally unstable personality disorder and depression who developed severe mandibular dystonia with sertraline in the absence of concurrent prescription of medications , which have potential action on the dopaminergic system .
this case highlights the need for clinicians to be aware of this alarming acute adverse effect with sertraline , which is conventionally considered to be well - tolerated and safe . |
in the past 5 years there has been a substantial shift in thinking regarding the optimal therapy of patients with severe community - acquired pneumonia ( cap ) , particularly with respect to pneumococcal disease . , martinez et al . , baddour et al . and weiss et al . have all identified significant mortality reductions in patients with bacteraemic pneumococcal pneumonia who received combination antibiotic therapy in comparison with patients who received monotherapy . additional observational studies in more general cap cohorts have also identified outcome benefits of combination therapy over monotherapy [ 6 - 9 ] . despite the limitations of these primarily retrospective observational studies , the similar findings in different populations makes it very likely that the association is real . however , it remains unclear whether there is a true survival advantage of combination therapy or whether there are common confounding factors related to patient selection , to the process , to quality or to care . in this issue , mortensen et al . demonstrate that , at least in their region , physicians have widely adopted combination therapy in patients with severe cap . in contrast with previous studies , an important strength is that a large proportion of patients were intubated by severe respiratory failure . the findings of mortensen et al . that fluoroquinolone/-lactam combinations were associated with worse outcome than other combination regimens is both enlightening and disturbing . the most consistent finding across the retrospective studies favouring combination therapy is that it is the addition of a macrolide to a third - generation cephalosporin that has the best outcome [ 1 - 3,6,7,9 ] . what is not clear is the mechanism by which the addition of a macrolide is beneficial . possible explanations include coverage of unrecognized co - infection with atypical pathogens , non - ribosomal anti - pneumococcal activity such as impairment of epithelial adherence , and their increasingly used immunomodulatory actions . , if proved correct , indicate that coverage of atypical pathogens is not the mechanism of benefit because there is no evidence that fluoroquinolones are inferior to macrolides for these pathogens and may even be superior . assuming that the findings of mortensen et al . are real and can be replicated by other groups , what possible explanations are there for the poor performance of fluoroquinolone/-lactam combinations ? first , it is important to remember that this was not a study of single compared with combination antibiotic therapy . no data were presented that suggested that the combination of a -lactam and a fluoroquinolone is worse than either agent separately and there is no in vitro evidence of antagonism between these classes of antibiotics . however , one potential adverse impact of the much broader spectrum of coverage provided by a fluoroquinolone/-lactam combination is the selection of highly resistant nosocomial ( hospital - acquired ) pathogens , particularly pseudomonas aeruginosa , which is the first cause of superinfection in intubated patients . although no data on nosocomial infections were presented by mortensen et al . , it is notable that the survival graph shows a continued disadvantage of initial fluoroquinolone/-lactam combination therapy well beyond 7 days and into the time frame in which nosocomial sepsis would be expected to contribute to mortality . a second possibility , put forward by mortensen et al . , is that their findings favouring macrolides are due to the immunomodulatory properties of this class of antibiotics . in healthy subjects macrolides substantially reduce the in vitro pro - inflammatory response to infectious stimuli , including the key cytokines tumour necrosis factor , il-1 , il-6 and il-8 . however , the reduction in immune response is not global , with minimal to no change in response to interferon- , a key cytokine in the restoration of immune function after sepsis - induced immunoparalysis . macrolides have also been reported to downregulate the production of reactive oxygen species , blocking the activation of nuclear transcription factors , inhibiting neutrophil activation and mobilization , accelerating neutrophil apoptosis , and improving the clearance of mucus . in contrast to macrolides , quinolones seem to have a more global immunosuppressive effect , including significant impairment of interferon- production . the combination of selection for multiresistant pathogens and potential prolongation of post - sepsis immunoparalysis certainly could explain the survival disadvantage observed with fluoroquinolones in comparison with macrolides . are worth exploring , but only if prospective , randomized , double - blind trials confirm the benefit of combination therapy in pneumococcal disease , including a clear benefit of having a macrolide as part of the combination . for a disease as common as cap , with a mortality rate approaching or exceeding 20% in severe disease the large number of different combinations chosen by physicians in the study by mortensen et al . is a clear indication that the therapeutic uncertainty in severe cap is perceived by physicians at the ' front line ' . indeed , other studies [ 18 - 20 ] have suggested that a substantial proportion of clinicians select the empirical antibiotic regimen by using a patient - based policy rather than by following general guidelines . now that there is a strong suggestion that fluoroquinolones may be suboptimal compared with macrolides as one arm of combination therapy in severe cap , conducting prospective , randomized clinical trials including a large proportion of pneumonia severity index of v patients should be a priority . gww is supported by a grant from the national health and medical research council of australia . | recent studies have suggested that combination antibiotic therapy is preferable to monotherapy for severe community - acquired pneumonia ( cap ) . in this issue mortensen and colleagues present retrospective data suggesting that combination therapy with a cephalosporin and a fluoroquinolone is inferior to combination therapy with a cephalosporin and a macrolide .
several mechanisms exist by which quinolones could be inferior to macrolides in combination therapy , so if these findings are confirmed by other groups they have significant implications for physicians treating patients with severe cap . |
computer users who work continuously have frequent work - related neck and shoulder
problems1 . it is difficult to identify a
clear reason for neck and shoulder pain and impairment , which contribute to a poor patient
prognosis2 . mclean suggested that the
sustained forward head posture during computer work decreases muscular efficiency and
increases activity of the upper trapezius muscle3 . however , other researchers have reported that a flexed - relaxed
trunk posture can be easily adopted to maintain posture with low muscular requirement . this
posture is maintained with passive structural tension rather than active muscular
effort4 . park and yoo reported muscle
activity changes over time and showed a decrease in upper trapezius activation in the last
phase compared to the second and third phases of sustained typing5 . they suggested further study to evaluate pain and changes
in activation of the upper trapezius during sustained typing5 . therefore , this study was conducted to compare activation and
changes in parts of the upper trapezius muscle between subjects in pain and those not in
pain during computer work . ten right hand - dominant male computer workers ( age , 2632 years ; mean height and weight ,
173.6 6.2 cm and 68.4 5.5 kg , respectively ) participated in this study . the subjects
used computers for 6 hours / day as full - time workers . subjects with conditions that may have
affected mobility of the cervical spine injury or who had neurological deficits in the neck
or upper extremities during the previous year were excluded from the study . the subjects
received an explanation about the purpose and methods of the study prior to their
participation and provided informed consent according to the ethical principles of the
declaration of helsinki . all subjects performed computer work for 1 hour using the same
computer workstation , in which the monitor was inclined back 20 , and their eyes were 0.8 m
from the monitor . to ensure that the hips and knees were flexed at 90 , an adjustable - height
table and chair without a backrest were used to set the initial sitting posture . during the
experiment , all subjects performed selected keyboard typing in the korean version of the
hansoft program . electromyographic ( emg ) data were collected using the mp150 acquisition
system unit , the acknowledge software package , and surface emg electrodes ( biopac systems
inc . , the channel of the emg device was used to detect activation of the
right side of the upper trapezius muscle . the electrodes were placed approximately 2 cm
laterally from the mid - distance between the c7 spine and the acromion on the upper trapezius
muscle . the subjects pressed the switch when
they felt pain in the right upper trapezius muscle . the spss for windows software
package ( ver . 18.0 ; spss inc . , chicago , il , usa ) was used to analyze differences and changes
( difference between minimum and maximum activation ) in upper trapezius muscle activation in
the feel - pain and non - feel pain sections during computer work . an independent t - test was
utilized to detect the differences and a value of p < 0.05 was taken to indicate
statistical significance . mean normalized upper trapezius muscle activity ( 33.1 14.4% ) did not differ between the
feel - pain and non - feel pain muscle sections ( 29.3 12.1% ) . the mean change in upper
trapezius muscle activity in the feel - pain section ( 7.6 4.2% ) decreased significantly
compared to the non - feel pain section ( 15.0 9.5% ; p < 0.05 ) . the upper trapezius is an important component of the shoulder pain associated with computer
work6 . clinicians usually focus on the
upper trapezius when treating shoulder pain in computer workers7 , 8 . we found no
difference between the feel - pain and non - feel - pain sections in mean normalized upper
trapezius muscle activity . higher activity of the upper trapezius muscle can be interpreted
as bad or good , and suggests muscle hypertension or postural variation , respectively . muscle
hypertension is present if more activity is needed to maintain the same posture . however , if
more muscle activity is used to vary posture , this may help relieve musculoskeletal loading .
therefore , we hypothesized originally that muscle activity would not be useful to detect
pain . hgg suggested that degeneration of type-1a muscle fibers is caused by overuse , which
induces pain in the upper trapezius region9 . this hypothesis suggests that the pain is caused by a long period
of low upper trapezius motor unit recruitment9 . sustained activation of this specific muscular region could promote
muscular damage , even with a low level of muscular recruitment9 . we found that the mean change in upper trapezius muscle activity decreased
significantly in the feel - pain muscle section compared to the non - feel pain section . thus ,
changes in the activities of the muscle sections were of more value when studying static or
sustained muscle stress , such as that associated with computer work . | [ purpose ] activation and changes in parts of the upper trapezius muscle during painful
and non - painful computer work were compared .
[ subjects ] ten male computer workers were
recruited .
[ methods ] surface electromyography was used to compare upper trapezius muscle
activation and changes ( difference between minimum and maximum activation ) during painful
and non - painful computer work .
[ results ] mean normalized upper trapezius muscle activity
did not differ between the feel - pain and non - feel - pain
muscle sections .
the mean
change in upper trapezius muscle activity in the feel - pain section decreased significantly
compared to the non - feel - pain section .
[ conclusion ] measuring changes in the activity of
muscle sections was useful to study static and sustained muscle stress during computer
work . |
angiomatosis is a rare vascular lesion characterised by diffuse proliferation of blood vessels with accompanying mature adipose tissue . it affects a large segment of the body in a contiguous fashion either by vertically involving multiple tissue types ( e.g. , subcutis , muscle , bone ) or by involving similar tissue types ( e.g. , multiple muscles ) . it is primarily seen in the first two decades of life with a slight female predilection . surgical removal of angiomatosis is very difficult and is associated with a high recurrence rate . angiomatosis is usually seen in lower extremities followed by the chest wall , abdomen and upper extremity . we report an unusual case of angiomatosis seen in the face of a 4-year - old boy with an initial clinical diagnosis of hemangioma . a 4-year - old boy presented with a diffuse swelling involving left cheek and left side of upper lip since birth causing disfigurement of his face . the diffuse lump started as a small red nodule at birth which gradually increased to the present size with the growth of the child . , there was a diffuse , ill defined , soft , non - compressible swelling in the left cheek and left side of the upper lip [ figure 1 ] . hyperpigmented spots were also noted on the skin of the left cheek . there was no bruit on auscultation . large diffuse swelling seen on the left lower face involving left cheek and upper lip doppler ultrasound was done which revealed a vascular lesion with doubtful communication with left facial vein . in view of the clinical presentation and inconclusive doppler report , a possibility of hemangioma followed by arteriovenous malformation was kept . propanolol was started at the dose of 0.5 mg / kg and increased to 2 mg / kg but the swelling persisted . however , there was no improvement in the size of the lump , despite treatment for 8 months . the aim of the surgery was to explore the cheek and lip and attempt debulking . during surgery , it was found that there was dense fibrosis in the cheek with infiltration into underlying tissue and muscle resulting in difficult excision . microscopically , haphazard proliferation of capillary - sized vessels were seen mostly adjacent to vein wall along with abundant adipose tissue . numerous nerve bundles , few of them showing myxoid change were also seen [ figure 2 ] . however , no arteries or arterioles were seen and vessel wall was negative for elastic tissue ( verhoffs stain ) . based on the clinical details and histopathological findings , a final diagnosis of angiomatosis was given . photomicrographs showing clusters of capillaries adjacent to vein wall infiltrating the adipose tissue with admixture of thick walled blood vessel , mature adipose tissue and nerve bundles . the diagnosis and management of vascular lesions continue to pose a challenge for the histopathologists as well as the treating surgeon . however , it differs from hemangioma in its infiltrative nature and propensity for local recurrence . angiomatosis be used to connote a histologically benign vascular lesion that extensively involves a region of the body or several different tissue types in a contiguous fashion . angiomatosis is a histologically benign vascular lesion which involves a region of the body or several different tissue types in a contiguous fashion . lesions reported radiologically as vascular malformation may in some instances correspond to what pathologists term angiomatosis . congenital form maybe sporadic or seen in association with certain syndromes such as klippel trenaunay syndrome , sneddons syndrome or gorham disease . reported the first documented case of klippel trenaunay syndrome associated with visceral angiomatosis ( gastrointestinal and genitourinary ) . angiomatosis usually presents in the first two decades of life mostly in childhood or adolescence with a slight predilection for females . reported 17 cases of angiomatosis presenting in children with recurrence seen in 10 patients and multiple recurrences in four patients . we present a rare case of angiomatosis in the face ( cheek and lip ) . angiomatosis has also been reported from other sites such as heart , abdominal wall , forearm , retroperitoneum and genitalia . the latter usually involutes by 2 year of age but vascular malformation grows persistently with age and does not disappear . as our patient did not respond to the bleomycin treatment for hemangioma , excisional biopsy and subsequent histopathological examination was planned . the most common pattern consist of haphazard proliferation of varying sized vessels and clusters of capillary vessels adjacent to vein walls . in second pattern , a central large vessel is surrounded by clusters of capillary - sized vessels arranged in nodules . however , a distinctive feature common to both types is presence of large amount of adipose tissue . in the present case , large amount of mature fat frequently accompanying the vascular elements seen in angiomatosis suggest that this lesion may possibly be a generalised mesenchymal proliferation rather than an exclusively vascular lesion . histologically , this lesion needs to be differentiated from angiolipoma because of the coexistence of vascular proliferation and abundant fat . however , in angiolipoma proliferating vessels are usually concentrated at the periphery of intratumoral lobules of adipocytes and the lesion is quite well circumscribed . angiomyolipoma can be differentiated from angiomatosis as it contains smooth muscle in addition to blood vessels and fat while angiomyxolipoma is distinguished by the presence of myxoid change in adipose tissue and absence of haphazard blood vessel proliferation . val bernal et al . reported a case of soft tissue angiomatosis closely mimicking liposarcoma due to the presence of large mass with prominent myxoid adipose tissue . features such as mitotic figures , cellular atypia and presence of typical lipoblasts help to differentiate liposarcoma from angiomatosis as they are not seen in latter . large amount of mature fat frequently accompanying the vascular elements seen in angiomatosis suggest that this lesion may possibly be a generalised mesenchymal proliferation rather than an exclusively vascular lesion . complete resection is preferred in local angiomatosis while radiotherapy or interferon 2a is the treatment of choice in extensive angiomatosis . however , about 90% of patients demonstrate local recurrences on follow up . through this article , we wish to highlight in a young patient presenting with diffuse persistent vascular swelling of the head and neck region , a possibility of angiomatosis , though rare , should be considered . the correct histopathological identification of this uncommon entity and its differentiation from the more innocous vascular lesion , i.e. , hemangioma is important , considering its high recurrence rate . | angiomatosis is a diffuse vascular lesion which clinically mimics hemangioma or vascular malformation .
it usually involves multiple tissues and is histopathologically characterised by proliferation of vessels of varying calibre intimately admixed with large amount of adipose tissue .
its surgical removal is very difficult because of its infiltrative nature .
therefore , a precise histopathological diagnosis is of utmost importance .
it is usually seen in females in the first two decades and commonly involves lower extremities .
angiomatosis of head and neck region is very rare . here
we present a rare case of angiomatosis of the lower face involving right cheek and lip in a 4-year - old boy clinically diagnosed as hemangioma .
histopathological differential diagnosis of angiomatosis is also discussed . |
motions like reaching are influenced by lesions affecting the sensorimotor system , and an
understanding of the influence of such lesions on movement control and physical motion
compensation is therefore essential for rapid rehabilitation1 . altered upper - limb function in patients with brain damage can make
it difficult to perform daily activities2 .
one study suggested that improving trunk control in hemiplegic patients greatly reduced the
problems related to motion during task performance and had a large impact on improving task
performance ability3 , 4 . to fully understand arm motion , it is important to study trunk
motion and compensatory strategies of the elbow joint3 . after comparing performance in ipsilateral and contralateral
reaching tasks , adamovich et al.4 proposed
guidelines for proper coordination of trunk movements . additionally , levin et al.5 reported that when the arm and body were in
close proximity , they worked together during the final stage of a reaching process , whereas
when they were farther apart , they worked together in the initial stage . many other studies
have emphasized the need for coordinated motion of the trunk to ensure proper reaching
movement . however , few studies have examined the change in the center of mass associated
with changes in the coordinated motion of the trunk . therefore , this study compared the
reaching velocity of the upper trunk and the center of force ( cof ) during a reaching task
with the dominant and nondominant hands . ten males ( age 2030 years , mean height 174.1 3.2 cm , mean weight 64.2 5.0 kg )
participated in this study . the subjects had no history of musculoskeletal disorders or pain
associated with the upper extremity in the past 6 months . this study was approved by the
inje university faculty of health science human ethics committee , and all subjects provided
written informed consent before participating . a three - dimensional ( 3-d ) ultrasonic
motion - analysis system ( cms - hs , zebris , medizintechnik , isny , germany ) was used to determine
the reaching velocity and frontal distance of the upper trunk . the sensor , which consisted
of three microphones used to record the ultrasonic signals , sampled two markers at 30 hz . one marker , located on the middorsal aspect of the wrist , was used to measure the reaching
velocity . the other marker , located on the acromion process , was used to measure the forward
movement of the upper trunk . the windata software ( cms - hs , zebris , medizintechnik , isny ,
germany ) was used to analyze the movement in the reaching task . the cof during the reaching
task was measured using a conformat system ( model # 5330 , tekscan , boston , ma , usa ) . this
system is a portable pressure - mapping system that captures the seat pressure distribution
and contact area . during the reaching task , the subjects sat on a chair with a flat seat and
no back support in front of a height - adjustable table . the hip , knee , and ankle joints were
positioned in 90 flexion , and the feet were positioned shoulder - width apart with the
subject seated on a conformat sensor on the chair . the subjects were required to maintain an upright
trunk with both hands resting on the thighs . the target was positioned directly in front of
the subject at a distance equal to 2/3 of the subjects arm length . arm length was defined
as the distance between the acromion and the tip of the middle finger of the subject s
left / right arm . differences in reaching velocity , trunk movement , and cof during the reaching task with the
dominant and nondominant hands were analyzed using independent t - tests . there was no significant difference in reaching velocity between the dominant and
nondominant hand during the reaching task ( 0.720.10 sec vs 0.790.23 sec ; p<0.05 ) . the
forward movement of the upper trunk reaching with the nondominant hand ( 4.73.1 cm ) was
significantly decreased when compared with reaching with the dominant hand ( 13.37.2 cm ;
p<0.05 ) . the cof movement in reaching with the nondominant hand ( 2.52.0 cm ) was
significantly decreased when compared with reaching with the dominant hand ( 7.82.7 cm ;
p<0.05 ) . this study compared the reaching velocity and movement of the upper trunk and center of
force during a reaching task using the dominant and nondominant hands . postural stability is
the ability to maintain the center of pressure within a specific space6 . reaching tasks in the sitting position generate a voluntary
perturbation and require postural control to change the center of gravity relative to the
support surface . to complete seated reaching tasks , coordinated movements are necessary , and
weight transfer to the lower extremities is essential6 . crosbie et al.7
reported that when young adults were instructed to reach to 160% of their arm lengths as
rapidly as possible , over 70% of the body mass was transferred to the feet . using 3-d motion
analysis , yoo et al.8 found that the arm
maintains the same position as it reaches for a target and that the trunk moves according to
the position , with different strategies used depending on whether the distance the hand
moves is longer or shorter than the arm length . the dominant and nondominant hands can be
considered the technical and nontechnical hands , respectively . for the hand that had not
practiced the reaching task in the present study , the reaching velocity was not very
different from that of the dominant hand . however , no forward movements were observed in the
trunk and center of pressure . this can be considered the result of performing a nontechnical
reaching task with the nondominant hand . clinically , when therapists train patients to
perform a reaching task , the performance of the task is generally evaluated as improved if
the reaching velocity is increased . previous researchers conducted an experiment in which 30
hemiplegic patients pressed a switch in front of them with either one hand or two hands when
it lit up9 . however , an evaluation can not
be made using velocity alone . according to our findings , the trunk and center of the body
need to move forward for an effective technical movement . therefore , when evaluating the
reaching performance of patients clinically , it is necessary to evaluate trunk movement ,
reaching velocity , and the subsequent movement of the center of the body . | [ purpose ] the purpose of this study was to compare the reaching velocity of the upper
trunk and the center of force ( cof ) during a reaching task with the dominant and
nondominant hands .
[ subjects ] ten males between the ages of 20 and 30 years participated
in this study . [ methods ] this study measured the reaching velocity , upper trunk movement ,
and center of force movement during a reaching task using the dominant and nondominant
hands . [ results ] there was no significant difference in reaching velocity between the
dominant and nondominant hands in the reaching task
. the forward movement of the upper
trunk and cof movement in reaching with nondominant hand were significantly decreased
compared with those of the dominant hand .
[ conclusion ] therefore , when evaluating the
reaching performance of patients clinically , it is necessary to evaluate trunk movement ,
reaching velocity , and the subsequent movement of the body center . |
revision of the acetabular component in total hip arthroplasty occurs for reasons such as polyethylene wear and osteolysis , recurrent dislocation , and mechanical failure . contrary , the removal of a well - fixed cemented acetabular can cause bone fractures , excessive bleeding , as well as extended bone loss , namely in osteoporotic bone . the indications for revision of a well - fixed acetabular component include malposition , infection or polyethylene wear . this can be achieved with the use of drills , screws , reamers , curved blades , gouges , chisels , and osteotomes . in revision hip arthroplasty one of the priorities of the surgeon we report a technique using 2 cork - screws for removal a stable cemented acetabular component due to malposition . a 74-year - old woman underwent a revision hip surgery for the treatment of a recurrent dislocation of a cemented total hip arthroplasty ( eight dislocations ) , by malposition of the acetabular component ( fig . the all - polyethylene acetabular liner was perforated with a 4.5 mm drill , and two cork - screws were firmly screwed in the rim as far as possible , in order to extrude the liner from the cement mantle , and to create also fissures into the cement ( fig . manual torsional shear forces were carried out , which led to a total disruption of the polyethylene liner at the polyethylene - cement interface . adequate manual torsional shear forces were carried out , which led to the disruption of the polyethylene liner at the cement - polyethylene interface , with no technical difficulties . the cement mantle was removed in a piecemeal fashion and the cemented plugs were carefully curetted out . a roof reinforcement ring and a cemented cup of uhmw polyethylene were implanted using the standard technique . no complications were reported in the perioperative course or during the hospitalization period . at 2 month well - fixed acetabular component may be removed in cases of recurrent dislocations by malposition of the acetabular prosthesis . removal of a stable cemented acetabular component in revision total hip arthroplasty can be challenging , time consuming , and potentially detrimental to the remaining host bone . the technique depicted here has been used successfully in our unit for several years , in cases where the polyethylene cemented liner presented no signs of loosening . the acetabular implant and the cement may be removed safely , under direct vision . on the other hand , if this technique fails it is possible to carry out others methods for the extraction of the acetabular implant . the removal process can be performed with sequential acetabular reamers to thin the liner , and allow easier extraction of the implant . the cork - screw femoral head extractor is a standard instrument in orthopedic surgery , found in most hip hemi - arthoplasty instrument sets the bone cement ( polymethylmethacrylate ) is strong in compression , but breaks when twisted . greater manual control is obtained , facilitating the manipulation of the acetabular component in different directions , tensile forces are applied , and the cup - cemented bond can be disrupted . the cement mantle may be removed in a piecemeal fashion , the host bone is preserved and the risks of complications are minimized . utilizing simple instrumentations , a new acetabular prosthesis may be implanted in adequate technical conditions . to our knowledge , this procedure using two cork - screws for removal an acetabular implant has not been described in orthopedic literature . one of the more difficult procedures in revision total hip arthroplasty is removal of a well - fixed femoral stem or acetabulum . the procedure described here for removal a well - fixed cemented acetabular component using 2 cork - screws is a simple , reproducible , non - timing consuming , non - costly , and bone preserving technique . written informed consent was obtained from the patient for publication of this case report and any accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal upon request . all authors contributed to the writing and collection of data , and they have participated in the surgery . | highlightsremoval of a stable all - polyethylene cemented line in revision hip arthroplasty can cause bone fractures , excessive bleeding , as well as extended bone loss , namely in osteoporotic bone.one of the priorities of the surgeon must be the preservation of the remaining bone stock , and the reimplantation of a new acetabular prosthesis with no technical difficulties and risks.the surgical procedure described here contributes to this objective . |
a 32-year - old female visited our hospital due to her nonspecific neck discomfort for several years . sonography was performed on an acuson unit with a 15-mhz linear transducer ( acuson , mountain view , ca ) , and a 0.5 cm sized hypoechoic nodule with ill - defined margins and a taller shape was found in the right portion of the thyroid gland . another 1.8 cm sized anechoic cystic lesion with oval shape and well - defined margin was also noted at the posterior aspect of the mid - portion of the thyroid , and it was superior to the suspicious malignant nodule ( figs . the patient underwent sonography - guided fine needle aspiration of the suspicious malignant nodule . at the same time , we also aspirated the cystic lesion in right portion of the thyroid gland . the aspirated fluid was measured as 1 cc and showed as a thick yellowish pus - like fluid material . the cystic lesion was thought of parathyroid cyst or other complicated cyst , so aspirated fluid was sent for cytology cultures for bacteria and a check of the parathyroid hormone ( pth ) level . the cytology results of the suspicious malignant nodule were consistent with a papillary cancer ; there were no follicular cells and a few squamous cells in the cytology results of the cystic lesion . the pth level of cystic fluid showed to be normal and there were no bacteria on culture . histopathologic examination about the suspicious malignant nodule on us revealed micropapillary carcinoma , the same as the preoperative cytology report . the 1.8 cm sized cystic lesion superior to the cancer was lined by ciliated pseudostratified columnar epithelial cells , and its diagnosis was a branchial cleft cyst ( fig . branchial anomalies may result from the abnormal persistence of branchial apparatus remnants , and they present around each of the developed brachial derivates . a branchial cleft cyst is an epithelial - lined structure without an external opening , and it is usually located in the lateral areas of the head and neck ( 1 , 2 ) . first branchial cleft cysts are intimately associated with the external auditory canal and the parotid gland ( 1 ) . second branchial cleft cysts are found along the anterior border of the sternocleidomastoid muscle and most commonly present just lateral to the internal jugular vein at the level of the carotid bifurcation ( 3 ) . anomalies of the third and fourth branchial clefts are relatively uncommon and the distinction between third and fourth branhcial anomalies remains controversial , primarily because both lesions similarly present around the piriform sinus ( 1 ) . the exact histogenesis of a intrathyroidal branchial cleft cyst is unclear , but probably the failure of the third or fourth branchial pouches to atrophy and dissipate in utero results in the cysts or the sinus tracts that lie in close proximity to , or inside , the thyroid gland ( 2 , 5 ) . whatever their origin , histologic analysis reveals that they are generally well - circumscribed cysts lined by straight squamous or pseudostratified columnar epithelium , and they have abundant lymphoid tissue and follicles beneath the epithelium . the cystic contents may be clear , watery to mucinous fluid , or the cyst may contain desquamated , granular cellular debris and if this becomes infected , it may be yellowish pus - like fluid ( 3 , 4 ) . generally , the diagnosis of branchial cleft cysts is relatively easy when it is located in its classical position , and sonographically the cyst appears as an anechoic mass or a predominantly hypoechoic , cystic mass with faint internal debris and posterior enhancement . however , on real - time images , after the application of transducer pressure on the cyst , the entire contents may shift , suggesting their true cystic nature ( 4 ) . preoperatively , how can we arrive at the the diagnosis of intrathyroidal branchial cleft cyst ? although some prior reports have suggested that intrathyroidal branchial cleft cysts should be considered in the differential diagnosis of thyroid cystic lesions , and especially in the patients with hashimoto 's thyroiditis , yet in view of the rarity of the lesion , the diagnosis can be suggested only by histopathologic confirmation , as was the case in all the previous case reports ( 2 , 6 ) . generally , in clinical practice , when thyroid cysts are detected by sonography , we first suggest that it is a colloid cyst or a true epithelial thyroid cyst . if cysts are presented in the posterior portion of the superior or inferior pole of the thyroid , we also should consider the possibility of parathyroid cysts ( 7 ) . but , fine - needle aspiration of yellowish , green viscous fluid or the sonographic appearances of a pseudosolid , cystic nodule in the thyroid should alert the sonographer to the possibility of a congenital developmental cyst ( 6 ) . thyroglossal duct cysts , the one of congenital developmental cysts , also have been reported to present as thyroid masses . the distinguishing features between a thyroglossal duct cyst versus a branchial cleft cyst include presentation of the mass , with thyroglossal duct cysts more likely to be midline neck masses while branchial cleft cysts are typically lateral neck masses , and frequency of infection , branchial cleft cyst are more likely to be infected ( 8) . histology of the cellular components of the cyst wall and surrounding tissue is the most valuable method of differentiating the two ( 8) . but , preexisting inflammation may cause metaplasia of the lining of a thyroglossal duct cyst , while may make histologic differentiation from a branchial cleft cyst difficult ( 8) . when we reviewed the previous case reports for intrathyroidal branchial cleft cysts , there was no specific finding on the sonography studies . but in a few cases , there was creamy green or turbid yellowish fluid aspirated during fine needle aspiration cytology for intrathyroidal branchial cleft cyst , as like our case ( 4 , 8) . in conclusion , the differential diagnosis for a cystic lesion that lies in close proximity to , or inside , the thyroid gland on sonography includes colloid cyst , true epithelial thyroid cyst and parathyroid cyst . the definite diagnosis of thyroidal branchial cleft cyst will be made postoperatively ; yet if on fine - needle aspiration of the cyst a yellowish , pus - like viscous fluid is aspirated , branchial cleft cyst should be considered in the differential diagnosis of the thyroid cystic lesion . | we report here on an extremely rare case of an intrathyroidal branchial cleft cyst .
intrathyroidal branchial cleft cyst is rare disease entity and it has nonspecific findings on sonography , so the diagnosis of the lesion is very difficult .
however , during aspiration , if pus - like materials are aspirated from a thyroid cyst , we should consider the possibility of intrathyroidal branchial cleft cyst in the differential diagnosis . |
major complications such as injury of the bowel or the bladder happen in < 1% of the cases . postsurgical fever and small hematoma formation on the abdominal wall at the trocar entries are more common but less severe . incidental appendectomies used to be performed frequently , but a reversal of trend happened in the 1990s . the most popular theory of pathogenesis of acute appendicitis is the obstruction by fecalith , lymphoid hyperplasia or adhesions of the appendiceal lumen with subsequent secondary infection . however , most of the patients with appendicoliths composed of firm feces and some mineral deposits are asymptomatic , but appendicoliths may also cause serious appendicular inflammation and peritonitis . moreover , in case of abdominal pain in the postoperative period , most physicians would probably first suspect a complication such as bleeding , infection or injury of the bowel or bladder . as it is important to be aware of other causes for the condition , such as acute appendicitis , we herein describe a case of acute appendicitis 3 days after a laparoscopic ovarian cystectomy . a 26-year - old nulliparous woman presented with right lower and upper abdominal pain 3 days after laparoscopic right ovarian cystectomy ( dermoid ovarian cyst ) and myomectomy of a small subserous myoma . she was discharged from the hospital with more abdominal pain than expected , but still in a normal range . the pain increased on her second postoperative day in the evening and the next day , it became severe with concomitant nausea and vomiting . inconspicuous appendix during first laparoscopy . physical examination revealed normal vital signs , severe diffuse right abdominal tenderness , generalized rebound tenderness , guarding accentuated in the right lower abdomen and rare bowel movements . laboratory evaluation showed a slightly elevated c - reactive protein ( crp ) ( 17 mg / l , reference < 5 mg / l ) and an elevated leukocyte count ( 18 000 cell / mm , reference < 10 200 cell / mm ) . transvaginal and transabdominal ultrasound scanning revealed a normal uterus , a small fluid collection in the douglas space , an already normal - appearing right ovary , no free fluid in the morison s pouch , no pathology of the gallbladder and no lesions suspected to be an abscess . but it showed an increased diameter of the appendix of 8.2 mm , target sign on axial scan of the appendix , an appendicolith and periappendiceal fluid of ~2 ml ( fig . 2 ) . therefore , an immediate re - laparoscopy was indicated , which showed an acute appendicitis and peritonitis ( fig . pathologic evaluation of the appendix showed an ulcero - phlegmonous , partly abscessing appendicitis , a distal appendicolith of 0.5 cm in diameter and acute peritonitis . ( picture by markus buerge , md )
figure 3:appendicitis with local peritonitis 3 days after fig . ( picture by markus buerge , md ) appendicitis with local peritonitis 3 days after fig . 1 . however , we hypothesize that appendicitis particularly in cases of appendicolithiasis might be triggered by laparoscopic abdominal surgery by influencing yet - unknown factors . therefore , post - laparoscopic appendicitis might be rather a complication than a coincidence . as we did not find any other case in the literature , there might be very few cases of post - laparoscopic appendicitis or these cases might be underreported . however , one should be aware of appendicitis as a cause of acute postoperative abdominal pain , even if the appendix appeared inconspicuous during the first operation . appendicoliths appear to play a role in the pathogenesis of acute appendicitis and are associated with complicated appendicitis . whether an elective appendectomy in patients with incidentally recognized appendicoliths is indicated or not , is discussed controversially . some authors recommend considering an elective appendectomy in such cases , whereas others conclude that the incidental finding of an appendicolith without signs of appendicitis on imaging studies is no indication for appendectomy [ 7 , 8 ] . however , if an elective laparoscopy is indicated for another reason , such as ovarian cystectomy , myomectomy or endometriosis , according to certain authors incidental appendectomy may be of benefit in young patients in the prevention of future morbidity and mortality following an acute appendicitis as well as it may reduce disability , time and costs of diagnostic work - up in case of future abdominal pain [ 9 , 10 ] . incidental appendectomies in women undergoing gynecological laparoscopy have an extremely low complication rate , require a short amount of time and are cost - effective . however , the extent of long - term complications of this potentially superfluous additional trauma , such as adhesions followed by bowel obstructions and infertility , is unknown . also , one should keep in mind the potential adverse effects of an appendectomy , such as the possible association between appendectomy and an increased risk for colorectal cancer and crohn s disease . nonetheless , further study about the risk and benefit of incidental appendectomies particularly in young patients with appendicolithiasis undergoing elective abdominal surgery might be of great value as one might reduce morbidity and costs by reintroducing incidental appendectomies in cases of appendicoliths . and one should always consider acute appendicitis as a possible cause of post - laparoscopic abdominal pain . | diagnosis of appendicitis , particularly in young women , may be challenging . in case of abdominal pain in the postoperative period of laparoscopic surgery
, one should not only think of complications such as bleeding and injury of the bowel but also such as acute appendicitis .
we report a case of a 26-year - old female patient with a post - laparoscopic acute appendicitis with appendicolithiasis 3 days after a laparoscopic ovarian cystectomy during which the appendix appeared inconspicuous .
appendicitis after gynecologic laparoscopy is a rare but potentially dangerous condition .
one should consider the possibility of a postoperative appendicitis in case of an acute abdomen after laparoscopic surgery .
further studies might be of value to re - evaluate incidental appendectomy especially in cases of appendicolithiasis . |
a 30-month - old boy initially presented with hip joint pain and fever , which failed to improve despite treatment that was given under the impression of a septic hip at a local hospital . one month later , he was admitted to another hospital due to progressed multifocal bone pain . the imaging work up , including bone scanning and mri for the head , neck and pelvis , suggested multiple metastatic lesions in the skeleton and bone marrow . a 2.5 cm sized left adrenal mass was detected on the contrast - enhanced ct ( fig . 1a ) . on the laboratory studies , the serum neuron - specific enolase ( nse ) and ferritin , and the urine vanillymandelic acid ( vma ) were elevated to 72 ng / ml , 1,245 ng / ml and 42 mg / day , respectively ( normal ranges : 0 - 12 ng / ml , 22 - 322 ng / ml , and < 3 mg / day , respectively ) . therefore , the patient was diagnosed with stage 4 neuroblastoma of a left adrenal origin , according to the international neuroblastoma staging system . subsequent chemotherapy was started , and this was followed by surgical resection of the residual adrenal lesion several months later . the pathologic diagnosis of the primary lesion was ganglioneuroblastoma with the large portion of the tumor being differentiated into ganglioneuroma , which was caused by the previous chemotherapy . after additional high dose chemotherapy followed by autologous peripheral blood stem cell transplantation , he was clinically considered to be in nearly complete clinical remission . the patient did well until recurrent abdominal pain developed at four years of age . at that time , contrast - enhanced ct scan of the abdomen demonstrated multiple small low attenuated nodular lesions in the pancreas ( fig . three weeks later , the laboratory examinations revealed that the amylase and lipase were elevated to 538 u / l and 1,023 u / l , respectively ( normal ranges : 13 - 100 u / l and 13 - 60 u / l , respectively ) . contrast - enhanced abdominal ct showed diffuse swelling of the pancreas with an increase in the size of multiple pancreatic masses throughout the entire pancreas ( fig . the chest ct that was performed for a palpable nodule in the sternal area also showed a destructive soft tissue mass of the sternum . the nse was also elevated to 95 ng / ml ( normal range : 0 - 12 ng / ml ) . after two weeks , progressive dilatation of the intrahepatic duct was noted on the follow up sonography ; this was due to obstruction of the common bile duct by the pancreatic mass ( fig . the patient 's pancreatitis was presumed to be present secondary to pancreatic metastasis . because the patient was in very poor general condition and he showed a rapid clinical decline with severe cholestasis the little boy was managed both conservatively and compassionately ; sadly , he expired one month after the diagnosis of relapse . neuroblastoma accounts for 8 - 10% of all malignancies in childhood and it occurs in the adrenal medulla and along the sympathetic nervous system by tumor arising from neuroblasts ( 1 , 2 ) . the prognosis depends on the age and stage at the time of diagnosis . infants younger than one year or patients with localized tumor have a good prognosis , whereas approximately 70 - 80% of patients older than one year have metastatic disease at diagnosis and they have a poor prognosis with a high recurrence rate in spite of receiving the best current multimodality therapy ( 1 , 2 ) . common sites of metastasis , via lymphatic and hematogenous routes , are the bone , bone marrow , lymph nodes , liver , dura and lung ( 1 , 2 ) . bone metastasis is common in children older than one year , and this often involves the long bones and orbit , which causes bone pain or proptosis . the unusual metastatic sites that were previously reported include the cns , kidney , and the cardiac and skeletal muscles ( 3 - 5 ) . to the best of our knowledge , pancreatic metastasis is generally uncommon from any source , even if it has been reported that the colon , lung , and kidney are the most common sites of primary tumors that metastasize to the pancreas ( 6 ) . especially in children , pancreatic metastasis was reported in patients suffering with leukemia , lymphoma , ewing sarcoma and rhabdomyosarcoma ( 7 , 8) . in the presented case , we were not certain at first that the multiple nodules found in the pancreas represented metastasis of neuroblastoma . however , as the pancreatic nodules increased in size , and this was associated with metastatic lesion in sternum and with the corresponding increased mibg uptake and increased nse value , then the diagnosis of pancreatic metastasis was made . performing tissue diagnosis for the pancreatic lesion was difficult to achieve in this seriously ill little boy and it was simply not justified when the clinical , laboratory and imaging findings were sufficient to make the diagnosis of metastatic relapse . it 's likely that there were residual tumor cells that became disseminated through the blood and lymphatic systems , and this caused early relapse at multiple sites , including the pancreas . as in our case , pancreatic metastasis may be complicated by biliary obstruction and pancreatitis . metastasis - induced acute pancreatitis can occur as an initial manifestation of tumor or it can happen later during the course of disease ( 9 , 10 ) . although the mechanisms to explain this phenomenon still remain controversial , it has been assumed that obstruction of the pancreatic duct by tumor , and the secretion of plasminogen activating enzymes that in turn activates trypsinogen - inducing autodigestion , may cause pancreatitis ( 9 , 10 ) . whatever the mechanism is , metastasis induced pancreatitis may result in a very poor outcome as in our case ; this is especially likely when it occurs together with extensive disease or severe pancreatitis ( 10 ) . in conclusion , we present here a very rare case of neuroblastoma that metastasized to the pancreas in a 4-year - old boy . pancreatic metastasis should be taken into consideration for those patients who are found to have pancreatic nodules concurrent with neuroblastoma . | we present here a very rare case of metastatic relapse in the pancreas of a 4-year - old boy who had been treated for stage 4 neuroblastoma .
computed tomography showed multiple metastatic masses in the pancreas with secondary pancreatitis . to the best of our knowledge ,
this is the first case report of pancreatic metastasis in a child with neuroblastoma . |
a subclavian artery aneurysm ( saa ) is a rare peripheral aneurysm.1 although rare , an saa can cause life - threatening complications such as rupture , thrombosis , and embolism.2 recently , we experienced a case of a ruptured saa presenting as hemoptysis , which was successfully treated by endovascular stenting . two weeks previously , he had visited the local hospital for atypical chest discomfort that had increased over the past 2 weeks . at that time , computed tomography ( ct ) of the thorax had revealed a small aneurysm of the left proximal subclavian artery ( fig . contrast - enhanced ct showed 7 cm of loculated fluid collection with peripheral wall enhancement in the upper hemithorax and an approximately 2.5-cm irregular contrast filling sac in the proximal portion of the left subclavian artery with scattered vascular calcification ( fig . no other aneurysms were noted in the brain , neck , chest , or abdomen ct scans . because of the patient 's refusal to undergo an operation , endovascular treatment with a graft stent was performed . the procedure is shown in fig . 3 . an angiogram using a pigtail catheter via the right femoral approach was performed to delineate the anatomy and confirm the aneurysmal leakage . because the diameter of the saa was very large on the angiogram , the endovascular stent was approached by a bilateral access technique in which the bulky stent graft was introduced retrogradely via cutdown of the left axillary artery and an 8-fr sheath in the right femoral artery.3 under local anesthesia , , usa ) measuring 135 mm was then deployed over the stiff wire in the subclavian artery under fluoroscopic guidance , with particular attention to not cover the orifice of the internal mammary or vertebral artery . the sheath and the wire were removed , and manual pressure was placed over the axillary artery . after the procedure , no hemoptysis was found and the patient was discharged on the seventh day after the procedure . three months later , follow - up ct showed luminal patency of the left proximal subclavian artery and considerable reduction of the hematoma ( fig . this is the first case report of the successful treatment of a ruptured saa with a covered stent in korea . because a large stent can not be easily introduced into the subclavian artery via a femoral access , we used bilateral arterial access with cutdown of the left axillary artery and right femoral artery . saas are rare , as seen in a report by dent et al.1 in which saas represented only 0.1% of an extensive series of atherosclerotic aneurysms . the common causes of saas were atherosclerosis , thoracic outlet syndrome , infection , trauma , and congenital arterial anomalies such as marfan syndrome.1,2 in particular , proximal aneurysms were caused by atherosclerosis ( 19% ) , collagen disorder ( 18% ) , trauma ( 15% ) , infection ( 13% ) , and in - hospital procedures ( 12%).2 our reported case did not have a history of trauma , infection , or vasculitis . instead , the scattered vascular calcification of the aneurysm suggested that the cause might be atherosclerosis . an atherosclerotic saa in an intrathoracic location usually occurs in patients over 60 years of age of either sex , but appears to be more common in men . although saas can be asymptomatic , they can also cause life - threatening complications such as rupture , thrombosis , or embolism and can result in symptoms caused by local compression.2 in a meta - analysis of 394 native saas , rupture occurred in 9% of patients , with an associated mortality of 19%.4 rupture is a rare complication with a high mortality and can produce intra- or extrathoracic bleeding , including hemoptysis from rupture and erosion into the apex of the lungs . most patients have an episode of hemoptysis , which might be an early sign of rupture.2 the indication for treatment of an saa is usually based on prevention of complications , such as thrombosis , embolization , and rupture . the development of these complications may depend on variable aneurysm - related characteristics including etiology and localization . since halsted first successfully combined ligation with resection of an saa in 1892,5 surgical repair was recommended for all patients with an saa to prevent potential complications.3 endovascular stent graft treatment for arterial injuries began appearing in the literature over a decade ago.6,7,8,9 the evolution of endovascular technology has improved the stents available to vascular surgeons and interventionists in the management of saas . the subclavian artery is mobile and is exposed to rotational forces during abduction and anteflexion of the arm . therefore , a flexible stent graft might be a better option in the saa in particular patients.10 in our case , we successfully applied a self - expandable viabahn covered stent . the natural history of saas is unknown and there are no guidelines concerning the timing of intervention or treatment modalities . in our case , the severe complication of aneurysmal rupture occurred after 2 weeks despite the small size of the aneurysm and optimal medical therapy . regardless of the size of the aneurysm , high mortality from rupture has been reported . thus , early intervention including open surgery or endovascular stenting might be needed to prevent potential complications if patients have symptoms . | an aneurysm of the subclavian artery is rare .
recently , we experienced a case of a ruptured subclavian artery aneurysm presenting as hemoptysis .
the patient had experienced atypical chest discomfort , and computed tomography ( ct ) revealed a small aneurysm of the left subclavian artery ( sca ) .
hemoptysis occurred 2 weeks later .
follow - up ct showed a ruptured aneurysm at the proximal left sca .
endovascular treatment with a graft stent was performed by bilateral arterial access with a 12-fr introducer sheath placed via cutdown of the left axillary artery and an 8-fr sheath in the right femoral artery .
a self - expandable viabahn covered stent measuring 135 mm was introduced retrogradely via the left axillary sheath and was positioned under contrast guidance with an 8-fr jr4 guide through the femoral sheath .
after the procedure , hemoptysis was not found , and the 3-month follow - up ct showed luminal patency of the left proximal sca and considerable reduction of the hematoma . |
although hepatic metastases from breast cancer is not an uncommon presentation with reported incidence at 4050% hepatic failure as a presentation of metastatic breast cancer is very rare . has described an incidence at 0.44% for hepatic metastases for malignancies with very few cases reported in the literature . hepatic failure following hepatic metastases though rare has been reported more for non - hodgkins lymphoma unlike metastatic breast cancer . an aetiological association between fertility drugs and breast cancer has not been reported to exist . however increase in oestrogen levels is associated with use of clomiphene , a selective estrogen receptor modulator . we report the case of a 41 year old para 1nigerian woman , who had been managed for secondary infertility with clomiphene , that presented with a t2 tumour of 3 months duration which emerged as hepatic metastases with liver failure . the rapidity of progression of this disease with subsequent demise within a week of presentation is remarkable . we believe that the prior use of clomiphene may have escalated the progression of this t2 tumour . though it has been reported widely that clomiphene raises levels of oestrogen to supraphysiological levels we are not aware of a report associating the prior use of this selective estrogen receptor modulator with the rapid progression of a previously existing breast cancer . again previous reports of liver failure from hepatic metastases have occurred in subjects who have been managed for breast cancer in the past . in addition , our case deserves mention being a fresh diagnosis following a 3 month history of a t2 breast tumour . a 41 year old para 1 lady was referred from a fertility clinic with a 3 months history of a left breast lump and nipple bleeding . she had been managed for secondary infertility with an extended use of clomiphene having visited other fertility clinics . breast examination showed bloody nipple discharge and a hard left breast lump measuring 4 by 3 cm with no attachment to skin or underlying tissue . breast biopsy revealed an invasive ductal carcinoma , positive progesterone receptor and doubly overexpressed human epidermal growth factor receptors . she was staged t2n1m1 . in the course of investigation , she developed fever , increasing scleral icterus , malaise , drowsiness and right hypochondrial pain . she continued to deteriorate with deepening scleral icterus , drowsiness and confusion dying within 9 days following presentation . our case was being managed for infertility at other fertility clinics before presenting with a 3 months history of a breast lump / nipple bleeding and diagnosed with a t2 tumour with diffuse liver metastases . this is considered a short duration for such metastases and a rational explanation would be the presence of a growth promoter , a history of clomiphene use which would result in exponential increases in oestrogen levels , the breast cancer being a hormone positive tumour . acute liver failure with liver metastases is rare being seen with diffuse parenchymal infiltration as in our case . very few cases have been reported in the literature with the prognosis more or less dismal . the fertility treatments before presentation may explain the aggression of this disease within the short duration of three months . although several reports have not determined an aetiological association between fertility drugs and breast cancer , other reports do not deny a growth promotion in a preexisting breast cancer being hormone dependent especially in our case which was hormone positive . again the tumour in our case was overexpressing for the her 2neu protein another factor in its aggression and rapidity of spread . our case tested negative for hepatitis b , a common case of hepatic failure in our environment . this further reiterates the need for breast cancer screening before women are placed on fertility drugs . a much needed call in this era of an upsurge in fertility treatments . patients who had been adequately treated for breast cancer in the past have been the subject of most reports with invasive ductal carcinoma being the histological type as in our case . however our case was exceptional being a diagnosis at first presentation thus had not received any treatment nor had a history of liver disease . prodromal symptoms of fever , malaise , right upper quadrant pain have been reported to precede the onset of liver failure as occurred in our case prior to commencing any treatment . . even though our case had chemotherapy to attempt to curtail the metastases , the patient s presentation with signs of deteriorating liver function ; elevating liver enzymes , increasing bilirubin levels , right hypochondrial pain , drowsiness predated the administration of chemotherapy . although we were unable to obtain an abdominal ct to determine the extent of liver involvement of the liver metastases , the abdominal ultrasound scan findings showed extensive hepatic involvement . nieto et al . reported the peculiar case of fatal acute liver failure from a previously 4 year old indolent breast neoplasm in a patient with a previously known liver disease . the patients liver metastases was reported to be widely infiltrating as in our case with diffuse metastatic liver nodules . the occurrence of diffusely infiltrating liver nodules has been reported to be most commonly associated with acute liver failure . anorexia , jaundice , and different levels of altered mental status have all been described as features in these cases of diffuse liver parenchymal involvement from an aggressive breast cancer as occurred in our case . martelli et al . have reported a metastatic breast cancer with rapidly progressive liver failure but radiographically occult liver lesions ; massive hepatic infiltration being demonstrated at autopsy . biochemical elevations in the levels of aminotransferases , lactate dehydrogenase and alkaline phosphatases were prominent features in our case as has been reported . however coagulopathy , disseminated intravascular coagulopathy and thrombocytopaenia were not observed in our case as has been described by other reports . a rapid and aggressive course of the disease is described for this disease with a very poor prognosis . the reported cases were described as uniformly succumbing to the condition in a short time . some workers have suggested that up to 8090% involvement of hepatic parenchyma by the tumour with hepatic failure likely provides explanation for the early demise of these reported cases , as in our case . we believe that the elevation of oestrogen levels from fertility drugs over a period of time may have promoted the growth of this tumour and its consequent liver metastases with widespread liver nodules within an observed 3 months clinical history . with the increase in utilisation of fertility drugs , there comes a need for pretreatment screening and increased surveillance for breast cancer in patients managed with fertility drugs . breast cancer screening for patients before infertility treatments and continual surveillance while on such treatment is highly recommended . the poor prognosis of metastatic breast cancer with acute hepatic failure with no effective treatments in published literature marks it as requiring more studies in a bid to upturn its dismal outlook . | highlightsmetastatic breast cancer presenting with liver failure is rare.this case had fertility treatments with clomiphene for an undisclosed duration before presenting with a t2 tumour with diffuse hepatic metastases.the extent of metastases was not consistent with the duration of the t2 tumour of 3months.the rapidity of progression and demise within a week of presentation is remarkable.this would be a cautionary report emphasizing the need for increased screening and surveillance during fertility treatments . |
trichoadenoma is a rare benign tumor , with multiple cystic structures closely resembling the infundibular structures of the hair follicle . it presents as a non - specific nodule over the face or buttocks , however , unusual sites such as the neck , upper arm , thigh shoulder , and shaft of the penis may also be affected . ours is a case of a 60-year - old female with a trichoadenoma over the left labia majora , which presented as a slow growing nodular plaque . a 60-year - old married female patient presented to us with asymptomatic gradually increasing skin lesion on vulva since 2 years . examination revealed a firm , nontender , nodular plaque of 3.5 cm 2.5 cm along with a few shiny papules along the right labia majora [ figure 1a ] . the left labia majora , labia mijora , clitoris , and perianal areas were not involved . differential diagnoses considered were fibrosis lymphangioma circumscriptum , giant trichoepithelioma , sarcoidosis , lupus vulgaris , and deep fungal infection . ( c ) post - treatment - 2 months , healing with post - inflammatory hyperpigmentation histopathology from the lesion showed numerous horn cysts present throughout the dermis surrounded by eosinophilic cells [ figure 2a ] . few of the cyst walls were lined by eosinophilic epidermal cells and contained keratin [ figure 2b ] ; thereby leading to a diagnosis of a less known adnexal tumor trichoadenoma . ( a ) 10 view : showing numerous horn cysts present throughout the dermis . ( b ) 40 view : showing cyst walls lined by eosinophilic epidermal cells , containing keratin trichoadenoma of nikolowski is a rare , follicular tumor considered as a neoplastic process by some authors and benign malformation by some others . clinically it presents as a solitary slow growing grayish nodule measuring up to 1.5 cm in diameter , seen over the face ( 57.5% ) and buttocks ( 24.2% ) . other uncommon sites of involvement are the neck , upper arm , thigh , shoulder and shaft of penis . it may also present as a chronic discharging nodule or as an ulcerated growth . rare case reports of trichoadenoma in association with intradermal melanocytic nevus , sebaceous carcinoma , basal cell carcinoma , syringocystadenoma papilliferum have also been recorded . verrucous variants of trichoadenoma have also been reported . though it is a tumor of adulthood , infants as old as 20 months numerous horn cysts are present surrounded by eosinophilic cells . in some instances , a single layer of flattened granular cells is interpolated between the horn cysts and surrounding eosinophilic cells . moreover , the histological similarity of trichoadenoma with trichoepithelioma suggests the development of immature hair structures . however , because the cyst wall consists of epidermoid cells and keratinization may take place with the formation of keratohyalin , it has been suggested that the tumor differentiates largely toward the infundibular portion of the pilosebaceous unit . the keratin profile expression of this tumor supports the theory that it differentiates towards the follicular infundibulum and the follicular bulge region . , we used co2 laser at 9.0 watts with 100 mm hand piece ( 1.0 mm spot size ) in superpulse mode to ablate the growth under local anesthesia [ figure 1b ] . there has been no recurrence so far and the normal vulvar anatomy has been maintained . the trichoadenoma is a mystifying follicular tumor . though it is known to occur on the face and buttocks , newer cases with unusual manifestations are being reported . to the best of our knowledge , a histopathologically proven large trichoadenoma over the vulva has not been reported . | trichoadenoma of nikolowski is a rare , benign , well differentiated , slowly growing tumor of the hair follicle which was first described in 1958 by nikolowski .
it usually occurs as a solitary nodular lesion between 3 and 15 mm in diameter .
it commonly occurs on the face or the buttocks .
herein we report a case of a slowly growing nodular plaque over the vulva of a 60-year - old female , histopathologically proven to be a trichoadenoma .
the lesion was completely removed by ablative carbon dioxide laser ( co2 ) . |
initially described after treatment with flutamide , responses to withdrawal of hormonal therapies in prostate cancer patients have been documented after cessation of other antiandrogens such as nilutamide , bicalutamide , or cyproterone acetate and of megestrol acetate , diethylstilbestrol or estramustine . in the southwest oncology group trial 9426 , 21% of 210 patients had confirmed psa decreases of 50% after treatment with nonsteroidal antiandrogens with a median progression - free survival of 3 months . it has been proposed that withdrawal responses could result from mutations in the androgen receptor ( ar ) . transfection experiments revealed that ar point mutations in the hormone - binding domain allow activation by ligands other than dihydrotestosterone . mutated ar from clinical samples was shown to be activated by progesterone , estradiol , adrenal androgens , hydrocortisone or hydroxyflutamide . however , a prospective study failed to demonstrate a clear association between detectability of ar mutations and antiandrogen withdrawal responses . an improvement in overall survival has been reported with abiraterone acetate ( aa ) , a selective inhibitor of cytochrome p450c17 ( cyp17 ) , in castrate - resistant prostate cancer patients who have failed docetaxel chemotherapy . the addition of low - dose corticoids to aa is mandatory in daily practice since the combination has been shown to minimize the syndrome of secondary mineralocorticoid excess related to cyp17 inhibition . here , we report the case of a patient who had a confirmed psa decrease of 50% after stopping aa , mimicking a withdrawal syndrome . a 60-year - old patient was diagnosed with a prostate adenocarcinoma ( gleason score 8 , 4 + 4 ) with synchronous bone metastases in april 2007 . he successively received an lhrh agonist , a combined androgen blockade with lhrh agonist and bicalutamide , without subsequent response to bicalutamide withdrawal , docetaxel ( 10 cycles ) , then mitoxantrone ( 3 cycles ) and diethylstilbestrol . in april 2011 , aa was started at the standard dose of 1,000 mg daily in combination with prednisone ( 5 mg twice daily ) . psa slightly decreased to a nadir of 68 ng / ml one month after the start of treatment . in parallel , a metabolic partial response according to consensus criteria on 18-fluorodeoxyglucose positron emission tomography ( pet ) and choline pet ( suvmax = 37% ) was observed . however , aa and prednisone were stopped in august 2011 because of back pain related to vertebral metastases and concomitant psa progression ( psa 128 ng / ml ) . one month later , a confirmed psa decrease to a nadir of 62 ng / ml occurred , defining a partial biological response according to standard criteria , with a concomitant improvement in back pain . to the best of our knowledge , this is the first case ever reported of a withdrawal response with aa . currently proposed mechanisms of resistance to aa include ligandless activation of ar by constitutively active variants lacking the ligand - binding domain , cross - talk with relative signaling pathways and/or activation of amplified or promiscuous ar by nonandrogenic ligands such as corticosterone or other steroids . following the latter hypothesis , the withdrawal response observed in the present case could be related rather to prednisone than to aa itself as it was stopped at the same time . | in 2011 abiraterone acetate ( aa ) was approved for the treatment of castrate - resistant metastatic prostate cancer patients who have failed docetaxel chemotherapy .
we report the case of a patient who experienced a confirmed psa decrease of 50% after stopping aa , mimicking an antiandrogen withdrawal syndrome . |
though occasionally reported , cardiac involvement is a rare phenotypic feature of facio - scapulo - humeral muscular dystrophy ( fsh - md).1 ) cardiac involvement in fsh - md includes cardiomyopathy and arrhythmias.1 ) though various arrhythmias have been found in patients with fsh - md , pre - excitation - syndrome has not been reported . the patient is a 39-year - old veterinarian who developed slowly progressive muscle weakness and wasting of the shoulder girdle muscles since age 3 years . since age 29 years he additionally developed diffuse weakness and wasting of the left leg accompanied by muscle stiffness . since then , he also noted generalized mus - cle tenderness , muscle aching after exercise , and contractures . needle - elec - tromyography was myogenic and deoxyribonucleic acid analysis revealed a reduction of the fsh - md tandem repeat size in the d4z4 locus on chromosome 4q35 ( fig . follow - up investigation in july 2012 revealed a facies myopathica with weakness of the upper lids , inability to voluntarily balloon the cheeks , and weak anteflexion of the head ( m5- ) . there was prominent scapular winging , bilateral diffuse weakness of the upper limbs with right - sided predominance , hypotonia , diffuse wasting , and re - duced deep tendon reflexes . on the lower limbs , there was bilateral weakness of hip flexion ( m4 ) and foot extension ( m4- ) . there was asymmetric wasting with left - sided predomin - ance , bilateral hypotonia , lumbar hyperlordosis , and a waddling gait . nevertheless , he was able to play the guitar and drive an automatic car , even over long distances . cardiologic investigation at follow - up revealed mild myocardial thickening , two intra - ventricular aberrant bands , and , surprisingly , intermittent pre - excitation on routine electrocardiography ( fig . 2 ) . stress testing to see if there was predominance of prolonged qrs - complexes and thus indication for ablation or predominance of normally - sized qrs - complexes , clearly demonstrated a reduction of prolonged qrs - complexes . since he was asymptomatic and pre - excitation - syndrome occurred only intermittently , he was not recommended to undergo ablation . the presented case is interesting for the association of pre - excit - ation syndrome and fsh - md . rhythm abnormalities so far reported in fsh - md include impulse generation abnormalities , such as sinus node dysfunction.2)3 ) supraventricular arrhythmia,3)4 ) and bradycardia , 3)4)6)7)8 ) or impulse propagation abnormalities , such as short pr - interval,4 ) tall p - waves,2)3 ) abnormal atrio - ventricular conduction with complete atrio - ventricular block,3)4 ) abnormal q - waves,8 ) intra - ventricular conduction delay,3)4)8 ) incomplete bundle branch block,2 ) ventricular tachycardia,3 ) abnormal st - segment,3 ) high t - waves,3)4 ) prolonged qt - interval,6 ) increased r / s - ratio in v1,7 ) or hypertrophy.2 ) in a study of 83 patients with fsh - md , 12% had cardiac arrhythmias.4 ) in a study of 24 patients with fsh - md , 9 had positive ventricular late potentials and increased qt - dispersion compared to controls.9 ) in the two patients described by lafort et al.4 ) a shortened pr - interval was not associated with a delta - wave . discrete concentric thickening of the myocardium was also interpreted as cardiac involvement in the presented case , since the history was negative for arterial hypertension or a thoracic deformity . single patients with fsh - md also develop cardiomyopathy.10 ) accordingly , echocardiography in fsh - md may show enlarged right cardiac cavities or restricted right ventricular movement , attributed to thoracic deformities2)8 ) or hypertrophic cardiomyopathy.4)10 ) additionally , myocardial scintigraphy revealed reduced thallium-201 uptake due to suspected myocardial fibrosis.7 ) this case shows that there is indeed cardiac involvement in fsh - md , manifesting either as hypertrophic cardiomyopathy or arrhy - thmias . among various different arrhythmias described in fsh - md , single patients may also develop pre - excitation - syndrome . | pre - excitation - syndrome has not been reported as a phenotypic feature of facio - scapulo - humeral muscular dystrophy ( fsh - md ) . in a 39-year - old male with fsh - md due to a reduced tandem repeat size in the d4z4-locus on chromosome 4q35 , cardiac involvement , manifesting as an incomplete right bundle - branch - block , tall t - waves in v 3 - 5 , st - elevation in v 2 - 4 , and mild thickening of the left ventricular myocardium ,
was first recognised 10 years earlier .
follow - up at age 39 years revealed mild myocardial thickening , two intra - ventricular aberrant bands , and , surprisingly , intermittent pre - excitation on a routine electrocardiography .
cardiac involvement in fsh - md may manifest as hypertrophic cardiomyopathy or various arrhythmias , of which one may be pre - excitation - syndrome . |
a 54 year - old man was referred to the outpatient clinic having been injured in his right eye by the hook of a clothes hanger while falling . swelling and bruising on the right eyelid he complained of image tilting in the right eye compared to the image inthe left eye . non - dilatating fundusphotos ( vx-10 ; kowa optimed , tokyo , japan ) showed a 12-degree excyclotorsion in the right eye and an 8-degree excyclotorsion in the left eye . image j ( national institutes of health , bethesda , md , usa ) was used to measure the angle between the line that crosses the center of the optic disc and parallels the fovea and the line that extends from the optic disc center to the fovea . non - enhanced orbit computed tomography was performed to rule out eyeball rupture , intraocular foreign body , or orbit wall fracture as well as any other abnormalities . during surgery , a 15-mm horizontal conjunctival laceration over the superior rectus muscle was found . the tendon of the superior oblique muscle protruded inferio - nasally through the lacerated conjunctiva ( fig . the cut margin of the ruptured tendon was irregular and the width was about 4 mm . it was assumed that the location of the rupture was in the middle of the tendon under the superior rectus muscle . the superior rectus muscle seemed to be intact . to correct torsional diplopia , we attached the anterior and posterior borders of the superior oblique tendon 5 and 9 mm apart from the nasal margin of the superior rectus muscle insertion ( fig . one week postoperatively , the patient showed vertical diplopia and 4-prism dioptor right hypertropia by the alternative prism and cover test in the primary position . however , the patient did not complain of torsional diplopia . in the fundus photographs , three months postoperatively , right hypertropia gradually increased to 20-prism diopters and the patient complained of vertical diplopia . it loops through the trochlea , and passes under the superior rectus muscle and inserts into the sclera on the posterotemporal surface of the eyeball . traumatic injury to the superior oblique muscle is extremely rare , and mostly arises from injuries caused by hook - shaped objects , and is frequently associated with penetrating eyelid injury . when the ruptured muscle belly from the origin could not be found and was left unconnected , the patient showed a pattern of superior oblique muscle palsy . however , harish et al . reported that excision of the loose hanging tendon near the medial border of the superior rectus muscle did not result in progressive muscle sequelae until six months later . that might be possible because some of the posterior fibers of the superior oblique tendon were still intact . in our case , the patient initially complained of torsional diplopia , which coincided with the excyclotorsion of the right eye on the fundus photograph . to correct this , we planned to reattach the ruptured tendon to the middle of the original track where the superior oblique muscle might have passed . dow found that anchoring the avulsed superior oblique muscle to the medial orbital wall adjacent to the trochlea resulted in slight hypertropia with full ocular motility . however , when the ruptured tendons were reapproximated with each other , postoperatively , asymptomatic brown 's syndrome could develop . because the ruptured superior oblique muscle shortened in our case , we recessed the muscle 5 mm from the medial border of the superior rectus muscle . according to parks and helveston , the distance between the nasal edge of the superior rectus and anterior border of the superior oblique muscle in the primary position was 3 to 5 mm . torsional diplopia , the main complaint of the patient , improved and the degree of excyclotorsion of the right eye decreased in the fundus photographs . however , three months after the operation , hypertropia increased to 20 prism dioptors . this could have been due to the torn tendon that was not attached far enough laterally to avoid a weakening effect . additionally , during the exploratory procedure to find the distal remnant of the superior oblique tendon , the superior rectus muscle could have been injured , although initially the superior rectus muscle seemed to be intact . upon healing , it might have undergone localized fibrosis that caused contraction and secondary hypertropia . to correct the hypertropia an inferior rectus resection was chosen for adhesion around the superior rectus muscle and to avoid contralateral inferior rectus recession . two months after the second operation , the patient was comfortable and muscle function was stable . when the superior oblique muscle is ruptured , intraoperative reapproximation of the muscle can be performed . however , because the function of the muscle is hard to restore perfectly , postoperative complications should be anticipated and careful follow - up is needed to determine further treatment . | traumatic rupture of the superior oblique muscle is rare .
we report a case of a 54-year - old man injured by the metal hook of a hanger , resulting in a rupture of the superior oblique muscle tendon .
he complained of torsional diplopia when in the primary position .
the distal margin of the superior oblique muscle was reattached to sclera 5 and 9 mm apart from the medial insertion of the superior rectus muscle .
one week after the operation , torsional diplopia disappeared .
however , a 4-prism diopter ipsilateral hypertropia was observed .
three months later , hypertropia gradually increased to 20 prism dioptors and the second operation was done to correct vertical diplopia . |
most of the dual infections cause a diagnostic dilemma and require a high degree of suspicion in susceptible individuals . however , dual infections with mucormycosis may follow a fulminant course and may be associated with higher mortality and morbidity . we hereby report coinfection of pulmonary tuberculosis ( tb ) with mucormycosis in a diabetic male that worsened rapidly in spite of the antimicrobial therapy targeted toward the same . a 72-year - old nonsmoker male , known case of diabetes mellitus type ii since 10 years , on oral hypoglycemic agents , presented with the complaints of altered sensorium with low - grade fever associated with pain abdomen and vomiting and was diagnosed to have severe urinary tract infection . he was being managed in the ward for diabetic ketoacidosis for the same and was doing well until the 9 day of admission when he developed cough , expectoration , and difficulty in breathing . subsequently , the patient was intubated and transferred to intensive care unit ( icu ) for mechanical ventilation with synchronized intermittent mandatory ventilation mode and further managed according to the institutional protocol . all routine biochemical and hematological investigations including the chest x - ray ( cxr ) were sent . blood sugar levels were found to be 460 mg% and total lymphocyte count was 1200 mm , and urine ketones were found to be positive . arterial blood gas on the day of admission at fio2 of 0.6 had a pao2/fio2(pf ) ratio of 261 . empirical antibiotic therapy and vasopressor support with noradrenaline ( 0.5 g / kg / min ) was started as per protocol . sputum examination undertaken in the ward itself was negative for acid fast - bacilli ( afb ) . the cxr showed bilateral cavitary lesions in all the zones on the very 1 day of icu admission . with a high index of suspicion for fungal pneumonia based on the clinical and radiological findings and negative sputum for afb , antifungal ( caspofungin 70 mg intravenous [ iv ] loading dose followed by 50 mg iv once daily ) however , no clinical improvement was noticed over 5 days , and radiological deterioration was evident by the appearance of new cavitary lesions developing over every 24 h [ figure 1 ] . the pf ratio also started deteriorating consistently with the values of 161 on the 3 day falling to 114.5 on the 9 day with fio2 going up to 0.9 . with patient condition deteriorating as evidenced by higher ionotropic support and decreasing pf ratio , bronchoalveolar lavage ( bal ) was performed , and wastings were sent for afb staining , pyogenic culture , and fungal culture . subsequently , antitubercular treatment was also initiated as per the revised national tuberculosis control program guidelines for the same . although the potassium hydroxide wet mount revealed no fungal elements , fungal culture showed growth of rhizopus species on the 2 day of inoculation [ figure 2 ] , following which amphotericin b ( in the dose of 0.5 mg / kg iv loading dose followed by 1 mg / kg once daily ) was introduced as per culture sensitivity . on the 11 day , the pf ratio had further fallen to 71 on fio2 of 1.0 . however , the patient failed to improve and succumbed on the 12 day of admission to icu . multiple cavitatory lesions on day 5 lactophenol cotton blue mount showing rhizopus microsporus species ( lcb 200 ) opportunistic fungal organisms such as candida species , aspergillus species , mucor species , and cryptococcus neoformans may be encountered in diabetic patients who are usually immunocompromised by virtue of the disease itself . diabetes mellitus may predispose to mucormycosis in 3688% of patients which is a serious , potentially fatal fungal infection that needs a high degree of suspicion for the diagnosis . patients with uncontrolled hyperglycemia , particularly those with ketoacidosis , are the most susceptible and it may be the first manifestation in patients with undiagnosed diabetes mellitus . the most common presentation is rhino - orbital - cerebral involvement followed by pulmonary infection . patients with diabetes are prone to develop tb and have high chances of treatment failure . a large proportion of people with diabetes further developing tb are not diagnosed , or diagnosed too late , may be due to lack of awareness on the part of the patient to report early for any development of chest symptoms . coinfection with pulmonary tb and mucormycosis in immunocompromised patients may present a diagnostic dilemma owing to similar clinical presentation and need a high degree of clinical suspicion and early aggressive treatment . both clinical entities in addition , the radiological features of consolidation , cavitation , infiltrates , and effusion are common to both the diseases and , therefore , histopathological examination or culture sensitivity should be undertaken at the earliest to make definitive diagnosis . it is possible that the high relapse cases , treatment failures , resistance , and high mortality associated with tb infection are partly attributed to coinfection with opportunistic fungal pathogens and drug - resistant non - tb bacteria . in the present case , an immunocompromised state prevailing as a result of diabetes and tb made the patient prone to acquire coinfection with mucormycosis . although cavitation was evidenced on the very 1 day of admission to icu , negative sputum examination in the ward made tb an unlikely diagnosis . however , conclusive diagnosis was made only after bal which demonstrated the presence of afb on smear and ubiquitous filamentous fungus rhizopus species , of mucorale order , colonization on culture . there are isolated reports of coinfection of mucormycosis and tb in immunocompromised patient where the patients were successfully treated . in the present case , the unfavorable outcome may be the result of delayed diagnosis which was probably missed for the first 8 days in a ward where primarily the focus was on treating diabetic ketoacidosis . meanwhile , the hyperglycemic environment that favors immune dysfunction ( e.g. , damage to the neutrophil function , depression of the antioxidant system , and humoral immunity ) flared up coinfection of mucormycosis with latent tb progressing to active tb and followed a fulminant course . latent tb and drug - resistant tb in patient with diabetes make them susceptible to opportunistic infections with potentially fatal fungal infections . simultaneous infection with mycobacterium tuberculosis and mucormycosis is a rare finding and requires high clinical vigilance . now - a days , it is imperative to consider fungal infections as an important differential diagnosis . early and aggressive treatment targeting the isolated organisms may help reduce the mortality and morbidity . meanwhile , screening the patients with diabetes for coinfections , especially in nonresolving pneumonias are imperative and should be practiced in a protocolized form in early stages of diabetes . | herein , we present the case report of an adult male diabetic patient who had coinfection with mycobacterium tuberculosis and mucormycosis , which otherwise is a rare clinical entity .
diabetes mellitus may predispose a patient to tuberculosis ( tb ) infection which further weakens immune system thus making him susceptible to other fungal or bacterial infections which may pose various treatment difficulties . therefore , there is a need for mycological and bacteriological investigations in patients with pulmonary tb to rule out secondary coinfections thus contributing to better management . |
a 45-year - old man presented with a history of blurred vision in his right eye since past 15 days . fundus examination showed multiple cotton wool spots inferior to the papillomacular bundle , extending toward macula with retinal hemorrhages temporal to macula [ fig . 1 ] . color fundus photo showing numerous soft exudates in inferotemporal part extending toward macula in some part , multiple vein constrictions , and occlusions red free and fluorescein angiography [ figs . 2 and 3 ] showed areas of blocked fluorescence due to hemorrhages and multiple brvos . fuorescein angiography showing multiple branch retinal vein occlusions asterix and arrows showing partial vein obstructions optical coherence tomography [ figs . patient underwent complete systemic workup including hemogram with erythrocyte sedimentation rate ( esr ) , lipid profile , renal function tests , ana , c - anca , p - anca , homocysteine levels , coagulation profile ( prothrombin time , aptt , bleeding time , clotting time , protein c and s levels ) , syphilis serology , echocardiography , and electrocardiogram . except a raised level of homocysteine ( 35 m ) ( normal range : 5 - 15 m ) , all other levels were within normal limits . oct showing normal anatomy of macula he was started on anticoagulants and homocysteine lowering agents under guidance of an internist . as macular perfusion was not affected and there was no associated cystoid macular edema , no active ocular intervention was done . he was advised close follow - up for early detection of any further vision threatening vein obstruction . retinal vein occlusion ( rvo ) is the second most common sight - threatening retinal vascular disorder after diabetic retinopathy . patients with rvo have a higher prevalence of stroke and a greater risk of cardiovascular disease than similarly aged individuals without rvo . brvo is divided into two distinct entities : major brvo , when one of the major branch retinal veins is occluded , and macular brvo , when one of the macular venules is occluded . in 66% of eyes with brvo , there is occlusion of the major branch in the superotemporal quadrant , followed by 22 - 43% of eyes with occlusion of the major branch in the inferotemporal quadrant . major brvo can be asymptomatic or with visual blurring , usually involving the sector of visual field corresponding to the area of the retina involved . in macular brvo , there is always a central visual disturbance with normal peripheral vision . among all conditions leading to a hypercoagulable state , hyperhomocysteinemia was the commonest cause found in 21.9% patients . homocysteine is an amino acid derived from methionine , which can be converted into cysteine . the metabolic pathways involving homocysteine require vitamin b12 , vitamin b6 , and folate for proper functioning . various reports on hyperhomocysteinemia depict that arterial as well as venous vessels are involved in the disease . the rare syndrome of homocystinuria was first described in 1962 among subjects with mental retardation , ocular , and skeletal abnormalities , as well as a high risk of thromboembolism . increased plasma homocysteine is associated with both rvo and retinal arterial occlusion , the elevation being greater in the arterial occlusion group . in young patients , rvo is less associated with systemic diseases and is more strongly associated with plasma homocysteine than in older patients . atherosclerotic plaques occur in young people with homocystinuria who suffer from premature , aggressive occlusive disease . plasma concentrations of homocysteine are an independent risk factor for cardiovascular and cerebrovascular disease in the general population . mild to moderate elevation of plasma homocysteine is reported as a risk factor for occlusive disease . specifically , many studies demonstrate hyperhomocysteinemia as an independent risk factor for atherosclerosis in the coronary , cerebral , and peripheral vasculature . the mechanisms by which homocysteinemia damages the blood vessel wall by supporting prothrombotic effects seems to be multifactorial . the various mechanisms reported include homocysteine - induced oxidative stress ( redox stress ) , decreased bioavailability of nitric oxide , altered expression of various thrombotic factors , mutagenic effect on arterial smooth muscle cells , and expression of acute stress - related genes . patients presenting with such kind of clinical picture should be investigated thoroughly to rule out any modifiable risk factor such as hyperhomocysteinemia to prevent any further vaso - occlusive event . | hyperhomocysteinemia is a well - known modifiable risk factor for thromboembolism .
retinal vascular occlusion in patients having hyperhomocysteinemia is a known entity , particularly in young patients .
however , multiple extra macular branch retinal vein occlusion ( brvo ) is a rare condition , which can be a presentation of this disease .
we present a patient who had multiple extra macular brvo ; on complete systemic workup , he was found to have raised homocysteine levels . |
they are formed as earlier indicated as a result of aggregation of three or more molecules of surfactant existing in a particular liquid medium in thermodynamically stable equilibrium that create highly anisotropic interfacial region lining the boundary formed by polar aqueous and nonpolar hydrocarbon regions , impacting new chemical and physical properties to the system [ 27 ] . determination of reaction rates in micellar is usually based on the pseudophase mode , which treats aqueous , organic , and/or surfactant components of the solvent medium as constituting distinct phases in which reaction occurs and between which reagent and product are distributed in accordance with conventional laws of kinetic and mass transfer . it has the greatest application in the field of alcohol and carbohydrate chemistry . under controlled conditions , periodate will selectively oxidize 1,2-diol , 1,2-amino alcohols , 1,2-hydroxyl aldehyde , and ketones and various other groupings . periodate oxidation has a lot of advantages which are responsible for its being widely studied . for example , it can be applied in aqueous solution over a wide range of ph . ethylene glycol is an organic compound primarily used as a raw material in the manufacture of polyester fibers and fabric industry . a small percent is used in industrial application like antifreeze formulation and other industrial products . the kinetic oxidation of ethylene glycol by various oxidizing agents has been investigated and was found to involve two electron transfers through the formation of a negatively charged cyclic intermediate . kinetics and mechanism of the reaction between ethylene glycol and periodate in micellar system remain unexplored . in this work , i have explored the effect of cationic ( cetyltrimethylammonium bromide , ctabr ) and nonionic ( dodecyl amine , da ) surfactant micelles on the kinetics and mechanism of the reaction between ethylene glycol and periodate . cetyltrimethylammonium bromide ( ctabr ) from fluka , dodecyl amine ( da ) from sigma , and sodium periodate from bdh ( 99% pure ) were used without further purification . reaction kinetics were studied on the perkin - elmer uv / vis spectrophotometer , lambola e 2150 , using a cell of path length 1 cm by recording the change in absorbance due to disappearance of periodate ( at 225.4 mn ) in a thermostated reaction cell . the concentration of ethylene glycol was kept in large excess over the concentration of periodate . the integrated first order equation is as follows :
( 1)lnata=lna0ak0 t ,
where a0 , at , and a are the absorbance time zero , t , and infinity was fitted to the kinetic data by using algorithm to give the first order pseudoconstant k0 ( k0 = observed rate constant ) . the value of the observed rate constant was reproducible within the experimental error ( 3% ) . the conductivity measurement was performed with jenway 4510 digital conductometer using a dip - type cell of constant 0.88 cm . all measurements were done in a jacketed vessel , maintained at desired temperature , with circulating water thermostat bath . the conductometric method was used to determine the cmc value of ctabr and da solution at different experimental conditions : ctabr , and da only , ctabr + io4 , ctabr + eg , da + io4 , and da + eg . the cmc value was determined from the specific conductivity versus [ ctabr ] and [ da ] in the presence and absence of io4 and eg . the cmc was found to be 9.1 10 , 1.54 10 , 9.7 10 , 1.01 10 , 2.06 10 , and 1.50 10 mol / dm for water + ctabr , water + da , ctabr + io4 ( 1.051 10 mol / dm ) , ctabr + eg ( 3.580 10 mol / dm ) , da + io4 ( 4.20 10 mol / dm ) , and da + eg ( 2.87 10 mol / dm ) , respectively , at 25c as shown in tables 1(a ) and 1(b ) . the reaction was carried out in the presence of ctabr ( 0.0002.743 ) 10 mol dm and fixed concentration of eg and io4 . addition of ctabr results in partial increase in rate up to the concentration of 1.83 10 mol / dm after which inhibition predominate as shown in figure 1 . the initial catalytic role of ctabr below 1.83 10 mol / dm can be explained on the fact that small aggregate of the ctabr exists below the cmc which interacts physically with the reactants forming active entities . therefore , the catalytic role is due to the presence of premicelle and preponement of micellization by reactant ; the two reactants are assumed to have penetrated the stern layer electrostatically . below 1.83 10 mol / dm of ctabr , inhibition occurs . these could be interpreted using the kinetic model of the pseudophase proposed by menger and portnoy , which , taking the micelles as a pseudophase uniformly distributed in the aqueous phase , put forward a reaction scheme with a micelle - substrate equilibrium governed by an equilibrium constant ks ( scheme 1 ) . this scheme represents the micellized surfactant as dn where [ dn ] = [ d ] cmc and [ d ] is the concentration of surfactant where m and w refer to the micellar and aqueous pseudophases , respectively . the scheme predicts a value for kob given by ( 2 ) which is the overall reaction rate . this value is equal to the rates at the micellar and aqueous pseudophases ( scheme 1 )
( 2)kob = kw+kskm[dn]1+ks[dn ] . the inhibition observed occurs because of the low concentration of io4 near the cationic surface causing the reactivity of the associated substrate to be much less than that of the substrate in the aqueous phase preventing the formation of [ eg . if km = 0 , io4 is completely excluded from the stern layer of the micelle and ( 2 ) becomes
( 3)kob = kw1+ks[dn ] ,
where kh2o represents the observed rate constant in the absence of surfactant . the reaction was also studied in the presence of da and the result ( figure 2 ) can be interpreted in the same way . added surfactant increases the relative concentration of eg and io4 in the stern layer which increases the reaction rate as shown by the ascending branch of the curve . as the concentration of ctabr and da increases , the concentration of the reagent in the micellar pseudophase decreases and furthers the excess of unreactive io4 in the stern layer so that the reaction rate decreases . finally , it can be concluded that , within the experimental range of studies , the pseudophase ion - exchange model has been found to be successful in explaining the result obtained in the kinetics and mechanism of the reaction between ethylene glycol and periodate in micellar system . | the oxidation of ethylene glycol by periodate ( io4 ) was studied in different micellar aggregates of cetyltrimethylammonium bromide ( ctabr ) and dodecylamine ( da ) by means of uv / vis spectroscopy .
the observed constant ko was obtained by monitoring the disappearing of ethylene glycol with time at a suitable wavelength under pseudofirst condition .
addition of ctabr and da inhibits the reaction rate while the kinetic behavior was explained on the association of one of the reactants with the micelles leaving the other reactant in the bulk solution ( pseudophase model ) . |
sjgren 's syndrome is a slowly progressing autoimmune disease characterized by lymphocytic infiltration of the exocrine glands , mainly the lacrimal and salivary glands , resulting in impaired secretory function . simultaneously , systemic features of cutaneous , respiratory , renal , hepatic , neurologic , and vascular nature often occur . the syndrome can present either alone ( as primary sjgren 's syndrome ) or in the context of underlying connective tissue disease ( as secondary sjgren 's syndrome ) . renal involvement is well - recognized extra glandular manifestation of primary sjgren 's syndrome ( pss ) . most common manifestations are related to tubular dysfunction resulting from chronic interstitial nephritis and can manifest as distal renal tubular acidosis ( rta ) , proximal rta , tubular proteinuria , and nephrogenic diabetes insipidus . hypokalemic paralysis rarely occurs as the first manifestation of a renal tubule disorder due to pss . we herein present two cases who presented to us with hypokalemic paralysis , secondary to pss . a 19-year - old unmarried lady presented to our hospital for evaluation of two episodes of generalized weakness . both the episodes were acute in onset , with weakness of all four limbs and neck muscles , and occurred during recovery from febrile illness . meq / l ) on both the occasions and complete recovery occurred in a span of 48 h on receiving intravenous and oral potassium . there was no history of fever , joint pain , skin rash , photosensitivity , or parotid swelling . there is no family history of a similar illness . at presentation to us , she had normal general physical and neurological examination . laboratory parameter of patients a diagnosis of distal rta was made in view of an alkaline urinary ph ( > 5.5 ) in the setting of metabolic acidosis . she was evaluated for etiology of distal rta , which revealed positive rheumatoid factor ( rf ) and antinuclear antibody ( ana ) , while serum c3 level was normal and anti - dsdna was negative . schirmer 's test was positive in both eyes and her serum anti - ro and anti - la antibody levels were unequivocally elevated . she was discharged on oral potassium and alkali supplementation . a 22-year - old married lady presented for evaluation of an episode of acute onset flaccid quadriparesis without any sensory , bladder , or bowel involvement . the episode occurred 15 days before she presented to us . at the time of episode she had been taking oral potassium supplementation regularly since then and did not develop any further episodes of weakness . there was no history of fever , joint pain , skin rash , photosensitivity , or parotid swelling . there is no family history of a similar illness . her general and systemic examination including neurological examination was normal . bicarbonate loading test ruled out an associated proximal tubular dysfunction . on working up for the etiology of distal rta , she was found to have elevated titer of rf and ana , her serum c3 level was normal , and anti - dsdna was negative . a 19-year - old unmarried lady presented to our hospital for evaluation of two episodes of generalized weakness . both the episodes were acute in onset , with weakness of all four limbs and neck muscles , and occurred during recovery from febrile illness . meq / l ) on both the occasions and complete recovery occurred in a span of 48 h on receiving intravenous and oral potassium . there was no history of fever , joint pain , skin rash , photosensitivity , or parotid swelling . there is no family history of a similar illness . at presentation to us , she had normal general physical and neurological examination . laboratory parameter of patients a diagnosis of distal rta was made in view of an alkaline urinary ph ( > 5.5 ) in the setting of metabolic acidosis . she was evaluated for etiology of distal rta , which revealed positive rheumatoid factor ( rf ) and antinuclear antibody ( ana ) , while serum c3 level was normal and anti - dsdna was negative . schirmer 's test was positive in both eyes and her serum anti - ro and anti - la antibody levels were unequivocally elevated . a 22-year - old married lady presented for evaluation of an episode of acute onset flaccid quadriparesis without any sensory , bladder , or bowel involvement . the episode occurred 15 days before she presented to us . at the time of episode she had been taking oral potassium supplementation regularly since then and did not develop any further episodes of weakness . there was no history of fever , joint pain , skin rash , photosensitivity , or parotid swelling . bicarbonate loading test ruled out an associated proximal tubular dysfunction . on working up for the etiology of distal rta , she was found to have elevated titer of rf and ana , her serum c3 level was normal , and anti - dsdna was negative . pss is a disease of exocrine glands presenting with manifestations related to dry eyes and dry mouth . nonexocrine organ systems may also be involved , including skin , lung , gastrointestinal tract , central and peripheral nervous system , muscular skeletal apparatus , and the kidney . the reported rate of renal involvement in pss in literature is variable ranging from 4.2% to 50% . the spectrum of renal disease includes interstitial nephritis , which can manifest as distal rta , proximal rta , tubular proteinuria , nephrogenic diabetes insipidus , glomerular diseases , or renal failure . the most common manifestations are related to tubular dysfunction which results from chronic interstitial nephritis . . the mechanisms of distal rta - induced hypokalemia include decreased distal tubular na delivery , secondary hyperaldosteronism , defective h - k atpase , and bicarbonaturia . although hypokalemia is frequent sequel of rta , a severe symptomatic decrease in serum potassium concentration has been described in a few cases only . subsequently , approximately 25 more cases has been reported.[821 ] here , we present a report of two cases that presented to us with hypokalemic paralysis secondary to distal rta , which on further work up , was found secondary to pss . both of our patients did not have any evidence of proximal tubular dysfunction . in conclusion , the kidney involvement in pss can uncommonly present as hypokalemic paralysis in the absence of significant sicca symptoms or may precede sicca symptoms . sjgren 's syndrome should be instigated in any patient presenting with hypokalemic paralysis from rta , even in the absence of the sicca syndrome . | primary sjgren 's syndrome ( pss ) is a chronic autoimmune disease characterized by a progressive lymphocytic infiltration of the exocrine glands with varying degrees of systemic involvement .
overt or latent renal tubular acidosis ( rta ) , caused by tubulointerstitial nephropathy , is a common extraglandular manifestation of pss .
hypokalemic paralysis is a well known , albeit rare complication of severe distal rta from any cause .
cases of pss manifesting for the first time as hypokalemic paralysis caused by distal rta have been rarely reported .
we herein present our experience of two cases , who presented to us for evaluation of hypokalemic paralysis and on work up found evidence of distal rta , which on further work up found to be secondary to pss .
a high index of suspicion for pss should be kept in all patients with hypokalemic paralysis . |
amyotrophic lateral sclerosis ( als ) is a neurodegenerative disorder affecting motor neurons that is characterized by progressive skeletal muscle atrophy and eventual death , usually due to respiratory failure . various structural , biological and physiological alterations of the skin have been reported in patients with als . the pincer nail deformity is characterized by distortion of the shape of the nail with transverse overcurvature . we herein describe the case of a patient who presented with a pincer nail deformity associated with rapidly advancing als . a buddhist priest began to experience weakness of the right upper and lower limbs at 64 years of age . six months after onset , a clinical examination revealed weakness in all four limbs [ medical research council ( mrc ) grade 4 ] with widespread fasciculation and hyperreflexia in the lower extremities . plantar reflexes were in flexion on both sides , and there were no sensory abnormalities or cognitive disturbances . magnetic resonance images of the brain and spinal cord were normal ; however , electromyography revealed changes indicating acute and chronic denervation in the tongue , facial muscles and all limb muscles . in contrast , motor and sensory nerve conduction studies were normal , as were routine laboratory tests . based on the revised el escorial criteria , als was confirmed as the clinical diagnosis , and the patient was treated with 100 mg of riluzole daily . approximately 13 months after onset , the patient s clinical features rapidly worsened , with progressive weakness in all limbs , predominantly on the right side ( mrc grade 2 ) . the rapidly progressive weakness was accompanied by a mild pinching sensation in the fingertips in both hands , although predominantly in the right hand . a physical examination revealed transverse overcurvature of the nails to the extent that the nails were now pinching the fingertips ( figure 1 ) on both hands , consistent with a pincer nail deformity . although the mild pinching sensation continued , no treatment was performed due to the patient s lack of pain . although an alternating pressure air mattress helped to prevent bed sores , the dysphagia gradually worsened and he was unable to take solid food during the last two months of his life . he subsequently stopped taking riluzole because allowing the medication to dissolve in his mouth induced numbness of the tongue . he thereafter received palliative care , with morphine to combat dyspnea during the last three weeks of his life . several skin alterations have been reported in patients with als , including elastosis , increased mucopolysaccharides , altered collagen , thickened blood vessel walls and reduced sweat secretion in association with advancing disease . the pincer nail deformity is characterized by distortion of the shape of the nail with transverse overcurvature , resulting in pinching of the soft tissue of the digit . pincer nails may be associated with systemic diseases , such as psoriasis , systemic lupus erythematosus , kawasaki disease , osteoarthritis of the distal phalanx or renal failure , as well as -blocker intake . however , there have been no previous reports of als patients presenting with pincer nails . due to the absence of -blocker use or systemic disease other than als in this patient , we speculate that the nail deformity was related to the advancing als . compared with that observed in age - matched controls , the activity of matrix metal - loproteinase ( mmp)-9 in the skin is increased in patients with als.4 an elevated mmp-9 level is associated with more rapid disease progression , and mmp-9 upregulation may be linked to collagen degradation and shrinking of the nail bed , which subsequently contributes to nail incurvation . furthermore , a disturbance in the sympathetic skin response and hypohidrosis on the palm may also occur in those in an advanced stage of als , and the use of -blockers , which depress the sympathetic activity , may also induce pincer nail formation.9 these observations suggest a potential link between rapidly advancing als and the pincer nail deformity , as observed in the current case . | although several manifestations of skin involvement in cases of amyotrophic lateral sclerosis ( als ) have been reported , the pincer nail deformity has not been previously reported in a patient with als .
the pincer nail deformity is characterized by distortion of the shape of the nail with transverse over curvature and may be associated with systemic diseases and certain medications .
we herein describe the case of a patient presenting with a pincer nail deformity associated with rapidly advancing als .
this case suggests a potential link between quickly advancing disease and the pincer nail deformity . |
the ao - fixed - angle blade plate is popular among surgeons for fixation of proximal femoral osteotomies [ 1 , 2 ] and is a common procedure for patients with significant hip displacement as a result of cerebral palsy . there are several complications associated after femoral osteotomy including wound infection , bursitis and peroneal nerve palsies , but fracture rates are relatively rare . this suggests little evidence is available to determine the best surgical management following a significant femoral fracture . the musculoskeletal difficulties commonly seen in quadriplegic cerebral palsy patients can add to the complexity of these cases , as younger patients may not be suitable for standard adult proximal femoral fixation techniques . we report a case of using of a proximal humeral plate for a revision fixation in a paediatric femoral fracture . an 18-year - old female with a history of quadriplegic cerebral palsy ( gmfcs - v ) was admitted to our orthopaedic department following a peri - prosthetic fracture of the right femur . she is well known to our department , as her non - ambulatory status has resulted in a number of surgeries including a right adductor tenotomy , aged 11 , femoral derotation osteotomy and dega acetabular osteotomy , aged 13 and right distal hamstring and knee capsule release , aged 14 . she presented to the emergency department with a painful right thigh after getting her leg caught in doorway while being mobilized in a wheelchair . the trauma resulted in lateral bending of the leg , with examination revealing a significant deformity of the thigh with audible crepitus . radiographs revealed a peri - prosthetic fracture of the right femoral shaft extending distal to the ao blade plate ( fig . 1 ) . the blade was also seen to be encroaching on the inferior cortex of the femoral neck leading to concerns about creating a stress riser or femoral neck fracture in the event of revision blade plate fixation . there was also concern that the longest replacement blade plate available would not be of sufficient length to stabilize the fracture distally . the complexity of this case along with the patients low body weight ( 26 kg ) , small skeletal morphology and underlying neurological disorder deemed revision with a long - blade plate , or standard adult femoral fixation devices unsuitable . therefore , we proposed a long philos proximal humeral locking plate ( synthes , uk ) as a suitable device for internal fixation of the fracture to provide adequate plate osteosynthesis , with secure proximal and distal fixation , bridging the fracture and the femoral neck defect . figure 1:ap - xr demonstrating a peri - prosthetic fracture of the right femur below the blade plate . ap - xr demonstrating a peri - prosthetic fracture of the right femur below the blade plate . open reduction and internal fixation using a 10 hole philos plate was performed with the patient in a supine position on the traction table utilizing a lateral approach . using c - arm image intensification , the height of the plate was judged , then applied to the bone and secured both proximally and distally to the fracture with proximal locking screws inserted where possible ( fig . the proximal femur proved challenging due to the femoral neck defect left by the removal of the blade plate . final reduction was judged to be near anatomical with the wound thoroughly irrigated with saline before closure . post - operative treatment included iv antibiotics with mobility instructions unnecessary due to the patient 's non - ambulatory status . the patient was discharged after 5 days with a follow - up period over 11 months . there were no post - operative complications during this time and radiographs showed an anatomically healed fracture ( fig . figure 2:image intensifier intra - operative ap highlighting 10 hole philos applied with near anatomical reduction ( a c ) . figure 3:ap - xr demonstrating radiographic union at 11 months with 10 hole philos insitu . image intensifier intra - operative ap highlighting 10 hole philos applied with near anatomical reduction ( a c ) . however , two studies have reported several fractures following proximal femoral osteotomies utilizing an ao - fixed - angle blade plate . , reported 14 various complications after performing proximal femoral osteotomies on a total of 157 paediatric patients . they reported only five fractures occurring in patients with cerebral palsy , with fractures of proximal fixation ( two patients ) , distal plate fixation , proximal tibia and distal femur . management of these fractures included cast immobilization , anterior plate and an interfragmentary screw with little comment found regarding the repair of the distal femur . performed 58 femoral osteotomies utilizing an ao 90-fixed - angle blade plate in children with cerebral palsy and noted only one fracture , which was revised using flexible intramedullary nails . with the vast majority of femoral fractures treated by conservative means , a number of difficulties can arise when considering management of femoral fractures in cerebral palsy ( cp ) patients , with previous studies having noted severity of contractures and non - ambulatory status impacting on higher malunion rates and adding to the complexity of these cases . the few studies that have examined treatment of femoral fractures in cp patients have noted the majority of cases are treated conservatively , particularly in non - ambulaters . therefore , little is known of the most appropriate surgical management for patients unsuitable for long leg or spica cast application . to our knowledge only one retrospective study review exists that evaluated the treatment of femur fractures in both ambulators and non - ambulatory patients . reported that although the majority of non - ambulators were treated conservatively at their centre , 6 of the 38 fractures were treated operatively , with procedures including percutaneous pinning , plate fixation , external fixation and intramedullary nailing . to our knowledge , this is the first reported case of using a philos plate ( proximal humerus internal locking system ) to treat a paediatric proximal femur fracture . the philos plate is a locking compression plate that utilizes locking screws to provide stable fixation and hold in osteoporotic bone . a number of studies have advocated its use in proximal humeral fractures , with encouraging evidence of both short- and long - term clinical outcomes [ 9 , 10 ] . although philos plates have been exclusively designed for the treatment of proximal humerus fractures , this does not prevent their use at alternative sites . we found that application of a philos plate provided a suitable alternative for stable fixation of a proximal femoral fracture with no long - term complications identified so far . it is important that trauma surgeons retain the ability for lateral thinking when approaching complex and challenging patients and fractures , though care should be taken when using implants in areas they were not specifically designed for . | in this case an 18-year - old female with cerebral palsy sustained a peri - prosthetic femoral fracture adjacent to a blade plate previously inserted for a femoral varus osteotomy .
the injury was treated using a long proximal humeral locking plate .
the existing blade plate was removed .
the fracture was reduced and held , and a 10-hole philos plate applied with near anatomical reduction .
there were no post - operative complications .
radiographic union was confirmed at 11 months . to our knowledge , this is the first reported use of a philos plate in the management of a femoral peri - prosthetic fracture and successfully demonstrated a straightforward method for revision fixation . |
c57bl/6j , b6.cg-tg(cd4-tgfbr2)16flv/j ( cd4dntgfrii ) , c57bl/6-tg(tcra2d2,tcrb2d2)1kuch / j ( tcr(2d2 ) ) and b6.129s6-tbx21/j ( t - bet ) mice were purchased from jackson laboratory ( bar harbor , me ) , b6.129s6-rag2 ( rag2 ) mice from taconic ( hudson , ny ) , tgfbr1 mice were bred with cd4-cretransgenic mice and tgfbr1/cd4-cre littermates were used as controls . ot - ii cd45.1 mice , bac - transgenic rorc(t)-gfp mice and rorc(t)-gfp littermate controls were previously described . all animal studies were performed according to the nih guidelines for the use and care of live animals and were approved by the institutional animal care and use committee of niams or the iacuc of schering - plough biopharma in accordance with guidelines of the association for assessment and accreditation of laboratory animal care . cd4 t cells from spleens and lymph nodes of 6- to 8-week - old mice were purified by negative selection and magnetic separation ( miltenyi biotec , germany ) followed by sorting of naive cd4cd62lcd44cd25 population using facsaria ii ( bd , nj ) . cells were activated by plate - bound anti - cd3/cd28 ( both 10 g ml ; ebioscience , ca ) in serum - free medium for 34 days either under neutral conditions or with il-6 plus il-1(each 20 ng ml ) and either human tgf-1 ( 0.5 ng ml ; th17( ) ) or il-23 ( 50 ng ml ; th17(23 ) ) ( all from r&d systems , mn ) . in all cell cultures anti - ifn- neutralizing antibodies ( 10 g ml , bd pharmingen ) were added . il-12 ( peprotech , nj ) was used at 20 ng ml , huil-2 at 100 u ml . procedures for antagonizing tgf--signaling , isolation of lamina propria lymphocytes and cns - infiltrating lymphocytes are given in methods . freshly isolated t cells or nave t cells activated under the indicated conditions were analyzed . for statistical analysis , all p values were calculated with student s t - test and p<0.05 was considered as significant . c57bl/6j , b6.cg-tg(cd4-tgfbr2)16flv/j ( cd4dntgfrii ) , c57bl/6-tg(tcra2d2,tcrb2d2)1kuch / j ( tcr(2d2 ) ) and b6.129s6-tbx21/j ( t - bet ) mice were purchased from jackson laboratory ( bar harbor , me ) , b6.129s6-rag2 ( rag2 ) mice from taconic ( hudson , ny ) , tgfbr1 mice were bred with cd4-cretransgenic mice and tgfbr1/cd4-cre littermates were used as controls . ot - ii cd45.1 mice , bac - transgenic rorc(t)-gfp mice and rorc(t)-gfp littermate controls were previously described . all animal studies were performed according to the nih guidelines for the use and care of live animals and were approved by the institutional animal care and use committee of niams or the iacuc of schering - plough biopharma in accordance with guidelines of the association for assessment and accreditation of laboratory animal care . cd4 t cells from spleens and lymph nodes of 6- to 8-week - old mice were purified by negative selection and magnetic separation ( miltenyi biotec , germany ) followed by sorting of naive cd4cd62lcd44cd25 population using facsaria ii ( bd , nj ) . cells were activated by plate - bound anti - cd3/cd28 ( both 10 g ml ; ebioscience , ca ) in serum - free medium for 34 days either under neutral conditions or with il-6 plus il-1(each 20 ng ml ) and either human tgf-1 ( 0.5 ng ml ; th17( ) ) or il-23 ( 50 ng ml ; th17(23 ) ) ( all from r&d systems , mn ) . in all cell cultures anti - ifn- neutralizing antibodies ( 10 g ml , bd pharmingen ) were added . il-12 ( peprotech , nj ) was used at 20 ng ml , huil-2 at 100 u ml . procedures for antagonizing tgf--signaling , isolation of lamina propria lymphocytes and cns - infiltrating lymphocytes are given in methods . freshly isolated t cells or nave t cells activated under the indicated conditions were analyzed . a detailed description of procedures and associated references are given in methods . for statistical analysis , all p values were calculated with student s t - test and p<0.05 was considered as significant . | cd4 + t cells that selectively produce interleukin ( il)-17 , are critical for host defense and autoimmunity14 .
crucial for t helper17 ( th17 ) cells in vivo5,6 , il-23 has been thought to be incapable of driving initial differentiation .
rather , il-6 and transforming growth factor ( tgf)-1 have been argued to be the factors responsible for initiating specification710 .
herein , we show that th17 differentiation can occur in the absence of tgf- signaling .
neither il-6 nor il-23 alone efficiently generated th17 cells ; however , these cytokines in combination with il-1 effectively induced il-17 production in nave precursors , independently of tgf-. epigenetic modification of the il17a / il17f and rorc promoters proceeded without tgf-1 , allowing the generation of cells that co - expressed rort and t - bet .
t - bet+ rort+ th17 cells are generated in vivo during experimental allergic encephalomyelitis ( eae ) , and adoptively transferred th17 cells generated with il-23 without tgf-1 were pathogenic in this disease model .
these data suggest an alternative mode for th17 differentiation .
consistent with genetic data linking il23r with autoimmunity , our findings re - emphasize the importance of il-23 and therefore have may have therapeutic implications . |
central nervous system ( cns ) may be affected by vasculitides as part of systemic illness or the vasculitic process may afflict the cns in isolation . patients with the angiitis may present with ischemic and/or hemorrhage stroke , progressive cognitive impairment , parkinsonism , ataxia , ocular movement abnormalities , dysarthria , dysphonia , dysphagia , myelopathy , headaches , seizures and other manifestations . we present a case of a male patient in whom the disease masqueraded as various other conditions until finally pacns was diagnosed . a 28-year - old non - hypertensive , non - diabetic , male tailor presented with recurrent episodic hemicranial throbbing headache associated with nausea for 4 years and generalized tonic - clonic seizures for around 1 year . his ct scan head showed multiple white matter hypodensities with evidence of speckled enhancement in some areas post contrast [ figure 1 ] . provisional diagnosis of epilepsy due to multiple neurocysticercosis and migraine was considered and the patient was started on antiepileptic drug , phenytoin . while his brain mri was being planned , he became clumsy , inattentive and started taking extraordinarily long time to tailor - cut the cloth material . meanwhile , mri brain showed multiple white matter t1w hypointensities and t2w hyperintensities with evidence of speckled enhancement post - gadolinium [ figure 1 ] . his fundus examination did not reveal any abnormality and his pupillary light responses were noted to be normal . mr angiography ( mra ) of intracranial and neck vessels did not reveal any vascular anomaly . the patient was lost to follow - up over the next few months . during this time , he wandered away from his home without being able to find his way back . when he was traced subsequently to a city away from his home , he could not recall what transpired during the days . subsequently , he had convulsions and developed acute onset right hemiparesis resulting in his fall from stairs . he was admitted to another local medical facility and his ct scan head done there showed left frontal hematoma . he improved only to have recurrence of sudden onset weakness of right half of body with aphasia following an episode of seizure and was admitted in our hospital . brain ct scan showed resolving hematoma in the left frontal region . in the hospital , the patient developed acute confusional state and a repeat ct scan showed appearance of fresh right sided frontal parenchymal hematoma . after re - evaluation of patient 's history and imaging findings , the diagnosis of cns angiitis was considered . the patient tested negative for various investigations including antinuclear antibody , rheumatoid arthritis factor , cytoplasmic and perinuclear antineutrophilic cytoplasmic antibody , venereal disease research laboratory test for syphilis and elisa for human immunodeficiency virus . cerebrospinal fluid ( csf ) analysis of the patient was done once the patient stabilized and it showed raised protein levels ( 111 mg% ; normal range : 15 - 45 mg% ) , normal glucose ( 52 mg% normal range 45 - 80 mg% ) with normal cytology . gram stain , zn stain , india ink stain and culture of csf were negative . the biopsy of brain showed non - caseating granuloma in the dura and brain parenchyma confirming the diagnosis of granulomatous angiitis of cns [ figure 2 ] . pulse of cyclophosphamide was repeated twice before the patient was lost in the follow up . on his last hospital appearance bilateral frontal and parietal hypodensities ( arrows ) seen on brain ct scan ( contrast enhanced ) on the left . these appear as iso or hypointese lesions exhibiting speckled enhancement on mri brain ( with gadolinium enhancement ) histopathological appearance of brain / meningeal biopsy showing giant cells and mononuclear cell infiltration of vessel walls in the meninges ( h and e stain ) neurological manifestations are diverse , the most common being headache , cognitive impairment , ataxia , seizures , and focal neurological deficits such as hemiparesis , aphasia , dysarthria , decreased visual acuity due to transient ischemic attack , ischemic stroke and intracranial hemorrhage . focal neurological manifestations , headache and seizures are present in various other disorders affecting cerebral blood vessels . the condition characterized by vasoconstriction of cerebral vessels is seen in postpartum state and due to exposure to vasoactive drugs . cerebral angiography shows segmental vasoconstriction of cerebral blood vessels similar to the appearance in pacns . the condition is not associated with inflammatory changes of the vessels as are seen in pacns . another important differential diagnosis is systemic vasculitis which is commonly associated with the presence of constitutional symptoms and serological markers . he subsequently had 2 episodes of acute hemorrhagic stroke over a period of 2 months . the patient developed insidious onset cognitive dysfunction involving multiple domains including attention , recent memory , judgment , visuospatial orientation , language function and praxis . intracranial hemorrhage followed by subarachnoid hemorrhage are the presenting feature in 11 - 12% of pacns . compared to patients without intracranial hemorrhage , those with it are reportedly less likely to have altered cognitive function and require therapy less frequently on subsequent follow - up . the hallmark of the pacns is that the inflammatory process is limited to the cns . sensitivity of cerebral angiography for detection of vasculitis in various studies is between 27% and 90% and for brain biopsy it is between 36% and 83% . the classical findings suggestive of granulomatous angiitis are observed in around 50 - 60% of cases . lymphocytic and necrotizing patterns are observed in 25% and 14 - 22% respectively of cases of pacns . the inflammation and fibrinoid necrosis leads to thickening of vessel walls which increases the risk of vascular rupture and aneurysmal dilatation . although there are no controlled therapeutic trials for cns vasculitis , current treatment recommendation is steroid along with intravenous cyclophosphamide for severe disease . the combination of corticosteroid and rituximab was recently reported to be effective in a report of patient who did not tolerate cyclophosphamide . as yet , there is scarcity of information about etiology , pathogenesis , appropriate diagnostic strategies and treatment approaches for pacns . | primary angiitis of central nervous system ( pacns ) is characterized by non - caseating granulomatous angiitis restricted to cns .
the condition often masquerades as migraine , stroke , epilepsy , dementia , demyelinating disorder and cns infection .
the protean manifestations frequently lead to misdiagnoses .
we present a case of a young male from rural background that remained undiagnosed for years as the possibility of pacns was not considered .
he had history suggestive of migraine - like headaches followed by seizures .
subsequently , he developed rapidly progressive dementia and two episodes of hemorrhagic strokes over a short period .
the diagnosis was finally clinched by the absence of evidence of systemic vasculitis and the presence of characteristic non - caseating granuloma around vessels of duramater and cerebral parenchyma on brain biopsy .
he was started on pulse therapy with intravenous cyclophosphamide and methylprednisolone .
the current literature about the condition and its management is reviewed in this report . |
the lyophilized extract was dissolved in distilled water and applied to a sephadex g-50 column ( 3.0 35 cm ) , equilibrated with distilled water , and eluted with distilled water at a flow rate of 1.5ml / min . the active fractions obtained were then applied to a c18 solid - phase extraction column ( sep - pak c18 cartridges ; waters co. , milford , ma , usa ) and equilibrated with 5% acetonitrile . acetonitrile step gradient elution was carried out in water at 5% , 25% , 50% , and 100% ( v / v ) acetonitrile . the active fraction was then applied to an analytical reverse phase high - performance liquid chromatography column ( rp - hplc ; vydac 218tp54 , c18 column , 5 m , 4.6 250 mm ; discovery science co. , deerfield , il , usa ) equilibrated with 5% acetonitrile . a linear acetonitrile elution gradient was carried out with 0.1% trifluoroacetic acid in water from 5% to 25% . male sprague - dawley rats ( age , 7 weeks ; weight , 180~200 g ) were maintained on a 12-hr light / dark cycle in a temperature and humidity - controlled room for 1 wk . all rats were randomly distributed into experimental groups ( n = 5/group ) . the uricase inhibitor potassium oxonate was used to induce hyperuricemia in the rats [ 18 , 19 ] . to increase serum urate levels , the rats were injected intraperitoneally with potassium oxonate ( 280 mg / kg ) 1 hr before the p. ostreatus extract test samples were administered . then , various concentrations of p. ostreatus ( 1,000 mg / kg and 500 mg / kg ) and the anti - gout agent allopurinol ( 50 mg / kg ) were administered orally . blood was obtained from the rats by cardiac puncture , and was allowed to clot for approximately 1 hr at room temperature . then , it was centrifuged at 3,000 rpm for 10 min to obtain serum , which was stored at -20 until use . each experiment was performed at least three times , and all quantitative data were expressed as mean standard deviation ( sd ) values . xod inhibitory activities of the water extracts of the fruiting bodies of six species of edible mushrooms were determined ( table 1 ) . the water extract from p. ostreatus had the highest xod inhibitory activity ( 78.3% ) , and the xod inhibitory activity of the extract of p. salmoneo - stramineus was also high ( 67.2% ) . the xod inhibitory activity of p. ostreatus was higher than that of the edible mushroom agaricus brasiliensis ( 72.9% ) . in contrast , the 95% ethanol extracts had very low ( < 10% ) xod inhibitory activity or not detectable activity ( data not shown ) . the effect of temperature and time on the extraction of the xod inhibitor from p. ostreatus was evaluated at temperatures ranging from 30 to 60 and extraction times ranging from 24 to 72 hr . water extracts obtained at 30 and 40 showed higher xod inhibitory activities ( 72.2~83.2% ) than those of the extracts obtained at 50 and 60 ( 17.0~41.7% ) . the maximal xod inhibitory activity ( 83.2% , ic50 12.2 mg ) of the p. ostreatus fruiting body was demonstrated by the extracts obtained with distilled water at 40 for 48 hr ( fig . purification of the water extracts of p. ostreatus was performed by sephadex g-50 gel filtration chromatography and the xod inhibitory fraction had an ic50 of 10.4 mg / ml . after ultrafiltration of the active fraction using the centriprep ym-50 , 30 , and 3 ultrafiltration units ( millipore co. , billerica , ma , usa ) , we obtained active filtrates of less than 3 kda by ultrafiltration ( ic50 , 1.9mg / ml ) . the active sub-3 kda filtrates were subjected to c18 solid - phase elution with an acetonitrile gradient ranging from 5% to 100% . the eluates from 5% acetonitrile had the highest xod ic50 ( 1.5 mg / ml ) . the active extracts from the 5% acetonitrile elution were subjected to rp - hplc using a vydac protein / peptide reverse - phase 218 t p54 column , and purified xod inhibitor was obtained at a yield of 3.0% ( w / w ) and an xod ic50 of 0.9 mg / ml ( 13.6-fold purification ) ( table 2 , fig . the purified xod inhibitor was analyzed by lc - ms / ms and a tripeptide with the amino acid sequence phenylalanine - cysteine - histidine was obtained ( fig . the anti - gout action of the ultrafiltrates from p. ostreatus was evaluated in normal rats and potassium oxonate - treated hyperuricemic rats . as shown in fig . 4 , the serum uric acid level in the non - hyperuricemic rats ( normal rats ; administered distilled water ) was 2.4 0.8 mg / dl , while that in the potassium oxonate - treated hyperuricemic rats that received no xod inhibitor was 8.3 0.14 mg / dl . when hyperuricemic rats were administered p. ostreatus extracts at an oral dose of 100mg / kg or 500mg / kg , uric acid levels decreased to 6.35 0.5 mg / dl and 7.75 0.4 mg / dl respectively , but these decreases were not statistically significantly . allopurinol treatment significantly decreased serum urate levels to values nearing the range in normal rats . while the degree of the effect of the p. ostreatus extract was not as high as that of allopurinol , these results suggest that the decrease in serum uric acid levels was caused by the xod inhibitory activity of the p. , we conclude that the extract obtained from p. ostreatus is a potent bioactive agent for the development of foods or drugs beneficial for the treatment of gout . | we selected pleurotus ostreatus from among several edible mushrooms because it has high anti - gout xanthine oxidase ( xod ) inhibitory activity .
the maximal amount of xod inhibitor was extracted when the pleurotus ostreatus fruiting body was treated with distilled water at 40 for 48 hr .
the xod inhibitor thus obtained was purified by sephadex g-50 gel permeation chromatography , ultrafiltration , c18 solid phase extraction chromatography and reverse - phase high - performance liquid chromatography with 3% of solid yield , and its xod inhibitory activity was 0.9 mg / ml of ic50 . the purified xod inhibitor was a tripeptide with the amino acid sequence phenylalanine - cysteine - histidine and a molecular weight of 441.3 da .
the xod inhibitor - containing ultrafiltrates from pleurotus ostreatus demonstrated dose - dependent anti - gout effects in a sprague - dawley rat model of potassium oxonate - induced gout , as shown by decreased serum urated levels at doses of 500 and 1,000 mg / kg , although the effect was not as great as that achieved with the commercial anti - gout agent , allopurinol when administered at a dose of 50 mg / kg . |
a 63-year - old male patient was regularly followed up because of chronic hepatitis b virus infection and diabetes mellitus . his history of present illness was a right incidental renal tumor that was found by ultrasonography ( us ) . abdominal and pelvic computed tomography ( ct ) revealed a 50x45 mm right renal tumor with an indistinct border . the right renal contour was smooth , and there was no extrusion by the tumor . the ct images also revealed two left adrenal tumors ( 2015 mm and 1510 mm ) ( fig . 1a ) . by enhanced ct ( dynamic study ) with the use of contrast material , the right renal tumor was heterogeneously enhanced and the degree of enhancement was weaker than the normal renal parenchyma in the early phase . the two left adrenal tumors demonstrated the same enhanced pattern as the right renal tumor ( fig . the differential diagnoses of the right renal tumor on the basis of the ct findings included rcc , transitional cell carcinoma , malignant lymphoma , and metastasis . in addition , the left adrenal tumors were suspected to be metastases , adenomas , or pheochromocytomas . including hormonal examination , laboratory data were normal except for an elevated hba1c ( 8.5% ) and positivity for hbs antigen . no lesions had been pointed out during the patient 's past periodic checkups , including ct and us . before the operation , i - mibg scintigraphy revealed no uptake in the left adrenal region and we ruled out pheochromocytoma . macroscopically , the size of the right renal tumor was about 5045 mm and the sizes of the left adrenal tumors were 1815 mm and 1510 mm ( fig . pathological examination revealed that the tumor form was alveolar growth and there were many atypical cells that were composed of swelling nuclei in the clear cytoplasm . the pathological diagnosis of the resected left adrenal tumors was also rcc . since the operation about 25% of patients with rcc show evidence of metastasis at the time of presentation . the lungs , bone , lymph node , and liver are the major sites of metastatic spread . malignant involvement of the ipsilateral adrenal gland is noted in only 1.2% to 10% of cases . moreover , contralateral adrenal involvement by rcc is extremely rare . to study the frequency of metastasis in patients with rcc , saitoh et al conducted autopsies in 1,828 patients . they reported that solitary and synchronous contralateral adrenal metastases were seen in 0.19% of the patients . in 2003 , lau et al described that the type of rcc that metastasized to the contralateral adrenal gland was only the clear cell type . contralateral adrenal metastasis of rcc is thought to occur via the hematogeneous route , as is the case for other organ metastases . some studies reported that the adrenal gland was suggested to have a higher affinity for the spread of rcc than other organs because contralateral adrenal metastasis occurred . zornoza et al mentioned that the abundant blood flow and sinusoidal structure of the adrenal gland may affect this unique metastasis . concerning adrenal tumors , it is said that 50% to 75% of incidental adrenal tumors in oncology patients are metastases and 30% to 50% are nonfunctional adenomas . to distinguish a benign adenoma from a malignant adrenal mass , it is necessary to first identify the presence of fat in the tumor by nonenhanced ct or magnetic resonance imaging ( in / opposite phase ) . second , a ct protocol to evaluate the wash - out rate of contrast media in an adrenal mass may be useful . in about one - third of rcc patients with isolated adrenal metastasis , surgical resection of the adrenal metastasis leads to an apparently curative outcome . in general , rcc patients with a solitary metastasis survive significantly longer than do those with multiple metastases . complete resection of the primary renal and metastatic adrenal tumors should be the most important clinical treatment in cases such as this case . to our knowledge , we are the first to report a case of rcc with double synchronous contralateral adrenal metastases . in cases of a unilateral renal tumor and contralateral adrenal tumor | a 63-year - old male patient visited our hospital with a right incidental renal tumor , which was found by ultrasonography for the follow - up study of chronic hepatitis b virus infection and diabetes mellitus .
consecutive computed tomography revealed a right renal tumor and two left adrenal tumors .
further systemic imaging study and hormonal examination suggested one right renal cell carcinoma and left adrenal metastases .
we performed right nephrectomy and left adrenalectomy .
the pathological diagnoses of all resected tumors were renal cell carcinoma .
the patient has been in good health without any recurrence for 12 months since the operation . in patients with renal cell carcinoma , contralateral adrenal metastasis
is usually associated with multiple metastases to other organs .
there are a few cases of solitary and synchronous contralateral adrenal metastasis in the english literature . to our knowledge
, this is the first report of a case of renal cell carcinoma with double synchronous contralateral adrenal metastases . |
to describe a patient with intrachoroidal cavitation in the normal eye that caused self - limiting recurrent macular detachment and retinoschisis . an 80-year - old female patient with intrachoroidal cavitation in the normal eye presented with macular detachment and retinoschisis after cataract surgery . one year after cataract surgery , the patient presented with macular detachment and retinoschisis in the same eye again . this case suggests that similar cases of cystoid macular edema after cataract surgery can occur , and that intrachoroidal cavitation is observed not only in eyes with pathologic myopia but also in normal eyes with peripapillary atrophy ; intrachoroidal cavitation can cause macular detachment and retinoschisis . macular detachment and retinoschisis are often associated with x - linked retinoschisis , pit - macular syndrome and coloboma [ 13 ] . however , recent studies have reported other causes of macular detachment and retinoschisis [ 47 ] . intrachoroidal cavitations are often observed in pathologic myopia , but not in normal eyes , and can result in retinoschisis and macular serous detachment [ 5 , 8 ] . other studies have shown peripapillary retinoschisis in glaucomatous eyes [ 9 , 10 ] , which resolved without treatment in some cases . here , we present a patient with intrachoroidal cavitation in the normal eye , with peripapillary atrophy ( ppa ) , that caused self - limiting recurrent macular detachment and retinoschisis . an 80-year - old woman presented with visual loss in her right eye to the sensho - kai eye institute . her best - corrected visual acuity measured 20/100 od and 20/16 os . in the right eye , a grade 2 nuclear cataract based on the emery scale was observed with an axial length of 23.80 mm and a refractive error of 1.0 diopters . the intraocular pressure measured 15 mm hg od and 14 mm hg os . no significant changes were observed in other parts of her eyes except small temporal conus . macular serous detachment and cysts were observed using rtvue-100 fourier - domain optical coherence tomography ( rtvue - oct ; optovue inc . , although rtvue - oct showed prominent serous detachment and cysts , fluorescent angiography showed slight pooling in the cystic space and no robust leakage comparable to configuration obtained by rtvue - oct ( fig . the eye was treated as cystoid macular edema with intravitreal bevacizumab ( 1.25 mg ) 2 months after surgery . the patient presented with decreased vision and central scotoma in her right eye to kyoto medical center . rs-3000 spectral - domain oct examination ( sd - oct ; nidek co. ltd . , tokyo , japan ) showed macular detachment and retinoschisis extending to ppa ( fig . intrachoroidal cavitations connecting to the cystic space were observed adjacent to the disc ( fig . the connection between the intrachoroidal cavitation and cystic space appeared as breakage of elschnig 's collagenous limiting tissue , which connects the choroid to the optic disc . since the patient refused further examinations , such as fluorescent angiography , sd - oct was used to monitor the condition monthly . serous retinal detachment and retinoschisis gradually absorbed , and was almost fully absorbed 4 months after onset ( fig . the left eye also showed intrachoroidal cavitation ; however , no incidence of retinoschisis or retinal detachment has been noted ( fig . no coloboma or optic disc pit was observed on ophthalmoscopy , but some ppa was found in the patient 's eyes . in her right eye , a yellow - orange lesion was also found that was reported as peripapillary detachment in pathologic myopia ( fig . 2 g ) . the hyporeflective space observed with sd - oct suggested the presence of fluid was not in large blood vessels but in the space of the intrachoroidal cavitation because no corresponding large vessels were visible in the ppa- area . the first incidence was resolved within 9 months with intravitreal bevacizumab , whereas the second incidence was resolved without treatment . this is similar to irvine - gass syndrome that is characterized by cystoid macular edema following cataract surgery . however , fluorescent angiography did not show the robust leakage associated with serous detachment and retinoschisis , suggesting another source of fluid like pit - macular syndrome could exist . retinoschisis appeared to extend to the disc , unlike that observed in common cystoid macular edema . recent studies have reported that peripapillary retinoschisis can be seen in glaucomatous eyes [ 9 , 10 ] and resolved without treatment . in our case , however , macular detachment and retinoschisis occurred immediately after cataract surgery , and may have therefore caused the fluctuation of intraocular pressure . the period of self - resolution of the second incidence was even shorter than that of the first . we have encouraged the patient to follow up periodically to assess any changes . although previous studies on peripapillary detachment in pathologic myopia showed small pit formation connecting the cystic space and vitreous , no such pit formation was observed in our case on thorough observation using sd - oct . the subretinal fluid in other cases may have been derived from the vitreous ; however , the fluid in our case may have been derived from intrachoroidal cavitation or spinal fluid . since there was no connection between vitreous and macular detachment , such a condition may facilitate self - resolution . previous studies have reported intrachoroidal cavitation or peripapillary detachment in pathologic myopia but not in normal eyes [ 47 ] . here , we reported a case of intrachoroidal cavitation in an eye with normal length and having ppa , resulting in self - limited recurrent macular detachment and retinoschisis . our case suggests that similar cases of cystoid macular edemas after cataract surgery exist , and that intrachoroidal cavitation is observed not only in pathologic myopia but also in normal eyes with ppa ; intrachoroidal cavitation can cause macular detachment and retinoschisis . in cases of cystoid edema following cataract surgery , we should carefully observe not only the macular area but also the peripapillary area . | purposeto describe a patient with intrachoroidal cavitation in the normal eye that caused self - limiting recurrent macular detachment and retinoschisis.case reportan 80-year - old female patient with intrachoroidal cavitation in the normal eye presented with macular detachment and retinoschisis after cataract surgery .
these were treated with intravitreal bevacizumab and then absorbed within 9 months .
one year after cataract surgery , the patient presented with macular detachment and retinoschisis in the same eye again .
these were absorbed within 4 months without treatment.conclusionthis case suggests that similar cases of cystoid macular edema after cataract surgery can occur , and that intrachoroidal cavitation is observed not only in eyes with pathologic myopia but also in normal eyes with peripapillary atrophy ; intrachoroidal cavitation can cause macular detachment and retinoschisis . |
a recent report of bilateral retinal injuries from hand - held lasers by yiu et al raised awareness of the hazards of recreational lasers.1 the report referred to a class 4 , high - powered 1,250 mw laser that was manufactured from the 445 nm blue diode of a dismantled home theater projector . retinal injuries from commercial lasers have been shown in previous papers to have a fairly low incidence.2 however , the incidence of ocular injuries has appeared to increase as personal lasers have come into widespread use.1,35 we recently encountered two patients who presented with sub - internal limiting membrane hemorrhage after exposure to a 2 mw - powered 510 nm diode laser , which is usually considered relatively safe , during a laser show . a 20-year - old healthy female presented with decreased visual acuity in her right eye that developed several hours after visiting a party with laser shows . on examination , her best corrected visual acuity ( bcva ) in the right eye was 20/800 . retinal examination revealed a sub - internal limiting membrane hemorrhage and a small , round , whitish lesion superior to the fovea that was consistent with a mild laser burn ( figure 1a ) . a pars plana vitrectomy was performed to remove the hemorrhage , and bcva recovered to 20/20 without scotoma on visual field examination . fluorescent angiography and spectral - domain optical coherent tomography were performed 2 months after the incident , and two small hyperfluorescent dot lesions were observed on fluorescent angiography ( figure 1b d ) . spectral - domain optical coherent tomography showed mild atrophic changes in the inner retina ( figure 1e ) . a 20-year - old female without any significant medical history visited the emergency department because of decreased vision in her left eye . she had been to the same dance club as the patient in case 1 the day prior to presentation in the emergency department , and described similar events ( figure 2a ) . after removing the sub - internal limiting membrane hemorrhage , a tiny intraretinal hemorrhage at the lower margin of the sub - internal limiting membrane hemorrhage was detected , along with a small burn at the superior margin ( figure 2b and d ) . one week after vitrectomy , bcva recovered to 20/20 without scotoma on a 24 - 2 visual field examination . fluorescent angiography taken 2 weeks after the incident revealed small window defects ( figure 2c ) . spectral - domain optical coherent tomography showed mild atrophic changes in the inner retina ( figure 2e ) . they each recalled that a randomly aimed laser beam might have been directed into their eyes . they stated that they had drunk a couple of beers but denied the use of illicit substances that night . the findings of mild retinal burns apart from the fovea in both patients support these statements . although the precise mechanism of injury in the two patients presented herein is unknown , it is highly probable that exposure to the diode laser resulted in contraction of the retina and disruption of the adjacent microvasculature . the importance of these cases is that the 510 nm diode laser used in these laser shows was graded as a class 3r device by the international electrotechnical commission 60825 - 1 standard.6 it has been considered safe under 5 mw if handled carefully with restricted beam viewing . the persons in charge of the shows denied any kind of manipulation of the laser device , especially in terms of power output . damage to the eye by accidental laser exposure depends on the power of the laser , wavelength , exposure time , and various individual factors . we speculate that multiple factors , such as repeated exposure to lasers that lasts for several hours , high body temperature while dancing , and the possibility of beam viewing while in a drunken state resulted in retinal damage from a relatively low - powered laser diode . importantly , both patients in this report had otherwise normal eyes and vision , and the retinal damage appeared to be caused by a class 3r device . although a low - powered class 3r device has been considered relatively safe , to our knowledge , no comprehensive studies have investigated its effects on the eyes . our cases emphasize the importance of strictly regulating the production and operation of laser devices for indoor laser shows , regardless of the safety level . | here we report two cases of retinal damage after exposure to a 510 nm laser diode during a laser show .
the first patient was a 20-year - old female who presented with decreased visual acuity in her right eye after visiting a dance party with a diode laser show ( wavelength 510 nm , power 2 mw ) , although she did not directly see the light .
retinal examination revealed a sub - internal limiting membrane hemorrhage and a small laser burn .
the second patient was a 20-year - old female who visited for decreased vision in her left eye .
she described similar events as the first patient .
an exposure to green diode laser can result in retinal damage .
it is strongly recommended that certified personnel operate laser devices used in indoor laser shows under strict regulation . |
thyroid disease , especially hypothyroidism , is very common in india , especially in women . positivity to thyroid antibodies is also common in the general population , particularly in women . being part of a mini - review series , this article will not provide an extensive review of the subject , but would rather cover selected controversies of interest to the practicing endocrinologist . in general , studies have suggested that early maternal hypothyroxinemia affects the offspring subject , as the fetal brain development occurs in the first trimester , and because fetal thyroid gland develops after the 12 week . this has often put clinicians in a quandary , as patients often present with pregnancy and very high tsh values , and gynecologists are often considering termination of pregnancy in view of hypothyroidism . as there are no indian studies correlating maternal hypothyroxinemia and fetal risk , we must continue to rely on international data . from these , international research papers , it is well known that - ( a ) untreated hypothyroidism in pregnancy may lead to lowered intelligence quotient in the offspring ( b ) children born to mothers with untreated hypothyroidism had higher learning disabilities and ( c ) iodine deficiency in pregnancy can also lead to neuropsychological problems in offspring . in order to understand this , it is important to analyze the mtp act of 1971 . according to this act , mtp can be advised up to 12 weeks , and the decision can be made by one qualified doctor . in case two qualified doctors can concur , mtp can be done up to 20 weeks . from the list of qualifications to be categorized as a doctor qualified to advise or perform mtp , it is clear that being an endocrinologist does not qualify a doctor as far as mtp is concerned . mtp is generally performed to save the life of the mother , for social indications and for what is termed eugenic indications are when there is a substantial potential of a baby being born with serious mental or physical disability . these reasons include - anencephaly , chromosomal disorders down 's syndrome , genetic diseases like hemophilia and exposure to teratogens , radiation , and rubella . obviously , it is better to discuss the current state of evidence with the patient . however , some generalizations come to mind - if the pregnancy is more than 20 weeks , there is no doubt that mtp should not be done . similarly , in cases of subclinical hypothyroidism too , there is no indication for mtp . in severe cases of hypothyroidism where the conception has occurred without difficulty - the benefits and risks may be discussed with the patient - and unless there is a request from the patient , mtp can not be recommended for hypothyroidism . in the management of hypothyroidism in pregnancy it may be remembered that the baby 's intelligence quotient is not the sole overriding consideration and that there are other problems that can be a consequence of hypothyroidism in pregnancy . this includes miscarriages , pre - eclampsia , anemia , abruptio placentae , and postpartum hemorrhage . this article will assume that readers are familiar with the following aspects of managing hyperthyroidism in pregnancy - the need to treat with the minimum dose of anti - thyroid drugs to keep the tsh normal and the ft4 in the upper half of the normal range , the need to monitor maternal and fetal health and the current guidelines that suggest the use of propylthiouracil in the first trimester and carbimazole / methimazole in the rest of pregnancy , as well as the trimester specific tsh cutoffs in pregnancy . one area of ambiguity is the need for a recommendation on tsh - receptor antibody testing in pregnancy . recently , a review has suggested that international consensuses are concordant about the indications for tsh - receptor ( tshr ) antibodies in pregnancy . in a pregnant patient on anti - thyroid drugs , known to have graves disease , tshr antibodies must be measured during the 22 week . in pregnant women with a past history of graves disease treated with radio - iodine or surgery - tshr must be measured at about 22 - 28 weeks . finally , tshr may also be measured in mothers with a previous history of tshr antibody positivity , and also in mothers with previous history of neonates born with neonatal thyrotoxicosis . is there a need for fetal monitoring in thyrotoxicosis , and if so , what are the fetal ultrasound criteria to be used ? this has long been unclear and present guidelines have attempted to shed light on this clinically relevant issue . fetal ultrasound should look for fetal heart rate and fetal goiter both signaling that the anti - thyroid drugs may have crossed the placenta and also for signals of hyperthyroidism like fetal tachycardia , intrauterine growth retardation , and features like advancing bone age as well as fetal hydrops . it is best that fetal ultrasounds be done when the tshr antibodies are more than 2 - 3 folds higher than normal , and from the 18 to the 22 week of gestation . after fetal surveillance is begun , the tshr antibodies may need to be tested every 6 weeks or so . in the preceding lines , the article has looked at what happens if tshr antibodies and anti - thyroid drugs cross placenta . a third possibility is that of thyroxine crossing the placenta : this happens in cases of severe , long - standing thyrotoxicosis . this may suppress the pituitary of the fetus and thus , after birth the suppressed pituitary may take a while to recover - resulting in a period of transient - central hypothyroidism . this suggests that babies of hyperthyroid mothers need to be screened with tsh and free t4 estimations . a final issue in question has been the use of anti - thyroid drugs in lactation . this has been recently settled , with studies showing that neither methimazole 30 mg / day nor propylthouracil 300 mg per day has any detrimental effect on the breast - feeding neonate 's thyroid function . however , the drug of choice is methimazole / carbimazole - so that the liver of the baby as well as the mother may be protected from liver necrosis attributable to propylthiouracil . in general , the thyroid gland has not received the importance that it deserves - the diseases being so common and widespread . other than hyperthyroidism and hypothyroidism , other thyroid diseases also require study such as for instance thyroid nodules . clinicians are also aware of the occurrence of thyroid nodules that enlarge in pregnancy ; if the lesion is suspicious for malignancy , thyroidectomy may be considered in the 2 trimester , but if diagnosed later then surgery may be done after delivery and suppressive thyroxin treatment instituted . a third obstetric controversy is the hyperthyroidism and treatment during pregnancy as well as lactation . further research is required to establish answers to some of the controversies in the management of thyroid disease in pregnancy . | it is well known that thyroid disorders commonly affect women .
the care of pregnant women affected by thyroid disease is an important clinical challenge for endocrinologists .
hypothyroidism is the commonest problem , and maternal hypothyroxinemia has been linked to adverse feto - maternal outcomes .
this article would discuss the controversy regarding first - trimester thyroid hormone deficiency and fetal brain development .
certain obstetric controversies in the management of hyperthyroidism in pregnancy , including the indications of tsh receptor antibody measurements and fetal thyroid status monitoring would also be discussed . |
stilgoe j , owen r , macnaghten p ( 2013 ) developing a framework for responsible innovation . policy 42 : 15681580 von schomberg r ( 2013 ) a vision of responsible innovation . in : responsible innovation : managing the responsible emergence of science and innovation in society , owen r , heintz m , bessant j ( eds ) . post ( 2015 ) regulation of synthetic biology . parliamentary office of science and technology . available at : http://researchbriefings.files.parliament.uk/documents/post-pn-0497/post-pn-0497.pdf
sutcliffe h ( 2011 ) a report on responsible research and innovation . available at : http://www.marketsandmarkets.com/market-reports/synthetic-biology-market-889.html
synthetic biology project ( 2015 ) synthetic biology products and applications inventory . | scientists are often called upon to discuss their research publicly .
these discussions are harder if the research is controversial or might frighten or worry the public .
when is the right time to start a public debate and who should start the debate by highlighting potential risks and benefits ? |
mahmood and colleagues performed a retrospective analysis , published in this issue of critical care , of 261,255 adult patients admitted to intensive care units ( icus ) . the results indicate that women younger than 50 years old had lower icu mortality in comparison with age - matched men , whereas mortality rate was similar in older patients . female mortality , compared with male mortality , was increased following coronary artery bypass graft ( cabg ) surgery but decreased with chronic obstructive pulmonary disease ( copd ) exacerbation . gender - specific mortality rates were not evident for patients with acute coronary syndrome , sepsis , or trauma . numerous experimental studies report gender - specific immune and cardiovascular responses [ 2 - 4 ] . male gender was associated with suppressed immune responses and impaired cardiovascular function as opposed to maintained responses in proestrus females in experimental models of trauma - hemorrhage or sepsis ( cecal ligation and puncture ) . in diestrus mice , proestrus mice are characterized by elevated estrogen plasma levels in comparison with mice in other phases of the estrus cycle . gender - specific immune responses were reversed in aged mice [ 2 - 4 ] . in contrast , the referenced study did not demonstrate gender - specific outcome in septic and trauma patients in any age group . similarly , other clinical studies failed to consistently reproduce experimental findings ; a large cohort of 22,332 patients with blunt injury did not demonstrate gender - specific outcome . a retrospective analysis of blunt and penetrating trauma at the university of alabama ( birmingham , al , usa ) , however , showed a significantly increased mortality rate in males younger than 50 years of age . those results are supported by deitch and colleagues , who conducted a prospective cohort analysis ( n = more than 4,000 trauma patients ) that showed that , despite higher injury severity scores ( isss ) , females younger than 50 years tolerated trauma better than males did . higher proinflammatory cytokine levels appear to be responsible for diminished outcome in male victims of trauma . in burn patients , however , young females had an increased mortality rate , suggesting that different trauma mechanisms ( blunt versus burn trauma ) alter gender - specific outcome . in experimental studies , sex hormones have been shown to affect gender - specific immune responses . male sex hormones are deleterious whereas female hormones are protective [ 2 - 4,12 ] . hormonal status is not evaluated in most clinical studies . to define pre- versus furthermore , the percentage of postmenopausal females on hormone replacement therapy is not defined . according to hersh and colleagues , this should be taken into consideration when analyzing gender - specific outcomes in critically ill patients . failure to measure hormone plasma levels significantly limits most clinical studies investigating gender differences . in summary , the prevailing hormonal milieu , and not gender , different immune responses to various disease entities have been shown in experimental and clinical studies trauma and sepsis severity is known to affect pathophysiological mechanisms [ 2 - 4,12 ] . in the present study , diseases were categorized into five groups ( acute coronary syndrome , cabg surgery , sepsis , trauma , and copd exacerbation ) . within those categories severity was based on apache ( acute physiology and chronic health evaluation ) score . specific scoring systems ( that is , iss for trauma ) were not mentioned , possibly limiting the conclusiveness of the data . in clinical conditions ( in contrast to experimental conditions ) , genetic background and comorbidities vary between critically ill patients , certainly affecting outcome . in the present study , chronic health conditions ( that is , aids , cirrhosis , hepatic failure , lymphoma and leukemia , and tumors with metastases ) were registered . long - term medication as well as icu medication ( that is , heparin , cyclooxgenase inhibitors , steroids , and immunesuppressants ) may alter immune responses and should be considered potential confounders . in this study , however , only immunosuppressants were registered . most studies investigating gender - specific outcome and mortality are retrospective , involving data from large registries . despite enrolling huge numbers of patients , there is no control over comprehensiveness or detail of data recorded , and information important for gender analysis ( that is , hormone plasma levels , state of the estrus cycle , and pre- versus postmenopausal status ) is not documented . to enhance data relevance , consideration of those potential confounders would require prospective registration of gender - specific parameters . because clinical studies suggest that females require less icu treatment in comparison with males , incorporating icu patients may reflect a selection bias of females with reduced prognosis in comparison with the overall female population . it is also important to consider age and hormonal status when investigating outcome in critically ill patients . specifically designed prospective studies are required to better define the exact role of gender and sex hormones in the clinical arena . female gender itself can not be considered a protective factor in critically ill patients . continued research into potential sex hormone - based differences may close the gap between bench and bedside and ultimately promote therapeutic interventions to improve outcomes in males and females . in this respect , cabg : coronary artery bypass graft ; copd : chronic obstructive pulmonary disease ; icu : intensive care unit ; iss : injury severity score . the authors wish to thank bobbi smith for her skill and assistance in preparing this commentary . this work was funded by national institutes of health grants 5 ro1 gm037127 and 5 ro1 gm39519 . | investigators continue to debate whether gender plays any role in patient outcome following injury / critical illness .
we submit that age and hormonal milieu at the time of injury , rather than gender , are the critical factors influencing patient outcome under those conditions . |
nephrotic syndrome is a heterogeneous group of childhood disorders associated with proteinuria , hypoalbuminaemia , oedema and failure to thrive , and is usually managed by glucocorticoid treatment . between 10% and 20% of the patients fail to respond to this therapy and are diagnosed with steroid - resistant nephrotic syndrome ( srns).the prognosis for srns is usually poor , due to the increased risk of developing end - stage renal disease . defects in several genes result in srns : nphs1 , nphs2 , wt1 , actn4 and cd2ap [ 15 ] . mutations in the wt1 gene are the second most common cause of sporadic srns following defects in nphs2 . wt1 encodes a zinc - finger transcription factor with multiple isoforms , which has critical role for development of the genitourinary tract and maintenance of podocyte differentiation . dominant wt1 mutations have been implicated in a large number of disorders such as frasier , denys drash and wagr syndromes . srns alone is found to be associated with defects in exons 8 and 9 of the gene . here , we report on the case of a female srns patient with onset of the disease at 22 months of age and frequent relapses . the renal pathology in this child is due to a novel missense mutation affecting the dna - binding zinc - finger domain of wt1 . the patient was born to parents with no known family history of kidney disease , at the end of 28th gestational week , weighing 950 g with signs of respiratory distress syndrome and anaemia . g / l per 24 h ) at 22 months of age . ultrasound examination revealed hyperechogenic parenchyma . the female sex of the child was confirmed by str analysis . due to lack of response to steroid treatment , g / l per 24 h , blood pressure 115200 mmhg systolic and 70100 mmhg diastolic ) . an alternate regimen of immunosuppressant ( cyclosporine and mycophenolate ) , ace inhibitor and corticosteroid was established . at present , the child is 6 years old , with levels of proteinuria 1.11 g / l per 24 h. genetic testing for mutations in the most commonly affected genes , nphs2 and wt1 , was carried out . while no defects were found in the podocin gene , a previously unknown nucleotide substitution , c > a in position 1184 , was identified in wt1 . on the amino acid level , this results in replacement of serine with tyrosine in position 395 . the substitution was found neither in the parents of the patient nor in any of the 120 unrelated control samples from individuals with no known kidney disorder . a clustalw2 multiple species alignment of the sequence surrounding serine 395 indicated that the affected residue is conserved among organisms as diverse as xenopus , mouse , chimpanzee and human ( figure 1 ) . , ser395 is located at the first turn of the -helix in zinc - finger 3 ( zf3 ) . to investigate the effect of s395y on wt1 analysis of the predicted mutant model revealed that the tyrosine s bulky side chain would clash with dna in two of three possible rotamers , while in the third rotamer , there would be a collision with phe383 of the protein ( figure 2 ) . the -helix of wt1 zinc - finger 3 is shown as secondary structure model with the three possible rotamers of ser395 mutated to tyr ( designated as s395ya , s395yb and s395yc , respectively ) as well as the neighbouring residue phe383 . we identified a novel heterozygous nucleotide change , c1184a , in exon 9 of wt1 in a srns patient . on the protein level the absence of the mutation in both parents indicates a de novo origin . according to the data in the human gene mutation database ( hgmd ) , missense mutations are the most common cause of wt1-associated diseases . this poses difficulties in elucidating the genotype phenotype correlations since the effect of a substitution on the protein level may be difficult to predict . the absence of the respective variant in healthy individuals and the evolutionary conservation of the affected residues are often reliable indications for the pathogenic character . it was found to be absent among healthy controls and affects an evolutionarily conserved residue in a functionally important domain of the protein . however , in order to understand the correlation between genetic defect and pathology , one could use molecular models based on the crystal structure data for predicting the effect on the protein level . a study of the molecular structure of wt1 reveals the important role of serine 395 for correct folding of the zf3 and localization of the -helix in the major groove of dna . the residue is conserved in all wt1 zfs and also in the homologous domain of the zif268 protein . the structure of the wt1 complex with the 14-bp dna duplex showed that zf3 along with zf2 and zf4 makes base - specific contacts in the major groove . by introducing ser395tyr in the model , we could show that the bulky tyr side chain would either prevent the proper folding of the protein or interfere with the dna binding ; hence , the mutation would have a negative impact on the function of wt1 ( figure 2 ) . in summary , we have identified a new nucleotide change in wt1 , which results in serine to tyrosine substitution in position 395 . ser395tyr affects an important , evolutionarily conserved part of the transcription factor responsible for dna binding . molecular modelling indicated that the serine residue is essential for the proper functioning of wt1 . taken together , these results allow us to conclude that c1184a is pathological by nature . | we report the case of a paediatric patient with steroid - resistant nephrotic syndrome due to a novel dominant wilms tumour 1 mutation .
the nucleotide change c1184a , identified in exon 9 , results in amino acid substitution ser395tyr .
genotyping of parents and healthy controls indicated that this is a de novo mutation not present in healthy individuals .
the affected amino acid is evolutionarily conserved and is located in a functionally important domain of the protein involved in dna binding .
molecular modelling based on crystallography data indicated that the substitution would have a deleterious effect on the protein function . |
a study conducted in a saudi hospital assessed the opinion of nurses on self report of pain as the indicator of pain intensity revealed that nurses underestimated pain intensity in smiling patient and overestimated it in the grimacing patient ( kaki et al . ( 2000 ) conducted a survey of nurses ' decisions about assessment and treatment of pain in two patient situations and confirmed that nurses continue to under treat severe pain . as shown in previous studies , nurses may be more influenced by the patient 's behavior than the patient 's self - report of pain . surveys have shown that patient self - report of pain is often not considered as a reliable indicator of pain ( pederson 1997 , sloman et al . in a recent report , desai , chaturvedi ( 2009 ) reported that majority of the nurses considered pain behaviors and ignored the subjective component of pain when they were asked to rate the pain on two case vignettes . this study analyses nurses rating of intensity of pain after assessing two mock clinical situations using role plays . the sample was formed by the participants of series of workshop on chronic pain that are being conducted for the health professionals . a workshop was conducted for the postgraduate nursing students at national institute of mental health and neuroscience , bangalore india in november 2009 . two role plays were done by the participants on assessment of pain when the other participants were asked to assess the severity of pain . the participants were asked to observe the interview and rate the severity of pain and suffering on a visual analogue scale ( vas ) of 0 - 10 in both the cases . a 35-year - old woman has been admitted with history of pain in lower back since 1 year . you are asked to assess the severity of pain and rate the pain on vas . you are a 35-year - old woman who has been suffering from pain in her lower back since 1 year . you have been having very severe pain at times . on the day of your assessment your pain is severe and you are unable to sit comfortably , moaning in pain , holding your back with your hands . a 39-year - old man has come with history of pain the upper back since 1 year . you are asked to assess the severity of pain and rate the pain on vas . on examination vitals you are a 39-year - old man who has been suffering from pain in his upper back since 1 year . you have been having very severe pain at times . on the day of your assessment put 1 next to the rating you would record for case 1 and 2 next to the rating you would record for case 2 pain . 0 - --------1 - --------2 - -------3 - -----4 - -----5 - -----6 - -----7 - ------8 - -----9 - ------10 give reasons for your rating : how would you rate the suffering for case 1 and 2 ? put 1 next to the rating you would record for case 1 and 2 next to the rating you would record for case 2 . 0 - --------1 - --------2 - -------3 - -----4 - -----5 - -----6 - -----7 - ------8 - -----9 - ------10 give reasons for your rating : the sample consisted of 14 ( 61% ) women and 9 ( 39% ) men . in case scenario 1 , only 4 ( 17% ) participants rated the correct reported intensity of pain as 7 . in case scenario 2 also , four ( 17% ) rated the intensity of pain as 7 . the pain was underrated , more so in the case scenario 2 . in case scenario 1 : only 2 ( 8.6% ) participants received a score 2 - 3 , 15 ( 65.2% ) received a rating of 4 - 6 . in case scenario 2 : 10 ( 43.5% ) participants received a score 2 - 3 , 8 ( 34.7% ) received a rating of 4 - 6 . the mean , median , and mode for the above cases are shown in table 1 . in both the cases , mean , median , and mode of the observer rating scores of pain as compared to the patient 's rating of pain the mean for suffering for the above cases were 5.48 1.53 for case 1 and 4.30 2.12 for case 2 . in both cases the mean rating for suffering is below 7 which is the expected rating . the reasons for rating pain intensity in case scenario 1 :
patient showed grimacingappeared uncomfortablecause was clear
patient showed grimacing appeared uncomfortable the reasons for rating of pain in clinical scenario 2 :
functioning was not much affectedthe patient was not using analgesics on daily basisthe patient did not show discomfortthe patient did not express pain and feelings
functioning was not much affected the patient was not using analgesics on daily basis the patient did not show discomfort the patient did not express pain and feelings the findings illustrate that self report of pain is often not relied for rating the pain intensity by professionals . nurses relied on their observation of the patient 's behavior rather than on the self - report . the observed factors which they took into consideration for rating were facial expression , discomfort in maintaining posture during interview , interference with daily activities , use of analgesics and the cause of the pain . this study corroborates the findings of studies done in other clinical settings ( mccaffrey et al . the mean , mode , and median scores were lower for case 2 who was not showing signs of distress and was trying to distract from pain . despite pain being highly subjective experience , the self - report is not taken into account as indication of pain intensity . a recent report used the written case scenarios methods and concluded that overt behavior and emotions determined pain ratings ( desai , chaturvedi 2009 ) , and this report using clinical interview by role play method confirms that pain ratings are based on many factors and not actually by self report . this exercise was done as a part of microteaching , hence role play was used and the observers were asked to do the assessment of severity of pain along with assessment of other aspects of pain . this method for assessing observer bias in pain assessment the above findings highlight the need for increasing awareness among nurses regarding assessment of pain . health professionals and nurses should not get misled by overt behaviors and emotional distress expressed or suppressed , while assessing pain intensity , and should give credence to the subjective rating mentioned by the person in pain . gender bias in assessment of pain is a possibility that has been raised . in order to maintain anonymity of the responses the participants were asked not to mention their personal details including gender . this is to have an authentic and reliable response by the participants . it is also important to study other factors which may influence the nurses ' assessment of pain . a manual of chronic pain assessment and management describes various methods for pain measurements ( chaturvedi , desai 2009).[for a copy of the manual , please contact the author ] the study highlights that the overt behaviors of pain are given more precedence than the subjective report of the patient in assessment of pain by the nurses , which is misleading . a comprehensive pain assessment is more desirable and is likely to be accurate measure of pain . since nurses play an important role in pain assessment and management it is important to increase the awareness among them by various educational methods . | context : nurses are involved in assessment of pain in most clinical settings . pain continues to be underestimated and undertreated.materials and methods : this study assessed the rating of pain severity by nurses who observed the two case scenarios using role plays .
the data were computed and frequency distribution was derived.results:majority of the nurses underrated the pain and based their ratings on various factors.conclusions:it is important to increase the awareness among nurses about comprehensive assessment of pain for better management . |
ipsilateral injury of more than one component of the knee extensor apparatus is rare and it is mostly associated with previous trauma , surgery , immunosuppression therapy and systemic disease . we report the first documented case of a spontaneous bifocal disruption of the knee extensor apparatus ( i.e. floating patella ) associated with lymphoedema in a 54-year - old man . a 54-year - old man presented to our accident and emergency ( a&e ) department complaining of pain in his right knee . the patient has slipped off the bottom two steps of the staircase , without suffering any direct impact to his knee . his past medical history included atrial fibrillation and squamous cell penile carcinoma of the glans penis , requiring partial penectomy and urethral reconstruction , and subsequent radical right inguinal lymph node ( iln ) dissection 16 years ago ( limited to borders of the femoral triangle ) for lymphatic metastases , with clear dissections margins . he has been free from disease recurrence since , and continued to have annual surveillance follow - up . following iln dissection , patient developed right lower limb lymphoedema , with episodes of intermittent swelling of his right thigh , which became more frequent over the last 3 years . there was no family history of musculoskeletal , autoimmune disease , diabetes or other systemic disease . physical examination showed knee swelling , a high riding patella with a palpable gap below the patella . the patient was unable to weight bear and could not perform active knee extension on his right knee . apart from ectopic ossification of the patellar tendon , plain radiograph showed no evidence of acute fracture or joint effusion ( fig . 1 ) . because of the unusual mechanism of injury and the initial ultrasound performed in a&e which suggested a rupture of the quadriceps and patella tendon , magnetic resonance imaging ( mri ) was performed and demonstrated complete rupture of the patellar tendon from the inferior pole of patella , an almost complete shearing of quadriceps tendon off the patella and chronic tendinosis in the mid - portion of patellar tendon ( fig . 2 ) .
figure 1:lateral radiograph of right knee , demonstrating patella alta and ectopic ossification of the patellar tendon ( marked * ) . demonstrating rupture patellar tendon attachment from inferior pole of patella , high riding patella , chronic tendinosis of patellar tendon and clustering of sheared quadriceps tendon . lateral radiograph of right knee , demonstrating patella alta and ectopic ossification of the patellar tendon ( marked * ) . patellar tendon attachment from inferior pole of patella , high riding patella , chronic tendinosis of patellar tendon and clustering of sheared quadriceps tendon . a midline longitudinal incision was made , revealing a complete rupture of the patellar tendon from the inferior pole of the patella , torn medial and lateral retinacular fibres , and near complete shearing of 90% of the quadriceps tendon ( fig . the patellar tendon was anchored trans - osseously with 5 ticron ( covidien ) sutures , and further strengthened with 2/0 vicryl sutures using the kessler technique . postoperatively , the knee was immobilized in a long leg back slab for 2 weeks , followed by cylinder cast for 4 weeks . patient was gradually worked towards full weight bearing afterwards . at 8 weeks post - surgery , patient was able to fully extend his knee , and perform straight leg raise against gravity . his range of flexion improved with physiotherapy from 30 to 100 at 3 months following surgery . figure 3:intra - operative image demonstrating shearing of the quadriceps tendon , complete patellar tendon rupture , and injuries to medial and lateral retinaculum . intra - operative image demonstrating shearing of the quadriceps tendon , complete patellar tendon rupture , and injuries to medial and lateral retinaculum . injuries to more than one knee extensor apparatus are rarely encountered in the literature . the term bifocal injury was used in the literature to describe any two injury involving patella / patellar tendon / quadriceps tendon / avulsion of tibial tubercle . with a ruptured patellar tendon and shearing of quadriceps tendon , our patient has sustained a type 5 bifocal injury . rupture of the knee extensor apparatus commonly affects pathological tendons microtrauma in athletes , chronic corticosteroid intake , immunosuppressant therapy , prior knee surgery , and systemic disease such as renal failure , diabetes , hyperparathyroidism , systemic lupus erythematous , gout and obesity [ 25 ] . it is well known that radical iln dissection for penile cancer metastases is associated with lymphoedema of the ipsilateral leg . although lymphoedema following breast surgery has been associated with rotator cuff tendonitis / tear ; so far , the association between lymphoedema and rupture of the knee extensor apparatus has not been documented in the literature . herrera and stubblefield first described the pathophysiology of rotator cuff tendonitis secondary to lymphoedema following axillary clearance breast cancer surgery . they believed the increased size and weight on the upper limb caused by interstitial stagnation of protein - rich fluid lymphoedema subjects the rotator cuff tendons to impingement , functional overload and intrinsic tendinopathy . with vascular studies demonstrating the presence of a hypovascular zone in quadriceps tendon ( 12 cm from superior pole of patella ) frequently associated with spontaneous rupture , and the mri image in our patient showing chronic tendinosis of the patellar tendon ( fig . 2 ) , it is most likely that chronic lymphoedema was the main aetiological factor in this spontaneous non - traumatic bifocal injury of the knee extensor apparatus . this is likely secondary to the resultant chronic inflammation and fibrosis from chronic lymphoedema , which compromised perfusion and tendon / connective tissue homeostasis , therefore predisposing the retinaculum , quadriceps and patellar tendon to a risk of rupture . to the best of our knowledge , this is the first reported case of spontaneous quadriceps tendon , patellar tendon and retinaculum rupture in an adult with lymphoedema in the literature . our case highlighted the importance of considering lymphoedema as another pathoaetiological risk factor of knee extensor apparatus rupture . it also highlighted the importance of assessing all components of the extensor apparatus in patients presenting with acute knee injuries . spontaneous rupture of several components of the knee extensor apparatus can sometimes be overlooked and missed , which could lead to significant implications during operative management . | ipsilateral injury of more than one component of the knee extensor apparatus is rare .
it is mostly associated with previous trauma , surgery , immunosuppression therapy and systemic disease .
we present the first documented case of a spontaneous bifocal disruption of the knee extensor apparatus ( i.e. floating patella ) associated with lymphoedema .
this case highlights the importance of considering lymphoedema as another risk factor for rupture of the knee extensor apparatus .
it also highlights the importance of assessing all components of the knee extensor apparatus in patients presenting with acute knee injuries . |
the differential diagnoses of a groin mass include inguinal hernia , round ligament varicosities ( rlv ) , mesothelial cysts , lymphadenopathy , endometriosis , subcutaneous lipoma , cyst of nuck ( persistent embryonic remnants of the process vaginalis with cyst formation ) , vascular aneurysms , soft tissue malignancies , abscess , and cystic lymphangiomas [ 1 - 5 ] . the rlv can easily be mistaken for an inguinal hernia ( especially a strangulated or incarcerated groin hernia that needs an emergency operation ) , because they share the same clinical appearance . we report a patient with rlv during pregnancy diagnosed by doppler sonography and managed with conservative therapy ( repeated sonography and obstetric practice ) . a 29-year - old woman presented at 36 weeks gestation with a palpable mass and pain in the right groin during her second pregnancy . the opinion of the obstetrician was inguinal hernia , and he transferred her to a general surgeon . the general surgeon diagnosed a right inguinal hernia , and he transferred her to our hospital . on physical examination , the mass ( 2 2 cm ) was palpable softly , but tender , and was reducible completely by supine position change ( fig . gray - scale sonography showed a mass in the right groin composed of multiple , echo - free , tubular channels . the color doppler sonography confirmed hypervascular and abundant venous flow consistent of a mass ( fig . 2 ) . there was no sonographic evidence of a herniated bowel , or thrombus , or adenopathy . based on sonographic results , we diagnosed round ligament varicosities , and performed repeated sonography and obstetric practice . the patient had an uncomplicated vaginal delivery at 40 weeks , and by two weeks postpartum , the mass subsided . the most common diagnosis of a female groin swelling during pregnancy is an inguinal hernia . however , inguinal hernias during pregnancy are relatively rare , with a reported incidence of 1 in 1,000 to 3,000 pregnant women . a thorough literature search revealed only 16 cases internationally of rlv during pregnancy reported in the last 53 years . the rlv was first reported in 1941 by verovitz and first reported in korea in 2010 by jung et al . . the rlv are prominent veins within the round ligament and are more common in pregnancy because pregnancy promotes increased venous return and reduced venous tone . the varicose vein formation of the round ligament during pregnancy have several mechanisms , including progesterone - mediated venous smooth muscle relaxation , a raised cardiac output causing increased venous return from the limbs and leading to engorgement of venous tributaries , and most importantly , pelvic venous obstruction by the gravid uterus [ 1 - 3,5 ] . the clinical manifestation of rlv usually includes swelling and tenderness in the groin region , which can be provoked by increased intra - abdominal pressure in cases of coughing or valsalva maneuver . a clue that might suggest rlv is the coexistence of vulvar or lower limb varicosities . our case , however , absence of vulvar or lower limb varicosities did not exclude rlv . the characteristic sonographic findings include a prominent venous plexus with accompanying dilated draining veins and the typical " bag of worms " appearance of smaller veins . color doppler imaging can confirm venous flow and augmentation of this flow with valsalva maneuver . according to mckenna et al . , ultrasound criteria that may be used in the diagnosis of rlv includes : multiple dilated veins passing through the inguinal canal , absence of bowel or lymph nodes in the inguinal mass , and veins seen to drain into the inferior epigastric vein . the differential diagnoses of rlv include inguinal hernia , lymphadenopathy , endometriosis , subcutaneous lipoma [ 1 - 5 ] . the sonographic findings of inguinal hernia are a superficial , well - delineated echogenic mass that appears adjacent to , yet distinctly separate from , the uterine wall . the sonographic findings of lymphadenopathy are hypoechoic round or oval masses of varying size in the region of the inguinal area . the characteristic sonographic appearance of endometrioma is that of a well - defined unilocular or multilocular , predominately cystic mass containing diffuse homogeneous , low - level , and internal echoes . the sonographic findings of lipoma show a homogeneous , and echogenic mass that is surrounded by a thin echogenic capsule . after a correct diagnosis of rlv , a wait - and - see policy is justified . after delivery , when pelvic venous obstruction by the gravid uterus is relieved , spontaneous resolution will occur in most patients [ 2 - 5,9 ] . however , rlv requires close monitoring during pregnancy as rupture of varices and acute variceal thromboses have been reported . rupture and thrombosis of the rlv may occur and give an intense painful swelling groin , in which case emergency surgical exploration is recommended . this recommendation comes first of all to rule out a strangulated hernia , and secondly to reduce pain or discomfort caused by the inguinal mass . when a pregnant patient with an inguinal mass visits our hospital , we have to know that rlv is a part of the differential diagnosis of an inguinal mass during pregnancy , and we must perform a diagnosis using duplex sonography . if the rlv is diagnosed by sonography with the absence of a herniated bowel , or thrombus , we manage it conservatively ( repeated sonography and obstetric practice ) . however , if rlv presenting with rupture of varices and variceal thromboses are observed , emergency surgical exploration is recommended . | round ligament varicosities during pregnancy are rare , and can easily be mistaken for an inguinal hernia . on physical examination ,
round ligament varicosities and groin hernia are difficult to distinguish .
the diagnosis of round ligament varicosities can be established on gray - scale and color doppler sonography .
we experienced a case of round ligament varicosities in which a 29-year - old woman presented symptoms at 36 weeks gestation .
the patient was diagnosed using doppler sonography , managed with conservative therapy , and had an uncomplicated vaginal delivery at 40 weeks .
the symptoms were resolved completely by two weeks postpartum .
we report a case of round ligament varicosities that was diagnosed at 36 weeks of gestation with a review of the literature . |
a 44-year - old male was visited for back pain and mass around left buttock . he had a medical history that included a diagnosis of pulmonary tuberculosis , from which he had recovered completely 20 years previous . he was under medication from a local clinic and pharmacy for 1 month prior to visiting the hospital due to cold symptoms and lower back pain . vital signs on visit were blood pressure at 130/80 mmhg , heart rate at 72 beats / minute , respiratory rate at 18/minute and body temperature was 38.3. the mass was painful and palpated on the left buttock area , and is seen as a reddish skin color around the anterior superior iliac spine ( fig . laboratory investigations on admission were the following : white blood cell count was 16,660/mm , hemoglobin was 10.5 g / dl , platelet count was 631,000/mm , c - reactive protein was 21.9 mg / dl , and erythrocyte sedimentation rate was 120 mm / hour . abdominal computed tomography ( ct ) and magnetic resonance imaging ( mri ) showed a large psoas abscess in retroperitoneal space and abscess on left lower quadrant anterior abdominal wall ( fig . 2 ) . based on a diagnosis of psoas abscess , the patient was given ciprofloxacin and metronidazole as an empirical injection , then an incision and drainage was performed . ciprofloxacin and metronidazole were injected for an additional 7 days after this procedure , however streptococcus agalactiae and streptococcus aginosus were cultured . a skin defect , 4 3 cm in size , developed around left anterior superior iliac spine . ten days post discharge , the patient experienced fecal discharge from the local flap site ( fig . the patient underwent an operation for enterocutaneous fistula , which was performed by the department of general surgery . during the surgical procedure , a tumor mass was found in the descending colon and was adherent to the retroperitoneum . the tumor had ruptured spontaneously and formed enterocutaneous fistula track , but there were no peritoneal seeding . the histopathologic results of the fistula track and capsule of abscess were granulation tissue with acute suppurative inflammation without tumor cell . reported cases of psoas abscesses have been attributed to tuberculous diseases of the spine , infective spondylitis , crohn 's disease , and diverticulitis.3 ) the incidence of perforated colon cancer ranges from 3% to 10%.4 ) development of intra - abdominal abscess from spontaneous rupture of colon cancer is very rare , having an incidence of 0.3 - 0.4%.1,2 ) the psoas muscle is a retroperitoneal structure lying outside the endoabdominal fascia , such that it is protected from intra - abdominal muscle.5 ) similar to this case , a psoas abscess from colon cancer usually has been preceded by perforation of cancer and commonly with fistula formation . abdominal ct is useful in diagnosis and mri helps in judgment of abscess location , determination of lesion boundaries , and the spread to surrounding soft tissue . in this case , the patient was relieved of symptoms from the psoas abscess by routine incision , drainage and antibiotic therapy in this case . however , the wound culture from the operation was positive for s. agalactiae and s. aginosus which are unusual findings from this location . this led the authors to doubts about the original diagnosis of an uncomplicated psoas abscess and prompted the taking of abdominal ct and mri . we discovered that the patient 's intra - abdomen was connected with retroperitoneum and fecal discharge , which were observed on the local flap . during the operation under enterocutaneous fistular impression , we found a ruptured tumor mass in the descending colon , which had adhered and connected to the retroperitoneum . the case report concerned a thigh abscess with perforated colon cancer ; the route by which the abscess spread from abdominal sources were by 2 means in this case : one was the direct soft tissue extension of infection from the extraperitoneal portion of colorectum ; the second was the extension of infection into the other site via naturally occurring defects in the abdominal wall.6 ) as previously described , perforated colon cancer is rare . fistular formation is uncommon and occurs only in approximately 15% of all perforated colon cancers.7 ) in this case , abscess and fistula of perforated colon cancer were coexistent . it must be recognized that ruptured colon cancer can be a rare cause of psoas abscess . if the result of wound culture is an uncommon strain or normal flora of bowel and the patients have continuous wound problem , surgeons should consider spontaneous rupture of colon cancer as part of the differential diagnosis in these cases . | spontaneous rupture of colon cancer , combined with psoas abscess formation , is rare .
a 44-year - old male visited for back pain and left buttock mass .
abdominal computed tomography and magnetic resonance image revealed a large abscess in the left psoas muscle and in the left lower quadrant area .
ten days after incision and drainage , a skin defect around the left anterior superior iliac spine remained .
a local flap was performed using a superficial skin graft .
ten days after the stitches had been removed , fecal discharge was observed around the anterior superior iliac spine at the flap site .
an operation was performed by a general surgeon who had diagnosed this as a case of enterocutaneous fistula .
operative findings included a ruptured tumor mass in the descending colon , which was connected to a retroperitoneal abscess .
pathologic report findings determined adenocarcinoma of the resected colon .
herein , we report a case of psoas abscess resulting from perforating colon cancer . |
dental anomalies such as alterations in shape , size , position , color , or texture may have a negative influence on the harmony of smile . even though both prenatal and postnatal period are blamed for anomalies in tooth dimensions , position , and number ; events in the prenatal period has the most influence on these defects . peg - shaped laterals are dental anomalies that are likely to be connected to defects in certain genes . there is a strong component of heredity , and peg - shaped lateral incisors have been linked genetically with tooth agencies . a peg - shaped incisor has a marked reduction in diameter , extending from the cervical region to the incisal edge . although the prevalence of unilateral and bilateral lateral incisors are the same , the left side of arch is twice as common as the right side . peg shaped mandibular incisors are mostly associated with syndromes [ table 1 ] . very few case reports have mentioned peg - shaped central or lateral incisors in the mandible . this case report will help clinicians to look at a different possibility of occurance of peg - shaped tooth in a non syndromic condition and will help to explore more . we present a very rare case report of nonsyndromic peg shaped mandibular incisors and maxillary lateral incisors in an individual . a healthy 11-year - old boy reported to our dental clinic with a complaint of his pointy shaped front tooth . ethical clearance was taken from a local review board of indian dental association and informed consent was taken from the parent especially mentioning for the publication purpose . his past medical history was not contributory , and family history revealed that his maternal uncle had congenitally small sharp teeth . the left permanent canine is partially erupted hence there is no confirmation of that tooth being peg shaped . in the maxilla , permanent lateral incisors are peg shaped ; central incisors were of normal shape with mamelons . based on these findings , it was diagnosed as nonsyndromic bilateral microdontic peg - shaped mandibular incisors [ figures 1 and 2 ] . peg - shaped maxillary incisors peg - shaped mandibular incisors permission from his parents for orthopantomogram ( opg ) to be taken for further diagnosis and given the treatment options as composite restorations to improve the esthetics of the tooth . parents advised that a periodic check up every 6 months to follow - up the eruption and fate of the canine and other permanent teeth should be scheduled . ethical clearance was taken from a local review board of indian dental association and also informed consent was taken from the parent to publish this rare case . anomalies of tooth number , structure , and morphology can occur within human dentition secondary to genetic and epigenetic influences . koch et al . defined tooth size as abnormal , when dimensions deviate two standard deviations from average . microdontia is used to describe teeth which are smaller than normal , i.e. outside the usual limits of variation . the deciduous dentition appears to be affected more by maternal intrauterine influences ; while the permanent teeth seem to be more affected by the environment . one of the most common forms of localized microdontia is that which affects the lateral maxillary incisors , called a peg lateral . peg - shaped teeth are anterior teeth in the primary or permanent dentition with a crown diameter that decreases markedly from cervical margin to incisal edge , thus resulting in the characteristic peg shape . the endocrine disturbances may affect the size / form of the crown of teeth in utero or in the first year of life . size and shape of the root , however , may be altered by disturbances in later periods . disturbances in morphodifferentiation may affect the form and size of the tooth without impairing the function of the tooth and the function of ameloblasts / odontoblasts , thus , the result may be a peg - shaped / malformed tooth with enamel and dentine that may be normal in structure . documentation of discrepancies helps in assessing the evolutionary structural and morphological changes in human dentition . the reports of peg - shaped laterals in the maxilla is more prevalent than any other tooth as 1.8% of incidence is seen and 2.15% in the turkish population , 4.3% in the iranian population , and 2.58% in the indian population . many studies report maxillary teeth being peg shaped yet only five cases report that the mandibular tooth is affected . four studies report mandibular central incisor being peg shaped and one study reports the mandibular lateral incisor being affected . the treatment aspect of these peg shaped tooth is always esthetic and orthodontic corrected [ table 2 ] . treatment options for peg shaped laterals the present case report highlights the alteration of the shape of mandibular incisors , which can affect the psychological well - being of the patient if not treated at an early stage . since dental esthetics is known to affect the overall quality of life , it is important that a multidisciplinary approach is adopted in the treatment of patients with tooth deformity . | microdontia is a term used to describe teeth which are smaller than normal .
peg - shaped teeth are type of microdontia , a developmental disturbances of teeth mainly due to congenital reasons . most commonly affecting single teeth , that is maxillary lateral incisors .
incidence of peg shaped tooth in maxillary laterals are usually seen and reported .
but in mandibular arch it is very rare .
present case , it affected all the incisors of mandibular arch and also the laterals of maxillary arch which is rarest of condition .
this case report presents a non syndromic , peg shaped mandibular incisors in a 11 year old male patient . |
clinically evident renal medullary fibroma is a rare occurrence . these lesions are usually small and escape clinical detection . benign fibromatous tumors have also been described to affect other urogenital sites such as testis and paratesticular soft - tissues . it is difficult to distinguish benign renal medullary fibroma from renal malignancy pre - operatively and most of these clinical scenarios end up in radical nephrectomy . a 32-year - old male patient presented with left flank pain and a single episode of gross hematuria . the radiologic features suggested renal carcinoma ; however , after surgery and histopathology investigations the lesion was identified as renal fibroma . computed tomography ( ct ) scan of the abdomen revealed a calcified , partially exophytic soft - tissue mass involving the mid and interpolar regions of the right kidney [ figure 1 ] . contrast enhanced ct revealed doubtful enhancement with perirenal mild inflammatory stranding [ figure 2 ] . right renal cell carcinoma ( rcc ) was suspected and patient underwent laparoscopic right radical nephrectomy . 32-year - old male with left flank pain and gross hematuria diagnosed with renal medullary fibroma . unenhanced computed tomography scan of the abdomen reveals a calcified , partially exophytic soft - tissue mass ( arrow ) involving the mid and interpolar regions of the right kidney . 32-year - old male with left flank pain and gross hematuria diagnosed with renal medullary fibroma . contrast enhanced computed tomography reveals doubtful enhancement around the calcified partially exophytic mass with perirenal mild inflammatory stranding ( arrow ) . on cut section , the right kidney contained a well - circumscribed 4 cm 4 cm , grayish - yellow tumor with areas of calcification [ figure 3 ] . 32-year - old male with left flank pain and gross hematuria diagnosed with renal medullary fibroma . gross photograph shows a firm grayish yellow calcified tumor ( black arrow ) , renal hilum ( white arrow ) . histological analysis revealed an unencapsulated , paucicellular tumor with large areas of hyalinization , fibrosis and variable infiltrate consisting of lymphocytes and plasma cells . scattered among the sclerotic stroma a few spindle cells in myxoid background with prominent nucleoli were observed . thick collagen bundles with psammomatous and dystrophic calcification with osteoid formation were also noted [ figures 4 and 5 ] . 32-year - old male with left flank pain and gross hematuria diagnosed with renal medullary fibroma . photomicrograph of hematoxylin and eosin , stained tissue ( 100 ) shows bland spindle - shaped cells occurring singly within a loose stromal matrix ( black arrow ) and separated by thick collagen bundles ( white arrow ) . 32-year - old male with left flank pain and gross hematuria diagnosed with renal medullary fibroma . photomicrograph of h and e stained tissue ( 100 ) shows foci of thick collagen bundles with calcification ( white arrow ) and osseous metaplasia ( black arrow ) . convalescence was uneventful and the patient was discharged on the 3 day post - operation . renomedullary fibromas are very common benign tumors arising from the interstitial cells of the renal medulla and commonly present as an incidental finding . the lesion localizes in the renal medulla and almost always measures less than 0.5 cm in diameter although tumors up to 8 cm have been reported . they are unencapsulated , but well - defined nodules that are usually solitary , but may sometime be multiple and bilateral . few renal medullary fibromas are large enough to become clinically evident and cause symptoms due to torsion of the tumor about a pedicle causing venous stasis , infarction , vascular rupture , and subsequent hematuria . pre - operative diagnosis of renal medullary fibroma is difficult and the radiological spectrum of calcifications on plain x - rays , echogenicity on ct , neovascularity on arteriogram and filling defects on retrograde pyelogram , all simulate either renal cell carcinoma ( rcc or transitional cell carcinoma . on ultrasonography , on ct scan , a predominantly soft - tissue attenuation tumor with lack of hemorrhage and necrosis should draw attention toward this differential diagnosis . osteosarcoma of the kidney is a rare tumor , which generally presents as a large mass with dense chunky calcification and may even replace whole of the kidney . xantho - granulomatous pyelonephritis also present as a heterogenous non - enhancing mass and a staghorn calculus is present in 80% of the cases . the kidney is enlarged with paradoxically contracted renal pelvis with dilated calyces giving the appearance of the paw - print of a bear ( bear paw sign ) . magnetic resonance imaging ( mri ) renal medullary fibroma shows low signal intensity in both t1- and t2-weighted images presumably because of their dense collagen content and little cellularity malignant fibrous histiocytoma is yet another densely collagenous , but malignant tumor , which shows low intensity in t2 images . rcc on the other hand have an intensity intermediate between renal cortex and medulla on t1-weighted image and are isointense or hyperintense relative to normal parenchyma on t2-weighted image . because it is unusual for rcc to have low signal intensity on both t1- and t2-weighted images , renal medullary fibroma should be considered in the differential diagnosis . hematomas can also can have low signal intensity on both t1- and t2-weighted images especially when their hemosiderin content is substantial . inflammatory pseudotumor is another rare lesion , which presents as hypointense lesion on t1- and t2-weighted images due to fibrotic changes and shows delayed gadolinium enhancement due to the accumulation of extravascular contrast media in the fibrotic component of the mass . contrast enhanced ct picture is highly variable and may show homo- or heterogeneity and hypo- , iso- , or hyperdensity . renal leiomyoma may be considered in the differential diagnosis since renal leiomyoma manifests with homogenous soft - tissue density on ct and low signal intensity on t1- and t2-weighted images . these patients usually undergo radical nephrectomy as malignancy can not be ruled out with certainty . however , these entities are entirely benign and patients have an excellent prognosis and thus there may be a role for biopsy if this lesion is suspected radiologically . role of positron emission tomography ( pet ) scan in renal masses is yet not clearly defined . in general , benign tumors are photopenic and malignant tumors shows fluorodeoxyglucose avidity , but exception has been reported . mri and pet scan evaluation of these clinical scenarios can prevent unnecessary removal of normal renal parenchymal tissue . prudent use of the available modalities of imaging can help us in deciding to perform nephron sparing surgeries in these cases . radiologists and clinicians need to be aware of the possibility of these kinds of lesions that warrant further evaluation . medullary fibroma is a very frequent small tumor that most commonly presents as an incidental finding , but in rare cases maybe large and symptomatic . these patients usually undergo radical nephrectomy as malignancy can not be ruled out with certainty . however , these entities are entirely benign and patients have an excellent prognosis and thus there may be a role for biopsy if this lesion is suspected radiologically with available modalities including mri . | renomedullary interstitial cell tumor or medullary fibroma , is a small tumor that commonly presents as an incidental finding , but in rare cases maybe large and symptomatic .
although it is a benign tumor , it is difficult to differentiate this lesion from other malignancies of the kidney on radiological basis and hence many patients undergo radical nephrectomy .
we present a case of renal medullary fibroma and various nuances associated with radiological identification of this lesion and its management related dilemmas . |
there is a specific hierarchy of laboratory services in minisrry of health ( moh ) , iran .although there are 31 provinces in iran , moh has divided the country to 49 regions each of which is under supervision of its dedicated medical university . there is an office of laboratory affairs in each university that is responsible for supervising the laboratories in that province . there are nearly 5000 laboratories throughout the country in different public and private sectors with different range of activities and capabilities and different levels of quality performance . each of them had their own policy to maintain and improve their quality ( 1 ) . moreover , there are fundamental differences in domestic situation and limitations in different provinces of iran ( 2 ) . after four years of publishing the national standard , it was necessary to evaluate the achievements and failures of implementation of standards throughout the country , as well as analyzing the gaps and planning the interventional activities to resolve the problems ( 3 ) . in this article , the challenges of establishment of the laboratory standard in iran will be discussed and strategy of reference health laboratory ( rhl ) to overcome the obstacles will be presented . assessing the achievements and failures of the laboratories , all around the country , in implementing standard requirements carried out through several different methods . the most effective source of data gathering was analysis of the results obtained by benchmarking programs . this program has been the best opportunity to assess the challenges of implementation of laboratory standards in different provinces ( 4 ) , in this program the laboratory auditors from all around the country gather together in one province to inspect almost all of the medical laboratories there . the final report is sent out to the dean and deputies of the host university in order to take appropriate actions . another important means of investigating the condition at district levels is random inspection of performance of the universities ( 3).to do so , expert teams from reference health laboratory carry out on - site evaluation to monitor the tasks that the universities have been required to perform . the cumulative analysis of the laboratory inspection 's reports by office of the laboratory affairs provides valuable information about the performance of laboratories over a period of time in different provinces . the external quality assessment schemes ( eqas ) was extended in terms of covering parameters and the frequency of holding the program . analyzed information of this countrywide program is utilized for policy - making in different fields such as quality of laboratory equipment and assessing training needs , a thorough evaluation revealed that the progress of implementation process varies considerably in different provinces . probably the most important and affective factor is intent and willingness of cooperation in the offices of laboratory affairs in respective universities . the responsibility of establishment of standards in each province is laid on the dedicated university , which supervises the laboratories there . according to local situation each university must have its own schedule for implementation and certainly it should be stepwise , flexible , practical and feasible within specified time frames . it is shown where the university authorities desire to cooperate in establishing the standard , they usually succeed to remove the barriers and proceed . financial problems and shortage of resources such as personnel , equipment , supplies and facilities are highly prominent obstacles in implementing laboratory standard . in many cases the problem is not the lack of resources but the allocation or distribution of resources and budgets make serious troubles . taking appropriate policies for resource management could resolve many problems . diversity of laboratories as well as laboratories in many different public and private sectors and at different level of quality development make it nearly impossible to set a same plan or follow a same approach for implementing the standards in all these heterogeneous laboratories throughout the country . even the best laid national plan may go awry ; there would be many different plans and approaches according to domestic situation in each province and laboratories in different sectors ( 6 ) . the universities and the subsidiary laboratories should be considered free to choose where to start and how to move forward . insufficiency of academic training in laboratory sciences has resulted in necessity of holding pervasive and comprehensive post - graduate training courses for laboratory personnel . adjoining the principles of quality management system in academic curriculum of laboratory sciences could be mostly helpful . until then it is necessary to fix an organized , training courses with pre - determined practical topics ( 6 ) . cascading training programs were held throughout the country , although evaluating the effectiveness of these training courses is very difficult and challenging . it seems necessary to provide specific technical guidelines , clarifying the required technical details in some processes such as specimen management , waste management and preventive maintenance of laboratory equipment ( 7 ) . shortage of human resources and inadequate number of competent auditors in many provinces led to ineffective tracking of the implementation process in the laboratories , there should be sufficient number of laboratory auditors to monitor accomplishments and failures of the laboratories in this process . it definitely requires arrangements to get the trained and qualified auditors to workforce ( 2 ) . reviewing the overall results of external quality assessment schemes in the country give us a valuable idea about status quo . evaluating the eqas results revealed that an important challenge we encountered is related to laboratory equipment . to develop a national controlling system to manage the laboratory equipment in terms of quality and accessibility is really crucial ( 8) and it is one of the rhl priorities . the last but not the least barrier to implement the laboratory standard is cultural problems and resistance to change that make it difficult to enforce a new decipline ( 2 ) . it takes time and needs to rationalize the necessity of establishing laboratory standards , and bring up the subject repeatedly in related seminars and forums . naturally enforcement of a new regulation may come up against certain barriers and reluctant reactions . establishment of national iranian laboratory standard was confronted some difficulties that reduce the pace of implementation process , but the problems are potentially resolvable through revision of the strategies and rearrangement of resources . full cooperation of universities ' authorities plays a key role , especially in designing a stepwise plan to implement the standard in laboratories in different sectors in a timely manner , concerning the local situation in their province , as well as efficient resource management could solve many problems . revising academic curriculums and organized post graduate training courses improves the knowledge of laboratory staff , as the most common cause of laboratory errors . providing supplementary technical guidelines is also beneficial . to improve the quality of laboratory equipments is crucial for quality performance of laboratories , upgrading the related regulation is important . in order to trace the implementation process in the laboratories , there should be sufficient number of auditors and formation of laboratory auditor pool is necessary . finally the most challenging part of laboratory standardization is to overcome the cultural problems and reluctance to change . ethical issues ( including plagiarism , informed consent , misconduct , data fabrication and/or falsification , double publication and/or submission , redundancy , etc ) have been completely observed by the authors . | after four years of publishing the iranian national laboratory standard and following a strategic plan to implement its requirements , it was decided to review the taken actions , evaluating the achievements and the failures , as well as analyzing the gaps and planning the interventional activities to resolve the problems .
a thorough evaluation revealed that the progress of implementation process varies considerably in different provinces , as well as in laboratories in different public and private sectors .
diversity and heterogeneousity of laboratories throughout the country is one of unresolvable problems .
although we encounter shortage of resources in the country , improper allocation or distribution of resources and budgets make the problems more complicated .
inadequacy of academic training in laboratory sciences has resulted in necessity of holding comprehensive post - graduate training courses .
revising academic curriculum of laboratory sciences could be mostly helpful , moreover there should be organized , training courses with pre - determined practical topics . providing specific technical guidelines , to clarify the required technical details could temporarily fill the training gaps of laboratory staff .
inadequate number of competent auditors was one of the difficulties in universities .
another important challenge returns to laboratory equipment , developing the national controlling system to manage the laboratory equipment in terms of quality and accessibility has been planned in rhl . at last cultural problems and resistance to change are main obstacles that have reduced the pace of standardization , it needs to rationalize the necessity of establishing laboratory standards for all stakeholders . |
laparoscopic cholecystectomy ( lc ) , as compared to open procedure , has higher incidence of biliary and vascular injuries ( 0.31.0% ) . it is still rare for such lesions to bleed into lower gastrointestinal ( gi ) tract and present as haematochezia . a 45-year - old male patient presented with pain in right upper quadrant of abdomen . ultrasound of abdomen showed cholelithiasis and choledocholithiasis for which patient underwent ercp and stenting with stone extraction . intraoperative course was uneventful except for minor bleeding at calot 's triangle which was successfully cauterized . after 15 days of discharge , patient presented in the emergency with giddiness and history of haematochezia since 2 days . on examination , he was hypotensive and pale . ct angiography suggested active bleeding from a pseudoaneurysm into sub - hepatic space [ figure 1 ] . triple - vessel conventional angiography revealed pseudoaneurysm of right hepatic artery [ figure 2 ] . however , embolisation was not successful due to partial thrombosis at celiac artery origin . contrast extravasation into right sub - hepatic space right hepatic artery pseudoaneurysm on conventional angiography hence , an emergency laparotomy was contemplated . an actively bleeding right hepatic artery pseudoaneurysm and its fistulous communication with hepatic flexure of the colon were found [ figure 3 ] . the bleeding aneurysmal vessel was ligated . in view of poor general condition and intraoperative hemodynamic instability , the site of fistulous communication on colon was freshened and brought out as loop colostomy . patient was discharged on postoperative day 10 and is doing well on follow - up . ( a ) ligated right hepatic artery pseudoaneurysm and ( b ) opening in hepatic flexure of colon vascular injuries occur in upto 0.8% of cases following lc . in the presence of a bile duct injury upto 25% will have a concomitant vascular injury . most of the vascular injuries present as intraoperative bleeding . while most of vascular injuries present as intraoperative bleeding , rarely minor injury to the vessel may go unrecognised and present later as a pseudoaneurysm . possible mechanisms of vascular injury during lc could be direct injury due to dissection , use of diathermy leading to direct trauma or accentuated conduction through the surgical clips ( as may have occurred in our case ) , vascular erosions due to encroachment by metal clips or sepsis and inflammation secondary to biliary injury and bile leak causing damage to the vascular wall . common sites of injury are right hepatic artery ( 61% cases ) , common hepatic and cystic artery . these kinds of injuries can be avoided by taking simple precautions like careful usage of diathermy near the metal clips in the cystic duct or cystic artery and staying close to the gallbladder wall during dissection of the calot 's triangle . symptoms may appear in the early postoperative period or as late as 120 days after operation . common presentations are right upper quadrant pain , jaundice , altered liver function tests and rupture with internal haemorrhage . the risk of rupture is high about 80% with associated mortality rate of upto 50% . common sites of bleeding are biliary and upper gi tract or peritoneum leading to hypovolemic shock , gi haemorrhage , biliary colic , haemobilia or obstructive jaundice . pseudoaneurysm bleeding in lower gi tract presenting as haematochezia , as in our case is extremely rare . rupture of a pseudoaneurysm into the peritoneal cavity may present as acute cardiovascular collapse or be temporally contained by surrounding tissue , which is often called double rupture phenomenon. a high index of suspicion is necessary for diagnosis in patients presenting with gi haemorrhage following lc . ct scan of abdomen with ct angiography may aid diagnosis by demonstrating haemorrhage or pseudoaneurysm . however , most reliable diagnostic test is triple - vessel conventional angiography . surgery is indicated if there is failure of angioembolisation , hemodynamic instability , compression of bile duct or fistula . several surgical options such as primary repair , patch angioplasty , saphenous vein or graft interposition are available . however , surgeon might decide to ligate the artery if repair is not feasible as in our case . surgical ligation carries a 2029% complication rate and a mortality rate of upto 50% in unstable patients . review of literature showed that in 82% cases embolization was successful , while 18% required surgery . to conclude , hepatic artery pseudoaneurysm after lc is a rare but recognized and potentially fatal complication . hence , main concern is the prevention of the iatrogenic injury . a high index of suspicion is warranted in patients who present with lower gi bleeding following lc . | pseudoaneurysm of hepatic artery is a rare but known complication of laparoscopic cholecystectomy ( lc ) .
such pseudoaneurysms may bleed in biliary tree , upper gastrointestinal ( gi ) tract or peritoneal cavity leading to life - threatening internal haemorrhage .
it is very rare for them to present as lower gi bleeding .
we report an unusual case of right hepatic artery pseudoaneurysm developed following lc , which ruptured into hepatic flexure of colon resulting in catastrophic lower gi bleeding .
this was associated with partial celiac artery occlusion due to thrombosis . due to failure of therapeutic embolisation
, the patient was subjected to exploratory laparotomy to control haemorrhage .
postoperatively , patient recovered well and was discharged on postoperative day 10 .
a strong index of suspicion is necessary for early diagnosis of such condition and to limit resultant morbidity .
angioembolisation is the first - line treatment and surgery is indicated in selected cases . |
a 61-year - old male presented to the emergency room with complaints of fatigue , dizziness and bright red blood per rectum ( brbpr ) for two days . on presentation , his vital signs were unstable ( heart rate : 110 bpm ; blood pressure : 90/64 mmhg ) , with a hemoglobin level of 9 g / dl ( baseline 1011 g / dl ) . his blood pressure responded appropriately to fluid resuscitation with a 2 l normal saline bolus . past medical history was significant for gastro - esophageal reflux disease , non - steroidal anti - inflammatory drug ( nsaid ) induced ulcer , and end - stage renal disease ( gfr < 30 ) status following two failed renal grafts . pertinent medications include pantoprazole and sodium polystyrene sulfonate in sorbitol ( kayexalate 30 g orally ) . on esophago - gastroduodenoscopy ( egd ) , there was a single shallow , flat , non - bleeding gastric ulcer ( 3 mm ) in the pre - pyloric region of the stomach , with no stigmata of bleeding . however , the patient continued to have intermittent episodes of brbpr , necessitating multiple blood transfusions during his hospitalization . subsequently , a colonoscopy was performed showing evidence of colitis and localized ulcerations in the cecum , which were biopsied . histopathology revealed basophilic , non - polarizable , rhomboid - like crystals without evidence of necrosis ( figure 1 ) . kayexalate is a cation - exchange resin that acts in the large intestine by exchanging sodium ions for potassium ions . it is used in the treatment of hyperkalemia and can be administered orally or as an enema . the mechanism of mucosal damage is not clear , and reported to be more common in patients with uremia . it is speculated that it is caused by its osmotic action and vasospasm of the intestinal vasculature . previous studies have suggested that kayexalate in sorbitol may be associated with intestinal necrosis and inflammation in uremic patients [ 2 , 3 ] . abraham and colleagues focused on endoscopic and histological findings in patients with kayexalate crystals from upper gastrointestinal biopsies . kayexalate - induced intestinal injury reveals rhomboid or triangular basophilic crystals adherent to the surface epithelium . our study highlights kayexalate as a cause of lower gastrointestinal mucosal injury and ulceration with no evidence of necrosis , as confirmed histologically by evidence of kayexalate crystalline resin in a patient with end - stage renal disease . although kayexalate - induced colonic injury is rare , recognition of kayexalate crystals as a marker can be instrumental in providing the correct diagnosis and should be considered in patients with end - stage renal failure with mucosal injury . | a 61-year - old male presents to the emergency room with complaints of fatigue , dizziness and bright red blood per rectum ( brbpr ) for 2 days .
past medical history was significant for gastroesophageal reflux disease , non - steroidal anti - inflammatory drug ( nsaid ) induced ulcer , and end - stage renal disease ( gfr < 30 ) status post 2 failed renal grafts .
pertinent medications include pantoprazole and sodium polystyrene sulfonate in sorbitol ( kayexalate 30 g / d orally ) .
on esophagogastroduodenoscopy ( egd ) there was a single shallow , flat , non - bleeding gastric ulcer ( 3 mm ) in the pre - pyloric region of the stomach with no stigmata of bleeding .
a colonoscopy was performed showing evidence of colitis and localized ulcerations in the cecum which were biopsied .
histopathology revealed basophilic , nonpolarizable , rhomboid - like crystals without evidence of necrosis . |
we analyzed 82 type and other culture collection strains encompassing all validly described staphylococcal species ( n = 38 ) and subspecies ( n = 21 ; according to the current list of bacterial names with standing in nomenclature , updated may 14 , 2005 ) ( 7 ) . two strains of the recently proposed candidate species s. pettenkoferi ( 8) were added to complete the rpob sequence reference database . using this database , we analyzed 55 clinical staphylococcal isolates collected from human ( n = 52 ) and animal ( s. intermedius , n = 2 ; s. felis , n = 1 ) specimens ; 6 of the human isolates exhibited the small - colony variant ( scv ) phenotype . this strain collection was previously analyzed by the api i d 32 staph and vitek 2 systems ( both obtained from biomrieux , marcy l'etoile , france ) , partial 16s rdna sequencing , chemotaxonomy , and riboprinting to determine species designation ( 3 ) . the thermal cycling condition to amplify the partial rpob gene ( 899 bp ) was 35 cycles of denaturation at 94c for 45 s ( 300 s for the first cycle ) , annealing ( 60 s at 52c ) , and extension ( 90 s at 72c , 600 s for the last cycle ) . the staphylococcus - specific primers used for amplification and sequencing of rpob are shown in table 1 . sequencing reactions were performed in a total volume of 10 l containing 0.5 l premix ( abi prism bigdye terminator v3.0 ready reaction cycle sequencing kit , applied biosystems , darmstadt , germany ) , 1.8 l 400 mmol / l tris - hcl , 10 mmol / l mgcl2 , 10 pmol sequencing primer , and 2 l polymerase chain reaction product . the sequencing products were purified by using the centri - sep spin columns ( princeton separations , adelphia , nj , usa ) and analyzed with the abi prism 3100 avant genetic analyzer ( applied biosystems ) according to the manufacturer 's instructions . for further analysis , nucleotides 14441928 ( corresponding to s. aureus rpob gene positions of the genbank accession no . the sequences were analyzed by using ridom traceeditpro version 1.0 software ( ridom gmbh , wrzburg , germany ) . staphylococcal partial rpob reference sequences determined in this study were deposited in genbank under accession nos . * primers are numbered from the 3 end of the primer on the forward strand of staphylococcus aureus ( genbank accession no . all staphylococcal type strains were distinguishable by rpob ; the only exception was the s. equorum subspecies that shared the same sequence ( figure a1 ) . the mean pairwise distance of all type and other culture collection strains exhibiting a unique rpob sequence ( n = 68 ) was 13.7% ( range 0%21.4% ) and the standard deviation was 3.3% . when assuming a normal distribution for the distances and choosing a reporting criterion > 94.0% , the similarity for a distinct species correlates with a statistical error probability of 1.0% ( 9 ) . the definitive identification of 55 clinical isolates and the rpob gene sequence similarity search results are shown in table 2 . at the species level , the correct species designation for all 55 clinical isolates was made by rpob sequence similarity search ( sequence similarity > 94.0% ) . of 21 clinical isolates belonging to species currently divided into subspecies , 17 isolates were correctly identified to the subspecies level . subspecies identification for isolates m26 and m53 was unsuccessful by rpob or partial 16s rdna sequencing , riboprinting , and chemotaxonomy ( data not shown ) . only isolates m20 and m39 were misidentified by rpob sequencing as s. saprophyticus subsp . our previous study demonstrated the superiority of sequence - based methods over phenotypic approaches using the api i d 32 staph and vitek 2 systems ( 3 ) . the advantage of a sequence - based method became most evident when differentiating isolates with the scv phenotype , in which the api i d 32 staph and vitek 2 systems misidentified 2 and 4 isolates , respectively . when both sequence - based approaches used were compared , rpob sequencing was superior to partial 16s rdna identification . although the 16s rdna procedure differentiated 50 ( 90.9% ) of all tested clinical isolates at species level , rpob identified 100% . therefore , if an unknown organism needs to be identified , 16s rdna sequencing is the method of choice because of the availability of universal primers ( 10 ) . however , if the genus is already known , the rpob method should be used . compared with other published molecular probes , rpob showed the highest discriminatory power , e.g. , hsp60 and soda sequencing did not differentiate subspecies of s. carnosus , s. cohnii , s. hominis , s. schleiferi , or s. succinus ( 4,5 ) . in a previous study , rpob sequence - based identification of staphylococcus species has been reported ( 6 ) . however , a limited number of taxa were included , and the primers used were not appropriate to detect all staphylococcal subspecies . a higher discrimination with rpob sequencing compared with 16s rdna sequencing has been demonstrated for the genera corynebacterium ( 13 ) and bacillus ( 14 ) . dna sequencing is a rapid alternative to biochemical and other phenotypic procedures for the differentiation of bacterial pathogens because of its decreased costs and increased automation ( 15 ) . thus , rpob is a useful molecular target for differentiating staphylococcal isolates to the species and subspecies level . | the emerging clinical importance of staphylococcal infections prompted us to establish a reference database for partial rna polymerase b ( rpob ; nucleotides 14441928 ) gene sequences from type strains of all staphylococcal species and subspecies .
this database correctly identified 55 clinical staphylococcal isolates ; all were correctly identified at the species level . at the subspecies level , rpob misidentified only 2 isolates . |
class iii malocclusion is considered to be one of the most difficult and complex orthodontic problems to treat . prevalence of class iii malocclusion in caucasians ranges from 0.8 to 4.0% and rises up to 1213% in chinese and japanese populations , while in north indian population , class iii malocclusion is found in up to 3.4% of the population.[13 ] individuals with class iii malocclusion frequently show combinations of skeletal and dentoalveolar components . several distinct cephalometric features have been reported in class iii patients , such as a short anterior cranial base length , acute cranial base angle , a short and retrusive maxilla , proclined maxillary incisors , retroclined mandibular incisors , an excessive lower anterior face height and obtuse gonial angle . skeletal class iii malocclusion may either be associated with maxillary retrusions , mandibular protrusion , or a combination of the two . a poor facial appearance is often the patient 's chief complaint , but it may be accompanied by functional problems , temporomandibular disorders , or psychosocial handicaps . in this case report , we present the treatment of an adult girl with skeletal class iii malocclusion . a 21-year - old female presented with the chief complaint of an unesthetic facial and dental appearance [ figures 14 ] . she had a severe class iii malocclusion with 2-mm anterior crossbite and 2-mm reverse overbite . lateral view and oblique view showed pronounced mandibular prognathism and midface deficiency with concave profile . intraorally , the molar relationship was class iii with a complete anterior crossbite [ figures 59 ] . pretreatment extraoral fontal view pretreatment extraoral oblique view pretreatment extraoral lateral view pretreatment extraoral smile view pretreatment intraoral frontal view pretreatment intraoral right lateral view pretreatment intraoral left lateral view pretreatment intraoral maxillary occlusal view pretreatment intraoral mandibular occlusal view cephalometric analysis [ table 1 ] showed maxillary deficiency and mandibular protrusion . cephalometric findings before and after surgery the skeletal problem was due to a combination of maxillary deficiency and mandibular prognathism [ figures 1012 ] . pretreatment lateral cephalogram pretreatment orthopantogram pretreatment posteroanterior cephalogram bilateral sagittal split osteotomy with presurgical and postsurgical orthodontics was planned to achieve esthetically acceptable and functionally optimum occlusion with straight facial profile and minimum traumatic surgical exposure to the patient . maxillary and mandibular arches were aligned upto 0.019 0.022 stainless steel wire with 0.022 slot edgewise appliances . the mandibular incisors were decompensated by proclining them in normal inclination and the archforms were coordinated [ figures 1317 ] . presurgical orthodontics , intraoral frontal view presurgical orthodontics , intraoral right lateral view presurgical orthodontics , intraoral left lateral view presurgical orthodontics , intraoral maxillary occlusal view presurgical orthodontics , intraoral mandibular occlusal view before orthognathic surgery , the template was prepared using tracing paper . the cut section of mandible was then fitted back to tracing in desired occlusal relation and the probable postsurgical changes were checked . in this case , 8 mm of mandibular setback brought class i molar relation with an esthetically pleasing profile . medial pterygoid muscle was detached after performing the split and 8 mm setback was achieved . the patient was followed closely after the procedure and was guided to perform opening and lateral movements . one year later , fixed appliances were removed and a retention appliance was delivered [ figures 1828 ] . postsurgical extraoral frontal view postsurgical extraoral oblique view postsurgical extraoral lateral view posttreatment extraoral smile view postsurgical extraoral frontal view postsurgical intraoral right lateral view postsurgical intraoral left lateral view postsurgical intraoral maxillary occlusal view postsurgical intraoral mandibular occlusal view postsurgical lateral cephalogram postsurgical orthopantogram patient 's cooperation was excellent throughout the treatment . bilateral sagittal split osteotomy with presurgical and postsurgical orthodontics was planned to achieve esthetically acceptable and functionally optimum occlusion with straight facial profile and minimum traumatic surgical exposure to the patient . maxillary and mandibular arches were aligned upto 0.019 0.022 stainless steel wire with 0.022 slot edgewise appliances . the mandibular incisors were decompensated by proclining them in normal inclination and the archforms were coordinated [ figures 1317 ] . presurgical orthodontics , intraoral frontal view presurgical orthodontics , intraoral right lateral view presurgical orthodontics , intraoral left lateral view presurgical orthodontics , intraoral maxillary occlusal view presurgical orthodontics , intraoral mandibular occlusal view before orthognathic surgery , the template was prepared using tracing paper . the cut section of mandible was then fitted back to tracing in desired occlusal relation and the probable postsurgical changes were checked . in this case , 8 mm of mandibular setback brought class i molar relation with an esthetically pleasing profile . retromolar area was exposed using modified third molar incision . bilateral sagittal split osteotomy with short lingual split medial pterygoid muscle was detached after performing the split and 8 mm setback was achieved . the patient was followed closely after the procedure and was guided to perform opening and lateral movements . one year later , fixed appliances were removed and a retention appliance was delivered [ figures 1828 ] . postsurgical extraoral frontal view postsurgical extraoral oblique view postsurgical extraoral lateral view posttreatment extraoral smile view postsurgical extraoral frontal view postsurgical intraoral right lateral view postsurgical intraoral left lateral view postsurgical intraoral maxillary occlusal view postsurgical intraoral mandibular occlusal view postsurgical lateral cephalogram postsurgical orthopantogram patient 's cooperation was excellent throughout the treatment . this case report describes the treatment of an adolescent girl with dental and skeletal class iii relationships . orthodontic treatment was the best option for achieving an acceptable occlusion and a good esthetic result in this case . presurgical orthodontics removes all the dental compensations and suggests the location and extent of the skeletal discrepancy . normal skeletal base relationship is achieved by osteotomy and setback of the prognathic mandible , postsurgical orthodontics guides the normal occlusal rehabilitation by correcting any emerging dental discrepancies . | for patients whose orthodontic problems are so severe that neither growth modification nor camouflage offers a solution , surgery to realign the jaws or reposition dentoalveolar segments is the only possible treatment option left .
one indication for surgery obviously is a malocclusion too severe for orthodontics alone .
it is possible now to be at least semiquantitative about the limits of orthodontic treatment , in the context of producing normal occlusion as the diagrams of the envelope of discrepancy indicate . in this case report
we present orthognathic treatment plan of an adult female patient with skeletal class iii malocclusion .
patient 's malocclusion was decompensated by orthodontic treatment just before the surgery and then normal jaw relationship achieved by bilateral sagittal split osteotomy . |
ependymomas are rare forms of primary brain tumors , accounting for 37% of all brain tumors [ 1 , 2 ] . ependymomas , composed of neoplastic ependymal cells , are thought to arise from the ependymal cells in the ventricular system . the majority occur infratentorially in the pediatric population , while a smaller fraction occurs in adults , usually in the spinal cord . rare variants of ependymomas have been described and can include cellular , papillary , clear - cell , and tanycytic variants . tanycytic ependymomas are particularly rare and typically occur in the spinal cord as an intramedullary mass . first described by friede and pollak in 1978 , tanycytic ependymoma represents a who grade ii ependymoma with characterized pathology described as containing bipolar cells with absent mitosis and low - to - moderate cellularity . here the patient is a 58-year - old male with a history of hypertension and type ii diabetes mellitus who presented to an outside hospital after developing chin and right arm tremors , followed by a seizure . the seizure was complicated by a fall and urinary incontinence ; the seizure resolved on its own . a ct completed at an outside hospital revealed a large , complex cystic mass in the frontal lobe and left hemispheric subarachnoid hemorrhage . mri analysis revealed a 5.6 8 5 cm complex cystic lesion in the left frontoparietal lobe , with a mass effect on the left lateral ventricle ( fig . there was some enhancement in the cystic components that was also visualized on t1 imaging with gadolinium . surgery was performed , and a left frontoparietal brain lesion , which was initially suspected to be a low - grade glioma , was removed . however , the tumor was surprising in its grayness and more firm than it would be expected for a low - grade glioma . the surgery was repeated 3 days later for microdissection and tissue harvesting . during his hospital stay , the patient developed waxing and waning expressive aphasia , with the development of global aphasia , a right facial droop , and right - sided weakness . a ct demonstrated hemorrhage in the resection cavity , effacement of the left superior frontoparietal sulci suggestive of cerebral edema , and a slight increase in left to right midline shift from 1.3 to 1.5 cm . however , it was unclear if the aphasia stemmed from the clot or cerebral edema following the surgery . on the day following evacuation of the hematoma , the patient 's symptoms improved . the patient 's symptoms included residual slurred speech , which improved over the subsequent month . at his last visit , 18 months following surgery , he continued to do well with no evidence of clinical or radiographic recurrence . pathological analysis revealed brain tissue infiltrated by well - differentiated piloid cells with low - to - moderate cellularity ( fig . analysis of immunohistochemical staining revealed glial fibrillary acidic protein - positivity solely in reactive astrocytes . the proliferative index of the tumor was low , with only 2% of cells positive for ki67 staining . this case report shows the diagnosis and current clinical outcome of a male adult patient with a supratentorial tanycytic ependymoma , who grade ii . this rare variant of ependymoma commonly occurs in the spinal cord . in a review of the literature , we identified only 6 cases of ectopic supratentorial tanycytic ependymomas ( table 1 ) [ 4 , 5 , 6 , 7 , 8 , 9 ] . four cases were identified after the onset of seizures , one after the onset of headaches , and one was incidental . locations varied among the cases , including both cortical and subcortical locations as well as right and left hemispheres . tumors were frontal , temporoparietal , temporo - occipital , parietal , and parieto - occipital . in this series of patients , the wide age ranges of the patients and the presence of the tumors throughout the hemispheres underscore the importance of considering ependymomas in the diagnosis of tumors occurring throughout the brain . this is especially crucial with regards to tanycytic ependymomas , which can be easily mistaken for pilocytic astrocytomas or fibroblastic meningiomas . this can occur due to the bipolar nature of the tumor cells on pathology along with low - to - moderate cellularity . nonetheless , a number of nonconventional ependymoma variants can also mimic other tumors within the brain . papillary ependymomas may demonstrate features similar to those of choroid plexus tumors , while myxopapillary ependymomas may mimic chordomas . in undiagnosed tumors that lack the typical staining pattern for more common intraparenchymal tumors , alternative diagnoses , including variants of ependymoma , must be considered . in conclusion , this case report represents the rare presentation of a tanycytic ependymoma in the supratentorial region in an adult male patient . proper pathological diagnosis of the rare variant of ependymoma is warranted as it can impact prognosis and treatment . | ependymomas , tumors of the ependymal cells , are very rare and usually present in the pediatric population . furthermore , there are even rarer variants of ependymomas that can include cellular , papillary , clear cell , and tanycytic subtypes .
we present a case of a supratentorial tanycytic ependymoma in an adult male and review the literature in regard to this rare primary central nervous system neoplasm . |
a 50-year - old , female patient presented to the dermatology outpatient department with complaints of itchy lesions all over the body for 1 month . the lesions were erythematous , scaly plaques which developed over the thigh followed by new lesions which appeared over the chest , back , upper limb , and face . she gave a history of being diagnosed with sle , 10 years ago , and was on treatment with prednisolone 10 mg once a day . hydroxychloroquine 400 mg once a day and methotrexate 7.5 mg twice a day pulse therapy every week were started 2 months back . cutaneous examination showed diffuse erythema of skin over the face , upper limb , trunk , and lower limb with exfoliation of the skin [ figures 1 and 2 ] . nail examination showed beau 's lines over the finger and toenail [ figure 3 ] . complete hemogram showed hemoglobin-11.8 g / dl , white blood cell count 12,400 cells / cumm , and erythrocyte sedimentation rate 45 mm/1 h. blood sugar , liver function test , renal function test , and urine examination were normal [ table 1 ] . exfoliation of skin over the palm in a patient with hydroxychloroquine - induced erythroderma exfoliation of skin over the foot in a patient with hydroxychloroquine - induced erythroderma beau 's lines over the fingernail in a patient with hydroxychloroquine - induced erythroderma laboratory investigations in a patient with hydroxychloroquine - induced erythroderma based on the history and clinical examination , a provisional diagnosis of drug - induced erythroderma was made . the biopsy done from erythematous scaly plaque showed hyperplastic stratified squamous epithelium with spongiosis and mild acanthosis . focal areas of neutrophilic inflammatory infiltrate admixed with parakeratosis were also present which confirmed erythroderma [ figure 4 ] . direct immunofluorescence showed granular staining of basement membrane zone with igg and c3 consistent with sle . the patient was treated with calamine lotion twice a day topical application , topical liquid paraffin once a day , topical fluticasone twice a day for new lesions , and tablet hydroxyzine 25 mg once a day . the patient was in the recovery phase after a week of treatment and was discharged . causality assessment with both who - umc causality assessment scale and naranjo adr probability scale showed that the adr is probable . microscopic appearance of a biopsy done from the erythematous scaly plaque in a patient with hydroxychloroquine - induced erythroderma . erythroderma , also sometimes called exfoliative dermatitis , is defined as diffuse erythema and scaling involving more than 90% of the total body skin surface area . exfoliation begins within few days , but hair and nail changes occur after weeks to months . the most common cause is an exacerbation of preexisting dermatoses such as psoriasis and atopic dermatitis . uncommon causes include cutaneous t - cell lymphoma and other internal malignancies . in the case of drug - induced erythroderma , onset may be abrupt with morbilliform or urticarial eruptions which coalesce later into red erythematous patches with islands of sparing , which resolves faster than other causes . hydroxychloroquine and chloroquine are 4-aminoquinolone derivatives which have shown to be effective in sle since quinine was first used more than 100 years ago . they are structurally similar , only differing in the replacement of ethyl group in chloroquine by hydroxyethyl group in hydroxychloroquine . they have a variety of action ; the most important among them is the lysosomotropic action . hydroxychloroquine is a weak base which accumulates and raises the acidic ph of lysosomes and interferes with biologic functions which are dependent on it . this may lead to its immunomodulatory , anti - inflammatory , antiproliferative , and photoprotective effects . hydroxychloroquine is preferred to chloroquine because of its safety profile and is the first - line drug in patients with sle . cutaneous adrs are also less common in patients with sle compared with those with dermatomyositis . roughly 25% of all dermatomyositis patients experience hypersensitivity reactions , drug eruptions during antimalarial treatment , and may represent a disease - specific idiosyncratic reaction in such patients . drugs implicated include carbamazepine ( 57.1% ) , phenytoin ( 14.3% ) , phenobarbital ( 9.5% ) , lithium ( 4.8% ) , penicillin ( 4.8% ) , vancomycin ( 4.8% ) , and co - trimoxazole ( 4.8% ) . the exact pathogenesis of erythroderma is unknown but is thought to be due to complex interaction of cytokines , chemokines , and intercellular adhesion molecules which lead to recruitment of inflammatory cells and increased epidermal turnover . the increased mitotic rate and decreased transit time of epidermal cells lead to exfoliation and loss of proteins and nucleic acid through the skin . the initial management remains similar irrespective of the cause including monitoring of hemodynamic status and temperature ; correction of fluid and electrolyte imbalance ; and nutritional support . symptomatic treatment of skin inflammation and pruritus includes the use of emollients , low to mid - potency corticosteroid , and oral antihistamines . cutaneous adrs are usually not severe , but can sometimes be life - threatening or result in disabling sequelae . drug - induced erythroderma is one such adr where early diagnosis can avoid serious outcomes . early recognition of the condition can also be achieved with adequate patient education when drugs with the potential to cause erythroderma are prescribed . the patient should also be educated that reexposure to the offending agent can lead to more serious adr in the future . | erythroderma is characterized by diffuse erythema and scaling of the skin involving more than 90% of the total body skin surface area .
drug - induced erythroderma has rarely been reported with hydroxychloroquine .
we report a case of a 50-year - old female patient , with systemic lupus erythematosus , who developed itchy lesions all over the body 1 month after starting treatment with hydroxychloroquine .
drug - induced erythroderma was suspected .
hydroxychloroquine was withdrawn and the patient was treated with emollients , mid - potency corticosteroids , and oral antihistamines .
a biopsy was done which confirmed the diagnosis of erythroderma .
she recovered with treatment and was discharged . a careful history and clinical examination to search for potential causative factors will help prevent disabling sequelae in erythroderma . |
amyloidosis whether generalized or localized can affect the breast . by definition , amyloidosis is the extracellular deposition of amorphous congophilic protein within tissues . many reports have described the image findings of primary breast amyloidosis as a mass on mammography . breast amyloidosis manifesting only as calcifications have been rarely reported ( 1 - 5 ) . herein , we report a case of localized amyloidosis of the breast presenting suspicious microcalcifications which are not associated with a mass as seen on the mammography . a 72-year - old woman was referred to our hospital for further evaluations of calcifications detected on a screening mammography taken at another hospital . the patient had a 5-year history of diabetes mellitus . no breast symptoms were evident such as pains , palpable masses , or nipple discharges . mammography demonstrated multiple , irregular calcifications in the subareolar area of the left breast ; they were regionally distributed , generally smooth - branched , linear , and rod - like , and varied in sizes and shapes ( fig . these calcifications were classified as category " 4a " : low suspicious findings at final assessment according to the breast imaging reporting and data system ( 6 ) . thus , a biopsy was recommended and the patient underwent surgical excisional biopsy with mammoguided needle localization . histopathology examination revealed dystrophic calcifications in ectatic mammary ducts , amorphous eosinophilic material deposition ; the infiltration of periductal lymphocytes and multinucleated giant cells were revealed upon hematoxylin - eosin staining ( fig . the calcifications were stained with congo - red and exhibited apple - green birefringence under polarizing microscopy ( fig . our patient refused further investigations , and there was no clinical evidence suggesting generalized amyloidosis in her physical or laboratory results . there was no interval change in calcifications of the left breast on follow - up mammography for five years . during the follow - up period of five years , no clinical or laboratory evidence of systemic it is divided into the systemic and localized form , according to the extensions of the disease and it can be classified as a primary or secondary form according to the etiology . amyloidosis can also be classified as the aa or al type based on chemical compositions . in clinical practice , the most commonly diagnosed form of amyloidosis is primary idiopathic amyloidosis , the al type ( 7 , 8) . breast amyloidosis typically appears as diffuse breast involvements in the systemic form of amyloidosis with the primary al type rather than aa type ( 8) . a less common manifestation is the isolated breast involvement that occurs as a localized form of disease ( 7 , 8) . the clinical course tends to be benign , and typically , patients complain for hard , non - painful , and palpable masses in the affected breasts ( 5 ) . previous studies have reported that common mammographic findings of breast amyloidosis have a variety of solid shapes or multiple masses or nodules , whether including or excluding calcifications ( 9 , 10 ) . primary breast amyloidosis which consists of only microcalcifications excluding the associated mass is very rare . only five cases that were presented solely as microcalcifications have been reported ( 1 - 5 ) . one case was breast involvements of systemic amyloidosis within a patient with a 15-year history of multiple myeloma ( 3 ) . the remaining four reports were in localized forms . in the previous reports , the shapes of the microcalcifications varied according to clustered ( 4 , 5 ) , pleomorphic ( 4 , 5 ) , fine linear and branching ( 1 , 2 ) , and smooth branching rod - like ( 2 , 3 ) shapes . all the previous mammographic findings were assessed either as intermediate or suspicious of malignancy , and pathological confirmations were recommended ( 1 - 5 ) . in this case , there were irregularly linear distributed , multiple , smooth , rodlike calcifications as evident by mammography , similar to the two previously reported cases ( 2 , 3 ) . among these two prior reports , one is of localized form , and the other being breast involvements of systemic amyloidosis ( 2 , 3 ) . this implies that mammographic findings between systemic amyloidosis and localized amyloidosis are not different which is probably due to the same pathophysiology . in breast amyloidosis , the amyloid is histologically evident as depositions at periductal , interstitial , or perivascular spaces with multiple multinucleated giant cells and calcifications . amyloid fibrils have an affinity for calcium and deposition around mammary ducts and is found in blood vessels ( 7 ) , thus , this pathophysiology is strongly correlated with the mammography - evident branching or linear distribution of microcalcifications deposited in or around the vasculature or mammary ducts . few cases of breast amyloidosis were associated with breast cancers such as ductal carcinoma in situ , invasive carcinoma , invasive lobular carcinoma , or tubular carcinoma ( 8 - 10 ) . and , some cases were associated with diffuse skin thickening which mimics inflammatory breast cancer ( 10 ) . in our case , although no distinctive findings have been reported for breast amyloidosis , the previously reported cases presenting suspicious masses with or without microcalcifications that mimic malignancy and pathologic diagnosis were needed . in summary , breast amyloidosis is a rare entity that is usually evident as clinical or radiological palpable masses . breast amyloidosis which only presented microcalcifications without mass is still rare , and only a few cases have been reported ( 1 - 5 ) . herein , we report a case of breast amyloidosis detected upon mammography which was solely presented as suspicious microcalcifications without mass as diagnosed by surgical excisions . | amyloidosis is a rare disease characterized by the formation of pathological protein deposits in organs or tissues .
it is typically a systemic disease which can occur in a localized form .
amyloidosis of the breast is uncommon .
common mammographic findings of breast amyloidosis are multiple nodules with or without calcifications .
we report a case of primary localized breast amyloidosis presenting suspicious microcalcifications on mammography without associated masses .
mammography in a 72-year - old woman displayed multiple , linearly distributed , irregular and rod - like calcifications in the subareolar area of the left breast .
the patient underwent surgical excision under mammo - guided needle localization and the pathology was confirmed to be breast amyloidosis . |
the 25-gauge curved vitrectomy probe was a prototype
manufactured by bausch and lomb ( rochester , ny ) . the length of
the probe is 25 mm with a uniform 25-g diameter ( 0.5 mm ) . construction of the illuminated vitrectomy probe is similar
to that described previously with a 20-g vitrectomy probe.5
a seamless strong polyester ( polyethylene terephthalate ) heat
shrunk tubing ( advanced polymers , inc . salem , nh ) is used
to secure a fiberoptic endoilluminator with a curved 25-g
vitrector ( [ fig . 1a , b , and 2a ] : vitrector and em).6 the tubing
has an internal diameter of 0.05 inches and thickness of 0.0005
inches . the resultant illuminated vitrector has a diameter of
1.0 mm ( 0.5 mm fiberoptic light source + 0.5 mm 25-g vitrector )
[ fig . the assembly is sterilized with gamma radiation ,
ethylene oxide or cold gas sterilization . standard surgical technique with microvitreoretinal blade is
used to create the sclerotomies.7 the posterior ppv is completed
using a 20-g vitrector . at the end of the procedure the 20-g
cutter is replaced with the 25-g illuminated curved vitrector
[ fig . 3 ] . the illumination of the probe facilitates visualization
of the vitreous on the opposite vitreous base ( 180 away ) , while
the curved design of the probe avoids crystalline lens touch
[ figs . 4 , 5 ] . this allows the surgeon to use the other hand to
depress the opposite sclera , which enables viewing of the
internal sclerotomy with its surrounding vitreous through the
wide - angle lens system [ fig . position of the endoilluminator
tip from the vitrector port can be varied by a gentle sliding
maneuver during the surgery to optimize the illumination
of the incarcerated vitreous . the vitreous incarcerated in the
internal sclerotomy opening is shaved completely under direct
visualization . excision of the peripheral vitreous in a phakic eye with a
clear lens is one of the most challenging tasks in vitreoretinal
surgery . with the advent of the wide - angle viewing systems ,
visualization of the peripheral vitreous base the potential of iatrogenic trauma to the posterior
lens surface by the straight vitrectomy probe restricts access
to the peripheral vitreous near the sclerotomy site.8 a curved
instrument allows access to the internal sclerotomy site without
damaging the posterior lens surface [ fig . 5].9,10 while using the curved or conventional vitrector , the surgeon
holds the endoilluminator while the assistant depresses the
sclera for view of the anterior vitreous . this makes the surgery
assistant - dependent , time - consuming , and limits the dynamic
viewing of the vitreous base with the endoilluminator . the 20-g curved illuminated vitrector addresses the
problems of illumination of the peripheral vitreous and safety
of the crystalline lens.11 however , it requires a larger sclerotomy
( 18-g ) than conventionally used , thereby increasing the risk
of sclerotomy - related leakage and vitreous incarceration . due to the smaller diameter of the shaft , the 25-g curved
illuminated vitrector allows removal of the vitreous from the
internal sclerotomy site through a conventional sclerotomy
( 20-g ) while preserving the safety of the posterior lens surface .
clinical examination as well as ultrasonographic examination
confirmed this in both pseudophakic and phakic patients
[ fig- 6a , b ] . an illuminated sleeve ( synergetics , inc . charles , mo )
that wraps around a straight 25-g vitrector provides the benefit
of illuminated 25-g vitrector in a pseudophakic eye . however ,
the illuminated sleeve does not conform to the curved 25-g
vitrector and can not be used in phakic eyes without risk of
lens touch . effective and complete removal of vitreous from the
internal sclerotomy prevents postoperative complications
associated with wound healing . sabti et al . , studied 22 eyes
with ultrasound biomicroscopy ( ubm ) of which 11 underwent
ppv with complete shaving of the vitreous from the internal
sclerotomy site by indentation with a straight conventional
vitrector , while 11 eyes in the control group underwent
conventional ppv only . complete vitreous shaving around the
sclerotomy site significantly reduced vitreous incarceration.4
removal of vitreous from the internal sclerotomy assumes
added importance during implantation of intra - vitreal drug
delivery systems where large sclerotomies are necessary to
accommodate the size of the implant ( 2 to 4 mm ) . in a prospective study utilizing ubm to study the internal
sclerotomy site in diabetic patients undergoing vitrectomy ,
bhende et al . , noted that eyes with postoperative vitreous
incarceration developed fibrovascular proliferation six months
after surgery.3 this is a known high - risk factor for recurrent vitreous
hemorrhage after ppv in diabetics we have successfully employed this instrument in 100
complicated vitreoretinal procedures , of which 41 were phakic
eyes . no lens - related complications occurred , though novice
surgeons may experience a learning curve with the new device .
in summary , the 25-g curved illuminated cutter combines the
advantage of a self - illuminated vitrectomy probe and custom
curved design that avoids crystalline lens touch . in addition ,
its usage through a standard 20-g sclerotomy in facilitating
complete removal of vitreous from the internal sclerotomy
prevents sclerotomy - related complications . | incarceration of vitreous in sclerotomy sites during pars plana
vitrectomy can lead to wound - related complications similar
to vitreous incarceration in cataract surgery .
we describe an
illuminated curved 25-gauge vitrectomy probe for removing
vitreous from sclerotomy sites .
polyester tubing is used to
secure a fiber optic endoilluminator ( 0.5 mm ) with the curved
25-gauge vitrector ( 0.5 mm ) .
the resultant illuminated curved
vitrector ( 20 g ) has a diameter of 1.0 mm .
it facilitates complete
removal of vitreous around the internal sclerotomies under direct
visualization in both phakic and pseudophakic eyes . the same
was confirmed with ultrasound biomicroscopy of the sclerotomy
sites .
curved vitrector reduces postoperative complications
related to incarcerated vitreous in phakic and pseudophakic eyes
and other sclerotomy - related wound complications . |
for companies in highly industrialised countries , high environmental standards and the responsible use of environmental resources are the norm . particularly for companies operating on a regional level , such as healthcare facilities , public perceptions are increasingly important . in this context , the presentation of ecological behaviour within the company is assuming greater importance . various instruments exist to demonstrate the willingness of a company to operate in an ecologically responsible manner to the general public , and promote environmental protection within the company . these instruments include certification , e.g. the eu emas ( environmental management and audit scheme ) on the basis of iso 14000 ) , the use of eco - labels , e.g. corporate social responsibility , , or environmental reporting , . environmental reporting , a prerequisite for certification , documents and communicates the relationship of a company to environmental protection and its activities in this regard . the growing number of companies issuing environmental reports shows that there is an increasing discourse on environmental protection within companies . unfortunately , relatively few healthcare facilities have issued environmental reports , and these vary widely in terms of scope and quality , , , , , , , , . the university medicine greifswald has been working on the publication of an environmental report for a number of years . to this end , several projects have been carried out to investigate the suitability of ecologically relevant topics for environmental reporting purposes . the motivation and commitment of hcws are a decisive factor in the implementation of corporate ecological responsibility . in this regard in the first instance it is necessary to assess the existing significance attached to ecological behaviour by staff , in order to then promote ecological awareness and ecologically responsible behaviour in a targeted manner . for this reason we conducted , in consultation with the medical director / ceo , an employee survey on environmental behaviour and the value attached to the protection of the environment . copies of a questionnaire with a demographic section , 12 items relating to environmental behaviour and 10 items relating to the significance attached to the protection of the environment , were left in a central place highly frequented by staff on randomly selected wards ( n=10 ) and in randomly selected departments ( n=2 ) . the results of the items relating to environmental behaviour are shown in table 1 ( tab . 1 ) and the results for the significance attached to the protection of the environment are given in table 2 ( tab . as no suitable questionnaire for healthcare facilities was found in the literature , the questionnaire was developed in collaboration with the institute of psychology . we decided to include a demographic section , items relating to environmental behaviour and items relating to the significance attached to the protection of the environment by staff . the response rate of 51.8% did not meet expectations , but can nonetheless be considered representative given that responses were made during normal business and on a purely voluntary basis . the share of responses from females , 77% , corresponds approximately to the overall employment structure of the university hospital . the most interesting and perhaps most surprising outcome was that 97.3% of those questioned considered ecologically responsible behaviour to be one of the duties of a hospital . therefore there is high potential to promote ecological awareness and ecologically responsible behaviour in the university medicine greifswald . with regard to the use of paper , we subsequently discovered that recycled paper was not available to staff in sufficient quantities , so that the use rate for recycled paper can certainly be increased . only 43.1% of respondents printed paper on both sides and 31.9% of respondents used paper on both sides for note taking etc . there is a high level of acceptance for the sorting of rubbish , as 77.7% of respondents sort their waste . therefore as a next step , we will investigate whether staff has sufficient time and facilities for sorting rubbish . the item relating to the use of ecological writing utensils such as pencils proved to be irrelevant for assessment of environmental behaviour . more than 92% of staff used biros because data protection and documentation regulations stipulate the use of permanent ink . consequently this item almost 90% of respondents use ecologically preferable glasses and ceramic mugs / cups rather than paper or plastic cups for hot drinks . that 48.9% of respondents leave electronic equipment in stand - by mode when not in use is unsatisfactory . more than 80% of respondents clean their crockery in a dishwasher , which is only ecologically acceptable if the machine is fully loaded . as far as the items relating to the significance attached to the protection of the environment are concerned , more than 90% of respondents replied that the protection of the environment was important for them . this allows us to draw an important conclusion for the hospital management and for the environmental report : the protection of the environment should not be neglected ! just 25% of respondents had previously received training in environmental matters , which provides significant potential for improvement , even if only 42% of respondents expressed interest in such training . one reason for this is certainly the heavy burdens on the time of staff , which is lower in age than the average , due to family and shift working . further potential for improved environmental protection can be deduced from the answers to item 17 . whilst 61.7% of respondents claimed to apply their knowledge relating to the protection of the environment in the work place , the remainder did not . for almost 40% of the sample . this could be due to a lack of opportunities in the work place and/or a lack of time . in this regard , the aforementioned training measures are important to raise awareness of the importance of environmental considerations in the work routine . a further aspect that should not under - estimated is that 87.8% of respondents assume that ecologically responsible behaviour leads to financial savings . this can be interpreted positive as the commitment of staff to economic efficiency on the one hand , or as still unharnessed ecological and economic potential on the other hand . the responses to the final question show that the employees have a positive attitude towards that environment . amongst the respondents 82.1% this is a huge resource that obliges the hospital management to use and develop this potential in a responsible manner . an environmental ideas competition could bring many concrete improvements and further sensitise staff to the environmental issues . with the exception of question 2 the used questionnaire was suitable to survey environmental behaviour and the significance attached to the protection of the environment amongst the staff of medical facilities . regularly conducted surveys would allow the identification of trends . the results of the survey show that the protection of the environment was of unexpectedly high personal importance to staff within the university medicine greifswald . based on this finding , this potential should be used to promote the optimal implementation of ecological - economic behaviour within the university hospital . marek zygmunt , medical director / ceo of the university medicine greifswald for his support in the hcw survey . marek zygmunt , medical director / ceo of the university medicine greifswald for his support in the hcw survey . | background : environmental reporting is increasingly important for medical facilities .
currently , hospitals can determine the content of an environmental report as they see fit .
objective : to examine the utility and scope of an employee survey as an instrument for the preparation of an environmental report at the university hospital greifswald . method : for this purpose a questionnaire was developed with a focus on environmental behaviour and the significance attached to the protection of the environment.results : the employees of the university medicine greifswald attach an unexpectedly high significance to the protection of the environment . based on this finding
, this potential should be used to promote the optimal implementation of ecological - economic behaviour within the university medicine .
conclusion : an employee survey is a useful instrument in the preparation of an environmental report . |
skillful experienced personnel are mandatory . in order to have a good outcome with minimal risks and maximal success in airway management , should be in collaboration with the anesthesiologist or trauma team leader is must . hutchinson et al . addressed six specific situations associated with maxillofacial trauma , which may adversely affect the airway : 1 . postero - inferior displacement of a fractured maxilla parallel to the inclined plane of the skull base , 2 . hemorrhage , 4.soft tissue swelling and edema , 5.trauma to the larynx and trachea , 6.foreign bodies dentures , debris , shrapnel , exfoliated teeth , bone fragments . on the other hand , nasal intubation is common procedure for airway management during maxillofacial surgery especially for those that they need intermaxillary fixation like panfacial fractures . dental occlusion is the key point for proper reconstruction during surgery even in post operative period . in many instances , comminuted fracture of maxilla concomitant with nasal floor and septum may block nasal route due to its accordion type of fractured segments and overlapping of fractured bones . moreover , folding of their overlying mucosa is the main cause of nasal obstruction . in this situation , in emergency situation it may manage by cricothyrotomy in operating room . if a difficult airway is not anticipated , the tracheal tube is passed through the nose after induction of anesthesia and neuromuscular blockade . this is followed by direct laryngoscopy to forward the tube into the trachea under direct vision by either manipulating the tube directly or using magill forceps . we report a case who suffered from combine crashed lefort i lefort iii and zigomatico - orbital fracture due to the road traffic accident and a difficult nasal intubation during anesthesia . a 27-year - old male patient after discharging from neurosurgery ward with gcs 15 was referred to maxillofacial surgery department for treatment of his left lefort iii , right lefort ii , leforti , bilateral orbital , and left blow out fractures . past medical history did not show any remarkable systemic diseases and nasal obstruction due to his septal deviation . the patient was traumatized severely on a crashed road traffic accident 5 days ago without any brain involvement and unconsciousness [ figure 2 ] . surgeon and anesthesiologist made a joint bedside consultation about evaluation of patient airway management during and after surgery . patient presented acceptable neck movements ; mallampati test was not performed due to being painful and inability of the patient in mouth opening , open nasal airway tests , which were not acceptable in both sides and normal thyromental distance . overall , due to the multiple fractures in both sides of the face , patient was considered as a difficult intubation case , so required equipments including video laryngoscope , fiber optic laryngoscope , difficult intubation set with its complete equipments and emergency tracheostomy set were standby . he was scheduled for surgery using mandibular dental arch as a base reference for repositioning of other displaced facial compartments . the goal of anesthesia was to secure the airway immediately after induction with ready available equipments . the induction of anesthesia was achieved with lidocaine%2 ( 1 mg / kg ) , midazolam ( 0.03 mg / kg ) , fentanil ( 2 gr / kg ) , propofol ( 2 mg / kg ) and atracorium ( 0.6 mg / kg ) . preoperative dish face profile view of patient axial ct scan shows crashed midface fracture anesthesiologist faced a difficult intubation due to the obstruction of the nasal route . nasal fiberoptic endoscopic investigation and computed tomography ( ct ) scan showed obstruction of nasal airway by folding and overlapping of nasal mucosa because of the severe retrusion of the midface and crashed maxillary bone . a 30 cm 0.4 stainless ligature wire inserted from distal interdental space of first premolars embrasures . flouting midface pulled forward and slightly downward by surgeon hand force . by this maneuver overlapped bony segments concomitant with overlying mucosa were straightened up and nasal obstruction was opened . nasal fiberoptic endoscope passed through nasal fossa into the trachea then fiber optic nasotracheal intubation easily was performed . nasotracheal tube was softened with warm normal saline and it lubricated for prevention of mucosal laceration [ figure 3 ] . the patient did not present any airway obstructive complications immediate post surgically and latter six months follow - up . dental occlusion is essential reference for surgical reconstruction of displaced facial bony fragments in panfacial fractured patients . during operation surgical team needs to check the patient 's dental occlusion for proper reduction and fixation of fractured bone . elective tracheostomy is another airway management , but it has its own side effects and complications . in the case of maxillofacial fractures without fractures of skull base and brain involvement nasal intubation can be a favorite airway management for both surgeon and anesthesiologist due to its low postoperative complication rate and its safe airway management during the surgery . nasal obstruction can occur due to severe septal deviation , massive turbinate hypertrophy , tumors , foreign bodies , comminuted maxillofacial fractures . in the case of lefort type floating maxilla , obstruction can be owing to the overlapping of fractured nasal floor and maxillary bone and their mucosa . forward movement of crashed maxilla aligns fractured bony segments inside of periostal sac and crooked overlying nasal mucosas were unfolded . present technique provides a good visualization for fiberoptic nasal endoscopy and consequently safe nasal intubation ; moreover it may prevent anterior dental damage in the case of direct laryngoscoy . retruded position of dental occlusion due to the backward fractured maxilla causes some difficulties during laryngoscopy . close cooperation between anesthesiologist and the surgical team is essential for proper performance of this technique . present technique can be performed only before fibrosis and consolidation of the fracture segments in early post traumatic period . overlapped cartilaginous and bony segments of nasal septum can be straightened by this maneuver during intubation . based on above mentioned points , maxillary forward movement technique can be an extremely useful maneuver for nasal intubation of patients with midface fractures . additionally , it can be considered an alternative technique beside the other airway management methods for early reconstruction of panfacial fractures . | dental occlusion is key point for proper maxillofacial reconstruction . in this way
nasal airway management is extremely important for both oral and maxillofacial surgeons and anesthesiologists .
we report a challenging case with severe maxillofacial trauma and nasal obstruction that it managed with a novel anesthetic - surgical procedure . |
endophytic actinobacteria have a capacity to produce numerous secondary metabolites with a mass of biological activity , such as antibiotics , antitumor and anti - infection agents , plant growth promoters and enzymes , and may promote plant establishment under adverse environmental stresses . introducing such bacterial strains to plant tissues can result in increased plant growth , usually due to suppression of plant pathogenic microorganisms . it seems to be pivotal for obtaining a healthy microfloral balance within plants , soil appearing to be an important and moderating source of bacterial endophytes . recently , our group has isolated from a carrot sample from xinjiang uyghur autonomous region ( china ) a novel species of paenibacillus dauci sp . 100608 = jcm30283 ) , which can produce potential antimicrobial substances playing the part of endophytic actinobacteria . comparisons with 16s rrna gene sequences as shown in fig . 1 revealed that the novel strain had the highest similarity to paenibacillus hunanensis fel05 ( 97% ) . however , the phylogenetic distances from recognized species ( fig . 2 ) indicated that p. dauci sp . nov . is not affiliated to any of these recognized species . we can therefore conclude that this strain represents a novel species of the genus paenibacillus . what 's more , high nitrogenase activity , strong antagonism against plant pathogenic fungi , extensive carbon source utilization , and stress resistance were also uncovered . in consequence , investigation of the genetic information and characteristics of p. dauci knowledge of the genome sequence and bioinformatics will be of great help in this regard . here we present the draft genome sequence of strain p. dauci h9 obtained using the illumina hiseq 2000 system , which was performed by shenzhen bgi . tech . the reads were assembled with soapdenovo , , the version is 2.04 , and the sequence was annotated using the rast annotation server ( fig . sequencing was performed based on the paired - end strategy of 473 reads to produce 790 mb of filtered sequences , representing a 126-fold coverage of the genome . the sequence of paenibacillus algorifonticola xj259 is 5,449,237 bases with a g + c content of 46.5% , which was assembled into 26 contigs and 19 scaffolds . it contains 4766 open reading frames ( orfs ) , 77 trna genes , and 1 rrna gene ( table 1 ) identified by glimmer 3.02 , genemark , trnascan - se , and rnammer . according to the genomic analysis of strain p. dauci , we analyzed 36 orfs related to antibiotic metabolic process . additionally , 12 orfs were also discovered related to trehalose , which makes us believe that it could be related to the shock - resistant mechanism since the trehalose is regarded as a molecular chaperone . what 's more , the biosynthesis of vitamin b was annotated in the strain p. dauci as there were 19 orfs related to vitamin b12 production and vitamin b6 metabolism . further studies will be performed to confirm their functions , and a complete genome sequence will be included in the future to reveal the unique molecular characteristics of strain p. dauci . this whole genome shotgun project has been deposited at ddbj / embl / genbank under accession number laqq00000000 . the version described in this paper is the first version , with accession number laqq01000000 . the authors declare that there is no conflict of interest on any work published in this paper . | paenibacillus dauci sp . nov . ,
a new kind of endophytic actinobacteria , is separated from the inner tissues of carrot sample , which forms intimated associations with carrot acting as biological control agents .
here we report a 5.37-mb assembly of its genome sequence and other useful information , including the coding sequences ( cdss ) responsible for biological processes such as antibiotic metabolic process , antimicrobial metabolism , anaerobic regulation and the biosynthesis of vitamin b and polysaccharide .
this novel strain can be a potential source of novel lead products for exploitation in the field of pharmaceutical , agriculture and industry . |
inflammatory myofibroblastic tumor ( imt ) of the liver is a very rare lesion , benign in nature . differential diagnosis of imt from malignant lesions of the liver is very important because surgical resection is not mandatory for imt and the prognosis is very good by conservative treatment . but , preoperative diagnosis is very difficult due to its radiologic similarity with intrahepatic cholangiocarcinoma , metastatic tumor and hepatocellular carcinoma ( hcc ) . lipiodol computed tomography ( ct ) , which is checked following lipiodol infusion via the hepatic artery , is a very sensitive and specific imaging modality for hcc . herein , we describe a case of imt that had dense lipiodol uptake in the tumor mimicking hcc . a 60-year - old woman was admitted at our hospital for evaluation and management of liver tumor . thus , she was treated with transhepatic arterial chemoembolization ( tace ) by using adriamycin ( 50 mg ) , lipiodol and gelfoam . abnormal laboratory results were decreased hemoglobin , 10.0 g / dl ( reference range , 12.0 to 16.0 g / dl ) ; elevated erythrocyte sedimentation rate , 122 mm / hr ( reference range , 0 to 22 mm / hr ) ; and elevated gamma - glutamic transpeptidase , 81 iu / l ( reference range , 7 to 32 iu / l ) . tumor markers were all nor mal ; alpha - fetoprotein , 3.2 ng / ml ( reference range , 0 to 10 ng / ml ) ; carcinoembryonic antigen , 0.4 ng / ml ( reference range , 0 to 5 ng / ml ) ; carbohydrate antigen 19 - 9 , 10.2 u / ml ( reference range , 0 to 37 u / ml ) . abdomen ct checked prior to tace showed a gross 5 cm spherical tumor in segment vii ( couinaud 's segment ) and segment vi of the liver . on arterial phase , this tumor showed general high attenuation with central stellate slight low attenuation ( fig . on portal phase , a major portion of the tumor showed remarkable low attenuation with irregular septa - like and peripheral thick rim - like high enhancement ( fig . lipiodol ct , which was taken 14 days following tace , revealed a 4.3 cm densely lipiodol uptaken spherical mass in segment s6 and s7 of the liver ( fig . 3 ) . high power view demonstrates interlacing bundles of myofibroblasts and fibroblasts in a collagenous stroma , with an admixture of polymorphic inflammatory cells , including plasma cells , lymphocytes and eosinophils ( fig . immunohistochemical stain revealed positive activity of vimentin ( diffuse + ) , smooth muscle actin ( mainly + ) and desmin ( focal + ) . the final diagnosis was imt . regular follow - up studies including abdomen ct showed no recurrence of tumor for 5 years after operation . imt , which also has been called ipt , is characterized by a mixture of myofibroblasts , fibroblasts , lymphocytes , and plasma cells in varying proportions . imts of the liver have rarely been reported , but nowadays reported cases are increasing probably due to the recent development of image modality . reported the incidence of imt was 0.7% by analyzing 473 patients with focal liver lesion that had been resected and the patients with imts accounted for 20% of the wrong preoperative diagnoses and 33% of wrong indications for surgery . the majority of imt can be managed successfully by medical treatment and the prognosis is very good . therefore , the accurate preoperative diagnosis of imt is essential for avoiding unnecessary surgery , which may have some morbidity and mortality . however , preoperative diagnosis by radiological modalities only is very difficult because there are few specific radiologic findings for imt . liver dynamic ct can reveal the enhancement pattern of the tumor during the different phases . this finding can easily differentiate imt from hccs but not from metastatic liver tumors or cholanigiocellular carcinomas . however , this point can not be applicable to all cases of imt . some cases had high enhancement on arterial phase and faded out on portal and delayed phase . in our case , the tumor showed general high attenuation on arterial phase and low attenuation with peripheral rim enhancement on portal phase . there were a few cases of imts that had been diagnosed as hccs preoperatively and resected . for fear of tumor spillage through needle track , lipiodol ct checked after intra - arterial injection of iodolized oil is widely used for the detection of hcc . ngan reported when dense homogenous uptake was present in a discrete mass , the chance of it being an hcc was high with post - test probability being 92.8% . in our case , contrast enhanced ct showed a well - enhanced tumor on arterial phase and lipiodol ct revealed dense homogenous uptake of lipiodol in tumor , by which findings were most compatible with hcc . to our knowledge , previously , only one case of imt with dense lipiodol retention has been reported . in general , lipiodol ct is checked 2 to 4 weeks following intra - arterial lipiodol infusion . in normal hepatic parenchyma , lipiodol is almost completely washed out at that time . however , lipiodol in hcc tissue mostly remains and can help in detecting small hcc . the reason for the discrepancy of lipiodol retention rate in normal parenchyma and tumor tissue has not been clearly explained yet . one plausible hypothesis is that it may be caused by the difference of blood flow . the tumor vessels that are tortuous and irregular and often lack both a muscular layer and elastic lamellae do not have sufficient blood flow to clear away the adhesive iodized oil . in this case , it was supposed that hepatic artery embolization for presumed hcc decreased the blood flow about the tumor and dense lipiodol remained in the tumor . choi et al . reported that hcc with complete intratumoral retention of lipiodol had 98% necrosis but tumors with incomplete retention had 64% necrosis . in conclusion , radiologic findings of imt including liver dynamic ct and lipiodol ct can mimic hcc . therefore , if other clinical data including viral status or tumor marker are not compatible for hcc , a more aggressive diagnostic work - up should be needed . | inflammatory myofibroblastic tumor ( imt ) of the liver is a very rare lesion that has radiologic similarity with malignant liver tumor .
differential diagnosis of imt from a malignant lesion of the liver is very important because surgical resection is not mandatory for imt .
lipiodol computed tomography is a very sensitive and specific diagnostic tool for hepatocellular carcinomas ( hcc ) .
herein , we describe a case of imt that had dense lipiodol uptake in the tumor and mimicked hcc . to our knowledge , previously ,
only one case of imt with dense lipiodol retention has been reported . |
a 12-year - old girl visited the emergency medical care center of busan paik hospital due to chest pain and dyspnea . a chest x - ray revealed a large left pneumothorax ( fig . 1a ) . after the insertion of a chest tube in the left thoracic cavity , she was referred to the department of thoracic and cardiovascular surgery of busan paik hospital for further treatment . after two days , she underwent high - resolution computed tomography ( hrct ) to identify the presence of other pulmonary lesions . intraoperatively , a large bulla measuring approximately 1.5 cm with multiple small bullae surrounding it was found ( fig . 2 ) . we resected the bullae using a 60 mm-4.8 mm stapler . after inserting the chest tube ( 16 fr ) the wedge resection specimen contained the bulla and atypical adenomatous hyperplasia ( aah ) , which was found incidentally ( fig . the resection margin was not involved and the safety margin was 0.7 cm from the resection margin . the patient is still undergoing follow - up , and no evidence has been observed of any other ground - glass opacity lesions . the world health organization defines aah as a focal proliferation of atypical cells lining the involved alveoli or respiratory bronchioles . it is usually 0.5 cm or less and located in the peripheral area of the lung . these lesions are mostly detected as incidental findings in lung tissue resected due to other problems , especially primary lung cancer . the incidence of aah has been reported to be approximately 9%21% in patients with primary lung cancer , while the incidence of aah in patients without lung cancer has been reported to be 4%10% . most nodules and masses are reactive lesions or congenital malformations , such as bronchogenic cysts , pulmonary sequestration , congenital pulmonary airway malformations , and congenital lobar overinflation . the incidence of primary lung tumors in children has not been established because most of the literature contains individual case reports and diagnosis - specific case series . in the texas children s hospital , the total number of surgical pathology specimens was 227,655 during a 25-year period , and only 3,980 surgical specimens ( 1.7% ) were designated as originating from the trachea , bronchus , or lung , including both biopsies and resections . cohen and kaschula reported a ratio of primary tumors to metastatic tumors to non - neoplastic lesions of 1:5:60 . previously reported cases of aah in children occurred in combination with a congenital cystic adenomatoid malformation or meta - static osteosarcoma of the lung . however , the number of reported cases is very small and no cases of solitary aah in a child have been reported . the youngest case of solitary aah was a 17-year - old male in japan , reported in 2003 . histologically , aah is a focal proliferative parenchymal lesion , and the alveolar septa are lined by rounded low columnar cells with round to oval nuclei . mild to moderately atypical cells , hyperchromatic nuclei , and prominent nucleoli are usually seen , with an increased nuclear - cytoplasmic ratio . in addition , it is often difficult to differentiate aah from adenocarcinoma in situ ( ais ) only based on small biopsy specimens or frozen sections . however , some features are helpful in differentiating between aah and ais in specimens . at low power additionally , ais exhibits a more abrupt transition to the adjacent lung parenchyma , and the presence of goblet cells is a strong indicator of ais . however , aah usually measures less than 5 mm in diameter and exhibits polymorphic cellular proliferations of variably sized and shaped cuboidal cells . in the periphery of the lesion , blending with normal alveolar lining cells no guidelines exist for the surgical or medical treatment of patients without cancer who are incidentally found to have aah , and no consensus exists regarding the risk of their lesions developing into invasive adenocarcinoma . in this case , the lesion had a sufficient resection margin relative to the nodule size , so we decided that no further resection was necessary . recently , imaging techniques have undergone continual improvement , resulting in the increased diagnosis of aah . furthermore , most authors have agreed that a genetic correlation is present between aah and ais , but this is also controversial . if solitary aah was not exceedingly rare in childhood , it would be easier to determine whether aah slowly progresses into ais or no relationship exists between aah and ais . this case is the youngest patient with solitary aah who has been reported , and continuous follow - up will be necessary . in the future , we expect appropriate treatment plans to be developed based on the accumulation of more data . | atypical adenomatous hyperplasia is a premalignant lesion reflecting a focal proliferation of atypical cells .
these lesions are usually observed as incidental findings in lungs that have been resected due to other conditions , such as lung cancer .
we report the youngest case of atypical adenomatous hyperplasia on record in a 12-year - old girl . in this patient ,
the lesion was found in association with pneumothorax . |
acute generalized exanthematous pustulosis ( agep ) is a rare acute reaction that is drug - induced in 90% of the cases , characterized by a widespread , sterile pustular rash . cefepime is a fourth generation cephalosporin antibiotic used to treat febrile neutropenia , severe infections related to the urinary tract , skin , nosocomial pneumonia , brain abscess , and intra - abdominal and septic lateral / cavernous sinus thrombosis . a 67-year - old man with renal failure who had been on dialysis during the last 2 years and with an 8-year history of cardiac insufficiency was admitted to the hospital complaining of 6 days of diarrhea . the patient was taken to the semi - intensive care unit and treated with ciprofloxacin . as a consequence , his long - term medications had not been changed and consisted of acetylsalicylic acid , furosemide , captopril , carvedilol and clonazepam . on the seventh day , the patient became dyspneic and his chest radiograph showed a left lower lobe opacity . treatment for nosocomial pneumonia was promptly initiated with cefepime ( 1 g / day ) . five days later , he presented with a pruritic , erythematous , maculopapular eruption affecting the abdomen , neck and skin folds . one day later , he developed disseminated pustular lesions ( fig . 1 ) and his temperature was 37c . laboratory exams evidenced c - reactive protein 136 mg / l , white blood cells 14,700 cells/l ( normal 3,50010,500 cells/l ) with 11,995 cells/l neutrophils ( normal 1,7008,000 cells/l ) . histology showed a toxic pustuloderma with spongiform subcorneal pustules , edema in the papillary dermis and perivascular inflammatory infiltrate consisting of neutrophils ( fig . after withdrawal of cefepime and introduction of imipenem , the disseminated skin nonfollicular pustules cleared within 4 days following a desquamation . the patient denied previous adverse reaction to other drugs and no personal or family history of psoriasis was evident . agep is a disease characterized by the rapid onset of many sterile , nonfollicular pustules usually arising on an edematous erythema and frequently accompanied by leukocytosis and fever . skin symptoms usually arise rapidly after an insult and resolve spontaneously ( within a few days ) . agep often starts predominantly in intertriginous areas or on the face , spreading rapidly to the trunk and lower limbs . the mean duration of the pustules is 9.7 days , and an annular desquamation typically follows for a few days . complications are rare [ 1 , 3 ] . the agep validation score of the euroscar study group has been used to establish the diagnosis . a score between 8 and 12 for agep is a definitive diagnosis ( table 1 ) . the case score was 11 , according to the validation score of the euroscar study group ( table 2 ) . the main differential diagnosis of agep is pustular psoriasis . because the pustules clinically and histologically resemble the lesions of pustular psoriasis and because in a number of reports patients had a history of plaque psoriasis , some authors assume that agep is nothing more than an acute exacerbation of psoriasis caused by a variety of exogenous triggers however , many studies strongly suggest that agep is not associated with psoriasis [ 1 , 5 ] . up to now agep has been attributed to a variety of causes such as viral infections , chlamydia pneumoniae infection or hypersensitivity to mercury , but the skin reaction is primarily an adverse response to drugs . antibiotics , other than cefepime , have been implicated as the causative agents in 80% of individuals . in this group , the present case of agep has well defined criteria , and because correct diagnosis generally leads to spontaneous resolution once the causative drug is withdrawn , clinicians should keep the possibility of this cutaneous drug reaction in mind . | acute generalized exanthematous pustulosis ( agep ) is a rare cutaneous rash characterized by widespread sterile nonfollicular pustules .
cefepime is a fourth generation cephalosporin , used to treat severe infections .
a 67-year - old man was admitted with acute gastroenterocolitis . on the seventh day
, the patient developed a nosocomial pneumonia and cefepime was initiated . on the fourth day of cephalosporin treatment , he presented with a maculopapular , pruritic eruption affecting the face , neck , abdomen and limbs .
one day later he developed disseminated pustular lesions and his temperature was 37c .
laboratory analysis evidenced leukocytosis and skin biopsy showed subcorneal pustule , edema in the papillary dermis , perivascular inflammatory infiltrate consisting of neutrophils , leukocytoclasia and red cell extravasation in the epidermis .
cefepime was suspended and within 4 days the non - follicular pustules cleared following a desquamation .
agep is a disease attributed to a variety of causes , but in 90% of the cases it is due to an adverse drug reaction .
antibiotics are implicated in 80% of these cases , mostly penicillins and macrolides .
there are few cases associated with cephalosporins .
it is very important to consider agep in cases of acute pustular rashes and drugs should be investigated as causative agents . |
interference in modern immunoassays caused by heterophilic antibodies ( ha ) is well documented . according to various published reports , in the general population these antibodies can be found in up to 40% of clinical samples . in many cases , however , for reasons not well understood , they can cause false - positive results in the analysis of different markers , even in the absence of antigen . interference by ha is described in the analysis of different tumor markers ( e.g. , prostate - specific antigen [ psa ] , human chorionic gonadotropin , alpha - fetoprotein , cancer antigen 125 , and calcitonin ) , infectious diseases , hormones , drugs , and cardiac markers ( e.g. , troponin - i ) . in this report , we describe an unusual case of a repeated spurious elevation of psa possibly caused by ha . a 52-year - old man presented with repeated elevation of psa serum values ( initial value , 19.7 ng / ml ) . during 2 years , the patient had a progressive increase in psa levels reaching 108.7 ng / ml ( psa doubling time , 9.8 months ; psa velocity , 3.6 ng / ml / mo ; fig . the results of a digital rectal examination were not suspicious for prostate cancer and the results of three transrectal ultrasound - guided biopsies performed were all negative . moreover subsequently , a new serum test was performed in another laboratory using a chemiluminescent enzyme immunoassay ( chl ) . contrary to the previous analysis , the new recorded psa value was 1.02 ng / ml . this number was confirmed by a second determination . as a result of the discrepancy in values , a false - positive result of the initial psa tests was suspected . to evaluate the possibility of an interfering factor in the immunoassays , we studied the same sample divided in 3 aliquots that were analyzed by three different laboratories using different techniques ( table 1 ) : elisa ( drg psa equimolar , drg international inc . , nj , usa ; psa values were 110.3 ng / ml and 114.1 ng / ml ) , immunochromatography ( vedalab psa - check , vedalab , alencon , france ; psa values were 115.7 ng / ml and 98.7 ng / ml ) , and chemiluminescence ( maglumi total psa , snibe diagnostic , shenzhen , china ; psa values were 0.8 ng / ml and 0.9 ng / ml ) . because the medical literature describes the existence of spurious high psa concentrations because of ha , a possible test interference was investigated by two different laboratories . rheumatoid factor ( rf ) and human antimouse antibodies ( hama ) were studied as possible causes of falsely raised psa levels . the value of rf ( 24 mcg / l ) was determined by elisa . on the basis of the preceding results , rf and hama values could not explain the observed interference . despite our failure to validate our working hypothesis by measuring rf and hama levels , we can not rule out that other serum ha may contribute to causing false - positive psa determinations if the analysis is not performed by use of the chemiluminescence technique . most of them are natural antibodies with polyspecific characters that are derived from b cells ; less frequently they are auto - antibodies . it is rarely possible to determine the etiology of ha , but their appearance is often described after contact with animals or after therapeutic treatments with drugs containing animal immunoglobins ( e.g. , iatrogenic immunization , in vivo diagnostic tests , or immunoglobin therapy ) . within specific antianimal antibodies , there exist different types : hama , human antirabbit antibodies , and human antigoat antibodies . rf is an auto - antibody that can also have hama - like activity , but its concentration in plasma is not high enough to cause significant interference . levinson and miller reported that ha interference in a healthy population is mainly due to natural polyspecific and idiotypic antibodies . by contrast , in allergic or diseased patients , the auto - antibody - type polyspecific or rf may be found more frequently . the prevalence of spurious elevated psa values is around 0.3% . there are eight cases in the medical literature of falsely elevated psa due to ha . six of these patients were diagnosed with prostate cancer and continued to have detectable false values of psa after radical prostatectomy . in some of these patients , an unnecessary salvage treatment was performed . in the other two patients , the interference was detected during the screening , presenting with significantly high psa values ( up to 83 ng / ml ) , but only one patient did not receive an unnecessary therapeutic treatment . in this case , we employed three " sandwich " immunoassays . these techniques use two monoclonal anti - psa antibodies : a capture antibody ( immobilized on a solid phase ) and a detector antibody coupled to a signal transducer , such as an enzyme ( elisa ) or a chl . in elisa the intensity of the color produced is measured by spectrophotometry and indicates the amount of psa in the sample . in chl , there is an emission of light as the result of a chemical reaction and this intensity is also measured . the presence of ha links capture and detector antibodies in the absence of the antigen , creating a test interference and a false - positive result . the chl technique is faster , is less expensive , and has higher sensitivity than elisa . it may be more accurate at detecting false - positive results of elevated psa due to ha , as was observed in the patient reported previously . the simplest approach is to analyze the sample in another laboratory using a different formulation . another option for removing or identifying ha is the use of blocking agents . with use of this technique , the incidence of ha interference has been reduced from the 2%-5% observed in unblocked assays . unfortunately , those investigators were unable to completely eliminate the problem because the antibodies have significant polyclonality and natural variability . therefore , it is complicated to identify the specific type of ha responsible for the interference , but in clinical practice this point is not essential . in the case described herein , despite our efforts to find the ha responsible for the observed interference , we were unable to obtain a positive outcome . in our case , changing the analysis technique was sufficient to confirm that the initial psa value was a false - positive result . to the best of our knowledge , this is the second case in the medical literature reporting spurious elevation of psa values diagnosed before unnecessary therapy . we recommend a close communication between the urologist and laboratory staff in cases in which the results do not correlate with the clinical scenario in order to avoid unnecessary overtreatment by misdiagnosis . | heterophilic antibodies are human immunoglobulins directed against various animal antigens .
they can produce false - positive results in the analysis of different tumor markers , including prostate - specific antigen .
this interference can lead to misdiagnosis , unnecessary tests , and overtreatment in some cases .
we present herein the case of a 52-year - old man with repeated spurious elevation of prostate - specific antigen , reaching levels of 108.7 ng / ml , that were suspected to be caused by heterophilic antibodies .
the interference was solved by changing the analysis technique .
real values of prostate - specific antigen were less than 1 ng / ml . |
neurological assessment remains a valuable tool for monitoring severely brain - injured patients and the need for a reliable evaluation conflicts with sedation , routinely administered to severely brain - injured patients . most available sedative agents , reducing the cerebral metabolic rate and cerebral blood flow , reduce intracranial pressure ( icp ) compared with no sedation . precise assessment and clinical modification tracking are essential for planning neuroimaging and surgical or medical interventions . for the clinical assessment of neuro critical patients , this interruption is usually short term , aimed to evaluate the patients and plan further management strategies , including the definitive sedation interruption once the clinical picture and icp are no longer a concern and is does not provoke patients ' distress and metabolic imbalance . the definitive interruption is possible once the clinical and cerebral state of the patient does not justify any sedation , whereas the brief interruption allows a neurological reassessment . the strategy of frequent sedative interruptions as well as the use of sedation algorithms allows a downward titration of sedative infusion rates over time , streamlining administration of these drugs and minimizing the tendency for accumulation . unfortunately , even if almost all of the severely brain - injured patients receive sedative agents , is in the clinical setting has received little attention . in this issue of critical care , helbok and colleagues prospectively evaluated in a small cohort of 20 sedated severely brain - injured patients the effects of a wake - up test on icp , brain tissue oxygen tension and brain metabolism . the study population was com posed of severe brain - injured patients with multiple modality monitoring . is has been considered potentially risky on 34% of the study days due to pre - existing hemodynamic instability and critical icp . the benefit of having clinical evolution clues in the more severe subset is overwhelmed by the possible negative effect on icp and , consequently , on cerebral blood flow . in this setting , multimodality monitoring and neuroimaging remain the clinicians ' guide and we have to postpone clinical evaluations that could cause secondary insults . if is could not be performed , the assessment of brainstem responses during sedation is feasible and loss of selected responses is predictive of mortality and severe disorder of consciousness . second , icp and cerebral perfusion pressure increase , usually slightly and tolerably , during is when compared with baseline levels recorded during sedation . in the majority of patients , these changes are mild , transient and acceptable and do not preclude repeating is in the neurointensive care setting . nevertheless , is is a stressful condition . third , the price paid in some patients is too high and unacceptable . in a subgroup of patients with lower cerebral compliance , is induced marked icp and cerebral perfusion pressure changes that could potentially negatively impact on the injured brain . those patients should be excluded from repeated is to assess the neurological status , and information should instead be gathered from other multimodality monitoring methods in combination with neuroimaging . of all is events performed , the trial had to be stopped frequently ( that is , in one - third ) due to icp surge , agitation or systemic desaturation . in the aborted is trials , a decrease in brain tissue oxygen tension and a tendency to brain metabolic distress were observed . one of the major strengths of daily awakening trials is the additional information gained from a reliable clinical assessment in those patients who tolerate is . unfortunately this was not the case : evidence for a new focal neurologic deficit was found in only one ( 2% ) patient with known cerebral vasospasm who developed motor weakness of the lower limb . given the current little knowledge about the benefits of is in brain - injured patients , it is extremely important to adopt multiple monitoring modalities in neurocritical care in order to escape is in those patients who will potentially be harmed by this procedure . once the clinical condition will improve , sedation needs to be tapered and suspended as soon as possible . studying the phenomena of severely brain - injured patients is similar to a nightly sail on an uncharted sea . we can find some important landmarks with the neurological exami na tion in less severe patients . but when the night is without stars ( that is , the neurological evaluation with is can not be safely performed ) we need to rely on multi modality neuromonitoring . th is monitoring needs to be in place and interpreted , helping us to find the correct way to treat the patient , taking decisions - including the one of performing is when the patient is improving - and , finally , stopping sedation . | the need for a reliable neurological evaluation in severely brain - injured patients conflicts with sedation , which is routinely administered .
helbok and colleagues prospectively evaluated in a small cohort of 20 sedated severely brain - injured patients the effects of a wakeup test on intracranial pressure ( icp ) , brain tissue oxygen tension and brain metabolism .
the test has been considered potentially risky on 34% of the study days . when the test is performed , icp and cerebral perfusion pressure increase , usually slightly , except in a subgroup of patients with lower cerebral compliance where marked icp and cerebral perfusion pressure changes were recorded . in this cohort ,
the information gained with the wake - up test has been negligible . given the current little knowledge about the benefits of interruption of continuous sedation in brain - injured patients , it is extremely important to adopt multiple monitoring modalities in neurocritical care in order to escape wake - up tests in those patients who will potentially be harmed by this procedure .
once the clinical condition will improve , sedation needs to be tapered and suspended as soon as possible . |
ewing 's sarcoma ( es ) is a rare malignant small round cell tumor that primarily affects the skeletal system . it accounts for 4 to 10% of all types of bone cancer , with long bones and pelvis being the most common locations . it affects mainly adolescents and young adults and is rarely seen before the age of 5 and after the age of 30 . clinically , this tumor has an aggressive behavior characterized by rapid growth and high probability of micrometastasis at diagnosis . an 11-year - old girl presented to department of oral and maxillofacial surgery , yenepoya dental college and hospital , deralakatte , mangalore , with the complaint of swelling on the left side of the face [ figure 1 ] . the swelling started 6 months back which gradually enlarged to present size . on examination , extra orally a diffuse swelling was seen on left side of face which extended superiorly up to ala tragus line , anteriorly from about 2 cm posterior to commisure of mouth to angle of the mandible . intra oral examination revealed mixed dentition and swelling extending from left mandibular canine to left mandibular second molar [ figure 2 ] . on palpation , bi - digital palpation revealed expansion of the buccal plate , no perforation of buccal / lingual cortex and no paresthesia of mucosa or lower lip were seen . grade iii mobility was seen on all the teeth from left mandibular canine to left mandibular second molar , which were non tender on percussion . the intra oral periapical radiograph shows radiolucency near the mandibular teeth [ figure 3 ] . intra oral periapical radiograph showing radiolucency near the mandibular teeth the ct scan findings revealed the following : an ill - defined expansile cystic lesion was seen in the body of the mandible on left side and also thinning and erosion of buccal cortex was also noticed [ figure 4 ] . the results of hematological and biochemical investigations were within normal limits . under local anesthesia , extraction of the mobile teeth left mandibular first molar was performed and the tissue sample obtained through the extraction socket was sent for histopathological examination . extracted tooth specimen for biopsy on microscopic examination , h and e stained sections showed sheets of uniform , small , round cells arranged in diffuse pattern , with indistinct outline , scanty cytoplasm , and well - defined nuclear outline , with round to oval nucleus and inconspicuous nucleoli . a diagnosis of malignant small round cell tumor was made based on the above histopathological findings . histopathological picture a panel of immunohistochemical markers namely cd99 , cd3 , cd20 , chr , mpo , desmin , and syn were used to rule out other small round cell tumors . tissue sections showed positive expression for cd99 ( mic2 ) , with characteristic membranous pattern , confirming the diagnosis of ewing 's sarcoma bone scan was also advised which revealed reduced uptake over the body of left mandible without rim of enhanced uptake . a surgical procedure was performed to resect the malignant tissue with generous surgical margins and to reconstruct the resulting defect . intraoperative surgical pictures the postoperative panoramic radiograph revealed a well - aligned reconstruction with intact plating ( 6 months post operative orthopantamograph ) adjuant chemotherapy comprised 20 weeks ( 3 cycles ) of treatment with a four - drug regimen which were vincristine [ vc ] , dactinomycin [ ac ] , cyclophosphamide [ cp ] and doxorubicin [ ad ] after surgery . ewing 's sarcoma ( es ) is a rare malignant small round cell tumor that primarily affects the skeletal system . it accounts for 4 to 10% of all types of bone cancer , with long bones and pelvis being the most common locations . it affects mainly adolescents and young adults and is rarely seen before the age of 5 and after the age of 30 . clinically , this tumor has an aggressive behavior characterized by rapid growth and high probability of micrometastasis at diagnosis . es is a malignant neoplasm that primarily affects long bones of the extremities with nearly 50% of reported cases involving the femur and pelvis . the majority of the patients affected are between the ages 5 and 20 , whereas the disease is distinctly uncommon in individuals before age 5 and after age 30 ( braz dent ) . es arising from the bones of the head and neck region is exceedingly uncommon . when it occurs in the jaw , mandible is more frequently affected than the maxilla . modern treatments are based on combined modality of treatment : local therapy ( surgery and/or radiotherapy to the main tumor ) followed by chemotherapy(for management of micrometastasis ) . the present case was positive for cd99 , vimentin and desmin and negative for other immunomarkers , leading to a diagnosis of es . if root amputation surgery is performed due to an extensive radiolucent lesion , histopathological evaluation should be mandatory . primary bony reconstruction bears the risk of second intervention when dealing with es in the mandible . | ewing 's sarcoma is a malignant tumor of bones that primarily affects children and young adults .
the true origin of this small round cell lesion still remains controversial .
it was originally described by james ewing in 1921 as arising from undifferentiated osseous mesenchymal cells ; however , recent studies suggest that ewing 's tumor might be neuroectodermally derived from various degrees of differentiation of the primitive neural tissues
. this paper reports a rare case of es of the mandible in an 11-year - old girl , which had been previously misdiagnosed and treated as a dental abscess . in the clinical examination , a hard immobile expansive mass of 2 cm diameter
was observed on the left side of the mandible .
radiographic examination revealed a diffuse radiolucent lesion with ill - defined borders and wide vestibular bone plate destruction .
microscopically , the tumor was composed by monotonous small round cells that exhibited immunoreactivity for cd99 , vimentin and desmin .
surgical resection of mandible followed by mandibular reconstruction was adopted .
the patient was subjected to multiagent chemotherapy with vincristine [ vc ] , dactinomycin [ ac ] , cyclophosphamide [ cp ] and doxorubicin [ ad ] ) . |
congenital , traumatic or extrinsic causes can lead to paraplegia ; some of these are potentially reversible and others are not . paraplegia can cause hip flexion contracture and consequently , pressure sores , scoliosis , and hyperlordosis . scientific literature contains many studies about children with hip flexion related to neurological diseases , mainly caused by cerebral palsy ; only few papers focus on this complication in adults.1 in this study , we report our experience on surgical treatment of a 20-year - old female with paraplegic fixed flexion deformity of both hip joints of 15 years duration secondary to an irrecoverable spinal cord disease , with complete motor and sensory loss and multiple pressure sores . a 20 year old college going female student was admitted in our institution with a gradual onset of longstanding flexion deformity of 120 of both hip joints . she was paraplegic for last 15 years due to irrecoverable spinal cord disease with complete motor and sensory loss . the patient had grade zero power in both her lower limbs with complete anesthesia of both the lower limbs . she was unable to sit and lie on her back , slept on her legs [ figure 1a ] . the pressure sores were mainly on the weight bearing areas , which included the knees and the lateral part of the upper thigh in this case . clinical photographs showing ( a ) patient can not lie on her back ( b ) patient rests on her chest and elbow ( c ) pressure sores at the back of knee ( d ) patient is unable to lie on her back clinical photograph ( a ) x - ray pelvis with both hips and pelvis after surgery showing proximal femoral resection ( b ) clinical photograph immediate postoperative showing patient lying supine ( c ) clinical photograph showing comfortable wheelchair life ( d ) clinical photograph showing sitting balance restored the resection was done adequately through posterior approach without anesthesia . both the hips were done in one sitting alternatively in right and left lateral positions . the resection was approximately about 4 inches on the both sides , which was assessed intraoperatively till the deformity was corrected . in postoperative period , no splints or plaster was used , and the patient was immediately put on her back and in wheelchair after removal of stitches that is , 2 weeks [ figure 1b ( b - c ) . in short time , she started sitting on her buttocks , led a comfortable wheelchair life with a sitting balance [ figure 1b ( d ) ] . there was no recurrence of flexion deformity in either hip up at 6 weeks , 3 months , 6 months , 1 year , 2 years , and 3 years followup . hip flexion contracture beyond 90 in adult paraplegic patient is a relatively difficult condition to treat because of the marked contracture of hip flexors in addition to blood vessels and nerves anterior to hip . five cases of flexion contracture of the hip in adults have been reported by nicodemo et al.1 in their study , which were either treated by girdlestone or replacement arthroplasty or by myoarthrolysis . ackerly et al . in their study reported their experience with proximal femoral resection in 12 paraplegic children with spastic and painful dislocation of hip.2 our study is unique because our patient had painless flaccid paraplegia with complete sensory - motor loss with a longstanding flexion contracture of both hip joints . michaelis3 had good results with iliopsoas and obliquus externus myotomy ; graham et al.4 described 3 cases of recurrent dislocation of the hip in adult paraplegics successfully treated with open reduction and bone block augmentation of the acetabulum ; becker et al.5 focused on periarticular ossification in paraplegics , reporting the results of six patients treated with a total hip replacement . he recommends avoiding femoral head resection in favor of total hip arthroplasty even in complete paraplegia , as the former has the risk of posterior trochanteric dislocation and consequent formation of pressure sores . however , on the other hand , in case of hip replacement , high dislocation rates , risk of infection ( especially in presence of pressure sores ) , osteoporosis , loosening risk , and blood loss must be taken into account . in all these cases , flexion contracture was < 90. ackerly et al.2 in their study reported their experience with proximal femoral resection in seven quadriplegic nonambulatory children with spastic painful dislocation of hip.2 the operative technique of castle and schneider ( 1978 ) was employed . the proximal femur was exposed through a lateral approach , extraperiosteally dissected , and resected below the level of the lesser trochanter . the capsule was sutured over the acetabulum and femoral stump was closed by suturing the vastus lateralis over it . he performed this technique due to pain secondary to dislocation of the hips and through lateral approach with minimal resection below the lesser trochanter . we are reporting a case of a young college going girl who presented with longstanding flexion contracture of both hip joints due to irrecoverable spinal cord disease with complete motor and sensory loss . she was unable to sit and lie on her back , slept on her legs . she underwent bilateral proximal femoral resection through posterior approach without anesthesia . in our patient , proximal femoral resection , until fully straight hip joint , was accomplished through posterior approach without anesthesia . both hip joints were straight immediately after the operation , and she could lie on her back . after 2 weeks she started sitting on her buttocks , led a comfortable wheelchair life with a good sitting balance . there is no incidence of proximal femoral migration or heterotopic ossification . proximal femoral resection thus offered a sound solution to an apparently difficult orthopedic condition of flaccid paraplegic acute hip flexion contracture , never reported before . | paraplegic flexion contracture of hip joints beyond 90 is a difficult condition to treat for any orthopedic surgeon .
there is no fixed protocol of treatment described , by and large it is individualized .
a 20 year old female presented with paraplegia for last 15 years due to irrecoverable spinal cord disease with complete sensory and motor loss of both lower extremities and was admitted with acute flexion contracture of both hip joints with trunk resting on thighs .
she underwent bilateral proximal femoral resection .
both hip joints were straight immediately after surgery and patient could lie on her back .
in a course of time , she started sitting on her buttocks , led a comfortable wheelchair life with a sitting balance .
proximal femoral resection is an effective method to treat long standing irrecoverable paraplegic acute flexion deformity of the hip joint . |
she complained of headaches , and rnm revealed a tumor mass occupying the whole hypothalamic area . she underwent a biopsy , and the histological examination showed a germinal cell tumor ( fig . 1 , fig . she was treated with radiotherapy above the hall skull and medullae with 30 and 24 gy , respectively . she did not undergo chemotherapy . at 13.58 years of age , the patient was examined at our endocrinology outpatient clinic . she had a height of 137 cm and a weight of 26.4 kg , corresponding to 3.16 sds and 4 sds , respectively , and she did not have any pubertal signs . bone age was delayed 2 years . at first analyses we found the following : hypernatremia in several days ( 165/170/159 , n = 136145 mmol / l ) ; hypercloremia ( 123/117/124 , n = 98107 mmol / l ) ; normal urine osmolality ( 630/720 , n = 300900 mosm / kg ) ; low thyroid hormones ( tsh 0.66 ui / ml , ft4 0.71 , n = 0.91.7 ng / dl ) ; high plasma osmolality ( 325/309 , n = 275295 mosm / kg ) ; low gonadotropins ( lh 0.1 mui / ml , fsh 0.51 mui / ml ) ; low acth ( 12.8 pg / ml ) for plasma cortisol level ( 6.6 g / dl ) and urine level ( 17.3 g/24 h , n = 2185 ) ; undetectable somatomedin 1 , and elevated prolactin ( 37.9 ng / ml ) . potassium and calcium levels were normal . the patient did not listen to our claims for drinking and eating , so we had to insert an endogastric tube . she began eating , drinking water , and taking medication : levotiroxine ( 0.1 g / kg / day ) , hydrocortisone ( 0.05 mg / kg / day ) , and desmopressin with dosages slowly increasing up to 0.72 mg / day . after 1 month , the tube was removed and she began eating and drinking by herself . water electrolytic balance was now completely normal as were thyroid hormones and cortisol levels . at 16 years of age she started growth hormone treatment with 0.08 mg / kg / week and growth velocity improved considerably ( from 0 cm to 5 cm / year ) . after 18 months of treatment , she is quite well , with energy , a collaborative mood and is doing her scholar works with great strength . the tumor has disappeared . after the attainment of a better stature , we hope to start estrogen therapy with the aim of getting sexual morphology and maturation . central diabetes insipidus ( cdi ) is caused by a deficiency of arginine vasopressin , an antidiuretic hormone . patients manifest polyuria , which usually is compensated with increasing water intake . however , some patients are not able to sense thirst due to hypothalamic osmoreceptor destruction and so they do not feel the need to drink . described a case of very dangerous , severe rhabdomyolysis due to an adipsic hypernatremia , and arima et al . reviewed 149 patients with cdi and found 23 patients with adipsia . a total of 6 patients died during the follow - up , and 4 of them were adipsic , and infections were pointed out as a potential final aggression . hypernatremia is an electrolytic abnormality with very difficult understanding and above all without the occurrence of thirst . in a recent national survey in denmark , . showed a prevalence rate of 23 cdi patients per 100,000 inhabitants in 5 years . timely diagnosis for initiating specific treatment is very important because damage of the central nervous system is a very probable consequence . in the presence of adipsia , diagnosis is more challenging and many times difficult to achieve . in a study by gonzlez briceo et al . of 159 patients , only 2 had hypernatremia due to adipsia . treatment includes forcing water intake because low vascular volume promotes a high sodium concentration and cellular lesion . sometimes it is necessary recurring to a nasogastric tube or a vascular infusion . in our case , we tried chlorpromazine because it seems to improve osmoreceptor sensibility , as it was proposed by malossi . in fact , with a very low dosage we reached a better patient collaboration and with cumulative desmopressin treatment we were able to hydrate the girl . rising osmotic reactivity by hypothalamic cells by chlorpromazine have been demonstrated several years ago by allen et al . . | central diabetes insipidus is a very common disorder after brain surgery or / trauma or even in the presence of brain inflammatory diseases .
polyuria and polydipsia are the clinical markers , but sometimes clinical situations are presenting with no thirst .
these are not frequent but are life - treating conditions .
diagnosis is not easy , and for this reason some cases are treated late .
we describe here a very infrequent oncological case of dangerous adipsic diabetes insipidus in a young girl who survived . |
this video is also available to watch on http://videos.springer.com/. please search for the video by the article title . the objective of this study was to identify new tactile imaging and muscle contraction markers to characterize female pelvic floor conditions . vaginal tactile imaging [ 1 , 2 ] allows 3-d quantitative elasticity assessment of pelvic floor support structures and carries a potential in assessment of surgical repair . we designed a new vaginal tactile imaging probe that images the entire vagina , the pelvic floor support structures , and pelvic floor muscle contractions . the probe has an orientation sensor , temperature sensors , and 96 pressure sensors positioned every 2.5 mm along both sides of the probe . the examination procedure includes four steps.step 1.probe insertion : this step provides the pressure responses ( p ) for vaginal anterior and posterior compartments along the entire vaginal length . we can use this information to calculate pressure gradients ( grp ) and anatomical dimensions.step 2.probe elevation : this step provides the pressure responses for the apical anterior and posterior compartments that are related to pelvic floor support structures.step 3.probe rotation : this step provides the pressure patterns for the left and right sides of the vagina ( circumferential tactile image from vaginal walls).step 4.pelvic floor muscle contractions : this step provides the muscle dynamic pressure responses ( dp ) of the pelvic floor muscle contraction recorded from opposite sides along the entire vaginal length . probe insertion : this step provides the pressure responses ( p ) for vaginal anterior and posterior compartments along the entire vaginal length . we can use this information to calculate pressure gradients ( grp ) and anatomical dimensions . probe elevation : this step provides the pressure responses for the apical anterior and posterior compartments that are related to pelvic floor support structures . probe rotation : this step provides the pressure patterns for the left and right sides of the vagina ( circumferential tactile image from vaginal walls ) . pelvic floor muscle contractions : this step provides the muscle dynamic pressure responses ( dp ) of the pelvic floor muscle contraction recorded from opposite sides along the entire vaginal length . in 2013 two patients were excluded from the data analysis because they had previously had pelvic floor surgery . the analyzed data set included 20 subjects aged from 41 to 70 years . among them four had normal pelvic floor conditions , four stage i , seven stage ii , four stage iii , and one stage iv prolapse . a standard physical examination was performed , including a bimanual pelvic examination , pelvic organ prolapse quantification ( pop - q ) , assessment of tissue rigidity , and assessment of pelvic floor muscle tone . the tactile imaging data from all examinations were reviewed in a blinded fashion with no knowledge of the subject s pelvic floor conditions to avoid bias in the data review process . the clinical information was then added to this data set after the tactile imaging data ( pressure , pressure gradients , muscle contracting response ) were finalized . one - way analysis of variance ( anova ) ( pa ) , paired t test ( pt ) , and pearson s correlation coefficients ( r ) were calculated to determine whether the various parameters showed dependence on the pelvic floor conditions . the subjects were asked to complete an assessment of comfort and pain levels for the tactile imaging procedure . we identified the following parameters as potential markers to characterize the female pelvic floor conditions . site 1 corresponds to the lower one third of the vagina and site 2 to the upper one third of the vagina . we found that nine parameters are sensitive to prolapse conditions ( p < 0.05 for one - way anova and/or p < 0.05 for t test with correlation factor r from 0.73 to 0.56 ) . these parameters demonstrate a mild - moderate correlation with women age and parity ( see table 1 ) . during step 4 part of the identified markers also demonstrates correlation with patient age and parity . a typical examination consisting of four steps takes 12 min . of the patients , 54 % classified vti comfort level as more comfortable than manual palpation , 36 % as the same , and 10 % as less comfortable than manual palpation . of the patients , 73 % classified vti pain as none , 24 % as mildly painful , and 3 % as a painful.table 1summary for 11 parameters identified as potential markers for pelvic floor characterizationno.marker locationmeasured / calculated valueinterpretationexamination step ( no.)one - way anova , p vs prolapse staging
t test , p vs prolapse stagingcorrelation coefficient , r vs prolapse stagingcorrelation coefficient , r vs agecorrelation coefficient , r vs parity1anterior site 1pressuretactile feedbackprobe insertion ( 1)0.0110.0060.730.170.222anterior site 1pressure gradienttissue elasticityprobe insertion ( 1)0.0890.0200.610.130.233posterior site 1pressuretactile feedbackprobe insertion ( 1)0.0080.0120.710.710.390.374posterior site 1pressure gradienttissue elasticityprobe insertion ( 1)0.0140.0200.690.400.315posterior site 1pressuretactile feedbackprobe elevation ( 2)0.0300.0130.630.520.266posterior site 2pressuretactile feedbackprobe rotation ( 3)0.350.0610.400.260.137sides site 1pressuremuscle strengthprobe rotation ( 3)0.0910.0480.660.260.118anterior site 1pressure changemuscle strengthmuscle contraction ( 4)0.1020.0570.580.240.009anterior site 2pressure changemuscle strengthmuscle contraction ( 4)0.0670.0160.560.110.3310posterior site 1pressure changemuscle strengthmuscle contraction ( 4)0.0800.0190.610.150.2111posterior site 2pressure changemuscle strengthmuscle contraction ( 4)0.0460.580.390.200.27 summary for 11 parameters identified as potential markers for pelvic floor characterization our findings suggest that the tactile imaging markers such as pressure , pressure gradient , and dynamic pressure response during muscle contraction may be used for further quantitative characterization of female pelvic floor conditions . h. van raalte : grant / research support ; shareholder of advanced tactile imaging , inc . v egorov : grant / research support ; ceo and shareholder of advanced tactile imaging , inc . | introduction and hypothesistactile imaging ( ti ) is the high - definition pressure mapping technology which allows recording pressure patterns from vaginal walls under applied load and during pelvic floor muscle contraction .
the objective of this study was to identify new tactile imaging and muscle contraction markers to characterize female pelvic floor conditions.methodsthe study subjects included 22 women with normal and prolapse conditions .
they were examined by a new vaginal tactile imaging probe that images the entire vagina , the pelvic floor support structures , and pelvic floor muscle contractions.resultswe identified 11 parameters as potential markers to characterize the female pelvic floor conditions .
these parameters correlate with prolapse conditions , patient age , and parity.conclusionsour findings suggest that the tactile imaging markers such as pressure , pressure gradient , and dynamic pressure response during muscle contraction may be used for further quantitative characterization of female pelvic floor conditions.electronic supplementary materialthe online version of this article ( doi:10.1007/s00192 - 014 - 2549 - 9 ) contains supplementary material , which is available to authorized users .
this video is also available to watch on http://videos.springer.com/. please search for the video by the article title . |
urethral duplication ( ud ) is a rare congenital anomaly and is characterized by two urethras , which may be either partial or complete . the females ud is classified according to plane ( frontal or sagittal ) of duplication into different types : ( 1 ) double urethra and double bladder , ( 2 ) double urethra with single bladder , ( 3 ) accessory urethra posterior to the normal channel , ( 4 ) double proximal urethra and single distal urethra , and ( 5 ) single proximal urethra and duplicated distal urethra . we are discussing a case of type five females ud presented as ambiguous genitalia with incontinence over continence . the child had normal voiding and dribbling of urine from the abnormal perineal orifice ( vaginal orifice ) and had abnormal genitalia ( hypertrophied clitoris ) . physical examination revealed posterior displacement of the vaginal introitus and prominent clitoris with a narrow opening at the tip . no scrotal or labial structures were present and the perineum appeared flat and fused [ figure 1 ] . on further evaluation , the child had female karyotype ( 46xx ) and sonography revealed normal mullerian structures with normal kidneys and bladder . retrograde urethrogram ( rgu ) was attempted through the opening at the clitoris but catheter could not be passed through it . subsequently , evaluation under anesthesia , cystogenitoscopy , and contrast study was done ; we could identify one tiny opening at the clitoral tip , which was very narrow and only admitting guide wire for 2 cm . during vaginoscopy , a urethral opening was present on the anterior wall of the vagina ( 2 cm proximal to vaginal orifice ) and it ( urethra ) led into the normal bladder . urethrogram and genitogram were done under fluoroscopy and delineated the anatomy , suggestive of ud [ figure 1 , inset ] . the dorsal urethra was dissected , the distal 2 cm of the urethra was very narrow and atretic hence excised . the proximal portion of the dorsal urethra was well developed ( good caliber ) , joined to the ventral urethra near its confluence into the vagina [ figure 2a ] . the rent in the anterior vaginal wall and ventral urethra was repaired and soft tissue interposed in between the two suture lines [ figure 2b ] . the distal continuity of the ventral urethra with the dorsal urethra ( good caliber ) was maintained and neourethra ( i.e. , proximal part consisted of the ventral urethra and the distal part consisted of the dorsal urethra ) reconstructed [ figure 2 ] . postoperative period was uneventful and the child was discharged after removal of all the catheters . on subsequent follow - up , the child had developed urethrovaginal fistula of 0.5 cm 0.5 cm size , 2 cm distal to the bladder neck , and presented as postmicturition dribbling . the child is under follow - up and continent for urine ( no urinary dribbling ) at last visit with normal upper tracts . ( a ) hypertrophied clitoris ( black arrow ) , flat perineum ( white arrow ) , and vaginal orifice ( blue arrow ) . u : urinary bladder , v : vagina , r : rectum , du : dorsal urethra ( red color ) , vu : ventral urethra ( a ) distal dorsal urethra ( black arrow ) and proximal dorsal urethra ( white arrow ) . the vagina ( blue arrow ) and its confluence ( yellow arrow ) with the ventral urethra . there is no embryological theory that explains all ud abnormalities . currently , widely accepted theory proposed by patten et al . suggested an abnormal relationship between the lateral anlagen of the genital tubercle and the ventral end of the cloacal membrane . indications for surgery include annoying symptoms such as a double stream ( most common ) , urinary incontinence , ambiguous genitalia , perineal sinuses , urinary obstruction or infection , and associated genitourinary anomalies . the preoperative evaluation requires a variety of radiological investigations and endoscopic procedures to define the anatomy . these include voiding cystourethrogram ( vcug ) , rgu , intravenous urography , sonourethrography , magnetic resonance imaging , and cystogenitoscopy . vcug and rgu should be carried out in lateral projections for visualization of the size , shape , and position of the two channels . patients with ud can be asymptomatic and in such situation a conservative approach is acceptable . the range of management includes observation to trivial intervention like meatotomy / urethral dilatation to complex urethral reconstruction in severe cases . less commonly , openings may lie collaterally ( side by side ) in the frontal plane . it is imperative to be aware of anatomy , continent status , and caliber of both the urethras during the preoperative evaluation . usually the ventral urethra is continent and good caliber compared to the dorsal urethra ; similar findings were present in the index case . during surgical planning , the facts to be considered are continent urethra should be preserved , good caliber urethra should be used for reconstruction purpose , and excision of accessory urethra should be accomplished . hence , we had started investigations on two lines : ectopic ureter and disorder of sex development . after cystogenitoscopy and contrast study , diagnosis was confirmed and anatomy of both urethras was well delineated . the incontinence was might be pseudo - incontinence because of the vaginal voiding , i.e. child might have been passing urine into the vagina during micturition and probably led to postvoid vaginal dribbling . we had planned to preserve ventral urethra as it was a continent and well developed . moreover , well - developed part of the dorsal urethra had been used for urethral reconstruction to achieve adequate length . the occurrence of urethrovaginal fistula in the postoperative period was might be because of inadequate soft tissue interposition or overlapping suture line or due to vascularity - related issues . as urethrovaginal fistula was distal to the bladder neck area , the eventual continence status was not compromised . ud is a rare cause for ambiguous genitalia in female and requires a high degree of clinical suspicion to diagnose the condition . the optimum management must be individualized and requires thorough investigations to delineate anatomy , which is crucial for surgical planning with the aim to provide excellent functional and cosmetic outcome . | urethral duplication ( ud ) in females is a rare congenital anomaly and requires a high degree of clinical suspicion for diagnosis .
the preoperative evaluation requires thorough investigations to delineate anatomy which is imperative for surgical reconstruction to provide excellent functional and cosmetic outcome .
we describe the successful management of a 6-year - old girl with ud ( presented as ambiguous genitalia and urinary incontinence ) along with a review of pertinent literature . |
little is known about longus colli tendinitis ( lct ) , a rare inflammatory condition believed to be caused by the deposition of calcium hydroxyapatite crystals in the longus colli muscle . also known as calcific tendinitis of the longus colli muscle or retropharyngeal tendinitis , lct is characterized by the acute presentation of severe neck pain , dysphagia , torticollis , and/or a painful restriction of neck movement . its presentation may resemble more serious conditions requiring immediate treatment , such as meningitis , retropharyngeal abscess , foreign body aspiration , or traumatic injury . patients also typically present with mild leukocytosis , which should prompt investigation to first rule out infectious etiologies . characteristic radiographic findings of the neck include calcifications in the superior longus colli muscle tendons at the level of c1 or c2 , which confirm the diagnosis . the incidence of lct has been reported to be 1.31 per 100,000 person - years but may actually be higher due to the belief that the disorder is underdiagnosed . therefore , we would like to make practitioners aware that lct is an important etiology for neck pain that must always be included in the differential diagnosis for acute neck symptomatology . a 44-year - old female patient with a history of gastric ulcers , gastritis , gastric polyps , schatzki ring , and hiatal hernia presented with the complaint of progressive dysphagia to solid foods . this dysphagia first developed after an incident of food aspiration 4 weeks prior to presentation . three weeks after this event , she experienced persistent odynophagia with left - sided occipital headaches and neck pain radiating to the left ear and mastoid process . physical examination was remarkable for decreased neck range of motion with flexion and extension and bilateral neck soft tissue tenderness to palpation . after an infectious etiology had been ruled out , an upper gastrointestinal series with barium swallow confirmed the patient 's known schatzki ring and hiatal hernia . a ct scan , mri , and mra of the brain were negative for abnormalities ; however , a ct scan of her neck was positive for calcified longus colli tendons just interior to the anterior ring of her c1 vertebrae ( fig 1 , fig 2 ) . the patient underwent a trial of oral nonsteroidal anti - inflammatory drugs ( nsaids ) and corticosteroids that quickly improved her dysphagia and odynophagia , which soon also resolved . this case presentation emphasizes the importance of having a vast differential diagnosis for relatively common complaints , such as neck pain and dysphagia . it is important to carefully contemplate these differentials in order to not misdiagnose less common yet benign etiologies for these complaints , such as lct . the longus colli muscle is a prevertebral muscle of the anterior neck that consists of a superior oblique , inferior oblique , and vertical portion . the longus colli muscle assists in cervical flexion and ipsilateral side - bending and rotation . the mean annual crude incidence of lct was found to be 0.50 cases per 100,000 person - years , with a standardized incidence of 1.31 for the age - matched population . it most commonly affects both males and females between the ages of 30 and 60 years , as seen in our 44-year - old female . the pathophysiology behind lct is explained by a reactive inflammatory process induced by calcium hydroxyapatite crystal deposition on the longus colli tendon . these calcifications , which are usually present in the superior oblique portion of the longus colli muscle at the c1c2 levels , are the cause of symptom development . the most common presenting symptoms are neck pain ( 94% ) , limited neck range of motion ( 45% ) , odynophagia ( 45% ) , neck stiffness ( 42% ) , dysphagia ( 27% ) , sore throat ( 17% ) , and neck spasm ( 11% ) . our patient displayed all of these signs and symptoms except for sore throat and neck spasm . laboratory findings typically include mildly elevated white blood cell count , erythrocyte sedimentation rate , and c - reactive protein , but this case featured only an isolated white blood cell count of 11.88 . ct is the gold standard for diagnosing lct , as it is readily available and can detect both prevertebral edema and calcium hydroxyapatite crystal deposition at the longus colli tendon [ 4 , 7 ] . mri can show prevertebral edema and corresponding effusion , but it is difficult to recognize calcific deposits [ 4 , 7 , 8 ] . this patient demonstrated longus colli calcifications in the anterior ring of the c1 vertebrae found on ct . the differential diagnosis between lct and other diseases showing similar clinical features includes meningitis , retropharyngeal abscess , neoplasm , cervical disc herniation , and traumatic fracture dislocation . the most important mimickers of lct are meningitis and retropharyngeal abscess [ 4 , 7 ] . it is critical to thoroughly work up the case in order to make a correct initial diagnosis and avoid unnecessary interventions . unlike meningitis and retropharyngeal abscesses , which are often associated with high morbidity , antibiotics use , and surgical intervention , lct is a benign , self - limiting disease . nsaids are the first - line treatment for lct and often alleviate symptoms , but in severe cases , corticosteroids and opioids may be necessary to abate acute symptom exacerbations [ 4 , 6 , 8 ] . symptoms typically resolve within a few days of treatment initiation , and the patient often becomes symptom - free after 13 weeks [ 6 , 9 ] . our patient required both oral nsaids and corticosteroids , which quickly improved her dysphagia and odynophagia within 1 day of treatment initiation . lct is a rare , unusual clinical entity believed to be underdiagnosed due to its often nonspecific presentation . increased awareness of the disease and its progressive presentation would perhaps yield more timely identification to avoid diagnostic errors and unnecessary patient and physician anxiety . therefore , it is imperative that practitioners be aware of lct as an important etiology for various presentations of unexplainable neck pain , dysphagia , and odynophagia . dominic m. colella and fiorela caldern sandoval wrote the manuscript , david w. powers and nimal patel revised the manuscript , and javier sobrado critically revised the manuscript and is the guarantor . | longus colli tendinitis ( lct ) is an acute inflammatory condition with symptoms typically consisting of acute neck pain and stiffness with or without dysphagia .
once more severe etiologies for these symptoms are ruled out , this self - limiting condition usually resolves spontaneously with nonsteroidal anti - inflammatory drugs and corticosteroids .
we present a case of lct that presented as acute neck pain , dysphagia , and odynophagia that rapidly resolved once diagnosed and treated with anti - inflammatory agents .
though exceedingly rare , lct must be considered in the differential diagnosis of acute neck pain , dysphagia , and odynophagia when more common etiologies do not correlate with the clinical presentation . |
nonmuscle - invasive bladder cancer seldom shows distant metastasis . in addition , it is extremely rare for bladder cancer staged as ta under the international union against cancer guidelines to result in distant metastasis . we present the case of a patient with a distant pulmonary metastasis of ta bladder cancer ; the patient did not have a history of muscle - invasive disease . we review the literature concerning non - muscle - invasive bladder cancer with distant metastasis and discuss management and treatment outcomes . a 66-year - old man had been diagnosed with ta bladder cancer 6 years before he complained of dry cough in july 2009 . he had a history of recurrent non - muscle - invasive bladder tumors and had therefore undergone 7 transurethral resections ( turs ) and had received intravesicular chemotherapies ( 8 cycles of weekly mitomycin - c after the 4th tur and 5 cycles of biweekly pirarubicin after the 5th tur ) . all tumors were < 1 cm , well pedunculated , and papillary . the number of tumors ranged from 1 to 6 in each respective tur . the pathological findings of all turs indicated the presence of urothelial carcinoma ( stage ta without carcinoma in situ , grade 1 or 2 ) without lymphatic or vascular invasion . the last tur in january 2009 showed the same findings as the previous turs , i.e. , there were 3 tiny papillary tumors located at the right lateral wall , at the posterior wall , and adjacent to the left ureteral orifice , and pathological examination indicated results similar to those of the previous turs . chest radiography at 6 months after the last tur detected a round nodule ( diameter , 7.5 cm ) in the right lower lung . we carefully rechecked the chest radiograph obtained in april 2008 , which showed a small nodule ( diameter , 1 cm ) in the same area . the initial diagnosis based on the computed tomography findings was primary lung cancer ( fig . the histopathologic features of the pulmonary lesion and the tur specimen of the bladder cancer were qualitatively similar . immunohistochemistry showed that both the tur specimen and the pulmonary lesion were positive for the urothelium - specific protein uroplakin ia ( up - ia ) ( fig . subsequently , he received 2 cycles of postoperative chemotherapy with the same gemcitabine and cisplatin regimen reported by von der maase et al . . although we had planned 3 cycles of chemotherapy , the patient refused the third course . his bladder cancer recurred at 3 and 8 months after chemotherapy and hence he underwent tur both times . the results of the pathological examination were the same results as for the previous turs . two and a half years after chemotherapy , chest and abdominal computed tomography once every 3 months detected no recurrence of the pulmonary lesions or other distant metastasis . for nonmuscle - invasive bladder cancer , the probability of recurrence at 5 years ranges from 31% to 78% , whereas the risk of distant metastasis is extremely low . matthew et al . reviewed cases of more than 1,000 patients treated for bladder cancer , regardless of the stage , and identified 9 patients with superficial urothelial carcinoma and distant metastatic disease ( including 3 patients with stage pta disease ) . three patients underwent radical cystectomy , and muscle invasion was not identified on pathological examination . because vessels are absent in the bladder mucosa , it is thought that ta bladder cancer does not have the potential for lymphatic or hematogenous metastasis . several iatrogenic mechanisms of metastasis in clinically diagnosed ta cancer have been postulated : microscopic invasion by an understaged tumor , intravascular dissemination of tumor cells during transurethral resection , and degeneration of the basal membrane caused by intravesicular therapy . histopathologic features of non - keratinized squamous - cell carcinoma of the lung are similar to those of urothelial carcinoma . uroplakins ( ups ) are urothelium - specific proteins and have 4 subtypes : ia , ib , ii , and iii . reported that up - ia has higher sensitivity for urothelial carcinoma than ups ii and iii . they investigated the expression of up - ia in primary and metastatic urothelial carcinoma . among primary and metastatic lesions , the detection rate of up - ia was 96.8% and 72.2% , respectively . up - ia can be a promising marker for identifying urothelial carcinoma . to the best of our knowledge , 15 cases of non - muscle - invasive bladder cancer with distant metastasis have been reported in the english literature . all patients with bone metastasis ( including 2 with stage pta disease ) died shortly after diagnosis , and 1 patient with solitary brain metastasis died less than 3 months after brain surgery . on the other hand , as indicated in table 1 , the outcomes in most patients with lung metastasis were good , except for 1 patient ( stage ta , grade 2 ) who died 1 month after diagnosis . other studies have demonstrated a good prognosis in 3 patients with ta bladder cancer and lung metastasis after a combination of lobectomy and neoadjuvant or adjuvant chemotherapy [ 7 - 9 ] . death due to pulmonary metastasis of ta bladder cancer has not been reported , because the malignant potential of the metastatic lesions is low , and metastatic lesions can be completely resected with ease . although the effects of adjuvant chemotherapy remain undetermined , some authors have reported that adjuvant chemotherapy may contribute to the removal of systemically circulating cancer cells . a combination of complete resection of the lung lesions and systemic chemotherapy may lead to good prognosis for patients with non - muscle - invasive bladder cancer . | a 66-year - old man with a history of multiple transurethral resections for recurrent bladder tumors , staged as ta according to the international union against cancer staging guidelines , presented with a complaint of dry cough .
a round nodule with a diameter of 7.5 cm was detected in the lung by chest computed tomography , and a video - assisted thoracoscopic lobectomy was performed .
pulmonary metastasis of recurrent bladder cancer was diagnosed by immunohistochemistry staining for the urothelium - specific protein uroplakin ia .
subsequently , 2 cycles of systemic chemotherapy were administered .
two and a half years after treatment , no recurrence of pulmonary lesions has been detected .
a combination of complete resection of pulmonary lesions and systemic chemotherapy may result in a good prognosis for patients with non - muscle - invasive bladder cancer . |
insects that sting to defend their colonies or subdue their prey belong to the order hymenoptera , which includes bees , wasps , hornets , yellow jackets and fire ants.1 honeybees are beneficial to man because they produce honey , which has nutritional and medicinal benefits . they are also important pollinators essential for the propagation of plants , including many agriculturally important crops.2 despite these benefits , being stung by a bee is an unforgettable painful experience and can lead to untimely death . bee stings occur as accidents or occupational exposure , especially in rural areas , within close proximity to forests,3 or in bee farms . the clinical manifestations of bee sting range from local or benign to systemic life threatening multisystem involvement , which result from allergic and/or anaphylactic to toxic reactions . bee venom contains many toxic fractions , the most important being mellitin , which alters capillary permeability , causes local pain , haemolyses red cells and lowers blood pressure.4 the venom also contains antigenic components which are capable of invoking an allergic response in the form of hypersensitivity in a significant proportion of the population , if subjected to a subsequent challenge . multiple stings , usually in excess of 100 , may result in significant haemolysis with acute anaemia and subsequent renal failure.4 a bee dies after stinging her victim , leaving the barbed end of her stinger apparatus , or ovipositor , firmly embedded in the skin.5 there are a few reports on the uncommon reactions to bee sting which include acute renal failure and haematuria.36 however , there is no documentation of bee sting in a sickle cell anaemia patient in medical literature to the best of our knowledge . it is against this background that we report an uncommon presentation of gross haematuria following multiple bee stings in a 8-year - old nigerian male with sickle cell anaemia and as well to highlight therapeutic challenge in the management . an 8-year - old nigerian male presented with a history of multiple bee stings on the head and passage of bloody urine 50 minutes prior to hospitalisation . he was initially rushed to a private clinic where he received intravenous ( i.v ) 5% dextrose saline fluid , i.v ampiclox , i.v hydrocortisone and intramuscular diclofenac before referral to our facility for further care . he is a known sickle cell anaemia patient diagnosed at the age of 8 months in a tertiary health facility . he has been in stable state of health for over 1 year prior to the bee stings . on admission , physical examination revealed extensive swelling on the head and face , he was moderately pale , febrile ( temperature 37.6 c ) , and mildly jaundiced . his laboratory investigations revealed a packed cell volume of 20% , white blood cell count of 28,700/mm3 with normal differential count . serum electrolyte urea and creatinine were within normal limits , malaria parasite test was positive ( + ) . dipstick urinalysis showed blood ( + + + + ) , other parameters were normal . he was transfused with 1 unit of fresh whole blood and was also commenced on iv antibiotics , iv antihistamines , i.v antimalarial , zinc and acetaminophen tablet . he developed incoherent speech and became delirious within 24 hours , and died after 36 hours on admission . the sight of passing overtly bloody urine is scary and needs urgent evaluation to determine its cause and institute appropriate treatment . gross haematuria following bee sting is a rare occurrence and the exact mechanism is not known . it is possible that mellitin contained in the venom might have triggered widespread alteration of capillary permeability coupled with extensive haemolyses of red blood cells , which eventually resulted in gross haematuria . the patient had features in keeping with red cell haemolysis ( moderate anaemia , thrombocytopenia , poikilocytosis , anisocytosis ) , which might have resulted from the haemolytic action of mellitin or from sickle cell disease . our patient also had other features of anaphylactic reaction to bee sting that include : local pain , fever , swelling over the head , vomiting and passage of watery stool . these effects are caused by mellitin and other substances contained in the venom like histamine , hyaluronidase , apamine and phospholipase a.78 the incoherent speech and delirious state of our patient 24 hours on admission might have been due to neurotoxic effect of apamine and hyaluronidase in bee venom.78 it is also possible that he had evolving stroke as a complication of sickle cell anaemia precipitated by the bee sting . a transcranial doppler ultrasound would have been beneficial but it was not done because we lacked the equipment in our facility . our patient did not receive i.v adrenalin for management of anaphylactic reaction because it causes vasoconstriction and might trigger or worsen vasoocclussive crises in children with sickle cell anaemia . autopsy was not performed because parents declined consent ; therefore , the cause of sudden death of our patient is not certain . it could have been caused by overwhelming toxaemia due to the venom , which has been reported to cause acute myocardial infarction in humans and myocardial necrosis in animal studies.910 this highlights the need for autopsies to be carried out routinely on every case of sudden death of unknown aetiology . clinicians should be aware that the risk of developing gross haematuria following bee envenomation appears to be increased by co - existing sickle cell anaemia . therefore , every patient with bee sting should be closely monitored for the possibility of this life threatening rare occurrence , especially if they suffer from sickle cell disease . | gross haematuria is an unusual complication of honey bee stings . herein , we report a rare case of gross haematuria following multiple honeybees stings in an 8-year - old nigerian child with sickle cell anaemia .
the patient had evidence of massive intravascular haemolysis and was transfused with a unit of fresh whole blood .
however , he died within 36 hours on admission despite medical intervention . |
the occurrence of squamous cell carcinoma in actinic keratosis is well - known and has been documented time and again . non - melanoma skin cancer ( nmsc ) is the most common variant of cutaneous malignancy , white population being the most susceptible . photo - protective effect of eu - melanin seemed to have a role to play in determining its pattern of incidence . although its worldwide incidence is skewed toward basal cell carcinoma ( bcc ) , dark - skinned indians are less prone to develop the condition . however , in the settings of genodermatoses , comprising oculo - cutaneous albinism ( oca ) , and hermansky - pudlak syndrome ( hps ) , bcc may be a relatively common finding . reduction / complete absence of melanin may lead to photosensitivity and high - risk cutaneous signs such as freckles , actinic keratosis , and bcc . here we report an indian native of oculo - cutaneous albinism , freckling , actinic keratosis , and ulcerative bcc . a 56-year - old farmer , a known case of type ia oca , presented with the complaint of discomfort in daylight , diminished vision , and a slowly progressive asymptomatic , red , raised eruption over the back for the past 10 years . he had irregular , dark , flat lesions , initially overexposed parts , which later spread to cover the whole body . some of the flat lesions turned darker in color , hard , and raised with the increasing age . skin surface examination , was conspicuous by the absence of color in the skin ( chalky white ) , hair , and eyes . multiple irregular , brown - to - amber color branched macules , the dendritic freckles were located over exposed and covered parts of the skin . in addition , a keratotic , speckled pigmentation plaque with rolled out margins of the size of 5 - 7 cm was present over the left scapular region [ figure 1a ] . smaller lesions of similar morphology were also identified over the arms and pre - auricular area [ figure 1b ] . there was no evidence of pallor , cyanosis , icterus , lymphadenopathy , and organomegaly . ( a , b ) basel cell carcinoma occupying left scapular region of the back and pre - auricular region of the face complete hemogram , liver and renal function tests were unremarkable . hematoxylin - eosin - stained sections prepared from the biopsy taken from the plaque revealed a basaloid proliferation of cells arranged in nests , with peripheral palisading [ figure 2 ] . retraction artifact was seen separating some of these cell nests from the surrounding stroma [ figure 3 ] . excision biopsy of the keratotic plaque from the auricle revealed keratinocyte atypia , solar elastosis , and melanophages in the dermis consistent with actinic keratosis [ figure 4 ] . proliferation of basaloid cells disposed in nests in the dermis , ( h and e , 40 ) sections prepared from the biopsy from an exophytic plaque . higher magnification depicting prominent peripheral palisading , and retraction space , ( h and e , 100 ) sections prepared from the biopsy of the keratotic plaque from the auricle . note the keratinocyte atypia in the lower two - thirds of the epidermis ; and melanophages , and solar elastosis in the dermis , ( h and e , 40 ) for the darker races , where melanin confers natural photo - protection , albinism poses as an established risk attribute for all three forms of cutaneous malignancies . although squamous cell carcinoma ( scc ) is usual , basal cell carcinomas have also been reported in albino . however , recent studies have incriminated basal cells in the histo - genesis of actinic keratosis . another fascinating entity that has emerged in recent times is baso - squamous cell carcinoma , where coexisting features of both scc and bcc have been encountered . such a case has also been reported in an albino indian . in our case , the clinical picture , in the setting of albinism and cutaneous features of photo - damage suggested a diagnosis of scc , but consideration of the sites of involvement , duration and absence of metastasis suggested otherwise . our case represents the documented cohabitation of cutaneous signs of photo - damage , actinic keratosis , dendritic freckles in the perspective of oca . the occurrence of bcc , therefore , is fascinating for it is infrequently reported in these settings . oca is an established risk factor for all the three major forms of skin cancers , due to reduction and/or complete absence of melaninalthough scc is common , the presence of bcc is not that infrequent and should be suspectedirrespective of the clinical morphology , histopathology is imperativeunder the circumstances , apart from identification of pure scc / bcc , keratinizing bcc and baso - squamous carcinoma should be kept in mind . oca is an established risk factor for all the three major forms of skin cancers , due to reduction and/or complete absence of melanin although scc is common , the presence of bcc is not that infrequent and should be suspected irrespective of the clinical morphology , histopathology is imperative under the circumstances , apart from identification of pure scc / bcc , keratinizing bcc and baso - squamous carcinoma should be kept in mind . basal cells in the histogenesis of actinic keratosis has recently been incriminated , enforcing this new concept . | the report highlights the occurrence of basal cell carcinoma in a native indian with oculo - cutaneous albinism , an association not frequently encountered .
the clinical and histopathological features , which assisted to form the diagnosis , are outlined .
a high degree of suspicion and timely recognition of the potentially aggressive neoplasm , under this unusual circumstance , is the key to its diagnosis . |
dna transposons are ubiquitous elements that allow genetic fragments to be integrated into host genomes and in nature are used , for example , to confer drug resistance in bacteria . they also have potential use as the delivery vehicle of new or modified dnas in gene therapies . the mechanism of transposition is multi - step , similar in many ways to recombination - type events , and can randomly insert a transposon into the host or show some degree of specificity in integration . one particularly interesting dna element is the is608 transposon ( a member of the is200/is605 class of transposable elements ) found in heliobacter pylori that lies close to genes that are associated with gastric epithelial invasion factors . this element is associated with antibiotic resistance and is inserted 3 of specific four- or five - nucleotide sequences in the host by the tnpa transposase . the question is , how can this relatively small 155-amino acid enzyme affect the cleavage and rejoining steps required to integrate the associated is608 transposon in a specific manner into the host genome ? the structures implicate unique sequence - dependent dna fold - back conformations as the primary determinants both in recognition and in the protein conformational change that switches the transposase into the active configuration . tnpa forms an obligate dimer that provides for dna cutting and splicing at the two ends of is608 transposable elements . thus , unlike other transposases that have two catalytic sites within the same protein , the tnpa dimer creates two sites in trans . this would be sufficient to deal with the mechanism for integrating the transposon if the two ends of insertion were identical and single - stranded , but they are not . the is608 transposon includes two imperfect inverted repeats ( ips ) that are nearly identical but differentially located relative to their cleavage sites . the left ip is 20 nucleotides from the left end ( le ) cut site , whereas the right ip is only 10 nucleotides from the right end ( re ) cut site . there must be an asymmetry in the system that would not obviously come from a symmetric dimer . furthermore , earlier crystal structures from ronning and colleagues of tnpa alone and in complex with an isolated dna hairpin elucidated the dimer structure , but both showed an inactive catalytic site and thus provided no additional insights into the mechanism of action . the recent paper by barabas and colleagues presents five single - crystal structures of tnpa in the presence of the left and right ip elements that extend toward the respective les and res of the transposon . the key finding is that the left and right ips and le and re dna elements form specific structures that not only define their recognition by tnpa , but also induce the catalytic sites of the enzyme to adopt their active conformations . together , the series of structures and biochemical and biophysical studies allow the authors to propose a detailed mechanism for the complete transposition cycle . the most intriguing structures in this series are the complexes between the tnpa dimer and the ip hairpins extended with the le or re dna of the transposon . the re dna , however , is not a dangling end but folds back into a specific loop structure through gcg and taa base triplets , resulting in a topology that is similar in many respects to rna topologies . this end of the dna specifically induces a conformational change in which a helix that contains the catalytic tyrosine residue ( y127 ) is remodeled from the inactive conformation seen in the previous structures to an active form , where y127 is now in position to serve as a nucleophile that can attack the dna backbone . a similar , though not identical , fold - back structure is formed by the le ip and the associated le element , which has a similar effect of creating an active conformation in the enzyme . thus , the dna itself plays a major role in its own recognition - the fold - back structures of the ip and le or re elements are unique and are required to induce the protein to assume its active conformation . this allows the enzyme to work only on the top strand of the transposon . the authors raise the question of whether the dna sequences , in themselves , can fold into the structures seen in the le and re complexes . if so , this would be a dramatic example of the concept known as indirect readout [ 6 - 8 ] , in which the specificity for protein recognition of a dna sequence is not provided by direct protein - dna contacts in the form of , for example , pairing hydrogen bond donor and acceptor groups between protein side chains and nucleotide base pairs , but through the effects of base sequence on the conformational shape of the dna element . this is reminiscent of most rna - binding proteins , which recognize the tertiary structure of the fold rather than the sequence of the single - stranded polynucleotide . an exhaustive map of dna sequences and their structures has shown that sequence can affect both the overall conformation and , more subtly , details of the double - helix . in the latter case , there are now a sufficiently large number of structures of b - dna in a self - consistent dataset to permit investigators to define sequences that adopt an average conformation for this standard double - helix and sequences that deviate significantly from this average , including those of known transcriptional regulators . a recent study by little and colleagues demonstrates the concept of indirect readout at the double - helix level in convincing fashion . in this study , the authors show that the hinc iii endonuclease can still effect site - specific dna cleavage even in a mutant in which all direct protein - dna contacts have been eliminated . the structures of the tnpa transopoases by barabas and colleagues , however , rely on larger effects of sequence on dna structure , a role that is more analogous to the one that indirect readout is proposed to play in the sequence - dependent recognition of holliday junctions during dna recombination . four - way dna junctions have been shown to be stabilized explicitly by core ( a / g)cc trinucleotides within perfect inverted repeat sequences and this has been proposed as the means by which junction - resolving enzymes , such as t7 endonuclease i and t4 endonuclease vii , show sequence specificity for their dna substrates . in the case of the tnpa transposase , the is608 transposon ends form sequence - specific fold - back structures that are specific for the enzymes ' active form . the question , therefore , is one of the chicken or the egg : does the dna fold to form a substrate that induces an active enzyme , or does the shape of the functional tnpa catalytic site induce the dna shape ? the current structures support the former scenario , but one must wait to see whether the le and re dnas by themselves or , more specifically , in the context of the transposon , can fold to the conformation that is uniquely recognizable by the tnpa transposase , and it would be interesting to see whether this could influence the rate of integration . if so , this may have implications for the design of molecular systems for gene therapy applications . | a series of single - crystal structures determined by barabas and colleagues provides a detailed mechanism for how the tnpa transposase from helicobacter pylori recognizes , cleaves , and integrates the is200/is605 class of transposable elements .
an interesting aspect of the mechanism is that the transposase recognizes the transposon through the unique fold - back structure adopted by the sequences of the dna components , rather than through direct protein - dna interactions .
this is an example of indirect readout that is reminiscent of how four - stranded junctions are recognized by recombination proteins , but is also analogous to ribonucleoproteins , in that the dna facilitates formation of an active nucleic acid - protein complex . |
Subsets and Splits