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several guidelines have been published regarding the treatment and prevention of deep vein thrombosis ( dvt)1 , 2 . the guidelines underscore the fact that comprehensive measures for prevention of dvt are necessary3 . guidelines for the diagnosis , treatment and prevention of pulmonary thromboembolism and deep vein thrombosis ( jcs 2009)2 state that elastic stockings are effective for the prevention of dvt . the mechanisms by which wearing elastic stockings prevents dvt are prevention of blood stasis by increasing the blood flow volume and decrease of the caliber of venous blood vessels by compression of the lower limbs4 . however , these effects are still under debate5 , 6 . nara et al . reported that there is little effect when elastic stockings are used as a single application7 , but scurr et al . indicated that a combination of elastic stockings and intermittent pneumatic compression was effective for prevention of dvt8 therefore , examination of the effects of combining elastic stockings with something else on prevention of dvt may be of clinical significance . previously reported that ankle positions affect the blood velocity in the common femoral vein during ankle pumping exercises9 , which was recommended for the prevention of dvt . the aim of this study was to identify how to effectively use elastic stockings to change the blood flow velocity in the common femoral vein under different ankle positions . the study subjects were 10 males without a history of cardiovascular diseases and no contraindications for exercise testing and training according to the guidelines of the american heart association10 . one experimenter ( y.f . ) measured the blood flow velocity in all of these experiments . time - averaged maximum flow velocity ( tamv ) in the left common femoral vein was measured using a pulse doppler method with a diagnostic ultrasound system ( acuson p300 , siemens healthcare , erlangen , germany ) . tamv is the averaged blood flow velocity per unit time in the left common femoral vein . the subject s heart rate , systolic blood pressure ( sbp ) , and diastolic blood pressure ( dbp ) were monitored ( suntech tango+ , suntech medical , usa ) continuously during measurement of tamv . in this study , stocking were used , and the right size for each subject according to the instruction manual was selected . subjects were asked to assume three different positions : supine ( supine position ) , supine with the legs up after the examination bed was raised to an 18-degree angle ( leg - up position ) , and supine with the head up after the examination bed was raised to a 30-degree angle ( head - up position ) ( fig . 1.experimental protocol ) . at the beginning of each condition , the subjects had a 3-min rest period to acclimatize themselves to each position . after the 3 min of rest , the tamv in the left common femoral vein was measured for 20 seconds . in total , the subjects were asked to undergo testing under six conditions , that is , each of the 3 positions with and without stockings . experimental protocol two - way anova for repeated measures was used to compare the tamv values of blood flow velocity . all analyses were performed using spss statistics ver . 17.0 ( spss inc . , chicago , il , usa ) , and statistical significance was accepted at an alpha level of 0.05 . this study was approved by the human ethics review of tokyo university of technology ( approval number ; e14hs-026 ) . under the without stockings condition , the tamv in the head - up position ( 16.6 6.1 cm / sec ) was significantly lower than those in the supine ( 30.1 17.8 cm / sec ) and leg - up positions ( 28.9 17.5 cm / sec ) ( supine vs. head up , p<0.05 , leg up vs. head up , p<0.05 ) . tamv showed no significant difference between the supine position and leg- up position ( table 1table 1.time-averaged maximum flow velocity in the common femoral vein in three different positions with and without stockings ) . under the with stockings condition , the tamv in the head - up position ( 13.6 4.4 cm / sec ) was also significantly lower than those in the supine ( 33.1 15.5 cm / sec ) and leg - up positions ( 27.7 9.4 cm / sec ) ( supine vs. head up , p<0.01 , leg up vs. head up , p<0.01 ) . tamv showed no significant difference between the supine position and leg - up position ( table 1 ) . in this study , elastic stockings did not affect the blood flow velocity in the common femoral vein . this can be attributed to the fact that subjects were young health males who had no problem in their venous valves and venous vascular walls . generally , the venous diameter is wider than the arterial diameter , but the venous vascular wall is thinner than the arterial vascular wall because it has a thinner layer of smooth muscle and fewer elastic components . for this reason , veins have great compliance and thus a high blood volume , and compression can easily change the venous shape11 . in venous incompetence , the number of collagen fibers increases , and the fibers are randomly aligned12 , 13 . furthermore , veins can not constrict and expand adequately in response to the stimulation of noradrenalin , potassium chloride , angiotensin ii , nitric oxide , calcium ion , etc . , and they do not function normally14 . additionally , expansion of veins and blood stasis occur easily because dysfunction of venous valves causes the blood pressure to increase in veins . thus , it is likely that elastic stockings are effective in preventing blood stasis in venous incompetence by compressing veins continuously and preventing them from expanding15 . in this study , therefore , it is suspected that compression by elastic stockings does not lead an increase in blood flow velocity , as their venous valves and venous vascular walls functioned properly . on the other hand , it is known that an increase in hydrostatic pressure disturbs venous return16 , thus in venous incompetence , the blood velocity in the common femoral vein can decrease markedly because venous incompetence disturbing venous return may be affected profoundly by gravity . this study has some limitations : ( 1 ) the number of subjects in this study was relatively low , and ( 2 ) the subjects were only young healthy male volunteers . this could have affected results , and a study for different age groups of people should be performed .
[ purpose ] the aim of this study was to identify how to effectively use elastic stockings . [ subjects and methods ] ten young healthy men participated in this study . time - averaged maximum flow velocity in the common femoral vein was measured for 20 seconds using a pulse doppler method with a diagnostic ultrasound system under six conditions ( three different positions and with and without stockings ) . changes of blood flow velocity were compared . [ results ] elastic stockings did not affect the blood flow velocity in the common femoral vein . for both the with stockings condition and without stockings condition , the time - averaged maximum flow velocity in the head - up position was significantly lower than that in the supine and leg - up positions . time - averaged maximum flow velocity showed no significant difference between the supine position and leg - up position . [ conclusion ] elastic stockings did not affect the blood flow velocity in the common femoral vein , but ankle positions did affect it . further studies of patients with venous insufficiency are needed .
when do such problems become abnormal and merit a specific psychiatric diagnostic label , remains a question under study with no clear cut answers . we present a case of a young girl for whom clinical attention was sought for transient eating problems . a 12-year - old young lady belonging to lower socioeconomic rural background was seen by the psychiatry services when referred for problems of eating since past 2 months . the patient had a history of delayed development and achieved milestones slower than other siblings . she would eat what was given to her by her mother , and ask for specific food items occasionally . around 2 years back , the patient developed generalized tonic clonic seizures during sleep which were followed by myoclonic jerks . the electroencephalograpm ( eeg ) findings were consistent with a diagnosis of juvenile myoclonic epilepsy ( jme ) . the episodes of generalized tonic clonic seizures were well controlled with valproate , but myoclonic jerks continued to recur . due to the occurrence of jerks at school , other children would tease her . she became irritable with her family members quite often , and would hit the siblings when provoked . she did not have depressive cognitions or change in sleep or appetite then . due to behavioral problems of refusal to go to school and irritability , intelligence quotient ( iq ) assessment was done and reported as 57 with a diagnosis of mild mental retardation . since around 2 months prior she would eat rice at the previous amounts , but would refuse to eat rotis , the staple diet of the family . if persuaded , she would get irritable and start crying and go to sleep without eating anything . at times she said that she was hungry and would ask for food , and would eat rice but refuse rotis . she did not have any associated loose stools , abdominal pain , vomiting , or regurgitation . she would not demand for specific food items like chocolates , etc . ; would not appear sad , withdrawn or fearful , or have any disturbances in sleep . over the period of 1 month , the weight reduced by about 1 kg . on further exploration , no preoccupation with body image , of previously fussiness of eating was reported by the family members . the child had a slow to warm up temperament and there was no history suggestive of eating inedible objects , delusions , hallucinations or repetitive behaviors , significant obstinacy , hyperactivity or inattentiveness . the general physical examination was within normal limits , the height and weight was over the 10 percentile and there were no signs of chronic malnutrition . rapport could be established with difficulty and the child answered briefly only after urging from the mother and therapist . when patient returned in 2 weeks , there was no further decrement in weight and the patient started to eat all kinds of food stuffs again and to the previous amounts . even on recovery , she did not elaborate the cause of decreased intake of certain foodstuffs and that of resumption . multiple possibilities can be entertained in this child while evaluating a child presenting with such eating problems . the age appropriateness of such behaviors , development level aptness , sociocultural background , parenting style , and medical conditions need to be ascertained before considering whether such a behavior can be assessed as abnormal . feeding habits in adolescents reveals deficits in calories and proteins in apparently healthy school - going children from relatively well - to - do backgrounds . with such a premise , is diagnosing the transient problems of eating as a separate disorder in this child a useful and relevant exercise , or whether these problems can be passed off as developmentally appropriate erratic behaviors ? even if a diagnostic label is required , where does the child fit in ? is the diagnosis of mental retardation sufficient to subsume these symptoms or an additional diagnosis is needed ? since the diagnostic categories of affective , psychotic , and anxiety spectrum disorders do not apply in this case , whether the problems can be better characterized as eating disorder not other specified ( eating disorder nos ) ? however , there was no evidence of body image disturbances . does feeding disorder of infancy and childhood better characterize this case , though the obvious atypicality being age of presentation and transient symptoms in this case ; or does this case remain a many researchers have proposed alternate pragmatic classification systems for characterizing the problems of eating in childhood . fox and joughin have described a variety of eating problems that are encountered in the childhood and adolescence . among these , the index case exhibits symptoms closest to food avoidance emotional disorder which occurs in the age range of 5 - 16 years and is characterized by avoidance of food and emotionality in the absence of preoccupation of body weight or body image . many children who present with behavioral problems of eating can not be pigeonholed into diagnostic class of present nosology , but still benefit with interventions . hence , we suggest that these disorders should be treated pragmatically after detailed assessment without being excessively concerned about the diagnostic label , at the same time not overlooking the obvious diagnostic possibilities .
eating problems are commonly encountered in childhood and adolescents , and may be manifestation of a variety of psychiatric disorders when medical causes are excluded . we present the case of a young lady presenting with problems of eating which presented with difficulties of ascribing a diagnostic label for eating problems . the patient , a 12-year - old girl with history suggestive of mild mental retardation and juvenile myoclonic epilepsy presented with selective eating of foodstuffs which improved spontaneously in 2 months . the different diagnostic possibilities entertained for the case are discussed .
to report surgical results of canaloplasty surgery for primary open - angle glaucoma ( poag ) in japanese patients . three eyes of 3 patients underwent canaloplasty alone and 8 eyes of 6 patients underwent canaloplasty combined with cataract surgery . canaloplasty was performed with a 10 - 0 polypropylene tensioning suture and an itrack 250a microcatheter . mean number of antiglaucoma drops was 2.8 0.6 before canaloplasty and decreased to 1.2 0.8 at 12 months after canaloplasty ( p < 0.01 ) . mean iop decreased postoperatively , being 13.7 2.8 mm hg at 1 month , 12.8 3.5 mm hg at 3 months , 14.0 4.4 mm hg at 6 months , and 15.0 4.1 mm hg at 12 months . the most frequent postoperative complication was mild hyphema ( 45.5% ) , which disappeared within 14 days after surgery . canaloplasty may be an alternative surgery for poag patients to reduce iop to a value of approximately 15 mm hg . canaloplasty is a new nonpenetrating glaucoma surgery that is expected to increase physiological aqueous outflow from the anterior chamber through the trabeculo - descemet 's window into schlemm 's canal [ 1 , 2 ] . using the flexible itrack 250a microcatheter ( iscience interventional , menlo park , calif . , usa ) , designed especially for canaloplasty , 10 - 0 polypropylene tensioning suture is installed into schlemm 's canal circumferentially . we studied the surgical results of canaloplasty surgery for japanese primary open - angle glaucoma ( poag ) patients . nine patients ( 11 eyes ) of poag treated at toyama university hospital between december 2008 and june 2009 were included in this study . three eyes of 3 patients underwent canaloplasty alone and 8 eyes of 6 patients underwent canaloplasty combined with phacoemulsification / aspiration and intraocular lens implantation ( pea + iol ) . written informed consent was obtained from each of the patients after they were provided with sufficient information about the procedures . the mean age of the patients was 71.7 8.5 years ( mean standard deviation ) . surgical techniques were as follows . briefly , after limbal conjunctival peritomy , a superficial parabolic flap approximately 4 mm wide and 5 mm long was created at the 12 o'clock limbus . after injection of viscoelastic material into the ostia of schlemm 's canal , 10 - 0 polypropylene tensioning suture was installed using an itrack 250a microcatheter . the iop was measured at 1 , 3 , 6 , and 12 months postoperatively . mean iops were 13.7 2.8 mm hg at 1 month , 12.8 3.5 mm hg at 3 months , 14.0 4.4 mm hg at 6 months , and 15.0 4.1 mm hg at 12 months ( fig . statistical analysis was carried out by a paired t test and dunn 's multiple comparison analysis . all postoperative mean iops were significantly decreased compared to the preoperative iop of 23.4 5.5 mm hg ( p < 0.001 or p < 2 shows an image of the anterior segment optical coherence tomography ( casia ; tomey corporation , nagoya , japan ) , which indicated the tensioned and dilated schlemm 's canal ( arrows ) at 12 months after canaloplasty surgery . a kaplan - meier survival model was used for cumulative probability of qualified success with or without medications ( fig . the qualified success rate of an iop of 21 , 18 , and 16 mm hg or less at 12 months was 81.8 , 54.5 , and 54.5% , respectively ( fig . the mean postoperative number of antiglaucoma drops was significantly reduced to 1.2 0.8 ( p < 0.01 ) . the most frequent postoperative complication was mild hyphema ( 45.5% ) , which disappeared within 14 days after the surgery without detrimental effect . we showed a similar mean postoperative iop of 15.0 4.1 mm hg at 12 months after canaloplasty . because this was a retrospective study canaloplasty reduced the mean postoperative number of antiglaucoma drops and it may be suitable to reduce the iop to a value of approximately 15 mm hg . mild hyphema was the most frequent postoperative complication ( 45.5% ) . during the installation of the itrack 250a microcatheter into schlemm 's canal , mild bleeding from the trabecular tissue into the anterior chamber koch et al . reported that anterior chamber hemorrhage with hyphema was found in 15 of 21 eyes ( 71.4% ) on day 1 after canaloplasty , and the eyes without hyphema showed higher iop than those with hyphema . it is possible that microperforation may be one of the mechanisms of iop reduction by canaloplasty surgery . although long - term results of canaloplasty have not been clarified yet , canaloplasty may be an alternative method for japanese patients with poag when the desired pressure is around 15 mm hg .
purposeto report surgical results of canaloplasty surgery for primary open - angle glaucoma ( poag ) in japanese patients.methodseleven eyes of 9 poag patients underwent canalopasty surgery at toyama university hospital . three eyes of 3 patients underwent canaloplasty alone and 8 eyes of 6 patients underwent canaloplasty combined with cataract surgery . canaloplasty was performed with a 10 - 0 polypropylene tensioning suture and an itrack 250a microcatheter . all patients were followed up for 12 months . changes in intraocular pressure ( iop ) and postoperative complications were examined.resultsmean preoperative iop was 23.4 5.5 mm hg . mean number of antiglaucoma drops was 2.8 0.6 before canaloplasty and decreased to 1.2 0.8 at 12 months after canaloplasty ( p < 0.01 ) . mean iop decreased postoperatively , being 13.7 2.8 mm hg at 1 month , 12.8 3.5 mm hg at 3 months , 14.0 4.4 mm hg at 6 months , and 15.0 4.1 mm hg at 12 months . the most frequent postoperative complication was mild hyphema ( 45.5% ) , which disappeared within 14 days after surgery.conclusionscanaloplasty may be an alternative surgery for poag patients to reduce iop to a value of approximately 15 mm hg .
, it ranked first among both kuwaiti and non - kuwaiti women . during the period from 2000 to 2008 , it constituted 36.0% and 39.5% from the newly diagnosed cancer cases in kuwaiti and non - kuwaiti females , respectively . excision of the primary tumor ( by mastectomy or breast conserving surgery ) and sentinel lymph node or axillary lymph node dissection are standard procedures for the treatment of most cases . surgical site infection ( ssi ) the development of ssi can lead to prolonged hospital stay with increased costs , poor cosmetic results , psychological trauma , and , occasionally , a delay in postoperative adjuvant therapies . to the best of our knowledge , no published studies reported ssi rate after breast cancer surgery in kuwait . hence , the present investigation was aiming to determine the ssi rate after breast cancer surgeries and the causative microorganisms as a step to improve the management and the outcome of such patients . all patients undergoing breast surgery in kuwait cancer control center which is a comprehensive cancer center devoted exclusively to the care of patients with cancer as a treatment for breast cancer from january 2009 to december 2010 were prospectively followed for the development of ssi . indirect prospective detection of ssi was used to identify patients with ssis [ 5 , 6 ] . patients were followed up to 30 days after surgery if there was no implant and up to one year when there was an implant placed during the operation . ssis were identified using centers for disease control and prevention / national healthcare safety network ( cdc / nhsn ) surveillance definition of health care - associated infections . diagnosis was based on collecting information from patients medical records including reviewing of clinical data ( symptoms and signs ) , investigations ( laboratory , histopathology , radiological , etc . ) , microbiological culture and sensitivity results , and medication charts in addition to discussions with the treating surgeons [ 5 , 6 ] . infections were identified either during the original surgical admission , at readmission to the hospital , or during outpatient follow up of the surgical wound by reviewing of medical records of surgery clinic patients [ 5 , 6 ] . ssi rate during the study period was calculated using the following formula : ( 1)number of ssis in breast cancer surgery100number of breast cancer surgery . the total number of operations performed for breast cancer during the study period was 438 . females represented the majority of patients , 434 ( 99.1% ) , while males constituted only 4 ( 0.9% ) cases . breast surgeries done were excision of the tumor either by breast conserving surgery or mastectomy accompanied with sentinel lymph node biopsy or axillary lymph node clearance . two patients 2/10 ( 20% ) required secondary wound closure or incision and drainage of the wound in the operating room . most of the ssis ( 8 cases , 80% ) were detected after patients discharge , during outpatient follow up of the surgical wound . out of them 5/8 ( 62.5% ) all patients with ssi except one ( 90% ) received systemic antibiotic therapy for management of their wound infection . staphylococcus aureus ( s. aureus ) was the most common pathogen ( isolated from 40% of all cases ) . details of microbiological results were reported in table 1 . the majority ( 6/8 ( 75% ) ) of empirical therapy it was reported that around 75% of breast cancer cases had undergone surgery as a part of their treatment . however , any surgical procedure has the potential risk of infection . the potential morbidity caused by infection can lead to serious complications . delaying postoperative radiotherapy results in poorer control of local diseases . a significant increase in metastatic relapse and reduced survival exist when adjuvant chemotherapy is delayed . ( 2.7% ) , 4.5% by leinung et al . by olsen et al . most of ssis were diagnosed after patients ' discharges . with the current trends favoring a shortened postoperative hospital stay , it becomes more likely that a significant percentage of surgical site infections will occur after these patients ' discharges . other investigators reported that an estimated 47% to 84% of ssis occur after discharge ; most of these are managed entirely in the outpatient setting [ 13 , 14 ] . minor infections are usually managed on outpatient basis while complicated wounds require readmission with the possibility of surgical interference . this is usually accompanied by delay in wound healing and consecutively delays of the start of postoperative adjuvant therapy . most of our patients who developed ssi were readmitted for management of their complicated infections ( including reoperation ) adding extra cost on the healthcare settings and may worsen the patient outcome . we believe that the rate of ssi in our study may be higher than we reported . as the postoperative length of stay is decreasing , the follow up of the patient is mainly carried out on an outpatient basis . during outpatient visits , when the ssi develops and requires no readmission , surgeons may not document the infection in the patient 's records and may not request microbiological sampling of the wound . this is primarily due to fear of medical malpractice claims or negligence especially in a surgery classified as a one like breast surgery . in the present investigation , despite the fact that s. aureus ( mssa and mrsa ) was the primary pathogen isolated from ssis , gram negative bacteria were isolated in 40% of cases representing a significant finding . however , mukhtar et al . reported gram negative bacteria as the most common isolated pathogens . these findings support the importance of the use of empirical broad - spectrum antimicrobial ( not only targeting s. aureus ) coverage ( if any ) until culture results become available .
background and objectives . surgical site infection ( ssi ) is the most common postoperative complication associated with breast cancer surgery . the present investigation aimed to determine the ssi rate after breast cancer surgeries and the causative microorganisms . patients and methods . all patients who underwent breast surgery in kuwait cancer control center as a treatment for breast cancer from january 2009december 2010 were prospectively followed for the development of ssi . indirect detection was used to identify ssis through medical record to review and discussion with the treating surgeons . results . the number of operations was 438 . females represented 434 ( 99.1% ) cases while males constituted only 4 ( 0.9% ) cases . ssis were diagnosed after 10 operations , all for female cases . most of the ssis ( 8 cases ; 80% ) were detected after patients were discharged , during outpatient followup . out of those 5/8 ; ( 62.5% ) were readmitted for management of ssi . nine patients ( 90% ) received systemic antibiotic therapy for management of their wound infection . the ssi rate was 2.3% . the main causative organism was staphylococcus aureus ( s. aureus ) which was responsible for 40% of infections . gram negative bacteria were isolated from 40% of the cases . conclusion . ssi is an important complication following breast cancer surgery . microbiological diagnosis is an essential tool for proper management of such patients .
meigs syndrome is defined as the coexistence of benign ovarian fibroma , hydrothorax , and ascites . by contrast , pseudomeigs syndrome is limited to other ovarian or pelvic tumors associated with hydrothorax and ascites 1 . both are treated by resectioning of the tumors , which could control the fluid accumulation in the thorax and abdomen 2 . although many different types of operative methods are chosen to treat rectal prolapse , rectopexy is considered the most appropriate in terms of curability , but its use is limited because of its invasiveness . laparoscopic rectopexy is expected to expand its indication even to elderly patients ; however , it is unclear whether patients with a large volume of ascites due to pseudomeigs syndrome would be candidates for laparoscopic rectopexy . we herein report a case of rectal prolapse caused by ascites from pseudomeigs syndrome that was successfully managed with laparoscopic surgery . a 64yearold female presented to our surgical outpatient department complaining of discomfort caused by rectal prolapse and abdominal distension attributable to ascites . she had been receiving outpatient chemotherapy to treat breast cancer with metastasis for 13 years , which she had commenced after a leftsided mastectomy , and her survival prognosis was estimated to be more than 1 year according to previous reports 3 , 4 , 5 . computed tomography ( ct ) revealed complete rectal prolapse ; disseminated masses , including an ovarian metastasis ; pleural effusion ; and large amounts of ascitic fluid ( especially in the pelvic cavity ) ( fig . approximately 3500 ml of slightly yellow transparent ascitic fluid was aspirated . as large ovarian metastases covered the pelvic cavity and impeded the procedure , we first performed a bilateral oophorectomy . the rectum was then dissected in the mesorectal plane to the level of the levators and subsequently stapled to the sacral bone around the region of the promontory ( fig . the patient was discharged on day 10 after surgery with no apparent complications and continued to receive outpatient chemotherapy . pathological examination revealed that the ovarian tumors were compatible with breast cancer metastases . at the 1year followup , no relapse was evident in terms of abdominal or thoracic fluid or rectal prolapse ( fig . 1 ) . thus , laparoscopic surgery was useful to treat both the rectal prolapse and pseudomeigs syndrome . ( left ) preoperative computed tomography ( ct ) scan : enlarged bilateral ovaries ( white arrows ) and a large amount of ascites fluid are evident in the pelvic cavity . ( right ) postoperative ct scan taken 2 months after surgery : both ovaries have been resected and the ascites has disappeared . ( a ) both ovaries were enlarged within the pelvic cavity and covered the rectum . ( b ) the ovarian artery and vein were ligated , and both ovaries were then resected using an ultrasonic coagulation device . ( c ) the rectum was completely mobilized down to the level of the levators . this case emphasizes that laparoscopic rectopexy can be used to treat rectal prolapse in the presence of comorbidities and that treatment of pseudomeigs syndrome improves quality of life and reduces the rectal prolapse relapse rate . a comparative study demonstrated that laparoscopic suture rectopexy is a safe and feasible procedure and has comparable results with regard to operative time , morbidity , bowel function , and recurrence ; it even has slightly better results than mesh rectopexy . furthermore , the cost of the mesh used in mesh rectopexy is absent in suture rectopexy 8 , and it is unclear whether mesh rectopexy might be safe and feasible for patients with multiple peritoneal dissemination . a recent systematic review showed that laparoscopic colorectal surgery in the elderly afforded significant advantages in terms of shortterm outcomes 9 . as the incidence of rectal prolapse increases with age , the use of laparoscopic methods might be able to extend surgical indications to elderly , fragile patients . one small randomized clinical trial showed that laparoscopic rectopexy was associated with less postoperative pain , an earlier return to alimentation , an earlier discharge from the hospital , and lowerlevel relapse , at the expense of needing a longer operation time compared to open methods 10 , 11 , 12 . additionally , laparoscopy permits visualization of the entire abdominal cavity , facilitating simultaneous treatment of several pathological lesions . in our present case , laparoscopic management was chosen not only to reduce the relapse rate but also to prevent the development of an abdominal incisional hernia attributable to increased intraabdominal pressure from ascites . as large ovarian metastases covered the pelvic cavity and impeded the procedure , we first performed a bilateral oophorectomy . this effectively treated the pseudomeigs syndrome . additionally , mitigation of the ascites prevented prolapse recurrence . palliative treatment ( i.e. , the miwagant procedure ) would not be expected to prevent early relapse because of the abundant ascites associated with pseudomeigs syndrome . in this case , neither pleural effusion nor ascites were clinically evident ; therefore , they did not affect the decision to perform the oophorectomy . if the patient were obese , it may have impeded the ability to clinically identify the presence of large ascites . however , the ascites contributed to the rectal prolapse , and the oophorectomy was thus useful . to the best of our knowledge , this is the first case in which rectal prolapse and pseudomeigs syndrome have been simultaneously treated and successfully managed by laparoscopic surgery . such surgery may thus be considered as the first choice when treating patients with rectal prolapse and pseudomeigs syndrome . tk , mt , hh , ko , ks and yk : designed the research ; tk , mt , and th : collected clinical information ; tk and mt : wrote the paper .
key clinical messagewe report a rare case of rectal prolapse with pseudomeigs syndrome in which laparoscopic bilateral oophorectomy and rectopexy were performed simultaneously and resulted in improved quality of life due to the loss of ascites and the repair of rectal prolapse . laparoscopic surgery is feasible for rectal prolapse with pseudomeigs syndrome .
bacteria of the genus actinomyces are gram - positive bacilli which generally colonize mouth , colon and vagina . , if actinomycosis occurs , it often presents as an indolent , slowly progressive infection characterized by abscess formation , which can progress across tissue boundaries , can present with mass like features which simulate malignancy , can develop into a sinus tract and/or develop clinically into a refractory or relapsing infection after a short course of therapy . a 43-year old woman with no significant medical history presented with complaints of an unilateral slowly growing painful mass in the left breast . mammography ( fig . 1 ) and ultrasonography were conducted and showed a small abscess - like density retroareolarly.fig . . 1 mammography of the left breast showing a confined density in the retroareolar area . species identification was performed using matrix - assisted laser desorption ionization - time - of - flight mass spectrometry ( maldi - tof ms ; bruker ) . susceptibility testing for a. neuii was performed using e - tests ( biomerieux ) . at the time that culture findings became available , therefore , the cultured actinomyces was considered as skin commensals and a wait and see policy was installed . after several months , the patient returned to the outward clinic with complaints of pain in the breast . psychical examination of the breast showed mild erythema and ultrasonography showed a minimal increase of the lesion . the abscess was surgically drained and new purulent fluid from the abscess was sent to the laboratory . the material was processed as described above . in contrast to initial culture findings , determination by the maldi - tof ms showed peptostreptococcus magnus ( score value 2.056 ) . after four days there was also growth observed in the brewer thioglycollate medium . the isolate was shown to be susceptible to penicillin , amoxicillin , clindamycin , meropenem and vancomycin . histopathological examination of the obtained tissue showed abscess formation in an infection with a sinus tract . subsequently , the patient was treated for six weeks of intravenous penicillin followed by six months of oral amoxicillin successfully . secondly , a 73-year old woman with a history of bilateral breast cancer was seen at with severe pain and swelling of the left breast ten days after her ablation which was complicated by a large hamatoma treated conservatively . gram strain showed many leukocytes , some gram - positive cocci and many gram - positive rods . determination using maldi - tof ms identified a staphylococcus aureus after 2 days there were colonies visible on the anaerobically incubated schaedler agar . determination by the maldi - tof ms revealed an a. neuii susceptibility testing was performed using e - tests . unfortunately , patient returned four days after discharge with a relapse of the hematoma which was surgically evacuated . a month later at the outward clinic she showed full recovery.fig . other isolates that have been identified as pathogens causing primary breast actinomycosis include actinomyces viscosus , actinomyces turincensis and actinomyces radingae . it differs from the other actinomyces species because of its aerobic growth and its microscopic morphology without branching . we were able to retrieve only three former cases of a. neuii causing primary actinomycosis . in the first case , treatment with antibacterials was unsuccessful and the lesion had to be surgically removed . in the second case , patient was successfully treated with amoxicillin for 21 days . in the third case , actinomycosis , regardless of species , should be treated with high doses of antibacterials for a prolonged period of time although therapy needs to be individualized . current guidelines recommend high dose of penicillin intravenously for two to six weeks , followed by oral therapy with penicillin or amoxicillin for 6 to 12 months . alternative antibacterial treatments include doxycycline , erythromycin or clindamycin . if actinomycosis presents with well - defined abscess then surgical drainage followed by long - term antibacterial therapy is indicated . we describe this combination of treatment in our cases . in the first case initial cultures showed islolated a. neuii . because signs of infection had decreased this finding was interpreted as skin flora and not as a pathogen . therefore medical treatment was considered unnecessary . in retrospect , if antibacterial therapy had been given at first presentation recurrence of the infection might have been prevented . actinomyces species morphologically resemble diphtheroids and could be dismissed as skin commensals , even when isolated from an abscess sample . in both of our presented cases we found different organisms in the culture additional to a. neuii . in the first case we found p. magnus and in the second case s. aureus was present . although a. neuii infection is known for its chronicity , the influence of these mixed organisms on the severity of infections remains unclear . further research on the effect of mixed organism on the severity of an a. neuii infection is required .
actinomycosis is a slowly progressive infection caused by anaerobic bacteria , primarily from the genus actinomyces . primary actinomycosis of the breast is rare and presents as a mass like density which can mimic malignancy . mammography , ultrasonography and histopathologic examination is required for diagnosis . treatment should consist of high doses of antibacterials for a prolonged period of time and possibly surgical drainage . primary actinomycosis infections are commonly caused by a. israelii . actinomyces neuii is a less common cause of classical actinomycosis . we present two cases of primary actinomycosis of the breast in two female patients caused by a. neuii .
from may through october 2008 , we trapped small mammals at 5 sites in southern sweden ( figure 1 ) . our primary objective was to obtain samples from bank voles , myodes glareolus , but other species were also sampled when captured . a nested pcr was performed with primers for anaplasmataceae specific for the 16s rrna gene ( 10 ) . prevalence of infection : hglinge , n = 45 infections , 0% candidatus n. mikurensis , 44.4% bartonella spp . ; revinge , n = 623 infections , 9.3% candidatus n. mikurensis , 33.7% bartonella spp . ; istaby , n = 53 infections , 3.8% candidatus n. mikurensis , 34% bartonella spp . ; hemmestr , n = 64 infections , 4.7% candidatus n. mikurensis , 39.1% bartonella spp . ; herseby , n = 49 infections , 12.5% candidatus n. mikurensis , 45.0% bartonella spp . all amplified fragments were sequenced , and a blast ( http://blast.ncbi.nlm.nih.gov/blast.cgi ) search showed that 68 animals were infected by candidatus n. mikurensis . the primers were chosen to be specific for bacteria belonging to the families rickettsiaceae and anaplasmataceae ( 10 ) , but they also amplified bartonella spp . under the given pcr conditions . in total , , as indicated by double peaks on the sequencing chromatogram , occurred in 12 cases . to further characterize the obtained candidatus n. mikurensis , we sequenced 1,426 bp of the 16s rrna and 1,233 bp of the groel gene ( 1 ) . the obtained 16s rrna candidatus n. mikurensis sequences in this study were identical ( 1,426/1,426 bp ) to sequences obtained from human patients in germany and switzerland ( 2,3 ) . the groel sequence was identical to the isolate from germany ( 1,233/1,233 bp ) but differed slightly from the isolate from switzerland ( 1,072/1,084 bp , 98.9% pairwise identity ) . a phylogenetic network containing unique 16s rrna sequences from candidatus n. mikurensis available at the national center for biotechnology information was made with the program network 4.5.1.6 ( www.fluxus-engineering.com ) by using the median - joining algorithm ( 11 ) ( figure 2 ) . phylogenetic network of 16s rrna sequences ( 1,231 bp ) from candidatus neoehrlichia mikurensis , southern sweden , 2008 . numbers on branches represents mutations , numbered according to nucleotide position in the alignment . the sequence obtained in this study is shown in boldface and is identical with sequences from human patients in germany ( 3 ) and switzerland ( 2 ) . the japanese reference strain tk4456 ( 7 ) showed 99.2% similarity with our sequence , and the north american candidatus n. lotori strain ( 12 ) showed 98.3% similarity . the prevalence of candidatus n. mikurensis at the 5 sites ranged from 0% to 12.5% , and bartonella spp . occurred in 33.7% to 45.0% of the animals ( figure 1 ) . candidatus n. mikurensis occurred in all 4 rodent species , but not in the shrews ; the difference in prevalence between rodents and shrews was statistically significant ( p = 0.011 , by fisher exact test ) . in the present field survey of anaplasmataceae in swedish rodents , we have amplified 16s rrna and groel sequences identified as candidatus n. mikurensis identical to sequences obtained from humans . this organism has been amplified from humans with febrile illness on 4 occasions ( 13 ) , demonstrating that candidatus n. mikurensis can cause human infections , at least occasionally . apart from the first human case , to our knowledge , candidatus n. mikurensis has not been detected in sweden . however , we identified candidatus n. mikurensis at 4 of 5 sites , indicating that this organism is widespread in southern sweden . identical or similar sequences have been detected in ixodes ricinus ticks from several european countries ( 36,13 ) , showing that it is distributed over a large area of europe . i. ricinus is the dominating tick species in sweden , and animals at all our study sites were heavily infested with larvae of this species and occasionally with nymphs ( m. andersson and l. rberg , unpub . reservoir hosts are essential for several tick - borne pathogens that lack the capacity for transovarial transmission from female ticks to larvae , such as a. phagocytophilum ( 14 ) . approximately 70% of the tick larvae in sweden engorge on small mammals , such as voles , mice , and shrews ( 15 ) . the prevalence of candidatus n. mikurensis was similar in all investigated rodent species in our study with a mean value of 8.8% . these results are consistent with several studies in japan , which also found candidatus n. mikurensis in several different rodent species ( 7,10 ) . this finding indicates that rodents play a role in the natural cycle of candidatus n. mikurensis in europe and that rodents are likely to be competent reservoir hosts . in contrast , the closely related candidatus n. lotori , found in the united states , seems to use raccoons rather than rodents as hosts ( 12 ) . whether the european variant of candidatus neoehrlichia is capable of infecting animals other than rodents and humans remains to be investigated . we found the organism was completely absent in shrews , which suggests that they might not be competent hosts for these bacteria . we conclude that candidatus n. mikurensis is geographically widespread in southern sweden and that several rodent species , the main source of blood meals for larvae of i. ricinus in sweden ( 15 ) , can be infected . the relatively high prevalence in common rodent species suggests that the risk for humans and domestic animals to encounter infected i. ricinus nymphs and adults is substantial . thus , candidatus n. mikurensis should be considered when diagnosing bacterial infections associated with tick bites .
we examined small mammals as hosts for anaplasmataceae in southern sweden . of 771 rodents , 68 ( 8.8% ) were infected by candidatus neoehrlichia mikurensis , but no other anaplasmataceae were found . candidatus n. mikurensis has recently been found in human patients in germany , switzerland , and sweden , which suggests that this could be an emerging pathogen in europe .
the typical clinical presentation of poi is pain in the right side of the abdomen , and this may lead to a misdiagnosis of acute appendicitis or cholecystitis . here , we present a case of poi , with pain in the left lower quadrant . a 50-year - old woman admitted to the emergency room with a history of worsening abdominal pain for 5 days . the pain began in the upper abdomen and later localized to the left lower quadrant , and the pain intensity exacerbated with activities . the pain was sharp and constant in nature without nausea or vomiting . on physical examination , the patient was febrile with a temperature of 38.1c and had severe left lower quadrant tenderness and abdominal rigidity . the previous history included an open ectopic pregnancy surgery in 1996 , and no atrial fibrillation , no venous thrombosis , no contraceptive , or aspirin usage . the laboratory test showed white blood cell 9.71 10/l , d - dimer 1.58 mg / l , and no other abnormalities . an abdominal computed tomography ( ct ) was performed and showed a left ovarian cyst measuring 6.0 cm 4.5 cm with no other remarkable findings [ figure 1a ] . after about 1 day , the patient complained of worsening abdominal pain and absence of passage of feces and gas . ( a ) axial image showed a left ovarian cyst measuring 6.0 cm 4.5 cm . median sagittal section showed an increased fat - density mass consequently , we performed laparoscopic exploration . during the procedure , we found a segment of congested necrotic omentum adhering to the abdominal wall with a segment of small intestine [ figure 2a ] , bloody ascites ( 200 ml ) accumulated in the abdominal cavity [ figure 2b ] , and an ovarian cyst measuring 6.0 cm 4.5 cm in pelvic cavity [ figure 2c ] . we resected the nonviable omental segment , and the gynecologist removed the ovarian cyst using laparoscopic procedures . ( b ) a dark purple mass on the left side in the lower part of the omentum and bloody ascites in the left abdomen . ( c ) a huge ovarian cyst by reanalyzing the preoperative ct , a segmental fat mass with an increased density was noted in the left lower quadrant which was consistent with the intraoperative findings [ figure 1b ] . final pathological examination revealed hemorrhagic infiltrations , thrombosis of the tissue , acute inflammatory cellular infiltrate , and fibrinoid necrosis , and poi was diagnosed [ figure 3 ] . histological examination of the resected nonviable omentum revealed hemorrhagic infiltrations , thrombosis of the tissue , acute inflammatory cellular infiltrate , and fibrinoid necrosis up till now , only a few hundred cases have been published in the english literature . susceptible factors include ( 1 ) anatomic malformations such as a bifid or accessory omentum causing the spontaneous torsion , ( 2 ) sudden movement , violent exercise , and hyperperistalsis , and ( 3 ) obesity . most authors support the hypothesis that it is associated with an anomalous and fragile blood supply of the right lower portion of the omentum , which is consequently susceptible to infarction . thus , 90% of all the cases have initial pain in the right side of the abdomen . our patient had pain in the left lower quadrant , so we did not include poi in the differential diagnosis spectrum . poi has no early specific signs and is often characterized by progressive , persistent abdominal pain . clinical features include unspecific gastrointestinal symptoms , slightly febrile , and mild leukocytosis . in the present case , d - dimer can be used as an objective parameter in the differential diagnosis of poi as it is used in other thromboembolic diseases . the most diagnostic finding is an ill - defined heterogeneous fat density with surrounding inflammatory changes . by retrospective reanalysis of the preoperative ct in this study , ultrasound is specific but not sensitive for diagnosing poi ; suspected imaging features include hyperechoic , incompressible , ovoid mass , and detectable in < 50% of cases , even when reviewed retrospectively . two patients underwent surgery , one because of the absence of spontaneous regression and the other because of extremely severe clinical symptoms . in the other four patients , conservative management was given and successful . if a confirmed diagnosis of poi can be made with the typical clinical signs and imaging studies , conservative treatment is the first - line therapy during the first 2448 h while resuscitation and antibiotics are initiated . however , if the diagnosis is in doubt or if conservative treatment fails , laparoscopy should be performed without delay . as in the present case , the pain was not relieved after conservative treatment for 24 h. intestinal obstruction occurred as a segment of small intestine adhered to the abdominal wall . poi should be considered in the differential diagnosis of any patient presenting with acute abdominal pain . laparoscopic exploration should be performed if new symptoms occur or do not relieve within 2448 h. there are no conflicts of interest .
primary omental infarction ( poi ) is a rare cause of acute abdomen . most patients have aggravating abdominal pain without gastrointestinal symptoms . here , we report a case of omental infarction in a 50-year - old woman , who had left abdominal pain and intestinal obstruction . preoperative computed tomography ( ct ) of the abdomen showed a left ovarian cyst measuring 6.0 cm 4.5 cm but otherwise seemed normal initially . the white blood cell count was 9.71 109/l , and d - dimer was 1.58 mg / l . laparoscopic exploration was performed 1 day after admission because of peritonitis and intestinal obstruction . during the exploration , a segment of congested necrotic omentum was found adhering to the abdominal wall with a segment of small intestine . bloody ascites was also observed in the abdominal cavity . we resected the nonviable segmental omentum , and the ovarian cyst was removed by the gynecologist using laparoscopic procedures . final pathological findings confirmed poi . while reanalyzing the preoperative ct , a segmental fat mass with an increased density was noted in the left lower quadrant , which was consistent with the intraoperative view 6 days after surgery . the patient recovered uneventfully and was discharged .
early childhood caries ( ecc ) is one of the most common chronic diseases of the childhood . according to the american academy of pediatric dentistry ( 2003 ) ecc may be defined as the presence of one or more decayed , missing ( due to caries ) or filled tooth surfaces in any primary tooth in a child 71 months of age or younger . the prevalence is 1 - 12% in developed countries and 70% , while in india , a prevalence of 44% has been reported for caries in 8 - 48 months old children . the early loss of primary anterior teeth may result in reduced loss of vertical dimension , masticatory efficiency , development of parafunctional habits ( tongue thrusting , speech problems ) , esthetic functional problems such as malocclusion , psychological problems that can interfere in the personality and behavioral development of the child . this case report describes the challenging task of treating a 3-year - old child with severely decayed maxillary anterior teeth that were restored using modified omega loop post followed by strip crown . a 3-year - old child reported to the department of pedodontics and preventive dentistry , with a complaint of severely decayed upper front teeth . the child was shy , and behavior of the child was definitely negative according to frankl behavior rating scale . intraoral examination revealed a complete set of deciduous and caries involving with 61 ( maxillary left primary central incisor ) . coronal portions of 61 were severely damaged , and most of the tooth structure was lost with pulpal involvement . 61 . after taking parents , consent diet analysis , counseling , and oral prophylaxes were performed . 61 using metapex , followed by custom - made half omega - shaped post with 0.9 mm stainless steel orthodontic wire using no.130 to make omega loop , and serrations were done to increase the stability of the esthetic restoration and the mechanical retention of the core . about 5 mm of the metapex was removed from the coronal end of the root canal , and 1 mm of glass ionomer cement ( gic ) was placed . the incisal end of the wire projected 3 - 4 mm above the remaining root structure . after gic set , the canal was prepared to get a space of about 3 mm [ figure 1 ] . ( a - f ) pulpectomy procedure with modified omega loop post followed by strip crown i.r.t . 61 the root canal was etched with 35% phosphoric acid for 20 s , followed by bonding agent was placed and cured for 20 s. the flowable composite was injected into the root canal along with the loop . the composite was light - cured for 40 s. crown was reconstructed using strip crown . finishing and polishing treatment of preschool children and restoration of primary anterior teeth with the severe loss of coronal structure is a challenging task for the pediatric dentists . the main aim is to preserve teeth and restore them so that child is able to perform normal like mastication , speech , and esthetics . the failure rate is high in such type of cases due to the absence of tooth structure , poor adhesion of bonding agent of primary teeth , limited availability of material and technique . after the successful endodontic treatment and placement of intracanal retainers , the remaining coronal structure can be restored with indirect or direct technique or single tooth prostheses , such as strip crown , stainless steel crown , metal plastic crown , porcelain veneers , polycarbonate crowns , and acrylic resin crown . nowadays , esthetic crowns are introduced in pediatric dentistry like pedo jacket crowns , fuks crowns , new millennium crowns , preveneered crowns , cheng crowns , dura crowns , pedo pearl crowns and kinder crowns to overcome the disadvantages of strip crown like low retention and poor adhesive of bonding agent . many studies advocate the use of nonmetallic posts such as ceramic post , polyethylene glass fibers , carbon fibers , etc . , but in such kind of cases , it has some disadvantages like technique sensitive , time - consuming , multiple steps , and expensive . a simpler and effective method is to use an omega loop that was introduced by mortada and king . in this technique , omega loops wire extensions are placed at the depth of around 3 - 4 mm inside pulp chamber and the projected portion of the loop is used for retention of the coronal restoration . the biggest advantage is that the wire does not cause any internal stresses in the root canal as it is incorporated in the restorative material mainly , and it can be done with minimal chair side time . in the present case report , a simple and effective method for reconstruction of severely destroyed primary anterior teeth has been used . this technique can be done directly in the oral cavity without involving any laboratory procedures . the complete procedure can be completed in one appointment , easy for the pediatric patient and less cooperation required from the child . as the core length of the omega loop , which is placed intracanal , is around 3 mm thus occupies only the cervical one - third of the canal and does not interfere with deciduous tooth root resorption and permanent tooth eruption . omega loop technique can be an easier , simpler , and inexpensive treatment of choice for severely damaged primary anterior teeth . the modified omega loop with serration used in this case report demonstrated good retention , good esthetics , and masticatory function to the child . however , it is a long time success , and its durability in children having parafunctional habits like bruxism , deep bite , etc .
the esthetic concern of severely mutilated primary anterior teeth in the case of early childhood caries has been a challenge to pediatric dentist . early childhood caries is the most common chronic disease of the preschool child . the case report presented here is of a three year old boy with severely decayed maxillary anterior teeth . after root canal treatment , the primary maxillary central incisors were reinforced using modified omega post and followed by using celluloid strip crowns . the technique described here offers a simple and effective method for restoring severely decayed primary anterior teeth that reestablishes shape , function , and esthetics .
treatment of metastatic kidney cancer has changed dramatically over the past years with the use of vegf - targeted therapies and mtor inhibitors . however , resistance occurs . we report here two cases of patients who benefited from the addition of bevacizumab to temsirolimus . a 48-year - old man presented with several episodes of loss of consciousness in 2005 . the brain metastases were treated with gamma - knife radiosurgery . in 2006 , he developed lung metastases and mediastinal lymph nodes . in march 2007 , the mediastinal lymph nodes increased and he was started on sunitinib ( 50 mg 4 weeks on treatment and 2 weeks off ) . the lesions were stable but the lymphangitic infiltration was extensive and the patient still experienced dyspnoea . he improved dramatically , his oxygen consumption decreased . on the ct scan the mediastinal lymph nodes and the lymphangitic carcinomatosis lesions improved . a 54-year - old man presented with hematuria revealing a left kidney tumour in january 2004 . the pathological examination showed a pt2 clear cell carcinoma , furman grade ii tumour . in march 2005 , he progressed with liver and lung metastases and he was enrolled in the phase iii study comparing interferon to sunitinib . he had slowly progressive disease on interferon and crossed over to sunitinib in march 2006 . he had a partial response . in july 2008 , the lung and liver lesions were stable but he developed peritoneal carcinomatosis with ascitis . he was started on temsirolimus : ascitis was less abundant , the other lesions were stable . there were probably several causes to his anaemia ( a side effect of temsirolimus and the disease ) . in january 2009 , the liver lesions increased and he was started on a combination of bevacizumab ( 10 mg / kg every 2 weeks ) and temsirolimus ( 20 mg iv weekly ) . his general condition improved , the anaemia disappeared , the lesions were stable and the ascitis much less abundant . he felt that he had fewer side effects on the combination treatment than on temsirolimus alone . these two cases show the effect of adding bevacizumab to temsirolimus for patients who were progressing on the mtor inhibitor . treatment of metastatic kidney cancer has changed dramatically in the past years with the use of vegf - targeted therapies and mtor inhibitors . the vegfr tyrosine kinase inhibitor , sunitinib , is usually used in the first - line setting . another tyrosine kinase inhibitor or an mtor inhibitor can be used in the second - line setting , after progression on initial treatment . resistance to vegf pathway inhibition can be caused by upregulation of alternative pro - angiogenic factors ( fgf , angiopoietin ) , inadequate target inhibition or enhanced receptor signalling . there are a few cases in the literature of patients who were progressing on sunitinib and who benefited from the adjunction of bevacizumab ; however , the first data show that these combinations are very toxic . this could drive upregulation of mtorc2 and further activation of akt and hif2. this could therefore lead to increase of expression of hif2 target genes , such as vegf . combining vegf - blocking agents with mtor inhibitors could contribute to reversing resistance when used in a sequential manner . however , results from the torava phase ii randomized trial show no improvement of non - progression rates with the upfront combination of bevacizumab and temsirolimus but increased toxicity leading to a high drop - out rate . there is no data in the literature on why patients should feel fewer side effects when adding bevacizumab to temsirolimus . however , some reports already mention the development of polyglobulia with anti - angiogenesis agent . this effect may correct a pre - existing anaemia [ 4 , 5 , 6 ] .
treatment of metastatic kidney cancer has changed dramatically in the past years with the use of vegf - targeted therapies and mtor inhibitors . however , resistance occurs . we report here two cases of patients who benefited , both on disease control and side effects , from the addition of bevacizumab to temsirolimus , after progression on the mtor inhibitor alone .
delusional disorder is an uncommon psychiatric condition characterized by nonbizarre delusions of a single theme , in the absence of other mood or psychotic symptoms . it is differentiated from schizophrenia by the absence of bizarre delusions and impairment of functioning only in relation to the delusional belief . the lifetime prevalence rate is reported to be about 0.2% in the united states , whereas in india rates of about 1% have been reported in a psychiatric clinic population . the disorder is divided into erotomanic , grandiose , jealous , persecutory and somatic subtypes based on the content of the delusion . the most commonly reported subtypes are the persecutory subtype in western literature , whereas delusional parasitosis is most commonly reported in indian literature . although delusions of a sexual nature are not unusual in schizophrenia and affective disorder , reports of this presenting as a delusional disorder are uncommon . we report a case of a young male who presented with a single delusion regarding his sexual physiology . mr . a is a 19-year - old single engineering student , from a middle socioeconomic background . over the previous year and a half , he firmly believed that every time he assumed an upright posture , he attained penile erection . he believed that others were able to identify his physiological state by the appearance of his groin and therefore were laughing at him and making derogatory comments . he had made attempts to mask these perceived bodily changes by changing the way he dressed . secondary to these beliefs he had also become socially withdrawn , was frequently absent from class and had had significant academic decline . he also avoided situations , which required him to stand upright , such as crowded buses , elevators , and shops . he had attempted self - harm a year earlier by slashing his wrist due to the distress related to his beliefs . the patient had been on fluvoxamine and risperidone for about 7 months at the time of presentation . physical examination , including a detailed neurological and genital examination did not reveal any abnormalities . mental status examination revealed a well groomed young man with normal psychomotor activity and speech . he was distressed by the sexual problems that he perceived he had , but denied suicidal ideation . he had a fixed belief that he attained penile erection whenever in an erect posture . the degree of conviction with which the patient held his belief despite evidence to the contrary suggested it to be a delusion , upon which he was acting . a diagnosis of delusional disorder was considered in view of the single delusional theme in the absence of first rank symptoms or affective features . the management focused on establishing rapport with the patient along with eliciting and understanding the explanatory model for his beliefs . socratic questioning was used to identify negative thoughts and dysfunctional assumptions , which were reflected to the patient . a hierarchy of situations based on distress and avoidance were charted down and he was asked to expose himself to the least anxiety provoking situation . the patient was also encouraged to do some behavioral experiments in these situations to confirm or disprove his assumptions . the antidepressant medication was tapered and stopped , while the dose of risperidone was gradually increased to 4 mg / day . delusions with sexual content , although not as common as other delusions , have been reported in psychotic disorders such as schizophrenia , depression , and organic conditions . the content of these delusions include that of self - mutilation , delusional pseudo - transsexualism , bizarre sexual mechanisms , threat to genitalia , variations of erotomania , pregnancy , procreation , and homosexuality . in a qualitative review of bizarre delusions among a sample of patients with schizophrenia in india , de et al . found that delusions with sexual content included false beliefs about sexual relationship between humans , animals and/or supernatural beings ; forceful change in sexual gender or identity and threats to genitalia . while sexual delusions are encountered as one among other types of delusions in schizophrenia , it is not commonly reported in the form of a delusional disorder . in this case , organic illness was ruled out and the delusional nature of the belief was confirmed . in the absence of other psychotic symptoms , the patient reported distress secondary to the belief and its consequences , and there was no evidence of a primary mood disorder or other psychotic symptoms . delusional disorder is managed with pharmacological and nonpharmacological strategies . in this case treatment with antipsychotic agents was combined with supportive measures along with cognitive and behavioural strategies , to allay the patient 's anxiety , acknowledge his distress , explore the consequences of his beliefs and modify them . isolated delusions with a sexual theme are rare . as with any other delusional disorder , the treatment is challenging and involves both psychopharmacology and psychotherapy . given the chronic nature of this condition , treatment strategies should be tailored to the individual needs of the patients with a focus on maintaining social function and improving quality of life .
delusions with a sexual theme are rare , but when present are usually seen in patients with schizophrenia or other chronic psychotic disorders . we report a case of delusional disorder , with a single belief of a sexual theme . this report focuses on the management issues , wherein a combination of pharmacological and nonpharmacological interventions proved helpful .
a 40-year - old man presented with involuntary movement of his right hand and left foot . the symptom started when he was approximately 35 years of age and had slowly worsened . his disability progressed over the following years and was restricted to his right hand and left foot . he had no significant medical history and no history of exposure to neuroleptic drugs , hypoxic environments , or toxic metabolites , except for the intermittent inhalation of silica dust at his workplace . there was no family history of neurological disorders . on examination , the patient had dystonic posture of his right hand , which caused alternating dorsiflexion and neutralization with supination of the forearm and flexion of the metacarpophalangeal joint . an ophthalmologic evaluation showed no kayser - fleischer rings , optic atrophy , or pigmentary retinopathy . serum ferritin and copper levels were normal , and a blood smear was negative for acanthocytes . the results of other laboratory tests , including serum electrolytes , erythrocyte sedimentation rate , thyroid function test , liver function test , autoimmune antibodies , ferritin , copper , ceruloplasmin , and a blood smear were normal , except for borderline - low 24-hour urine copper ( 10.2 g / dl , normal 1530 g / dl ) . a t1-weighted brain mri study showed hyperintensity bilateral anteromedial globus pallidi ( figure 1a ) . a t2-weighted mri showed hypointensity in the bilateral globus pallidi with hyperintense core , the typical eye - of - the - tiger sign ( figure 1b ) . genomic dna was extracted from peripheral blood , and polymerase chain reactions ( pcr ) were preformed . the dyt1 gag deletion ( dele302/303 ) automated dna sequence analyses revealed two single - base variants in exon 3 and exon 4 . both pathological variants result in changes to conserved amino acids in the pank2 protein : asp268 gly ( p.d268 g ) and arg330prp ( p.r330p ) . to our knowledge , this is the first patient identified with the mutation p. r330p and the second patient with the mutation p.d268 g . the dystonia of the patient s right hand and left foot did not respond to levodopa / carbidopa ( 100/25 mg t.i.d ) . treatment with diazepam ( 5 mg t.i.d ) and trihexyphenidyl ( 2 mg t.i.d ) slightly improved the dystonia . however , his dystonia worsened after one year , and he could not walk without a cane . our patient had an atypical phenotype of the pank2 gene : compound heterozygous mutations in exon 3 ( p.d268 g ) and exon 4 ( p.r330p ) . to our knowledge , our patient is the first to be identified with mutation p.r330p and the second to be identified with mutation p.d268 g . the onset of atypical pkan is known to be 1st three decades ( mean age 13.6).2 in a previous study , 23 patients with atypical pkan were analyzed , and the mean age at onset was 13.7 years ( range 128 years ) . patients with atypical pkan had variable clinical features , including parkinsonism , corticospinal tract involvement , speech disturbances such as palilalia , and psychiatric symptoms.3 our patient presented with dystonia and gait disturbance , which are the predominant symptoms of classical pkan . the implication of the eye - of - the - tiger sign on a brain mri is controversial.3,4 the eye - of - the - tiger sign on mri is not pathognomic of pkan , and some patients with pank2 mutations do not have this sign and sometimes , it may appear only transiently . this sign has also been reported in corticobasal degeneration , progressive supranuclear palsy , anoxicischemic leukoencephalopathy , and early - onset levodopa - responsive parkinsonism.4 however , a previous study reported that all patients clinically suspected of nbia with the eye - of - tigersign have mutations in pank2 and that all patients without the typical mri finding do not have these mutations.3 in our patient , the mri finding and presenting symptoms were consistent with classic pkan , while the age at onset and the absence of other features such as retinal degeneration , optic atrophy , and delayed development were consistent with atypical disease or other subtypes of nbia . genetic studies identified compound heterozygous missense mutations ( p.d268 g , p.r330p ) in exons 3 and 4 of pank2 . to our knowledge , this is the first description of the p.r330p mutation . the mutation p.d268 g was reported previously in a chinese patient with heterozygous missense mutations ( p. d268 g , p.i391n).5 others have attempted to establish a correlation between the genotype and phenotype . although the linkage between mutations and residual enzymatic activities related to the age of onset has been partially demonstrated , the phenotype is still difficult to predict based on the presence of pank2 mutations.3,68 there were no overlapping clinical features between our patient and the chinese patient with the p.d268 g mutation , except for the typical mri finding . the chinese patient s disease began at 17 years of age , and his predominant symptoms were tremor and rigidity . it is presumed that modifier effects of allelic genes and environmental factors played a roll in the phenotypic differences between these two patients.3 the pank2 gene codes for pantothenate kinase , which is an essential regulatory enzyme in coenzyme a ( coa ) biosynthesis , catalyzing the cytosolic phosphorylation of pantothenate ( vitamin b5 ) the enzyme of the initial and rate - limiting step in coa biosynthesis : the phosphorylation of pantothenate , n - pantothenoyl - cysteine and pantetheine.9 to compensate for the partial enzymatic deficiency in patients with pkan , supplement of pantothenate ( vitamin b5 ) have been tried without conclusive benefit.3 previous case reports using pallidal deep - brain stimulation reported improvement of dystonia in patients with pkan , but it was also restrictive effect.10 further study is needed for early diagnosis of pkan , correct prediction of disease progress , and rational treatment .
pantothenate kinase - associated neurodegeneration ( pkan ) is an autosomal recessive disorder that is characterized by mutations in the pantothenate kinase 2 gene ( pank2 ) and typical magnetic resonance imaging findings . we report a case of atypical pkan presenting with generalized dystonia . our patient had compound heterozygous mutations in the pank2 gene , including mutation in exon 3 ( p.d268 g ) and exon 4 ( p.r330p ) . to our knowledge , this patient is the first to have the p.r330p mutation and the second to have the p.d268 g mutation .
radiologically , they are similar to ependymomas , and histopathologically , they are similar to neuroendocrine tumors . most of them are non - secreting tumors , although they are of sympathetic origin . back pain is the most common symptom , and they are not associated with tachycardia , flushing , or hypertension . the treatment of choice is total surgical resection , and no additional treatment is needed with total resection . most of them are benign , but , recurrence can occur in cases involving a failure of the total resection . we describe a rare case of a paraganglioma that was adherent to the cauda equina that presented with low back pain and radiating pain in both the lower extremities . a 76-year - old female visited our department with complaints of low back pain and radiating pain in both the lower extremities for more than two months . magnetic resonance ( mr ) imaging revealed a lobulated sausage - like intradural mass ( fig . 1 ) . she underwent a total laminectomy and near - total tumor removal . during the operation , after opening the dura , a reddish , friable , and well - marginated mass was observed ( fig . we removed the mass near - totally except for the lesion that was adherent to the cauda equina . extradural paragangliomas may compress the spinal cord , resulting in paraparesis5 ) . from a pathologic point of view , paragangliomas are similar to parasympathetic tumors . however , functional paragangliomas are rare6 ) . histopathologically they are similar to parasympathetic tumors that occur in the carotid body or glomus region . althogh they are also similar to catecholamine - secreting tumors , symptoms of flushing , tachycardia , or hypertension are rare . however , a case in which hypertension was controlled after thoracic paraganglioma removal has been reported12 ) . preoperative diagnosis is difficult with radiological tools . mr images of paragangliomas in the cauda equina region are non - specific and are relatively isointense on t1-weighted images and hyperintense on t2-weighted images . immunohistochemically , neuron - specific enolase , synaptophysin , and chromogranin staining are positive2 ) . in the cauda equina region , it is very difficult to distinguish paragangliomas from ependymomas . cauda equina paragangliomas are classified as grade i on the world health organization ( who ) classification . recurrence after surgery on a paraganglioma in the cauda equina region , especially if it is encapsulated , is rarely encountered2 ) . the surgical goal is complete total resection with preservation of the surrounding nerve roots1 ) . thus , we did not treat the patient any additional therapy , and conducted a close follow - up . after incomplete surgical resection , prolonged postoperative observations are mandatory because of the slow evolution of paragangliomas4 ) . distant metastases very rarely occur5 ) , and metastatic paragangliomas of the spine have been reported7,11 ) . total resection was difficult in this case due to adhesion to the cauda equina , and close follow - up may therefore be needed .
paragangliomas are rare among intradural spinal tumors . most of them are benign , but aggressive behavior and local recurrence can occur . cases of paraganglioma are , difficult to diagnose radiologically ; hence , diagnosis is confirmed histopathologically . radiologically , paragangliomas are similar to ependymomas , and , histopathologically , they are similar to neuroendocrine tumors . we evaluated the case of a 76-year - old woman with a spinal paraganglioma that was associated with back pain and radiating pain in both the lower extremities . she underwent an operation , and her symptoms were relieved . here , we describe a rare case of paraganglioma that was adherent to the cauda equina .
, neurological side effects of carcineurin inhibitor are becoming controversial recently . the neurological change related to tacrolimus was first presented as posterior reversible leukoencephalopathy in 1996 . until now , there have been many clinical symptoms reported and they are referred to various names including posterior reversible encephalopathy syndrome . we report an 11-year - old girl 's reversible cerebral infarction and cerebral vascular stenosis after using tacrolimus for her kidney transplantation . an 11-year - old girl with end - stage renal disease had underwent a transplantation of kidney from her mother . pre - transplant donor - specific t- and b - cell cross - matches were negative . initial immunosuppressive treatment consisted of tacrolimus ( 0.2 ng / kg / day orally , target level 15 to 20 ng / ml ) , mycophenolate mofetil ( 1,000 mg twice a day orally ) , and methylprednisolone ( 25 mg twice a day orally ) . renal function was quite good after kidney transplantation . on post - operative day 12 , hypertension ( 160/100 ) , headache , and left motor weakness ( grade i ) suddenly occurred . the brain - magnetic resonance imaging ( mri ) and magnetic resonance angiography ( mra ) findings showed acute cerebral infarction of subcortical white matter of the right hemisphere and multiple stenosis of both anterior cerebral artery ( aca ) and middle cerebral artery ( mca ) ( fig . conservative treatment was done for several days and tacrolimus continued with dosage adjusted to maintain a serum level of 5 to 10 ng / ml . the repeated brain - mri and mra scaning the findings showed newly developed acute cerebral infarction on the subcortical white matter of the left hemisphere , cortex of the left parietal lobe and mildly improving status of stenosis in both aca and mca ( fig . 2 ) . this neurological change seems to be acute cerebral infarction due to cerebral vascular stenosis caused by either vasculitis or vascular spasm , which is highly suspected to be the consequence of tacrolimus . afterwards , neurologic symptoms improved and follow - up ( post - operative day # 29 ) brain mri and mra findings showed an improving status of multifocal acute infarction and no stenosis of either aca or mca ( fig . in 1996 , hinchey et al . described a syndrome of acute but reversible clinical findings including headache , mental status alteration , seizures , hypertension , and acute visual changes associated with abnormalities seen on mri of symmetric white matter lesions , usually in bilateral parietal and occipital lobes . although it was initially thought to affect the subcortical white matter only , additional studies supported by improved radiologic imaging modalities have revealed that the cortical gray matter may also be involved . the term " posterior reversible encephalopathy syndrome ( pres ) " proposed by casey et al . is widely used recently because it expresses its clinical manifestation and radiologic findings appro priately . there have been many reports of this syndrome in the literature since its initial definition . as pres for example , reports have linked pres to hypertension , immunosuppressive / chemotherapeutic agents , eclampsia , porphyria , and renaldysfunction . the incidence of neurotoxicity , which is one of the major adverse events of calcineurin inhibitors , was higher in patients receiving tacrolimus rather than cyclosporine . as both sensory and motor functions may be adversely affected , patients thus present with a wide range of neurological and psychiatric disorders . severe symptoms could manifest as psychoses , hallucinations , cortical blindness , seizures , cerebellar ataxia , motor weakness , or pres . fluid - attenuated inversion recovery is the most sensitive sequence for recognition of cortical and subcortical edema in pres where hyperintense signal alterations are more prevalent than in conventional sequences . this girl showed slightly different radiologic findings from those of typical pres what causes vasogenic edema with vasculopathy , such as vasoconstriction and vasodilation . thus , the neurological complication of this patient seems to have been caused by the use of tacrolimus . pres induced by tacrolimus can usually be diagnosed on the basis of a characteristic clinical and radiographic pattern and is usually reversible by reducing the dosage or with holding the drug for a few days . therefore , careful examination of patients receiving immunosuppressive agents was needed to discover this uncommon but good prognostic complication .
recently significant neurotoxicity has been reported with the use of carcineurin inhibitors . an 11-year - old - girl had undergone a transplantation of kidney from her mother . on post - operative day 12 , hypertension , headache , and left motor weakness ( grade i ) suddenly occurred . the brain - magnetic resonance imaging and magnetic resonance angiography showed acute cerebral infarction at subcortical white matter of the right hemisphere and multiple stenoses of both anterior cerebral artery and middle cerebral artery . while stopping tacrolimus treatment , we experienced clinical and radiological improvement . so , the neurological complications of this patient seem to have been caused by the use of tacrolimus .
eq . 2 can be generalized to the case where the charge moves continuously , corresponding to an infinite number of steps . if we makezi = qmax / n , i=1 n , vi = vo , i=1 n , then all i = , and we can write eq . 4 can also be written as(5)qnor(v)=12[1+coth[qmax(vvo)2kt]2ktqmax(vv0)],which is of the same form of the classical equation of paramagnetism ( see kittel , 2005 ) . typically , the experimental value of the charge plotted is normalized to its maximum because there is no knowledge of the absolute amount of charge per molecule and the number of molecules . the normalized q - v curve , qnor , is obtained by dividing q(v ) by the sum of all the partial charges . 1 shows qnor computed using eq . 2 for one , two , three , four , and six transitions and for the continuous case using eq . 5 ( squares ) with superimposed fits to a two - state boltzmann distribution ( eq . 1 , lines ) . the computations were done with equal charge in each step ( for a total charge qmax = 4e0 ) and also the same vi = 25 mv value for all the steps . it is clear that fits are quite acceptable for cases up to four transitions , but the fit significantly deviates in the continuous case . examples of normalized q - v curves for a qmax = 4 computed with eq . 2 for the cases of one , two , three , four , and six transitions and the continuous case using eq . 5 ( squares ) . all the q - v curves were fitted with eq . 1 ( lines ) . the insets show the fitted valence ( qmax ) and half - point ( v1/2 ) . considering that experimental data normally have significant scatter , it is then quite likely that the experimenter will accept the single - transition fit even for cases where there are six or more transitions ( see fig . 1 ) . in general , the case up to four transitions will look as a very good fit , and the fitted qmax value may be inaccurately taken and the total charge transported might be underestimated . to illustrate how bad the estimate can be for these cases , we have included as insets the fitted value of qmax for the cases presented in fig . it is clear that the estimated value can be as low as a fourth of the real total charge . the estimated value of v1/2 is very close to the correct value for all cases , but we have only considered cases in which all vi s are the same . it should be noted that if i of the rightmost transition is heavily biased to the last state ( vi is very negative ) , then the qmax estimated by fitting a two - state model is much closer to the total gating charge . in a three - state model , it can be shown that the fitted value is exact when v1 and v2 because in that case , it converts into a two - state model . although these values of v are unrealistic , the fitted value of qmax can be very close to the total charge when v2 is much more negative than v1 ( that is , v1 > > v2 ) . on the other hand , if v1 < < v2 , the q - v curve will exhibit a plateau region and , as the difference between v1 and v2 decreases , the plateau becomes less obvious and the curve looks monotonic . these cases have been discussed in detail for the two - transition model in lacroix et al . we conclude that it is not possible to estimate unequivocally the gating charge per sensor from a single - boltzmann fit to a q - v curve of a charge moving in multiple transitions . the estimated qmax value will be a low estimate of the gating charge qmax , except in the case of the two - state model or the case of a heavily biased late step , which are rare occurrences . it is then safer to call apparent gating charge the fitted qmax value of the single - boltzmann fit . the most general case in which transitions between states include loops , branches , and steps can be derived directly from the partition function and follows the general thermodynamic treatment by sigg and bezanilla ( 1997 ) , chowdhury and chanda ( 2012 ) , and sigg ( 2013 ) . the reaction coordinate is the charge moving in the general case where it evolves from q = 0 to q = qmax by means of steps , loops , or branches . in that case , the partition function is given by(6)z=iexp(qi(vvi)kt).we can compute the mean gating charge , also called the q - v curve , as(7)q(v)=q=ktzz = ktdlnzdv=iqiexp(qi(vvi)kt)iexp(qi(vvi)kt).the slope of the q - v is obtained by taking the derivative of q with respect to v:(8)dq(v)dv=(kt)2d2lnzdv2 . the charge fluctuation will depend on the number of possible conformations of the charge and is expected to be a maximum when there are only two possible charged states to dwell . as the number of intermediate states increases , the charge fluctuation decreases . now , a measure of the charge fluctuation is given by the variance of the gating charge , which can be computed from the partition function as:(9)q2=q2q2=(kt)2(zz(zz)2)=(kt)2d2lnzdv2.but the variance ( eq . this implies that the slope of the q - v is maximum when there are only two states .
the voltage dependence of charges in voltage - sensitive proteins , typically displayed as charge versus voltage ( q - v ) curves , is often quantified by fitting it to a simple two - state boltzmann function . this procedure overlooks the fact that the fitted parameters , including the total charge , may be incorrect if the charge is moving in multiple steps . we present here the derivation of a general formulation for q - v curves from multistate sequential models , including the case of infinite number of states . we demonstrate that the commonly used method to estimate the charge per molecule using a simple boltzmann fit is not only inadequate , but in most cases , it underestimates the moving charge times the fraction of the field .
radiofrequency ablation of the slow pathway is considered to be the gold standard treatment for patients with atrioventricular nodal reentrant tachycardia ( avnrt ) . pulmonary artery agenesis is a rare anomaly that may occur during embryological development of the heart.1 this agenesis may be accompanied by a complete or partial absence of the lung . in the great majority of the cases , the diagnosis is usually made at or soon after birth and it can be associated with multiple anomalies . an otherwise normal heart may be displaced into the right hemithorax by unilateral pulmonary agenesis.2 catheter ablation of avnrt in the setting of dextrocardia is potentially challenging . there have been a few reports of successful slow pathway ablation in patients with dextrocardia , but usually at the expense of prolonged procedure and fluoroscopy times.37 to our knowledge , slow pathway ablation of a patient with dextrocardia due to pulmonary agenesis has not been described to date . a 35-year - old woman was admitted to our cardiology clinic with complaints of palpitation . she had a 7-year history of paroxysmal palpitations . the tachycardia , at a rate of 170200 bpm , was associated with dyspnea and chest tightness and lasted for up to 60 minutes at a time . her physical examination revealed no breathing sound on the right and a normal breathing sound on the left hemithorax , while the heart s sound was heard from the right hemithorax . the chest radiogram incidentally showed a rudimentary , opacified right hemithorax with mediastinal shift and herniation of the contralateral lung . except for dextroposition of the heart , a 12-lead resting electrocardiogram ( ecg ) showed sinus rhythm at a rate of 64 bpm , a positive qrs complex in avr , a positive p wave in avr and a biphasic p wave in avl , and a prominent r wave in v1 with undetermined horizontal axis ( figure 1 ) . the clinical tachycardia was a regular , narrow qrs tachycardia at a rate of 170 bpm , during which the p wave was indiscernible ( figure 2 ) . computed tomography showed a complete absence of the right lung with dextroposition of the heart ( figure 3 ) . electrophysiological study was performed after obtaining written informed consent and discontinuation of all drugs for five half - lives . the right atrium and the right ventricle were imaged on left lateral and anteroposterior views by contrast injection through inferior vena cava to reveal the cardiac anatomy ( figure 4 ) . then , a 4 mm - tipped ablation catheter ( cordis webster , baldwin park , ca , usa ) was advanced through the 6f sheath into the right atrium and a four - pole fixed - curve diagnostic catheter was placed on the bundle of his . during the electrophysiological study , a narrow qrs tachycardia with a cycle length of 270 ms was reproducibly induced by atrial premature stimulation with an extrastimulus ( figure 5a ) . the ventriculoatrial interval , measured from the onset of ventricular activation on the surface ecg to the earliest deflection of the atrial activation in the his bundle electrogram , was < 60 ms , and the diagnosis of typical slow fast avnrt was confirmed . his bundle records were taken with an electroanatomi - cal approach . then , slow pathway potentials were recorded ( figure 5b ) . fluoroscopic views of the catheters during the ablation and mapping of the coronary sinus ostium are shown in figures 6 and 7 . during ablation , the radiofrequency energy was adjusted to obtain a catheter tip temperature between 50c and 60c . the current was applied for 60 seconds after junctional tachycardia was observed during ablation ( figure 8) . after the ablation , there was no residual slow pathway conduction and the clinical arrhythmia was not inducible without or with infusion of isoproterenol . the total procedure time was 30 minutes with a total radiofrequency delivery time of 8 minutes and fluoroscopy time of only 6 minutes . no complication occurred after the procedure and the patient was free of symptoms at 1-year follow - up . dextrocardia or complex cardiac anatomy of the heart may be challenging to electrophysiologists during catheter ablation procedures . there have been only a few case reports of catheter ablation of supraventricular tachyarrhythmias in patients with dextrocardia.37 pulmonary agenesis can be localized to a single lobe and it can affect an entire lung or , in rare cases , both lungs.1 although a majority of patients with unilateral agenesis die soon after birth or in early childhood , in some extreme cases , patients can survive up to adulthood , like our patient . the most common and familiar form is the mirror image of normal or mirror - image dextrocardia , in which the migration of the apex of the l - bulboventricular loop is into the right hemithorax , as expected . the anterior posterior relationship of the various parts of the heart is normal , but their right - to - left orientation is reversed . the second most common type is dextroversion , in which the heart appears to be rotated into the right hemithorax relative to its normal position . 3 ) the third type of dextrocardia is dextroposition , in which an otherwise normal heart is displaced or shifted into the right hemithorax by other extracardiac causes such as agenesis or fibrosis of the right lung . our case was a type 3 dextrocardia . to recognize the ecg findings of dextrocardia necessitates a clear understanding of the electrical axis . global negativity in lead i , a positively deflected qrs complex in avr , and right axis deviation and reverse r wave progression in precordial leads are the mean ecg findings in dextrocardia . the finding of a positively deflected qrs complex in avr and a negatively deflected ors complex in lead i may lead to misdiagnosis . the most common cause for this finding is reversed electrode placement of arm leads ( left and right ) . during a standard catheter ablation procedure , the catheter is targeted to the ablation region based on typical local electrogram characteristics ( slow - pathway potentials ) and anatomical landmarks . in contrast to patients with normal anatomy , localization of the slow pathway and stabilization of the catheter are more difficult in patients with dextrocardia . therefore , the time spent in the catheterization laboratory and the fluoroscopy time are markedly longer than in cases with normal hearts . our case illustrates the role of imaging by contrast injection in a patient with dextrocardia . the identification of the accurate anatomy using imaging modalities such as computerized tomography and magnetic resonance imaging and three - dimensional image reconstruction using mapping systems may be useful not only to help the electrophysiologist , enabling a safe and successful catheter ablation procedure , but also to understand the complex anatomical structures and to guide optimal catheter access . however , these modalities are quite expensive and are not easily accessible in every unit . before the ablation procedure , we easily localized the coronary sinus ostium and his bundle position . as such , we did not need an additional catheter in this ablation procedure , but an additional catheter might further facilitate the procedure . we are presenting this pulmonary agenesis case in view of its atypical presentation in adulthood , which is exceptionally rare . as demonstrated in this case , revealing cardiac anatomy by contrast injection or other imaging modalities may shorten the procedure time and decrease the complication rates .
radiofrequency catheter ablation of the slow pathway is considered to be the treatment of choice for patients with atrioventricular nodal reentrant tachycardia . we report a 34-year - old female with mirror image dextrocardia due to unilateral pulmonary agenesis who underwent successful slow pathway ablation for typical atrioventricular nodal reentrant tachycardia . using contrast injection , cardiac anatomy was identified in a short time and successfully ablated .
transesophageal echocardiogram ( tee ) has been introduced to the operating room over 30 years ago . we report a case of esophageal perforation caused by tee during an aortic valve replacement operation , treated successfully with a new endoscopic clip . to the best of our knowledge , this is the first case of post - tee esophageal perforation treated endoscopically with this new clip device . a 72-year - old female was admitted to the operating theater for aortic valve replacement . during the operation and in the first hours in the intensive care unit ( icu ) the patient had to undergo tee in order to assess postoperative left ventricle performance status . a deep 2 1.5 cm ulcer covered with blood thrombus was found approximately 2 cm above the gastroesophageal junction . the rest of the endoscopy was unremarkable . within the next 48 h , thoracic and only a few blood clots were found in the area between the stomach and the left liver hilum , but no signs of mediastinitis or free intra - abdominal air . four days later , as the patient became febrile , a second gastroscopy was performed . a 2 1.5 cm perforation was seen at the same site of ulcer with no bleeding ( fig . due to the patient 's clinical condition and the size of the gap , an endoscopic intervention was decided . a new 12-mm clip ( otsc ; ovesco endoscopy , tbingen , germany ) was engaged . the clip is made of nitinol and approximates large perforation margins like a surgical clamp . the edges of the perforation were approximated by using a specific endoscopic double grasping forceps and applying suction through the cap . thus , the tissue was pulled into the cap and the clip was released by rotating the wheel attached to the shaft of the endoscope . a nasogastric levin tube was uneventfully left in the stomach under direct vision for long - term enteral feeding . further , the patient was offered two intra - abdominal drainage catheters in the operating theater so that intra - abdominal air and blood clots be drained and high fever get under control . all intra - abdominal and peripheral blood cultures were sterile . the patient was kept on intravenous antibiotics , proton pump inhibitors and parenteral nutrition over the next 10 days followed by enteral feeding through the levin tube . two weeks later she was transferred to the clinical ward , experiencing no dysphagia , and was discharged fully recovered 3 months after the operation . tee has safely been applied in patients undergoing cardiac surgery and in icu departments for diagnostic purposes and monitoring over 30 years . factors that may determine the way of intervention following esophageal perforation are age , clinical condition , location and size of the perforation , cost of intervention and elapsed time since the injury . the time since perforation has occurred is crucial . a diagnosis delayed for more than 36 h is associated with high mortality ( 50% ) , despite surgical intervention . up to date , there have been no randomized controlled trials comparing surgical to endoscopic intervention regarding patient outcome . in our case , the tee performed in the operating theater and the icu led to esophageal perforation due to the patient 's esophagus anatomy . the delayed diagnosis of perforation is attributed to the blood thrombus on top of the ulcer seen in the first endoscopy that behaved as a sealant . our patient 's serious clinical condition precluded major surgical intervention , and it was decided to provide her with endoscopic therapy . additionally , due to the size of perforation and cost , a single clip that could seal the gap was very appealing . last , by leaving this clip in place for a long time , longer than common clips , could possibly guarantee safe sealing of this perforation . according to clip manufacturers , the clip may remain in place for over 6 months and is magnetic resonance imaging - safe . esophageal perforations closed endoscopically range from 3 to 25 mm in size . due to the new device technology ( twin grasper and the cap at the tip of endoscope ) , a single clip is enough and able to create a full - thickness closure of perforations up to 3 cm in diameter . this new clip provides a secure and successful esophageal perforation closure rate , up to 100% in perforations diagnosed within the first 24 h . according to this study , the clip remains in place for over 6 months and the mean repairing time of an esophageal perforation after successful multiple clipping is 18 days ( interquartile range 626 ) . most importantly , chronicity of perforation ( > 10 days ) was the only independent predictive factor for successful perforation closure in univariate and multivariate analysis . however , esophageal perforation as a complication of this clip during patient intubation has been reported in up to 3% of patients , but probably this can be attributed to the learning curve . indeed , in another study with 94 patients regarding the use of the otsc clip to narrow the pouch in patients who underwent gastric bypass surgery , no esophageal perforation occurred . in conclusion , although tee is a safe procedure , it may be complicated by esophageal perforation . we clearly showed and strongly suggest that esophageal perforation of 5 days can safely and successfully be treated endoscopically by applying this new otsc clip device .
esophageal perforation due to transesophageal echocardiogram ( tee ) during cardiac surgery is rare . a 72-year - old female underwent tee during an operation for aortic valve replacement . further , the patient presented hematemesis . gastroscopy revealed an esophageal bleeding ulcer . endoscopic therapy was successful . although a ct scan excluded perforation , the patient became febrile , and a second gastroscopy revealed a big perforation at the site of ulcer . the patient 's clinical condition required endoscopic intervention with a new otsc clip ( ovesco endoscopy , tbingen , germany ) . the perforation was successfully sealed . the patient remained on intravenous antibiotics , proton pump inhibitors and parenteral nutrition for few days , followed by enteral feeding . she was discharged fully recovered 3 months later . we clearly demonstrate an effective , less invasive treatment of an esophageal perforation with a new endoscopic clip .
thrombotic microangiopathies result from damage to the endothelium leading to a cascade of thrombosis and resultant anemia , thrombocytopenia , and renal damage . atypical hemolytic uremic syndrome ( ahus ) , a rare genetic disorder of this class stems from a rapid inappropriate activation of the complement system , termed atypical due to the lack of a triggering event akin to conventional hus which starts with exposure to the shiga - like toxin . the mortality in pediatric - onset ahus is reported to be more ( 6.7% ) while adult onset has higher chances of progression to end - stage renal disease . newer therapy such as the complement binding antibody eculizumab is financially unviable at present in the indian setting . a 14-year - old male , presented with complaints of sudden reddish discoloration of urine 5 days back followed by yellowing of eyes and skin , nausea , and vomiting associated with feeds . on routine investigation , he was anemic ( hemoglobin 4.9 g% ) with thrombocytopenia ( platelets 97,000 ) . renal functions were deranged with a serum creatinine of 4.27 and blood urea of 116.8 . a peripheral blood smear revealed dimorphic anemia with thrombocytopenia with polymorphonuclear leukocytosis with abundant schistocytes and tear drop cells suggestive of hemolytic uremic syndrome with a reticulocyte count of 30% . anti - nuclear antibodies were absent with low complement 3 ( c3 ) and normal c4 levels . anti - complement factor h ( cfh ) antibodies were found to be significantly raised at 2043 au / ml . he was initiated on plasma exchange with 7 initial daily cycles and a total of 16 cycles titrated to clinical response . anti - cfh antibodies were repeated and found to have decreased to 191.07 au / ml . he later developed sudden onset severe headache followed by loss of vision progressing to generalized tonic he was intubated and later successfully weaned off mechanical ventilation . a magnetic resonance imaging ( mri ) brain revealed posterior reversible encephalopathy with altered signal intensity in a subcortical white matter of the bilateral parietal , occipital , posterior temporal lobes and cerebellar hemispheres [ figure 1 ] . severe skin rashes developed almost 2 months into admission : centripetal in development with mucosal involvement and was diagnosed as steven he was started on further immunosuppression with steroids and azathioprine and discharged on day 91 of admission in complete clinical and hematological remission . ( b ) thermal - infrared images show symmetrical hypointense lesions in bilateral parietal lobes . ( c ) fluid - attenuated inversion recovery hyperintensities in occipital lobe typical of posterior reversible encephalopathy . ahus is a thrombotic microangiopathy resulting from mutations in cfh , complement factor i , membrane cofactor protein ( cd46 ) , c3 , thrombomodulin , cfh - receptor 5 , or from autoantibodies to cfh . autoantibodies to cfh reported in 4%14% of all cases , however , are much more common in cases with early onset of disease and are present in up to 25% of such cases . autoantibodies to cfh prevent cell surface protection by cfh , chiefly by inhibiting binding to c3b . ahus may be suspected in patients with typical history along with proven negative stool cultures for shiga - like toxin and normal adamts13 activity and can be confirmed by genetic assays , though empiric treatment is often initiated . the autoimmune variant , however , as in our case may not have a genetic focus and can be diagnosed by high levels of antibodies to cfh ( 2043 au / ml in our case ) . the overall ahus has incomplete penetrance , and an infectious trigger is often associated with the disease precipitating . our case presented with a high - grade fever of undocumented etiology which was probably the trigger for complement activation . globally , eculizumab , the monoclonal antibody to c5b , has found acceptance in the treatment of ahus . guidelines issued in 2009 recommended initial daily plasma exchange ( 5070 ml / kg ) with further titration of frequency according to clinical response . our patient received an initial seven cycles of daily plasma exchange followed by gradual tapering with monitoring of hematological and renal parameters which showed complete normalization by the 12 cycle . cfh - related ahus , similar to our patient , has been documented to respond better to plasma exchange . our patient did not develop any requirement for renal replacement therapy with hemodialysis as is the norm in a majority of cases of ahus . hypertension is a common complication of ahus but developed late in the course of treatment in our patient . posterior reversible encephalopathy with associated loss of vision may have an identical presentation as cerebral thrombotic microangiopathy . the latter can be differentiated on the basis of asymmetry of the lesions on mri . although neurological sequelae respond best to eculizumab , our patient was already in remission at the time of onset . johnson syndrome was probably a coincidental reaction to phenytoin and does not relate to ahus in the available literature . cyclophosphamide , mycophenolate mofetil , azathioprine and steroids , all have supporting literature for maintenance therapy . 36.5%63% progress to mortality or end - stage renal disease in the long - term . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .
atypical hemolytic uremic syndrome ( ahus ) is a rare disorder resulting from a dysregulated activation of the alternative pathway of the complement system . it results in significant morbidity and mortality if not diagnosed and treated promptly . it lends itself to myriad renal and extrarenal manifestations , all potentially disabling . eculizumab , a monoclonal antibody to complement c5 is now the widely accepted norm for treatment . however , in resource - limited settings , plasma exchange if instituted early may be as beneficial . we report a case of ahus treated with extended plasma exchange with excellent results . critical care monitoring is essential for the management of the disease in view of a tendency to develop multiple complications . long - term immunosuppression may be successful in maintaining remission .
skeletal muscle metastasis as the initial presentation of an unknown primary lung cancer is unusual . f-18 fluorodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) imaging is useful in the identification of primary in carcinoma of unknown origin . we describe a patient showing fdg - avid metastases to the skeletal muscles along with a previously unknown primary tumor in the right lung , in a patient presenting with initial muscular symptoms without any pulmonary manifestations . the patient underwent a whole body f-18 fdg pet / ct to identify the site of the primary malignancy . increased fdg avidity ( standardized uptake value [ suvmax ] 9.0 ) was detected in an irregular heterogeneously enhancing soft - tissue mass in the right paravertebral region in the upper lobe of the right lung with a focus of calcification within the mass [ figure 1b and d , white arrow ] . abnormal fdg uptake was also noted in a presacral mass [ figure 1c and e ] , the bulky left adrenal gland , several dorsal vertebrae and multiple lesions in the trapezius [ figure 1b and d , red arrow ] , right brachioradialis [ figure 2 ] , deltoid , and right external oblique muscles [ figure 3 ] , suggestive of metastatic involvement . a diagnosis of primary squamous cell carcinoma of the lung was pathologically the patient was treated with 4 cycles of chemotherapy , after which significant decrease in fdg uptake ( suvmax = 5.1 ) was seen in the primary as well as the right brachioradialis muscle lesion ( not shown here ) . fluorodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) maximum intensity projection ( mip ) image ( a ) showing multiple foci of abnormal tracer uptake . transaxial thoracic ct ( b ) and fused pet / ct image ( d ) show increased fdg uptake in an irregular , heterogeneously enhancing soft - tissue mass ( white arrow ) in the paravertebral region in the upper lobe of the right lung with calcification within the mass . increased fdg uptake is also seen in a peripherally enhancing ring like lesion in the trapezius ( red arrow ) . axial ct ( c ) and fused pet / ct ( e ) images at the level of the rectum show increased fdg uptake in a heterogeneously enhancing pre - sacral soft - tissue deposit coronal and transaxial computed tomography ( ct ) ( a and c ) and fused positron emission tomography / ct ( b and d ) images of the right hand showing increased fluorodeoxyglucose uptake in the right brachioradialis muscle with no increase in attenuation ( probably because the images were acquired after completion of the whole body pet scan ) axial computed tomography ( a ) and fused positron emission tomography / computed tomography ( b ) images showing increased tracer uptake in a ring - like hyperenhancing lesion in the right external oblique muscle suggestive of muscle metastasis found that only 4 patients ( 0.16% ) among 2,557 patients with lung cancer developed metastasis to the skeletal muscle . whole - body fdg pet / ct imaging is useful in detection of muscle metastases in lung cancer patients . multiple muscle metastases from lung cancer are rare , and fdg pet / ct imaging is useful in the identification of unsuspected metastatic sites . primary presentation of a skeletal muscle metastasis , such as in our case , remains an unusual occurrence . the present case , where the initial presentation was of metastatic muscular involvement , highlights the role of fdg pet / ct in tracing the location of primary lung malignancy and unsuspected sites of multiple muscle metastases in a patient with muscle metastases of unknown primary .
skeletal muscle metastasis as the initial presentation of the unknown primary lung cancer is unusual . a 65-year - old male patient presented with pain and swelling of the right forearm . fine needle aspiration of the swelling revealed metastatic squamous cell carcinoma . the patient underwent whole body f-18 fluorodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) to identify the site of the primary malignancy . the authors present pet / ct images showing fdg - avid metastases to the skeletal muscles along with a previously unknown primary tumor in the right lung , in a patient presenting with initial muscular symptoms without any pulmonary manifestations .
although it comprises only 10 - 15% of all hepatobiliary neoplasms , its incidence is increasing.1 frequent metastatic sites of biliary cancer are the liver , peritoneum , intra - abdominal lymph nodes , and lungs.2 here , we describe a very rare case in which metastatic cholangiocarcinoma of the stomach was mistaken for primary gastric cancer in a patient who underwent whipple 's operation . however , histopathologic and immunohistochemical findings suggested that the gastric tumor was a metastatic adenocarcinoma originated from a distal cholangiocarcinoma . a 67-year - old woman presented with a 4-month history of abdominal pain after meals . laboratory findings was as follows : aspartate aminotransferase 141 iu / l ( normal ; 10 - 40 iu / l ) and total bilirubin 0.9 mg / dl ( 0.2 - 1.0 mg / dl ) . computed tomography showed a malignant tumor of the common bile duct ( cbd ) , located just above the intrapancreatic segment , with consequent dilatation of the proximal biliary tree including the gall bladder ( fig . 1 ) . the periportal , common hepatic , and portocaval lymph nodes were enlarged . endoscopy revealed a 2-cm , flat , elevated lesion with convergence of the surrounding folds , situated at the gastric angle ( fig . based on the biopsy results , an adenocarcinoma , thought to be an early gastric cancer , was diagnosed . the patient underwent whipples 's operation . the surgical specimen consisted of a whitish mass , 2.11.3 cm in diameter , removed from the distal cbd and showing invasion of the pancreas and peripancreatic fat . pathologic examination of the resected stomach demonstrated that the tumor was very clearly demarcated from the surrounding nontumorous gastric mucosa and submucosa , and also showed invading lymphovascular spaces . in addition , the adenocarcinomatous tissue had infiltrated into the gastric mucosa and submucosa , while the gastric superficial mucosa was intact ( fig . immunohistochemical stains showed that the tumor tissue was strongly positive for cytokeratin ( ck)-7 and weakly positive for ck-19 , while the surrounding gastric mucosa was negative for both cytokeratins ( fig . according to a number of reports in the literature , metastasis to the stomach is a rare occurrence , with a reported incidence of less than 1% . the main primary metastatic tumors are those of the breast ( 33% ) and lung ( 25% ) , and malignant melanoma ( 22%).3 in our patient , it was difficult to distinguish gastric metastasis of a cholangiocarcinoma from a primary gastric cancer on the basis of clinical , endoscopic , and radiologic features . thus , results obtained from complete histopathologic and immunohistochemical studies of gastric biopsies should be compared with the characteristic features of cancer of the cbd . the anatomic distribution of ck-7 and ck-19 is generally restricted to epithelia of primary sites and their neoplasm . primary gastric cancer has the same staining pattern , because ck-7 and ck-19 expressions are reported to be positive in about 80% of stomach cancer cases . however , both the normal gastric mucosa surrounding the tumor and the gastric carcinoma , if it originates from the stomach , are positive for ck-7 and ck-19.4,5 metastasis to the gastrointestinal tract initiates from the serosa and submucosa and progresses to cause intraluminal lesions , as occurred in this case.6 based on these considerations , we diagnosed metastatic gastric carcinoma from a distal cholangiocarcinoma . another important immunohistochemical marker , cdx-2 is a very useful marker to distinguish stomach cancers from bile duct cancers , because cdx-2 expression is reported to be positive in 61% of stomach cancer cases but only in 13% of bile duct cancers , however cdx-2 was not performed in our study.7 until now , unusual metastastic sites from cholangiocarcinoma were reported to be the colon , adrenal gland , skull bone , epididymis , corneal limbus , meninges , ovary , skeletal musle , and skin.2,8 - 15 metastasis of a distal cholangiocarcinoma to the stomach has not been reported previously to our knowledge but , as in the above - mentioned sites , appears to be a rare site of metastasis of this tumor . in conclusion , a careful histopathologic and immunohistochemical review is very important in diagnostic differentiation of metastatic tumors from primary sites in this case .
we report an unusual case of distal cholangiocarcinoma with gastric metastasis mimicking early gastric cancer . a 67-year - old woman presented with a 4-month history of abdominal pain after eating . computed tomography showed a malignant tumor of the common bile duct located just above the intrapancreatic segment , and endoscopy revealed a 2-cm , flat , elevated lesion with convergence of the surrounding folds , situated at the gastric angle . based on the endoscopic biopsy results , an adenocarcinoma , thought to be an early gastric cancer , was diagnosed . the patient underwent whipples 's operation . histopathological findings showed that the adenocarcinomatous tissue was clearly demarcated and infiltrated the gastric mucosa and submucosa , leaving the gastric superficial mucosa intact . both tumors showed similar pathological features and were positive for cytokeratin ( ck)-19 and ck-7 . these finding suggest distal cholangiocarcinoma with gastric metastasis .
diphenyl cyclo propenone ( dpcp ) is used as a topical immunomodulator in alopecia areata . it is a potent contact allergen in humans and animals ; 98 - 99% of the cases of alopecia areata can be sensitized on the scalp skin . although its use has been increasing of late , the process of procuring , dilution and storage at a particular concentration is cumbersome and limits its wide use . this short communication aims to make the dpcp preparation and application easy for the readers . dpcp is available as 1 and 5 g powder in amber - colored glass bottles . the standard solvent , acetone , is a strong uv light absorber and inhibits this process . for dilution , first , the dpcp is weighed in a weighing scale and then diluted with the required quantity of acetone . initial sensitization is carried out with a 2% solution , which is made by dissolving 20 mg in 1 ml of acetone . further dilution can be prepared by making a stock solution of 2% and diluting with acetone taken in a pipette as per the concentration [ tables 1 and 2 ] . dilution for diphenyl cyclo propenone dilution from the stock solution 2% percentage = weight / volumedilution100 ( % = w / vdilution100 ) to prepare a 0.001% solution , 0.1 ml of stock solution is mixed with 200 ml of acetone , and 5 ml of the resulting solution can be used for application over the scalp . the rest of the solution should be stored in air tight , screw - capped amber - colored containers for further use . storage of the diluted solution is difficult as acetone often evaporates , leading to a change in the concentration . fresh solution can be made every time to avoid this . for concentrations of 0.01% and above , dpcp can be weighed prior and stored in glass containers to which acetone can be added in the required amount at the time of application as this saves time and prevents wastage ( e.g. , 0.01% can be made by adding 10 ml acetone in 1 mg preweighed dpcp containers ) . the diluted solutions are placed in a wide - mouthed glass beaker and applied on the scalp with cotton swab . application needs to be done fast to cover the entire scalp before acetone starts evaporating in the beaker . health care professionals should take proper precautions of wearing a glove , face mask and apron during the dpcp application as the spill of solution may cause an irritant / urticarial reaction . patients are advised to wait for 5 - 10 min in the out patient department before covering their head with a cap or cloth to protect from sunlight . after 48 h , patients should be advised to shampoo the scalp to remove the residual dpcp . during these 48 h , patients should be advised not to touch the scalp accidentally either by themselves or others . the common adverse effects after dpcp applications are local eczema with blistering , regional lymphadenopathy and contact urticaria . rare adverse effects include an erythema multiforme - like reaction , hyperpigmentation , hypopigmentation and vitiligo .
diphenyl cyclo propenone ( dpcp ) is used as a topical immunomodulator in alopecia areata . it is a potent allergen . the process of procuring , dilution and application limits its wider use . this short communication aims to make the dpcp application easy to use .
epidermolysis bullosa ( eb ) is a rare genetic disorder characterized by abnormal fragility of skin and mucosal surface . the separation of skin layers occurs after application of friction or shearing forces and results in intradermal fluid accumulation and bullae formation . in addition to considerations associated with positioning , monitoring , infection , and prevention of skin and mucosal trauma , anesthetic management of eb is uniquely challenging because of the effects on the airway . this case report describes the successful anesthetic management of a patient with eb presenting for syndactyly release . a 6-year - old male child , weighing 14 kg ; a known case of eb since birth presented with congenital left hand syndactyly [ figure 1 ] . significant preoperative findings were generalized scars , bed sores , pustules , and joint contractures . airway assessment revealed mallampatti class iii , microstomia , loose teeth , and poor oral hygiene with thyromental distance 3 cm . left hand shows syndactyly and right shows iv canula secured with nonadhesive technique airway assessment shows anticipated difficult airway oral antibiotics were administered preoperatively . intravenous ( iv ) line 22-g secured with use of emla and fixed with vaseline gauze . premedication with iv fentanyl 20 g , midazolam 0.4 mg , and dexamethasone 2 mg was given . were given with iv ranitidine 1 mg / kg and cefotaxime 50 mg / kg , respectively . intraoperative heart rate , rhythm , and oxygen saturation were monitored with lubricated clip on pulse oximeter . noninvasive blood pressure ( nibp ) and electrocardiogram monitoring were avoided [ figure 3 ] . patient was positioned supine and pressure points were padded with cotton . due to anticipated difficult airway , a difficult airway cart was prepared with various size masks , laryngoscope , endotracheal tubes ( ets ) , ventilating bougie , and stylet . though supraglotic airway devices were inappropriate in this patient they were kept ready for an emergency airway situation . minimal monitoring was used intraoperatively preoxygenation was followed by induction with iv propofol 20 mg along with oxygen and sevoflurane under gentle mask holding with vaseline gauze . after confirming ventilation , with use of lubricated macintosh laryngoscope trachea was intubated with uncuffed et no . 5 and tube secured with nonadhesive lubricated bandage [ figure 4 ] . et secure with nonadhesive technique anesthesia was maintained with oxygen , nitrous oxide , and sevoflurane with manual ventilation using jackson rees circuit . as rectal suppositories , subcutaneous and intradermal routes are generally not recommended ; postoperative analgesia was provided with iv diclofenac sodium 25 mg 8 hourly . regional block was avoided due to joint contracture and scars , which made location of anatomical landmarks difficult . neuromuscular blockade was antagonized at the end of the procedure , and trachea extubated when adequate signs of spontaneous recovery were evident . postoperatively , oral examination revealed small intra - oral blister formation not requiring active management . eb encompasses an array of autosomal dominant and recessive conditions that may have either localized or generalized dermatological manifestations . the loss or absence of normal intracellular bridges is due to a collagen abnormality , which makes patient susceptible for blister formation by friction / shearing forces and subsequent scarring . equipment and techniques routinely used in the induction and maintenance of general anesthesia can be the source of serious postoperative complications . anesthesia is frequently required for multiple surgeries like daily dressings , dental procedure , esophageal dilatation , gastrostomy , contracture release , and syndactyly release . malnutrition , anemia , and decreased immunity are present in patients due to decrease oral intake secondary to oropharyngeal and esophageal lesions . malnutrition can leads to hypoprotienemia , anemia and electrolyte imbalance which may affect pharmacokinetic effects of anesthetic agents . infection is common as patients often have both poor immunity and long - term corticosteroid treatment . antacid prophylaxis is required due to history of reflux , regurgitation , or esophageal stricture . patients with eb are prone for ophthalmic complications like corneal erosion , conjunctival symblepharon , and ectropion . dental problems like cleaning difficulty , poor eating patterns , enamel dysplasia , carious , and loose teeth are frequently associated . iv or intra - arterial access should be secured with bandage , cotton wrap , or suture . all persons involved in handling these children must be aware of the extreme vulnerability of skin . during transport or mobilization of the patient , the most important task is to maintain the integrity of the skin and avoid friction and trauma . direct pressure to the skin is not as damaging as frictional or shearing forces , so nibp and tourniquet can be used with pressure and duration limitations . as our case was of a short duration with no expected major blood loss and hemodynamic shifts , use of nibp and tourniquet therefore , clinical use of nibp and tourniquet in such patients depends on its merits and demerits . microstomia , neck contracture , oropharyngeal lesion , ankyloglossia ( decrease mobility of the tongue ) , thickened epiglottis , and possible tracheal stenosis make maintenance of a patent airway challenging . fiberoptic intubation is less traumatic to the mucosa than direct laryngoscopy and should be the first choice in eb patients with a difficult airway . laryngeal , tracheal involvement in eb is rare because that tissue is pseudostratified , columnar , ciliated epithelium and whereas oropharyngeal , esophageal mucosa is stratified squamous . supraglottic airway increases bullae formation but can be used in difficult airway scenario with appropriate care . after application of moisturizing ophthalmic gel , eyes should be covered with moistened gauze to protect from mechanical trauma . regional anesthesia should be considered whenever possible but contractures , scars , and infections are the issues associated with regional anesthesia . perioperative blister should be treated with liquid paraffin , silver sulphadiazine , or steroids . in ambulatory careful monitoring , transport and positioning was instituted in the present case to avoid undue skin trauma . atraumatic ventilation and intubation was possible with use of vaseline gauge and gentle mask holding . minimal mandatory monitoring and no touch principle remains the key to successful anesthesia management of eb cases . patients with eb present a unique challenge for all anesthesia - care providers . with maximal skin and mucous membrane protection , anesthesia in children with eb can be conducted with few sequelae . hence , meticulous execution of preoperative planning is indispensable for ensuring a favorable intraoperative and postoperative course .
epidermolysis bullosa ( eb ) is a rare genetic mechanobullous disorder , with excessive fragility of the skin and mucous membranes . avoiding mechanical injury to the skin and mucous membranes is essential in the anesthetic management . shearing forces applied to the skin result in bullae formation , while compressive forces to the skin are tolerated . the challenge is to use monitoring technology without damaging the epithelial surface . difficult airway , positioning issues , nutritional deficiencies , poor immunity , and carcinogenic potential add to the comorbidities . we managed a child with eb undergoing syndactyly release . ensuring maximal skin and mucous membrane protection , anesthesia in children with eb can be conducted with few sequelae .
during 20042007 , as part of a broader biodiversity survey and inventory program , we sampled birds from mostly forested sites in guangxi and guizhou provinces in the southern part of the people s republic of china ( figure ) . sampling was conducted by mist netting and selective harvesting with shotguns ; all birds in the study were apparently healthy and behaving normally at the time of collection . because initial sampling was focused on endoparasite communities , samples from 20042005 consisted of complete gastrointestinal tracts frozen in liquid nitrogen . in 20062007 southeastern asia , showing 5 sites in the people s republic of china where land birds were collected and tested for influenza a virus . prevalence values were 4% ( n = 103 ) in dashahe in 2006 ; 6% ( n = 194 ) in kuan kuoshui in 2006 ; 0.3% ( n = 328 ) in shuipu in 2007 ; 3% ( n = 184 ) in jing xi , in 2004 ; and 0% ( n = 130 ) in shiwandashan in 2005 . a total of 184 samples were collected from jing xi municipality in guangxi ( 21.122n , 105.964e ) in 2004 , 130 from shiwandashan nature reserve in guangxi ( 21.840n , 107.880e ) in 2005 , 103 from dashahe nature reserve in guizhou ( 29.167n , 107.575e ) in 2006 , 194 from kuan kuoshui nature reserve in guizhou ( 28.226n , 107.160e ) in 2006 , and 328 from shuipu village , guizhou province ( 25.485n , 107.882e ) in 2007 ( figure ) . samples were tested for influenza a virus by real - time reverse transcription pcr ( 6 ) in 2 diagnostic laboratories ( southeast poultry research laboratory , us department of agriculture , athens , ga , usa , and national wildlife health center , us geological survey , madison , wi , usa ) . of 939 samples tested , 24 were positive for influenza a viruses ( prevalence 2.3% , table ; complete summary in appendix table ) . if migratory behavior ( species classified as migratory or nonmigratory on the basis of descriptions by mackinnon and phillipps ) , was considered , 11 ( 4.8% ) of 231 samples from species showing marked seasonal migrations were influenza positive . however , only 13 ( 1.8% ) of 708 samples from nonmigratory species were positive . the cumulative binomial probability that such a high number ( 11 ) of positive samples would result among the 231 migratory - species samples , were the true prevalence to be 1.8% , is low ( p = 0.0013 ) . thus , migratory species appear to have higher influenza infection rates . in terms of general habitat use ( 7 ) , open - country species were slightly more prone to be influenza positive ( 8 [ 2.9% ] of 274 samples ) than forest species ( 16 [ 2.4% ] of 665 samples ) , but the difference was not significant ( cumulative binomial probability , p>0.05 ) . interactions between migratory behavior and habitat use were not significant ( contingency test , p>0.05 ) . although all infections detected were among songbirds ( passeriformes ) , the sampling also concentrated on songbirds ( 94.3% ) . thus , we could not test adequately the hypothesis that influenza prevalence was equivalent between songbirds and other birds . an obvious question is whether the influenza a viruses we detected belong to the highly pathogenic subtype h5n1 strain currently circulating across much of asia . pcr ( 6 ) , although this result does not exclude the possibility that h5 viruses were among the positive samples . the preservation status of samples we tested prevented virus isolation or full , strain - level characterization of influenza viruses . the subtype h5n1 strain of influenza virus has spread rapidly and has been detected across much of central and southern eurasia . although movements of wild birds have been implicated in this spread ( 8) , other studies question ( 9,10 ) or contradict ( 11 ) this idea . an important part of the argument centers on the question of the occurrence of the virus in wild birds without obvious illness , which can be difficult to interpret given the low prevalence of influenza . for instance , a recent study based on sampling > 13,000 migratory birds in china detected the subtype h5n1 strain of influenza virus only 8 times ( 12 ) , and similar results have been obtained elsewhere ( 2 ) . our study , although not successful in characterizing influenza viruses to specific strains , nonetheless shows that influenza a virus infection occurs in more bird species than previously assumed and that influenza a infections can be found in birds that behave normally and show no sign of illness . although a review of avian influenza virus ecology ( 1 ) discussed the occurrence of influenza viruses across all groups of birds ( and other vertebrates ) , subsequent studies have assumed that waterbirds are the primary reservoir ( 8,13,14 ) . in this study , although waterbirds could have higher prevalences , we have demonstrated broad occurrence of influenza viruses in diverse taxa of passeriformes ( songbirds ) in southeast asia . this result suggests that land birds may also be a major reservoir of influenza viruses . we have taken a step toward a more complete understanding of influenza virus ecology among wild birds . our partial survey of influenza virus distributions across the rich avifaunas of the southern region of china demonstrated frequent infections . we suggest that to be effective future surveillance efforts will need to include the full diversity of wild birds .
water birds are considered the reservoir for avian influenza viruses . we examined this assumption by sampling and real - time reverse transcription pcr testing of 939 asian land birds of 153 species . influenza a infection was found , particularly among migratory species . surveillance programs for monitoring spread of these viruses need to be redesigned .
peutz - jeghers is a rare autosomal dominant disorder characterized by hamartomatous polyps and discoloration of mucosal membranes . the polyps can occur anywhere in the gastrointestinal tract and can grow large enough to cause bowel obstructions . a 16-year - old male presented to the emergency department with signs and symptoms of an acute bowel obstruction . he had a previous history of a colonoscopy with polypectomy at age 4 , and hyperpigmentation of his mucous membranes . an exploratory laparoscopy found an intussusception of the mid jejunum . a laparoscopic - assisted small bowel resection was performed . colonoscopy and upper endoscopy revealed 5 more polyps in the stomach and colon that were removed . the treatment of obstruction in these patients is to remove the offending hamartomatous polyp(s ) . the rest of the intestine needs to be examined and those polyps found should be removed . peutz - jeghers syndrome ( pjs ) is a rare autosomal dominant condition characterized by hamartomatous polyps and mucocutaneous pigmentation of the lips , buccal mucosa , and digits . polyps can occur anywhere in the gastrointestinal tract and can grow large enough to cause bowel obstructions . a 16-year - old male presented to the emergency department with signs and symptoms of an acute bowel obstruction . he had a previous history of a colonoscopy with polypectomy at age 4 , had hyperpigmentation of his mucous membranes , and his mother and maternal grandfather had a history of gastrointestinal polyps . the patient underwent an exploratory laparoscopy and was found to have an intussusception of the mid jejunum ( figure 1 ) . pathology showed a 5-cm polyp that had acted as a lead point for the intussusception ( figure 2 ) . the patient had an uncomplicated postoperative course and was discharged home on postoperative day 3 . he is one year out from surgery and has resumed his normal activities with no evidence of recurrence . pjs was first described in 1921 by peutz and subsequently elaborated upon by jeghers in 1949 . germline defects in the tumor suppressor gene serine / threonine kinase 11 ( stk11 ) are implicated in this rare autosomal dominant inherited disease . its incidence is calculated in 1 in 200,000 liveborns , and its mean age of onset is 25.2 years . the most common location of the hamartomatous polyps is the small bowel ( 78% ) , followed by the colon ( 42% ) , stomach ( 38% ) , and rectum ( 28% ) . these polyps can cause obstruction in up to 43% of cases and rectal bleeding in up to 14% of patients . the syndrome is associated with a 2% to 10% increased risk of cancer of the intestinal tract , from the stomach to the rectum . there is also an increased risk of extraintestinal malignancies , including cancer of the breast , ovary , cervix , fallopian tubes , thyroid , lung , gallbladder , bile ducts , pancreas , and testes . however , recurrence of intussusception episodes occurs in at least 10% of cases , resulting in repeated surgical intervention . thus , the rest of the intestine needs to be examined , and those polyps found should be removed . once the gastrointestinal tract has been cleared of polyps , the recommended interval of small bowel follow - through is from 2 years to 3 years . the presence of polyps larger than 1.5 cm in diameter mandates another complete gastrointestinal evaluation with endoscopic removal of polyps . patients should also be screened periodically for malignancies of the breast , cervix , ovary , testis , stomach , and pancreas . to our knowledge , there are at least 2 other published case reports regarding the laparoscopic management of bowel obstructions in pjs . the ideal way to remove a pedunculated polyp acting as a lead point , laparoscopy offers a safe and effective method for surgical management with reduction of the intussusception and small bowel resection . the diagnosis of pjs should be considered in patients presenting with a clinical picture of bowel obstruction and mucocutaneous hyperpigmentation . if the diagnosis is made preoperatively , optimal management should include laparoscopic treatment of the bowel obstruction and intraoperative enteroscopy . if the diagnosis is made after the operation , the patient needs complete evaluation of their gastrointestinal tract .
introduction : peutz - jeghers is a rare autosomal dominant disorder characterized by hamartomatous polyps and discoloration of mucosal membranes . the polyps can occur anywhere in the gastrointestinal tract and can grow large enough to cause bowel obstructions.case report : a 16-year - old male presented to the emergency department with signs and symptoms of an acute bowel obstruction . he had 2 days of abdominal pain , obstipation , and vomiting . he had a previous history of a colonoscopy with polypectomy at age 4 , and hyperpigmentation of his mucous membranes.results:computed tomographic ( ct ) scan revealed an intussusception of the small intestine . an exploratory laparoscopy found an intussusception of the mid jejunum . a laparoscopic - assisted small bowel resection was performed . pathology showed a 5-cm polyp that acted as a lead point for the intussusception . colonoscopy and upper endoscopy revealed 5 more polyps in the stomach and colon that were removed.conclusion:small bowel obstructions can be managed successfully with minimally invasive approaches . the treatment of obstruction in these patients is to remove the offending hamartomatous polyp(s ) . the rest of the intestine needs to be examined and those polyps found should be removed . this can be done intraoperatively with laparoscopic - assisted enteroscopy and colonoscopy .
from november 2008 through december 2011 , mrsa of companion animal origin was routinely isolated from specimens submitted for diagnostic purposes to vet med labor gmbh in ludwigsburg , germany , or to the institute of microbiology and epizootics , freie universitt berlin , in berlin , germany . s. aureus was confirmed as described ( 7 ) and stored in glycerol stocks at 80c . pcr routinely used to confirm methicillin resistance and species identity had failed to produce a positive signal for meca in 10 mrsa isolates from companion animals ( 2 isolates from dogs , 7 from cats , and 1 from a guinea pig ) ( 8) . we screened these 10 isolates for the meca homologue by using the pcr method published by cuny et al . ( 5 ) and sent the amplicons obtained to lgc genomics gmbh ( berlin , germany ) for sequencing . automated antimicrobial drug susceptibility testing was performed by using the biomrieux vitek 2 system ( nrtingen , germany ) according to the manufacturer s instructions . the following drugs were tested according to clinical and laboratory standards institute guidelines m31a3 : penicillin , ampicillin sulbactam , oxacillin , gentamicin , kanamycin , enrofloxacin , marbofloxacin , erythromycin , clindamycin , tetracycline , nitrofurantoin , chloramphenicol , and trimethoprim sulfamethoxazole , ( 9 ) . all isolates were further characterized by spa typing , multilocus sequence typing , and microarray hybridization by using the alere identibac s. aureus genotyping chip ( alere technologies gmbh , jena , germany ) as described ( 1012 ) . all pcr amplicons demonstrated 100% identity with the dna sequence of mecalga251 ( national center for biotechnology information no . the strains originated from geographically diverse areas ( 5 federal states of germany ) and were isolated from different infection sites ( table ) . all strains were identified as mrsa by the vitek 2 system ( growth in the presence of 6 g / ml cefoxitin according to the vitek 2 advanced expert system ) , although oxacillin mics were rather low ( 0.5 g / ml ) or moderately high ( 4 g / ml ) ( table ) . imt , institute of microbiology and epizootics , freie universitt berlin , berlin , germany ; oxa , oxacillin ; st , sequence type ; vb , vet med labor gmbh , ludwigsburg , germany . all isolates were positive for nuc and negative for meca according to pcr to detect mrsa ( 8) , and all were positive for mecalga251 according to pcr to detect the novel meca homologue ( 5 ) . as has been described for atypical mrsa , 4 strains belonged to st130 and 1 strain belonged to st1945 ( differs from st130 by 1 allele ) ( 35 ) . the remaining 5 isolates were assigned to st599 ( differs from st121 by 2 alleles ) ( table ) . st599 has been reported for methicillin - susceptible isolates from humans in europe , asia , and africa ( www.mlst.net ) . the figure shows a minimum spanning tree based on 4,197 entries of the s. aureus multilocus sequence type database ( http://saureus.mlst.net/ ) as of january 19 , 2012 ( figure , panel a ) and a detailed view of the branches and sts harboring strains with the novel meca homologue published ( figure , panel b ) ( 35 ) . minimum spanning tree based on multilocus sequence typing data from 4,197 staphylococcus aureus strains ( a ) and an enlarged view of 1 phylogenetic group ( b ) . each circle represents a distinct sequence type ( st ) , and circle size is proportional to st frequency . green indicates mecalga251-positive s. aureus strains of companion animal origin reported in this study and sequence data from published multilocus sequence typing results ( 35 ) ; red indicates st599 methicillin - resistant s. aureus ; and blue represents st2024 methicillin - sensitive s. aureus isolated from a wild rat . microarray hybridization data revealed that the agr type i and capsule type 5 seem to be associated with st599 and agriii and that clonal complex ( cc ) 130 isolates harbor the capsule type 8 encoding gene . cc130 and st599 isolates were positive for the surface - associated proteins clfa , clfb , fnba , and bbp . all st599 strains produced a positive hybridization result for 1 of the gene variants encoding the toxin responsible for toxic shock syndrome ( tst1 or tst - bov ) , and all but 1 of them harbored the enterotoxins c ( sec ) and l ( sel ) , indicating the presence of an s. aureus pathogenicity island that encodes superantigens ( 13 ) . positive or ambiguous hybridization signals for ccrb1 , ccra3 , and ccrb3 were obtained for 5 isolates , suggesting the presence of the sccmecxi in those strains , according to the results of shore et al . our findings of cc130 and st599 mrsa harboring mecalga251 in several companion animal species suggest that in germany , the presence of the meca homologue in mrsa is not exclusively associated with cc130 . this finding supports the hypothesis that some , if not all , mrsa strains that harbor the novel meca variant can cause infections among a broad variety of hosts , as has been shown for mrsa of human , equine , canine , and other companion animal origins ( 1,7 ) . all currently known mecalga251carrying mrsa were observed in a distinct section of the s. aureus population ( figure , panel b ) . whether this phylogenetic group possesses the ability to integrate the novel meca variant needs to be further investigated . in the past however , all isolates were correctly identified as mrsa by the vitek 2 system , as reported ( 4 ) . of the 10 isolates , a recent study identified cats as a potential natural reservoir for s. aureus of cc133 , a genetic lineage that has also been reported for s. aureus of ruminant origin ( 14 ) . moreover , we have identified a cc130 strain ( mssa_st2024 , t8403 ) from a wild rat ( imt21250 ; id4035 ) ( www.mlst.net ) . in addition , cc130 mrsa containing the meca homologue has only recently been reported for isolates from humans in germany ( 5 ) . although many investigators focus on livestock - associated mrsa , and because particular companion animal lineages of mrsa seem to be lacking , transmission of mrsa between companion animals and human family members in close proximity might be underestimated , especially in cases of recurrent infection ( 15 ) . the emergence of mrsa harboring the novel meca homologue has consequences for the verification methods for mrsa used in veterinary medicine ; implementation of new methods will be inevitable . their supposed restriction to only a few genetic lineages and the potential risk for interspecies transmission of atypical mrsa between companion animals and their owners in household environments needs further elucidation .
methicillin - resistant staphylocoocus aureus ( mrsa ) harboring mecalga251 has been isolated from humans and ruminants . database screening identified this mrsa variant in cats , dogs , and a guinea pig in germany during 20082011 . the novel mrsa variant is not restricted to ruminants or humans , and contact with companion animals might pose a zoonotic risk .
today , the treatment of choice is surgical excision of the tongue , but before 1900 , surgery was often temida1 . only in 1673 niels the size and shape of the teeth are directly influenced by the size of tongue3 . the shape of the teeth is determined by forces employed on the teeth , especially the muscles of the tongue , lips and cheek . due to the effects caused by the aesthetic and functional macroglossia the macroglossia is classified as true when there is excessive enlargement of the language and a relative when there is an imbalance between the size of the tongue and oral cavity , resulting in insufficient space for organ4 . the goal is to establish technical bases associated with partial glossectomy orthodontic treatment of dentofacial deformity in patients with macroglossia . three patients underwent orthognathic surgery associated with partial glossectomy from 1995 to 1999 , a multidisciplinary team - doctor and dentist . all patients had macroglossia relative and underwent clinical assessment taking into account the respiratory function , swallowing and speech deficit , as well as changes in dental occlusion and was also performed radiological evaluation . the main problem was manifested by the presence of steep lower dent alveolar , destabilizing the use of dentures causing joint dysfunction with severe pain . she underwent a single surgical intervention in the partial glossectomy and orthognathic surgery for targeting sub apical segment dent alveolar anterior inferior . the main problem of the second patient was the anterior open bite accompanied by difficulty in breathing and articulation of words . after treatment for orthodontic tooth alignment leveling , underwent surgery for a single suspension by corticotomy posterior maxillary le fort i type , reduction of mandibullary prognathism by sagittal technical branches , targeting sub apical posterior - inferior right and partial glossectomy . the third patient had mandibullary prognathism and , during orthodontic treatment in preparation for orthognathic surgery , the surgery was anticipated , since the interposition of the tongue between the back teeth did not allow the evolution of orthodontic treatment . he underwent a partial glossectomy to reduce the transverse diameter of the tongue and mandibullary prognathism by sagittal technical branches . we used rigid skeletal fixation with titanium plates and screws so that patients could stay without intermaxillary block in the immediate postoperative period . to control tongue edema , the technique used consist of segmental resection along the median raphe of the tongue and suture by planes - figure 1 . as a routine the symptoms regressed completely and all skeletal segments remained stable . demarcation and resection of the lingual and final appearance . the classification of macroglossia is not yet consensus in the literature . according to shafer ( 1968)5 , or primary congenital macroglossia is due to the excessive development of the musculature , which may or may not be associated with generalized muscular hypertrophy or unilateral hypertrophy . since the secondary macroglossia may occur as a result of a tumor in the tongue , as a diffuse hemangioma or lymphangioma , neurofibromatosis , and occasionally blocking efferent vessels in cases of malignant neoplasm of the tongue . wolford et al . ( 1996)6 refer to as macroglossia on pseudomacroglossia , separating it from the true macroglossia . ( 1980)7 macroglossia consider the functional as a third classification , occurring when the language does not fit into the oral cavity after a surgical procedure . the true when there are histological abnormalities associated with the increase of the tongue , such as vascular malformation , stretching and tumors . relative macroglossia includes cases of apparent increase in volume without an explanation of the language exam , as in down syndrome . the decision to refer the patient to partial glossectomy should be based on the volume of the language , mobility , position , function , symptoms , speech intelligibility , skeletal anterior open bite , interference in orthodontic treatment , drooling , swallowing and tongue recurrent trauma9 . the language has increased in volume expansive effect on the lower dental arch , being blamed as the cause and maintenance of open bite , bimaxillary protrusion or diastemas10 . being interposed between the arches , is an important etiologic factor for malocclusion listed ( figures 2 and 3 ) . a partial glossectomy performed simultaneously with mandibullary osteotomy for treatment of patients with mandibullary prognathism and anterior open bite is advantageous to prevent recidivas11 . the tongue can cause deformity increased dental - muscle - skeletal , instability in orthodontic treatment and orthognathic surgery , masticatory disability , communication problems and respiratory6 . there are several clinical and radiographic findings , but not all features are always present and their existence is not necessarily path gnomonic for the diagnosis of macroglossia . should be included the clinical , radiographic and functional for the interference with speech , mastication , airway and stability of orthodontic treatment and orthognathic surgery . there are basically three choices in the surgical sequence : ( i ) stage 1 : partial glossectomy , stage 2 : orthognathic surgery ( ii ) stage 1 : orthognathic surgery , stage 2 : partial glossectomy and ( iii ) partial glossectomy and orthognathic surgery in a single stage surgery .
summary introduction : macroglossia is a condition which influences the size and shape of the teeth employed due to the forces on teeth . objective : to establish bases for the indication of partial glossectomy associated with orthodontic treatment and surgical dento - facial deformity in patients without tumors and down syndrome as a cause of macroglossia . case reports : three patients underwent orthognathic surgery associated with partial glossectomy under general anesthesia . all patients had macroglossia relative and underwent clinical assessment taking into account the respiratory function , swallowing and speech deficits and radiological evaluation . the technique used consist of segmental resection along the median raphe of the tongue and suture by planes . we used rigid skeletal fixation with titanium plates and screws so that patients could stay without intermaxillary block in the immediate postoperative period . were followed over five years . the symptoms regressed completely and all skeletal segments remained stable . discussion : the decision to refer the patient to partial glossectomy should be based on the volume of the language , mobility , position , function , symptoms , speech intelligibility , skeletal anterior open bite , interference in orthodontic treatment , drooling , swallowing and tongue trauma applicant .
these viruses infect a variety of species , including aquatic birds , poultry , pigs , horses , dogs , and humans , causing significant morbidity and mortality . in the case of human influenza viruses , in addition to the burden of yearly epidemics , there is the ever - present threat of an influenza pandemic . pandemics occur when a novel strain of influenza virus of animal origin evolves the ability to infect and efficiently transmit among humans . while some influenza pandemics , like the 1918 h1n1 pandemic that killed more than 40 million people worldwide , have had devastating consequences , others , including the h1n1 pandemic in 2009 , which resulted in an estimated 18,000 deaths , have been considerably milder . unfortunately , the emergence of new human pandemic viruses , as well as the subtype and virulence of the causative viruses , is still unpredictable . although there are vaccines available for certain influenza viruses , they are strain specific , and the generation and general distribution of a new influenza virus vaccine take more time than the spread of a new virus , severely limiting the impact of vaccines in the first wave of a pandemic . influenza antiviral drugs are of broader specificity , but resistance to those drugs and problems with availability limit their use . in order to mitigate the possible impact of an influenza pandemic , we need more research and development in the generation of improved vaccines and antivirals with broad cross - reactivity against multiple influenza viruses . in addition , by investigating the factors responsible for the generation of human pandemic viruses , we can better recognize the risks that animal influenza virus strains pose to humans and build eradication campaigns to target specific viral strains circulating in animals . critical to this issue is the question of what makes an influenza virus transmissible in humans and animals . a better understanding of influenza transmission will lead to the development of countermeasures for viral transmission that can enhance our pandemic preparedness plans . several mutations and changes associated with increased airborne transmission of avian influenza viruses in ferrets have been identified in recent years ( 13 ) . however , previous attempts to identify adaptive changes in highly pathogenic avian h5n1 viruses associated with transmission have failed , supporting the possibility that h5n1 viruses might be structurally unfit for mammalian transmission ( 4 ) . more recently , two independent laboratories lead by ron fouchier and yoshihiro kawaoka have ruled out this hypothesis and identified specific mutations that allow the h5n1 virus to accomplish airborne transmission in ferrets ( unpublished observations ) . while this provides important information on the adaptability of h5n1 viruses , more research needs to be conducted to understand the possibility that avian h5n1 could evolve to become transmissible in humans , to predict its pathogenesis in humans , and to find the molecular mechanisms responsible for host specificity in influenza virus transmission . while research on influenza virus transmission is critical for finding ways to better tackle this pathogen , it is important to conduct such research using appropriate biocontainment and biosafety conditions to minimize possible risks of virus release to the environment . risk assessment is a crucial tool in selecting biocontainment levels for research on potentially dangerous pathogens . according to the biosafety in microbiological and biomedical laboratories ( bmbl ) manual ( 5 ) , the definitive reference book for biosafety issues , biological risk associated with pathogen research is determined by three elements : the activities that can result in human exposure to the pathogen , the probability that such exposure would cause an infection , and the consequences of such an infection . although ferret - adapted h5n1 viruses probably have attenuated infectivity and pathogenesis for humans , to minimize all possible risks one should use biocontainment facilities and practices that prevent human exposure . preventing the escape of viruses from the laboratory can be achieved by housing research activities in a facility equipped with interlocked rooms with negative pressure and high - efficiency particulate air ( hepa ) filtered air circulation and using the appropriate decontamination and/or sterilization practices for material leaving the facility . since human infection with influenza viruses occurs via the respiratory route , infection of laboratory personnel can be prevented by the use of powered air - purifying respirators . these practices correspond to enhanced biosafety level 3 ( bsl3 ) , as described in the bmbl manual . the effects of accidental exposure to the virus in an enhanced bsl3 facility can be minimized by vaccinating personnel with an h5n1 vaccine and through the use of antiviral drugs . increased biocontainment , or bsl4 , h5n1 viruses , on the other hand , are sensitive to the antivirals zanamivir and oseltamivir , and infection with the virus is preventable by vaccination with the h5n1 inactivated vaccine . influenza virus research is important for the development of novel intervention strategies for preventing and mitigating influenza epidemics and pandemics . as scientists , we have the responsibility to avoid the undue restrictions of the highest level of biocontainment if enhanced bsl3 facilities can provide the appropriate biosafety . the use of bsl4 containment would not decrease the risk of virus release any more than enhanced bsl3 containment , but it would result in an unnecessary burden that would restrict research on h5n1 influenza transmission to a few facilities and considerably decrease the speed of research on this important pathogen .
abstractresearch on h5n1 influenza viruses has received much attention recently due to the possible dangers associated with newly developed avian h5n1 viruses that were derived from highly pathogenic avian viruses and are now transmissible among ferrets via respiratory droplets . an appropriate discussion , based on scientific facts about the risks that such viruses pose and on the biocontainment facilities and practices necessary for working safely with these viruses , is needed . selecting the right level of biocontainment is critical for minimizing the risks associated with h5n1 research while simultaneously allowing an appropriately fast pace of discovery . rational countermeasures for preventing the spread of influenza can be developed only by gaining a thorough knowledge of the molecular mechanisms at work in host specificity and transmission .
, it was found that the thyroid gland had the maximum amount of selenium per gram of tissue . autoimmune thyroiditis ( ait ) , the prototype of autoimmune diseases , is characterized by t - cell - mediated autoimmune destruction of thyroid cells . environmental factors , such as iodide intake , immunotherapeutic agents , or viral infections that may initiate the disease . in areas , where selenium deficiency is prevalent , higher incidence of thyroiditis has been reported due to a decreased activity of selenium - dependent glutathione peroxidase enzyme within thyroid cells . severe nutritional selenium deficiency leads to an increased rate of thyroid cell necrosis and invasion of macrophages . whether this it may be assumed , however , that thyroid cell damage may initiate or maintain autoimmune thyroiditis , especially in patients susceptible to the development of autoimmune diseases . to study the effect of selenium supplementation in patients with autoimmune thyroid disease . patients of all age groups and both sexes with autoimmune thyroid disease ( as defined by an anti- thyroid peroxidase antibody [ tpoab ] level more than 150 iu / ml ) irrespective of the baseline thyroid status . patients with overt hyperthyroidism who are on antithyroid drugs , patients on any other medication , which may alter the immunity status of the patients , and pregnant patients were excluded from the study . patients were randomized into two age and tpoab - matched groups ; 30 patients received 200 g sodium selenite / day , orally , for 3 months , and 30 patients received placebo . the differences in antibody concentrations at the beginning and end of the study were determined by t - test for paired samples . patients of all age groups and both sexes with autoimmune thyroid disease ( as defined by an anti- thyroid peroxidase antibody [ tpoab ] level more than 150 iu / ml ) irrespective of the baseline thyroid status . patients with overt hyperthyroidism who are on antithyroid drugs , patients on any other medication , which may alter the immunity status of the patients , and pregnant patients were excluded from the study . patients were randomized into two age and tpoab - matched groups ; 30 patients received 200 g sodium selenite / day , orally , for 3 months , and 30 patients received placebo . the differences in antibody concentrations at the beginning and end of the study were determined by t - test for paired samples . in the selenium treated group 27 patients were female , and three patients were male ( m : f = 1:9 ) , which was comparable with the ratio in the placebo - treated group ( 1:7.3 ) the mean ages at presentation in both the groups were 34 2.5 and 31 3.4 years , respectively . at study entry , the mean tpoab concentrations were identical for both groups ( selenium treated group , 669 205 iu / ml ; placebo , 729 277 iu / ml ) . out of the total 30 patients in the selenium treated group , 6 patients were overtly hypothyroid , 15 were subclinical hypothyroid , 6 were euthyroid , and 3 were subclinical hyperthyroid . there were comparable numbers of patients in each subgroup in the placebo - treated group also . the mean tpoab concentration decreased significantly by 49.5% ( p < 0.013 ) in the selenium treated group versus 10.1% ( p < 0.95 ) in the placebo - treated group . in subgroup analysis , the decrease in the mean tpoab titre was highest in the subclinical hyperthyroid group ( up to 64.42% ) , and comparable in the other three groups ( 41.13% , 47.18% , and 42.64% in the euthyroid , hypothyroid , and subclinical hypothyroid groups respectively ) . one patient with hypothyroidism in the selenium treated group with a tpoab concentration of > 1000 iu / ml , had completely normalized antibody concentrations after 3 months . it was also found that those patients with tpoab greater than 1000 iu / ml revealed a mean 31.38% reduction in the selenium - treated patients , compared with no significant change in tpoab in the placebo group . . glutathione peroxidase can reduce hydrogen peroxides and phospholipid hydroperoxides , and hence can reduce the production of free radicals and reactive oxygen species . these mechanisms may contribute to reduced inflammatory activity in the organ - specific autoimmune response , and may explain the improvement of autoimmune thyroiditis in our study . based on the link described above between selenium and the thyroid , several studies applying organic and inorganic selenium compounds were undertaken in patients , with ait in areas with low to borderline - low - selenium content . a prospective placebo - controlled clinical study with selenium in ait conducted in the selenium deficient area of bavaria in southern germany , by grtner et al . in 2002 showed a 36% reduction in anti - tpo titers in the selenium - treated group , whereas a further reduction of up to 60% was seen in a subgroup of patients with basal anti - tpo levels above 1200 iu / ml . supplementation of selenium has a significant impact on inflammatory activity in thyroid - specific autoimmune disease . it would be of interest to determine whether early treatment with selenium in patients with newly developed autoimmune thyroiditis may delay , or even prevent the natural course of these diseases .
introduction : in areas with severe selenium deficiency higher incidence of thyroiditis has been reported due to a decreased activity of selenium - dependent glutathione peroxidase enzyme within thyroid cells.aims and objective : to study the effect of selenium supplementation in patients with autoimmune thyroid disease.materials and methods : this is a blinded placebo - controlled prospective study done in 60 patients with autoimmune thyroid disease ( as defined by an anti - thyroid peroxidase antibody ( tpoab ) level more than 150 iu / ml ) irrespective of the baseline thyroid status . patients with overt hyperthyroidism who are on antithyroid drugs , patients on any other medication , which may alter the immunity status of the patients , and pregnant patients were excluded from the study . patients were randomized into two age and tpoab - matched groups ; 30 patients received 200 g of sodium selenite / day , orally , for 3 months , and 30 patients received placebo . all hypothyroid patients were given l - thyroxine replacement.results:of 30 patients in the selenium treated group , 6 patients were overtly hypothyroid , 15 were subclinical hypothyroid , 6 were euthyroid , and 3 were subclinical hyperthyroid . the mean tpoab concentration decreased significantly by 49.5% ( p < 0.013 ) in the selenium treated group versus 10.1% ( p < 0.95 ) in the placebo - treated group.conclusion:selenium substitution has a significant impact on inflammatory activity in thyroid - specific autoimmune disease . it would be of interest to determine whether early treatment with selenium in patients with newly developed autoimmune thyroiditis may delay or even prevent the natural course of these diseases .
vero e6 cells were infected with 100 focus - forming units of ebov expressing enhanced green fluorescent protein . after a 1-h incubation , the inoculum was removed and replaced with media ( dulbecco s modified eagle 's medium with 2% fetal bovine serum , penn / strep , l - glutamine ) containing chloroquine ( sigma , st . the supernatant was collected on days 1 , 3 , 5 , 7 , and 9 after infection and media replaced with fresh drug . viral rna was extracted from the supernatant and quantified by real - time quantitative reverse transcription pcr as previously described ( 10 ) . concurrently , cell viability was assayed by using cell titer96 aqueous one solution ( promega , madison , wi , usa ) according to the manufacturer s instructions . ec was determined by using prism6 ( graphpad software , san diego , ca , usa ) . when added 1 h after infection , chloroquine at 5 g / ml and 25 g / ml reduced the viral loads by 0.61 and 1.07 logs , respectively ( peak reduction observed on day 5 ) , without any significant cytotoxicity ( figure 1 ) . analysis of the data from day 5 resulted in an ec50 of 1.77 g / ml and an ec90 of 23.34 g / ml , concentrations that are comparable with previous data ( 8) ; however , reductions in viral loads at these concentrations at other time points were negligible . although concentrations of > 50 g / ml of chloroquine reduced viral loads by 24 logs starting on day 3 , this decrease was accompanied by a high level of cytotoxicity ( > 50% ) that was evident both in the cytotoxicity assay and microscopically resulting in poor selectivity of chloroquine . viral loads from supernatants derived from vero cells infected with ebola virus expressing enhanced green fluorescent protein and treated with chloroquine at the indicated concentrations ( 0 , 5 , 25 , 50 , 100 , and 200 g / ml ) . six - week - old balb / c mice or syrian hamsters ( both from harlan , indianapolis , in , usa ) were inoculated intraperitoneally with 100 50% lethal dose of ma ebov . the mouse ( 11 ) and the hamster ( 12 ) are well - established disease models of ebov infection . treatment groups ( mice and hamsters ) received 90 mg / kg of chloroquine alone ( intraperitoneally ) . an additional group of hamsters received 50 mg / kg of chloroquine ( intraperitoneally every 24 h ) in combination with 2.5 mg / kg doxycycline ( gavage every 12 h ) and 50 mg / kg azithromycin ( intraperitoneally every 24 h ) . after inoculation , animals were monitored at least twice daily and euthanized by using a humane endpoint scoring criteria as approved by the animal care and use committee at rocky mountain laboratories ( hamilton , mt , usa ) . two of 3 mock - challenged mice did not survive because of chloroquine ( 90 mg / kg ) treatment alone ( figure 2 , panel a ) . only 2 of 9 mice infected with ma ebov and treated with chloroquine survived , and 1 of 9 mice infected with ma ebov and treated with vehicle survived . with median survival of 7 , 8 , and 8 d for mock - challenged / chloroquine - treated mice , ma ebov infected / chloroquine - treated mice , and ma ebov infected / vehicle - treated mice , respectively , treatment had no significant effect on survival . this dose , although previously stated as the maximum tolerated dose in mice ( 8) , was not well tolerated by the animals in this study and clearly did not improve survival in animals challenged with ma ebov . survival of ma ebov - inoculated mice ( a ) and hamsters ( b ) treated with cq ( 90 mg / kg ) . c ) survival of ma ebov infected hamsters treated with a combination of cq ( 50 mg / kg ) , doxycycline ( 2.5 mg / kg ) , and azithromycin ( 50 mg / kg ) . combo , combination of chloroquine , doxycycline , and azithromycin ; cq , chloroquine ; ebov , ebola virus ; ma , mouse - adapted . when the same dose ( 90 mg / kg ) of chloroquine was given to hamsters challenged with ma ebov , the study had to be terminated on day 2 after treatment . nearly all the treated animals , in both the ma ebov and the mock - challenged groups , died of acute toxicity after administration of chloroquine intraperitoneally , typically within 30 min after treatment ( figure 2 , panel b ) . in a separate study , hamsters were treated with chloroquine ( 50 mg / kg ) in combination with doxycycline ( 2.5 mg / kg ) and azithromycin ( 50 mg / kg ) to additionally provide broad - spectrum antimicrobial drug coverage . reperfusion injury of the gut after ebov disease , which would subsequently result in bacterial sepsis , has been suspected as a possible cause of death . thus , broad - spectrum antimicrobial drugs were proposed to help in this regard . in this study , no toxicity was observed in the mock - challenged group as a result of the combination treatment . however , treatment had no effect on survival ; no combination - treated or vehicle - treated groups survived , and median survival times were comparable ( figure 2 , panel c ) . despite some activity of chloroquine against ebov in vitro , we observed no benefit to its administration in the mouse and hamster models . in the mouse model , a dose of 90 mg / kg resulted in toxicity but did not alter survival ; therefore , higher concentrations of chloroquine in the mouse would not be expected to be possible . in the hamsters , this dose was already lethal on its own . in the hamster model at a lower dose ( 50 mg / kg ) combined with doxycycline and azithromycin which together provide broad - spectrum antimicrobial coverage , in addition to doxycycline having a small antiviral effect against ebov previous anecdotal reports of the incidental use of chloroquine in patients with filovirus infections also do not support any benefit from its use ( 13,14 ) . together , these data suggest that chloroquine is unlikely to provide any protection from ebov infection in humans . given its in vitro activity against many different viruses and its longstanding use in humans , chloroquine has been put into multiple clinical trials . during dengue virus infection , viremia did not decrease ( 15 ) , and chloroquine neither prevented influenza virus infection ( 7 ) nor improved outcome of chikungunya virus infection ( 6 ) despite promising in vitro activity against these viruses . when taken together with previous findings for other less pathogenic viruses , the clinical use of chloroquine seems unlikely to provide any benefit for either prophylaxis or treatment of ebov . moreover , chloroquine has a small therapeutic window ; dosing for treatment of acute malaria is 15 mg / kg , and lethality starts at 50 mg / kg . thus , current preclinical data do not support the continued consideration of chloroquine for use against ebov infections in humans .
the antimalarial drug chloroquine has been suggested as a treatment for ebola virus infection . chloroquine inhibited virus replication in vitro , but only at cytotoxic concentrations . in mouse and hamster models , treatment did not improve survival . chloroquine is not a promising treatment for ebola . efforts should be directed toward other drug classes .
acute acalculous cholecystitis ( aac ) is defined as acute inflammation of the gallbladder in the absence of gallstones and has a multifactorial pathogenesis.1 aac occurs in about 10% of all cases of acute cholecystitis . aac has numerous causes that produce bile stasis and ischemia leading to inflammation and infection of the gallbladder . aac tends to have a more fulminant course , is frequently associated with gangrene , perforation and empyema , and has high morbidity and mortality.2 aac has traditionally been recognized to occur in patients with serious co - morbid illnesses especially after a major operation , severe trauma , burns , systemic sepsis and prolonged intravenous hyperalimentation.3 however , there has recently been an increasing number of reports in the literature of the occurrence of aac in patients with none of the established risk factors.4 - 6 aac arising as a complication of laparoscopic appendectomy has been reported in just 1 case and it was treated conservatively.7 herein , we present the case of a 38-year - old woman who developed aac after laparoscopic appendectomy . a 38-year - old woman who was previously in good health came to our emergency room 3 days after she received laparoscopic appendectomy at a local clinic . she had acute abdominal pain which initially occurred in the epigastric and umbilical area and then migrated to the right lower quadrant after 10 hours and was associated with nausea and anorexia . acute appendicitis had been diagnosed by abdominal computed tomography ( ct ) and operative findings at another hospital . her abdominal pain and associated symptoms improved after surgery . body temperature was 37.8. she exhibited tenderness and guarding in the epigastric region and right upper quadrant . results of the laboratory studies were unremarkable , except for elevated c - reactive protein ( crp ) which was 8.04 mg / dl ( normal range less than 0.5 mg / dl ) . her body mass index ( bmi ) was 21.7 ( height : 168 cm , weight : 61.2 kg ) . abdominal ct showed a thickened , contrast - enhanced wall of the gallbladder ( fig . her abdominal pain subsided and crp decreased to 1.33 mg / dl after the ptgbd . the patient 's postoperative progress was uneventful , and she was discharged 5 days after surgery . aac generally occurs in patients after major surgery , in the presence of critical illnesses such as trauma , burns , and sepsis , and in the elderly.1 however there have been recent reports of acalculous cholecystitis in young healthy patients who had none of the established risk factors.6,8 the patient described in this case report was young and healthy and exhibited none of the standard risk factors other than recent surgery , although laparoscopic appendectomy is not considered to be a major operation . the commonest postulated pathogenesis of aac is bile stasis resulting in a change of bile composition and ischemia.2 factors known to contribute to bile stasis in the postoperative patient are fasting , anesthesia , dehydration , fever , and narcotics for the relief of pain.9 i believe that the most probable mechanism of aac in this woman was bile stasis that resulted from the anesthesia given to her during the appendectomy and the narcotics given for the relief of pain . aac is difficult to diagnose because clinical signs such as abdominal pain and fever , and laboratory test results are non - specific . delayed diagnosis of aac is associated with high morbidity and mortality due to the high prevalence of gangrene and perforation.10 early diagnosis and appropriate treatment can improve outcomes in patients with aac.11 the treatment options for aac are cholecystostomy and/or cholecystectomy.9,12 cholecystectomy generally is considered the definitive therapy , and percutaneous cholecystostomy can be performed safely and rapidly.9 standard treatment methods of aac have not yet been established , but it is usually determined by the patient 's condition . i believe that early cholecystectomy in this case was an effective treatment tool for avoiding potential failure of conservative management and for preventing recurrence , although the patient was young and had none of the risk factors . as mentioned above , laparoscopic appendectomy may mask clinical signs and symptoms of aac . therefore , when right upper quadrant pain , fever , leukocytosis , and abnormal liver function tests are observed after surgery , physicians should consider the possibility of aac , and promptly check radiological findings . aac arising as a complication of laparoscopic appendectomy has been reported rarely , especially in young healthy patients . when abdominal pain , leukocytosis , fever and abnormal results of liver function tests are observed in patients after appendectomy , the possibility of aac should be considered . prompt recognition and appropriate treatment of aac is necessary to minimize the associated morbidity and mortality .
acute acalculous cholecystitis ( aac ) is defined as acute inflammation of the gallbladder in the absence of gallstones . aac occurs in patients after major surgery and in the presence of serious co - morbidities such as severe trauma , burns , sepsis , prolonged intravenous hyperalimentation and hemodynamic instability . aac is rare in patients with none of the established risk factors . we present a case of a 38-year - old woman who developed aac after laparoscopic appendectomy .
a 35-year - old woman was admitted complaining of a headache over the last two months . she had undergone a right temporal lobectomy for generalized tonic - clonic seizures 10 years earlier . laboratory tests were normal except for an increased erythrocyte sedimentation rate of 45 mm / hr . a pre - contrast ct showed a linear calcification surrounded by a lobular - shaped mass of increasing attenuation in the right frontal lobe ( fig . the mass was accompanied by extensive edema surrounding it , with a midline shifting to the left side . on mr imaging , the mass showed a heterogeneous dark signal intensity on t2-weighted images ( fig . moreover the mass had an irregular margin and was classified as an intra - axial mass with cortical involvement . three regions of interest ( roi ) were determined based on the gradient - echo echo planar imaging source images and the matching t2 and gadolinium - enhanced t1-weighted images . the roi with the highest regional cerebral blood volume ( rcbv ) was selected and was found to be almost the same as that of the contralateral side ( rcbv ratio = 1.04 ) ( fig . localized proton mr spectroscopy was performed using a multivoxel point - resolved spectroscopy sequence with an echo time of 144 msec . the spectroscopic data showed a slightly elevated choline ( cho ) and slightly decreased creatine ( cr ) and n - acetyl aspartate ( naa ) peaks with a small increase in the choline / creatine ( cho / cr ) ratio in the enhancing lesion ( fig . this diagnosis was based on the strong enhancement of the mass accompanied with severe peritumoral edema and a mass effect . however , the perfusion data and mr spectroscopy findings suggested that the mass was benign . the mass was surgically removed and the pathology revealed a foreign body - associated granulomatous inflammation with marked lymphocytic and histiocytic infiltration , microcalcification , and fibrous scar tissue formation ( fig . 1j , k ) . following surgery , the chemical agents used in neurosurgery to achieve intra - operative hemostasis can cause foreign body reactions . historically , several terms such as textiloma , gossypiboma , gauzoma , and muslinoma have been employed , reflecting the hemostatic materials involved . in our case the signal intensities of the foreign body granulomas are variable on the t1- and t2-weighted images . . variable signal intensity on t2-weighted images may reflect a complex pathology including acute or chronic inflammation , granuloma formation , fibrosis , collagen deposition , and degeneration of foreign materials . in our patient , the low signal intensity proportion of the t2-weighted images may be explained by the fibrosis seen in the histologic specimen . in the case of ring - enhancing mass lesions , these represent degenerated foreign material and may be helpful in distinguishing a muslinoma from brain abscess ( 7 ) . this was true of our patient based on the mr imaging findings showing intense mass enhancement with severe surrounding edema . however , on perfusion mri , the rcbv did not increase and , on multi - voxel mr spectroscopy , the mass cho / cr ratio increased only slightly , which was not consistent with a high - grade tumor . previous reports on perfusion and mr spectroscopic findings in patients with non - neoplastic tumor - mimicking masses showed mildly decreased - to - increased perfusion , and small rises in cho / cr ratios , with decreased naa . perfusion mri and mr spectroscopic findings from our patient were very similar to those of patients from previous reports with non - neoplastic mass lesions ( 8 , 9 ) . retrospectively , we think we could have excluded the possibility of malignancy in our patient based on the perfusion mri and mr spectroscopy , although benign tumorous conditions may not be totally excluded by such exploratory modalities . although the perfusion mri and mr spectroscopic findings are not specific for foreign body granulomas , these techniques may be helpful in excluding a high - grade tumor diagnosis , which may be the most important differential diagnosis . by combining clinical history of any previous operation , conventional mr imaging findings , and perfusion mri and mr spectroscopy , it may be possible to correctly diagnose a foreign body granuloma . in summary , conventional mri findings in foreign body granuloma patients may sometimes mimic those of brain tumors . to differentiate the diagnoses , the performing a perfusion mri and mr
we report a case of intracranial foreign body granuloma that showed features of a high grade tumor on magnetic resonance ( mr ) imaging . however , the relative cerebral blood volume was not increased in the enhancing mass on perfusion mri and the choline / creatine ratio only slightly increased on mr spectroscopy . the results suggest that the lesion is benign in nature . perfusion mri and mr spectroscopy may be helpful to differentiate a foreign body granuloma from a neoplastic condition .
organizing pneumonia ( op ) is characterized by an indolent clinical course and a favorable prognosis although a fulminant variant has been documented . pulmonary air leak comprising pneumothorax , pneumomediastinum , and subcutaneous emphysema is rarely a presenting feature of an op . the exact pathogenesis and an appropriate management guideline have not yet been established for this dreaded complication . review of medical literature reveals only a few case reports of air leak in op . here , we report a case of op secondary to bleomycin chemotherapy , where pulmonary air leak was the major presenting symptom , which has never been reported previously . a 43-year - old female presented to our emergency department with severe shortness of breath , diffuse neck swelling , and neck pain , of 1 day duration . she had a stage iii ovarian immature teratoma diagnosed 6 months back and was treated with bilateral salpingo oophorectomy followed by 6 cycles of chemotherapy with bleomycin , etoposide , and cisplatin . one week prior to the admission , she had fever and severe cough which was treated from a local hospital . on examination , she had respiratory distress with a respiratory rate of 32/min . her spo2 was 82% in room air , blood pressure 110/70 mmhg , and heart rate 110/min . examination of the respiratory system showed equal breath sounds on both sides with bilateral fine basal crepitations and muffled heart sounds . the values for the blood gas analysis were a ph of 7.42 , pco2 of 34 mmhg , and po2 of 65 mmhg . chest x - ray showed [ figure 1 ] features suggestive of subcutaneous emphysema and mediastinal emphysema with bilateral haziness and alveolar opacities in lower zones . a computed tomography ( ct ) thorax [ figure 2 ] with findings of ground - glass opacities and peripheral consolidation , confirmed the diagnosis of pneumonia and mediastinal emphysema . mediastinal emphysema and diffuse haziness in chest x - ray ( a - c ) serial computed tomography images showing bilateral diffuse ground - glass opacities , mediastinal emphysema , and peripheral consolidation with this clinical presentation of a pulmonary air leak syndrome and radiologic picture of bilateral predominant ground glass opacities with patchy consolidations , we considered the possibility of pneumocystis jiroveci pneumonia or a drug - induced lung disease like diffuse alveolar damage / op . since the patient had severe respiratory distress and hypoxemia , she was immediately taken up for surgical management . a video - assisted thoracoscopy was done [ figure 3 ] , and the mediastinum dissected revealing multiple paracardiac blebs . these blebs were ablated ; the pneumomediastinum drained into left hemithorax , and an intercostal drainage tube was inserted in left hemithorax . video - assisted thoracoscopy showing paracardiac blebs patient 's condition improved dramatically with rising oxygen saturation . she was shifted to the intensive care unit and was treated with antibiotics , steroids , and supportive treatment . biopsy of the lung specimen [ figure 4 ] revealed bronchiolitis obliterans organizing pneumonia ( boop ) . soon the air - leak disappeared , and she was discharged from the hospital with a long - term course of steroids and macrolide . ( a ) histology showed plugs of fibrous tissue in terminal bronchioles , ( b ) trichrome stain showing fibrous proliferation op is a clinicopathologic syndrome first described by davison and epler et al . in the 1980s . causes of secondary op include infections , drugs , connective tissue diseases , organ transplantation , and inflammatory bowel diseases . the manifestations of op are nonspecific ; hence , the diagnosis is delayed in many . high index of suspicion should be kept to diagnose secondary op , especially in patients with chemotherapy , organ transplantation , etc . a unique manifestation of bleomycin toxicity is multiple pulmonary nodules in ct mimicking metastasis and having histologic characteristic of op / boop . a study conducted by mokhtari et al . found that of the 43 patients with isolated boop in cancer patients , 3 were found to be due to bleomycin . kofteridis et al . , iwanaga et al . , and yang et al . have published case reports of air leak syndrome in boop . the hallmark of op is the presence of intraalveolar buds of granulation tissue consisting of fibroblasts - myofibroblasts embedded in connective tissue . these buds may extend from one alveolus to the next through the interalveolar pores and into the bronchioles obstructing the lumen ( bronchiolitis obliterans ) . the pathogenesis proposed for air leak is that , localized plugs of fibrous tissue in the bronchiole lumen in op , produce a ball valve mechanism and alveolar over - distension , eventually leading to rupture . the air dissects through the bronchovascular sheath and ascends toward the hilum to produce a pneumomediastinum and also along the subcutaneous connective tissue to cause subcutaneous emphysema . air leakage in the mediastinum may also occur due to the rupture of paracardiac bleb . in severe interstitial lung disease ( ild ) , we also considered the possibility of pneumocystis jiroveci pneumonia and metastasis as a differential in this immunocompromised patient with fever , cough , radiologically bilateral ground glassing , and air leak , but investigation profile proved against this . in our patient , the treatment of op is long - term steroid , and the treatment for massive pneumomediastinum is limited mediastinotomy and drainage . op should be included in our differential diagnosis list , whenever a patient presents with air leak . lung biopsy should be done to confirm the diagnosis and would prove to be lifesaving as this disease responds well to steroids . limited mediastinotomy and drainage of pneumomediastinum in massive air leak is lifesaving and gives rapid improvement .
organizing pneumonia ( op ) is a less common interstitial lung disease with varying clinical picture . the development of pulmonary air leak in a case of op is an extremely rare complication . here , we report the case of a 46-year - old female with carcinoma ovary , postchemotherapy who developed respiratory distress with pneumomediastinum , and subcutaneous emphysema . lung biopsy showed evidence of op . this turned out to be a rare case of op , secondary to bleomycin chemotherapy , presenting with pulmonary air leak .
a 66-year - old female was presented to the emergency room with sudden weakness on her left side and dysarthria while taking aspirin . diffusion weighted magnetic resonance imaging ( mri ) showed several small acute infarctions scattered in her right frontal cortex and basal ganglia . mr angiography revealed a severe stenosis of the right middle cerebral artery ( mca ) . digital subtraction angiography ( dsa ) confirmed the severe long segment stenosis of the mca and a significant decrease of flow velocity to the distal segment ( fig . 1a ) . we suspected that her current medical treatment would be ineffective to prevent another stroke , and thus , we planned an endovascular treatment . an endovascular procedure was performed 1 week later when her left - sided weakness was almost recovered . she received a daily oral administration of 325 mg of aspirin and 75 mg of clopidogrel before the procedure . endovascular procedure was performed under local anesthesia and with systemic heparinization . with a 6 fr guiding catheter located in the right internal carotid artery ( ica ) , after the microcatheter was advanced across the lesion , the microwire was exchanged with a 300 cm exchange wire that could facilitate balloon and stent navigation . the microcatheter was removed , and a 1.520 mm gateway balloon catheter was advanced over the exchange wire . the balloon was located within the stenotic segment and inflated to approximately 6 atm . on control angiogram after balloon angioplasty next , the wingspan delivery system was prepared and advanced over the exchange wire across the target lesion . during wingspan ( 3.520 mm ) passed cavernous ica , patient complained headache and nausea , and systolic blood pressure was elevated from normal range to approximately 160 mmhg . immediately , a stent was deployed at the stenotic segment and a control angiogram was achieved . although the stenotic segment was recanalized and the distal flow restored to normal velocity ( fig . 1b ) , a tiny irregularity of posterior parietal branch of right mca and extravasation of contrast media suggested that dissections were revealed ( fig . rotational flat panel ct was performed immediately and showed a small amount of extravasation of contrast media within the subarachnoid space along the right lateral sulcus ( fig . we dropped the blood pressure under normal range and reversed heparin with protamine sulfate . on the angiogram performed 10 minutes later , a small filling defect appeared within the stenosis , which was suspected as ist . because of a subarachnoid hemorrhage caused by an iatrogenic vessel injury , iib / iiia receptor antagonist or fibrinolytic agent should not be administrated for thrombolysis . to manage the ist rebar 18 " microcatheter ( ev3 , irvine , ca , u.s.a . ) was passed the wingspan stent , and a solitaire stent ( 420 mm , ev3 , irvine , ca , u.s.a . ) was deployed to cover the wingspan stent ( fig . ist was completely resolved on dsa obtained at 30 and 60 minutes after procedure ( fig . brain ct at immediate and 24 hours after procedure did not reveal any new low attenuation or progression of subarachnoid hemorrhage . in - stent restenosis occurs in approximately 30% of patients after delivery of the wingspan stent , although many of these incidents of re - stenosis remain asymptomatic . although the actual rate of periprocedural ist of intracranial stent is not obvious , the most recent report demonstrated that the rate of acute or subacute ist after intracranial stent ranged from 10% to 14.6% [ 4 , 6 ] . contrary to chronic in - stent stenosis , acute ist can result in permanent disability of the patient . . aspirin and clopidogrel resistance may be the actual causes of ist during the periprocedural period , but it is likely there are other factors that have not been elucidated yet . it should be noted that the incidence of ist in the wingspan and gateway system is more frequent than that in the stent for aneurysm neck remodeling . the reason can be explained that the underlying disease itself is different from cerebral aneurysms and may be more prone to acute thrombus formation . a balloon angioplasty before the wingspan placement may result in an endothelial injury or the exposure of atheromatous plaque material , promoting an in - stent thrombus formation . the present case showed that the secondary self - expandable stent had resolved acute ist without a new thrombus formation within the stent in spite of the non - administration of fibrinolytic agents . the second stent would open circumferentially to displace the thrombus , immediately reestablishing a channel of flow within the occluded vessel . once antegrade flow was restored , thrombus dissolution via endogenous thrombolysis would follow stent deployment . therefore , we suspected that the etiology of acute ist in our case might be not associated with aspirin or clopidogel resistance but with endothelial injury . stent in - stent would cover the exposure plaque material from the coagulation system , protecting re - growth of the thrombus within stent . unfortunately , we were unable to confirm drug resistance using blood assay , such as verifynow p2y12 test . the management described for the ist includes a systemic or local application of iib / iiia receptor antagonist or a recombinant tissue - type plasminogen activator with / without balloon angioplasty [ 4 , 6 , 8 ] . although these thrombolytic agents are effective solutions for acute thrombolysis , they can not be used and the mechanical technique should be considered for the recanalization of thrombotic occlusion of artery when there is a recent hemorrhage such as the present case . we have found several reports regarding the use of stent - in stent technique for recanalization of the late in - stent restenosis following the wingspan placement [ 5 , 8 , 9 ] . however , to the best of our knowledge , this present case is the first case that has recanalized acute ist using a stent in - stent technique and without using the thrombolytics . iatrogenic vascular perforation is a complication of major concern that can result in fatal intracranial hemorrhages during endovascular neurointervention . when vascular perforation is detected during a procedure , the treatment should immediately begin with a reversal of anticoagulant , lowering the blood pressure and/or temporary or permanent closure of the ruptured vessel . as in this case , when an ist following a wingspan stent placement for intracranial stenosis occurs with a coincidentally hemorrhagic complication , the deployment of another stent in - stent should be considered as a treatment option .
we presented a case that an acute in - stent thrombosis after the deployment of a wingspan stent was successfully managed with a stent in - stent technique . because vessel perforation and subarachnoid hemorrhage were iatrogenically developed during the procedure , we were unable to use the thrombolytic agents to correct the in - stent thrombosis . when a thrombotic complication following an intracranial stent placement occurs with a coincidentally hemorrhagic complication , the stent in - stent technique should be considered as a treatment option .
currently , nanoparticles ( nps ) are used in several scientific applications , but among these silver nanoparticles ( ag - nps ) are predominant . this is because of several characteristic features of this type of metal np.13 antimicrobials and nanocomposite fabrication are some of the highly useful applications , among others , of applications that were attributed to ag - np.4,5 in the fabrication of nps , it is very important to control particle size , shape , and morphology . laser ablation in liquid media is a simple and clean method for synthesis of nps.69 in this technique , there is no need to use any chemical reagents ( such as nabh4 ) in the fabrication process or any purification techniques to characterize the produced nps . for example , nps using fatty acids ( oleic and lauric acid ) and vegetable oils as stabilizers have been synthesized.1012 it was found that these materials consist of amphiphilic molecules with polar carboxylic groups which are able to adsorb nps , and their nonpolar long carbon chain prevents nps agglomeration through steric repulsion . castor oil is a vegetable oil obtained by cold pressing the seeds and subsequent clarification of the oil by heat . it has high viscosity , high polarity , and very low vapor pressure and optical activity in comparison with other oils . castor oil is classified as a safe and effective stimulant laxative by the us food and drug administration ( fda ) . it is a triglyceride containing fatty acid chains , 90% of which are ricinoleic acid . oleic and linoleic acids are the other significant components.12 with the above characteristics , it is expected that castor oil can be used as a good stabilizer in nps fabrication . in this study , we report fabrication of ag - nps in castor oil . to the best of our knowledge , fabrication of ag - nps in castor oil using laser ablation has not been reported previously . for the fabrication of ag - nps , a silver plate was ablated by laser in castor oil for 10 min . as shown schematically in figure 1 , the plate with high purity ( > 99.99% ) was located 5 mm behind a glass cell wall filled with 10 ml of castor oil . a nanosecond - pulsed q - switched nd : yag laser ( brilliant ; lambda photometrics ltd , hertfordshire , uk ) with 10-hz repetition rate , 5-ns pulse duration , and 360 mj / pulse energy at its original wavelength ( 1064 nm ) was applied . a lens with focal length of 250 mm was used to focus the laser pulses onto the silver plate . during plate ablation , an atomic absorption spectrometer ( aas - s series ; thermo scientific , san jose , ca ) was used to measure concentration of ag - nps in castor oil . the obtained concentration of ag - nps in castor oil was 0.13 mg / l . due to the high viscosity of castor oil the prepared sample was characterized using a uv - vis double beam photospectrometer ( shimadzu , columbia , sc ) , a transmission electron microscope ( tem , hitachi h-7100 ; hitachi , tokyo , japan ) , and a fourier transform - infrared ( ft - ir ) spectrometer ( 1650 ; perkin elmer , waltham , ma ) . figure 2a indicates the absorption spectrum of the castor oil immediately after laser ablation of the silver plate inside it . the appearance of the maximum absorption peak around 400 nm confirms that the solution contained ag - nps , and that this peak originated from the oscillation of collection of free conduction electrons of ag - nps , which is called surface plasmon resonance . the ultraviolet - visible spectrum of the ag - nps in castor oil was measured after a period of 2 months in order to check the ability of castor oil as a stabilizer . as shown in figure 2b , the spectrum does not show any significant change in comparison with the spectrum of the freshly prepared sample ; however , there is a small reduction in absorption . this showed that the ag - nps produced in castor oil using the laser ablation technique were stable and did not agglomerate during storage of the sample at room temperature for quite a long period of time . the slight decrease in absorption intensity was possibly due to the sedimentation of larger particles . the spherical shape nps can be very appropriately used for drug loading and for most biological applications such as antibacterial properties.13 figure 3 shows an approximate 5.06 nm average diameter for freshly prepared ag - nps obtained from two areas . nps are formed via nucleation , transition , and crystal growth of materials such as silver atoms , clusters , and droplets that were emitted from the silver plate upon laser ablation.14 castor oil can adsorb the produced ag - nps and long - chain hydrophobic moieties protect them through a steric stabilization mechanism . ft - ir spectroscopy of castor oil and castor oil containing ag - nps ( figure 5 ) shows absorption bands at 3448 , 2923 , 2857 , 1739 , and 1160 cm related to the vibrational - stretching mode of o h ( intermolecular hydrogen bond ) , c h ( sp ) , c h ( sp ) , co , and c o , respectively . in addition , the appearance of the absorption band at 3780 cm can be attributed to nonhydrogen - bonded o we believe that the existence of ag - nps prevents intermolecular hydrogen bonding , and therefore , the vibrational stretching modes of free o h appear at a higher wave number.15 ag - nps have been successfully prepared in castor oil by laser ablation from a silver plate . the results obtained showed that the produced ag - nps were well dispersed and also stable for quite a long period of time . this ability of castor oil to prevent agglomeration of ag - nps is due to the presence of long - chain hydrophobic moieties .
silver nanoparticles were fabricated by ablation of a pure silver plate immersed in castor oil . a nd : yag - pulsed q - switch laser with 1064-nm wavelength and 10-hz frequency was used to ablate the plate for 10 minutes . the sample was characterized by ultraviolet - visible , atomic absorption , fourier transform - infrared spectroscopies , and transmission electron microscopy . the results of the fabricated sample showed that the nanoparticles in castor oil were about 5-nm in diameter , well dispersed , and showed stability for a long period of time .
castleman 's disease ( cd ) is a rare lymphoproliferative disorder caused by faulty immune regulation resulting in excessive b - lymphocyte and plasma - cell proliferation in lymphatic tissue . marked interfollicular vascular proliferation leads to mass formation , which can encase bronchi and sometimes cause mural erosion leading to life - threatening hemoptysis . vascular supply to these lesions have been reported to arise from the bronchial , internal mammary , and intercostal arteries . we report a case of hilar intrathoracic cd with atypical radiological features where the left inferior phrenic artery and bronchial circulation provided codominant blood supply . a 24-year - old female with a 5-month history of wheeze and nonproductive cough underwent chest x - ray , which showed a well - defined mass in the left hilar region [ figure 1a d ] . a contrast - enhanced computed tomography ( ct ) scan of the chest revealed a heterogeneously enhancing 7 5 cm mass containing flecks of calcification at the left hilum . 18 fluorodeoxyglucose positron emission tomography ( 18-fdg pet ) scan demonstrated an inhomogeneous hypermetabolic lesion with no evidence of metastatic uptake . a ct - guided percutaneous core biopsy revealed chronic inflammatory tissue with no evidence of malignancy . due to worsening cough and wheeze , the patient underwent a thoracotomy and surgical resection . during the operation there was massive blood loss of 2000 ml . histopathology confirmed the mass to have marked lymphoid infiltrate with irregular b cell follicles and hyaline vascular - like change consistent with cd . chest x - ray of a 24-year - old female ( a ) showing well - defined left hilar mass . contrast - enhanced ct scan ( b and c ) shows heterogeneously enhancing mass with coarse calcifications within this hilar mass . 18-fdg pet / ct scan ( d ) shows hypermetabolic uptake within the lesion two months after the thoracotomy , the patient presented to the emergency department with a 1-day history of massive hemoptysis ( > 200 ml ) . a ct pulmonary angiogram for clinically suspected pulmonary embolism showed no pulmonary embolism , however , multiplanar reformats showed low attenuation soft tissue in the surgical resection bed at the left hilum receiving blood supply through hypertrophied left bronchial and left inferior phrenic arteries [ figure 2a f ] . the patient had an embolization through a right common femoral artery approach and the common ( conjoined ) origin of the right broncho - superior intercostal trunk and left bronchial arteries were catheterized with a 5 fr ( cordis , usa ) left amplatz coronary i catheter ( cordis , usa ) . the sharply angulated left bronchial artery could not be superselectively catheterized with a range of microcatheters . the main trunk was embolized with 300500 micron polyvinyl alcohol ( pva ) particles ( cook , usa ) . the medial division of the inferior phrenic artery was seen to supply the mass via its pericardial branch . this was super - selectively catheterized with the same 5 fr catheter and a renegade microcatheter ( boston scientific , usa ) and embolized with the same pva particles . no spinal , esophageal , or coronary supply was demonstrated from the bronchial and left inferior phrenic arteries . there were no procedure - related complications and the patient had an uneventful recovery with no further hemoptysis during the 3 years follow - up period . contrast - enhanced ct with mip images ( a and c ) showing blood supply to the lesion with bronchial artery and left inferior phrenic artery ( arrows ) . catheter angiogram demonstrates bronchial artery ( arrow in b ) and left inferior phrenic artery ( arrow in d ) and subsequent embolization with pva ( e and f ) cd may be localized or multicentric and primarily involves the mediastinum , neck , and mesentery . there are three known histological variates namely ( a ) hyaline - vascular , ( b ) plasma - cell , and ( c ) mixed variant . classic features on a ct scan is a solitary , intensely , and homogeneously enhancing mass with no local invasion . the standard treatment of the localized form is surgical resection , however , where there is encasement or invasion of the adjacent structures , preoperative embolization has been advocated . recently , embolization alone without surgical resection has been shown to terminate hemoptysis and alleviate shortness of breath due to airway compression in patients with unresectable disease . in our patient , preoperative embolization , which may have reduced the large volume intraoperative blood loss , was not considered . it was only when the patient presented with hemoptysis after surgical resection that the vascular supply was identified and an emergency referral to interventional radiology was made . previous reports have identified bronchial , internal mammary , and intercostal arteries as feeding vessels to cd . inferior phrenic supply to cd has not been reported before to the best of our knowledge . when basal lung segments are suspected as the source of hemoptysis , inferior phrenic supply should always be considered . inferior phrenic supply to hilar masses is much rarer , but when it occurs , the supply is usually via the pericardial branch of its medial division . the left inferior phrenic medial branch may supply the esophagus , and it is important to exclude any esophageal supply before particulate embolization . careful review of multiplanar reformats of the ct scan aided procedure planning and the consent process by identifying the hypertrophied arterial feeders . cd should be considered in the differential diagnosis for all benign intrathoracic masses . where a mass shows avid enhancement , whether homogeneous or heterogeneous , a ct angiogram ,
castleman 's disease ( cd ) is a rare lympho - proliferative disorder due to faulty immune regulation resulting in proliferation of lymphatic tissue . the vascular supply to these lesions have been reported to arise from the bronchial , internal mammary and the intercostal arteries . we report a case of hemoptysis secondary to intrathoracic cd with vascular supply arising from the left inferior phrenic artery which was successfully embolised with polyvinyl alcohol ( pva ) particles .
we report a 32-year - old iranian male diagnosed with a case of miliary tuberculosis affecting the prostate . computerized tomography increased the clinical suspicion of miliary tuberculosis extending to the prostate where a trans - rectal urethral biopsy was obtained . a strong clinical suspicion and availability of sophisticated tests with confirmation by biopsy , polymerase chain reaction , and culture are needed in order to avoid misdiagnosis of complicated miliary tuberculosis cases . miliary tuberculosis is a rare form of a tuberculosis infection that results from massive lymphohematogenous dissemination of the mycobacterium tuberculosis bacilli.1 this form of infection , which accounts for 2.8% of all tuberculosis infections , usually involves the extrapulmonary organs.2 from extrapulmonary tuberculosis only 22% affects the genitourinary system , while tuberculosis of the prostate gland is seen in only 2.7% of genitourinary tuberculosis.3,4 due to the rare involvement of the prostate , very scarce literature of the subject , and non - specific symptoms , the clinical suspicion of prostate tuberculosis is difficult . here we report a case of a young , previously healthy , immunocompetent patient with miliary tuberculosis involving the prostate . we present a case of a 32-year - old immunocompetent iranian male who presented to the emergency room with a generalized tonic - clonic seizure lasting for 3 minutes . furthermore , he did not have any genitourinary symptoms , including dysuria , hematuria , or penile discharge . a computerized tomography ( ct ) scan of the head failed to show any acute central nervous system insult . prior to this admission , he gave history of an admission 12 months ago to a hospital outside the country , where he complained of fatigue , weight loss , and fever . reports from the hospital showed that he also had chronic moderate ascites and a left pleural effusion . however , several laboratory and imaging tests were done and it was concluded that there was no evidence of infectious disease , chronic liver disease , or malignancy . it was recommended that he may need more investigations or a laparoscopy with peritoneal tissue biopsy , which was not done . upon the latest admission , the respiratory rate was 12 breaths per minute , the body temperature was 36.9c , and the blood pressure was 112/70 mmhg . digital rectal examination was not performed since the patient did not have any genitourinary or gastrointestinal symptoms . during this hospitalization , the laboratory investigations , urine routine and microscopy , cultures , virology screen , including human immunodeficiency virus , as well as the autoimmune markers did not show any significant issues . also , three sets of sputum acid - fast bacilli smears were done , which were all negative . a lumber puncture was also performed with a normal initial pressure , 0 mmcell count , glucose of 4.1 mm per liters , and protein of 764 mg per liter . at the same time a magnetic resonance image of the brain was done concluding multifocal enhancing foci suggestive of an infective process ( figure 1 ) . due to his previous history , ct - chest , the abdominal ct showed mild peritoneal ascites with evidence of mild splenomegaly . the ascetic fluid taken was translucent and yellow in color and the serum - ascites albumin gradient 5 gram per liter ( 0.5 gram per deciliter ) furthermore , the analysis was negative for ziehl neelsen , gram stain , and culture . the pelvis ct also showed an enlargement of the prostate and a right prostate lesion extending to the seminal vesicles ( faint ring enhancing lesion measuring 32.73 cm ) ( figure 2 ) . the case was discussed with the patient and he agreed to start anti - tuberculosis treatment and to have a trans - rectal biopsy of the prostate . the biopsy showed multiple slides of necrotizing granulomata suggestive of tuberculosis ( figure 3 ) . neelsen stain was also performed , and the diagnosis of miliary tuberculosis was confirmed ( figure 4 ) . he was started on anti - tuberculosis therapy with isoniazid , rifampicin , ethambutol , pyrazinamide , and pyridoxine for 12 months . at follow - up the patient was asymptomatic with a normal physical examination and laboratory tests with no drug side effects . this complication is seen in 1%3% of all tuberculosis cases.5 although most cases of miliary tuberculosis are treatable , the mortality rate among young adults with miliary tuberculosis remains 15% 20% and for adults 25%30%.2 one of the main causes for these high mortality rates includes late detection of disease caused by non - specific symptoms . tuberculosis of the prostate is rare and the literature is scarce . most often , the cases are under - reported , and easily missed . patients with prostatic tuberculosis usually present with non - specific symptoms except in rare cases . these rare cases present with irritative voiding symptoms , terminal dysuria or hemospermia.6 however this was not the case in our patient . the patient was asymptomatic and like most cases in the literature , tuberculosis of the prostate was diagnosed incidentally . as in our case , tuberculosis of the prostate is mainly diagnosed by pathologists where a trans - rectal ultrasound guided biopsy of the prostate is the mainstay of the diagnosis.7 as wang and chang have demonstrated , it was the clinical usefulness of the contrast enhanced ct for the diagnosis of tuberculosis of the prostate in which low density multiple and bilateral lesions with irregular borders are seen that prompted us to further investigate the prostate.8 once the disease is diagnosed , the patient should receive a full course of anti - tubercular therapy . often , the presentation can be non - specific and misleading , as in our case . a strong clinical suspicion and availability of sophisticated tests with confirmation by biopsy , polymerase chain reaction , and culture are needed in order to avoid misdiagnosis of complicated miliary tuberculosis cases .
introductiontuberculosis of the prostate is a rare complication of miliary tuberculosis.case presentationwe report a 32-year - old iranian male diagnosed with a case of miliary tuberculosis affecting the prostate . the patient was admitted to the hospital with convulsions . computerized tomography increased the clinical suspicion of miliary tuberculosis extending to the prostate where a trans - rectal urethral biopsy was obtained . the biopsy revealed multiple necrotizing granulomata suggestive of tuberculosis.conclusiona strong clinical suspicion and availability of sophisticated tests with confirmation by biopsy , polymerase chain reaction , and culture are needed in order to avoid misdiagnosis of complicated miliary tuberculosis cases .
persistent left superior vena cava ( plsvc ) is a congenital but benign vascular anomaly with a prevalence of 0.3 - 0.5% in the general population . plsvc is one of the most common cardiovascular anomalies but is generally asymptomatic , and it is thus often identified accidentally during cardiovascular ultrasound / ct scan screening or coronary interventional angiography . anatomically , plsvc causes the aberrant backflow vein to drain into the retrograde positioned coronary sinus . some researchers have called attention to the risk of atresia of the coronary sinus among the anomalies associated with plsvc , but there are no recommendations regarding anticoagulation therapy for patients with plsvc . nevertheless , coronary sinus atresia ( csa ) due to plsvc is a rare complication . in patients with hematological malignancies , the insertion of a central venous catheter ( cvc ) is required for the patient to undergo intensive chemotherapy . hematologists must make a decision whether to conduct a cvc insertion for patients with a given complication . there are a few reports of csa in plsvc induced by central venous catheterization , pacemaker wire placement , or cardiac surgery . the placement of a cvc might evoke arrhythmia due to an additive enhancement of the patient 's arrhythmogenic condition . considering such a situation , it may not be advisable to insert a cvc in a plsvc patient with a hematological malignancy . we treated a 42-year - old male with stage iv hodgkin 's lymphoma with bone marrow involvement . the initial chemotherapy ( abvd , doxorubicin 25 mg / m , bleomycin 9 mg / m , vinblastine 6 mg / m , and dacarbazine 250 mg / m , day 1 and 15 ) was effective , resulting in complete remission after 6 cycles of abvd . although the abvd chemotherapy had been administered via a peripheral vein , salvage chemotherapy was initiated via an implantable venous access port catheter because his peripheral blood accesses were ultimately unusable . he received an indwelling port catheter from the left subclavian vein , since the right subclavian approach was not possible . 1a ) , which was found at the time for the first time in his life . the patient successfully completed a total of 3 courses of salvage chemotherapy constructed with arac , carboplatin , etoposide , and methylpredonisolone ( aces ) . although obstruction of the coronary venous drainage caused by plsvc has been suggested , it is not always thrombogenic . a small plsvc flow can easily connect to hemostasis of the venous circulation return . in our patient , the plsvc cavity had enough space for a port catheter to be cannulated and positioned ( fig . . ideally the back flow and diameter of the plsvc to the coronary sinus should be evaluated by cardiovascular ultrasound or cardiography . although plsvc is a rare congenital anomaly , some risks including thrombosis and occlusion should be annotated when the patient receives a cvc insertion . the relationship between csa and plsvc remains unclear ; however , the hematologist should pay attention to the malpositioning of the cvc tip . informed consent to participate in the study was obtained from the participant . written informed consent was obtained from the patient for publication of this case series and any accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal .
persistent left superior vena cava is a congenital vascular anomaly , which is possibly arrhythmogenic and thrombogenic , rarely complicated with coronary sinus atresia . we treated a 42-year - old male with hodgkin 's lymphoma requiring central venous catheter placement for intensive chemotherapy . persistent left superior vena cava was revealed after the insertion of the central venous catheter by the radiological finding of the catheter tip cannulated into the vena cava cavity . the relationship between coronary sinus atresia and persistent left superior vena cava induced by central venous catheterization remains unclear ; however , the hematologist should pay attention to the malpositioning of the central venous catheter .
fried seeds subjected to grinding by adding sufficient water to it for 30 - 45 min . the mixture was filtered through muslin cloth and pea nut milk is separated . solvent { ethanol : acetone ( 1:1 ) } was added to the milk and then subjected to refrigeration for 30 min . indomethacin was dissolved in chloroform and bio - polymer solution was prepared by adding water to it . the drug solution was added to bio - material solution and mixed by magnetic stirrer until emulsion is formed . release of drug through egg shell membrane : egg shell membrane was separated using con . the egg shell membrane attached to the tube . measured quantity of micro particles 5 ml solution was withdrawal and 5 ml buffer solution was added to make the volume . same procedure was repeated for different formulated films of aceclofenac with phosphate buffer ( ph-7.4 ) . the dissolution studies were perform and subjecting it for dissolution for 3 hrs and analyze the samples by uv spectrophotometer at 320 nm . % cummulative release vs time in ph 1.2 % cummulative release vs time in ph 7.4 fried seeds subjected to grinding by adding sufficient water to it for 30 - 45 min . the mixture was filtered through muslin cloth and pea nut milk is separated . solvent { ethanol : acetone ( 1:1 ) } was added to the milk and then subjected to refrigeration for 30 min . indomethacin was dissolved in chloroform and bio - polymer solution was prepared by adding water to it . the drug solution was added to bio - material solution and mixed by magnetic stirrer until emulsion is formed . release of drug through egg shell membrane : egg shell membrane was separated using con . the egg shell membrane attached to the tube . measured quantity of micro particles 5 ml solution was withdrawal and 5 ml buffer solution was added to make the volume . same procedure was repeated for different formulated films of aceclofenac with phosphate buffer ( ph-7.4 ) . the dissolution studies were perform and subjecting it for dissolution for 3 hrs and analyze the samples by uv spectrophotometer at 320 nm . % cummulative release vs time in ph 1.2 % cummulative release vs time in ph 7.4 five different formulations were formulated using various proportion of bio loaded sustained release bio - micro particles was performed . it had t50% and t 80% of 1.56 hrs and 2.45 hr formulation fg-5 shows percentage yield t50% and t80% of 1.62 hrs and 2.61 hrs at hence these two formulations are the best among the five prepared formulations .
indomethacin microparticles prepared by extraction of bio polymer from arachis hypogen . in vitro drug release study was carried out through egg shell membrane for 3 hrs and analyzed sample by uv spectroscopy at 320 nm . a novel bio membrane from arachis hypogen was isolated by simplify economical process . the % yield was found to be 80% and particle range was 3265 m .
a 36-year - old japanese man was referred to us for the evaluation of bilateral retinal hemorrhages . he developed an acute vision decrease in his left eye , pain in his right chest area , and headache after he had trekked at an altitude of 4600 m in tibet for 1 week . because of the illness , he descended 3 days later , and he felt better at that time but the visual impairment remained . our examination showed that his visual acuity was 20/20 od and 20/200 os with refractive errors of 0.25 diopters ( d ) od and 0.50 d os measured 3 weeks after the onset of the visual decrease . funduscopic examination revealed multiple intraretinal hemorrhages bilaterally and a macular hemorrhage in the left eye [ fig . 1 ] . sd - oct ( spectralis , heidelberg engineering , heidelberg , germany ) showed that the macular hemorrhage was in the superficial layer of the retina beneath the inner limiting membrane of the left eye [ fig the thickness of the choroidal layer at the fovea was 530 m od and 490 m os which was thicker than the mean subfoveal choroidal thickness in normal subjects of approximately 300 m in the enhanced depth images obtained by spectralis sd - oct . most recent studies report that the average subfoveal choroidal thickness is 287 m in caucasians of an average age of 50.4 years and 272.6 m in the japanese of an average age of 30.5 years obtained by the enhanced depth imaging of the spectralis sd - oct . fundus photograph of the right eye ( a ) and the left eye ( b ) showing multiple intraretinal hemorrhages and a macular hemorrhage in the left eye optical coherence tomographic images at the first visit . the choroidal layers ( white arrowheads ) are thick in both eyes ( a : right eye , b : left eye ) . a macular hemorrhage located in the superficial retina beneath the inner limiting membrane of the left eye ( white arrow ) can be seen individuals with acute mountain sickness present with lethargy , nausea , headache , insomnia , anorexia , and disorientation . the proposed mechanism for the symptoms of acute mountain sickness is respiratory alkalosis from hyperventilation and increased cerebral blood flow . in hace , there is a breakdown of the blood however , hace can lead to long - lasting severe neurological and psychiatric disorders and even death in some cases . wiedman and tabin examined 40 climbers who had ascended to altitudes of over 4870 m whether they developed signs of har , symptoms of acute mountain sickness , and clinically signs of hace . thus , they concluded that when advanced har is recognized , treatment should be initiated for hace with oxygen , steroids , or diuretics , and immediate descent to prevent further progression of hace . a pronounced increase in retinal blood flow in mountaineers with retinal hemorrhage and dilated epipapillary network has been detected with heidelberg retina flowmeter after acute hypoxic stress at high altitudes . the increase in retinal blood blow and cerebral blood flow under hypoxic conditions may also be associated with an increase in choroidal blood flow resulting in an increase in choroidal thickness . the pathogenesis of the increased choroidal thickness may be similar to that of hace , namely a breakdown of the blood it is difficult to draw strong conclusions from a single case ; however , we suggest that measurements of the choroidal thickness may be useful in evaluating the status of hace . currently , data are not available from population - based studies on the normal values for the choroidal thickness of a large number of caucasians or asians . further studies are needed to evaluate the choroidal thickness in mountaineers with acute mountain sickness .
we report a case of high - altitude retinopathy with increased choroidal thickness detected by spectral - domain optical coherence tomography ( sd - oct ) . a 36-year - old japanese man developed an acute vision decrease in his left eye after he had trekked at an altitude of 4600 m in tibet for 1 week . his visual acuity was 20/20 od and 20/200 os with refractive errors of 0.25 diopters ( d ) od and 0.50 d os 3 weeks after the onset of the visual decrease . funduscopic examinations revealed multiple intraretinal hemorrhages bilaterally and a macular hemorrhage in the left eye . sd - oct showed that the thickness of choroidal layer at the fovea was 530 m od and 490 m os which is thicker than that in normal subjects of approximately 300 m . we suggest that the increase in the retinal blood flow under hypoxic conditions may be associated with an increase in the choroidal blood flow resulting in an increase in choroidal thickness .
from msm who attended the amsterdam municipal health service sti outpatient clinic in 2000 and 2001 , randomly selected stored specimens of c. trachomatis dna positive ( as assessed by ligase chain reaction , abbott laboratories , chicago , il , usa ) rectal samples were tested for the c. trachomatis variant by real - time pcr ( 6 ) . from 2002 to 2005 , msm with symptomatic proctitis ( i.e. , purulent discharge , rectal ulceration , bleeding , or edematous mucosa ) and msm without symptoms were included . from the san francisco region , 51 lgv positive isolates from symptomatic msm were analyzed ( 7 ) . the isolates were collected in medical clinics ( e.g. , ambulatory care , emergency room , screening , acute care ) from 1979 to 1985 ( table ) . lgv was assessed at the time of collection , according to phenotypic properties observed during cell culture . although the growth characteristics of lgv serovars can be distinguished from serovars d k , cell culture for c. trachomatis is no longer available in most clinical settings . * in 2002 and 2003 , 45 lgv l2b variants of 109 isolates have been described in detail ( 5 ) . c. trachomatis serovar typing was performed as described previously ( 5 ) . briefly , amplification of the ompa gene ( 1.1 kb ) was performed in a nested pcr format . the ompa nucleotide sequences were subsequently analyzed by automated dna sequencing on an abi 310 sequencer ( pe biosystems , foster city , ca , usa ) . the sequences obtained from c. trachomatis infected msm in 2000 and 2001 in amsterdam and from msm in san francisco were compared to the recently identified l2b variant to determine if the strain was present earlier . the table presents the results of this analysis . in the amsterdam c. trachomatis dna positive rectal samples , lgv strains were detected by real - time pcr in 2 of 67 samples in 2000 and in 4 of 28 samples in 2001 . sequencing showed that in all 6 lgv strain positive samples , the l2b variant was present . also in 2002 and 2003 , 109 l2b - positive samples of 403 c. trachomatis dna positive rectal samples were identified , of which 45 were strain l2b , and these have been described in a previous publication ( 5 ) . all 51 san francisco specimens ( from 51 patients ) were positive for lgv variants by real - time pcr . by sequencing variable segment 2 of the ompa gene ( vs-2 ) , we identified 15 as serovar l1 , 18 as serovar l2 prototype , and 18 as the l2b variant . we sequenced the complete ompa gene of 5 of these 18 l2b variants that originated in san francisco ; all were identical to the recently described l2b variant circulating in amsterdam . four nucleotide changes were found when compared to reference serovars l2 , l2a , and the variant l2 , including 1 change that encoded the previously undescribed change at amino acid 162 , aatagt ( 5 ) . the l2b lgv variant identified as the cause of all the lgv proctitis in the recent outbreak among msm in amsterdam appears to have been circulating in amsterdam in 2000 . moreover , we showed that this l2b variant was present in the 1980s in san francisco with exactly the same mutations in the complete ompa gene . however , since we only sequenced the ompa gene , and although the sequence was identical in old and new l2b strains , we can not exclude the possibility that it could involve different strains of c. trachomatis that differ in other parts of the genome , although this is unlikely . since lgv causes potentially severe infections with possibly irreversible sequelae if adequate treatment is not begun promptly , early and accurate diagnosis is essential . sequence - based nucleic - acid tests that can discriminate between lgv serovars and less invasive c. trachomatis species can help detect cases and prevent further transmission of lgv . in conclusion , our results suggest that we are dealing with the same lgv variant > 25 years later , and the current lgv outbreak in industrialized countries has most likely been a slowly evolving epidemic with an organism that has gone unnoticed in the community for many years and is now being detected by new technologies . the numbers detected in 2005 in amsterdam suggest that a considerable reservoir exists , which emphasizes the need for ongoing public health awareness .
we traced the chlamydia trachomatis l2b variant in amsterdam and san francisco . all recent lymphogranuloma venereum cases in amsterdam were caused by the l2b variant . this variant was also present in the 1980s in san francisco . thus , the current " outbreak " is most likely a slowly evolving epidemic .
subclassification and study of cancer patients based on mutational status presents opportunities to learn the significance of genomic alterations ( and their combinations ) and to develop additional therapies ( and combinations of therapies ) . however , the large number of mutations known to be important in cancer development and the presence of multiple mutations in any individual patient combines to create a great diversity in populations of patients with a specific tumor type . shrager and tenenbaum note that cancer is in effect , a large number of rare diseases occupying a very high dimensional space with very few opportunities for action and observation in each subtype . to efficiently search a space of this nature , one needs to capture the learnings from as many patients and treatment experiments as possible in a continuously updated knowledge base.1 the stratification of cancer patients by mutational status and resultant decrease in the proportion of patients available for study enrollment presents serious problems for observational and interventional research . the low prevalence ( 12% ) of many driver mutations in solid tumors precludes recruitment of sufficient numbers of subjects from traditionally sized research consortia and has led to new models of clinical research based on interinstitution collaboration , community outreach , and broad data sharing , with the goal of learning from every treatment encounter . this article reviews the challenges and opportunities of such collaborations from the perspective of the department of veterans affairs healthcare system , the largest integrated healthcare system in the united states . creating generalizable knowledge as well as informing current individual patient care is therefore enabled by learning from all available previous treatment experiences of every relevant patient in the entire system , aggregating data across many medical centers . familiar challenges to this approach include technical issues , such as data element provenance , data quality , and database variability across institutions , ensuring patient protections in data sharing related to informed consent , privacy / confidentiality , and health insurance portability and accountability act ( hipaa ) authorization , scalability and sustainability of aggregated databases , and cultural and financial barriers to data sharing in a research community . the ability to commoditize healthcare data has created opportunities for new consortium models that enable data sharing and patient access ( for clinical trials ) . data sharing is an important element of the collaborations exemplified by orien,2 medc,3 tapur,4 and apollo5 ( see table 1 ) . the national cancer institute ( nci ) has created the genomic data commons , a unified data repository6 that enables data sharing across these and other cancer genomic studies , in support of precision medicine . the repository houses clinical health record and genomic data ( fastq file format ) and complements the cancer imaging archive,7 another ncisponsored repository that contains radiographic and pathology images for cancer patients . sharing with the research community the department of veterans affairs ( va ) has begun to move consented and hipaaauthorized patient data from the va electronic medical records to these nci data repositories for subsequent sharing with the research community ( see table 1 ) . this approach replaces the need for cancer patients to sign multiple forms consenting to data sharing with a single broad consent that satisfies the requirements of the various project institutional review boards ( irbs ) . it also provides data collection and curation that meets requirements of the individual project aims . is a joint effort by the va and the nci to provide these data to early career scientists to develop analytics and other tools in support of clinical and research objectives.8 as discussed above , traditional recruitment approaches from single or limited groups of institutions do not provide sufficient numbers of eligible study subjects to fulfill inclusion criteria with specific tumor mutation combination requirements . furthermore , thirdparty payers seldom reimburse for mutational analysis required for patient screening prior to entry into research , thus shifting to the research enterprise the cost of screening large numbers of patients , of whom only a small fraction will be found eligible . the lack of data on the mutational status of patients is the major bottleneck for clinical trial execution and slows progress in precision oncology . in the era of precision oncology treatment , the new standard of care requires clinical reimbursement for expanded panel testing in cancer patients , with subsequent recruitment in clinical trials when appropriate for the individual patient . that standard then leads to sharing and reuse of patient data for clinical trials and observational research . major efforts underway to solve these and related problems are exemplified by the medc and orien initiatives . the medc program offers insurance coverage of testing for institutions and patients who agree to contribute clinical data to the n1 registry for subsequent analysis . the oncology research information exchange network ( orien ) is a research collaboration founded by the moffitt cancer center in tampa and the ohio state university comprehensive cancer center to match patients to targeted treatments and promote datasharing activities . the group engages industry partners on sponsored projects across the clinical trials continuum and serves as a broker between research and healthcare communities . these and other programs ( particularly ncisponsored clinical trial consortia ) that foster collaboration between clinical care and research communities represent new models to advance precision oncology . membership of va sites in nci consortia and programs such as medc , orien , and tapur makes data sharing and clinical trial participation opportunities available for veterans at participating va medical centers but leaves behind patients at facilities that lack oncology research programs and infrastructure . this structural problem is not unique to the va , as opportunities for patients to participate in cancer clinical trials are similarly limited in communities not located near cancer centers . if participation in clinical trials is considered a new standard of care , then this phenomenon exposes a new and important access disparity in a healthcare system . the distributed enrollment program under development at the va cooperative studies program presents a model to move clinical trials to patients remote from va cancer centers by seeking preapproval of cancer protocols by a central irb , reduction of researchspecific training requirements imposed on participating clinicians , and centralized trial management ( such as data collection and submission ) . indeed , much of the administrative overhead built into the clinical research apparatus in the name of quality assurance , while highly appropriate for research designed primarily to benefit the broader community ( such as registration studies for drugs where effective alternatives exist ) , may have reduced relevance in a precision oncology setting , where the patient 's motivation to participate is first and foremost to obtain study drug , whether or not alternatives exist . by its nature , precision oncology is a biomarkerdriven field critically dependent on acquisition of clinical biosamples and electronic medical record data for discovery and validation with lack of access to both resources as a central limitation to the pace of discovery . collaborations to costshare for data and tissue procurement between biotech , pharma , and healthcare systems , in a precompetitive fashion , are emerging.4 to reach full potential , such collaborations require more complete integration within healthcare systems , as exemplified in the apollo program , a partnership between the nci , dod , and va . in apollo , tumor tissue is made available for biomarker analysis ( proteomics in this case ) and the results transmitted back to healthcare providers if they are determined to offer incremental value to patient care , beyond mutational analysis . while observational data is useful in this regard , randomization accelerates learning , and results in more certain knowledge . for example , demonstration that patients randomized to genomic augmented with proteomic analysis had superior treatment outcomes to those randomized to genomic analysis alone supports a compelling argument to adopt this new biomarker of response to targeted therapy . problematically , the introduction of randomization ( to biomarkers ) into clinical care using traditional clinical trial methods is costprohibitive . the department of veterans affairs continues to make progress in this area through the pointofcare research9 and precision oncology10 programs whereby patients are randomized to minimal risk alternatives with relaxed regulatory requirements appropriate with the degree of risk ( riskbased monitoring ) . data generated from these embedded studies are derived exclusively from the ehr ( realworld evidence ) and require fda acceptance if used for registration of a new companion diagnostic .
cancer genomic research reveals that a similar cancer clinical phenotype ( e.g. , nonsmall cell lung cancer ) can arise from various mutations in tumor dna . thus , organ of origin is not a definitive classification . further , targeted therapy for cancer patients ( precision oncology ) capitalizes on knowledge of individual patient mutational status to deliver treatment directed against the protein products of these mutations with the goal of reducing toxicity and enhancing efficacy relative to traditional nontargeted chemotherapy .
primary chest wall chondrosarcoma is a relatively rare malignant tumor [ 1 , 2 , 3 ] . when a patient with a history of long - term asbestos exposure has a pleural tumor with multiple plaques on chest computed tomography ( ct ) , malignant pleural mesothelioma is usually suspected . a 62-year - old man with a history of long - term asbestos exposure as a construction worker was found to have a chest wall tumor on chest ct and referred to our hospital . chest ct showed a pleural tumor invading the sixth rib and multiple pleural plaques ( fig . additional wide resection of the chest wall was performed , that is , en bloc resection , including the fifth , sixth , and seventh ribs , with a 5-cm safety margin from the tumor along the sixth rib . the chest wall defect was 25 10 cm in size , and chest wall reconstruction was performed with a polypropylene mesh . microscopic examination revealed a lobulated growth pattern of cartilaginous cells of histological grade 1 ( fig . although the margins are free of tumor cells , the tumor widely spread into the sixth rib , to 17 cm long . chest wall chondrosarcoma is relatively rare [ 1 , 2 , 3 ] . to the best of our knowledge , this is the first reported case of chondrosarcoma of the rib mimicking malignant pleural mesothelioma . two cases of extraskeletal mesenchymal chondrosarcoma mimicking malignant pleural mesothelioma have been reported [ 4 , 5 ] . goetz et al . reported a case of myxoid chondrosarcoma with a history of asbestos exposure . in our case , the patient had a history of long - term asbestos exposure and multiple pleural plaques on chest ct . additionally , diagnosis of malignant pleural mesothelioma by using fnac is insufficient because it can not differentiate malignant pleural mesothelioma from adenocarcinoma . chondrosarcoma is relatively radio insensitive , and no effective chemotherapy has been established so far . thus , complete surgical resection is the only curative option for chondrosarcoma [ 1 , 2 ] . reported that local recurrence rate was highly dependent on surgical margins . therefore , wide resection with adequate safety margin is recommended . in our case , the tumor appeared as small as 3 cm on chest ct , but the tumor cells widely spread into the sixth rib , to 17 cm long . because it is difficult to diagnose the free margin of the rib in intraoperative frozen sections , preoperative planning for wide resection of the chest wall is important . chest wall reconstruction is recommended when at least three ribs are resected or when the resected size of the chest wall defect is more than 10 cm in order to avoid pulmonary complications . our case suggests that it is difficult to diagnose chondrosarcoma in preoperative imaging diagnosis , and wide resection is necessary for curative treatment .
a 62-year - old man with a history of long - term asbestos exposure was found to have a chest wall tumor invading the sixth rib on chest computed tomography . the computed tomography also revealed multiple plaques in the pleura . malignant pleural mesothelioma was suspected , and thoracoscopic surgery was performed . thoracoscopy revealed that the tumor location was extrapleural . thus , excisional biopsy was performed . the tumor was histologically diagnosed as chondrosarcoma . additional wide resection of the chest wall , including the fifth , sixth , and seventh ribs , was performed . chest wall reconstruction was performed with a polypropylene mesh .
clinical research in the intensive care unit ( icu ) setting is essential to ensuring that patients are treated with interventions that are both effective and safe . unfortunately , lack of clarity as to when research risks are acceptable in relation to anticipated benefits has impeded important clinical trials . federal regulation governing ' exception from informed consent requirements for emergency research ' considers research risk on the aggregate , and as a result it imposes considerable restrictions on the conduct of research without consent . recently , the us office for human research protections investigated three ardsnet clinical trials for purportedly exposing trial participants to undue risk . during the protracted review , enrollment in the fluid and catheters treatment trial if burdensome regulation and unnecessary trial suspension are to be avoided , then clear thinking about research risk is required . a comprehensive and systematic approach to the ethical analysis of research benefits and harms by institutional review boards ( irbs ) , called component analysis , was recently proposed . it was endorsed by the us national bioethics advisory commission in its final report and by a number of commentators [ 4 - 6 ] . the present commentary provides the reader with a brief introduction to component analysis and highlights its application to icu research . the central insight of component analysis is that clinical research often contains a mixture of study interventions . therapeutic procedures , such as a particular ventilation strategy , insertion of a pulmonary artery catheter , or administration of a drug , are given with therapeutic warrant . that is , they are administered on the basis of evidence supporting the expectation that the intervention may benefit the study participant . nontherapeutic procedures , such as downloading data from monitors , drawing extra blood for pharmacokinetic drug levels , or abstracting information from the patient 's chart , are administered without therapeutic warrant and are performed solely to answer the study question . because therapeutic procedures hold out the prospect of benefit to trial participants and nontherapeutic procedures do not , a separate moral calculus is required for each type of intervention . clinical equipoise requires in essence that therapeutic procedures in a clinical trial be consistent with competent clinical care . more formally , it requires that at the start of the trial there exist a state of honest , professional disagreement in the community of expert practitioners as to the preferred treatment . the irb ensures that this standard is met by reviewing the justification in the study protocol , the relevant literature and , if necessary , the opinions of impartial experts . therapeutic procedures are acceptable if the irb certifies that there is sufficient evidence supporting each of the procedures such that , were it widely known , expert practitioners would disagree as to the preferred treatment . nontherapeutic procedures do not offer the prospect of benefit to trial participants and hence a harm benefit calculus is inappropriate . risks of nontherapeutic procedures must be minimized consistent with sound scientific design and , furthermore , they must be deemed reasonable in relation to the knowledge to be gained . the irb ensures the first standard is met by asking whether all nontherapeutic procedures are necessary to answer the study question and , if possible , by identifying procedures that might equally well piggyback on routine clinical interventions . the second standard requires that the irb judge the scientific and social value of the study to be sufficient to merit the nontherapeutic risks posed to participants . when clinical research involves members of a vulnerable population , such as pregnant women , prisoners , children , or adults incapable of providing informed consent , additional restrictions may apply . a threshold may limit the amount of nontherapeutic risk to which vulnerable research participants may be exposed legitimately . in the case of children , nontherapeutic risks are limited to a minor increase over minimal risk , that is , a minor increase over the ' risks of daily life ' . it has been cogently argued that a similar degree of protection ought to be afforded to adults incapable of providing informed consent a vulnerable group comprising a large proportion of participants in icu research . to determine whether risks associated with nontherapeutic procedures meet this standard , the irb reasons by analogy . it asks whether risks posed by nontherapeutic procedures are the same as those ordinarily encountered in daily life or are sufficiently similar to those risks . the irb may deem a study acceptable only if the moral calculi for both therapeutic and nontherapeutic procedures are satisfied . component analysis allows us to disambiguate this claim , and focus attention on the incremental risk posed to icu patients who enter a clinical study . clinical equipoise ensures a rough parity in terms of benefit , harm , and uncertainty between the procedures that patients would receive as a part of clinical practice and therapeutic procedures in a clinical trial . thus , whatever incremental risks are posed to participants stem from nontherapeutic procedures . in icu research , these procedures are commonly limited to downloading data from monitors , abstracting chart information , and a few extra blood tests . in these cases , studies are properly understood as posing only minimal risk a finding with implications for both irb review and the informed consent process . we argued elsewhere that acute care research in which it is not possible to obtain the consent either of the patient or of their proxy decision maker might proceed under a simplified version of the waiver of consent . we argue that this approach offers a superior alternative to the unduly restrictive ' exception from informed consent requirements for emergency research ' . provocatively perhaps , component analysis also suggests a novel approach to informed consent . in this approach , the focus is shifted away from the life - threatening complications of the patient 's illness , which are present regardless of whether the patient participates in research , to the incremental risks posed by study participation . the consent negotiation is thereby allowed to concentrate on the question , ' what difference will it make to me to participate in this study , as opposed to being treated in accordance with routine clinical care ? ' professor weijer 's research is supported by a canadian institutes of health research investigator award and operating grant .
research in the intensive care unit ( icu ) is commonly thought to pose ' serious risk ' to study participants . this perception may be at the root of a variety of impediments to the conduct of clinical trials in the icu setting . component analysis offers a promising approach to the ethical analysis of icu research . because clinical trials commonly involve a mixture of study interventions , therapeutic and nontherapeutic procedures must be analyzed separately . therapeutic procedures must meet the requirement of clinical equipoise . risks associated with nontherapeutic procedures must be minimized consistent with sound scientific design , and be deemed reasonable in relation to the knowledge to be gained . when research involves a vulnerable population , such as adults incapable of providing informed consent , nontherapeutic risks are limited to a minor increase over minimal risk . understood in this way , the incremental risk posed by participation in icu research may be minimal . this realization has important implications for review by institutional review boards of such research and for the informed consent process .
illness in the intensive care unit is defined not by pathologic changes in a particular tissue or by structural changes in a specific organ , but by a constellation of quantifiable changes in physiological and biochemical measures . to round in an intensive care unit is to be exposed to a cacophony of numbers the ph , the glasgow coma scale , the fibrinogen level . to be an intensivist means to take this chaotic melange of digits and to transform them into a clinical profile that will support a therapeutic decision . an uninitiated visitor to a contemporary intensive care unit could be forgiven for concluding that the intensivity of intensive care referred to the zeal with which its practitioners measure things : the continuous recording of the pulse , the blood pressure and the transcutaneous oxygen saturation , and the frequent assay of circulating factors whose function is familiar ( e.g. potassium or hemoglobin ) as well as those factors whose biologic significance is less so . high on the list of those less significant factors is procalcitonin . in the present issue of critical care , level and colleagues report the results of a carefully conducted cohort study of 15 patients undergoing continuous venovenous hemodialysis . they show that procalcitonin ( pct ) is cleared by continuous venovenous hemodialysis at conventional filtration rates and that the protein adsorbs to the filter , so as much as 20% of pct is removed through the membrane . the consequences of this removal are modest , however , and are probably not clinically significant . what message should the beleaguered intensivist , struggling to maintain a focus in the face of an onslaught of new measures and new sources of uncertainty , take from this report ? i believe there are two : one message regarding the utility of pct as a diagnostic marker , and the second message addressing the more fundamental question of how to interpret the masses of numeric information generated within the intensive care unit . a report by assicot and colleagues a decade ago , evaluating 79 children suspected of being infected , suggested that elevated levels of pct could reliably discriminate patients who were truly infected from those patients in whom clinical signs of acute inflammation were initiated by noninfectious stimuli . since that report , and driven in no small part by the development of a reliable assay for pct , a medline search using the keyword ' procalcitonin ' currently identifies 483 publications these studies suggest that , although pct levels can be elevated in noninfectious conditions such as the treatment of transplant rejection with antibodies to cd3 , elevated levels of pct are a reliable and specific marker of invasive infection [ 4 - 6 ] , and that adequate treatment of such infection results in a reduction in the levels of circulating pct . the utility of pct as a diagnostic marker appears to be less in its sensitivity to detect infection than in its specificity to rule it out . in particular , a low level of pct permits the clinician to be confident that infection is not present with greater than 90% certainty . but if pct is a promising marker that permits us to conclude that a critically ill patient is not infected ( and so to avoid noninformative diagnostic investigations or exposure to unnecessary antibiotics ) , how confident can we be that the information it provides can be applied in all critically ill patients ? clinicians must make categorical , yes / no decisions based on data that are continuous in character . a culture of a venous catheter tip is considered positive if more than 15 colonies of bacteria are present following a standardized method of culture , or transfusion is administered if the hemoglobin level is less than 70 the validity of each of these thresholds has been established empirically , but their successful application depends on the reliability of the measure that is used . can that reliability be significantly jeopardized by an artifact resulting from the confounding effects of the underlying disease or its treatment ? this is the question that level and colleagues sought to address , and a question of practical importance to the interpretation of diagnostic tests in the intensive care unit . the circulating level of a given molecule depends on three factors : the rate of production and release of the molecule , the rate of its removal , and the volume within which it is diluted . when rates of production and removal are equal , a steady - state constant level results . however , the actual measured level of that steady state will depend on the volume of distribution . although the kinetics of the synthesis and release of pct are not well understood , its synthesis and release appear to be triggered by invasive infection , with the result that levels in the circulation increase . the magnitude of this increase is clearly large enough to offset the reduction in concentration that might result from the presence of a larger volume of redistribution in the resuscitated , septic patient . is it , therefore , either artefactually increased in renal failure or reduced by hemodialysis ? herget - rosenthal and colleagues studied pct levels in 68 patients with acute or chronic renal failure treated by intermittent hemodialysis . they found that elevated pct levels had an 84% positive predictive value and an 87% negative predictive value for the diagnosis of infection . low flux membranes did not alter these figures after the start of dialysis , while high flux membranes did result in a significant reduction in the negative predictive value to 54% . in contrast , in a study of 26 patients undergoing continuous venovenous hemofiltration , meisner showed that although pct was adsorbed to the membrane , and removed in the ultrafiltrate , plasma levels remained constant . it thus appears that continuous venovenous hemodialysis at conventional flow rates does not jeopardize the diagnostic utility of pct ; whether high - volume hemofiltration has an effect remains to be determined . from a broader perspective , the evolving literature on pct underlines the conceptual quandaries that confront the contemporary intensivist . why is the pro form of a calcium - regulating hormone released during bacterial infection ? is its release a marker of an appropriate host response to that infection , or is it a marker of a maladaptive response that might contribute to the morbidity of sepsis ? is pct simply a convenient diagnostic marker , or is it an appropriate target for therapy ? are the diagnostic criteria of infection used to evaluate pct performance indicative of a disease process ( infection ) whose timely and appropriate treatment might improve outcome ? or , rather , are the criteria surrogate markers of an alternate disease ( hyperprocalcitonemia ) that merits therapy in its own right ? the emergence of the intensive care unit as a locus for providing supportive care for critically ill patients has confronted us with challenges that are unprecedented in medical history . intensive care units care for a population of patients who , if nature were permitted to take her course , would die a rapid death . in the absence of fluid replacement and circulatory support with exogenous catecholamines and vasoactive agents , the end result of shock is a quiet death from circulatory insufficiency ; without the mechanical ventilator , hypoxemia similarly leads inevitably to a rapid demise . but if survival under these circumstances is unprecedented , how should we interpret the biochemical and physiologic events that occur in patients who remain alive only because of the intervention of the intensivist ? there is no compelling evolutionary argument to support the advantages of one physiologic state over another : those who in an earlier era would have died do not contribute to the gene pool , and even in our own brave new age reproduction while on the ventilator is distinctly uncommon . under these circumstances , what is normal may not be what is optimal , and what is abnormal may not be reliable .
intensivists depend upon a large number of measurements to make daily decisions in the icu . however , the reliability of these measures may be jeopardized by the effects of therapy . moreover , in critical illness , what is normal is not necessarily optimal . procalcitonin , a putative marker of occult infection , is emerging as a valuable diagnostic marker in the icu . although questions remain regarding its specificity , an increasing body of work suggests that it is reliably elevated in the setting of infection . as demonstrated by level and colleagues in this issue of critical care , its utility as a diagnostic marker is not affected by concomitant hemodialysis .
a 48-year - old man presented with acute lower extremity weakness and back pain developed while he was working in a crawl space hyper extending his back . muscle strength was 4/5 in right iliopsoas and hamstrings , and 0/5 in the remaining muscles of right and all muscles of the left lower limb . bilateral knee and ankle reflexes pinprick sensation was decreased over left leg up to t12 level , and right l3 through s2 dermatomes . vibration and proprioception sensation were normal . at a peripheral hospital , spine mri was unremarkable on day 1 . spine mri on day 2 showed a non - enhancing intra - axial bright t2-weighted signal preferentially involving the anterior part of the cord extending from the conus to t11 ( figure 1a&b ) . on day 4 , ncs showed normal sensory and motor studies except an unrecordable right peroneal motor response ( chronic , post ankle fracture ) . f - waves were not obtainable over the peroneal and tibial nerves ( figure 2a - c ) . cerebrospinal fluid protein , glucose , cell count , cytology and flow cytometry were normal . extensive metabolic , infectious , vasculitis , cardiac , malignancy and thrombophilia workup were unremarkable . he initially received intravenous methylprednisolone and plasma exchange for a presumed transverse myelitis . on day 10 , mri showed smooth enhancement of the ventral nerve roots and conus ( figure 1c&d ) . the diagnosis was revised to conus medullaris infarct based on the distribution and evolution of mri findings . . left peroneal and bilateral tibial compound muscle action potential ( cmap ) amplitudes decreased . left peroneal f waves reappeared with poor persistence ( 6.7% ) and prolonged latency ( 64 ms ) . although cmap amplitudes remained decreased , all f - waves were obtainable with a slightly prolonged latency and normal persistence ( figure 2g - i ) . spine mri axial ( a ) and sagittal ( b ) t2-weighted mri obtained 2 days after symptoms onset demonstrating t2 hyperintense signal within the conus extending to t11 . axial t2-weighted ( c ) and contrast enhanced ( d ) mri obtained 10 days after symptom onset demonstrating contrast enhancement of the anterior part of the conus and nerve roots . f - waves ( a - c ) f responses recorded by stimulating the left peroneal and bilateral tibial nerves at day 4 , ( d - f ) day 18 , and ( g - i ) day 56 after disease onset . m - direct motor response ; ml - minimal latency ; mv - millivolt , nr - no response ; ms - millisecond f - wave is not considered part of the workup in conus medullaris infarct . however , in the absence of upper motor neuron signs , especially with a normal early spine mri and absent f - wave , confusion about the diagnosis may arise . in a previous case report , f waves were absent in the hyperacute stage ( first 4 hours ) of anterior spinal cord infarct.2 in our patient , f waves were absent at the time of the first nerve conduction studies ( ncs ) ( day 4 ) and they may have been absent from the onset . hiersemenzel et al3 described reappearance of f waves after the stage of spinal shock in 12 patients with a traumatic paraplegia above t10 level , and attributed the early absence of f waves to reduced excitability of motor neurons at the stage of spinal shock . in our patient , the mechanism of injury was ischemia ( rather than trauma ) and the injury level was at the conus ( lumbosacral spinal cord segments ) with involvement of the motor nerve roots that showed enhancement on the follow - up mri . recovery of left tibial f waves on day 18 coincided with development of hyperreflexia in the same leg . in contrast , at the time of reappearance of right tibial f waves on day 56 , right ankle reflex was absent . thus , we hypothesize that the absent f - waves in the acute stages of conus medullaris infarct might have been due to temporarily unexcitable ahc as a result of spinal shock , spinal cord edema , and possibly oligemia ( figure 1 ) . we would not expect f waves to be recordable in the setting of a diffuse infarction of all ahc in the spinal cord segments subserving the tested nerve . we are not aware of any reports describing f wave as a prognostic indicator of conus medullaris infarct . we postulate that persistently absent f waves , beyond the stage of spinal shock ( 2 - 4 weeks ) , in conus medullaris infarct indicates severe damage to ahc in the lumbosacral region and poor chance for renervation through collateral sprouting . all f waves had reappeared by day 56 , at least 8 weeks before he regained walking . the area of infarct involved the ventral part of the spinal cord supplied by the anterior spinal artery . we suspect the mechanism of infarct was secondary to a hyperextension injury similar to the non - traumatic spinal cord infarct described in novice surfers ( surfers myelopathy ) . in such cases , the postulated mechanisms of ischemia include transient arterial compression during a prolonged back hyperextension in the setting of poor collaterals , vasospasm or thrombosis of the artery of adamkiewicz , and avulsion of perforating blood vessels.4,5 the prognosis of surfers myelopathy varies from complete recovery to persistent paraparesis.4,5 a normal mri in the first few days does not rule out spinal cord infarct . in reported case series , the earliest changes ( bright t2-weighted signals ) appeared after 1 - 2 days , followed by enhancement appeared within the first week and peaked at 14 -21 days.6,7 cauda equina enhancement post spinal cord infarct has been described in a few case reports and attributed to a possible disruption of blood - nerve barrier.2,8,9 in conclusion , further studies are required to test our hypothesis that reappearance of tibial and peroneal f waves has a prognostic value in predicting walking in patients with conus medullaris infarct .
absent f wave in the stage of spinal shock has been described in cases of traumatic spinal cord injury . the role of f wave in predicting prognosis after conus medullaris infarct has not been described . we describe herein a middle aged - man with a conus medullaris infarct . both tibial and peroneal f waves were absent on day 4 . the left tibial f wave reappeared in the following study on day 18 . all f waves reappeared on day 56 at which time the patient was still wheelchair bound . he regained walking on day 105 . we hypothesize that reappearance of initially absent f waves post conus medullaris infarct is a good prognostic sign for the return of ambulation . the applicability of this observation requires further research . we also discuss clinical and diagnostic caveats in this case .
a maximum mouth opening that is smaller than the size of a complete denture can make prosthetic treatment challenging . this article describes a simple technique used to fabricate maxillary and mandibular custom sectional impression trays for making definitive impressions in patients with microstomia . microstomia is defined as an abnormally small oral orifice.1 this disorder is described as a reduction in the oral aperture size associated with facial burns , diffuse scleroderma , traumatic injuries , and surgical reconstruction involving the orbicularis oris muscle . microstomia can result in multiple debilitating sequelae such as inability to masticate , droolin g , speech problems due to poor articulation , impaired delivery of oral hygiene and dental care , and psychological problems secondary to facial disfigurement.28 several methods of prosthodontic treatment for microstomia patients have been presented , and numerous devices to expand the oral commissure have been described.3,912 the prosthetic rehabilitation of microstomia patients presents difficulties at all stages , from preliminary impressions to prosthesis fabrication.13 because such patients have small oral openings , using conventional methods for making definitive dental impressions and fabricating dentures may be extremely difficult . making the accurate impressions represents the initial difficulty in the prosthetic rehabilitation of such patients . the recommended techniques for obtaining preliminary impressions for microstomia patients include the use of modeling plastic impression compound , the use of stock impression trays with heavy and light body silicone impression materials , and flexible impression trays with silicone putty . the casts obtained from these preliminary impressions are then used for making custom sectional impression trays . these trays typically consist of 2 locking devices or assemblies , 1 situated anteriorly and the other posteriorly , which join and provide stability to both the sections of the trays . after the definitive impressions are made , these trays are reassembled extraorally and are poured in dental stone to obtain definitive , working casts . several studies have described various techniques that are used for making custom sectional impression trays.1418 different devices used for connecting the custom sectional trays include hinges,14 plastic building blocks ( lego ; lego systems inc , enfield , conn . , usa),15,16 orthodontic expansion screws,17 or locking levers.18 this article describes a simple , cost - effective , and time - saving method for fabricating custom sectional impression trays using easily available dual die - pins and sleeves as potential devices for interlocking the sectional trays . the locking mechanism design includes an anterior locking assembly for the maxillary and mandibular custom trays and a posterior locking assembly for only the maxillary custom tray . by using the conventional method , fabricate the maxillary and mandibular custom sectional impression trays by using autopolymerizing acrylic resin ( dpi - rr , dental products of india , mumbai , india ) on the preliminary casts . make the handles of the trays ( minimum dimensions with 13 mm height , 10 mm length , and 10 mm width ) such that they incorporate the metal sleeves of the dual die - pins ( m.r . section both the custom impression trays at the midline by using a diamond disk ( dfs , germany ) . steps in the fabrication of the anterior lock assembly in the maxillary and mandibular custom sectional impression trays : - the assembly basically consists of 2 dual die - pins and 2 sleeves . closely juxtapose the 2 sleeves such that the smaller keyway of 1 sleeve faces the larger keyway of the other sleeve . join these sleeves by inserting the 2 dual die - pins and making the assembly a rigid joint ( figure 1a d ) . - make a slot on the inside portion of the handles on each half of the sectioned custom trays to incorporate the sleeve ( figure 2a & b ) . - attach the sleeves in the slots by using the autopolymerizing acrylic resin as mentioned previously ( figure 3 ) . - verify the position of the attached sleeves by inserting die - pins such that the halves juxtapose precisely in both the sectioned trays . - fabricate an anterior assembly for the mandibular sectional tray in a similar manner ( figure 7 ) . steps in the fabrication of the posterior lock assembly in the maxillary custom sectional tray : - cut the 2 dual die - pins that are attached to sleeves halfway through their heights ( figure 4 ) by using a carborundum disk ( dentorium , new york , usa ) . only the broader upper halves are used for fabricating the posterior assembly and the lower halves are discarded . - attach the half - cut sleeves on the posterior parts of the sectioned custom tray halves using acrylic resin such that the greatest dimension of each sleeve is oriented in the anteroposterior direction . check for parallelism between the sleeves using a dental surveyor and verify the fit of the die - pins in the respective sleeves ( figure 5 ) . - fabricate an acrylic resin block ( 6 mm height , 10 mm width , and 4 mm longer than the distance between the 2 attached sleeves ) using the autopolymerizing acrylic resin ( figure 4 ) . - transfer the respective points of the half - cut die - pin heads on the acrylic bar by using a pressure spot indicator ( coltene psi , switzerland ) and drill slightly oversized holes in the acrylic bar on the marked points by using a bur ( 261-ef023 , brasseler , usa ) - with the closely juxtaposed sectioned trays and the die - pins of the anterior assembly placed in position , secure the heads of the half - cut die - pins into the holes by using the autopolymerizing acrylic resin ( figure 5 & figure 6 ) . the 2-piece custom - made tray described above allows for a functional impression to be made despite the difficulties associated with microstomia . the anterior locking assembly described in this article does not require any special alteration in the conventional custom tray design . the die - pins with metal sleeves used in this technique provide a greater degree of stability and a precise union of the 2 sections of the tray . the joint can be made more rigid by activating the 2 prongs of each dual die - pin away from each other . this technique used for fabricating custom sectional impression trays does not require any special devices or complex locking joints . the only additional materials used are the dual die - pins which are commercially available at a minimum cost . it is often difficult to use conventional methods for fabricating dentures for patients with limited mouth opening . this article described a simple , time - saving , and cost - effective method used to fabricate custom sectional impression trays for making definitive impressions in patients with microstomia .
objective : a maximum mouth opening that is smaller than the size of a complete denture can make prosthetic treatment challenging . this article describes a simple technique used to fabricate maxillary and mandibular custom sectional impression trays for making definitive impressions in patients with microstomia .
heterogenous vancomycin intermediate staphylococcus aureus ( hvisa ) strains have been reported as indicators for reduced vancomycin susceptibility in s. aureus , and various studies associated its presence with vancomycin treatment failure . it has been shown that methicillin resistant s. aureus ( mrsa ) has the propensity to evolve into hvisa phenotype during in vitro exposure to subinhibitory concentrations of vancomycin . during the last decade , hvisas had been isolated in many countries including those in south east asia ; nevertheless , in our knowledge , its emergence has not been reported in malaysia . as a pilot study , we investigated the prevalence of hvisa among mrsa strains isolated at hospital kuala lumpur ( hkl ) in a 3-month period and determined factors associated with its infections . hospital kuala lumpur is the largest hospital in malaysia with the highest mrsa burden in the country . in this hospital , vancomycin is used as the standard first line treatment for mrsa infection ; however , recently , its efficacy has been a subject of discussion due to several anecdotal vancomycin treatment failure cases in hospital kuala lumpur . we also wondered if some of the mrsas isolated in the hospital were actually hvisas with reduced susceptibilities to vancomycin that could not be detected by routine microbiological tests used in our hospital diagnostic laboratory . to investigate this , from 25 february to 25 may 2009 , we collected a total of 320 index mrsa isolates ( first mrsa isolated from the corresponding patients ) and established them as strains for vancomycin resistance testing . as it is cost , time and labor consuming to perform vancomycin population analysis on all 320 strains to test for heterogenous vancomycin resistance , strains were first screened for the phenotype using glycopeptide resistance detection ( grd ) etest antibiotic strips ( ab biodisk , sweden ) . after grd screening , a total of 8 strains were defined as presumptive hvisa , no visa strain was detected . following that , to confirm the results of the grd screening interestingly , area under the curve ( auc ) analyses of the strains ' population analysis profiles confirmed that 7 out of the 8 tested strains were hvisa ( table 1 ) , giving a prevalence rate of 2.19% . table 1heterogenous vancomycin intermediate staphylococcus aureus strains and their corresponding patients in this study.specimen noagegenderprimary diagnosisspecimendiabetes mellitusrenal failuremalignancyadmission to icudays of hospital staydays of iv vcmon beta - lactamarea under curve ratio68214maleright hip osteomyelitisnasal swabnononoyes44 days0yes0.9058253femaleleft diabetic foot ulcerpus swabyesyesnoyes52 days14yes0.9318258maleacute ventriculitiscsfyesnonoyes98 days14yes1.0125220malegluteal sarcoma with hapsputumnonoyesyes24 days10yes0.9097871malepemphigus folliaceouspus swabyesnonono34 days7yes1.012154femaleacute encephalitis with haptracheal aspiratenononoyes32 days14yes0.9646029femalemeningo - encephalitis with hapsputumnononoyes38 days10yes0.98icu , intensive care unit ; iv , intravenous ; vcm , vancomycin ; csf , cerebrospinal fluid ; hap , hospital acquired pneumonia . icu , intensive care unit ; iv , intravenous ; vcm , vancomycin ; csf , cerebrospinal fluid ; hap , hospital acquired pneumonia . all hvisa strains isolated in this study were hospital acquired as they were isolated from their corresponding patients after 48 hours of hospital admission . to determine factors associated with the 7 hvisa infections , demographic data of all corresponding patients of each index mrsa isolate were retrieved from medical records . medical history of each patient such as diabetes mellitus , renal failure , malignancy , together with prescription history of vancomycin and beta - lactam antibiotics ( as these were the only classes of antibiotics prescribed to the corresponding patients of the study isolates during this investigation ) , length of hospitalization and intensive care unit ( icu ) admission were recorded . continuous variables were then assessed by independent samples t - test , while categorical variables were analyzed using pearson 's chisquare . calculations were performed using statistical package for social science ( spss ) 12.0 ( spss inc . , chicago , usa ) where a p - value of < 0.05 was considered as significant . after performing multivariate linear regression , we found that icu admission ( p<0.004 ) , hospitalization of more than 14 days ( p<0.014 ) and vancomycin administration of more than 7 days ( p<0.016 ) were independent factors associated with hvisa infections in our group of patients . our findings were in line with those of charles et al . in 2004 , where hvisa / visa infections were found to be associated with longer antibiotic treatment periods and longer hospitalization . in a separate report it seems that patients who are severely ill , hospitalized for long durations with icu admissions might have a higher chance of developing hvisa infections . as many patients in hkl fulfill some or all of the above criteria , taking it together , we suspect that the prevalence of hvisa in hkl might be high ; however , these strains are not being actively detected by the hospital diagnostics laboratory . as hvisa and mrsa with reduced vancomycin susceptibility has been reported to cause treatment failure , given the hvisa prevalence rate detected in this study , it is not surprising that vancomycin treatment failure cases among mrsa infected patients are increasing in hkl . in our study , we employed the grd test as a screening tool for hvisa before confirming the resistance with population analysis , and found that the grd etest was fairly specific with only one false positive result . in a review , howden and colleagues have reported the test 's sensitivity as 9394% with a 8295% specificity for hvisa detection . therefore , the grd might be considered a good screening tool for hvisa in hospitals where most hospitalized patients are severely ill with long hospitalization durations . once identified as hvisa infected , optimal treatment could be prescribed to the corresponding patient to prevent vancomycin treatment failure , thereby increasing the chance of a good clinical outcome for the patient . as the strains used in this study were collected in a short span of 3 months , and that vancomycin treatment failure is on the rise in hkl , we suspect that the actual prevalence of hvisa in this hospital might be even higher . we found the grd test useful for hvisa screening , nevertheless pap - auc analysis still remains the gold standard for hvisa confirmation . a more comprehensive , case control study involving major hospitals in the country would be important to better understand the significance and distribution of hvisa in malaysian hospitals .
in a 3-month study done in hospital kuala lumpur ( hkl ) , 7 out of 320 methicillin resistant staphylococcus aureus isolates were confirmed as heterogeneous vancomycin intermediate s. aureus ( hvisa ) using the glycopeptide resistance detection e - test and population analysis , giving a prevalence rate of 2.19% . this is the first report of hvisa in malaysia .
cadaver renal transplantation is fast becoming an important modality in the management of end - stage renal disease ( esrd ) , in india . in the present scenario it is imperative that we improve the donor pool , such that more chronic kidney disease patients are benefited . the existing renal malignancy is a contraindication for renal transplantation , but data regarding graft kidneys with suspicious solid renal masses are not widely available . a 50-year - old man with a history of road traffic accident ( rta ) was admitted to intensive care unit ( icu ) and declared brain dead . formal consent for organ donation was obtained from the relatives and organ harvesting was done . under the organ sharing network , our institution received the left kidney . a 36-year - old , same blood group , routine evaluation of the recipient was done . during bench dissection of the donor kidney , a 1.5 2 c - arm screening was done and no radio opaque lesion was seen . as the cadaver transplantation was done in odd hours , frozen section facilities were not available . as the lesion was found in the mid zone of the graft kidney , partial nephrectomy was not attempted and we proceeded with enucleation of the solid lesion . the nephrotomy wound was closed with absorbable sutures and the kidney was grafted to the recipient [ figure 1 ] . post - operative recovery was uneventful and urine output improved to 150 - 200 ml / day from post operative day two . donor autopsy was done and no evidence of any other malignant lesions or lesions suggestive of metastasis was found . renomedullary interstitial cell tumour , a benign lesion of the renal medulla [ figure 2 ] . renal imaging with a plain ct scan done two months postoperatively , found no lesion in the kidney . although the number of live and cadaver renal transplants have increased in recent years , there continues to be a significant deficit . the increase in the donor pool is not able to match the ever increasing population with esrd and many patients die each year waiting for a suitable graft . the presence of malignancy in a renal graft is considered to be a contraindication to renal transplantation . there are many studies that have studied the use of kidneys with incidental detected renal masses in renal transplantation . our decision in this case to go ahead with the transplantation of the graft after enucleation was due to the small size of the lesion , its grossly benign appearance , and to give the recipient a better chance of survival and quality of life . in july 2009 , reported a series of five cases where < 2.3 cm incidental renal masses were subjected to back table partial nephrectomy and transplanted into the matched recipients . there was no evidence of cancer - specific mortality or recurrence at a 15-month follow - up . mchayleh et al . , in 2008 , reported two cases of metastasis from renal cell carcinoma in a kidney allograft . hence , many articles have been published regarding the use of grafts with renal malignancies . however , data on the management of benign renal masses in donor kidneys are few . renomedullary interstitial cell tumour is a common autopsy finding in patients over 50 years , being found in about 30% of this group . these lesions are usually round to oval , pale gray to yellow with a mean size of 3 mm . clinically symptomatic tumours are rare . the term renomedullary interstitial cell tumour was coined by lerman et al . , in 1972 . the previous terminology used was renal medullary fibroma. the finding of a discrete renal mass during bench dissection for a cadaveric renal transplant raises many questions . forty to fifty percent of renal masses less than 2 cm in size are benign . meticulous back table dissection and subsequent histopathological confirmation are necessary when such kidneys are transplanted . hence , such transplant recipients should be diligently followed up to look for recurrence of renal masses and also for occurrence of metastasis . this situation also brings up the case of the need for imaging studies like a contrast - enhanced ct , for the evaluation of potential cadaveric donors . in some cases , it may not be possible to shift the brain dead donor with all supports to the ct room . hence , at least a bedside ultrasound may add to our preoperative assessment of the kidney . incidentalomas like in our case could have been picked up on imaging and the surgical team was better prepared to deal with the situation . therefore , routine imaging studies like the ct or ultrasound may be advocated for all potential cadaveric donors , preoperatively . the increase in the use of imaging studies for a variety of abdominal conditions has increased the rate of detection of small clinically insignificant renal masses .
the number of patients living with end - stage renal disease ( esrd ) is increasing in our country and demand for renal grafts is ever increasing . cadaver renal transplantation is being established as a viable supplement to live transplantation . we present a case where a mass lesion was encountered in the donor kidney from a cadaver . enucleation of the lesion was done and we proceeded with the grafting . histopathological examination showed a renomedullary interstitial cell tumour , a rare benign lesion . post transplant , the renal function recovered well and the patient is asymptomatic . such incidental renal masses present an ethical dilemma to the operating surgeon .
it could be an incidental diagnosis in adulthood or could present with obstructive symptoms from the herniated viscera . surgical treatment consists of direct closure or mesh placement for the diaphragmatic defect , or suturing by transabdominal or transthoracic access . we report a patient with morgagni hernia who underwent a laparoscopic mesh placement with reduced port surgery ( rps ) . an 85-year - old female presented in er with a 2-day history of upper abdominal discomfort and loss of appetite . she had never experienced the same symptoms previously , and she denied any history of abdominal trauma . physical examination revealed no palpable mass in the upper abdomen , and local tenderness on upper abdomen was shown without any peritoneal inflammatory signs . serum blood test showed a hyperlipidemia and mild elevation of urea nitrogen ( total cholesterol , 250 mg / dl ; urea nitrogen , 22.5 mg / dl ) . a ct scan showed a huge diaphragmatic anterior hernia with a segment of transverse colon and fat tissue migration ( fig . the diagnosis of morgagni hernia was made and the patient was considered for repair of the diaphragmatic defect by the laparoscopic approach . under general anesthesia in the lithotomy position , the sils port ( covidien , tokyo , japan ) was inserted into a 2.5-cm umbilical incision vertically . after inspection of the visceral space , a second port , 12 mm in size , was inserted into the left lower abdomen . the herniated bowel and fat tissues were gently pulled down with grasping forceps and placed entirely into the abdominal cavity ( fig . the defect was ovoid and approximately 5 cm in size , and difficult to close by the suturing technique ; therefore we performed mesh placement with gore - tex dual mesh ( gore inc . operation time and operative blood loss were 157 min and 5 ml , respectively . the patient started to eat a soft meal on day 1 after surgery and was discharged from our hospital on day 8 after surgery without any symptoms . after 6-month , 12-month , and 24-month follow - ups , the patient had no sign of recurrence of the morgagni hernia ( figs . 4 and 5 ) . giovanni - battista morgagni first described this type of hernia in 1769 . at present , it could be an incidental diagnosis in adulthood or could present with obstructive symptoms of the herniated viscera . surgical treatment is required to relive current symptoms or to prevent possible future complications such as strangulation ileus or incarceration . originally , direct closure of the hernia site or mesh placement was used as the surgical options by laparotomy or thoracotomy . recently , the laparoscopic procedure has become available to treat this type of hernia and has bought a number of advantages such as reduced pain , shorter recovery time , and cosmetic benefits . in addition , recent trends in laparoscopic procedures have been toward minimizing the number of incisions to achieve less invasiveness . examples of such approaches are single incision laparoscopic surgery ( sils ) and reduced port surgery ( rps ) . only one report previously described morgagni hernia repair using single port access and closing the hernia site by non - absorbable suturing into extra - abdominal region . this report was very successful , however postoperative pain might be occurred because of suturing tension by lifting the interrupted sutures to close the hernia site . some technical problems associated with sils have been reported : restriction of the working field and interference of surgical instrument . the sils technique does not rely on triangulation , which is one of the core principles of conventional laparoscopic surgery , allowing adequate operative exposure while maintaining an ergonomic position for the surgeon and assistant . consequently , the inherent technical challenge that arises from the sils technique is that of a compromised view and locomotive field . therefore unfavorite outcomes such as longer operative time and possibly higher complication rate have been reported in early period of sils operation . a small additional port or a reduced number of ports as with rps are modifications that may overcome these problems . in combination with the sils port and the additional port enable the performance of dual - port surgery , even for complex operation such as laparoscopic gastrectomy . this type of repair is technically easy and should have a high probability of success because of the tension free condition . also , less postoperative pain is also expected , compared to the external knot tying technique . thoracoscopic approach is also other option for this type of hernia as a less invasive treatment , although the benefits over conventional laparoscopic approach have not been cleared . although the benefits of sils or rps over conventional laparoscopic surgery have not been established , either sils or rps could be the first choice for symptomatic morgagni hernia repair because of the acceptable operation time , blood loss , and clinical outcome . basically , cosmetic benefits and less operative pain are also expected as advantages of sils or rps originating from fewer incisions . in addition , tissue trauma and port - related complications such as organ damage , adhesions , bleeding , wound infections and hernias could be decreased . for the future direction , additional this approach is safe and can be the good indication for this kind of hernia . written informed consent was obtained from the patient for publication of this case report and accompanying images .
highlightsmorgagni hernia is a rare diaphragmatic hernia and standard therapy has not been established.the laparoscopic repair of a rare diaphragmatic morgagni hernia in an elder patient using the reduced port approach is described.this reduced port approach is a good indication for this type of hernia because of less invasive ness , cosmetics and safety .
klotho is expressed mainly in the kidneys , parathyroid glands , brain choroid plexus , and testes ( 2 - 4 ) . studies have confirmed klotho expression in other tissues , including the aorta , colon , thyroid gland , and pancreas , but the kidney remains the strongest klotho - producing organ ( 5 ) . the latter functions as a co - receptor for fibroblast growth factor-23 ( fgf23 ) . the membrane - bound form , after losing its membrane domain , enters into the circulation as soluble klotho ( skl ) , acting as a hormone with anti - aging and anti - oxidative stress properties ; skl can also be directly generated by alterative splicing of the klotho transcript ( 2 , 5 ) . klotho deficiency is an early biomarker for chronic kidney disease , and its upregulation could protect the kidney from fibrosis progression ( 6 ) . the beneficial effect of physical activity in preventing premature mortality has been established by epidemiological studies showing that exercise may delay aging through various mechanisms . klotho upregulates nitrous oxide ( no ) production and inhibits angiotensin ii - induced reactive oxygen species production within endothelial cells ( 7 ) . in an epidemiological study , handgrip strength , an indicator of total body muscle strength , the purpose of this study was to determine whether plasma klotho levels are inuenced by aerobic exercise . for this purpose , plasma klotho levels were measured in a group of trained athletes . in this study , 30 healthy football players ( males aged 18 22 years ) participated . the controls were 28 healthy young males ( age range 18 27 years ) . all subjects were nonsmokers and free of cardiovascular disease , as indicated by their medical history . none of the subjects took cardiovascular medications or hormone replacement therapy , and they all maintained routine eating habits . in the experimental group , klotho concentration was measured the morning after a session of afternoon training , with blood samples collected from the antecubital vein . all participants had abstained from caffeine and fasted for at least 8 h before sampling . we did not measure the level of physical activity in the control group ; they were healthy young males with normal daily physical activity , but none were trained athletes . plasma klotho concentrations were measured with the elisa technique , using a soluble klotho elisa assay kit based on the manufacturer s instructions ( human klotho elisa kit ; hangzhou eastbiopharm co. , ltd . , hangzhou , china ) . the demographic characteristics and measurements of the athlete group and the controls , respectively , were as follows : age , 18 22 versus 18 27 years ; body mass index , 22.3 1.4 versus 24.9 1.3 kg / mg ; total cholesterol , 5.3 0.4 versus 5.7 0.3 mmol / l ; triglycerides , 1.5 0.1 versus 1.7 0.15 mmol / l ; serum calcium , 9.8 0.8 versus 9.7 0.6 ; serum phosphorus , 4.4 0.3 versus 4.93 0.34 ; systolic blood pressure , 117 5 versus 119 6 mmhg ; diastolic blood pressure , 70 4 versus 71 3 mmhg ; and plasma klotho , ng / ml 3.375 1.48 ng / ml versus 1.39 0.43 ng / ml ( p < 0.05 ) . we found no significant differences between the groups for total cholesterol , triglycerides , and blood pressure . the control subjects were within close range of the previously proposed klotho concentrations for normal individuals , while the athlete group had significantly higher plasma klotho concentrations . the results of this study showed that aerobic exercise training induces an increase in plasma klotho levels . plasma klotho levels were only measured one time , the day after exercise in the athlete group ; therefore , it is not known whether this elevation continues over time . our study population and the controls were healthy young adult males , and their serum calcium and phosphate levels were within the normal range . in the study group , we collected the blood samples the morning after the last evening exercise , so we can not rule out the acute effect of exercise on plasma klotho levels . it has been shown that aerobic exercise training induces increased plasma klotho concentrations and decreased arterial stiffness in postmenopausal women ( 9 ) . exercise training might increase circulating klotho due to increases in peroxisome proliferator - activated receptors ( ppar ) and decreases in angiotensin ii type i receptor ( at1r ) signaling ( 10 ) . aerobic exercise - induced increases in plasma klotho concentrations could be responsible for exercise - induced decreases in arterial stiffness ( 11 ) , enhancing vascular protection and ameliorating endothelin - induced arterial stiffness . secreted klotho protects endothelial cells and smooth muscle cells through no production ( 12 ) and suppression of oxidative stress ( 13 - 15 ) . no production regulates endothelial cell calcium inux ( 9 ) . transforming growth factor beta-1 ( tgf-1 ) and endothelin-1 ( et-1 ) receptor activation negatively affect arterial stiffness , and their levels are decreased by exercise training ( 16 ) . interestingly , in a cross - sectional study , low plasma klotho concentrations were independently associated with disability among the elderly ( 17 ) . exercise - induced increment of serum klotho could be due to increased klotho secretion or increased splicing of membrane - bound klotho ( 9 ) . the kidney is the major source of skl production ( 18 ) , and membrane - bound klotho is also a co - activator of fgf23 , which is prominently expressed in distal convoluted tubule ( dct ) and proximal convoluted tubule ( pct ) cells ; these locations are essential for its function as a phosphaturic substance ( 11 ) . klotho deficiency is an early biomarker for chronic kidney disease ( ckd ) , and a progressive decline in urine klotho occurs with ckd progression ( 6 , 11 , 19 ) . endogenous klotho may inuence the processes of inammation , oxidative stress , and vascular calcication and remodeling ( 20 ) . secreted klotho directly blocks phosphate - induced dedifferentiation of vascular smooth muscle cells into osteoblast - like cells . secreted klotho also prevents the transformation of endothelial cells to osteoblast - like cells ( 21 , 22 ) . angiotensin ii downregulates renal klotho protein expression ( 23 ) , and at1r blockade increases circulating klotho . further studies are needed in order to clarify the dynamics of klotho production and secretion , and to understand the mechanisms of exercise - induced klotho secretion or shedding .
background : klotho is an anti - aging protein that is predominantly secreted by the kidneys.objectives:the aim of the study was to measure and compare the circulating klotho levels in the serum of trained athletes and in healthy , non - athlete controls.materials and methods : thirty trained football players were enrolled and their serum klotho levels were measured the morning after their last evening exercise training.results:the plasma free klotho concentration was significantly higher in the athlete group ( 3.375 1.48 ng / ml ) compared to the non - athletes ( 1.39 0.43 ng / ml ) ( p < 0.05 ) . serum levels of cholesterol , triglycerides , calcium , and phosphorus were not significantly different between the two groups.conclusions:regular aerobic exercise could increase plasma klotho levels , and this could be an explanation for exercise - related anti - aging effects .
a 75-year - old man with a slightly increased serum creatinine level had developed proteinuria and hypertension in 2000 . in 2007 , he noticed a gradually progressive hearing loss and repeated bloody nasal discharge resulting from a refractory sinusitis . initial laboratory findings revealed elevated serine proteinase 3-anti - neutrophil cytoplasmic antibody ( pr3-anca ; 26.2 eu ) , blood urea nitrogen ( 35 mg / dl ) , and serum creatinine ( 1.62 mg / dl ) levels . because of these combined findings , he was diagnosed as having granulomatosis with polyangiitis ( gpa ) according to the american college of rheumatology 1990 criteria for gpa and watts algorithm [ 1 , 2 ] . the birmingham vasculitis activity score 2003 was 6 . because of the low disease activity , the slow progression and the advanced age , we did not initiate immunosuppressive therapy at this time . in october 2008 , he developed fever lasting for several days . laboratory data were as follows : c - reactive protein 11.91 mg / dl , pr3-anca 49.1 u / ml , fibrinogen 752 mg / dl , d - dimer 1.7 g / ml , and urinary protein 0.27 g / day . activated partial thromboplastin time , prothrombin time and fibrin degradation product were within the respective normal range . although we administered meropenem ( 2.0 g / day for 1 week ) , vancomycin ( 500 mg / day for 17 days ) and itraconazole ( 200 mg / day for 20 days ) , the fever and c - reactive protein level did not decrease . on day 12 of admission a brain mri revealed a mass lesion in the right frontal lobe and hypertrophic mucosa in the paranasal sinuses ( fig . 1a ) in addition to a thickened and strongly enhanced dura mater surrounding the mass lesion ( fig . however , he rapidly developed left hemiparesis and deterioration of consciousness [ glasgow coma scale ( gcs ) : e1v2m4 ] . a brain ct revealed that the parenchymal mass lesion had enlarged and the mucous effusion was invading the posterior wall of the right frontal sinus . due to an impending transtentorial herniation , a decompressive hemicraniectomy and partial right frontal lobectomy were performed , which moderately improved his consciousness ( gcs : e2v3m4 ) . after the surgery , he developed bloody sputa , and a chest ct revealed an infiltrative shadow in both lungs . the biopsied brain material from the surgery revealed a granulomatous inflammation with geographic necrosis and multinucleated giant cells in the perivascular area of the thickened dura mater and leptomeninges ( fig . small vessels in the meninges were involved in the granulomatous lesions , and the lumens of the veins were often occluded . in the cerebral cortices and the white matter in these areas , hemorrhagic infarction was widely observed . after treatment with prednisolone ( 20 mg / day ) and azathioprine ( 40 mg / day ) , the disturbance of consciousness substantially improved ( gcs : e4v3m5 ) , and the epistaxis stopped . this study was conducted with the approval of the ethics committee of niigata university graduate school of medical and dental sciences . cns involvement such as pachymeningitis and cerebrovascular events is uncommon in gpa , reported in only 28% of cases [ 3 , 4 ] . we previously clarified that leptomeningeal and parenchymal involvement in the brain were significantly more common in pr3-anca - positive hypertrophic pachymeningitis compared to myeloperoxidase anti - neutrophil cytoplasmic antibody - positive and idiopathic hypertrophic pachymeningitis in a study of 36 patients ( including this case ) . based on the study , mri findings showed an enhancement of both the pachymeninges and leptomeninges . moreover , the present case had the extension of the mass lesion with the granulomatous inflammation , and the venous obstruction resulted in severe edema and hemorrhagic infarction . emergency decompressive craniectomy and partial lobectomy for cerebral infarction with gpa likely contributed to our patient 's survival . to our knowledge , this is the first report successfully managed with surgical decompressive craniectomy .
central nervous system ( cns ) involvement , such as pachymeningitis and/or cerebrovascular events , is rare in patients with granulomatosis with polyangiitis ( gpa ) . furthermore , the details of pathological examinations of cases have rarely been described . we describe a case of gpa that manifested as an isolated paranasal sinus disease that invaded the subarachnoid space and caused a hemorrhagic venous infarction . we also describe the pathological characteristics of the biopsied brain material from the successful decompressive craniectomy . in particular , granulomatous inflammation with geographic necrosis and multinucleated giant cells were observed in the perivascular area of the thickened dura mater and leptomeninges . small vessels in the meninges were involved in the granulomatous lesions , and the lumens of the veins were often occluded . in the cerebral cortices and white matter in these areas , hemorrhagic infarction was widely observed . we suggest that our findings represent a novel mechanism of cns involvement in gpa . moreover , we believe that the emergency decompressive craniectomy and partial lobectomy for the cerebral infarction in this patient with gpa likely contributed to his survival .
a 59-year - old man presented with liver cirrhosis and esophageal varix bleeding . a ct scan ( lightspeed qx / i , general electric co. , milwaukee , wi , usa ) with contrast media was performed and it incidentally revealed a horseshoe kidney with a well - enhanced isthmus and a 3-cm enhancing mass in the left part ( fig . the tumor was limited to the kidney , but its extension into the renal pelvis was not definite . digital subtraction angiography ( dsa ) via the right femoral artery route was performed under local anesthesia just before the embolization procedure . the dsa of the aorta showed that the horseshoe kidney was fed by a five - vessel supply that consisted of both the two normal main renal arteries , two aberrant vessels that were originating from the aorta and they entered both sides of the isthmus ( fig . 1b ) that entered the left side of the isthmus and it originated from the left common iliac artery . the dsa of the left renal artery demonstrated hypervascular tumor staining that was supplied by the anterior inferior segmental branch of the left main renal artery ( fig . although the portion of the tumor that had invaded the collecting systems could not be completely excluded , the decision was made to perform organ - preserving surgery or simple enucleation . catheterization was performed via a transfemoral approach with the use of the standard coaxial technique . a 5-f end - hole catheter ( cobra catheter , cook , bloomington , in , usa ) was introduced over a 0.035-inch guide wire ( termo ; radifocus , tokyo , japan ) to the left main renal artery . the feeding vessel to the tumor was catheterized with the use of a 3-f microcatheter ( renegade ; boston scientific , watertown , ma , usa ) and it was embolized superselectively with contour ( 355 - 500 microns , boston scientific international , la garenne colombes cedex , france ) ( fig . the contour granules were slowly and carefully injected ( to prevent reflux of the particles ) under fluoroscopic guidance before embolization with the use of a 0.018-inch - diameter tornado microcoil ( 3 mm to 2 mm , cook ) . a postembolization angiography shows a successful segmental embolization of the anterior inferior segment of the left part kidney , including the tumor ( fig . for the patient to undergo an effective and comfortable intervention , analgesic ( midazolam 2 mg , i.v . ; roche , fontenay - sous - bois , france ) and sedative ( fentanyl 60 micrograms , i.v . ; co. , hwasung , korea ) were administered just before the dsa to achieve moderate sedation . under general anesthesia the tumor site was a mild brown color and it was easily identified at the left side of the horseshoe kidney because of the previous renal artery embolization . the tumor was enucleated by repeated cuts with the use of an electrosurgical generator ( valleylab inc . , boulder , co , usa ) . the parenchymal bleeding was easily controlled by suture because of the previous renal artery embolization . upon examination of the gross specimen , the tumor showed as a well - circumscribed , bright yellow , solid mass measuring about 3 cm at its greatest diameter ( fig . the tumor was confined to the kidney and it proved to be a renal cell carcinoma , grade 2 ( fig . 1f ) . a follow - up ct scan with contrast media 33 days after the operation showed a parenchymal defect at the previous tumor site with some postoperative change ( fig . the horseshoe kidney is probably the most common of all renal fusion anomalies ( 3 ) . this anomaly consists of two distinct renal masses lying vertically on either side of the midline ; the masses are connected at their respective lower poles by a parenchymatous or fibrous isthmus that crosses the midplane of the body ( 3 ) . most of the malignant tumors arising in horseshoe kidneys are renal cell carcinomas , but transitional cell carcinomas , squamous cell carcinomas , wilm 's tumors , lymphomas , carcinoid tumors and sarcomas have also been reported ( 4 , 5 ) . it has been stated that the occurrence of renal cell carcinoma in horseshoe kidneys is no higher than in non - fused kidneys , but that the incidence of transitional cell carcinoma in horseshoe kidneys is higher , and this is conceivably due to the presence of chronic urinary tract infections ( 4 ) . the blood supply to the horseshoe kidney can be quite variable ( 3 ) . in 30% of the cases , it consists of one renal artery for each kidney ( 6 ) , but the blood supply may be atypical , with duplicate or even triplicate renal arteries supplying one or both kidneys ( 3 ) . the isthmus and adjacent parenchymal masses may receive a branch from each main renal artery , or they may have their own arterial supply originating from the aorta either above or below the level of the isthmus ( 3 ) . not infrequently , this area is supplied by branches from the inferior mesenteric artery , the common or external iliac arteries , or the sacral arteries ( 7 ) . in this case , the isthmus was receiving two arterial supplies that originated from the aorta at the level of the isthmus , and there was an additional arterial supply from the left common iliac artery ( fig . an aberrant vascular supply is one of the major anatomic features in horseshoe kidneys ; thus , the vascular supply can not be easily predicted on the surgical field . this is especially true when preoperative renal artery embolization is necessary and a part of the organ has to be removed due to malignant disease while the maximal amount of functioning renal tissue needs to be preserved . radical nephrectomy is the standard therapy for renal cell carcinoma ( 8) . in cases of neoplasm in a horseshoe kidney , however , there is a place for limited resection or heminephrectomy , with special attention being paid to the abnormal arteries and the renal pelvis ( 8) . preoperative superselective renal artery embolization helps to prevent excessive bleeding complications during organ - preserving surgery , it allows the preservation of a maximum amount of functioning renal tissue and it enables easy detection of the tumor site via the discoloration ; thus , simple enucleation is then feasible during the operation . in conclusion , preoperative superselective renal artery embolization can be an effective tool to facilitate organ - preserving surgery in a case of a horseshoe kidney with renal cell carcinoma .
only rarely is renal cell carcinoma encountered in a horseshoe kidney . this is a case report on renal cell carcinoma in a horseshoe kidney , in which superselective renal artery embolization was performed preoperatively . ct and digital subtraction angiography revealed a horseshoe kidney with a 3-cm tumor in the left side . superselective renal artery embolization of the tumor was performed as a prerequisite procedure for the organ - preserving surgery of simple enucleation . preoperative superselective renal artery embolization can be an effective tool to facilitate organ - preserving surgery in a horseshoe kidney .
a previously healthy 36-year - old man presented with slowly progressive spastic paraparesis , impairment of vibration , and pain perception below the upper trunk associated with constipation , urinary difficulty , and sexual dysfunction . he had experienced lower abdominal pain and vomiting 7 months before the onset of these symptoms , and subsequently noticed a tingling sensation in the right leg and voiding difficulties . the neurologic examination performed at the time of his first visit to our clinic was compatible with a spinal cord lesion : there was bilateral hypesthesia below the t10 level , hyperactive knee and ankle jerks , and extensor plantar responses . t2-weighted magnetic resonance imaging ( mri ) of the spine showed a lesion with a high signal intensity and mild swelling that was not enhanced after the administration of contrast material in the cervical spinal cord ( fig . 1 ) . the cerebrospinal fluid ( csf ) was clear and colorless with 5 white blood cells/l , and the cells were mostly lymphocytes . electrophoresis of csf was negative for the oligoclonal band , and the igg index was within the normal range . the laboratory findings for rheumatoid factor , antinuclear antibody , antineutrophil cytoplasmic antibodies , cryoglobulinemia , hepatitis b and c , and human immunodeficiency virus were negative . the erythrocyte sedimentation rate and c - reactive protein and complement levels ( c3 , c4 , and ch50 ) were normal . the results for the visual evoked potential and brain mri were negative . the patient was treated with steroid pulse therapy under a diagnosis of transverse myelitis . he complained of intermittent chest discomfort when subsequently visiting our clinic , but electrocardiography , cardiac enzymes , and echocardiography findings were normal . the first steroid pulse therapy provided temporary relief from bilateral hypesthesia and paresthesia , but he still experienced spastic paraparesis with painful tonic spasm and bladder dysfunction including recurrent urinary tract infection and nonspecific anterior chest tenderness , especially in the costochondral joint area . another two cycles of steroid pulse therapy were administered during the 2 years following the first treatment due to progression of sensory and motor symptoms . follow - up spine mri after the second steroid pulse therapy revealed recurrence of intramedullary myelopathy ( fig . two years after the onset of myelopathy , he developed painful swelling of his costochondral joints and both knees . a tc methylene diphosphonate bone scan showed markedly increased uptake in the left costomanubrial junction and the first rib ( fig . pelvis radiographs showed ankylosis of bilateral hip joints and poorly delineated bilateral sacroiliac joints ( fig . he subsequently displayed multiple pustular skin eruptions on the palms of the hands , soles of the feet , elbows , trunk ( fig . administration of methotrexate and sulfasalazine markedly reduced the patient 's arthralgia and neurologic symptoms , which were stabilized by maintenance therapy with methotrexate and prednisolone . the spondyloarthropathies comprise a diverse group of inflammatory arthritis conditions that share certain genetic predisposing factors and clinical features . recent studies have provided insight into distinct pathogenetic mechanisms underlying ankylosing spondylitis and reactive arthritis that arise from a complex interplay between genetic factors ( including hla - b27 ) and environmental factors.2 the pathogenesis of reiter 's syndrome may involve molecular mimicry between bacterial fragments in synovial fluid and the hla - b27 molecule . most ( 70 - 80% ) patients with reiter 's syndrome are positive for hla - b27 , as compared with only 6% of the general population . the arthritis may be perpetuated by the induction of cytotoxic t lymphocytes by microbial fragments in the joints , but these cytotoxic t lymphocytes have specificity for hla - b27-positive cells . the presence of hla - b27 may allow stronger or persistent microbial invasion.3 reactive arthritis usually has a self - limited course of 3 to 12 months , but up to 50% of patients experience recurrent bouts of arthritis , and 15% to 30% of them develop chronic symptoms of the disease.4 extra - articular manifestations such as ocular inflammation , enteritis , mucocutaneous lesions , urethritis , and ( rarely ) carditis provide essential support for a diagnosis of reactive arthritis . however , neurological complications are rare.5 there have been only a few case reports of polyneuropathy , cranial nerve palsy , or myelopathy in reiter 's syndrome.6,7 whilst there were gastrointestinal symptoms in this case , stool examinations provided no laboratory evidence of preceding infection . the patient visited our clinic at 7 months after the onset of the first symptoms . the high dose of steroids administered might have inhibited a systemic inflammatory reaction . according to a previous report , approximately only 60% of such cases have evidence of previous infection detected either by serology or by cultures from urogenital or stool samples.8 nonsteroid anti - inflammatory drugs ( nsaids ) and sulfasalazine are effective treatments for reactive arthritis , and methotrexate can also be beneficial.9 our patient did not respond to nsaids , but methotrexate and sulfasalazine relieved his neurological symptoms and arthralgia , making them stable during a 5-year follow - up . the association between reactive arthritis and cervical myelopathy was not clear in this patient , but there were some features suggesting reiter 's syndrome as a cause of the myelitis . first , the gastrointestinal symptoms that appeared before the development of cervical myelopathy might have reflected a preceding infection that initiated an autoimmune reaction , which led to myelitis and systemic inflammation . initial urinary tract infection and costochondral tenderness at the time of the first attack of myelitis might be indicative of reiter 's syndrome . second , despite the application of steroid pulse therapy , recurrences of progressive myelopathy associated with systemic symptoms of the skin and joints , and the positivity for hla - b27 suggest other causes of myelitis . third , both neurological symptoms and the arthritis that was resistant to the steroid pulse therapy did not recur after sulfasalazine and methotrexate treatment . in conclusion , reiter 's syndrome should be considered in the differential diagnosis of cases of progressive myelopathy with multiple arthritis , urethritis , and skin lesions .
reiter 's syndrome belongs to the family of spondyloarthropathies that usually present with a triad of arthritis , urethritis , and uveitis . the diagnostic criteria include clinical , radiological , and genetic findings , and the response to treatment . nervous system involvement in reiter 's syndrome is extremely rare . we report here on a 36-year - old man who initially presented with progressive cervical myelopathy and was diagnosed as reiter 's syndrome 2 years later . the myelopathy was stable after treatment with methotrexate and sulfasalazine . this case suggests that reiter 's syndrome can present as progressive myelopathy and should be considered in the differential diagnosis of treatable myelopathies .
in may 2014 , a pig fattening farm in southern germany ( federal state of baden - wuerttemberg ) that continuously houses 1,400 fattening pigs reported watery diarrhea in pigs in all age groups ( feeders to slaughter animals ) . the first cases occurred after new feeder pigs from a large piglet producer were brought onto the farm . fecal samples from diseased pigs were submitted to the regional laboratory for diagnosis , and coronaviruses were detected by electron microscopy ( figure 1 ) . additionally , 3 pigs with catarrhal enteritis were euthanized ; postmortem examination at the regional laboratory confirmed coronavirus infection in all 3 animals . porcine epidemic diarrhea virus particles seen by negative - stain electron microscopy of fecal samples . subsequently , ped was diagnosed in a private laboratory ( ivd gmbh , hannover , germany ) by use of a published multiplex reverse transcription quantitative pcr ( 7 ) . selected positive samples were submitted to the friedrich - loeffler - institut , isle of riems , germany , for confirmation and further virus characterization . at this institution , 2 fecal samples with high genome load , as determined by 2 independent , published ( 8,9 ) reverse transcription quantitative pcrs selective for pedv nucleocapsid ( n ) and spike ( s ) genes , were chosen for routine virologic testing and next - generation sequencing . the phylogenetic tree , based on full - length genomes , was constructed by using phyml ( 11 ) in the geneious software suite ( http://www.geneious.com/ ) with a generalized time reversible substitution model , and the tree was supported by 1,000 bootstrapping replicates . virus isolates were obtained after inoculation of cells of different permanent cell lines ( pig kidney [ pk]-15 and vero ) with clinical material from the pigs . sequencing of nucleic acids isolated directly from diagnostic samples pedv / ger / l00719/2014 and pedv / ger / l00721/2014 resulted in 2 viral genomes ( table ) encompassing all typical pedv coding sequences . each sequence encodes a large replicase polyprotein , a spike ( s ) protein , an alphacoronavirus - specific accessory membrane protein , an envelope protein , a membrane protein , and a nucleocapsid ( n ) protein . * kj778615 , kf272920 , kf468753 , kj584361 , kj408801 , kf650374 , kf468752 , kf267450 , kc210147 , jn825712 , kc210145 ) . # aa 632f detected in reference strain kf267450 only . comparative analyses of the full genomes showed that the strains share a very high ( 99.5% ) identity with the new variant oh851 ( genbank accession no . a more comprehensive comparison of 21 full - length pedv genomes from different years and locations revealed lower similarities ( 98.7% ) with currently circulating highly virulent strains from the united states and from china ( technical appendix figure , panel a ) . in contrast , the new isolates from germany , pedv / ger / l00719/2014 and pedv / ger / l00721/2014 , are less similar ( 97.1% ) to the isolate from europe , cv777 , which dates back to the 1970s ( 12 ) . the nucleotide alignment of the obtained pedv genomes and the available references from the database revealed a region with high variability of the first 1,200 nt in the 5 portion of the s protein the n terminal s1 domain of the coronavirus s protein is necessary for virus attachment by interaction with host cell receptors ( 13 ) and might therefore be highly mutable . although in - depth analysis of the deep - sequencing data for pedv / ger / l00721/2014 revealed a genetically homogenous population , this analysis for pedv / ger / l00719/2014 uncovered a mixed viral population with a total of 8 single - nucleotide variants . one nonsynonymous single - nucleotide variant ( variant position g19042u , amino acid substitution s6348i ) was detected in the polyprotein coding sequences . of note , 7 single - nucleotide variants are located in the aforementioned variable region in the s protein coding sequences , 5 of which are nonsynonymous ( table ) , thereby confirming the high variability in the n terminal part of the s protein . because quite extensive differences ( 50 aa ) were found between the recent n terminal s protein region of the isolates from germany and the highly virulent pedv strains from the united states and china , the isolates from germany described in this article seem to not be directly linked to the highly virulent pedv strains circulating in the united states ( figure 2 ) . in contrast , the recent isolates from germany and strain oh851 share not only high identity over the entire genome , including the highly variable 5 region of the s protein coding sequences , but also their clinical phenotype observed under field conditions . phylogenetic analysis based on 21 full - length porcine epidemic diarrhea virus ( pedv ) genomes . the new strains from germany ( pedv / ger / l00719/2014 and pedv / ger / l00721/2014 , in boldface and italics ) and the new 2014 pedv variant from the united states ( oh851 , in boldface ) were included and compared with current circulating strains from the united states and china . numbers above branches indicate proportions calculated from 1,000 bootstrap replicates : the scale bar represents nucleotide substitutions per site . comparative analyses of full - length sequences revealed that the isolates from these pigs in germany show very high nucleotide similarity with strain oh851 found in the united states in 2014 . however , differences exist that distinguish the strains from germany from the highly virulent pedv strains that caused the major losses in the united states . given the fact that pedv surveillance has been lacking in germany , we can not exclude the possibility that the strains described here have already been circulating in europe for a longer time or were indeed recently introduced from the united states or asia to europe . therefore , our report provides useful information about recent pedv strains in europe , but a comprehensive evaluation is still difficult because of a lack of data about additional strains . future studies should therefore concentrate on analysis of additional pedv from different years and locations . pairwise similarity matrix based on full - length genomes and schematic representation of the nucleotide sequence alignment of the complete spike protein coding sequences .
since 2013 , highly virulent porcine epidemic diarrhea virus has caused considerable economic losses in the united states . to determine the relation of us strains to those recently causing disease in germany , we compared genomes and found that the strain from germany is closely related to variants in the united states .
divided vascular lesions in the maxillofacial area into two groups : hemangioma and vascular malformations . they are more common in women than men ( 3:1 ) . about 60% of hemangioma lips , tongue , and buccal mucosa are the most common sites of involvement . however , it is more likely to occur in the gingiva , mandible , palate , floor of the mouth , and parotid gland . this report introduces a rare incidence of hemangioma in the buccal fat pad ( bfp ) along with phlebolithiasis . the patient was a 28-year - old woman who referred with the chief complaint of a swelling and stiffness in the left cheek . from a clinical perspective on palpation , a moving mass with a stiff area was felt ; and in intraoral examination , its position was felt in the anterior ramus . the only point in the patient 's history was a course of laser therapy for skin rejuvenation in the left cheek and several other areas in her face . in the medical history of the lesion , there were 3 times of triamcinolone injection in the area during the last 3 years to treat the lesion by another physician . the patient said that reduction in tumor size was seen for a while after these injections . aspiration was performed for the patient through intraoral approach , whose result was negative . magnetic resonance imaging ( mri ) magnetic resonance imaging revealed a solid heterogeneous mass in the pterygopalatine fossa area with penetration and extension towards both buccal and masticator spaces on the left . the submandibular area and carotid space were normal and no abnormality was seen in the nasopharynx area . mri revealed a solid heterogeneous mass in the pterygopalatine fossa area with penetration and extension toward both buccal and masticator spaces on the left . ( b ) coronal view of t1 and t2 according to the results of clinical and radiographic examinations of the treatment plan , the excisional biopsy of the studied mass was selected under complete anesthesia . under general anesthesia , the patient underwent a surgery with intraoral access through a cut in the upper area of anterior ramus . after dissection in the upper - side direction , vascular lesion was seen in buccal extension of the bfp [ figure 2 ] . the bfp capsule was intact and the mass had offended no soft / hard adjacent tissue . the possibility of a vascular lesion and second , for the purpose of liposuction for cosmetic goal and remove of the swelling on the patient 's cheek , the vascular mass was removed along with anterior lobe , as excisional biopsy through intraoral approach and hence that we did not enter into the vascular lesion [ figure 3 ] . the clinical swelling of the cheek was removed immediately after the surgery . in the macroscopic viewpoint , the lesion was a yellow and dark purple mass measuring 2 cm 3 cm 4 cm along with a hard nodule - like area . microscopic results represented a vascular lesion composed of large amounts of small to large vascular structures covered with endothelial cells . view of the totally excised lesion showing hemangioma with phlebolith histopathologic view of the lesion showing vascular lesion composed of large amounts of small to large vascular structures covered with endothelial cells . hemangiomas usually appear within a few weeks after birth and have a growth rate that exceeds the growth rate of children . in this growth phase , hemangioma will have its own characteristics : endothelial cells getting fatter along with frequent mitotic division , increased number of mast cells , and multilayer basement membrane . following this stage , flat and inactive endothelial cells are located in a context called fibrous fatty tissue with a normal view . sometimes , they can even involve all layers of the skin and offend the muscles . at the cellular level , hemangiomas are characterized by increased birth and death rate of endothelial cells and proliferation of mastocytes during the postnatal proliferative phase in the lesion . derived from young proliferating hemangiomas , capillary endothelium is easily grown in cell culture mediums and forms tubes . in accumulated hemangiomas , hence , a normal hemangioma is an endothelial tumor with a very complex life cycle of cell proliferation and natural regression . . however , their clinical manifestations are not obvious sometimes until late infancy or even childhood . phleboliths consist of a mixture of calcium carbonate and calcium phosphate salts and are thought to form when a fibrous component attaches to a developing phlebolith and becomes calcified . radiologically , they have either a radiolucent or a radiopaque core , and repetition of this calcification causes an onion - like appearance or concentric rings . it should be noted that bfp is also a mass composed of fat tissue covered with a thin capsule membrane and is mainly located in the buccal space . bfp has a rich blood supply and it is a proven fact that bfp has multipotential cells . however , there was no report about the incidence of hemangioma in the bfp until 1956 . deighan and barton first pointed to the incidence of a hemangioma case with phlebolithiasis in the bfp mass in 1956 . after a review of english literature , only two cases of the incidence of hemangioma in the bfp mass were found , except the above case . ikegami and nishijima reported the incidence of hemangioma in the bfp mass in a 23-year - old patient in 1984 . in that case , the tumor was enucleated and the presence of a cavernous hemangioma was confirmed . the last report on the incidence of hemangioma in the bfp was published by tanaka et al . in 2000 . the patient was a 3-year - old boy whose tumor was diagnosed 4 months after birth . unlike the report of ikegami and nishijima in which the lesion surface was irregular , in this case , the lesion surface was reported smooth . however , the surface of the removed lesion was also irregular in this report . like the case described in this report , no involvement was reported in the bfp mass adjacent areas in the previously reported cases . when radiographic examinations reveal a radiopaque lesion in the tumor , the differential diagnosis will be easier and there will be two possibilities : hemangioma or sialolithiasis of the parotid gland . however , when phlebolithiasis is not seen , preoperative diagnosis of hemangioma will be very difficult . in the report by tanaka et al . , the tumor was removed through extraoral access . however , in the extraoral access , the facial nerve is more likely to be damaged and risk of scar is present . however , tanaka et al . suggested that they used extraoral access because they assumed the probability of large extension of the lesion . this shows the importance of careful radiographic examinations for accurate diagnosis of the lesion limits and the selection of appropriate surgical technique . therefore , if the tumor does not have too large extension according to clinical and radiographic examinations , then intraoral access is preferred . in such cases , the use of only one single imaging modality can not provide enough information with the physician about the diagnosis and treatment of vascular lesions . therefore , the use of mri and computed tomography ( ct ) is recommended in these cases . the use of ultrasound / color doppler will also be very helpful for validating the mri and ct interpretational results . in general , the incidence of hemangioma in the bfp will be very rare , but in cases where this lesion is suspected , precise preoperative clinical and radiographic examinations are recommended .
hemangiomas are benign vascular neoplasms characterized by an abnormal proliferation of blood vessels . buccal fat pad ( bfp ) is a rare place for hemangioma . in this report , clinical , radiographic , and histopathological findings are described in a rare case of hemangioma with phleboliths involving the bfp , and a review is made of the international literature on this subject .
molecular diagnostics of cancer predisposition is very important for the medical treatment of the patient and persons belonging to the high risk group . molecular studies enable the detection of mutation carriers and release from unreasonable stress of persons from the group with increased risk of cancer occurrence . the mutation spectrums in the genes predisposing to colorectal cancer in the polish population have been described [ 1 - 4 ] . in the present work we focused on recurrent mutations of the apc gene causing fap . familial adenomatous polyposis ( fap ) is characterized by the appearance of numerous polyps in the large intestine . the correlation between mutations of the apc gene and the occurrence of familial adenomatous polyposis was described in 1991 and since then , mutations of the apc gene have been investigated in research centres leading to identification of various mutation types . mutations of the apc gene , in most cases , are small deletions or insertions with the most frequent mutations , in the greater part of the described populations , being the aaaga deletion at codon 1309 and the acaaa deletion at codon 1061 . clinical diagnoses of fap patients were conducted in collaborating genetic centres or gastroenterology clinics in the place of residence of patients . so far , samples of dna belonging to 280 polish fap families have been collected in the dna bank of polish fap families established in 1997 at the institute of human genetics , polish academy of science in poznan . dna was extracted from peripheral blood cells by the classical phenol purification method and entire coding sequence of apc gene was screened for mutations by pcr - hd and sscp methods in 280 probands . dna fragments showing heteroduplex or additional pattern in sscp analysis were sequenced by direct pcr product sequencing and analyzed using a megabace 500 sequencer according to the manufacturer 's specifications . we identified 72 mutations in 124 of our 280 fap families and observed eight types of recurrent mutations . the mutations and age of onset two of them were localized in exon 11 and the remaining six in the 3 ' part of exon 15 . the most frequent mutation , 39273931delaaaga , occurred in twenty - eight families ( 10% ) ; the second one was 31833187delacaaa , occurring in eight families ( 2.8% ) ; and the third most frequent mutation was 32023205deltcaa , detected in 5 families ( 1.7% ) . in our fap patients y500x occurred in four families ( 1.4% ) while q978x was detected in three families . each of the remaining four types of mutation occurred in two families and the frequencies of these mutations were below one percent . fifty - four recurrent mutations identified in apc gene and age of onset in a group of 124 diagnosed polish fap families nd no data available in the human mutations database at the institute of medical genetics in cardiff , considered the most representative population in the world , seven hundred mutations are listed for the apc gene . the mutation reports describe the frequency of this mutation from 0% in northwest spain , 2.4% in the australian population , 5% in dutch , 7% in israeli to 16% in the group of italian fap patients [ 6 - 9 ] . the second most frequent mutation , 31833187delacaaa , is reported with frequency ranging from 0% in northwest spain , 1.5% in israeli populations to 8.4% in australia [ 6 - 9 ] . a study of over 100 dutch families revealed equal frequency of those two most frequent mutations ( 39273931delaaaga and 31833187delacaaa ) . the latest published report in 2005 involved the analysis of over 1000 patients . in comparison to this study , the representative study of mutation frequency in the neighbouring population indicated two times higher frequency of 39273931delaaaga , whereas a difference in frequency of 31833187del - acaaa was not observed ( germany 3.8% , poland 2.7% ) . the frequency of 32023205deltcaa was equal ( 1.7% ) in both populations . in worldwide comparison differences in the frequency of mutations the polish population of fap patients belongs to the group where 39273931delaaaga occurred with higher frequency , whereas the frequency of mutation 31833187del - acaaa occurred with medium frequency in comparison with other populations . the two recurrent mutations localized on exon 11 were observed only in the polish population . in our two unrelated families with 14901491inst brain tumours were observed . another mutation ( q978x ) did not occur with higher frequency as described for other populations . in our fap patient group q283x , which occurs with frequency of 4.5% in uk fap patients , screening for these mutations permitted us to diagnose 19% of all families in our population but eight types of mutations constitute 43.5% of all our diagnosed families . the mutation study in our population should involve these eight mutations to improve molecular diagnostics of the apc gene . the study was financed by grant 2p05a10728 from the ministry of science and higher education .
the molecular diagnostics of genetically conditioned disorders is based on the identification of the mutations in the predisposing genes . hereditary cancer disorders of the gastrointestinal tracts are caused by mutations of the tumour suppressor genes or the dna repair genes . occurrence of recurrent mutation allows improvement of molecular diagnostics . the mutation spectrum in the genes causing hereditary forms of colorectal cancers in the polish population was previously described . in the present work an estimation of the frequency of the recurrent mutations of the apc gene was performed . eight types of mutations occurred in 19.4% of our fap families and these constitute 43% of all polish diagnosed families .
the incidence of acute pancreatitis is increasing around the world , where it is associated with morbidity and even the risk of mortality . it may presents with various skin manifestations as rash and rarely , with erythematous tender subcutaneous nodules , known as pancreatic panniculitis . alcohol and gallstones are the most important causes , while routinely prescribed drugs have been linked to the causation of acute pancreatitis . metformin , one of the most widely prescribed oral hypoglycemic agents , was linked to pancreatitis , secondary to overdose or in case of impaired renal function . here , we report a case of metformin induce acute pancreatitis in young healthy man with normal renal function . nineteen year - old - man , known case of type 2 diabetes mellitus for 4 y on 1 g metformin twice daily since diagnosis of his diabetes . he was in his usual state of health till he presented to the emergency department reporting nausea , vomiting and epigastric pain for 3 d. on physical examination , his height was 170 cm and body weight 99 kg ; body mass index ( bmi ) 34.3 kg / m2 , looked mildly dehydrated . laboratory investigations showed hba1c 7.7% , creatinine 58 mol / l , amylase 462 units / l ( normal range < 100 ) , lipase 1378 units / l ( 060 ) , white blood cells 16.8/mm3 ( 411 ) 80% of which was neutrophils , crp 258 mg / l ( 05 ) , mg 0.76 mmol / l ( 0.71.05 ) , ca 2.17 mmol / l ( 2.22.6 ) , ast 18 units / l ( < 39 ) , alt 34 units / l ( < 41 ) , tg 0.95 mmol / l ( < 2.3 ) , lactate 1.4 mmol / l ( 0.51.6 ) . abdominal ultrasound and ercp were done for the patient , results showed no gallstones and clear biliary tract , respectively . normalization of amylase and lipase was reached after metformin cessation , and supportive treatment in the form of iv insulin and iv fluids . few days later , after re - exposure to metformin , he presented with recurrence of his previous symptoms , and elevation of amylase and lipase was documented . as a result , metformin was suspended with improvement of his symptoms and biochemical profile . metformin is one of the most effective and valuable oral hypoglycemic agents in the biguanide class . it has been selected as the drug of choice in management of majority of patients with type 2 diabetes mellitus , as it works by several mechanisms ; decreasing hepatic gluconeogenesis , increasing glucose transport into glucose utilizing cells , decreasing appetite and caloric intake and reducing intestinal glucose absorption . as any other medication , metformin is associated with undesirable side effects and it has been reported in few cases to cause lactic acidosis and pancreatitis . acute pancreatitis is attributed to many etiological factors ; one of them is medication , where a large number of single case reports on drug - induced pancreatitis were published . diuretics , antimicrobial agents , hiv therapy and neuropsychiatric agents as well as metformin are known medication to induce pancreatitis . the underlying pathogenesis in drug induced pancreatitis in some medications as codeine was due to spasm of sphincter of oddi , in others as azathioprine , immune- mediated mechanism or a hypersensitivity to the drug is the postulated pathogenesis . in metformin , the exact mechanism is not known , but toxicity is probably secondary to acinar cell injury leading to intercellular leakage of digestive enzymes from ductules . fortunately , drug - induced pancreatitis is an acute edematous pancreatitis of a benign course and good prognosis , but fatal outcome may ensue if proper management is not initiated . among the published case reports of metformin induced pancreatitis , one was attributed to metformin overdose , other was caused by metformin accumulation , resulting from combination of drug overdose and acute renal failure triggered by vomiting in patient with concealed renal insufficiency . additionally , two case reports were found to be associated with drug induced ( angiotensin converting enzyme inhibitor , angiotensin receptor blocker , non - steroidal anti - inflammatory drug as ibuprofen and celecoxib and diuretics ) renal failure , which reduced excretion of therapeutic dose of metformin causing toxicity , leading to sever lactic acidosis and pancreatitis . lastly , in a case report of patient presented with hyperglycemia after an ingested dose between 64- 85 g of metformin in a suicide attempt , the potential mechanism of hyperglycemia is not clear and nothing among metformin s known mechanisms would logically explain the progressive and severe hyperglycemia , pancreatitis remains a potential mechanism , as the patient s clinical presentation with reportts vomiting and abdominal pain is consistent with pancreatitis . in our case , several signs can guide to and support the diagnosis of metformin induced pancreatitis ; first is the clinical presentation of abdominal pain , nausea , vomiting and dehydration . second , high levels of lipase and amylase , which correlate positively with ct findings . the lack of other known causes of acute pancreatitis such as gall stones , alcohol abuse , hypercalcaemia , hypertriglyceridaemia , and trauma , added more evidence to the diagnosis . apart from the therapeutic dose of metformin ( 2 g daily ) , our patient has never consumed any other medications known to cause pancreatitis . finally , resolution of his symptoms upon metformin cessation and relapse upon re - exposure would strongly suggest that metformin is deemed responsible of our patient s clinical presentation . available evidence suggests that acute pancreatitis in our patient was probably precipitated by therapeutic dose of metformin with normal renal function , with unknown exact mechanism . in summary , our case demonstrates the possibility of metformin to induce pancreatitis in a healthy patient without preexisting renal disease . accordingly , every diabetic patient on metformin should be counseled about symptoms of acute pancreatitis and the urgency to discontinue metformin and visit the emergency department to receive the appropriate treatment . and for us as physicians , metformin should be kept in back of our minds as a cause that would contribute to acute pancreatitis even in a healthy patient .
acute pancreatitis frequently presents with abdomen pain but may presents with various skin manifestations as rash and rarely , pancreatic panniculitis . metformin , one of the most effective and valuable oral hypoglycemic agents in the biguanide class was linked to acute pancreatitis in few cases . here , we report a case of metformin induce acute pancreatitis in young healthy man with normal renal function .
methods are described in detail in the supplementary material online , below is a brief summary . we used publicly available chip - seq data produced from the livers of human , macaque , mouse , rat , and dog for four liver - enriched tfs : cebpa , foxa1 , hnf4a , onecut1 ( schmidt et al . reads were mapped to each reference genome with bowtie2 ( langmead and salzberg 2012 ) with default parameters . numbers of mapped reads and quality metrics are shown in supplementary table s2 , supplementary material online , for the liver - enriched tfs . tf peaks were called as reproducible in both replicates if they overlapped by 75% reciprocally ( supplementary table s3 , supplementary material online ) . ( 2010 ) and were screened by eye for whether they have liver expression in mouse , rat , and dog ( based upon previously published rna - seq data , see supplementary material online ) , as well as whether they have clear 1:1 orthologs based on ensembl annotations and/or based on sequence similarity . the set of 1,373 liver expressed genes that are one - to - one orthologs across the 5 mammals was constructed based on a cut - off of 5 read fragments per kilobase of exon per million reads within the livers of mouse , rat , and dog . the binding level for orthologs across species 2015 ) . before running these analyses , for each tf and species , read alignment files were downsampled to the number of reads mapped in the replicate with the fewest number of reads . this approach combines the number of binding events , their binding intensity , and their distance from the tss into a single measure . specifically , binding level ( ais ) , where i is an orthologous gene in species s , is given by ais = log ( jkgijksdijks + 0.1 ) where k is a peak within 100 kb of the tss of gene i , gijks is the intensity of peak k for tf j in species s and dijks is the distance ( in bp ) from the tss to the summit of peak k. the region size used is 100 kb and is discussed further elsewhere ( wong et al . a pseudocount of 0.1 is added to the denominator to ensure this value is never zero . the mean and variance in binding level was calculated for all tfs and species was determined for all liver expressed genes . because the signal - to - noise ratio differs greatly across chip - seq experiments , binding levels were converted into standard scores ( for each species and tf ) so that they could be better compared across species . the standard scores of binding levels for each tf were then summed to obtain a single measure of binding level per gene . maximum - likelihood ancestral binding levels were reconstructed under a bm model in r using the ace function of the ape package ( paradis et al . enrichment levels for the histone modification markers were calculated using the same steps as for binding level . all sequence analyses reported were performed on peak and flank coordinates sliced from the 38 eutherian epo alignment ( release 74 ; cunningham et al . 2015 ) . peak sequences were taken as 100 bp from peak summits , while flanking regions were taken as 2 kb from peak ends that did not intersect other peaks or exons . these alignments were realigned with mafft ( l - ins - i method ; v6.882b ; katoh and toh 2008 ) . shared peaks across species were called based upon overlapping summits ( taken as the peak center ) within 150 bp of each other within the alignment . ancestral sequences at each node in the primate phylogeny were reconstructed using the prequel program of phast ( v1.3 ; hubisz et al . lrts were performed with the phylop program of phast to test for accelerated evolution within the human supplementary tables s1s3 , figures s1s9 , and text are available at molecular biology and evolution online ( http://www.mbe.oxfordjournals.org/ ) .
characteristics of pseudogene degeneration at the coding level are well - known , such as a shift toward neutral rates of nonsynonymous substitutions and gain of frameshift mutations . in contrast , degeneration of pseudogene transcriptional regulation is not well understood . here , we test two predictions of regulatory degeneration along a pseudogenized lineage : 1 ) decreased transcription factor ( tf ) binding and 2 ) accelerated evolution in putative cis - regulatory regions.we find evidence for decreased tf binding levels nearby two primate pseudogenes compared with functional liver genes . however , the majority of tf - bound sequences nearby pseudogenes do not show evidence for lineage - specific accelerated rates of evolution . we conclude that decreases in tf binding level could be a marker for regulatory degeneration , while sequence degeneration in primate cis - regulatory modules may be obscured by background rates of tf binding site turnover .
obsessive compulsive disorder ( ocd ) can manifest with a wide range of clinical pictures . on the other hand , there has been a long - standing observation that patients with various types of epilepsy / seizures have a higher incidence of many psychiatric disorders including ocd . though the equation of seizures leading to ocd is a known phenomenon , but to our best knowledge , we could not find literature showing ocd to cause seizures directly or indirectly . here we present a case of undetected ocd presenting as repeated seizures , which is an indirect relationship might be first of its kind in literature . a 45-year - old female from rural kashmir ( india ) , married with four children , with previous three to four admissions for generalized tonic - clonic seizures from last 3 years was presently again admitted with two episodes of generalized tonic clonic seizures . as in previous instances she again had a low serum sodium level of 115 meq / l . rest of the investigations viz . , hemogram , liver function tests , kidney function tests , blood sugar , urine examination , calcium , potassium , chloride , protein levels and lipid levels were in normal range . every time her seizure was ascribed to the only abnormal finding of hyponatremia of 110 meq / l , 114 meq / l , 121 meq / l , in previous admissions and the 115 meq / l at the present . there was no apparent cause for this low sodium except for high intake of thiazides at the first admission . in spite of changing thiazides , she again had seizures with hyponatremia every time . she was evaluated for other possible causes of seizures and hyponatremia , but no concrete cause was found . one out of three electroencephalograms showed nonspecific epileptic discharges . considering her repetitive enquiring behavior and restlessness a psychiatric evaluation was sought . on detailed psychiatric evaluation she verbalized pathological doubts , excessive cleanliness , excessive worries , repetition of acts . upon further interview , she said that whenever she took water she felt as if she did not take and did not get satisfied , and she took more and more water for the same . many a times she knew that she has taken a lot of water , but she felt compelled to take more . she further said that her idea of repeatedly drinking water was useless , but she could not resist it and had taken about 6 - 8 l of water on the day of seizure . she also described the similar repeated intake of antihypertensive tablets ( thiazides ) prior to her first seizure around 3 years back . her husband further described her habits of taking medications over the counter , from her local health workers , changing and ill formed pain and ache complaints and corroborated her behaviors of intrusiveness , excessive washing , cleaning , checking and perfectionism . she described the intrusiveness of these thoughts and the disturbance in other psychosocial spheres for more than 15 years as was also reported by the family members . she had been put on tablet phenytoin sodium 300 mg daily since her first seizure , without any significant benefit . on psychiatric evaluation she was diagnosed as ocd ( international classification of diseases-10 criteria ) and put on fluvoxamine and cognitive behavioral therapy ( cbt ) , however other causes of hyponatremia and polydipsia were still being ruled out . tablet fluvoxamine was slowly titrated from 50 to 200 mg / day over 5 weeks , and cbt sessions were also given twice weekly . the patient showed a response to therapy . in the meantime , no apparent cause for his low serum sodium / seizure / excessive water intake could be found . after a consensus with the neurologist , phenytoin sodium was slowly tapered from 6 month of the start of flouvoxamine , repeated electroencephalograms and serum sodium levels were followed and she was off phenytoin in 9 month . at present , after 28 months of follow - up she is significantly improved in ocd features and had no further seizures since then . her yale - brown obsessive compulsive scale score at start and at 28 months is 29 and 10 respectively . she scored 5 ( markedly ill ) for clinical global impressions severity ( cgi - s ) at start and at 28 months she scores 1 ( very much improved ) for cgi improvement and cgi - s also scores 1 ( normal - not at all ill , symptoms of disorder not present past 7 days ) . presently , she is only on fluvoxamine 200 mg / day , without any antiepileptic . as our findings and investigations , we excluded our differential diagnosis for seizures such as central nervous system infections , space occupying lesions , drug intake , metabolic causes , and other possible causes for seizures . as has been reported , seizures could be the only obvious neurologic manifestation of more moderate levels of hyponatremia . hyponatremia seems to be most possible cause in this case , as a correction , and future avoidance of hyponatremia prevented further seizures . in light of normal renal functions and normal metabolic profile ; a subtle , undiagnosed renal / metabolic defect can explain the development of hyponatremia in an otherwise healthy female , although development of hyponatremia with excessive water intake is known . in this case our hypothesis of ocd leading to excessive water intake and subsequent development of hyponatremia and seizure is more tangible because treatment of ocd led to the overall improvement . more importantly our patient is off antiepileptics , has improved oral contraceptive features and had an overall improvement and functionality . since epilepsy affects more than 50 million people worldwide , 80% of them live in developing world and a substantial number has no attributable cause , our finding is important in finding an indirect etiology ( ocd ) of seizures . it also points to the unawareness and the load of the psychiatric presentations in the society . we suggest more involvement of psychiatrists in emergency units , and team approach with other specialties . it will help in avoiding unnecessary continuous antiepileptic and more importantly it shows a different presentation of ocd ( close to psychogenic polydipsia ) for which both emergency residents and the psychiatrists should scratch into .
chronic epilepsy is leading to behavioral changes including obsessive - compulsive symptoms has been well - studied and shown to be about 22% , but the converse has not been reported . here , we present a case discussion of a 45-year - old female , who presented with recurrent seizures with hyponatremia , which latter was ascribed to her undiagnosed obsessive compulsive disorder ( ocd ) . this patient later did well on anti - obsessional treatment without any antiepileptic . this embarks the need for detailed psychiatric evaluation for patients in emergency care settings and gives a rare presentation of ocd .
the attack on the world trade center had the potential to overwhelm new york 's health services . initial estimates spoke of 10,000 people in each of the two towers , all of them either killed or injured . local hospitals prepared for the worst , and in the first few hours there were hundreds of patients with crush injuries and burns . horror and sadness has now been replaced by anger , fear , and the determination to be better prepared next time . this determination not only exists in politics but also in health care , and as with all attempts to enforce change there needs to be a period of collecting opinions and data . in this issue of critical care , there is a series of nine articles offering varied perspectives of the events of 11 september 2001 . louis cook is an advanced life support coordinator with the fire department of new york . on page 301 , he describes how the fire department of new york took charge , set up command and control , and then had to evacuate , replace lost personnel , and resume control after the tower collapsed . " this was a monumental task " , writes cook , " given the psychological impact of the event " . the command and control center was also staffed by the new york police department and , on page 304 , the deputy chief surgeon , charles martinez , describes his experiences , including the part he played in helping police to ensure surrounding buildings were not " used by terrorists to launch a subsequent attack ... with the specific aim of harming the rescue effort " . the article , co - authored by dario gonzalez , medical director for clinical affairs of the fire department of new york , concludes that , while the incident was dramatic , the lessons to be learnt " are the same as those from previous disasters " . on page 307 , j david roccaforte of bellevue hospital , only 2.5 miles ( 4 km ) from the world trade center , tells us how they reacted from how they triaged " as well as could be expected " to how it was " difficult to anticipate needs " . donald b chalfin , an attending intensivist at maimonides medical center , new york , offers on page 310 his eye witness observations of the attack as he sat in the city 's traffic . " like so many other physicians ... " he writes , " i was paralyzed knowing that i was unable to give help ... " . on page 312 , david crippen draws on his experience of the 1988 earthquake in armenia to suggest that , while specialized search and rescue teams may be useful , " a more cost - effective approach [ would be ] to teach life supporting first aid to the general public " . but whoever intervenes , whether it be medical personnel or the general public , there is potential psychological sequela post - traumatic stress disorder . jeffrey hammond and jill brooks state , on page 315 , that treating post - traumatic stress disorder has " only a marginal effect " , and that early intervention , in the form of critical incident stress management , can " limit the establishment of maladaptive and disruptive cognitive or behavioral patterns " , both in health personnel and victims . ron simon and sheldon teperman of the jacobi medical center , new york , were part of the hospital 's disaster committee and offer some straight - talking criticism of the city 's reaction . " the lack of communication probably resulted in more problems than all other factors combined " , they say , going on to suggest that " time , effort , and resources were wasted ... because of lack of direction and information " ( page 318 ) . at the time of writing , on page 321 , vlad kvetan suggests that , while new york absorbed the blow of the world trade center attack well , " major stress was placed on ... biohazard resources " . he suggests that because the internet has disseminated sophisticated information about bioterrorism , comprehensive guidelines on managing all kinds of outbreaks , from anthrax to smallpox , are needed . finally , in the concluding article on page 323 , kenneth mattox questions to what extent we are prepared , or can be prepared , for the unexpected , and whether the bureaucrats behind health care have the ability to make those preparations . the frameworks for multifaceted care required during disasters can only be created , he points out , " by leaving egos and personal agendas at the front door and working for a common benefit " . we hope this 10-part series of articles in critical care facilitates the opinion and data collecting that is required before changes in disaster preparedness can be enforced . the series is dedicated to the first responders fire , police , and medical personnel who attended the world trade center disaster of 11 september 2001 . they did not hesitate to place themselves in harm 's way to rescue the innocent , and without their efforts many more would have perished . the authors acknowledge david crippen 's assistance in the creation of this 10-part review series . this article , and the series it is part of , is dedicated to the first responders fire , police and medical personnel who attended the world trade center disaster of 11 september 2001 . they did not hesitate to place themselves in harm 's way to rescue the innocent , and without their efforts many more would have perished .
the attack on the world trade center had the potential to overwhelm new york 's health services . sadly , however , the predicted thousands of treatable patients failed to materialize . horror and sadness has now been replaced by anger , fear , and the determination to be better prepared next time . this determination not only exists in politics but also in health care , and as with all attempts to enforce change there needs to be a period of collecting opinions and data . this article introduces nine reviews in critical care offering varied health care perspectives of the events of 11 september 2001 from people who were there and from experts in disaster management .
primary amenorrhea is usually the result of a genetic or anatomic abnormality . in young women with primary amenorrhea , it is commonly caused by x - chromosome aberrations such as in turner syndrome , but individuals with a normal chromosome complement and ovarian dysgenesis also exist . a 35-year - old female patient presented to our medical outpatient department with complaints of palpitation and chest pain of 6 months duration . patient had paroxysmal episodes of palpitations that lasted few minutes , resolved spontaneously , and were not associated with exertion or other aggravating factors . patient had chest pain that was retro - sternal , intermittent not related to exertion and resolved spontaneously . menstrual history revealed that she never attained menarche . on examination she had dolicocephaly , arachnodactyly , high arched palate , she was tall statured [ height 165 cm ; figure 1 ] , her arm span ( 176 cm ) was more than her height , arachnodactyly [ figure 2 ] , reduced upper to lower body segment ratio ( 0.83 ) , and she had positive wrist and thumb sign . patient had underdeveloped rudimentary breasts ( tanner stage 2 ) , sparse pubic hair ( tanner stage 1 ) , and absent axillary hair . cardiovascular examination revealed mid - systolic click with a late systolic murmur , other systems were essentially normal . her blood glucose , renal function tests , liver function tests , and serum electrolytes were within normal limits . echocardiogram echo / doppler revealed prolapse of anterior mitral leaflet , mitral regurgitation and normal left ventricular systolic function . she had low levels of estrogen ( estradiol-10 pg / ml ; normal 15 - 200 pg / ml ) , serum testosterone ( 0.16 pg / ml , normal 0.6 - 6.8 pg / ml ) , and progesterone ( 0.56 ng / ml ) . she had elevated levels of gonodotropins , follicle - stimulating hormone ( fsh ) ( 101.3 serum prolactin ( 12 ng / ml , normal 0 - 20 ng / ml ) and thyroid function tests ( free t4 - 9.2 mcg / dl , normal 4.6 to 11.2 mcg / dl ; tsh-3.49mu / l , normal 0.4 - 5.0 marfanoid habitus : tall stature , height 165 cm ; arm span to height ratio of 1.067 ; upper segment to lower segment ratio of 0.83 arachnodactyly with high metacarpal index mci list of investigations that contributed in diagnoses of primary amenorrhea perrault and his colleagues in 1951 published the first report on two sisters with gonadal dysgenesis and with additional sensorineural deafness . hypothesized that there is a form of ovarian dysgenesis that is inherited as an autosomal recessive , female - limited disorder . neurologic data are available on 14 of 21 girls ; 7 of 14 had neurologic abnormalities . they concluded that high incidence of neurologic anomalies suggest that ataxia or mental retardation may not be just coincidental findings , but pleiotropic manifestations of perrault syndrome . reported on two sporadic and two familial new cases with sensorineural hearing impairment and ovarian dysgenesis , which are the cardinal signs of perrault syndrome in females . reported on two pairs of sisters with gonadal dysgenesis and deafness , cerebral , and ocular involvement who developed a progressive , severe sensory , and motor neuropathy . based on the clinical observations of known patients ; two forms of ps may be distinguished : one apparently non - progressive form and another with apparently progressive axonal - cerebellar degeneration . nishi et al . reviewed 21 patients from the literature , added ataxic gait , pes equinovarus , nystagmus , limited extraocular movements to the spectrum of neurologic defects in ps . absence of deafness in a patient with xx female gonadal dysgenesis ( xx - gd ) does not rule out perrault syndrome because the patients could develop deafness much later , at an older age . most patients had moderate to severe sensorineural deafness with mutism among patients with early onset of deafness . the findings of abnormal body proportions noted in our patients have been previously reported by jacob et al . in their observation of two siblings with perrault syndrome with marfanoid habitus . the pathogenetic basis for the ps is still unclear . studied a small family of mixed european ancestry includes two sisters with well - characterized perrault syndrome . whole - exome sequencing of genomic dna from one of these sisters revealed exactly one gene with two rare functional variants : hsd17b4 . further studies on other families are awaited to confirm the homogeneity of the genetic defect and the underlying molecular defect . these issues were discussed with our patient . during the follow up visits , we noticed depression and suicidal tendencies . after a comprehensive psychiatric evaluation , she was diagnosed with major depression and was put on anti - depressant medication . we did not advice any therapy for mitral valve prolapse as the prognosis in asymptomatic patients with nonsustained arrhythmias and structurally normal hearts is quite good . thus , the potential deleterious effects of drug therapy probably outweigh any risk from the arrhythmia in this setting . similarly , no antibiotic prophylaxis or anti - thrombotic prophylaxis was suggested as present guidelines recommends endocarditis prophylaxis for patients only at the highest risk . perrault syndrome is a rare cause of primary amenorrhea or ovarian dysgenesis , but should be considered in a female child with deafness / mutism . several reported cases have some form of neurologic deficit but our patient did not have any obvious neurological signs or symptoms , but she had marfanoid habitus , an association that was described only once in the literature . the marfanoid features could be considered as a part of extended phenotype of perrault syndrome .
gonadal ( ovarian ) dysgenesis with normal chromosomes ( 46 , xx ) , xx female gonadal dysgenesis ( xx - gd ) is a rare genetically heterogeneous disorder . in 1951 , perrault reported the association of gonadal dysgenesis and deafness , now referred to as perrault 's syndrome . perrault syndrome is a rare autosomal recessive condition affecting both females and males ; only females have gonadal dysgenesis associated with sensorineural deafness , which is present in both sexes . we present a case of sporadic perrault syndrome in a 35-year - old female with primary amenorrhea , sensorineural deafness , marfanoid features and normal karyotype . there are very few case reports describing the condition , even lesser reports of association with marfanoid habitus . we report this case for its rarity and add to the spectrum of the disease that remains undetermined .
trauma to the popliteal arteries resulting in pseudoaneurysm formation is rarely encountered outside the setting of wartime surgery [ 1 , 2 ] . popliteal pseudoaneurysms ( pps ) account for < 4% of all popliteal artery aneurysms , and hence experience of their management in individual centres is limited [ 35 ] . the diagnosis of pp may also be delayed due to long - time interval between the initial trauma and symptom onset . we present the case of a large pp causing acute limb ischaemia to a 37-year - old man . an otherwise healthy 37-year - old man presented with acute limb ischaemia of recent onset . his past medical history was significant for smoking as well as a previous gunshot wound to the right lower limb 12 years previously that required fasciotomies . diagnostic work - up included an arterial duplex scan of the right lower limb , which demonstrated a 5.5 cm 5.2 cm aneurysm of the right popliteal artery and the lack of flow in the right anterior tibial and peroneal arteries . a ct angiogram was subsequently undertaken to establish the morphology of the aneurysm for preoperative planning , which confirmed the diagnosis but also demonstrated a large number of pellets in the soft tissues surrounding the right knee in keeping with the history of previous gunshot injury to the area . poor opacification of the right anterior tibial and peroneal arteries was also demonstrated , suggestive of thrombosis or embolization secondary to the popliteal aneurysm . angiography , via an antegrade right femoral approach , showed a false aneurysm of the popliteal artery at the knee joint level . a 6 mm 25 mm viabahn ( gore ) covered stent was deployed to exclude the pseudoaneurysm , and a thrombolysis catheter was left in situ in the below - knee popliteal artery ( fig . 1 ) . thrombolysis was started using alteplase at a rate of 1 mg / h , with a concurrent infusion of heparin 400 iu / h via the arterial sheath , as per local protocol . figure 1:a digital subtraction angiogram of the right popliteal artery demonstrating the pseudoaneurysm , pre- ( a ) and post- ( b ) stenting . a digital subtraction angiogram of the right popliteal artery demonstrating the pseudoaneurysm , pre- ( a ) and post- ( b ) stenting . a repeat angiogram after 24 h of thrombolysis demonstrated improved patency while at 48 h excellent patency of the infragenicular vessels was seen ( fig . 2 ) , and the patient was subsequently discharged on dual - antiplatelet and statin therapy . a follow - up arterial duplex performed at 6 weeks post discharge demonstrated excellent patency of the stent graft and infragenicular arteries . figure 2:a digital subtraction angiogram at presentation ( a ) and after 48 h of continuous alteplase thrombolysis ( b ) demonstrating the improved patency of the infrapopliteal arteries . a digital subtraction angiogram at presentation ( a ) and after 48 h of continuous alteplase thrombolysis ( b ) demonstrating the improved patency of the infrapopliteal arteries . pps are a clinical entity that is rarely encountered in civilian medicine , and account for < 4% of all aneurysms of the popliteal artery [ 1 , 2 ] . its development is considered to be secondary to the formation of a thick fibrous pseudosac following arterial wall injury and haematoma organization . this rather slow process may explain the usual delay in diagnosis which may range from days to years , with a median delay of 1.5 months . in our case , the delay in diagnosis was 12 years after the gunshot wound . the usual presentation is that of a painful , pulsatile mass that may have given rise to complications that may threaten limb function or viability , including thrombotic and embolic events . a wide range of options exist for the management of pps , including open surgical repair and endovascular treatment options . surgical methods include the resection of the pseudoaneurysm with interposition grafting , ligation and bypass and primary arterial repair without grafting . finally , aneurysm resection and primary end - to - end anastomosis can be considered for small aneurysms . the limiting factor , however , is the amount of perianeurysmal fibrosis and chronic inflammatory changes that are frequently encountered and hinder the safe performance of open surgical repair [ 1 , 6 ] . in this case , we elected to proceed with an endovascular repair . the reasons were 2-fold : on one hand , the previous gunshot injury and the subsequent fasciotomies had led to significant deformity of the patients lower limb , while the amount of scarring would make access to the popliteal artery difficult , thus not favouring an open surgical repair . on the other hand , the distal anterior tibial and peroneal artery thrombosis would require thrombolytic therapy and thus , the endovascular approach was considered more appropriate . the false aneurysm was excluded with the covered stent prior to starting thrombolysis in order to eliminate the risk of distal embolization of thrombus from within the false aneurysm into the calf arteries during thrombolysis . endovascular repair has been shown to be a safe and efficient way of treating pps associated with lower morbidity and shorter hospital stay . careful patient selection , however , is warranted , as increased risk of early occlusion has been reported , and hence may render this approach less desirable [ 7 , 8 ] . covered stent placement was considered appropriate in this case in order to enable thrombolysis , which would not have been possible immediately after open surgical bypass . it is unclear whether stenting in this case will be the definitive treatment , as due to the young age of the patient and the anatomical location of the stenting , stenosis or occlusion of the stent is likely in the future . to this end , the patient has been put on a surveillance programme and will be followed up closely . a short covered stent ( 25 mm ) was used to avoid compromising future graft anastomotic sites . thus , the endovascular treatment offered in this case was efficient in dealing with both the acute ischaemia secondary to thrombosis as well as the aneurysm itself in the short term . on the other hand , the presence of a stent does not preclude open surgery in the future , preferably in an elective setting , and this is the approach the patient selected having being informed of his options . this case report demonstrates the efficiency of stenting in the acute management popliteal artery pseudoaneurysms with encouraging early radiological and clinical outcomes .
pseudoaneurysms of the popliteal artery are a rare clinical entity , accounting for < 4% of all popliteal aneurysms . accurate diagnosis and effective intervention is required to prevent potentially limb - threatening complications . we present the case of a 37-year - old man with acute limb ischaemia due to distal calf vessel thrombosis secondary to a popliteal pseudoaneurysm that was managed with covered stent placement and thrombolysis .
posttraumatic avascular necrosis ( avn ) of the scaphoid is a common complication probably due to the precarious blood supply . the rate of incidence is about 13% to 40% and almost all are seen in the proximal fragment [ 2 , 3 ] . careful review of the literature revealed six cases of distal avn in three studies [ 4 , 5 , 6 ] . the diagnosis of avn of the scaphoid is made by combining the mri findings with direct observation of the punctuate bleeding during the operation . however , the diagnosis of avn is suspicious in these cases , as mri investigation had been performed in one case and preoperative bleeding was observed in the other . here , we report four new cases , all with the correlation of mri and punctuate bleeding . the series consisted of four patients aged 29 , 33 , 53 and 57 years . all had a history of scaphoid fracture following a fall on an outstretched hand 35 years ago , and all had been treated by casting before . radiographic examination revealed suspected areas , such as sclerosis and/or cystic changes , on the distal pole of the scaphoid ( figure 1 ) and mri views were identical with avn of the distal pole , showing hyperintense changes ( figure 2 ) . tourniquet was deflated in order to observe punctuate bleeding ; it was observed in three cases and the patients were treated by bone grafting and wire fixation ( figure 3 ) in the remaining , there was no punctuate bleeding despite drilling and the scaphoid was excised and radial advancement osteotomy ( figure 4 ) was performed . treated by bone grafting and wire fixation . scaphoid was excised and radial advancement osteotomy . all patients were evaluated by quick disabilities of shoulder , arm , and hand score 711 years after the operation by telephone interview . according to this score , 11 questions related to daily living posttraumatic avn of the scaphoid occurs as a result of impairment of the vascular supply to the involved bone fragment . several studies had demonstrated a strikingly poor blood supply to the proximal pole , particularly in comparison with the abundant supply to the distal two - thirds of the scaphoid [ 1 , 2 ] . review of the literature revealed six cases in three reports [ 4 , 5 , 6 ] . , avn in the distal fragment occurs if the volar vessel is damaged and the fracture line is distal to the line of entry of the dorsal vessels . another explanation maybe the anomalous different vascularization of the distal pole [ 5 , 6 , 9 ] . the diagnosis of avn of the scaphoid was made by combining the mri findings with direct observation of the punctuate bleeding during the operation . however , there is no such approach in the previously described cases [ 4 , 5 , 6 ] . in the case of sherman et al . , mri was not available and there was no chance to observe bleeding , as the patients had refused surgical treatment . similarly , in the case of the garg et al . , although mri suggested avn , the patient had been treated conservatively , so there was no chance for punctuate bleeding , and the fracture united . the diagnosis is also suspicious in this case as no correlation was possible and the fracture had healed . according to herbert , avn means irreversible necrosis or death of bone and healing is impossible . only one case had been operated . in this case , the authors had confirmed avn by the pinning attempt that had failed . there seems no such method for the diagnosis of avn , so the diagnosis is again in doubt . in our opinion , all these cases should be labeled as suspected avn . in three of our cases , although preoperative mri suggested avn ( figure 2 ) , punctuate bleeding was observed during the operation and all were treated by pinning and grafting and healed uneventfully . in those cases , the appearance of avn was probably due to the normal marrow of bone that has not been established yet , but bleeding was observed due to revascularition . in the remaining case , mri suggested avn along with lack of punctuate bleeding and scaphoid was excised and radial advancement osteotomy was performed . in conclusion , all six cases reported before [ 4 , 5 , 6 ] , and three of our cases that were treated by pinning and had healed , were not real avn or the diagnosis was doubtful . so our fourth case seems to be a unique case of distal pole avn of the scaphoid in the literature .
abstractavascular necrosis ( avn ) of the scaphoid predominantly occurs in the proximal pole . review of the literature revealed only six cases and all are suspect due to the lack of either mri investigation or investigation of bleeding preoperatively . we report four new cases and one of them appears to be a real distal pole avn of the scaphoid in the literature .
providing anaesthesia for tte is always a challenge for the anaesthesiologists because of intraoperative need for one - lung ventilation and its associated problems . post - operative pulmonary complications in the early post - operative period can lead to increased morbidity and mortality . pulmonary complications can also occur due to surgical issues that may mislead the perioperative team and result in unnecessary and avoidable interventions . we would like to share our experience of two cases that underwent tte and developed acute respiratory distress in the post - operative period . a 39-year - old woman , known case of oesophageal carcinoma , was scheduled for tte with extended two - field lymphadenectomy with gastric pull - up with cervical neck oesophago - gastric anastomosis and feeding jejunostomy . she had history of hypertension since 4 years and controlled on amlodipine ( 10 mg ) and atenolol ( 50 mg ) . the anaesthetic management and monitoring were as per institutional protocol and was administered epidural and general anaesthesia . as a part of operative procedure , she was shifted to intensive care unit ( icu ) for post - operative monitoring and analgesia . her oxygen saturation decreased from 97% to 84% over h in spite of oxygen supplementation with hudson mask . on auscultation , air entry was decreased on the right side and the percussion note was tympanic . she was put on oxygen at 15 l / min through venturi mask ( fio2 0.60 ) . the respiratory distress was only partially relieved and the air leak from the icd continued . due to on - going respiratory distress , the patient 's trachea was intubated . hence , we suspected air leak from the cervical drain and it was decided to remove the cervical drain and suture the defect . a 56-year - old man , case of carcinoma mid - oesophagus underwent tte with extended two - field lymphadenectomy with gastric pull - up and oesophago - gastric anastomosis in neck . the patient developed respiratory distress and air leak through right icd and saturation decreased to 86% despite oxygen supplementation . it was decided to conduct bronchoscopy . before bronchoscopy , we observed bubbles ( air leak ) from neck drain site . on removal of corrugated neck drain and resuturing the neck wound , air leak stopped and there was no further respiratory distress . a 39-year - old woman , known case of oesophageal carcinoma , was scheduled for tte with extended two - field lymphadenectomy with gastric pull - up with cervical neck oesophago - gastric anastomosis and feeding jejunostomy . she had history of hypertension since 4 years and controlled on amlodipine ( 10 mg ) and atenolol ( 50 mg ) . the anaesthetic management and monitoring were as per institutional protocol and was administered epidural and general anaesthesia . as a part of operative procedure , she was shifted to intensive care unit ( icu ) for post - operative monitoring and analgesia . her oxygen saturation decreased from 97% to 84% over h in spite of oxygen supplementation with hudson mask . on auscultation , air entry was decreased on the right side and the percussion note was tympanic . she was put on oxygen at 15 l / min through venturi mask ( fio2 0.60 ) . the respiratory distress was only partially relieved and the air leak from the icd continued . due to on - going respiratory distress , the patient 's trachea was intubated . hence , we suspected air leak from the cervical drain and it was decided to remove the cervical drain and suture the defect . a 56-year - old man , case of carcinoma mid - oesophagus underwent tte with extended two - field lymphadenectomy with gastric pull - up and oesophago - gastric anastomosis in neck . the patient developed respiratory distress and air leak through right icd and saturation decreased to 86% despite oxygen supplementation . , we observed bubbles ( air leak ) from neck drain site . on removal of corrugated neck drain and resuturing the neck wound , air leak stopped and there was no further respiratory distress . our two cases reveal a rare cause of respiratory distress and air leak in patients who underwent tte . in these cases , slipped chest drains , lung parenchymal injury , tracheobronchial injury are well - documented causes of post - operative air leak and subsequent respiratory distress and pneumothorax . only two cases of pneumothorax associated with neck drains have been described . in both the cases , air leak was seen following transhiatal oesophagectomy . in our cases , air leak and subsequent pneumothorax were seen after tte . to the best of our knowledge , there is no literature suggesting air leak / respiratory distress following tte and subsequent improvement after removal of neck drain . after oesophagectomy , apart from icd , corrugated neck drain insertion is an acceptable technique . during tte , pleura were opened and gastric pull - up is done thus creating a communication between thorax and neck . generally , this is not a problem because the stomach tube seals the communication between the chest and the neck and the negative intrathoracic pressure is not transmitted to the neck . however , in certain situations , the corrugated drain may provide ample space for sucking atmospheric air during patient 's spontaneous respiratory efforts which creates negative intrathoracic pressure . the performance of extended lymphadenectomy , especially in the upper mediastinum may result in a wider communication between the chest and the neck . the communication between the chest and neck might then transmit negative intrathoracic pressure to the neck . if the defect size is larger than two - third of tracheal diameter , the air will preferential enter through this defect leading to pneumothorax . use of corrugated neck drain may also precipitate emphysema of neck and thus may compromise the airway as well . the possible causes of post - operative pneumothorax after thoracotomy and oesophagectomy include lung parenchymal leak / injury , bronchopleural fistula , ruptured bullae and malpositioned chest drains . the airway injury ( bronchus and lung ) during the surgery may lead to continuous air leak through chest drain or cause surgical emphysema of the face and neck . furthermore , ensuring the absence of air leak from lung before closure of thoracotomy incision needs to be emphasised . chest drains may occasionally slip out in the post - operative period which can normally be recognised by identifying the tube fenestrations outside the thorax . in our case air leak from lung parenchymal injury usually settles spontaneously in 1 - 2 days post - operatively . alternative drainage systems or avoidance of neck drains may be considered to prevent this complication . we also emphasise on the need for careful monitoring of respiratory status in patients having corrugated neck drain after tte .
transthoracic oesophagectomy is a standard surgical procedure for oesophageal cancer . because of thoracotomy and lung handling , perioperative pulmonary complications make such procedures challenging . the issues related to respiratory complications may be predicted and managed accordingly . however , we report two cases of respiratory compromise caused due to a peculiar iatrogenic component .
steven johnson syndrome ( sjs ) is characterized by sudden onset of erosion of mucous membranes ( predominantly oral mucosa , lips , and conjunctivae ) with widespread blistering of the skin involving up to 10% of the body surface area . trimethoprim - sulfamethoxazole ( tmp / smx ) is a widely prescribed antimicrobial for the management of several uncomplicated infections . in immunocompetent persons , the adverse reactions to tmp / smx occur in approximately 1 - 3% of persons . in contrast , in hiv - infected population , a much higher incidence of adverse reactions has been reported with frequencies ranging from 40 - 80% . we report a hiv - infected man who developed sjs while receiving tmp / smx therapy . a 33-year - old male presented with complaints of intermittent low grade fever for two months , cough with expectoration for one and half month , loss of weight , and appetite for one month . the patient was afebrile with a pulse rate of 92/min , respiratory rate of 30/min and blood pressure of 120/70 mmhg . investigations revealed : hemoglobin 9.2 gm / dl , total leucocyte count 7500/cumm with an absolute lymphocyte count of 1500/cumm , platelet count of 2.1 lac / cumm and erythrocyte sedimentation rate of 76 mm at the end of 1 hr . hiv - elisa was positive . in view of the clinical presentation and investigations , a diagnosis of pneumocystis jirovecii pneumonia ( pcp ) was considered and the patient was started on tmp / smx doubles strength two tablets thrice daily . after ten days of starting tmx / smx therapy , the patient developed high grade fever , redness and pricking sensation in eyes , and burning sensation in throat . within the next 48 hours he developed blepharitis , conjunctival congestion , erythema of the eyelids , and oral mucosal erosions [ figure 1 ] . painful , erythematous , maculopapular , and vesicular lesions appeared all over the body including genitals [ figure 2 ] . there were multiple ulcerations in the buccal mucosa , floor of the mouth , and ventral surface of the tongue . the patient was hemodynamically stable with a pulse rate of 102 per / min and a blood pressure of 110/80 mmhg . the alanine aminotransferase and aspartate aminotransferase levels were 250u / l ( 10 - 40u / l ) and 155u / l ( 10 - 35u / l ) , respectively . the patient was treated aggressively with intravenous dexamethasone 6 hourly , intravenous fluids , prophylactic antibiotics , anti - allergic drugs and local treatment of lesions . re - epithelialization of the previously sloughed areas occurred and the general condition of the patient remained satisfactory . he was discharged after a month by which time the skin had healed with post - inflammatory hypopigmentation . desensitization to tmp / smx was not attempted and the patient was discharged on dapsone prophylaxis for pcp . clinical photograph showing conjunctival congestion , erythema of the eyelids , and blepharitis clinical photograph showing cutaneous lesions all over the body with oral mucosal erosions tmp / smx is commonly used for the treatment and prophylaxis of pneumocystis jirovecii pneumonia ( pcp ) in hiv - infected patients . in addition to its efficacy in preventing pcp , tmp / however , the administration of tmp / smx is hampered by the high incidence of hypersensitivity reactions in these patients . the most common adverse reactions include nausea , vomiting , anorexia , dermatological reactions such as pruritis , urticaria and less commonly steven johnson syndrome ( sjs ) , neutropenia , exfoliative dermatitis , erythema multiforme and toxic epidermal necrolysis . a pubmed search using the keywords trimethoprim - sulfamethoxazole and steven johnson syndrome and human immunodeficiency virus revealed less than ten cases being reported in literature till date . the increased incidence of adverse reactions to tmp / smx in hiv - infected patients is due to the hydroxylamine derivative of sulphamethaxazole , i.e. , sulphamethaxazole hydroxylamine . the deficiency of glutathione enzyme in hiv - infected patients results in a decreased capacity to scavenge these hydroxylamine derivatives . in vitro , it has been found that as compared to cd8 cells , purified cd4 cells are more resistant to the sulphamethaxazole hydroxylamine . in hiv infection , the depletion of cd4 cells leaves a greater proportion of circulating t - cells as the more susceptible cd8 subset . in addition , the immune dysfunction observed in hiv - infected patients , i.e. , the polyclonal b - cell activation , polyclonal gammopathy , and decreased t - suppressor cell functions leads to an exaggerated humoral immune response to smx or its metabolites . other factors such as viral load and concurrent medications may also increase cell sensitivity to reactive metabolites . it is not clear whether the adverse reactions to tmp / smx merely indicate or also induce progression of hiv disease . veenstra et al . found that patients with adverse reactions to tmp / smx have a more rapid decline in cd4 cell counts and rapid progression to aids and death . in patients with non life - threatening adverse reactions ( e.g. , fever , rash ) , rapid or slow oral desensitization to tmp / smx can be attempted . however , in patients with severe life threatening adverse reaction to tmp / smx , alternative drug like dapsone , pentamidine , or atovaquone should be given for pcp prophylaxis . those who do not tolerate or have recurrent pcp infections despite alternative therapy could be subjected to a trial of desensitization to tmp - smx under close supervision . this case illustrates a clinically important and life threatening adverse effect of tmp / smx in hiv - infected patients . due to the high incidence of such hypersensitivity reactions
trimethoprim - sulfamethoxazole ( tmp / smx ) is a widely prescribed antimicrobial for the management of several uncomplicated infections . it is commonly used for the treatment and prophylaxis of pneumocystis jirovecii pneumonia ( pcp ) in the hiv - infected population . the adverse reaction to tmp / smx is more frequent and severe in hiv - infected patients as compared to the general population . here , we report a case of stevens - johnson syndrome ( sjs ) secondary to tmp / smx . the patient had a generalized cutaneous reaction with involvement of the eyes , oral cavity , and genitals . he had elevated hepatic alanine aminotransferase and aspartate aminotransferase enzyme . tmp / smx therapy was stopped and supportive treatment was started . his condition improved after eight days of stopping tmp / smx therapy .
a study on serologic and molecular characterization of denv isolates was initiated in january 2005 at the fundao de medicina tropical do amazonas ( fmtam ) . parasite - negative patients who had clinical symptoms of malaria were invited to participate in the study . each patient received essential information and signed a consent form approved by the fmtam ethical committee . all serum samples were collected during the acute phase of illness and tested for denv infection by using 3 methods . the first method was virus culture , for which serum samples were placed on the aedes albopictus cell line c6/36 grown in leibovitz-15 medium containing 5% fetal bovine serum , followed by viral antigen identification with type - specific monoclonal antibodies in an indirect immunofluorescence assay ( 8) . the second method was detection of immunoglobulin m antibodies to denv by an elisa on serum samples from patients > 7 days after onset of symptoms ( 9 ) . pcr ( rt - pcr ) protocol on the basis of that described by lanciotti et al . briefly , viral rna was extracted by using the qiaamp viral rna mini kit ( qiagen , valencia , ca , usa ) , and reverse transcription was conducted on 5 l of extracted rna with superscript iii ( invitrogen , carlsbad , ca , usa ) and random primers . after incubation for 1 h at 50c , 2 l of each cdna was subjected to pcr amplification with d1 and d2 primers for 35 cycles consisting of 1 min at 94c , 1 min at 55c , and 1 min at 72c , and a final extension for 10 min at 72c . a second round of amplification was conducted with 10 l ( diluted 1:100 ) of the first amplicon , a mixture of type - specific reverse primers ( ts1ts4 ) , and the conserved forward primer d1 . denv-4 was detected in 3 samples ( am750 , am1041 , and am1619 ) by virus culture or rt - pcr . it was identified as a co - infecting virus with denv-3 in isolate am750 ; samples am1041 and am1619 represented single denv-4 infections ( table ) . to confirm these results , generated amplicons were cloned into a ta vector ( invitrogen ) , and > 3 colonies for each sample were sequenced in both directions by using the bigdye terminator cycle sequence kit ( applied biosystems , foster city , ca , usa ) . denv-3 and denv-4 nucleotide sequences obtained were subjected to a basic local alignment search tool ( blast ) ( www.ncbi.nlm.nih.gov/ ) analysis that used the megablast algorithm optimized for highly similar sequences . using this approach , we obtained sequences with identities ranging from 95% to 99% for denv-3 and 94% to 98% for denv-4 for isolates am750-d3 , am750-d4 , and am1619 . eu127898 ( am750-d3 ) , eu127899 ( am750-d4 ) , and eu127900 ( am1619 ) . * igm , immunoglobulin m ; m - pcr , multiplex typing pcr described by lanciotti et al . ( 10 ) ; s - pcr , single serotype - specific primer pcr ; d , dengue serotype ; blast , basic local alignment search tool ; ns , not sequenced . isolate am750 is from a person with a co - infection ; different clones were sequenced . the 3 denv-4positive samples were obtained from patients who lived and worked in manaus and reported no travel history for > 15 days before onset of symptoms . these samples were obtained during another study that identified 62 denv - positive samples from january 2005 through june 2007 ( 24 denv-2 , 35 denv-3 , and the 3 denv-4 cases in our study ) among 128 samples tested from patients in 14 municipalities in amazonas . since its introduction into the western hemisphere in 1981 , denv-4 has been associated with dengue fever and only sporadically associated with serious cases of dhf or dss ( 1 ) . a study in colombia found more dhf patients infected with denv-2 than with denv-3 or denv-4 ( 11 ) . conversely , another study showed an association of denv-4 with an epidemic of dhf that occurred in mexico in 1984 ( 12 ) . there are many host ( and perhaps viral ) factors in dengue infections that may lead to development of dhf . on the basis of the antibody - dependent enhancement hypothesis , the most important factors would be those generated by the patient s immune response upon secondary infections ( 13 ) . the 3 isolates reported in our study were from patients with no travel history , which indicates that denv-4 is present in manaus . detection of denv-4 in brazil co - circulating with other denv serotypes endemic to this country represents an increased risk for dhf or dss because many persons have been sensitized by previous dengue infections but are not protected against infection with denv-4 . the first report of denv-4 in brazil was in the state of roraima in 1982 . since that time , no other isolate of denv-4 has been reported in any part of brazil ( 14,15 ) . the resurgence of denv-4 in the amazon region of brazil most likely resulted from the proximity of brazil to denv-4endemic countries ( venezuela and colombia ) . additional genotyping studies are being conducted to verify this assumption and to obtain information on dengue epidemiology in brazil . our study documents the detection of denv-4 in manaus , amazonas , and the first isolation of this serotype in brazil in 25 years . these findings reinforce the need for continual epidemiologic studies and use of classic and molecular approaches in the surveillance of emerging or reemerging diseases .
we report dengue virus type 4 ( denv-4 ) in amazonas , brazil . this virus was isolated from serum samples of 3 patients treated at a tropical medicine reference center in manaus . all 3 cases were confirmed by serologic and molecular tests ; 1 patient was co - infected with denv-3 and denv-4 .
epigastric hernia is a type of abdominal wall hernias due to a weakness , gap or opening in the muscles or tendons of the upper abdominal wall , on the alba line between the umbilicus and the xiphoid process . the hernial sac content is usually properitoneal fat , vascular structures and , uncommonly , abdominal viscera . epigastric hernias are usually occult in obese patients , and their symptoms may mimic peptic ulcer or gallbladder disease . there is a male predominance with a male to female ration of at least 3:1 , diagnosis usually occurs in the third to fifth decade . defects of the fascia may vary in diameter from several centimeters to only a few millimeters . the larger ones usually readily reducible , whereas the smaller often became in - carcerated . operative management aims at reposition of the hernia sac contents and direct closure of the hernial opening with a continuous suture . due to high recurrence rates , tension - free hernia repair with mesh is becoming more common . even if is a common and relatively simple procedure , there is no exact protocol today on how the repair should be done . the purpose of epigastric hernia surgery is to repair the weakening area between rectus abdominis and put the hernial sac back into the abdomen . the best way to restore the anatomy of the abdominal wall in a tension free manner , the mayo technique and its alterations could not stand the test of time and it shows a recurrence rate of 20% and higher . although there is no consensus opinion , the anatomic repair without tension and without an artificial enlargement of the defect is clearly the new trend in hernia repair techniques . in 1987 lichtenstein reported on 6321 cases of inguinal herniorraphy with a tension free repair , and in 1994 stuart reemphasized that special importance in his editorial in the lancet . a newer study from brancato and coworkers in italy also states the advantage of a tension - free prosthetic repair in 16 patients with epigastric hernia ; in their work no recurrence has been recorded . they found the technique simple , safe and absolutely effective , allowing immediate rehabilitation with a low rate of complications ( figs . small epigastric hernias is usually not a medical emergency and can be healed without surgery . when the symptoms are frequent and the problem affects the quality of life of the patient , surgery is the solution . the traditional approach to incisional hernia repair usually involves a surgical approach via the vertical scar . in young woman midline vertical scar they would rather prefer not to be operated instead of having such a visible scar . techniques developed for the surgical approach to mini - abdominoplasty include the use of the transverse lower abdominal incision and the resection of excess skin above the navel . the laparoscopic access was not the first choice because it did not allow the resection of the excess of skin . we report a case of epigastric hernia repair through a transverse lower abdominal incision with the resection of excess of skin . a 37 year - old female patient was diagnosed an epigastric hernia with a little diastasis recti . patient 's symptoms were sense of discomfort , bloat , sometimes associated with dull pain increasing with cough . she had a mild laxity of the skin in the lower part of the abdomen and the position of the navel was 16 cm from the pubic symphysis . she refused classic approach to surgical hernia repair due to the visible midline vertical scar . the general surgeon asked the cooperation of a plastic surgeon who found another technique reliable to solve the functional problem with an esthetic approach combining the hernia repair with a mini - abdominoplasty hiding the scar in the bikini zone . the miniabdominoplasty technique was chosen in accord to the abdominolipoplasty classification system proposed by mejia ja and castellanos c and categorized as a type ii . preoperatory marking was done following the type ii abdominoplasty described by pontes r. because the patient had such criteria in fact this technique is suitable for females with high position of the navel . the patient was placed in the supine position with the break in the table at the patient 's waist . the lower abdominal incision was first demarcated in the pubic area 6 cm above the anterior vulvar cleft . the incision was made , the flap was elevated , hemostasis was done to avoid any bleeding . the umbilicus was detached from its aponeurotic implantation to prepare the access to the epigastric hernia . care was taken to elevate the skin flaps only as far as necessary to define the hernial defect and to find surrounding fascia of good quality . this area lacks important perforator vessels , and the tunnel was wide enough to expose the medial borders of the rectus muscles . the pannus was raised to the costal margins taking care to avoid undermining laterally to preserve the intercostal perforators to the flap . the navel was released from its insertion on the aponeurosis and left attached to the flap , as described by psillakis in his paper . alba line was opened and the hernial defect was identified , isolated and was safely reduced . the edges were dissected to free the hernial sac and identify an intact facial edge . the hernia was reduced and the posterior fascia of rectus abdominis was dissected from its adjacent structures . a polypropylene mesh was placed over the posterior rectus fascia to prevent the recurrence of the hernia . the navel was reattached 2.5 cm below its original position maintaining 13.5 cm from the pubic symphysis , using a 3/0 absorbable suture . the excess of skin was sectioned after marking the cranial border of the flap with the pitanguy marker . epigastric hernia repair through a mini - abdominoplasty incision is a reliable method to approach an abdominal wall defect with an esthetic procedure . the use of the mini - abdominoplasty approach isolates the incision from the hernial defect and repair . we found a little difficult to access to the epigastric area through the subcutaneous tunnel , but , when the defect is not too big to repair and the flaps are elevated till the xiphoid process , then it is easy to prepare the posterior fascia and to inset the polypropylene mesh . in conclusion we find this approach suitable for those people who need a surgical repair of an epigastric hernia and have such conditions:laxity of the skin in the lower part of the abdomen.high position of the navel.patients refusing midline vertical scar . written informed consent was obtained from the patient for publication of this case report and accompanying images .
the objectives of abdominal hernia repair are to restore the structural integrity of the abdominal wall . current techniques include primary closure , staged repair and the use of prosthetic materials . techniques for mini - abdominoplasty include the use of the transverse lower abdominal incision and the resection of excess skin . we report a case of epigastric hernia repair through a transverse lower abdominal incision with the resection of excess of skin . our purpose is to evaluate the results of the procedure by incorporating these aspects into an epigastric hernia repair , we found out that the procedures are made safer and the results are improved . proper indication and details of the technique are described .
there is heterogeneity in human mrna start sites ( 1 ) ; 4060% of human genes are transcribed alternatively ( 2 ) , and 49% of multi - exon transcripts are accompanied by alternative splicing of the initial exon ( 3 ) . transcription start sites might be altered in a variety of different cell types or affected by environmental conditions , such as methylation . although an extensive collection of transcription start sites for a large number of human genes is available ( 4 ) , the frequencies of individual start sites are unclear . there is a need for high - throughput technology to monitor the statistics of start site occurrences for a comprehensive understanding of the start site gene expression mechanism . microarrays are unsuitable for this purpose because of their inability to detect novel start sites . the serial analysis of gene expression ( sage ) method ( 5 ) has demonstrated its effectiveness at cataloging large quantities of expressed genes in cells or tissues from a variety of physiological , developmental and pathological states ( 611 ) . the original sage generates short ( 10 + 4 bp ) nucleotide sequences , called tags , derived from the 3 ends of transcripts ; however , typical tags are too short to be uniquely identified with their corresponding genes . this shortcoming was resolved using the longsage method ( 12 ) , a high - throughput means of profiling 21 bp tags , which are sufficiently long to be unambiguously identified with genes in most cases . however , existing sage methods are designed to monitor the 3 ends of transcripts , and the challenge was to extend the sage method so that it would be capable of capturing the novel 5 ends of transcripts and efficiently quantifying individual 5 end occurrences . recently , hashimoto et al . ( 13 ) developed such a system for human cell lines , while shiraki et al . the 5sage database stores a collection of data accumulated by using the hashimoto et al . hashimoto et al . ( 13 ) have described the details of the method , and we present a brief summary here . the method first profiles 21 bp tags by using a novel way of combining the oligo - capping method ( 15 ) , a modification of the oligo - capping method ( 16 ) and the longsage method ( 6 ) . subsequently , these 5sage tags are aligned with the human genome to locate their positions , to begin a search for neighboring mrna start sites . we found that 19 893 of 25 684 5sage tags in a human cell line , hek293 , were matched to the human genome . of the 15 448 tags that hit a locus within the human genome , 85.896.1% of the 5sage tags were assigned to within 500 to + 200 nt of the mrna start sites in the refseq , unigene ( 17 ) and dbtss ( 4 ) databases , while 1774 tags were within the introns of known genes or uncharacterized regions , indicating possible novel start sites . in the 5sage database server , users can browse transcription start sites and frequencies of individual genes by querying on the accession numbers of sequences in refseq , cluster identifiers in unigene or symbol names , such as hdac . to retrieve all the genes in the server , the word the user can impose additional conditions on the number of distinct start sites and the total frequency of 5sage tags monitored for individual genes of interest . for instance , one can look for genes by monitoring five or more distinct start sites with 10 or more 5sage tag occurrences . in response to the query clicking on each gene displays a window for browsing the transcription start sites ( figure 1 ) . the start site view initially displays the narrow , 150 bp region surrounding the transcription start site of the representation gene in refseq or unigene , while the global view presents entire structures of individual transcripts that are helpful in comprehending alternatively spliced transcripts at a glance . users can change the zoom magnification of each view independently by setting the ruler unit to an alternative base pair length . the orange vertical lines depict transcription start points ; the depth of each orange line below the axis shows the frequency of the transcription start site . the thick , green , horizontal lines are cpg islands , which are regions of 50 or more bp consisting of at least 50% g or c nucleotides . for instance , figure 1 shows the transcription start sites of neurofilament 3 ( nef3 ) . note the large number of start points detected for nef3 ; most are novel , and some start at the second or third exon . transcription start site view also lists 5sage tags , their distances from the representative start site , their frequencies and their nearest expressed sequence tags . we have performed long sage on the 3 ends of mrna in hek293 cells to validate the accuracy of our 5sage results ( 13 ) . the total frequency of 3sage tags associated with the representative gene is also displayed with the 3sage tag sequences and distances from the start site . 3sage tags are sampled independently at random , the pearson correlation coefficient between the frequencies of 5sage and 3sage tags indicates moderate similarity ( 13 ) . as on october 2004 , the 5sage database presents transcription start sites collected from human cell lines , hek293 and ramos . start site information in other human cell lines is being collected for the analysis of start point variation in different tissues , and will be made available at the same website . this work was supported in part by a grant - in - aid for scientific research on priority areas ( c ) from the ministry of education , culture , sports , science and technology of japan .
to comprehensively identify transcription start sites and the frequencies of individual mrnas in human cell libraries , a method of 5 end serial analysis of gene expression ( sage ) was developed recently , which makes it possible to collect a large amount of start site information , and subsequently , we have established a related database server called 5sage . this database displays the observed frequencies of individual 5 end sage tags and previously unknown transcription start sites in the promoter regions , introns and intergenic regions of known genes . 5sage will be useful for analyzing promoter regions and start site variation in different tissues , and is freely available at http://5sage.gi.k.u - tokyo.ac.jp/.
a total of 226 bengali medium students ( male 99 and female 127 ) , studying in class seven and eight , were taken from two urban and two rural schools of west bengal . jepqr - s developed by corulla is 48 item self - reported questionnaire having four dimensions extraversion , neuroticism , psychoticism , and lie scale each of which has 12 items . the items of the questionnaire were translated into bengali by two bilingual bengali personnel . where there were inconsistencies , both translation of the questionnaire by an expert was made and where there were any inconsistencies , the translators were consulted to get best possible solutions . internal consistency of each items of each of the four subscales of the translated jepqr - s was completed . inter - correlations between different subscales were also found . to find internal consistency of items under a subscale , item rest of test correlations were found . the internal consistency of the subscales of the translated questionnaire was found by calculating chronbach 's alpha . the statistical analysis was made with the help of statistical package for the social science ( spss ) statistics 17.0 . a total of 226 bengali medium students ( male 99 and female 127 ) , studying in class seven and eight , were taken from two urban and two rural schools of west bengal . jepqr - s developed by corulla is 48 item self - reported questionnaire having four dimensions extraversion , neuroticism , psychoticism , and lie scale each of which has 12 items . jepqr - s developed by corulla is 48 item self - reported questionnaire having four dimensions extraversion , neuroticism , psychoticism , and lie scale each of which has 12 items . the items of the questionnaire were translated into bengali by two bilingual bengali personnel . where there were inconsistencies , both the translators were consulted . also back translation of the questionnaire by an expert was made and where there were any inconsistencies , the translators were consulted to get best possible solutions . internal consistency of each items of each of the four subscales of the translated jepqr - s was completed . to find internal consistency of items under a subscale , item rest of test correlations were found . the internal consistency of the subscales of the translated questionnaire was found by calculating chronbach 's alpha . the statistical analysis was made with the help of statistical package for the social science ( spss ) statistics 17.0 . table 1 presents that the item rest of test correlations vary for the extraversion between 0.082 and 0.417 , for neuroticism between 0.127 and 0.552 , for psychoticism between 0.085 and 0.504 , and for the lie scale between 0.051 and 0.363 . item rest of test correlations alpha reliability found for the four subscales were 0.620 for extraversion , 0.663 for neuroticism , 0.604 for psychoticism , and 0.620 for lie scale . the test retest reliabilities were found 0.659 for extraversion , 0.652 for neuroticism , 0.631 for psychoticism , and 0.621 for lie scale . the present study was aimed to examine to what extent the bengali translation of the jepqr - s is functional in bengali cultured population . the results [ table 1 ] of the first stage of data analysis indicate that most of the items within a subscale have significant correlations with rest of the items . the alpha coefficients of all the four subscales recorded in this study do not cross 0.7 , the level recommended by kline , but they are very close to it and satisfactory . alpha coefficients for extraversion , neuroticism and lie scale recorded in this study are less well relative to main stream studies , perhaps , due to cultural difference , as a measuring tool like personality test is always culturally loaded . significant observation of this study is that the alpha coefficient of 0.604 for psychoticism is impressive against the historic difficulties encountered by the subscale . retest reliabilities found in this study are satisfactory and the results are very similar to the findings of eysenck and eysenck which were for 13 years boys and girls with an interval of 6 months ; extraversion 0.67 and 0.76 , neuroticism 0.72 and 0.74 , psychoticism 0.63 and 0.66 , and lie scale 0.65 and 0.66 . results relating to the main stream questionnaires provide that the dimensions of personality are not all orthogonal . the inter correlations [ table 2 ] between the dimensions of the translated jepqr - s of the present study also show that they are not all orthogonal . neuroticism and lie scale also showed negative correlation significant at the 0.05 level . on the other hand , extraversion and lie scale also neuroticism and psychoticism both showed positive correlations significant at the 0.05 level . however , there is a significant negative correlation at the 0.01 level between psychoticism and lie scale while extraversion is independent of neuroticism . on the basis of the present study , it may be concluded that the bengali version of the jepqr - s may be accepted as a functional tool for research work and educational purpose . it is further recommended to administer the bengali version of jepqr - s on a larger sample for determining the psychometric properties more accurately .
background : personality measuring instrument plays an important role in many fields of human civilization and therefore , present study was aimed to find such an instrument for bengali speaking juniors.materials and methods : bengali translation of the short - form of the revised junior eysenck personality questionnaire developed by corulla was administered on a sample of 226 bengali speaking students ( 99 boys and 127 girls ) studying in class seven and eight taken from two urban and two rural schools . internal consistency of each item under a subscale was calculated ; internal consistency of each of the four subscales of the translated questionnaire was calculated ; test retest reliability was found with an interval of 3 months and inter - correlations between different subscales were found.conclusion:the findings provided satisfactory psychometric properties of the extraversion , neuroticism , psychoticism , and lie scale .
bone scintigraphy is an important modality for the examination of various pathological conditions of the skeletal system . radionuclide bone imaging is quick , relatively inexpensive , widely available , and exquisitely sensitive and is invaluable in the diagnostic evaluation of numerous pathological conditions . these compounds accumulate rapidly in bone , and by 2 - 6 h after injection , about 50% of the injected dose is in the skeletal system . tc - methylene diphosphonate ( tc - mdp ) imaging , customarily directed toward identifying altered skeletal metabolism , has been used extensively to image a variety of extraosseous lesions , especially heterotopic ossification and metabolic , metastatic calcification . we report a rare case of accumulation of bone scintigraphy tracer in a phlebolith in a patient of adenocarcinoma of lung . a 43-year - old female patient , known case of adenocarcinoma of right lung was referred for a tc - mdp bone scan with complaint of persistent low backache since 3 months . the scan showed abnormal , focal increased tracer uptake in left pelvic cavity , not localizing to any bone of the skeleton [ figures 1a and b , arrows ] . the uptake was persistent even in post - void imaging done after adequate hydration , giving us a clue that it was not due to tracer hold up in the ureter . on single photon emission computed tomography and computed tomography ( spect - ct ) imaging , the focal tracer uptake localized to a small focus of calcification in the left pelvic cavity away from the ureter [ figure 2 , arrow ] . an additional ultrasonography of the pelvis confirmed that there was no ureteric calculus [ figure 3 ] . review of the patient 's recent contrast - enhanced computed tomography revealed a phlebolith in left iliac vein ( ct hounsfield unit : 338 ) . thus , we concluded the extraosseous tc - mdp uptake was due to phlebolith in the left iliac vein . planar bone scan ( a and b ) of a 43-year - old female patient , a known case of adenocarcinoma of right lung . focal , intense tracer uptake seen in left pelvic cavity ( arrow ) correlative single photon emission computed tomography and computed tomography fusion imaging localized this uptake to a phlebolith in left iliac vein ( arrow ) ultrasonography of the pelvis showing ureteric jets bilaterally ( arrows ) , ruling out obstruction ( calculus ) in the ureter tc - mdp , a bone scan tracer , has rapid blood clearance , excellent in vivo chemical stability , and a high bone - to - soft tissue ratio , which are ideal for bone imaging . although tc - mdp is customarily used to evaluate skeletal pathological conditions , its excellent clearance from normal soft tissues allows the detection of abnormal extraskeletal tc - mdp accumulation . it is hypothesized that tc - mdp and its analogues bind to bone by adsorption to the surface of the hydroxyapatite crystal . while in soft tissue , these pathways result in uptake of tc - mdp through what is believed to be chemisorption of the radiopharmaceutical onto the surface of the calcium salts . phleboliths are deposits which develop in the wall of a vein and are composed of calcium or lime . these are most commonly seen in the lower pelvis and sometime occur in large numbers . they are often confused with ureteric stones when seen around the urinary bladder in the pelvic cavity . however , on plain radiographs , these can be distinguished from a calculus due to the presence of a translucent center and circular or oval shape.[810 ] bone scan tracer uptake in a phlebolith has been described in past . dystrophic calcification occurs in patients with normal ca + and po4 levels and refers to ca + deposition in tissues secondary to histological disruption caused by trauma , ischemia , or cellular necrosis or in the enzymatic necrosis of fat . it is thought that calcium ion binds to phospholipids present in membrane bound vesicles , phosphatases generate phosphate groups which in turn bind to the calcium , and the cycle is repeated until local concentrations are elevated and crystals begin to form . hyaline collagen degeneration , a consequence of tissue damage , is particularly associated with ca + deposition . similar mechanism operates in other known causes of extraskeletal bone scan tracer uptake in tissue infarction , deep vein thrombosis , scars , and dermatomyositis . to conclude , we report a rare case of extraosseous uptake of bone scan tracer in a phlebolith . with growing use of integration of ct with spect , nuclear medicine physicians should become aware of such anatomical changes and variants so as to avoid misinterpretation . the awareness of the underlying pathophysiological basis of these changes and uptake of mdp in extraosseous soft tissue can significantly enhance the interpretation and diagnostic value of bone scintigraphy .
uptake in non - osseous , non - urologic tissues is occasionally found in the performance of bone scintigraphy . these uptakes reflect an atypical interaction between the radiopharmaceutical and the patient . knowledge of the rare extraskeletal uptakes and their clinical relevance helps in better interpretation of bone scans . we report an unusual occurrence of extraosseous accumulation of 99mtc - methylene diphosphonate in a phlebolith in bone scan , confirmed with the aid of photon emission computed tomography and computed tomography .
it imposes a large burden on society that is growing larger in terms of mortality or morbidity , quality - of - life and costs . resistant hypertension is defined as persistent elevation of blood pressure ( bp ) above the goal in spite of concurrent use of three to four antihypertensive agents of different classes at maximally tolerated doses including a diuretic . as defined , resistant hypertension may also include patients whose bp is controlled with the use of more than three medications . patients with resistant hypertension are at increased risk of stroke , cardiovascular ( cv ) disease , and chronic kidney disease ( ckd ) . the diagnosis of resistant hypertension requires optimal bp measurement techniques to confirm persistently elevated bp readings . pseudoresistance hypertension , including patients who lack bp control secondary to inaccurate suboptimal treatment regimen , poor adherence to antihypertensive therapy , life - style and diet , use of anti - inflammatory agents or white coat hypertension , must be excluded . ambulatory bp monitoring or home bp allows the identification of white coat effect . among the most common secondary causes of resistant hypertension in general , plasma renin activity ( pra ) is very low or undetectable in patients with primary aldosteronism . furthermore , in most patients with primary aldosteronism , the plasma aldosterone concentration is > 15 ng / dl . in general furthermore , in most patients with primary aldosteronism , the plasma aldosterone concentration is > 15 ng / dl . a plasma aldosterone level ( ng / dl)/pra ( ng / ml / h ) ratio of greater 20 is likely suggestive of primary hyperaldosteronism but further investigation is required to confirm the diagnosis . a high suspicion for primary hyperaldosteronism should be entertained for patients with following clinical history : spontaneous or unprovoked hypokalemia with renal potassium wasting , diuretics - induced hypokalemia that does not normalize after discontinuation of diuretics for at least 4 weeks and is unresponsive to angiotensin blockers and a family history of primary hyperaldosteronis . in a study of patients with resistant hypertension versus control subjects , plasma aldosterone levels , aldosterone - to - renin ratio and 24-h urinary aldosterone levels experts recommend screening patients with resistant hypertension for plasma aldosterone and 24-h urinary aldosterone levels , as well as pra , even if the serum potassium level is normal . in addition , patients with resistant hypertension should be evaluated for pheochromocytoma if they have suggestive manifestations such as episodic hypertension , palpitations and/or diaphoresis or tremor and if all else is negative , it is reasonable to begin an evaluation for renal artery stenosis . adrenal vein sampling ( avs ) is considered the gold standard for localizing aldosterone - producing adenomas in patients with primary hyperaldosteronism . most of patients with an aldosterone producing adenoma ( apa ) will have cortisol - corrected aldosterone lateralization ratios greater than 4.0 . in addition , the contralateral aldosterone to cortisol ratio is less than the inferior vena cava aldosterone to cortisol ratio in 93% of patients with surgically confirmed apa . it has been shown to have an accuracy rate as high as 96.6% and also has altered the course of clinical management in about 37.5% of patients . following the avs , an aldosterone - blocking agent can be added to test therapeutic response . avs is recommended for patients with primary aldosteronism when the computed tomography scan is normal , shows bilateral abnormalities , or shows a unilateral abnormality who would like to pursue surgical management ( unilateral adrenalectomy ) . adding a mineralocorticoid receptor antagonist , such as spironolactone or epleronone , has been shown to reduce resistant hypertension in patients with primary aldosteronism especially those with bilateral adrenal hyperplasia , which has been found to be a contributing factor in about two thirds of cases of primary aldosteronism . in this study , spironolactone reduced systolic bp by as much as 25 mm hg when added to a multidrug regimen that included a diuretic and an angiotensin converting enzyme ( ace ) inhibitor or angiotensin - receptor blockade ( arb ) and the reduction was seen in patients with and without primary aldosteronism [ table 1 ] . similar results were seen in a smaller study of patients with hypertension that was not responding to at least a dual therapy . antihypertensive drugs for management of resistant hypertension in children and adolescents studies combining an ace inhibitor with an arb have produced varied results , and one has stirred controversy . the cooperate trial showed the combination of an ace inhibitor and arb to be superior to ace - inhibitor monotherapy in slowing the progression of non - diabetic renal disease . other trials also have called into question the effectiveness and/or safety of dual renin - angiotensin system ( ras ) blockade in the treatment of hypertension associated with heart failure or cv disease . until studies demonstrate true benefit , experts recommend avoiding dual ras blockades . a recent systematic review suggested that treatment with beta - blockers improved all - cause mortality in patients with ckd and heart failure . recent studies recommends treating patients with resistant hypertension who are unresponsive to aggressive antihypertensive therapy with ablation of sympathetic nerves in the renal arteries . these studies also suggest that renal sympathetic denervation provides safe and sustained bp reduction up to 1 and 2 years . resistant htn is usually due to poor compliance , suboptimal therapy , renal vascular hypertension , or use of nsaid , oral contraceptives , steroids , and estrogen replacement therapy . primary hyperaldosteronism , renal vascular hypertension , and ckd are the most common secondary causes of resistant hypertension . a combination of ace - inhibitor , arb , plus a beta a blocker , and/or calcium channel blocker are highly effective and have an additive effect , controlling bp in up to 85% of patients . thiazide or loop diuretic is often indicated in edematous states including patients with heart failure or chronic kidney disease . renal nerve ablation is preserved for patients who fail to response to aggressive antihypertensive drug therapy .
resistant hypertension in adolescents is increasing in frequency and is increasingly recognized as having significant short- and long - term health consequences . it may be seen in up to 30% of all hypertensive patients cared for . adolescents with resistant hypertension are at higher cardiovascular ( cv ) risk due to a long history of severe hypertension complicated by other cv risk factors such as obesity . common causes of resistant hypertension include primary aldosteronism , sleep apnea , diabetes and chronic kidney disease . careful blood pressure ( bp ) measurement and thorough evaluation of patients with sustained bp elevation should make a possible early diagnosis of resistant hypertension . successful treatment requires identification and reversal of life - style factors contributing to treatment resistant and diagnosis and appropriate treatment of causes of hypertension . improved pharmacologic therapies may offer the potential for preventing or at least ameliorating early cv disease . this review highlights these and other important issues in the evaluation and management of adolescents with resistant hypertension and provides practical guidance to the practitioners involved in caring for such patients .
in a recent issue of critical care , morgan and colleagues present the results of a well - conducted systematic review and meta - analysis of the effect of statin therapy on inflammatory markers after cardiac surgery . observational evidence suggests that statin therapy may dampen the inflammatory response following exposure to a significant trigger and there is currently much interest in using statins to treat sepsis . in this context cardiopulmonary bypass ( cpb ) is appealing methodologically because it allows the study of preventive interventions [ 4 - 6 ] . fortunately for patients , mortality following cpb is low , but the methodological downside of this success is that very large trials of low risk patients ( or somewhat smaller trials of higher risk patients ) are needed to show a mortality effect . the randomised controlled trials included by morgan and colleagues measured post - operative inflammatory markers in adults receiving a statin or placebo prior to cpb . they found that statin therapy may reduce post - cpb inflammation as measured by il-6 , il-8 , c - reactive protein and tumour necrosis factor - alpha . for example , six of the eight apparently randomised studies provide no information on sequence generation and allocation concealment . three were unblinded and only two had a low risk of bias ( defined by applying the cochrane risk of bias tool ) . the median sample size was 43.5 ( range 20 to 200 ) and the confidence intervals around the mean differences in inflammatory markers for individual studies and for the summary estimates were fairly wide . other studies of inflammatory biomarkers are likely to vary widely between patients and within patients over time , suggesting that analysis of within - patient changes over time may detect differences between treatment groups with more statistical power . while the meta - analysis does not provide a definitive answer , it raises key methodological issues relevant to sepsis research in general , and to statin research in critical illness in particular . the methodological quality of many of these studies is variable and frequently the mechanisms ( at both biological and functional levels ) through which benefit are supposed to accrue were not robustly described . we agree with other authors that the logical sequence of questions to answer before performing pragmatic mortality trials should be : first , can statins theoretically beneficially modulate the immune response in these patient populations ? second , do statins beneficially ( and safely ) modulate the immune response and associated physiology ? third , does the modulated immune response translate into benefit at the level of organ function ? in this regard the critical care research community can learn much from colleagues in rheumatology , cardiology and oncology , who have explored and described mechanistic pathways - paths reliably connecting biological plausibility and effect with organ performance and then outcomes important to patients ( for example , mortality ) ; and developed reliable and validated surrogate endpoints . morgan and colleagues want to establish whether potential surrogate endpoints ( inflammatory markers ) are modulated , but herein lay the problems . second , while data from successful ' mortality ' randomised controlled trials may improve our understanding of surrogate outcomes , interventions that improve surrogate markers do not necessarily translate into improved mortality [ 12 - 14 ] . third , what is the minimum clinically important difference for a biomarker or combination of biomarkers ? how much should individual ( and aggregate ) marker levels differ between groups to translate into clinical benefit ? lastly , what if ' good ' markers are also suppressed and , indeed , how much inflammation is good for you ? it is clear that in this relatively new study area the knowledge space is already occupied by poor studies and potentially unreliable data . we would like to encourage investigators to design studies that are methodologically robust and provide reliable mechanistic data . icu researchers should work towards developing valid surrogate endpoints to allow robust and reliable translational research , although we acknowledge that previous success at improving organ performance has not always improved outcomes [ 12 - 14 ] . once validated , these surrogate endpoints should be used to establish the biological effectiveness of new treatments ( and probably some existing ones ) before moving on to pragmatic studies using mortality as outcome measure . without demonstrating first biological and then functional ( for example , organ performance ) effectiveness , we run the risk of wrongly adding statins to the wasteland of icu pharmacotherapy . on the positive side , the pleiotropic effects of statins and extensive experience with these agents in cardiology mean that we may be less likely to fall into a trap constructed of an insufficient understating of mechanisms combined with a single - target therapy .
statin therapy may prevent an excessive inflammatory response after cardiopulmonary bypass for cardiac surgery . in a recent issue of critical care , morgan and colleagues present data from a well - conducted systematic review and meta - analysis of randomised controlled trials using inflammatory markers as primary outcome measure . they find that pre - operative statin therapy , compared with placebo , may reduce various post - operative markers of systemic inflammation ( il-6 , il-8 , c - reactive protein , tumour necrosis factor - alpha ) . their ability to make definitive conclusions is limited , however , by the suboptimal methodological quality of the primary studies . their review suggests that icu researchers should focus on developing valid surrogate markers and use these to accurately describe the mechanisms and effectiveness of novel therapies before proceeding to large pragmatic trials using mortality as primary outcome .
pleomorphic carcinoma ( pc ) is a rare malignant lung tumor characterized by dual - cell components including spindle or giant cells as well as epithelial cells . pc has been reported to have a more aggressive clinical course and a poorer survival compared to other histological types of non - small cell lung cancer ( nsclc ) [ 1 , 2 ] . we report the case of a patient with pc and a single brain metastasis that achieved long - term recurrence - free survival following treatment with combined modality therapy . a 71-year - old male presented with aphasia and muscle weakness of the left side of his body . he was suspected of having a central nervous system disorder and underwent head brain ct that showed a 50 50 mm mass lesion in the right frontal lobe ( fig . his chest x - ray showed a mass shadow with a cavity in the right - middle lung field ( fig . the chest ct also showed a 70 68 mm mass with a cavity in the right lower lobe ( fig . the level of tumor markers like carcinoembryonic antigen , cytokeratin 19 fragments and pro - gastrin - releasing peptide were not elevated . based on these clinical findings , the patient was diagnosed with lung cancer with a single brain metastasis . craniotomy was performed because the patient 's central nervous system 's symptoms were severe and his eastern cooperative oncology group performance status was zero . the brain tumor in the right frontal lobe was completely removed , and the pathological diagnosis was confirmed as sarcomatoid pattern carcinoma . a ct - guided percutaneous lung biopsy proved that the pathological diagnosis of the right lung mass was spindle cell carcinoma . the patient was therefore diagnosed as having pleomorphic carcinoma of the lung with a single brain metastasis ( clinical t3n0m1b stage iv ) . after craniotomy , the patient underwent 6 courses of chemotherapy ( carboplatin auc 4 on day 1 and gemcitabine 800 mg / m on days 1 and 8 , repeated every 3 weeks ) . after chemotherapy , the chest ct showed a reduced nodular shadow ( 62 50 mm ) ( fig . 1d ) , while f - fluorodeoxyglucose positron emission tomography showed no uptake in the lymph nodes ( data not shown ) . the patient achieved a partial response by preoperative chemotherapy and underwent a right - lower lobectomy , partial resection of a right s2 segment , parietal pleurectomy , and mediastinal lymph node dissection through a posterolateral thoracotomy . this surgery was required because of the possibility of invasion into the right upper lobe and the parietal pleura . microscopically large , spindle - shaped atypical cells had sarcomatously proliferated into most of the tumor portions ( fig . immunohistochemically , the mib-1 index of the specimen obtained by ct - guided percutaneous lung biopsy before preoperative chemotherapy was high at 60% ( fig . 2c ) , whereas that of the resected tumor was low at 10% ( fig . pc is rare , accounting for 0.31.0% of all lung cancers ; it is defined as a poorly differentiated nsclc . in a recent large study on patients with stage i iii lung cancer , the overall 5-year survival rate and mean survival time of these patients were reported to be significantly lower and shorter than that of patients with other types of nsclc ( 36.7 vs. 59.4% , and 22.8 vs. 96 months , respectively ) . therefore , patients with pc have a significantly poorer outcome than patients with other types of nsclc . in the present case , the patient underwent preoperative chemotherapy after craniotomy . the national comprehensive cancer network recommends neoadjuvant chemotherapy combined with surgical treatment or surgical treatment combined with adjuvant chemotherapy in nsclc cases with brain metastasis ( tnm category t12 , n01 , t3n0 if the metastasis can be controlled ) . although previous papers showed that pc had a poor response to chemotherapy regimens , there is a report indicating the efficacy of chemotherapy for pc . in our case , gemcitabine - based chemotherapy was effective and suggested that this treatment was potentially useful for pc . the following discussion includes the possible reasons why the present case achieved long - term survival . . showed the following 4 factors that contributed to the poor prognosis for a pc survival : ( 1 ) tumor size > 5 cm , ( 2 ) stage greater than i at presentation , ( 3 ) presence of distant metastases , and ( 4 ) lymph node metastases . in this case , the absence of lymph node metastasis was a good prognostic factor . these 4 factors only include the tnm classifications and are common prognostic factors for pc and other nsclcs . however , patients with pc have poorer outcomes than those with other nsclcs , and it is therefore necessary to investigate specific prognostic factors for pc . in the present case , the mib-1 index of the immunohistochemical specimen obtained by ct - guided percutaneous lung biopsy before preoperative chemotherapy was high at 60% ( fig . 2c ) , although it was reduced to 10% in the resected tumor ( fig . mib-1 is present during all active phases of the cell cycle ( g1 , s , g2 , and mitosis ) , but absent in resting cells ( g0 ) . this property makes it an excellent marker for determining the so - called growth fraction of a given cell population . in patients with pc , a low mib-1 index ( < 35% ) moreover , there is a report in which the mib-1 index was significantly lower after gemcitabine - based chemotherapy and exerted a true antiproliferative effect on tumors in vivo . reduced proliferation with gemcitabine - based chemotherapy may partly be because of increased apoptosis in actively proliferating cells so that the residual population would be enriched by mib-1-negative cells . in our case , the mib-1 index was reduced by gemcitabine - based chemotherapy , which may have led to long - term disease - free survival . the mib-1 index may therefore be a useful biomarker of the response to chemotherapy , with the reduction in the index following preoperative chemotherapy representing a response marker that reflects the proliferation of tumor cells . the changes in the mib-1 index caused by preoperative chemotherapy may therefore provide useful information for deciding the regimen for adjuvant chemotherapy .
pleomorphic carcinoma ( pc ) is a rare malignant lung tumor with a poorer prognosis compared to other histological types of non - small cell lung cancer . however , several recent immunohistochemical studies revealed that a low mib-1 index is a good prognostic marker in patients with pc . we report the case of a patient with pc and a single brain metastasis that achieved long - term recurrence - free survival following treatment with combined modality therapy . in this case , the mib-1 index was reduced by gemcitabine - based chemotherapy , which may have led to long - term disease - free survival . the mib-1 index may be a useful biomarker for the response to chemotherapy .
rituximab is a relatively novel medication for immunomodulation in current clinical practice . with its use becoming fairly widespread and newer indications being approved at a fast rate it is also imperative to watch for any adverse reactions , which may need monitoring parameters during therapy a 54-year - old man was diagnosed with limited form of wegener 's granulomatosis 1 year before the current hospitalization . he had failed therapy with corticosteroids and was recommended rituximab for resistant disease state . besides lung and upper airway involvement , he had no other organ involvement from wegener 's disease . five days after receiving the infusion he developed postural dizziness and had near syncopal episodes , which led to hospital admission . he denied chest pain , shortness of breath , fever , palpitations , and any rash . vital signs on admission revealed hypotension with a blood pressure of 80/40 mmhg , pulse 75 beats / min , respiratory rate 20/min , and temperature 98f . electrocardiogram ( ecg ) showed a new onset left bundle branch block ( lbbb ) [ figure 1c ] as compared to his prior ecg performed a month back before the current admission [ figure 1a ] . in view of new lbbb , an urgent transthoracic echocardiogram was performed revealing normal left ventricular systolic function and absence of wall motion abnormalities . patient was admitted to intensive care unit and was treated with intravenous fluids and inotropic agents for hypotension . contrast - enhanced cardiac magnetic resonance imaging was conducted to rule out focal myocarditis as a cause of the new onset lbbb ; and failed to show any abnormality . on day 2 of admission , telemetry showed intermittent lbbb [ figure 1b ] and eventually the lbbb was replaced by a narrow qrs complex [ figure 1d ] with only supportive treatment . patient remained hemodynamically stable off inotropic agents and was discharged home and is doing well on follow up . telemetric strip new onset left bundle branch block ( lbbb ) intermittent sinus beats with lbbb and finally resolution of lbbb rituximab , the first monoclonal antibody approved by the united states food and drug administration ( fda ) for the treatment of malignant disease , is being used to treat a wide variety of conditions in modern practice , including non - hodgkin 's lymphoma , wegener 's granulomatosis , rheumatoid arthritis , thrombotic thrombocytopenic purpura , among many others . currently , four adverse effects that warrant a " black box " warning in the package insert are known : infusion reactions , tumor lysis syndrome , mucocutaneous reactions , and progressive multifocal leukoencephalopathy . it is also known to promote severe orthostatic hypotension often associated with allergic symptoms , urticaria , bronchospasm , and angioedema . most adverse events occur during or after the first infusion of rituximab , and the number and severity of adverse events decreases with subsequent infusions . as with any novel therapy , new adverse reactions are being noted with increasing time and usage . in a multicenter phase ii study conducted to assess the toxicity and response rates to rituximab in b - cell malignancies that express cd20 , 10 out of 131 patients developed an arrhythmia with treatment . these included bradycardia ( n = 3 ) , atrial fibrillation ( n = 2 ) , and nonspecified arrhythmia or tachyarrhythmia ( n = 5 ) . one other patient had palpitations , and another was noted to have premature ventricular complexes . in most cases , ventricular tachycardia during the infusion of rituximab and prompt disappearance after discontinuation of infusion was first reported in 2005 . complete atrioventricular block induced by rituximab monotherapy in an 83-year - old has also been described in literature . another recent report of polymorphic ventricular tachycardia causing syncope during initial infusion of rituximab raises concerns of arrhythmogenic side effects of this medication . despite these reports a study to determine the maximum tolerated infusion rate of rituximab with special emphasis on monitoring the effect of rituximab on cardiac function concluded with confirmation of lack of cardiotoxic effect of a fast infusion rate . however , we could not come across any reported case of lbbb associated with rituximab infusion . increased ventricular dysfunction after rituximab infusion has been seen which is attributed to growth of reticulin fiber in cardiac myocytes . reticulin deposition may have an effect on conduction system causing rhythm abnormalities and interventricular conduction delays as seen in our patient . it is also hypothesized that the drug affects conduction by inhibiting the calcium - ion channel properties of the cd20 antigen , which could lead to formation of early after depolarizations . still the mechanism by which rituximab may cause arrhythmias is presently unclear and needs more research . also , it would be prudent to keep patients on electrocardiac monitoring during drug infusions to monitor for possible conduction abnormalities , especially the initial infusions .
rituximab ( a monoclonal antibody directed against cd 20 ) therapy can be acutely complicated by infusion reactions and cardiac arrhythmia on rare occasions . we report the first case of a new onset left bundle branch block ( lbbb ) after rituximab therapy for wegener 's vasculitis .
gallstone ileus causing small bowel obstruction is a well - document phenomenon ; however , gallstones causing large bowel obstruction are much rarer and almost always secondary to existing bowel pathology . we report what we believe to be the first case of large bowel obstruction caused by an impacted gallstone without primary pathology distal to the stone and discuss potential management options from the literature to rationalize our choice of a defunctioning loop colostomy with the stone left in situ . a 92-year male , with no significant co - morbidities apart from peptic ulcers , was admitted with 6 days of obstination . on examination blood showed features of dehydration and plain abdominal x - ray demonstrated both small and large bowel dilatation , but no obvious causal pathology . a contrast - enhanced computed tomography scan revealed a 2.5-cm partially calcified gallstone impacted at the descending colon - sigmoid junction ( fig . 1 ) . 2 ) , with a further smaller gallstone in the caecum ( fig . 3 ) . the offending gallstone could be seen incidentally in the gallbladder on imaging 3 years previously ( fig . figure 1:large gallstone impacted in the descending colon - sigmoid junction , causing large bowel obstruction proximally . figure 2:cholecysto - colic fistula , with a thick - walled , dilated , gallbladder . figure 3:ct reconstruction showing the impacted gallstone and a smaller incidental gallstone in the caecum . large gallstone impacted in the descending colon - sigmoid junction , causing large bowel obstruction proximally . the patient was initially resuscitated , although he could not tolerate an naso - gastric tube . this failed due to a large amount of faecal matter distal to the stone , despite several phosphate enemas . in light of worsening abdominal distension and pain , the patient underwent a lower midline laparotomy where the hugely dilated small bowel was decompressed via the stomach , the descending colon mobilized and the diagnosis confirmed . there was indeed no primary pathology distal to stone ; however , the stone could not be disimpacted , and the overlying colonic wall was viable with no evidence of ischaemia . considering the patient 's age and physiological reserve , the duration of large bowel obstruction , high morbidity and mortality associated with emergency surgery , and the fear of leak following a colotomy , an on - table decision was made to perform a proximal defunctioning loop transverse colostomy to relieve the obstruction . the cholecysto - colic fistula was left untouched , and the impacted stone left in situ . he will be brought back to the clinic to discuss the options with regard to removal of stone and reversal of his colostomy . gallstone ileus is not an uncommon phenomenon ; however , large bowel obstruction due to stone impaction is rarely seen due to the large calibre of the colon , with few case reports in the literature [ 2 , 3 ] . the site of impaction is usually the sigmoid [ 3 , 4 ] , with retention of the stone as a result of a stricture from diverticular disease or previous radiotherapy . the stone is thought to enter the colon via a cholecysto - colic fistula , developing during a bout of acute cholecystitis affecting the hepatic flexure . cases usually present with features of large bowel obstruction ; however , colonic gallstones may also present atypically with non - specific symptoms such as diarrhoea , cholangitis or bleeding from the fistula tract . the diagnosis is best made on computed tomography ( ct ) . a trial of conservative treatment may be attempted , and colonoscopy with and without lithotripsy has been successful in relieving obstruction ; however , surgery may be needed if the stone can not be retrieved and will not pass spontaneously . there is no definite consensus as to how a gallstone impacted in the colon should be treated . the options are a combination procedure of enterolithotomy , cholecystectomy and fistulectomy , or enterolithotomy alone with or without a covering loop colostomy to resolve the immediate obstruction [ 2 , 3 ] . the former leads to relief of the obstruction as well as preventing further stone formation and episodes of cholangitis ; however , as these patients are invariably elderly and bearing significant co - morbidities , a minimalistic approach is often preferable . in our case a laparotomy was undertaken as endoscopy failed and the patient 's pain and distension continued to worsen . at laparotomy the small bowel was decompressed to view the colon and to rule out further contributing pathology . a defunctioning transverse loop colostomy was performed without removing the stone to avert the immediate crisis , while minimizing morbidity and mortality . this would have hastened patients post - operative recovery , while resulting in a similar functional outcome . we believe this to be the first instance that an impacted gallstone has been left in situ post - operatively with a proximal loop colostomy used for symptomatic relief . there are various options now for retrieval of the stone , should this be necessary . the gallstone could be accessed directly via the colostomy or anus , and it may be possible to use lithotripsy to shatter and remove the stone without resorting to surgery . on the other hand , it could be left alone , as although fears remain that such a stone might erode through the wall or cause a localized abscess , decompression of the bowel has significantly reduced the chance of pressure necrosis and related complications .
we present a rare case of large bowel obstruction secondary to colonic gallstones in a frail nonagenarian . uniquely , the stone was impacted in the descending colon - sigmoid junction , in the absence of underlying bowel pathology distal to the stone . in light of worsening pain and distension after failed endoscopic treatment , the patient was treated with an emergency laparotomy . after an on - table dilemma , a proximal defunctioning loop colostomy was fashioned and the stone left in situ , with the eventual fate of the stone currently undecided . we also discuss alternative treatment options and explain the thought processes that lead to our decision .
hailey - hailey disease ( hhd ) or familial benign chronic pemphigus first described in 1939 , is a rare autosomal dominant genodermatosis , which presents with recurrent vesicles , erythematous crusts and erosions in the areas subjected to friction , including , the neck and intertriginous areas . abnormalities in the tonofilament desmosome keratin complex results in marked acantholysis , giving the histological appearance of a dilapidated brick wall , which is pathognomonic of the disease . ultra violet b rays the treatment is often not satisfactory and hence , various modalities of treatment have been tried . we describe a case of hhd with a positive family history , in which various treatment modalities were tried without any benefit but responded well to thalidomide . a 30-year - old unmarried male presented with a history of recurrent painful fluid and pus filled lesions followed by painful erosions over the neck , axillae , trunk and groins of 15 years duration exacerbated over the past 10 - 15 days . he gave family history of similar disease in his father , paternal grandmother and paternal uncles . before coming to us , he had been treated with dapsone following which he developed agranulocytosis and dapsone was discontinued . several other treatment modalities including oral and topical antibiotics , corticosteroids were tried but resulted in only partial or temporary improvement . large macerated plaques studded with foul - smelling pus along with erosions , fissures , and crusts were present on the anterior and the lateral aspect of the neck , axillae , back , lower abdomen , pubic area , gluteal cleft , groins , upper medial thighs and scrotum [ figure 1 ] . erosions and crusts were seen on the glans with purulent foul smelling discharge in the prepucial sac , sparing rest of the penis . the skin biopsy specimen for histopathological reconfirmation of the diagnosis revealed focal suprabasilar clefting , few acantholytic cells , intercellular edema with dilapidated brick wall appearance which confirmed our diagnosis [ figures 2 and 3 ] . macerated plaques with erosions and crusts over the scrotum , glans penis and upper medial thighs focal suprabasal clefting and lacunae ( h and e , 10 ) suprabasalar clefting with few acantholytic cells , intercellular edema and dilapidated brick wall appearance ( h and e , 40 ) patient was started on intravenous ( iv ) betamethasone 4 mg , 8 h which was tapered off with the introduction of 40 mg of oral prednisolone , iv ceftriaxone 1 mg twice daily and later iv piperacillin - tazobactam based on pus culture and sensitivity reports along with a combination of fusidic acid and betamethasone for topical application . he continued to develop new lesions with the deterioration of his general condition and was febrile throughout . in spite of controlling the secondary infection , the vesicular eruptions persisted . therefore , he was started on oral thalidomide 100 mg three times a day and a combination of betamethasone and gentamycin for topical application . marked improvement was seen within a week of starting thalidomide [ figure 4 ] and hence we discontinued corticosteroid after rapid tapering of its dose and continued treating him with oral thalidomide 100 mg three times a day for one more week , which was then tapered off in 3 - 4 weeks and maintained on 50 mg daily . after 6 months of receiving 50 mg thalidomide , the drug was stopped in view of no recurrence of the lesions . he has improved well and comes to us for regular follow - up . mucosal involvement is unusual in hhd and has been very rarely reported and can involve oral , conjunctival , vaginal , or in very rare instances esophageal mucosa . our patient had oral , pharyngeal , and genital mucosal involvement , which were resistant to the symptomatic treatment . apart from the symptomatic therapy , drugs such as corticosteroids , dapsone , etretinate , methotrexate , cyclosporine , topical 5-fluorouracil , topical vitamin d analogs , topical zinc oxide , alefacept , psoralen plus ultraviolet a therapy and photodynamic therapy have been tried with varying results . as hhd is often recalcitrant to treatment , further options including invasive methods such as grenz ray therapy , carbon dioxide laser abrasion , erbium : yag laser ablation , dermabrasion , electron beam therapy , botulinum toxin and full - thickness excision of affected skin with repair by split - thickness grafting have been reported as useful in treatment of hhd . in the study conducted by galimberti et al . hhd is linked to mutations in the calcium - transporting atpase type 2c member 1 gene encoding the human secretory pathway ca / mn atpase . topical gentamicin was found to be very effective in inducing remission in a hhd patient carrying a premature stop mutation . it may be an important alternative in the treatment of hhd in patients not responding to the other modalities of treatment or when the conventional drugs are contraindicated . mucosal involvement is unusual in hailey - hailey disease ( hhd ) and has been very rarely reported . our patient had oral , pharyngeal , and genital mucosal involvement.thalidomide may be an important alternative in the treatment of hhd in patients not responding to the other modalities of treatment or when the conventional drugs are contraindicated . mucosal involvement is unusual in hailey - hailey disease ( hhd ) and has been very rarely reported . thalidomide may be an important alternative in the treatment of hhd in patients not responding to the other modalities of treatment or when the conventional drugs are contraindicated .
familial benign chronic pemphigus or hailey - hailey disease ( hhd ) is a rare autosomal dominant disorder characterized by the development of recurrent blisters and erosions in the intertriginous areas . various topical and systemic treatment options include corticosteroids , topical 5-fluorouracil , topical vitamin d analogs , topical zinc oxide , dapsone , psoralen plus ultraviolet a , systemic retinoids , cyclosporine , methotrexate , and photodynamic therapy . in recalcitrant cases , further options including , invasive methods such as grenz ray therapy , carbon dioxide laser abrasion , and erbium : yag laser ablation , dermabrasion , electron beam therapy , botulinum toxin , and full - thickness excision of affected skin with repair by split - thickness grafting have been reported as useful in treatment of hhd . we describe a case of hhd who was treated with several treatment modalities including antibiotics , corticosteroids , and dapsone earlier and when presented to us had a severe recalcitrant disease . thalidomide , as a modality of treatment has been successfully used in few cases earlier . our patient responded well to thalidomide .
udp - glcua ( ammonium salt ; > 98% purity ) , and nad ( sodium salt ; 98% purity ) were obtained from sigma aldrich . purified preparations of wild - type hugdh and e161q mutant were obtained using reported procedures . both enzymes were produced as fusion proteins containing an n - terminal extension , which comprised a solubility enhancement tag , a streptavidin tag , and a tobacco etch virus protease cleavage site . the n - terminal extension was then removed , and the enzymes were further purified by gel filtration and anion - exchange chromatography . a 50 mm potassium phosphate buffer at pd 5.9 , 7.0 , 7.9 or 8.8 was used . starting concentrations were 15 mm nad , 2 mm udp - glc and 0.38 m wild - type hugdh or 15 m e161q . after addition of all components , the reactions were incubated at 25 c for 16 h. for determination of the solvent isotope effect , enzymatic conversions in h2o or d2o were performed in a beckman coulter du800 spectrophotometer using a 50 mm potassium phosphate buffer with ph / pd 7.5 . reaction velocity was measured by nadh absorption ( = 340 nm ) . all spectra were recorded on a bruker drx-600 avance spectrometer ( bruker , rheinstetten , germany ) at 600.13 mhz ( h ) . the h nmr spectra were measured at 298.2 k with presaturation ( 1.0 s ) and acquisition of 32k data points . after zero filling to 64k data points , spectra were performed with a range of 7200 hz . chemical shifts were referenced to external acetone ( h = 2.225 ppm ) . the reactions were directly made in a 5 mm high precision nmr sample tube ( promochem , wesel , germany ) to measure in situ h nmr spectra . samples contained 2 mm udp - glc , 15 mm nad , and 0.084 m wild - type hugdh or 33 m e161q , as well as 50 mm potassium phosphate buffer in d2o ( 0.70 ml , 99.9% d , pd 7.5 or 8.3 ) . 12 h in the magnet by recording up to 64 h nmr spectra in regular intervals . the topspin 3.0 software from bruker was used for processing the nmr spectra after data acquisition . for quantitative analysis of the nmr data , a correction value was subtracted from the integral values of each proton to account for background noise . udp - glcua ( ammonium salt ; > 98% purity ) , and nad ( sodium salt ; 98% purity ) were obtained from sigma aldrich . purified preparations of wild - type hugdh and e161q mutant were obtained using reported procedures . both enzymes were produced as fusion proteins containing an n - terminal extension , which comprised a solubility enhancement tag , a streptavidin tag , and a tobacco etch virus protease cleavage site . the n - terminal extension was then removed , and the enzymes were further purified by gel filtration and anion - exchange chromatography . full details of the purification protocol are given elsewhere . enzymatic assays were performed in 1 ml of d2o . a 50 mm potassium phosphate buffer at pd 5.9 , 7.0 , 7.9 or 8.8 was used . starting concentrations were 15 mm nad , 2 mm udp - glc and 0.38 m wild - type hugdh or 15 m e161q . after addition of all components , the reactions were incubated at 25 c for 16 h. for determination of the solvent isotope effect , enzymatic conversions in h2o or d2o were performed in a beckman coulter du800 spectrophotometer using a 50 mm potassium phosphate buffer with ph / pd 7.5 . reaction velocity was measured by nadh absorption ( = 340 nm ) . all spectra were recorded on a bruker drx-600 avance spectrometer ( bruker , rheinstetten , germany ) at 600.13 mhz ( h ) . the h nmr spectra were measured at 298.2 k with presaturation ( 1.0 s ) and acquisition of 32k data points . after zero filling to 64k data points , chemical shifts were referenced to external acetone ( h = 2.225 ppm ) . the reactions were directly made in a 5 mm high precision nmr sample tube ( promochem , wesel , germany ) to measure in situ h nmr spectra . samples contained 2 mm udp - glc , 15 mm nad , and 0.084 m wild - type hugdh or 33 m e161q , as well as 50 mm potassium phosphate buffer in d2o ( 0.70 ml , 99.9% d , pd 7.5 or 8.3 ) . 12 h in the magnet by recording up to 64 h nmr spectra in regular intervals . the topspin 3.0 software from bruker was used for processing the nmr spectra after data acquisition . for quantitative analysis of the nmr data , a correction value was subtracted from the integral values of each proton to account for background noise .
graphical abstract
the surgical indication is difficult to establish in some cases ( more than 40% ) . acute abdominal pain in women may be a manifestation of a disorder of various organs . in addition to surgical , urological , orthopedic , neurological , and psychogenic problems , gynecological causes play a major role . incarcerated herniation of the bowel , occurring through a defect in the broad ligament , is extremely rare . we herein report the 2 cases of women with intestinal obstruction , neither of whom had a previous history of any surgical pelvic treatment . the laparoscopic approach revealed in both cases an incarceration of the small bowel herniated through a defect in the broad ligament . without the antecedent of uterine surgery , delivery trauma , and pelvic diseases , congenital abnormalities are considered the causes of these defects although the real mechanism is unknown . this cause should therefore be considered in the differential diagnosis of female patients presenting with an intestinal obstruction who have not had a prior laparotomy . a 38-year - old gravida 1 , para 1 , woman was admitted to our hospital with a chief complaint of lower abdominal colicky pain and vomiting of 1 day 's duration . her relevant past medical history included a laparoscopic appendectomy performed 7 years previously and a diagnosis , in the same setting , of endometriosis , since then successfully treated with medical therapy . laboratory findings showed slight leucocytosis , and a plain abdominal radiograph showed some loops of dilated small bowel with air fluid levels . ultrasonography confirmed the small bowel obstruction and disclosed the presence of fluid in the pelvis . with the diagnosis of intestinal obstruction of unknown origin because abdominal distension was moderate , a laparoscopic approach was performed with 3 trocars , instruments , and optics 5-mm in diameter . a 30-cm long ileal loop was found to have herniated through a defect in the right broad ligament . a 23-cm defect was noted , and it was closed by using a purse - string 2/0 monofilament absorbable suture . the postoperative course was uneventful , with bowel movement and hospital discharge on the first and fourth postoperative days , respectively . a 55-year - old gravida 2 , para 2 woman was admitted to our department with symptoms of colicky pain and nausea but no vomiting for the previous 2 days . most notable , she had no history of prior abdominal or pelvic surgery or pelvic inflammatory disease . on examination , as with the previous case , a plain abdominal radiograph showed some loops of dilated small bowel with no air fluid levels . ultrasonography confirmed the small bowel obstruction and disclosed the presence of fluid in the pelvis . due to a diagnosis of intestinal obstruction of unknown origin , the patient was taken into the operating room and a laparoscopic approach was used in the same manner as the case described above . a 20-cm long ileal loop was found to have herniated through a fenestration of the left broad ligament . as above , the small bowel , once reduced , appeared vital , and a defect of 13 cm was seen and closed by using a purse - string monofilament absorbable 2/0 suture . the postoperative course was uneventful , with bowel movement and hospital discharge on the first and third postoperative days , respectively . surgeons treating a patient with lower abdominal pain of uncertain origin are caught between the extremes of conservative and operative treatment . after clinical examination and ultrasonography , exploratory laparoscopy has been shown by several studies to solve this therapeutic dilemma . a corollary to the application of diagnostic laparoscopy is the potential for therapeutic manipulation during the same setting . internal hernias represent only 0.4% to 4.1% of all small bowel incarcerations , and of these , the hernias through a defect of the broad ligament represent only 4% to 7% . regarding their cause , they are thought to be either congenital or acquired . the congenital ones are a consequence of a spontaneous rupture of congenital cystic structures within the broad ligament reminiscent of the mesonephric or mllerian ducts . this hypothesis could account for those patients who are nulliparous or have never undergone pelvic surgery . an acquired defect may result from either operative trauma , pregnancy and birth trauma , or prior pelvic inflammatory disease . they have therefore been frequently reported in multiparous patients , patients who have undergone a surgical pelvic procedure , and patients with a history of salpingitis or endometriosis . one of our patients had only a history of multiple pregnancies and deliveries , and the other patient with endometriosis had an appendectomy . a classification of broad ligament defects has been proposed based on their anatomical position : type 1 defect , which occurs throughout the entire broad ligament ; type 2 , which occurs throughout the mesosalpinx and the mesovarium ; and type 3 , which occurs throughout the meso - ligamentum teres . plain film radiography because of its low sensitivity and specificity helps little in the diagnosis of most causes of abdominal pain due to gynecological disorders . other radiological investigations , such as either ultrasonography or ct scan , or both used together , are expensive and not possible to perform in all hospital situations , 24 hours a day . diagnostic laparoscopy , with an accuracy of more than 90% has been demonstrated to be superior to other diagnostic tools and may lead to the correction of an erroneous preoperative diagnosis in up to 40% of patients , or it may be used to exclude other pathologies . a corollary to the application of diagnostic laparoscopy is the potential for therapeutic manipulation during the same setting , which has been reported in the literature to be more than 80% . the goal of surgical treatment includes reduction of the intestinal loop ( its resection , if needed ) , and direct closure of the opening to avoid any recurrence . only one report exists in literature , to the best of our knowledge , of a pathology totally diagnosed and treated by using laparos - copy . for more than 10 years , we have been using laparoscopy routinely in both scheduled and emergency situations , if no absolute contraindication to the technique is present . in both cases reported herein , the laparoscopic approach was possible to perform due to the mild intestinal gas distension . intestinal resection was not necessary , and we closed the opening of the broad ligament with a purse - string absorbable suture . early diagnosis and , if possible , therapeutic laparoscopy is cost - effective in many scenarios dealing with managing nonspecific acute ( low ) abdominal pain , especially in women of reproductive age . thus , the following is perfectly applicable to the 2 cases we have report herein : both were rare gynecological pathologies causing small bowel obstruction that were diagnosed and treated in the same setting with good results and perceptions of the patients . we do think that laparoscopy is not an alternative to physical examination and conventional noninvasive diagnostic methods in any acute abdominal situation . however , it must be considered an effective option in patients in whom these methods fail , especially as surgeons become more experienced and skilled .
two cases of internal herniation through a defect in the broad ligament of the uterus are described . both were successfully treated laparoscopically . this rare condition should be borne in mind when a middle - aged woman presents with colicky lower abdominal pain . the cause is unknown , but both congenital and acquired origins have been proposed . as far as emergency situations are concerned , laparoscopy has proven to be both a diagnostic and a therapeutic tool .
we describe a case of pseudotumor cerebri in a young man developing 4 years post - traumatic brain injury ( tbi ) . this is a rare clinical scenario and delayed presentation following tbi with chronic headaches should raise this possibility . detailed clinical history and examination with appropriate investigations must be performed to diagnose this condition . a 29-year - old male was admitted to our unit with head injury following a road traffic accident . his glasgow coma score was 15/15 at admission and had no neurological deficits on examination . ct brain showed small right frontal extradural hematoma with associated pneumocranium , right orbital roof , and cribriform plate fracture . a repeat ct brain scan revealed an increase in the size of the extradural hematoma following which he underwent a frontoparietal craniotomy and evacuation of the extradural hematoma . he recovered well postoperatively and was discharged home . since discharge , he experienced intermittent frontotemporal headache and worsening of short - term memory . five months later , he developed a right - sided facial swelling and erythema , with frontal headache , fever , and episodes of vomiting . removal of infected bone flap was performed , and staphylococcus aureus was isolated from the bone flap . his headache persisted following treatment , and he had no signs suggestive of raised intracranial pressure . he remained well for the next 12 months apart from episodes of headaches on and off . a lumbar puncture was performed after imaging and was noted to have an elevated csf pressure of 20 mmhg and a diagnosis of pseudotumor cerebri was made . he underwent a lumbar - peritoneal ( lp ) shunt and postoperatively his symptoms improved transiently over the next few weeks . he had gained 9 kg of weight over the course of 4 years .two months subsequent to lp shunt insertion , he presented with worsening headaches and a new lumbar puncture showed an opening pressure of 25 mmhg , and closing pressure of 8 mmhg with no evidence of csf infection . ct brain revealed no evidence of increase intracranial pressure , and a right ventriculo peritoneal ( vp ) shunt was inserted . his headache persisted following vp shunt insertion and subsequent to this he underwent bitemporal decompression ( craniectomy ) in 3 weeks time with the removal of lp shunt [ figure 1 ] . he had two further vp shunt blockages and he developed a slit ventricle syndrome despite the shunt malfunction [ figure 2 ] . a ct venogram was performed 1 year later , which demonstrated a stenosis of the left transverse sinus and sigmoid sinus with hypoplastic right transverse and sigmoid sinuses . the cerebral venous pressure was measured with significant left transverse sinus stenosis , and pressure gradient of 2612 mmhg , > 50% gradient . this was managed nonoperatively , and it was found not to contribute to his on - going symptoms . he was also commenced on acetazolamide for the management of pseudotumor cerebri . ct brain demonstrating bitemporal decompressive craniectomy ct brain with vp shunt in situ and slit - ventricle with an underlying shunt malfunction pseudotumor cerebri is more commonly seen in young obese women than men . in a study by durcan et al . determining the incidence of pesuedotumor cerebri in iowa and louisiana , it was noted that obesity increased the incidence in both males and females with a overall female - to - male ratio of 8:1 . pseudotumor cerebri is a relatively common neurologic illness and may be an important preventable cause of blindness in obese young women . pseudotumor cerebri usually presents in adults with transient visual obscuration and blurred vision in contrast to development of diplopia in the pediatric age group . various clinical conditions and factors are associated with development of pseudotumor cerebri . in our case we focus on the possible etiology following tbi and have attempted to outline the possible causes for the development of pseudotumor cerebri . the question then arises as to why all the patients with tbi do not develop pseudotumor cerebri . the mechanism of csf flow dynamics alteration following central nervous system infection and cerebral blood flow alteration is usually seen during the period of insult to the brain and transient alteration in the physiology is a reversible phenomenon depending on the severity of head injury . the pathological process reverts to normal and very rarely , as in this case , may lead to the development of pseudotumor cerebri . possible etiologies contributing to pseudotumor cerebri mastoiditis and subsequent venous thrombosis is a known to cause pseudotumor cerebri , and there was no evidence of sinus thrombosis on imaging in our case . the bone flap infection may have altered the cerebral venous drainage and subsequently caused changes in the csf slow dynamics . the patient had gained weight following the head injury , and weight gain and obesity are important etiological factors in the development of pseudotumor cerebri . the management was aimed at preventing the possibility of optic nerve damage and blindness in benign intracranial hypertension ( bih ) , vp shunt , and lp shunt are the surgical options available for pseudotumor cerebri and our patients visual symptoms got better following csf diversion procedures . we experienced the difficulty in managing the patient in the later stage due to shunt dysfunctions with associated slit - ventricle syndrome . this procedure was resorted to , as a last option for controlling headaches and it is known to be effective in symptomatic control in the some group of patients described in the literature . the prognosis for vision in most patients with pseudotumor cerebri is excellent ; however , visual loss , which is the only serious complication , may occur either early or late in the course of the disease . when a post - head injury patient presents with history of chronic headache with visual disturbance , pseudotumor cerebri should be considered a strong possibility . neuroimaging in the form of brain ct , mri , and mr venogram must be considered to rule out vascular pathology and a diagnostic lumbar puncture is recommended . csf flow diversion techniques must be adopted to treat this condition , and these may require long - term follow - up .
we describe a case of pseudotumor cerebri in a young man developing 4 years post - traumatic brain injury ( tbi ) . a 29-year - old man was admitted after sustaining a fall with headache , and no clinical deficits were noted on examination . ct brain demonstrated an extradural hematoma . this was successfully evacuated after his symptomatic worsening . following this , he developed bone flap infection and had the infected bone flap removed . he developed chronic mild - to - moderate headache following these procedures , which failed to respond to medical treatment . pseudotumor cerebri was diagnosed . a lumboperitoneal ( lp ) shunt , ventriculo - peritoneal ( vp ) shunt , and bitemporal craniectomy were performed as a part of management at different stages . post - tbi patients may present with chronic headache and in such circumstances , a possibility of pseudotumor cerebri must be considered . investigations should include neuroimaging in the form of mri / mrv and fundoscopy to look for papilledema . management in the form of csf flow diversion techniques ( vp and lp shunt ) with medical management results in good clinical outcomes
skin metastasis from a thyroid carcinoma is rarely a presenting feature of an underlying malignancy . subcutaneous metastasis from differentiated thyroid carcinoma ( dtc ) is a rare manifestation of disseminated disease . some of the authors believe that follicular carcinoma of the thyroid has a higher propensity to metastasize to the skin , followed by papillary carcinoma , then anaplastic carcinoma and finally medullary carcinoma . others believe that papillary carcinoma is the most common thyroid carcinoma metastasizing to the skin . all agree that the scalp is the most common site of thyroid carcinoma skin metastases . there are no previous reports of positron emission tomography / computed tomography ( pet / ct ) in patients with loin metastasis from an unknown primary and identified as papillary cancer thyroid . this was a case report of a 45-year - old male patient who presented with subcuataneous swelling in the left loin , which on biopsy showed metastatic papillary cancer . pet / ct showed an intense uptake in the subcutaneous soft - tissue lesion in the left loin [ figure 1 ] . a diagnosis of primary papillary carcinoma of thyroid with subcutaneous loin metastasis was made and he was advised total thyroidectomy and excision of metastasis . whole body fluorodeoxyglucose - positron emission tomography/ computed tomography ( pet / ct ) maximum intensity projection image ( a ) axial ct ( b ) pet ( c ) fused pet / ct ( d ) showed a intense uptake in the subcutaneous soft tissue lesion in the left loin axial ct ( a ) pet ( b ) fused pet / ct ( c ) showed a intense uptake in the right lobe thyroid nodule cutaneous metastasis from dtc is also a rare manifestation of thyroid cancer . a review on roughly 60 cases of dtc skin metastases , which have been documented in the literatures , stated that pc has a greater preponderance for skin metastases dermal lesions typically present as slowly growing erythematous or purple plaques or nodules , usually on the scalp , face , or neck . this may relate to local vascular factors essential for the highly complex nature of metastases . pet / ct is very useful in identifying unknown primary cancer from the metastatic lesions . there are reports of pet / ct in identifying occult papillary cancer in a thyroglossal cyst . however , 18 fluorine - fluorodeoxyglucose - pet / ct has a role in 131i - whole body scan negative patients with elevated thyroglobulin . there are reports of pet / ct in identifying muscle and scalp metastasis from a papillary thyroid cancer . this is the first case of identifying primary papillary cancer with subcutaneous metastasis in pet / ct . recognizing and understanding the clinical findings
differentiated thyroid cancer frequently metastasizes but generally spreads to regional cervical lymph nodes and , in advanced cases , to the lungs and/or skeleton . metastases to the skin / subcutaneous tissue are rare . we report 45-year - old male patient presented with a loin swelling which on biopsy showed a papillary carcinoma and referred for fluorodeoxyglucose - positron emission tomography / computed tomography ( fdg pet / ct ) to find out the primary disease . pet / ct showed abnormal fdg uptake within a loin metastasis and right lobe thyroid nodule . fine - needle aspiration from nodule showed papillary carcinoma . because thyroid cancer can rarely metastasize to the skin , attention should be given to that region during interpretation of the images . he was advised total thyroidectomy and metastasis excision .
ectopia cordis is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity . ectopia cordis may occur as an isolated malformation or it may be associated with body wall defects that affect the thorax , abdomen , or both . with the advances in the medical field and surgical technique , a 24-year - old , unbooked primigravida delivered spontaneously , a 34-week , male stillborn weighing 1,870 g at kasturba hospital , mgims , sevagram . there was no history of intake of any teratogens or exposure to unusual environment in antenatal period . an anterior thoracoabdominal defect with extrathoracic heart , a cleft sternum , and omphalocele were recognized at birth . the physical examination revealed an exposed heart totally outside of the thoracic cavity without pericardium protection . the abdominal wall defect that caused evisceration of liver , stomach , and the intestines [ figure 1 ] . other abnormal features included asymmetrical face with medial epicanthal folds , low set ears , micrognathia , asymmetrical bossing of the skull , and high arched palate . ectopia cordis is a very rare anomaly with an estimated prevalence of 0.079/10,000 births and may occur more frequently in females . it is related to the malformation of the anterior wall of the thorax , with an extrathoracic location of the heart . in 1958 , cantrell described this syndrome , which occurs sporadically , with variable degrees of expression . ectopia cordis can be classified into five types : 1 ) cervical , in which the heart is located in the neck with sternum that is usually intact ; 2 ) thoracocervical , in which the heart is partially in the cervical region , but the upper portion of the sternum is split ; 3 ) thoracic , in which the sternum is completely split or absent , and the heart lies partially or completely outside the thorax ; 4 ) thoracoabdominal , which usually accompanies cantrell 's syndrome ; and 5 ) abdominal , in which the heart passes through a defect in the diaphragm to enter the abdominal cavity . our case had thoracoabdominal type ectopia cordis in which the bifid sternum , extrathoracic heart , absence of parietal pericardium , and an omphalocele was present . the formation of the thoracic and abdominal walls is complete in the 9 week of pregnancy and of the heart in the 8 week . complete or incomplete failure of midline fusion at this embryonic stage can result in a variety of disorders ranging from isolated ectopia cordis to complete ventral evisceration . ventricular septal defect , atrial septal defect , tetralogy of fallot , and diverticulum of the ventricle are the most commonly encountered heart lesions . ectopia cordis has also been reported with other congenital anomalies such as abdominal wall defects , cranial and facial malformations , cleft lip and palate , anencephaly , hydrocephaly , neural tube defects , pulmonary hypoplasia , genitourinary malformation , gastrointestinal defect , and chromosomal abnormalities . the defect of the abdominal wall can range from simple diastasis to huge omphaloceles with bowel , liver , and heart . the ectopic heart may either simply bulge out of the chest or be entirely out of the chest . the diagnosis has been made as early as 17 weeks , but in some cases complicated by oligohydramnios , these cases may be missed entirely . the differential diagnosis includes isolated thoracic ectopia cordis , amniotic band syndrome , and body stalk anomalies . the key features for distinguishing these conditions is the position of abdominal wall defect in relation to the umbilical cord insertion , eviscerated organs , the presence or absence of membranes or bands , and associated anomalies . omphalocele in cantrell 's pentalogy usually involves a midline defect at the umbilical cord insertion . an eccentric large lateral defect and adherence of the placenta to the defect the presence of an unexplained ventral wall defect along with extremity deformity with an adherent band suggests amniotic band syndrome . the prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity . in view of the poor prognosis , the prognosis depends on the degree of the intracardiac involvement and associated malformations , as well as the degree to which the heart is exposed . attempts at surgical correction are already widely performed , with immediate covering of the heart and exposed abdominal contents using silastic prosthesis being recommended . additionally , a complete evaluation and correction of the intracardiac defects should be performed before closing the abdominal wall . in conclusion , ectopia cordis with omphalocele is a rare congenital malformation from fatal to nonfatal , therefore it must be adequately evaluated for appropriate prenatal and postnatal management .
a rare congenital defect in fusion of the anterior chest wall resulting in an extrathoracic location of the heart . cantrell 's pentalogy is a congenital anomaly resulting from embryologic development defect and consists of the following : a deficiency of the anterior diaphragm , a midline supraumbilical abdominal wall defect , a defect in the diaphragmatic pericardium , congenital intracardiac abnormalities , and a defect of the lower sternum . here we report a rare case of ectopic cordis with omphalocele .
during the past two centuries , the anatomical variations of the axilla have been described in both textbook of human anatomy and more recently in those of operative surgery . a muscle extending from the latissimus dorsi to the pectoralis major muscle has been called axillary arch or langer 's axillary arch . this occurs in at least 7% of different populations but may not always be clinically apparent . among the muscles the usual lesion is absence of the sternocostal portion , with or without absence of the pectoralis minor muscle . in a period of one year , we identified two patients ( 4% ) with axillary arch and one patient ( 2% ) with absent pectoralis major and minor muscle among fifty subjects which undergoing axillary dissection for breast cancer surgery . the anatomy of axilla regarding muscular variations was studied in 50 patients who had an axillary dissection for the staging and treatment of invasive primary breast cancer over one year . the axillary vein was identified and all fatty and lymphatic tissue was removed inferior to the axillary vein , between the anterior border of latissimus dorsi muscle laterally and the lateral border of the pectoralis minor muscle ( level of first rib ) medially . during the procedure , two individuals with axillary arch muscle and one individual with absent pectoralis major and minor muscles were identified . of the 50 patients , 3 had a variation from the anatomy described in the standard textbooks of anatomy and operative surgery . there were two patients who had an abnormal band of muscle arising from the latissimus dorsi muscle and crossed the axilla medially towards pectoralis major muscle , pectoralis minor muscle and the coracoid process , without interruption by any type of tendinous fibres . axillary arch ( aa ) muscle crossing anteriorly over right axillary vein ( av ) there was left sided absence of pectoralis major and minor muscles in a 45 years old woman operated for left sided carcinoma of breast [ figure 2 ] . there was little interest in ramsay 's description until langer , in 1846 , described the muscle more accurately and it became known as the embryological derivation of langer 's arch remains unknown , but the most reliable theory supports its origin from the panniculus carnosus , which is an embryologic remnant of skin - associated musculature , lying at the junction between the superficial fascia and the subcutaneous fat . in lower mammals the panniculus carnosus is highly developed to form the pectoral group of muscles . however , in man it has regressed because its functional importance decreased during evolution in favour of wide upper limb mobility . langer 's arch is usually asymptomatic and its main importance is the confusion it can cause during routine axillary surgery for breast cancer . an axillary arch may be palpable in living subjects and should be borne in mind during clinical examination of the axilla as it may be mistaken for a tumor . the presence of axillary arch can impede adequate exposure of the true axillary fat and in particular may limit access to the lower lateral group of lymph nodes , thus resulting in an incomplete clearance of the axilla . because of its close proximity with neurovascular and lymphatic structures within the axilla , as the axillary arch crosses the vessels and nerves , it may present with axillary vein obstruction . the axillary arch may lead the surgeon one level above the axillary vein and as a result the neurovascular bundle of the axilla may be injured . the pectoral musculature is derived from dorsal limb bud masses which arise from myoblasts that migrate out of last five cervical and first thoracic myotomes into developing limb buds during fifth week of development . the pectoral muscles assume their final form through a combination of migration , fusion and apoptosis of muscle cell precursors . absence of one or more skeletal muscles is more common than is generally recognised ; common examples are the sternocostal head of the pectoralis major , the palmaris longus , trapezius , serratus anterior and quadrates femoris . usually only a single muscle is absent on one side of the body , or only part of the muscle fails to develop . occasionally the same muscle or muscles may be absent on both sides of the body . these structures fail to develop in the embryo.the muscles develop partly , fail to attach to the bone and subsequently atrophy.the premuscle mass , which in normal development goes to form the pectoralis minor and two portions of the pectoralis major , fails to differentiate into its separate parts.in one study , pectoralis major was absent in three of 15,000 cases and in another study , the muscle was absent in five of 54,000 cases . on average , the premuscle mass , which in normal development goes to form the pectoralis minor and two portions of the pectoralis major , fails to differentiate into its separate parts . paraskevas george noted that anomalies of the pectoralis major muscle are of prominent interest for plastic surgeons because that muscle is harvested during total , segmental or turn over flap graft removal for coverage of major sternal wound infections after cardiac surgery , breast reconstruction , or local mediastinal wounds and may serve as treatment for a paralytic elbow . furthermore , the pectoralis minor muscle is useful as a free flap in cases of facial palsy . clinical detection of this muscle is difficult ; however , it is possible to detect the presence of the axillary arch on performing computed tomography scan or magnetic resonance imaging of the axillary region . caution should be exercised while performing fnac , core needle or tru cut biopsy of breast lesions in patients with poland syndrome . the procedure should be preferably performed under image guidance in such patients in order to minimize the risk of complication of pneumothorax . the reported incidence of this complication varies between 3 in 100 and 1 in 10,000 . when present , axillary arch should always be accurately identified and formally divided to allow adequate exposure of axillary contents in order to achieve a complete lymphatic dissection . axillary arch can easily cause difficulty for the inexperienced surgeon if , by following the band , the dissection is carried higher than normal , into the region of the axillary artery and brachial plexus . the defects of pectorals usually cause little or no functional disability and often go unnoticed by the patients or relatives . however an understanding of the spectrum and complexity of this anatomical variation may be of benefit to the surgeon and pathologists while performing fnac , core needle or tru cut biopsy . absence of these muscles may increase the chances of direct spread of cancer breast through chest wall into cavity , which can worsen the prognosis .
aim : the present study was conducted to detect the musculature variations during axillary dissection for breast cancer surgery.methods:the anatomy of axilla regarding muscular variations was studied in 50 patients who had an axillary dissection for the staging and treatment of invasive primary breast cancer over one year.results:in a period of one year , two patients ( 4% ) with axillary arch and one patient ( 2% ) with absent pectoralis major and minor muscles among fifty patients undergoing axillary surgery for breast cancer were identified.conclusions:axillary arch when present should always be identified and formally divided to allow adequate exposure of axillary contents , in order to achieve a complete lymphatic dissection . complete absence of pectoralis major and minor muscles precludes the insertion of breast implants and worsens the prognosis of breast cancer .
vestibular schwannoma ( vs ) usually arises within the internal auditory canal ( iac ) from either the superior or inferior vestibular nerve . these tumors usually grow very slowly with the growth rate 0.66 to 2 mm per year6,7 ) . with time , they cause erosion of iac . the location of tumor in iac was closely related in erosion of iac , tumor that arise laterally along the course of eighth nerve could tend to cause varying degree of erosion before they extend into cerebellopontine angel ( cpa ) cistern4 ) . almost all vs usually widens iac only and could not invade the temporal bone beyond the confines of the bone of iac . in this report , we describe a patient without previously known bone disease who presented with aggressive temporal bone invasion of unilateral vs. a 51-year - old male presented with progressive hearing loss of last 4 years and dizziness of few months duration . he did not have a history of a head trauma or of any brain surgery . on admission , neurological and physical examination revealed left side deafness , no facial weakness , or no other low cranial nerve deficit like loss of gag reflex , uvular deviation , tongue deviation . the patient did not have any known systemic disease of bone and family history or physical characteristics of neurofibromatosis . his blood profile , including his serum calcium , phosphorus , and alkaline phosphates , was normal . ct scan showed that the tumor mass had destructed iac completely and temporal bone to petrous apex , grown around the cochlea , reached the carotid artery in its petrous portion , extended to jugular foramen ( fig . we noticed pneumatized area of opposite temporal bone corresponded to eroded area by tumor ( fig . the tumor was a 43.83 cm sized , heterogeneous lobulated , well - defined mass with destruction of iac and extended to cpa cistern . the tumor was confined to posterior fossa and not extended to meckel 's cave ( fig . the combined translabyrinthine with retrosigmoid suboccipital approach was used for the resection of the tumor . at the first time of surgery , we removed the tumor piece by piece within temporal area with translabyrinthine approach , but we could not find facial nerve with this approach . so we performed additional retrosigmoid suboccipiral approach , and it allowed the exposure the lateral recess area easily and origin site of facial nerve from brain stem . after detection of facial nerve with intraoperative facial nerve monitoring , we dissected facial nerve from proximal to distal area . the dissection of the facial nerve within the temporal bone was very difficult , because the facial nerve was severely adhered to the tumor mass ( fig . the tumor was extended to jugular foramen by destruction of superior wall of jugular foramen . the consistency of temporal bone was felt to be normal and the colors and the consistency of the tumor were typical of vs. we successfully achieved total removal of tumor and preserved facial nerve in continuity . the postoperative course was uneventful with normal facial nerve function and the vertigo was resolved . the patient was good condition after 18 months of follow - up without cranial nerve dysfunction . he also did not show any evidence of the recurrence or residual tumor in postoperative mri performed at 15 months of follow up . vs typically originates lateral to the glial - schwann junction , and this point is usually located within the iac , and thus can erode the iac . but the mechanism of widening of iac by a vs still unknown . takada et al.5 ) hypothesized that the mechanism involved in the destruction of iac is as follows : 1 ) the repeated strokes of the tumor caused by brain pulsation gradually eroded the bony wall of iac ( especially , that of the posterior wall ) . the apparently less destruction of iac in cases with mostly cystic large tumors might be due to the restriction of this pulsatile movement . 2 ) increased cerebrospinal fluid pressure on the site had some influence on the mechanism of bone erosion as also does solidity of the tumor . this case presented with aggressive pattern of invasion of temporal bone , which is more extensive than expected in case of unilateral vs. temporal bone destruction over iac in vs is very rare . to our knowledge feghali and kantrowitz2 ) reported 4 cases of temporal invasion of unilateral vs , in which one cases had experienced previous schwannoma surgery . they suggested that the invasion to temporal bone occurred through two mechanism in growth of vs. one of them was that the destruction of bone in the petrous apex can actually follow the pneumatized tracts when the cortex of the iac was eroded . the second mechanism was that a thick membranous scar had " trapped " and confined the tumor within the temporal bone and separated it from the recurrent tumor in the cpa . we examined various factors related in the excessive erosion of temporal bone in this patient . in this case , the patient showed no signs of osteoporosis and no abnormal laboratory findings including serum level of calcium , phosphorus and alkaline phosphatase . histologic findings demonstrated typical nature of benign schwannoma , and there were not excessive unusual vascularity nor any malignant features . if cortex of iac was eroded , the tumor could grow into the temporal bone through the pneumatized area . but we can not determine why this unilateral tumor first eroded cortex of iac and destructed temporal bone to the petrous apex before growing along the area of least resistance toward the cpa cistern . trigeminal schwannoma usually widen meckel 's cave and extend into middle fossa and posterior fossa , may destruct petrous apex and grow into temporal bone . mri showed that the tumor was confined to posterior fossa and not extended to meckel 's cave . and tympanic segment is the most frequently involved3 ) , so temporal bone might be destructed . the patient presented no facial palsy , no facial sensory change , and no facial pain . the most common surgical approaches for vs is the translabyrinthine or suboccipital approaches1 ) . because of hearing loss and mass in temporal bone , we performed combined translabyrinthine with retrosigmoid suboccipital approach to detect the facial nerve earlier and remove the tumor in temporal bone . the facial nerve was severely compressed and irregularly distorted by tumor , bony edges , and crests along the invaded petrous apex . temporal bone destruction beyond the cortex of iac in vs is very rare , which cause facial nerve compressed and distorted severely . during operation , it is very difficult to dissect and preserve the facial nerve because of uneven erosion of temporal bone .
vestibular schwannoma ( vs ) usually present the widening of internal auditory canal ( iac ) , and these bony changes are typically limited to iac , not extend to temporal bone . temporal bone invasion by vs is extremely rare . we report 51-year - old man who revealed temporal bone destruction beyond iac by unilateral vs. the bony destruction extended anteriorly to the carotid canal and inferiorly to the jugular foramen . on histopathologic examination , the tumor showed typical benign schwannoma and did not show any unusual vascularity or malignant feature . facial nerve was severely compressed and distorted by tumor , which unevenly eroded temporal bone in surgical field . vestibular schwannoma with atypical invasion of temporal bone can be successfully treated with combined translabyrinthine and lateral suboccipiral approach without facial nerve dysfunction . early detection and careful dissection of facial nerve with intraoperative monitoring should be considered during operation due to severe adhesion and distortion of facial nerve by tumor and eroded temporal bone .
left ventricular thrombus ( lvt ) is one of the most common complications of myocardial infarction ( mi ) . the incidence of lvt in patients with acute anterior mis in the prethrombolytic era is ranged from 20% to 40% and with a non anterior acute myocardial infarction ( ami ) and the risk of lvt was < 5% . although controversial , in the contemporary era of routine early revascularization and more aggressive anticoagulation , the incidence of lvt complicating as an anterior ami is likely reduced and is currently estimated at 5 - 15% . the risk factors for the development of lvt are consistently irrespective of infarct treatment and include large infarct size , severe apical akinesia , or dyskinesia left ventricular ( lv ) aneurysm , and anterior mi . two - dimensional transthoracic echocardiography is the imaging modality used most often for assessing the presence , shape , and size of an lv mural thrombus with an excellent specificity of 85 - 90% and sensitivity of 95% . here , we report a case of 40-year - old male patient who is presented in emergency department with the complaint of substernal chest pain radiating to left arm and back associated with sweating for the last 1 day , there was no history of dyspnea , palpitation , any limb weakness , or any history suggestive of transient ischemic attack and peripheral embolization . his 12-lead electrocardiogram was suggestive of inferior wall mi [ figure 1 ] . his total leukocyte and eosinophil counts were normal , prothrombin time / international normalized ratio and activated partial thromboplastin time were normal , and other tests for hypercoagulable states were planned for the follow - up evaluation . a transthoracic echocardiogram was performed bedside which revealed hypokinesia of basal , mid inferior , and inferoseptal wall [ figure 2 and video 1 ] . surprisingly , it also revealed a mobile mass which is the most probably thrombus attached to hypokinetic inferobasal septum just near the lv outflow tract ( lvot ) [ figure 3 and video 2 ] . considering the possibility that this mass was high risk for embolism , we planned for coronary angiogram followed by the surgical extraction of possible thrombus . however , unfortunately , despite the proper counseling and explanation of the risk of embolism , the patient refused for further intervention and got discharged against medical advice . small arrows showing hypokinesia of basal inferior septum , large arrow indicates left ventricle probable thrombus apical four chambers view thrombi formation at basal interventricular septum near lvot is extremely rare as it is a region of high - velocity blood flow . traditionally , the causes of lvt formation after acute st - segment elevation mi include segmental dysfunction of the infracted myocardium resulting in the stasis of blood , endocardial tissue inflammation that provides a thrombogenic surface and a hypercoagulable state . the higher mortality has been reported in patients with lv thrombi after infarction , especially when these develop within the first 48 h after infarction . the treatment for cardiac mass is a prompt surgical resection of the mass with the patients placed on cardiopulmonary bypass . therefore , better understanding of the circumstances in which lv thrombosis occurs may influence the patient management . in our case , thrombus formed at the very unusual site and could lead to dreaded complication for the patient . the possible etiology of thrombus formation , in our case , is hypokinesia of basal septum due to mi , but the presence of a subtle septal rupture which is not detectable on echocardiography could not be ruled out .
left ventricular ( lv ) thrombus formation is a frequent complication in patients with acute anterior myocardial infarction ( mi ) . its incidence is lower with inferior wall mi . risk factors for the development of lv thrombus are consistently irrespective of infarct treatment and include large infarct size , severe apical akinesia or dyskinesia lv aneurysm , and anterior mi .
bronchial atresia resulting from a localized defect in normal bronchopulmonary embryogenesis is a rare disease . it can produce emphysematous changes in the affected pulmonary segment or lobe with or without dyspnea and/or episodic pulmonary infection . we herein report a case of bronchial atresia associated with pneumothorax which was successfully treated with lobectomy . a 32-yr - old korean male patient was admitted to inha university hospital , incheon , korea due to dyspnea and right chest pain for 1 day . on physical examination , the breath sound in the right lung fields was decreased without shifting of the maximal point of the cardiac impulse . he had a history of the right pneumothorax one and a half years ago , which was treated with tube thoracostomy . the posteroanterior chest radiograph revealed increased radiolucency along with overinflated lung parenchyma and sparse vasculature in the upper half of the right lung . the chest ct taken in the state of full expansion of the right lung after tube thoracostomy showed a branching soft tissue density in the region of the posterior segment of the right upper lobe . the orifice of the posterior segmental bronchus was visualized but the orifice of the subsegmental branch of the posterior segmental bronchus could not be visualized separate from the origin ( fig . 2 ) . there were no endobronchial lesions in the bronchial tree on the bronchoscope and each orifice of the segmental bronchi of the right lung was seen normal . his forced expiratory volume at 1-sec ( fev1 ) was 3.49 l ( 91% ) and forced vital capacity ( fvc ) was 4.57 l ( 100% ) . there was a localized emphysematous change in the posterior segmental area of the right upper lobe with the apical pleural adhesion and the remaining lungs were normal . the pathological findings of the resected right upper lobe showed overinflation of the posterior segment . there were no obstructed lesions of the orifices of the three segmental bronchi of the right upper lobe . however , one of the subsegmental branch of the posterior segmental bronchus was obstructed and there was a 2.51.51.5 cm sized cystic mass containing brownish mucus material at the distal portion of the obstructed subsegmental bronchus . microscopically the distal air spaces of the atretic segmental bronchus showed overinflation only . however the foci of the subpleural bullae in the overinflated segment were observed ( fig . bronchial atresia is a rare disease , which develops due to the failure of embryogenesis of the segmental or lobar bronchial tree . since the first report of bronchial atresia by ramsay et al . in 1953 , there have been many illustrations and reports about bronchial atresia ( 1 - 8 ) . according to the reports , most of the patients with bronchial atresia were asymptomatic and it was diagnosed incidentally in the second or third decade of life on routine chest radiography ( 2 - 4 ) . the common radiographic findings of bronchial atresia are hyperinflation of the involved lung parenchyma and collapse of the affected lobe with a radioopaque extrahilar mass with mucocele in patients who had pulmonary symptoms such as fever , cough , and shortness of breath due to recurrent pulmonary infection or overinflation of the involved lung parenchyme ( 3 , 4 , 6 , 8) . however , bronchial atresia associated with pneumothorax is extremely rare in spite of overinflation of the involved lung parenchyma . we report a case of bronchial atresia in the subsegmental branch of the posterior segmental bronchus of the right upper lobe associated with mucocele and recurrent spontaneous pneumothorax , which was treated with right upper lobectomy . the resected specimen showed atresia of the subsegmental branch of the posterior segmental bronchus of the right upper lobe with hyperinflated lung with subpleural bullae . the cause of pneumothorax was thought to be due to the rupture of the bulla in the hyperinflated lung segment . the exact mechanism of bulla formation of the affected lung parenchyma could not be proved . however , we think the bullous change have been resulted from mechanical stress to the alveoli wall from hyperinflation of the lung parenchyma distal to the atretic bronchus due to aeration by check - valve mechnanism through the collateral channels of the interalveolar pores of kohn and bronchoalveolar channels of lambert .
a 32-yr - old male patient with recurrent pneumothorax associated with bronchial atresia of the subsegmental branch of the posterior segmental bronchus of the right upper lobe was successfully treated with right upper lobectomy . before surgery , the bronchial atresia with pneumothorax was suspected on the chest radiograph and ct scans , which showed the findings of bronchocele with localized hyperinflation of the right upper lobe . the examination of surgical specimen from the resected right upper lobe suggests that the cause of the recurrent pneumothorax was the rupture of the subpleural bullae in the hyperinflated lung segment distal to the atretic bronchus .
aspergillosis is the most common fungal infection of the paranasal sinuses and it usually occurs unilaterally in maxillary sinus . on the other hand , transverse maxillary deficiency ( tmd ) is one of the most common skeletal dysplasias observed in clinical practice . it can be treated with several techniques such as slow orthodontic expansion , rapid palatal expansion , and surgically assisted rapid palatal expansion ( sarpe ) , and the recommended technique for adult patients is sarpe because of limited osteogenic activity of palatal suture . the use of sarpe to treat tmd decreases adverse effects of orthodontic expansion such as lateral tipping of posterior teeth , extrusion , periodontal membrane compression , buccal root resorption , alveolar bone bending , fenestration of the buccal cortex , palatal tissue necrosis , inability to open the midpalatal suture , pain , and instability of the expansion . although sarpe is a relatively easy and an effective operation , complication possibility due to technical sensitivity of surgical approach and patient 's systemic condition must be considered . in this case report , perforation - related fistula formation on inferior meatus and unilateral aspergillosis infection in maxillary sinus associated with sarpe , treated with functional endoscopic sinus surgery ( fess ) , were evaluated . a 32-year - old systemically healthy woman with tmd was referred from the orthodontics department . the patient was operated under general anesthesia with nasal endotracheal intubation . before the surgical procedure , expansion device was activated . following the full - thickness sulcular incision on the deepest level of the vestibular sulcus incision , the lateral surface of the maxilla was exposed with subperiosteal dissection . nasal mucosa was elevated from the lateral nasal wall without any perforation , and bilateral corticotomy starting from the pyriform aperture to the pterygomaxillary fissure was performed . the medial walls of maxillary sinuses were separated bilaterally using guided osteotomes , and the palatal suture was separated using sharp chisel osteotome . the incision was closed primarily , and anterior nasal packs ( merocel standard dressing ) were applied . the patient was discharged on the same day , and the 1 week of healing period was uneventful . the patient consulted an ent specialist and computed tomography revealed that the left middle meatus was congested with radiopaque substance [ figure 1 ] . besides , endoscopic sinus examination showed that there was pus drainage from maxillary sinus to inferior meatus through fistula [ figure 2 ] , and augmented amoxicillin with clavulanic acid ( augmentin , 1 g ) was prescribed for 2 weeks to control sinusitis . computed tomography imaging of congested ostium and radiopaque appearance defluxion from fistula after 2 weeks , a granulation tissue in the maxillary sinus was observed during the endoscopic examination [ figure 3 ] , and fess was performed for decontamination of the maxillary sinus and correction of ostium function . augmented amoxicillin with clavulanic acid ( augmentin , 1 g ) was prescribed for 2 weeks again after fess , and histopathological examination of the substance removed from the sinus revealed aspergillosis [ figure 4a c ] . granulation formation ( a ) middle meatal antrostomy , ( b ) removal of substance located in the maxillary sinus , ( c ) postoperative appearance of the middle meatus after 3 weeks , it was observed that the ostium was functioning and there was no sign of aspergillosis . in 3 months correction of tmd in adult patients is more challenging because of changes in osseous articulations of the maxilla with adjoining bones . procedures described for the correction of tmd have conventionally been grouped into two categories as segmenting the maxilla during the le fort 1 osteotomy in a widened transverse dimension and sarpe . however , sarpe has become a common procedure and it has been considered as the procedure which has the lowest morbidity incidence , especially when compared with other orthognathic surgery procedures . sarpe allows clinicians to achieve satisfactory maxillary expansion in a skeletally mature patient and it decreases adverse effects of orthodontic expansion . although sarpe is a relatively simple procedure among other orthognathic surgery techniques , complications related with surgical technique and systemic condition of the patient must be considered . in this case , fistula formation between the maxillary sinus and the inferior meatus occurred during osteotomy step of sarpe procedure , and the alteration of the sinus ventilation was complicated by the presence of aspergillosis . it is well - known data that the use of wide broad spectrum antibiotics in a long period can cause fungal infection . the middle meatus dysfunction and the operation trauma are other reasons of aspergillosis formation in this case . although potential association between aspergillosis and sarpe was not described before in literature , it must be noted that sarpe causes perforation and fistula formation between the nasal cavity and maxillary sinuses . despite the frequent use of the procedure , only limited data on the prevalence of postoperative complications after sarpe are available in literature . on the other hand , some severe and unusual complications have been reported . it should be noted that fistula formation and meatus dysfunction related with sarpe operation cause fungal infection development .
surgically assisted rapid palatal expansion ( sarpe ) is one of the most common orthognathic surgery operations for the treatment of maxillary transverse deficiencies . although this operation is considered technically simple and has low complication rate , predisposing factors can complicate the postoperative period . in this case report , fistula formation and aspergillosis after sarpe operation were presented .
a 68-year - old male patient visited konkuk university hospital due to epigastric pain lasting 2 to 3 seconds 2 months before hospitalization . according to his anamnesis , he was taking 50 mg of atenolol because he had been diagnosed with hypertension 2 years before . he had been diagnosed with esophageal diverticulum 10 years before ; since then he had experienced no symptoms . he underwent a cardiac computed tomography ( ct ) scan 3 weeks prior to hospitalization . the ct scan showed that mild stenosis ( 20% to 30% ) had formed in left anterior descending artery , left circumflex artery , and right coronary artery . he underwent gastroesophageal endoscopy ; 5 cm of diverticulum was found 20 cm from the incisor tooth ( fig . 1 ) . there were no other findings except for erosive gastritis in the antrum of the stomach . the esophagography 2 weeks prior to the hospitalization showed that there was 10 cm of diverticulum projecting to anterolateral side ; killian - jamieson diverticulum ( kjd ) was considered ( fig . although the location of the diverticulum on the esophagogram was right side , we decided left cervical approach for surgeon 's convenience . left cervical incision was conducted under general anesthesia and the cervical esophagus was exposed following dissection along the sternocleidomastoid muscle . diverticulum ( 105 cm sized ) was found with a wide base and which contained a small amount of necrotic tissue ( fig . the diverticulum adhered to circumjacent tissues ; in particular , it strongly adhered to the prevertebral fascia in the rear of the trachea . cervical esophagus proximal to the diverticulum was dissected cautiously and looped with a silastic drain . the diverticulum was excised with a ta 60 stapler ( ethicon endo - surgery , cincinnati , oh , usa ) . esophageal myotomy of about 3 cm was conducted along the distal part of the esophagus after the excision of the diverticulum . reinforcement sutures were inserted with 3 - 0 silk along the excision area of diverticulum . the esophagography on the fourth day after the surgery showed that there were not abnormalities such as leakage or stenosis ; therefore , dietary treatment was initiated . the patient was discharged from konkuk university hospital on the sixth day after the surgery . at the time of discharge follow - up observation has been performed for 6 months , during which the patient has not shown any abnormalities such as diverticulum relapse , dysphagia , or stenosis . kjd is a rare form of esophageal diverticulum which appears through the killian 's dehiscence , a mucosal protrusion below the cricopharyngeal muscle . kjd is similar to zenker 's diverticulum ( zd ) due to the protrusion of esophageal mucosa . kjd is rare in that the incidence of kjd is a fourth of that of zd . the two diseases have similar symptoms ( e.g. , dysphagia , coughing , and chest pain ) . however , according to previous studies , kjd has more non - specific symptoms than zd . location is a standard for distinguishing them : zd occurs mainly in the rear of the esophagus in the upper cricopharyngeal muscle , and kjd occurs mainly in the front or side of the esophagus 2 cm away from the lower cricopharyngeal muscle . the two diseases , zd and kjd , have been observed simultaneously . in rare cases , because of its symptom or greater size , endoscopic treatment and surgical treatment are used for kjd . generally , endoscopic treatment is considered preferable in treating zenker 's diverticula smaller than 3 cm . endoscopic treatment has the possibility of occluded view when there is food or a foreign body in the diverticulum . the treatment of the kjd is more closely adjacent to the recurrent laryngeal nerves than zd . for kjd treatments that do not come into contact with the recurrent nerves , treatments other than endoscopic treatments are preferable . according to the previous studies , the safety of endoscopic treatment has not been established for kjd , due largely to the rarity of cases , and for zd the recurrence rate of endoscopic treatment is 10 times higher than that of surgical treatment . furthermore , myotomy should be adopted as a treatment for kjd , since its treatment is closely related to the prevention of the recurrence of zd from the perspective of the features of diverticular disease . in conclusion , konkuk university hospital experienced the successful treatment of a case of kjd that had been accompanied by rare symptom and discussed the result together with the review of the relevant literary works .
killian - jamieson diverticulum is a rare diverticular disease . this disease differs from zenker 's diverticulum in its location and mechanism . various treatment modality have been attempted , but traditional surgical treatment has been recommended for a symptomatic killian - jamieson diverticulum due to the concern of possible nerve injury . we performed surgical treatment by cervical incision . we report here on a case of killian - jamieson diverticulum and we briefly review the relevant literature .
pyoderma gangrenosusm ( pg ) is a chronic , rare , inflammatory cutaneous disorder and is manifested by the development of painful ulcers , bulla , pustules and rarely vegetating lesions and histologically by predominantly neutrophilic infiltration . we report a case of severe pg in a girl since her 4 months of age . the difficulty in diagnosis and methodical approach in such cases is highlighted . a 6-month - old hindu girl presented with progressive , extensive , and painful ulcers since her 4 months of age . ulcers developed from erythematous papules and vesicles , had purple red undermined margin , raw or crusted surface , merged with other ulcers in the neighborhood and formed large ( even > 8 cm ) lesions with annular , polycyclic or crescent shape . they were distributed over scalp , face , ear , trunk ( less over anterior trunk ) , buttocks , thigh , legs , dorsum of hands and feet without any mucosal involvement [ figure 1 ] . ( a ) face , ( b ) arm , ( c ) dorsum of hand , ( d ) leg , ( e ) thigh , ( f ) back , ( g ) encircled site where intradermal normal saline was injected , ( h ) same site after 48 h showing pustulation ( positive pathergy test ) the child was playful with normal physical and intellectual growth . there was mild fever , moderate pallor without any cyanosis , clubbing , organomegaly , lymphadenopathy , or joint abnormality . pustule developed following intradermal injection of normal saline on normal skin ( positive pathergy test ) [ figure 1 ( g , h ) ] . there was low hemoglobin ( 8.9 gm / dl ) , leucocytosis ( 22,600/cmm ) , neutrophilia ( 70% ) , and elevated sedimentation rate ( 69 mmhg ) . chest x - ray , mantoux test , dna - pcr for tuberculosis , immunoglobulin level , ra factor , ana ( hep-2 cell ) , vdrl , c - anca , hiv- elisa , pus culture , and colonoscopy of rectal mucosa revealed no abnormality . histopathology of the skin biopsies with h and e stain from the margin of the ulcer from two different sites showed epidermal ulceration and dense dermoepidermal collection of inflammatory exudates consisting predominantly of neutrophils with occasional multinucleated giant cells [ figure 2 ] . one of the biopsies also showed foci of granulomatous reaction [ figure 2 , inset ] . z - n stain ( for afb ) and pas stain ( for fungus ) were negative . photomicrograph showing ulceration of epidermis and collection of inflammatory exudates along with occasional giant cells ( h and e , 100 ) . inset showing ill formed granuloma with giant cells ( h and e , 400 ) it was diagnosed as a case of pyoderma gangrenosum . the patient responded satisfactorily to oral prednisolone ( 2 mg / kg / day ) . however , there were signs of relapse with dose less than 1 mg / kg / day . at that time , the patient became irregular in follow - up and finally lost to follow up . in most of the cases , pg is associated with some systemic diseases like inflammatory bowel diseases ( ibd ) , rheumatoid arthritis , myeloproliferative disease , etc . extensive distribution of the disease was unusual for an infant with pg who generally have ulcers on head and buttocks . onset at 4 months of age was extremely rare and possibly not reported so far . due to much atypicality , diagnosis was difficult and many possibilities were considered like pg , tuberculous , atypical mycobacterial infection , fungal , malignant and vasculitic ulcers , wegener 's granulomatosis , sweet 's syndrome , and epidermolysis bullosa . violaceous undermined margin with erythematous halo , pain , characteristic histological appearance and positive pathergy test were helpful for diagnosis . multiple site colonoscopic biopsies from even normal rectal mucosa could have increased the diagnostic yield for any subclinical evidence of ibd . the case
a female child developed multiple , progressive , therapy - resistant , painful large ulcers , vesicles , and pustules since her 4 months of age . the ulcers were large , some even measured more than 8 cm ; most had violaceous undermined margin with surrounding erythematous halo , raw and crusted surface and were distributed extensively over scalp , face , ear , trunk , buttocks , thigh , legs , dorsum of hands , and feet without any mucosal involvement . after detail clinical examination and investigation , it was diagnosed as a case of pyoderma gangrenosum . extensive search did not reveal any systemic abnormality or detect any infective etiology . the case highlights the problems of diagnosis of multiple ulcers at very early age .
the beijing genotype is commonly present in russian population and in eurasia as a whole . this lineage , especially bo\w subline is characterized by high - level virulence , , , . for our analysis , we chose the mycobacterium tuberculosis strain b9741 , isolated from a 33-years - old hiv - positive female patient from irkutsk oblast , russia , with firstly diagnosed fibrocavernous tuberculosis , provided by the scfhhrp , irkutsk , russia . this strain was resistant to isoniazid ( inh ) , rifampin ( rif ) , streptomycin ( sm ) and pyrazinamide ( pza ) . genome sequencing was carried out on roche 454 gs junior instrument ( roche , switzerland ) , in the laboratory of bacterial genetics , vigg ras ( moscow , russia ) . all reads were assembled to an initial draft genome : 4,322,170 bp ( total length ) nucleotides at 13-fold coverage using the gs de novo assembler ( version 3.0 ; roche ) ( table 1 ) . the automatic functional annotation results were obtained using ncbi prokaryotic genome annotation pipeline ( pgaap ) ( http://www.ncbi.nlm.nih.gov/genomes/static/pipeline.html ) . the b9741 genome contains 4193 genes ( total ) , 4 rrnas , and 46 trnas . a total of 250 pseudogenes , 3 noncoding rnas ( ncrnas ) , 1 clustered regularly interspaced short palindromic repeats ( crispr ) were predicted using the pgaap . according to housekeeping gene and toxin - antitoxin analysis , , we classified this strain to belong to b0/w beijing lineage . we compared our output sequence with dna sequence of the high - virulent m. tuberculosis w-148 strain which belongs to b0/w cluster of beijing group . in this announcement , we focused on genes , which determine virulence and drug resistance . in addition , we analyzed snps in genes , which are associated with drug resistance to inh , rif , sm , and pza . analysis we provide the comparison of sequenced dna with b0\w dna sequences and revealed the presence of three polymorphisms at virulence genes . in the gene mce3f , which essential for survival of mtbs in macrophages and invasion to the host cells , we have found substitution - d410a , in irtb gene , which encode the part of irtab iron importer - a175 t , . this protein play an essential role for iron homeostasis in stress conditions . and in vapc46 - a38 g , . thus , identified genes will be used for understanding of m. tuberculosis adaptation to patients with low immune level , including hiv + patients . othis whole genome shotgun ( wgs ) project has been deposited at genbank under the accession lvjj01000000 ( mycobacterium tuberculosis strain b9741 ) . this whole genome shotgun ( wgs ) project has been deposited at genbank under the accession lvjj01000000 ( mycobacterium tuberculosis strain b9741 ) . the beijing genotype is commonly present in russian population and in eurasia as a whole . this lineage , especially bo\w subline is characterized by high - level virulence , , , . for our analysis , we chose the mycobacterium tuberculosis strain b9741 , isolated from a 33-years - old hiv - positive female patient from irkutsk oblast , russia , with firstly diagnosed fibrocavernous tuberculosis , provided by the scfhhrp , irkutsk , russia . this strain was resistant to isoniazid ( inh ) , rifampin ( rif ) , streptomycin ( sm ) and pyrazinamide ( pza ) . genome sequencing was carried out on roche 454 gs junior instrument ( roche , switzerland ) , in the laboratory of bacterial genetics , vigg ras ( moscow , russia ) . all reads were assembled to an initial draft genome : 4,322,170 bp ( total length ) nucleotides at 13-fold coverage using the gs de novo assembler ( version 3.0 ; roche ) ( table 1 ) . the automatic functional annotation results were obtained using ncbi prokaryotic genome annotation pipeline ( pgaap ) ( http://www.ncbi.nlm.nih.gov/genomes/static/pipeline.html ) . the b9741 genome contains 4193 genes ( total ) , 4 rrnas , and 46 trnas . a total of 250 pseudogenes , 3 noncoding rnas ( ncrnas ) , 1 clustered regularly interspaced short palindromic repeats ( crispr ) were predicted using the pgaap . according to housekeeping gene and toxin - antitoxin analysis , , we classified this strain to belong to b0/w beijing lineage . we compared our output sequence with dna sequence of the high - virulent m. tuberculosis w-148 strain which belongs to b0/w cluster of beijing group . in this announcement we developed the catalog of 342 genes determinate virulence , , . for this analysis , in addition , we analyzed snps in genes , which are associated with drug resistance to inh , rif , sm , and pza . we provide the comparison of sequenced dna with b0\w dna sequences and revealed the presence of three polymorphisms at virulence genes . we carried out a deeper analysis of these genes involved in virulence . in the gene mce3f , which essential for survival of mtbs in macrophages and invasion to the host cells , we have found substitution - d410a , in irtb gene , which encode the part of irtab iron importer - a175 t , . this protein play an essential role for iron homeostasis in stress conditions . and in vapc46 - a38 g , . thus , identified genes will be used for understanding of m. tuberculosis adaptation to patients with low immune level , including hiv + patients . othis whole genome shotgun ( wgs ) project has been deposited at genbank under the accession lvjj01000000 ( mycobacterium tuberculosis strain b9741 ) . this whole genome shotgun ( wgs ) project has been deposited at genbank under the accession lvjj01000000 ( mycobacterium tuberculosis strain b9741 ) . the authors declare that there is no conflict of interests with respect to the work published in this paper . this research did not receive any specific grant from funding agencies in the public , commercial , or not - for - profit sectors .
we report a draft genome sequence of mycobacterium tuberculosis strain b9741 belonging to beijing b0/w lineage isolated from a hiv patient from siberia , russia . this clinical isolate showed mdr phenotype and resistance to isoniazid , rifampin , streptomycin and pyrazinamide . we analyzed snps associated with virulence and resistance . the draft genome sequence and annotation have been deposited at genbank under the accession nz_lvjj00000000 .
they are congenital benign tumor accounting for 15 - 20 % of intraventricular mass but only about 1% of intracranial ones ( 1 - 3 ) . they can be diagnosed at any age but usually become symptomatic in the third to sixth decades and more common in men than women . they usually found incidentally and are asymptomatic ; but in some instances may associate with rapid neurologic deterioration , herniation , and sudden death . so , recognition of this rare but important diagnosis may result in decreasing mortality ( 4 ) . in this report , we presented a 13-year - old boy with complaint of two times drop attack and final diagnosis of colloid cyst in the third brain ventricle . a 13-year - old boy was brought to the emergency department ( ed ) with complaint of two times drop attack . the patient mentioned that he felt sudden weakness in both lower limbs , which led to drop . these attacks were happened about 4 hours before admission and he did not have any same experience previously . he did not have complaint of nausea , vomiting , headache , vertigo , blurred vision , or palpitation . the subject did not have any known structural or congenital heart disease , but suffered from asthma and used salbutamol spray irregularly . the patient did not have trauma history and there was no positive history of any known medical illness in his parents or closed relatives . on arrival , he had 36.9c axillary temperature , 16/minute respiratory rate , 90/minute pulse rate , 120/80 mmhg blood pressure , and 96% oxygen saturation at room air . on physical examination , he did not have focal neurologic findings or even paresthesia or paraplegia . general examination of head and neck , chest , abdomen , and limbs did not reveal any positive findings . following the evaluation process , a brain computed tomography ( ct ) was performed ( figure-1 ) . a hyperdense round lesion was seen in the third ventricle consequently caused that the corresponding physician requested a neurologic consultation in the ed . finally , the brain magnetic resonance imaging ( mri ) confirmed the diagnosis of third ventricle colloid cyst and the patient was underwent surgery and discharged without any problem ( figure 2 ) . the colloid cysts commonly settle near the foramen of monro in the anterior third ventricle and so may encounter with drainage of the cerebrospinal fluid ( csf ) ( 3 ) . since even a small lesion can block the mentioned foramen , these cysts may result in hydrocephalus and increase the intra cranial pressure ( 5 ) . increased intracranial pressure can be manifested with headache described as severe and intermittent , with short duration , usually located frontally . in contrast with usual headaches , secondary to intracranial tumors , the colloid cyst induced headache can be relieved by lying down ( 4 ) . other symptoms include drop attacks , gait abnormalities , progressive dementia , and transient loss of consciousness . in children , the most common symptoms are nausea , vomiting , headache , diplopia , and papilledema ( 6 ) . the classic clinical description of intermittent headaches and drop attacks occurs in only one - third of patients . sudden obstruction of the ventricular system and following rapid rising of intracranial pressure can lead to herniation and rarely sudden death ( 7 , 8) . colloid cysts size varies from 3 - 40 millimeters in diameter , but the size do not related to their symptoms or outcome , as even small ones may lead to sudden death ( 9 ) . colloid cyst is usually diagnosed by non - contrast computed tomography ( ct ) as an oval or rounded hyperdense mass on the anterior aspect of the third ventricle . they may occasionally be hypodense or isodense to the brain , or found in other areas of the brain . colloid cysts have different manifestation on mri . despite their variable signal characteristics , their location and shape help to the correct preoperative diagnosis in most patients ( 6 ) . half of the cases are hyperintense on t1-weighted mri images and hypointense on t2-weighted mri images respected to brain . isointense cysts are not easily identified on mri , and in such instances ct scan is more useful ( 9 , 10 ) . small asymptomatic colloid cyst can be considered for close follow up by serial examinations and neuroimaging ( 11 ) . there is also the rare report of spontaneous resolving of the third ventricle colloid cyst ( 12 , 13 ) . all authors passed four criteria for authorship contribution based on recommendations of the international committee of medical journal editors .
colloid cysts are mucous - filled masses with an outer fibrous layer . these cysts are rare developmental malformation and not a true neoplasm . they usually found incidentally and are asymptomatic ; but in some cases may associate with rapid neurologic deterioration , herniation , and sudden death . recognition of this rare but important diagnosis may result in decreasing mortality . in this report , we presented a 13-year - old boy with complaint of two times drop attack and final diagnosis of colloid cyst in the third brain ventricle .
the etiologies of intracranial artery dissection are various , the exogenous triggers like trauma , infection as well as inherited connective tissue disorders like moyamoya disease , fibromuscular dysplasia , and marfan 's syndrome . alpha 1 antitrypsin deficiency and hyperhomocysteinemia are also distributing factors of carotid artery dissection19,25 ) . the internal carotid artery dissection is responsible for less than 2% of all stroke and for 10 - 20% of stroke of young adults22 ) . the dissection tends to occur in proximal internal carotid artery ( ica ) and rare in distal ica except when the cause is exogenous such as infection . dissecting aneurysms of the intracranial posterior circulation are also rare that produce acute brain stem infarction or hemorrhage in young , healthy patients13,28,29 ) . we report a case of multiple dissecting aneurysms of left superior cerebellar artery ( sca ) , left posterior inferior cerebellar artery ( pica ) and right pericallosal artery in patient who presented with subarachnoid hemorrhage . we will also discuss of the relevance between dissecting aneurysm with the patient 's medical history , thrombocythemia . a 42-year - old man was admitted to emergency room with a history of decrease in the level of consciousness . he had no previous event of motor weakness or dizziness before the onset of mental change . he had past medical history of hereditary spherocytosis and underwent splenectomy . during the follow up , his bone marrow biopsy reported megakaryocytic hyperplasia and he was diagnosed to essential thrombocythemia ( et ) . after a year , he was diagnosed to cerebral infarction and had been taking oral anti - platelet agent , since then . the routine laboratory tests were normal except the platelet count , 660 k / ul . serum elastase was also within normal limit . emergent brain ct showed diffuse subarachnoid hemorrhage ( sah ) in the basal cistern and acute hydrocephalus ( fig . the hematoma was located in posterior fossa , especially in interpeduncular cistern and prepontine cistern . the diffusion mri , which was done one year prior to the time of stroke , showed multiple cerebral infarctions on both cerebellum and body of corpus callosum ( fig . with mr angiography , we could identify the fusiform aneurysms on the left sca and the left pica . 2b ) . the conventional angiography revealed multiple dissecting aneurysms in left sca , left pica and right pericallosal artery ( fig . considering hematoma distribution , we concluded that the sca aneurysm was responsible for sah . since collateral circulation to midbrain , pons and cerebellum was abundant , we decided to occlude left sca with coil embolization ( fig . postoperative diffusion mri revealed cerebellar infarction ; however , the patient showed no clinical symptom and was able to discharge with alert mentality , recovered from hemiparesis after rehabilitation ( fig . intracranial dissection is rare event comparing to extracranial dissection , and is usually demonstrate ischemic event of young adults when it 's symptomatic5,22 ) . it usually relates to exogenous triggers like trauma , infection as well as inherited connective tissue disorders . our case was very rare one because it was a case of multiple intracranial dissecting aneurysms that resulted in sah as well as acute cerebral infarction which was related to no other risk factors except et . essential thrombocythemia is characterized by a high platelet count , originating from a pluripotent stem cell and usually affects middle aged to elderly . nearly half of the patients are asymptomatic while the other half has vascular occlusive or hemorrhagic event14 ) . it is known to cause a prothrombic state10,17 ) , and there are reports that describe the relationship between et with cerebral ischemic event . arboix et al.3 ) has reported several cases of large - vessel occlusion which resulted in ischemic stroke due to et . bogousslavsky et al.6 ) has reported that the ischemic stroke attributed to et was found in 0.4% of cases in the lausanne stroke registry . there have been reports of intrapetrous ica dissection of the patient with et9,12 ) , yet multiple intracrnial dissecting aneurysms of the patient with et has been rarely reported . piepgras et al.20 ) has reported the case of distal middle cerebral artery dissecting aneurysm secondary to proximal artery dissection , however , in our case , we could not find any dissection or occlusion in proximal arteries . et , by changing prothrombotic state , causes a disturbance of microcirculation of vasa vasorum and as a result , increases the vulnerability of the vessel walls10 ) . however , this theory only explains the dissection of extracranial arteries that the vasa vasorum is usually distributed to and does not fit in our case7 ) . et can induce endothelial damage by activating leukocyte and releasing the elastase and alkaline phosphatase that play a role of the pathogenesis of the prothrombic state8 ) . the most characteristic structure of intracranial artery is internal elastic lamina . instead of lack of external elastic membrane , thinner adventitia , and fewer elastic fibers of media , in addition , the media and internal layer of intracranial artery is lack of vasa vasorum7 ) . these changes occurs at the level of skull base in the carotid artery and at the 1 cm proximal to the dural perforation in vertebral artery27 ) . with the anatomical differences , there are pathological differences between dissecting aneurysm of extracranial and intracranial arteries . in the extracranial artery , dissection usually occurs between the media and adventitia or outer layer of the media11,13,23,28 ) . in extracranial artery dissection , for example , cystic medial necrosis and degeneration of elastic tissue in the media is observed4,18 ) . in the intracranial artery , on the other hand , intramural hematoma is usually formed between internal elastic lamina and media1,15,16,28,29 ) . when the dissection involves the subadventitia and rupture into the subarachnoid space , sah is presented2,21,24,26,29 ) .
the etiologies of intracranial artery dissection are various , the exogenous as well as inherited connective tissue disorders . we report on a patient who presented with diffuse subarachnoid hemorrhage who had been suffered from essential thrombocythemia . he was diagnosed to multiple dissecting aneurysms of left superior cerebellar artery , left posterior inferior cerebellar artery and right pericallosal artery and treated with endovascular coil embolization .
only several cases have been reported in the literature and most of them concerned mucous cysts ( 1 - 3 ) . although many theories have been suggested for the late cyst formation after rhinoplasty , migration or incorporation of mucosal tissue in the subcutaneous space during the surgical procedure has been accepted as an important mechanism ( 4 - 6 ) . careful dissection and meticulous manipulation of tissue and implant are necessary to prevent the mucosa from being grafted with the implant material ( 4 - 6 ) . rarely , a foreign body type cyst resulting from petroleum jelly impregnated with the packing material has been reported . the authors report a case of dorsal nasal cyst that was presumed to have a different pathogenesis . we describe this case with a brief review of the literature with an emphasis on its pathogenesis and treatment . thirty years earlier at a local clinic , she had undergone augmentation rhinoplasty with a material presumed to be silicone . she had had no complication afterwards . however , 5 yr previously , a nasal mass developed that had increased gradually . physical examination showed a 3.52.5 cm round , soft , painless mass on the midline of nasal radix ( fig . the nasal dorsum and the tip were firm on palpation and a small cruciate incision scar was found at the infratip lobule , which was supposed to be the entry of the augmentation material . on computed tomography ( ct ) scan , a heterogeneous cystic mass was observed at the radix and the cyst was continuous with a homogenous density at the nasal dorsum , which was first thought to be a silicone implant ( fig . 2 ) . a presurgical diagnosis of nasal radix cyst associated with previous augmentation rhinoplasty was made , and a direct , open approach was chosen to remove the cyst . after a skin incision on the center of the cyst , it was dissected down to the nasal bone . the cyst was connected to the dorsal mass , which were removed together in en - bloc fashion ( fig . 3a ) . for safe removal of the graft material over the tip area and effective tip - plasty , grossly , the removed cystic mass had a thick , fibrous wall with dirty cheesy material inside . the cystic wall was composed of dense fibrous tissue containing dispersed , microsized amorphous foreign materials ( fig . foreign materials were surrounded by foreign body type multinucleated giant cells and induced granulomatous reactions . a scanning electron microscopy ( sem ) showed a fragment of foreign body ( fig . 4a ) and energy dispersive radiography spectroscopy ( edx ) showed presence of silicone ( si ) in the removed specimen ( fig . the lesion was diagnosed as a foreign - body type cyst associated with silicone material used for the augmentation rhinoplasty . the depressed radix , dorsum , and tip after excision of the mass were reconstructed using auricular and septal cartilages . the redundant and thin skin of the radix was trimmed and reinforced with an underlay of the temporalis fascia . the patient has been followed for 9 months postoperatively with a good aesthetic result and no complication ( fig . exact pathogenesis of the cyst formation in our case is not clear because no information about the material that had been used for dorsal augmentation was available . however , considering the gross and histological findings as well as the results from sem - edx analysis , foreign body inflammatory reaction from silicone material , most possibly a liquid type , which had been injected 30 yr ago , probably caused the cyst . first , sem - edx analysis of the cyst revealed silicone in the cyst , which meant that a type of silicone material was used for dorsal augmentation . second , silicone , whether it is a liquid type or implant type , was and still is the most commonly used material for augmentation rhinoplasty in korea . third , a small , cruciate incision of the infratip lobule was too small to insert any type of hard material including silicone implant . although calcification and inflammatory cell infiltration around a silicone implant have been reported , total degradation is impossible considering the bio - characteristics of the silicone implant ( 7 , 8) . sem - edx used for analyzing the graft material in this case adopted the principles that when the electron generated from the electron microscope collides with the object , a specific radiography characteristic to the object is released from the surface . edx analysis clarifies the object by detecting this radiography , and this technique is widely used in material engineering and archeology . in our case , a small amount of silicone detected in the specimen led us to believe that the foreign body that caused cyst formation was a type of silicone material . silicone implant has been used for rhinoplasty since 1950 and it still remains one of the most widely used implant materials in asian countries , including korea ( 9 - 11 ) . injectable liquid silicone is useful in augmenting the chin , cheek , and glabella area ( 12 ) . it is not recommended for rhinoplasty because the nasal skin is thin and the silicone frequently results in ridging or beading ( 12 ) . serious complications such as severe edema or localized discoloration of the injected area has also been reported ( 13 ) . only one case of nasal dorsal cyst after augmentation rhinoplasty using silicone implant has been reported in the literature but it 's pathologic findings and pathogenesis were not clarified ( 1 ) . the mainstay of treating postrhinoplasty nasal dorsal cyst is complete resection and reconstruction . in this case , a direct open approach using the horizontal incision over the cyst center was used given the location and size of the cyst . to expose the tip , an open approach using separate transcolumellar incision was chosen because a previous cruciate incision was located at the infratip lobule and it was too small to expose the nasal tip . in revision rhinoplasties due to complications of the allograft implant , fortunately , we could augment the depressed dorsum and radix with autogenous septal and auricular cartilages without harvesting the rib cartilage despite the large defect .
development of a cystic mass on the nasal dorsum is a very rare complication of aesthetic rhinoplasty . most reported cases are of mucous cyst and entrapment of the nasal mucosa in the subcutaneous space due to traumatic surgical technique has been suggested as a presumptive pathogenesis . here , we report a case of dorsal nasal cyst that had a different pathogenesis for cyst formation . a 58-yr - old woman developed a large cystic mass on the nasal radix 30 yr after augmentation rhinoplasty with silicone material . the mass was removed via a direct open approach and the pathology findings revealed a foreign body inclusion cyst associated with silicone . successful nasal reconstruction was performed with autologous cartilages . discussion and a brief review of the literature will be focused on the pathophysiology of and treatment options for a postrhinoplasty dorsal cyst .
dd , caused by mutations in the er ca atpase atp2a2 ( sakuntabhai , et al . , 1999 ) , is an uncommon ( 1:30,000 ) blistering skin disease . patients with dd suffer from impaired cell - to - cell adhesion , defective keratinocyte differentiation , and non - physiologic keratinocyte apoptosis . histologically , dd manifests with suprabasal clefting in the epidermis , acantholysis , rounded dyskeratotic keratinocytes ( corps ronds ) , hyperkeratosis and parakeratotic keratinocytes in the stratum corneum ( grains ) . current treatments , such as retinoids , do not ameliorate the underlying defect in er ca sequestration , and are ineffective for many patients . this report , by savignac et al ( editor , please add reference ) , advances our understanding of dd in several important ways . first , it illustrates how er stress impairs the formation of both adherens junctions and desmosomes , contributing to dd pathogenesis . second , it expands our understanding of how er ca signaling may control , not only keratinocyte growth and differentiation , but also keratinocyte cell - to - cell adhesion . defects in desmoplakin redistribution have been associated with the impaired cell - to - cell adhesion seen in dd ( dhitavat , et al . , 2003 , defective desmoplakin redistribution after serca2 ca depletion is mediated by protein kinase c alpha ( pkcalpha ) ( hobbs , et al . , 2011 ) . pkcalpha also may act on desmoplakin to direct the hyperadhesive desmosomal state ( hobbs and green , 2012 ) , rearrange desmosome components during wound healing ( garrod , 2013 ) , and modulate desmosomal susceptibility to autoimmune attack in pemphigus vulgaris ( cirillo , et al . , more recently , cell - to - cell adhesion defects in dd also have been associated with defects in e - cadherin redistribution ( celli , a. , et al . , 2011 ) . because both desmoplakin and e - cadherin have been shown to have signaling as well as structural roles ( kowalczyk and green , 2013 , tu , et al . , 2012 ) , it is likely that interactions among adhesion components involve multiple feedback loops between each other and the serca2-controlled er ca store . mild and self - limited er stress , due to transient release and refill of er ca stores , is an important physiologic signal for epidermal permeability barrier repair and antimicrobial peptide synthesis ( celli , a. , et al . , 2011 , park , et al . , 2011 ) . once er ca depletion passes a critical threshold , the er unfolded protein response ( upr ) is triggered , and apoptotic mechanisms are initiated in many cell types ( oakes , et al . , 2003 ) . this report identifies er stress , induced by er ca depletion due to serca2 dysfunction , as an important contributor to dd pathogenesis . finally , this report demonstrates that treatment of dd keratinocytes with miglustat improves desmoplakin and e - cadherin redistribution and improves ( although it does not normalize ) cell - to - cell adhesion . the authors propose that miglustat acts as a chaperone that allows adhesion molecules to escape from the er stress - induced upr , thus enabling them to reach the plasma membrane and form adherens junctions and desmosomes . miglustat , used clinically for gaucher disease , also acts to inhibit glucosylceramide synthase ( reviewed in venier and igdoura ( venier and igdoura , 2012 ) ) , and an additional potential therapeutic pathway may be through its modulation of the ceramide / sphingolipid pathway previous described in dd pathogenesis ( celli , a , et al . , 2012 ) . lastly , since glucosylceramide synthesis is required for epidermal permeability maintenance ( jennemann , et al . , 2007 ) , some caution should be used in extrapolating these results from monolayer keratinocytes to a multilayered epidermis or to patients . as the authors note , however , therapeutic options for dd are limited , and miglustat may be the first in a series of agents that treat dd by facilitating redistribution of adhesion molecules to the plasma membrane .
darier 's disease ( dd ) is caused by mutations in the endoplasmic reticulum ( er ) ca2 + atpase atp2a2 ( protein serca2 ) . current treatment modalities are ineffective for many patients . this report shows that impaired serca2 function , both in dd keratinocytes and in normal keratinocytes treated with the serca2-inhibitor thapsigargin , depletes er ca2 + stores , leading to constitutive er stress and increased sensitivity to er stressors . er stress , in turn , leads to abnormal cell - to - cell adhesion via impaired redistribution of desmoplakin , desmoglein 3 , desmocollin 3 and e - cadherin to the plasma membrane . this report illustrates how er ca2 + depletion and the resulting er stress are central to the pathogenesis of the disease . additionally , the authors introduce a possible new therapeutic agent , miglustat .
metronidazole , a nitroimidazole antimicrobial agent is widely used in the treatment of anerobic and protozoal infections for more than three decades . common side effects are nausea , vomiting , abdominal discomfort , headache , and metallic taste . neurological effects such as ataxia , dizziness , peripheral neuropathy , and seizures were also reported . we report a rare case of metronidazole - induced encephalopathy ( mie ) in order to create an awareness of this unusual entity among practitioners . a 39-year - old male without any comorbidities was brought to the emergency department for acute onset of slurring of speech , generalized weakness , and unsteadiness . he was unable to button his shirt , and turn a doorknob due to clumsiness of hands and fingers for the past 24 hours . four days prior to this admission , he complained of numbness and tingling sensation of toes and dorsum of both feet . recently , he was treated for amebic liver abscess with ultrasound - guided aspiration and was given tablet metronidazole 800 mg three times a day for 2 weeks . since repeat ultrasound revealed re - accumulation of liver abscess after 2 weeks , he was advised to continue metronidazole 800 mg three times a day orally for 2 more weeks . thus , he had taken 67.2 grams of tablet metronidazole over a period of 28 days . neurologic examination revealed the patient was conscious but confused , with dysarthria , nystagmus , dysmetria on finger - to - nose test , positive romberg 's sign ; graded sensory loss to pain , temperature , touch , and proprioception over distal lower and upper extremities in a stocking and glove type , and impaired joint position and vibration sense . clinical assessment of cranial nerve , muscular system , deep tendon reflexes , and gait was normal with absent babinski sign . his hematological , metabolic panel , thyroid profile , cerebrospinal fluid ( csf ) analysis , viral markers , arterial blood gas , ammonia levels , vitamin b1 , b12 , and folate levels were within normal limits but for mild derangement of liver function . his magnetic resonance imaging ( mri ) brain revealed symmetrical areas of altered signal intensity , appearing hyperintense on t2w and fluid - attenuated inversion recovery ( flair ) images , and involving the dentate nuclei and splenium of the corpus callosum . there was an evidence of diffusion restriction on diffusion - weighted / apparent diffusion coefficient mapping without evidences of hemorrhage or infarct . imaging findings were in favor of metronidazole toxicity , which was supported by the prolonged history of metronidazole intake . moreover , his symptoms disappeared on third day after discontinuation of metronidazole , and mri 4 months later showed resolution of the earlier described signal changes . mie usually occurs at doses exceeding a total of 50 grams / month or 1.5 to 2 grams / day . although the exact mechanisms are unknown , the possible mechanisms with experimental evidences are furnished . furthermore , cerebellum of rats has been shown to uptake carbon labeled metronidazole . in vitro , metronidazole incorporated into thiamine analogs , inhibits the phosphorylation of thiamine , thereby antagonizing vitamin b1 effect . radiolabelled metronidazole binds ribonucleic acid ( rna ) in a significant manner , and inhibits neuronal protein synthesis and facilitates degeneration . catecholamine neurotransmitters are oxidized by metronidazole derivatives to produce semiquinone and nito anion radicals which are neurotoxic . the case is presented so as to facilitate practitioners to recognize the symptoms and signs of metronidazole toxicity , and consider them in their differential diagnosis . it is worth to remember the potential neurological abnormalities and imaging findings of this entity , as this agent is frequently prescribed and used in clinical practice .
encephalopathy associated with metronidazole administration is an uncommon but potentially reversible disease and depends on the cumulative metronidazole dose , and most patients with this condition recover rapidly after discontinuation of therapy . we present a case as well as a review of the literature regarding this rare but serious adverse event .
the success of radiotherapy ( rt ) depends not upon the expense and complexity involved , but upon the correctness of techniques . correct portal - design calls for correct knowledge regarding the location of the target volumes to be treated . traditional bony - landmarks may have been used as a surrogate , but they do not always correlate with the actual location of the soft - tissue target - volumes . the four - field - box ( 4fb ) technique for cervical carcinoma is often utilized to improve dose homogeneity . the exact placement of the posterior border on the lateral portals of this technique is unfortunately not supported by stone - hard consensus ; placing it at the s2-s3 junction may increase the chance of sparing rectum but at the risk of target - miss . we intend to demonstrate the potential benefits with the use of sectional imaging in safely delineating target - volumes . a lady on evaluation and referral from her gynecologist , presented to us with the diagnosis of stage - ib2 cervical - carcinoma staged as per the figo ( federation of international gynaecologists and obstetricians ) system . as we were preparing to initiate this patient on a course of concurrent chemo - rt , we had the opportunity to review her magnetic - resonance imaging ( mri ) films obtained earlier by her gynecologist . we noticed a stark retroversion of the uterus , which almost abutted the sacrum [ figure 1 ] . this finding made us ponder over the potential perils associated with the conventional 4fb technique , which is widely utilized worldwide for portal design for cervical - carcinomas . given that the uterine and cervical lymphatics are interconnected , and that disease extension from the cervix to the uterus is highly probable , the current consensus state that the entire uterine - corpus should be a part of the clinical - target - volume ( ctv ) for every patient of cervical - carcinoma . the uterus , being a mobile organ may manifest various positions , which can not be taken into account during bony - landmark based planning . unless the true position of the uterus can be determined with imaging , it would be risky to place the posterior - margin of lateral fields at the s2-s3 junction . magnetic resonance imaging showing a retroverted uterine corpus extending well beyond the posterior border of the s2-s3 junction concurrent chemo - rt is a standard of care in the curative approach for stage - ib2 cervical - carcinoma . though there has been a recent emergence of the use of 3d - imaging based techniques , however , a considerable majority of patients across the world are treated with traditional rt techniques even to this day , mainly owing to the fact that cervical - cancer is mainly a disease of the developing world which suffers shortages with regards to advanced planning and treatment systems . conventional techniques may involve either the opposed anteroposterior - posteroanterior ( ap / pa ) two - field technique , or the 4fb technique - planned using radiologically determined bony - landmarks . in the 4fb technique , lateral portals are added with an intention to reduce the dose to the bowel anteriorly and to the rectum posteriorly . conventional techniques have been found to provide equivalent results in comparison to 3d - rt , which is more expensive and complex . however , inadequate coverage with improper portal - design can preclude chances of cure . the ap and pa field definitions are similar with the two - field and the 4fb technique . the caveat with the implementation of the 4fb technique has always been ( and still continues to be ) the fact that are no unanimous guidelines regarding the margin definitions for the lateral portals . the controversy lies in the definition of the posterior - margin of the lateral - portal . some authorities recommend its placement at or 0.5 cm posterior to the anterior - border of the s2-s3 interspace . with particular reference to the treatment of stage - ib carcinomas , one definition suggests that the posterior border be placed in such a way as to cover atleast 50% of the rectum . however , our point of contention would be that such a definition would be oblivious to the status of the uterine - position [ figure 1 ] and rectal - distension . placing the posterior - margin of the lateral - portals at s2-s3 junction using bony references from radiographs was found to be inadequate to cover the ctv in patients with bulky - disease . an evaluation of the ctv coverage by using the s2-s3 junction for the posterior border of the lateral fields revealed an inability to cover the optimal target - volumes in about half of the stage - ib patients.[57 ] the consequent effects on local - control were also quantified . among stage - ib patients , the local - control at 3-years was 100% for patients who had adequate margins , compared to a drastically reduced value of 71% for patients of the same stage with inadequate margins . since cervical carcinoma is staged clinically with the figo - system ( which gives no regard to the utility of imaging to describe uterine - corpus involvement ) , it would be potentially dangerous to apply a one definition fits all philosophy in designing portals for patients with staged ib2 with the figo system . the current consensus recommends the inclusion of the entire uterine - corpus into the ctv mainly since the uterine and cervical lymphatics are interconnected . retroverted uterus ( after all , a normal variation of the uterine - position ) may extend well beyond the line falling from the s2-s3 junction [ figure 1 ] . the presence of uterine - retroversion is unlikely to be detected unless use is made of ct or mri . given that the ctv would be incomplete without the inclusion of the entire uterus , the design of lateral portals of the 4fb technique should never be based on bony references . it should be individualized to the patient 's soft - tissue imaging ( with ct / mri ) obtained in the treatment position . usage of bony - landmarks for portal definition is insensitive to uterine flexion / version , which would be influenced by bladder and rectal filling . mri , if used in treatment planning provides a very accurate definition of the individual morbid anatomy . lateral portals designed using sagittal mri would help in a safe and confident placement of posterior margins . ct would be a reasonable alternative if mri based planning is unavailable , given that vivid soft - tissue detail and accurate reconstructions can be had with helical ct - scanners . in concluding , we remind the reader that as per current consensus , the ctv for cervical - carcinoma would be incomplete without the inclusion of the entire uterus . the uterus is not a fixed organ , and has many possible variations in its position , which can not be encompassed by bony - landmark based planning . ct / mri based target delineation provides an opportunity to take the uterine position and bulk into account .
radiation therapy ( rt ) plays a pivotal role in the curative approach for carcinoma of the cervix . inspite of the emergence of various new conformal techniques in rt , conventional techniques still hold vital importance . majority of the patients worldwide are treated with 2d - rt techniques . 2d - rt techniques have been proven to be non - inferior and simpler in comparison to 3d - rt in the context of carcinoma of the cervix . however , inadequate target volume coverage with improper portal design can preclude the chances of cure . we demonstrate the need for abolishing guesswork in terms of target volume determination through the example of a patient 's sagittal magnetic resonance image showing a case of the retroverted uterus which would have been likely to be missed from the treatment portals if they were designed using definitions based on bony landmarks .
a 45-year - old male patient was admitted to an outpatient clinic with blunt thoracic trauma after falling from height ( work - related accident ) and was referred to kocaeli university faculty of medicine after the detection of ischemia on his electrocardiogram . in the physical examination , the vital signs were stable , but pain and tenderness was detected on his ribs in the thoracal examination . however , it was worth noting the patient had a history of hypertension . a t inversion was detected in d1 , avl , and v3 , v4 , v5 , v6 derivations in the electrocardiogram ( ecg ) . further , there were no additional pathologic findings in the blood tests and the serological tests echocardiography showed minimal mitral insufficiency and a cystic lesion ( size : 2527 mm ) next to the left ventricle anterolateral wall ( fig . cardiac magnetic resonance imaging ( mri ) documented a cystic lesion on the lateral wall of the left ventricle , approximately 3.52.5 cm in size ; this lesion was initially considered to be similar to a hydatid cyst ( fig . 1b ) . there were no additional cyst hydatid lesions found in the computerized tomography scan of the abdomen and the thorax . median sternotomy was performed under general anesthesia , and cardiopulmonary bypass was initiated after standard aorta and bicaval cannulation . the cyst was opened up ; cyst fluid was aspirated ; and the remaining contents , including the germinative membrane and the daughter vesicules , were removed ( fig . we paid maximum attention to the prevention of the dissemination of the aspiration fluid to the pericardial sac . the cyst cavity was irrigated with a highly concentrated sodium chloride solution , and then , the empty sac was closed with primary sutures . a therapy with albendazole was initiated immediately with a 10-mg / kg / day dose and maintained for three months . echocardiography and thorax computed tomography ( ct ) were repeated in the outpatient clinic control at 6 months postoperatively and showed no pathological findings . median sternotomy was performed under general anesthesia , and cardiopulmonary bypass was initiated after standard aorta and bicaval cannulation . the cyst was opened up ; cyst fluid was aspirated ; and the remaining contents , including the germinative membrane and the daughter vesicules , were removed ( fig . we paid maximum attention to the prevention of the dissemination of the aspiration fluid to the pericardial sac . the cyst cavity was irrigated with a highly concentrated sodium chloride solution , and then , the empty sac was closed with primary sutures . a therapy with albendazole was initiated immediately with a 10-mg / kg / day dose and maintained for three months . echocardiography and thorax computed tomography ( ct ) were repeated in the outpatient clinic control at 6 months postoperatively and showed no pathological findings . hydatid disease is endemic in farming areas but occurs worldwide . in particular , the incidence is greater in the cattleor sheep - raising areas of the world , such as australia , south america , south africa , and panama , the mediterranean countries , and the middle east . the most common site of the disease is the liver , followed by the lungs , kidney , bones , and brain . . any race can be affected , and this disease is common in both men and women . its symptoms depend on the affected organ ; for example , liver cysts cause jaundice and abdominal discomfort , while lung cysts cause cough , chest pain , and hemoptysis ( coughing up blood ) . hydatid cyst is a human parasitic disease in which multiple - visceral involvement is generally caused by the metacestode of the tapeworm or larva of the species of the genus echinococcus . however , the most frequently involved cardiac region is the left ventricle ; the right ventricle and the interventricular septum are the less affected regions , respectively . the age of the cyst , size of the cyst , and the extent of calcification are decisive for clinical presentation in patients . patients generally present at outpatient clinics with complaints of subjective symptoms such as palpitation , dyspnea , and atypical angina pectoris . diagnosis is performed using imaging techniques , examination of the cyst fluid , and serological tests . a cardiac cyst can lead to life - threatening consequences such as myocardial infarction caused by the compression of a coronary artery , pulmonary edema caused by a disturbance in the valvular mechanisms , or outflow tract obstruction and sudden cardiac arrest caused by a variety of conduction defects . a cardiac cyst is a diagnostic and therapeutic challenge due to the variability of signs and symptoms at its presentation , its numerous and often unpredictable preoperative complications , and the risk of complications associated with cardiac surgery . our patient was asymptomatic for years and did not have any complaints associated with hydatid disease before the accident when a cardiac hydatid cyst was diagnosed by the thorax ct . arrhythmia , electrical conduction system defects and bundle branch blocks , myocardial infarction , and non - specific st segment and t - wave changes can be seen in the ecg in the cases of cardiac hydatid cysts . a t inversion was detected in d1 , avl , and v3-v6 in the ecg of our patient ; however , the patient 's coronary angiography was normal . cardiac cyst hydatid involves many septums and occasionally , daughter cysts . because of the thin membrane surrounding the cyst it can be distinguished from other intracardiac masses by echocardiography . a hydatid cyst can be in the form of a solid mass or have a multiloculated cystic formation in some cases . cardiac mri and thoracic ct have been utilized in the diagnosis of hydatid disease . in our case , the cardiac mri revealed normal intensities , sizes , and wall thicknesses of the right ventricle , left atrium , and right atrium . further , a hypodense mass ( size : 3.52.5 cm ) was observed in the t1-weighted sequence ; the t1- and t2-weighted sequences of the cyst rim revealed hypointensity . the most common treatment strategy of echinococcosis is 6 months of medical treatment with albendazole or mebendazole or a combination of albendazole and praziquantel after surgical treatment . we chose surgical excision under cardiopulmonary bypass , and the albendazole treatment was pursued after surgical cyst excision in this patient . the patient is still being followed in the outpatient clinic of infectious diseases . in conclusion , the possibility of cardiac hydatid disease should be kept in mind , particularly in the endemic zones . due to the high risk of their associated complications ,
cardiac involvement in hydatid disease is more seldom than the involvement of the liver and the lungs . cardiac cyst hydatid disease is diagnosed incidentally or by means of symptoms such as dyspnea and angina pectoris . here , we present the case of a 45-year - old male patient who underwent open heart surgery for a randomly detected cardiac cyst hydatid during investigations carried out in a healthcare institution after accidentally falling from height . on the other hand , this patient did not have any complaints associated with hydatid disease before this event .
posttraumatic cerebral edema leading to refractory intracranial hypertension is the main prognostic factor in brain - injured patients . morbidity and mortality occur as a result of transtentorial herniation caused by mass effect due to cerebral swelling . the combined medical and surgical objective during the acute phase is to prevent raised intracranial pressure ( icp ) and maintain cerebral perfusion pressure in order to limit the development of secondary lesions . early decompressive craniotomy ( dc ) is a method for decreasing raised icp and has been associated with better outcomes . in neonates , as the cranial sutures are open , any associated fracture may relieve the raised icp due to craniotomy flap - elevation like effect . an 8-day - old male child was brought to the emergency room with a history of fall from a cradle of about 3 feet height followed by loss of consciousness for half an hour . there was no history of convulsions , vomiting or ear , nose , and throat bleed . on examination , the neonate was vitally stable , alert , conscious , moving all four limbs with no focal neurological deficit . the physical examination showed a large swelling in the left temporal region behind the ear ( battle 's sign ) [ figure 1 ] . photograph on the day of admission showing a swelling in the left temporoparietal region with battle 's sign computed tomography ( ct ) of the brain showed a large left sided frontoparietal contusion with brain herniation through the fracture segment [ figure 2 ] . plain axial computed tomography scan brain shows a large hyperdense area in the left frontoparietal region suggestive of a contusion ( black arrow ) , as well as brain herniation , with underlying sutural widening based on the clinical examination and ct findings , a decision was taken to medically manage the patient with anti - epileptic medications and monitor for any signs of raised icp . the child showed progressive improvement in the neurological condition and was discharged after 8 days and asked to follow - up for dural repair and cranioplasty after 3 weeks . the child was lost to follow - up and presented 2 months later with a cystic , nonpulsatile 5 cm 4 cm swelling in the left temporal region . ct scan showed a large cystic lesion in the left temporoparietal region with underlying bone defect [ figure 3 ] . a diagnosis of growing fracture skull ( posttraumatic leptomeningeal cyst ) was established , and surgical intervention planned . the neonate underwent duraplasty with pericranial graft and cranioplasty with titanium mesh plate 70 days after the head injury insult [ figure 4 ] . the infant tolerated the procedure well and was discharged after suture removal . on 6 months follow - up , the infant was doing well with no neurological deficits or wound - related complications . plain axial computed tomography scan showing a large 6 cm 3.2 cm 5.3 cm cystic lesion in the left temporoparietal region with underlying bone defect in the temporal bone ( black arrow ) intraoperative photograph showing titanium mesh plate with underlying pericranial dural graft head injury is likely to occur in children and adolescents ranging from 0 to 19 years in approximately 200 per 100,000 populations with a mortality rate of 29% , according to the national center for health statistic . different components , such as vasogenic and cytotoxic edema and possibly cerebral vasocongestion contribute to posttraumatic brain swelling . this brain swelling is associated with an exponential rise in the icp and eventually leads to brain herniation syndromes . in infants with open sutures , the relative increases in the icp and their tolerance remain unknown though there is a concern with regards to susceptibility to ischemic injury . any fracture extending across the cranial suture in an infant can cause bony separation leading to an open cranial vault . linear skull fracture in association with open sutures can lead to a condition wherein an effect similar to dc is achieved . in adults with a thick solid skull , the linear undisplaced fracture can not expand and therefore probably will not tolerate an increase in icp as well . the 2012 pediatric head injury guidelines state that decompressive craniectomy with duraplasty , leaving the bone flap out , may be considered for pediatric patients with traumatic brain injuries ( tbi ) who are showing early signs of neurological deterioration or herniation . hypothesized that early craniotomy ( within 6 h of injury ) in pediatric patients with refractory raised icp would result in better outcomes than following the historic standard of care , which reserved surgery as a final intervention in these patients . similar studies by kan et al . demonstrated good results with an early craniotomy . in our patient , we believe that the early dc achieved by linear undisplaced fracture and sutural diastasis prevented the development of raised icp leading to quick clinical improvement . adelson et al . has recommended the following criteria for selecting favorable patients for craniotomy in children : ( 1 ) diffuse cerebral swelling on cranial ct imaging ; ( 2 ) within 48 h of injury ; ( 3 ) no episodes of sustained icp > 40 mm hg before surgery ; ( 4 ) glasgow coma scale score > 3 at some point subsequent to injury ; ( 5 ) secondary clinical deterioration ; and ( 6 ) evolving cerebral herniation syndrome . while , our patient initially presented with diffuse cerebral swelling on ct , the infant was otherwise neurologically intact and showed no signs of raised icp . thus , an emergency surgery was not warranted , and early dural repair was planned . . found high rates of bone resorption following autologous bone grafting in pediatric tbi patients similar to our previous experience . due to this and the size of the cranial defect in our patient , we performed the cranioplasty using the titanium mesh plate . linear undisplaced fracture in neonates along with open sutures may act as a dc relieving the raised icp almost immediately . such infants in whom there are no signs of raised intracranial tension can be managed conservatively if the decompression effect achieved is adequate . hence , a close clinical and radiological follow - up is essential . as the effect of decompression
decompressive craniotomy ( dc ) is used to treat intracranial hypertension associated with traumatic brain injury . early dc is associated with better outcomes . we present a neonate with a history of fall with computed tomography scan showing a large frontoparietal contusion and associated parietal and temporal bone fracture . this acted as a spontaneous dc causing bony segment to separate due to which the edematous brain could be accommodated . despite the presence of a large contusion , the child was neurologically intact and medically managed . the neonate presented with a posttraumatic leptomeningeal cyst 2 months later , which had to be repaired surgically . we discuss how a linear undisplaced fracture acts as spontaneous dc and the role of early dc in improving outcomes .
epithelial - myoepithelial carcinoma ( emc ) is a rare biphasic low grade malignancy accounting for only 0.5% of all salivary gland tumors . there have been reports suggesting co - existence of another malignancy , like adenoid cystic carcinoma ( ad cc ) with emc , in the same tumor . if emc develops in a long standing case of pleomorphic adenoma ( pa ) , it is called emc - ex pa . ours is a case of a tumor in the submandibular and sublingual salivary glands diagnosed as emc , having clinical and histological resemblance to cellular pa . the present case report is about a 61-year - old woman who reported to us with a growth in the floor of mouth that gradually increased in size since last 3 years , without pain . on examination , a 3 cm 2.5 cm ovoid swelling was palpated lingual to the edentulous alveolar ridge , extending from midline ( lingual frenum ) to the right premolars [ figure 1 ] . it was a firm , non - tender , non - compressible , non - reducible , did not move with deglutition and showed no change in size with meals . complete hemogram , serum electrolyte levels , kidney function test and liver function test had all values within the normal range , but for a raised erythrocyte sedimentation rate ( 50 mm / h ) . magnetic resonance imaging revealed a 20 mm 18 mm 15 mm well defined well - marginated lesion in right sublingual space in the region of anterior aspect of sublingual gland [ figure 2 ] . it indented the inferior surface of tongue and right genioglossus muscle and did not cross midline . multiple mildly enlarged bilateral level ib , ii and iii lymph nodes were noted . a provisional diagnosis of pa of sublingual gland was made and local excision of the lesion along with the gland was planned under l.a . incision was placed from midline extending posteriorly near the alveolar ridge in order to protect the wharton 's duct . with careful dissection , , it was found that the mass involved the deep part of submandibular gland and duct with the sublingual gland being compressed by the mass . it was difficult to delineate the exact origin of the pathology ( sublingual or submandibular gland ) ; hence it was decided to sacrifice the sublingual gland , deep part of submandibular gland and its duct on the affected side [ figure 1 ] . initial histopathological examination , revealed a solid tumor tissue composed of myoepithelial and ductal cells presenting as double layered duct like structures with hyalinization of stroma . the epithelial component showed diversity in the form of sheets , nests and cords with different patterns of ductal arrangements and large areas of secretory material in the intervening areas , suggestive of cellular pa [ figure 3 ] . however since most of the tumors of submandibular and sublingual gland are malignant , a second examination of the specimen was undertaken . it showed that the entire tumor mass was covered by dense band of fibrous connective tissue . tumor cells were arranged in a background of predominantly mucous acini , as small tubules lined by cuboidal to oval cells containing prominent nuclei and eosinophilic cytoplasm and surrounded by clear cells and increased hyalinization [ figure 4 ] . on the basis of this dual cell population , a provisional diagnosis of emc was suggested . some focal areas were also observed with cells arranged in a cribriform pattern with pseudocystic spaces that pointed toward ad cc . immunohistochemistry showed p-63 reactivity in outer clear cells , s-100 and smooth muscle actin were focally positive and c - kit for ad cc was negative . hence , a diagnosis of hybrid carcinoma was ruled out and a final diagnosis of epi - myoepithelial carcinoma was made . there is a chance that being a long - standing tumor ; initial pa may have turned into emc . patient denied adjuvant radiotherapy ; is being followed - up since last 1 year and has no fresh complains or signs of recurrence until now [ figure 5 ] . magnetic resonance imaging of the lesion h and e section suggestive of cellular pleomorphic adenoma h and e section suggestive of epithelial - myoepithelial carcinoma post - operative clinical picture emc was first described by donath et al . , in 1972 and officially defined as a salivary gland tumor by who in 1991 . it commonly affects parotid gland ( 70% ) , sino - nasal glands , palatal and submandibular gland and rarer sites like lungs , breasts etc . , ( no reports on sublingual gland origin were found ) . it is a low - grade carcinoma of ductal origin characterized by its dual cell population of ductal epithelial cells and clear myoepithelial cells . apart from this characteristic picture , there are many histological variants such as oncocytic emc , double clear emc , emc ex pa , high grade or dedifferentiated emc , emc with myoepithelial anaplasia etc . many times , it is found that pre - operative fnac points toward a benign pathology ( pa ) . a diagnosis of pa would be incorrect due to the excessive hyalinization seen in the tissue , in spite of the pleomorphic picture of cells . differential diagnosis includes other clear cell tumors such as muco - epidermoid carcinoma , acinic cell carcinoma and sebaceous carcinoma , with immunohistochemical presence of myoepithelial markers , similar to those seen in emc . furthermore , there have been reports describing emc ex pa in parotid gland with the histological picture similar to ours . within the characteristic dual cell picture of emc , arrangements of cells in cribriform pattern with pseudocystic spaces reminiscent of ad cc were observed focally . , the tumor could have been a primary onset emc or an emc - ex - pa . there have been cases of recurrence of tumor by simple excision alone , hence we chose wider margins of healthy mucosa . the prognosis of emc is fairly good as the median disease free survival rate is reported to be 11.34 years . long - term follow - up is needed for detection of early signs of recurrence and proper management .
a 61-year - old female presented with a 3-year - old swelling in the right floor of mouth . clinical examination and fine needle aspiration cytology suggested a benign lesion . the mass was excised locally along with the involved sublingual and deep part of submandibular gland and duct . post - operative histopathological examination revealed features of pleomorphic adenoma . however , on revision of histological sections , features were predominantly of a rare malignancy of the salivary glands , epithelial - myoepithelial carcinoma ( emc ) , along with focal areas of adenoid cystic carcinoma ( ad cc ) . the tumor was p-63 , s-100 and smooth muscle actin positive but c - kit was negative , which ruled out ad cc and the possibility of a hybrid carcinoma . the aim of this article is to describe a rare case of emc in the floor of mouth and the confusing cytological picture that it created .
since couinaud 's segment viii is the largest segment of the liver and patients with hepatocellular carcinoma ( hcc ) often have impaired liver function , reduced anatomical resection of the ventral part of segment viii ( s8vent ) has been used as an alternative to full anatomical resection of segment viii in its entirety in actual clinical settings . anatomical resection of s8vent is generally considered a demanding procedure due to its anterosuperior location , its boundaries marked by tributaries of the middle or right veins and their tributaries , and the complete absence of anatomical landmarks on the liver surface , particularly in the cirrhotic liver . several techniques of anatomical resection have been proposed , including the technique involving puncture of the portal branch as proposed by makuuchi et al . that technique remains the most precise and practical method for anatomical demarcation on the liver surface . however , there are no accurate methods to identify the demarcation line inside the liver and divide the liver parenchyma along the intersegmental or intersubsegmental plane , which is not a straight line but rugged and irregular . the current authors have proposed a method of identifying the intersegmental and intersubsegmental border inside the liver to facilitate more precise anatomical subsegmentectomy . his liver function was normal , and his indocyanine green retention rate at 15 min was 6.5% . the tumor was located in the ventral part of segment viii ( s8vent ) ( fig . according to three - dimensional images ( iqqa - liver , edda technology , princeton , nj , usa ) , one tributary of the middle hepatic vein ( mhv ) , designated v8i , was running between s8vent and the dorsal part of segment viii ( s8dor ) . another tributary of the mhv , designated v8 - 5i , was running between s8vent and the ventral part of s5 ( s5vent ) ( fig . volumetric analysis indicated that the volume of the sviii was 297 cm ( 22.1% of the total liver volume ) and that the volume of s8vent was 165 cm ( 12.3% ) . after laparotomy and thoracotomy along the ninth intercostal space with a j - shaped incision , intraoperative ultrasonography was used to scan the entire liver to determine access to the vascular architecture and the tumor . thoracophrenolaparotomy facilitated mobilization of the right liver and provided excellent views of the insertion of the hepatic veins into the vena cava . guided by ultrasound , about 5 ml of indigo carmine dye ( indigocarmine injection 20 mg/5 ml ; daiichi sankyo , tokyo , japan ) was injected into the proximal part of portal branch of s8vent ( p8vent ) after clamping of the hepatic artery . the border of stained surface of the liver was marked using electrocautery and the tumor was located within the resection line ( fig . the locations of the aforementioned p8 vent , v8i , and v8 - 5i were then ascertained and marked on the surface ( fig . flow was intermittently occluded by clamping of the hepatoduodenal ligament , and the parenchyma was dissected using the clamp and crush method . the sequence of liver parenchymal transection is shown in fig . after the small tributary of v8 - 5i was exposed , it was followed all the way to the main trunk of the mhv . the portal pedicle of s8vent , which lay near the v8 - 5i , was then ligated and divided . after anatomical resection of s8vent , v8i and v8 - 5i were clearly exposed in almost their entirety and then preserved . the main trunk of the mhv between the two tributaries was also exposed ( fig . the operating time was 240 min and intraoperative blood loss was 50 ml . drains were inserted in the transected surface of the liver and right subphrenic space , and these drains were removed on day 6 postoperatively . an intercostal drain was left in the pleural space and removed on day 2 postoperatively . according to the oncological principle , it is reasonable to radically remove hcc by inclusion of the feeding portal area since the portal vein is the main gate for intrahepatic tumor spread . meanwhile , the volume to be resected is limited since patients with hcc often have background liver disease . anatomic segmental or subsegmental resection , taking into consideration both preservation , to the maximal extent possible , of liver functional parenchyma and eradication of intrahepatic metastasis , would be a theoretically reasonable procedure . several studies have demonstrated the superiority of anatomic resection in comparison to non - anatomic resection , showing better disease - free or overall survival . as for this patient , the tumor was located in the ventral part of s8vent , so the anatomical subsegmentectomy of s8vent was decided . however , anatomical segmentectomy , and especially s8vent resection , is technically demanding and two main difficulties exist . one relates to the demarcation of s8vent on the liver surface and the other relates to the direction of transection along the intersubsegmental planes inside the liver . several studies have described techniques for demarcation of s8vent on the liver surface . of these , the intraoperative ultrasound - guided portal branch puncture technique proposed by makuuchi et al . in the mid-1980s is still the most precise method . in relation to the direction of transection along the intersubsegmental planes inside the liver , sakairi and makuuchi proposed a method in which the portal pedicle is clasped and dye is injected . with this method , staining of the portal unit persists even after resection is complete , and the margin of the portal unit within the parenchyma is easily followed during transection . however , if a small branch of the portal vein is injured in an actual clinical setting , the dye spreads to the dividing planes and also stains the counter plane . moreover , a recent study showed that the subsegmental border visualized between the ventral and dorsal region always coincided with the plane of the v8i according to computer simulations , so the subsegmental plane can reasonably be divided along with v8i by preserving the very small tributaries near the liver surface and following them to determine where they meet as they run into v8i . the small venous tributaries meet along the way , gradually becoming a larger vessel that eventually joins the main three hepatic veins . the method is time - consuming but may be the only method to accurately divide the liver parenchyma along the intersegmental or intersubsegmental plane demarcation . in the current case , a tributary of the mhv ( v8 - 5i ) was found to be running on the segment border of s8vent and s5vent in three - dimensional images and it was detected and exposed during transection . the landmark vein of v8 - 5i in the transverse s8s5 intersegmental plane was determined for the first time since it had not been described in the literature . in recent practice , these subsegmental venous tributaries have been exposed as they are in anatomical studies . anatomical s8vent resection is technically demanding , and previously reported techniques for anatomical resection are not without their drawbacks . proposed here is a more accurate method of dividing the liver parenchyma along the intersubsegmental and intersegmental demarcation to facilitate more precise anatomical s8vent resection . 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 . xiang , dong , and makuuchi had done the study conception and design and drafted the manuscript . the acquisition of data was done by xiang , liu , dong , and sano .
highlightsanatomical resection of s8vent is generally considered a demanding procedure.there are no accurate methods to identify the demarcation line inside the liver by far.we proposed a new method to divide the intersegmental plane by exposing and following the small tributaries of hepatic vein near the liver surface.this method is time - consuming , but it is an effective way to divide the intersegmental plane .
a 10-year - old asian indian male child was brought to the emergency services of our tertiary care hospital with progressive bulging of the left eye for the past 7 days . it was associated with deep constant periorbital pain , inability to close the eye , fever with no rigors or chills , discharge , and decreased vision . the patient gave a history of scratching of boil over the left upper eyelid 3 days before start of symptoms . there was no history of upper respiratory tract infection , toothache , ear discharge , insect bite , or trauma . the patient had received oral penicillins before presentation at our center , but there was no relief . examination of the affected eye revealed visual acuity of 6/24 , hypoglobus , mechanical ptosis , restriction of ocular movements in all directions ; swelling of lids with overlying redness of skin with raised temperature and tenderness on palpation . anterior segment examination showed conjunctival chemosis and diffuse descemet folds while posterior segment examination was unremarkable . investigation showed hemoglobin of 13.6 g / dl , total leukocyte count of 12,500/mm , erythrocyte sedimentation rate of 45 mm in 1 h , and no pus cells in urine . noncontrast computerized tomography ( ncct ) scan showed ill - defined hypodense collection in the extraconal department of the left orbit , extending up to orbital septum and causing proptosis suggestive of orbital abscess . there was associated with thickening of superior rectus - levator palpebrae superioris ( sr - lps ) complex but no evidence of sinusitis [ fig . 1 ] . at presentation . ( a ) clinical picture , ( b ) ill - defined hypodense collection in the extraconal department of the orbit suggestive of orbital abscess , ( c ) thickening of superior rectus - levator palpebrae superioris complex , ( d ) clear sinuses on left a straightforward diagnosis of orbital cellulitis with abscess formation was made ; and intravenous amoxicillin ( 25 mg / kg / day in three divided doses ) and clavulanic acid and metronidazole ( 7.5 mg / kg / day in three divided doses ) and amikacin ( 15 mg / kg / day in three divided doses ) were started empirically . abscess was drained and pus samples were sent for microbiological examination . we were left in a fix when there was no improvement even after 3 days of treatment . microbiology report unveiled the mystery behind this odd behavior and guided us toward the correct path for further management . culture on blood agar showed greenish discoloration suggestive of alpha ( complete ) hemolysis while macconkey agar showed magenta - colored colonies . microscopy shows gram - positive spectacle - shaped cocci arranged at an angle to each other and in lines . the organism was catalase negative , grew on 6.5% sodium chloride , and produced black colonies on blood tellurite agar . sensitivity as per the clinical and laboratory standards institute revealed resistance to ciprofloxacin , penicillins , and cotrimoxazole and sensitivity to clindamycin , teicoplanin , linezolid , gentamicin , and doxycycline . after pediatric consultation , intravenous clindamycin ( 20 mg / kg / day in three divided doses ) and gentamicin ( 1.5 mg / kg / day in three divided doses ) were started . swelling and proptosis started reducing by day 2 of revised therapy . on day 10 , best - corrected visual acuity improved to 6/6 , swelling and proptosis disappeared , and extraocular movements became full and free . repeat ncct showed thickened lps - sr complex , with involvement of tendons suggestive of myositis [ fig . after 1 month of steroid treatment , there was improvement in the amount of ptosis [ fig . ( a ) greenish colonies on blood agar , ( b ) magenta colonies on macconkey agar , ( c ) black colonies on blood tellurite agar , ( d ) microscopy showing spectacle - shaped gram - positive cocci arranged at an angle to each other two weeks after treatment . ( a ) left persistent ptosis , ( b ) superior rectus - levator palpebrae superioris complex thickened with involvement of tendons . one month after treatment , ( c ) improving ptosis , ( d ) decreased thickness of superior rectus - levator palpebrae superioris complex after a thorough search of scientific literature , we could find only one case of orbital cellulitis caused by s. faecalis , reported by biedner et al . in 1986 . they reported a 2.5-month - old female who was hospitalized with septicemia , ethmoiditis , and orbital cellulitis and was managed with intravenous ampicillin . unlike the earlier reported case , our patient was healthy with no history of admission to a hospital . gamble suggested three routes for spread of infection : ( 1 ) extension from an adjacent tissue , ( 2 ) septicemia , or ( 3 ) a wound . in pediatric population , 90% of patients with orbital cellulitis have existing sinusitis while our patient had clear sinuses on ct scan . enterococcus is an emerging agent of upper and lower airway diseases , including paranasal sinuses . therefore , possibility of cases associated with e. faecalis which remained undiagnosed can not be ruled out . the undiagnosed cases may have simply responded to the therapy given , and no abscess formation may have taken place . enterococcus group was originally classified with streptococci as group d. lancefield classified hemolytic streptococci on the basis of nature of carbohydrate antigen on cell wall into twenty lancefield groups . on blood agar , they are usually nonhemolytic but may show alpha ( complete ) or beta ( incomplete ) hemolysis . on microscopy , they appear as oval cocci , arranged at an angle to each other or in lines . they can be distinguished from other streptococci as they grow in the presence of 40% bile , 6.5% nacl , 9.6 ph , at 45c , in 0.1% methylene blue and survive at 60c for 45 min . e. faecalis can be identified by its ability to produce black colonies on tellurite agar and ferment mannitol , sucrose , sorbitol , and esculin . chaudhry et al . in their review of 218 patients of orbital cellulitis reported 4 patients with persistent ptosis after resolution of orbital cellulitis and found delayed intervention to be the common factor . in our case , precise treatment was delayed for about 10 days pending the microbiological confirmation of the causative agent . thickening of lps - sr complex in our case suggests myositis of lps , which was responsible for persistence of ptosis . the material obtained from abscess drainage should be handled properly and sent for microbiological examination to identify the causative organism and sensitivity . this is especially important , like in our case , where the causative agent was an unusual one and would not have responded to the empirical therapy . an accurate diagnosis and early intervention are required to prevent the deadly complications of orbital cellulitis . we must understand that antibiotics need to be used judiciously to restrict the resistance being developed by the microbes .
orbital cellulitis is an infection of soft tissue behind the orbital septum . common pathogens isolated include staphylococcus aureus , staphylococcus epidermidis , and streptococcus pneumoniae . it is a straightforward diagnosis and usually responds to empirical treatment without any sequela . we report a case of orbital cellulitis caused by enterococcus faecalis , which was complicated by myositis of levator palpebrae superioris . to the best of our knowledge , only one case report exists dating way back to 1986 .