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guillain barr syndrome ( gbs ) is an acute autoimmune inflammatory demyelinating polyneuropathy that mostly affected the peripheral nervous system . gbs is clinically characterized by progressive symmetrical weakness of limbs , with or without autonomic or sensory disturbances . gbs may be induced by various factors , such as immunization , infection , trauma , or surgery . the pathogenesis of gbs was that neural antigens were cross - reactivated with antibodies to infections including cytomegalovirus , hemophilus influenzae , mycoplasma pneumonia , and herpes simplex . in this study , we reported a case of gbs in a 14-year - old patient with scoliosis . written informed consent was obtained from the patient 's parents on behalf of the child for publication of this case report and any accompanying images . a copy of the written consent is available for review by the editor of this journal . we present a 14-year - old patient admitted for a correction of his progressive scoliosis deformity . his spinal plain radiographs showed that the cobb angle of thoracic scoliosis was 114 ( fig . one week later , pain and weakness in the lower limbs , unsteady gait , and the inability to stand up from squatting position unassisted , developed , and progressed . two years later , the patient presented to the spinal service complaining of asymmetry of his shoulders . his spinal plain radiographs showed that the cobb angle of thoracic scoliosis was 114 , suggesting the need for surgical correction . magnetic resonance imaging revealed that cavernous hemangioma in the t4 ( thoracic 4 ) level of the spinal cord ( fig . , cavernous hemangioma resection was performed in the department of neurosurgery of our hospital and histological examination confirmed the diagnosis of cavernous angioma . magnetic resonance imaging revealed that spinal - cord cavernous hemangioma in the thoracic 4 level of the spinal cord . three months after the cavernous hemangioma resection surgery , a posterior correction and fusion at t5 ( thoracic 5)l5 ( lumbar 5 ) levels was performed , using the legacy spinal system ( usa ) . the total operation time was about 4 h. total amount of blood loss was 1200 ml and the amount of autologous blood transfusion was 650 ml . during the operation , the signal of this patient was normal using intraoperative spinal cord monitoring . postoperative plain x - ray film demonstrated a cobb angles correction from 114 to 45 ( correction rate 60.5% ) ( fig . his follow - up was symptomatic , well balanced in the coronal planes , with solid fusion ( fig . standing anteroposterior and lateral radiographs of 4 d after operation . standing anteroposterior and lateral radiographs of 12 mo after operation . gbs is an acute postinfectious autoimmune polyneuropathy , characterized by rapidly progressive , areflexia , and symmetrical limb weakness . the diagnosis of gbs depends on the clinical features including rapid development of areflexia , muscle paralysis , and albuminocytologic dissociation of cerebrospinal fluid . limited reports are available on neuromuscular scoliosis . in this study , we reported this case of a 14-year - old gbs case with scoliosis . in our case , lower limb pain occurred first , followed by weakness in the lower limbs , unsteady gait , and the inability to stand up from squatting position unassisted . the patient showed significant recovery in strength in lower extremities after treatment with intravenous immunoglobulin and steroids . some patients may develop neuromuscular complications such as neuromuscular scoliosis after acute onset of gbs . in this case , the patient presented to the spinal service with asymmetry of shoulders 2 years after the onset of gbs . as far as we know , there is no specific guideline for operations on patients with scoliosis secondary to gbs . the present case described a successful surgical management of a rapidly progressive scoliosis with contemporary posterior surgical instrumentation and fusion . in conclusion , gbs is a relatively rare syndrome described in recent years . doctors must keep in mind that neuromuscular scoliosis could develop secondary to gbs . when evaluating patients with acute inflammatory polyneuropathy , clinical examination of the spine is essential to identify patients with neuromuscular scoliosis .

abstractguillain barr syndrome ( gbs ) is an acute autoimmune inflammatory demyelinating polyneuropathy that mostly affects the peripheral nervous system . little is reported about spinal deformity associated with gbs . this study aims to present a case of scoliosis occurring in the setting of gbs.case report and literature review.the patient was a 14-year - old male with scoliosis . his spinal plain radiographs showed that the cobb angle of thoracic scoliosis was 114. history review revealed that he developed profound lower extremity pain , weakness , and numbness after catching a cold 5 years ago . these symptoms progressed to unsteady gait and inability to stand up from squatting position . the diagnosis of gbs was confirmed based on these symptoms . he underwent a posterior correction at thoracic 5lumbar 5 ( t5l12 ) levels using the ( legacy , usa ) spinal system . the cobb angle was corrected from 114 to 45 ( correction rate 60.5% ) . his follow - up was symptomatic , well balanced in the coronal planes , with solid fusion 12 months after the operation.neuromuscular scoliosis could develop secondary to gbs . when evaluating patients with acute inflammatory polyneuropathy , clinical examination of the spine is essential to identify patients with rare neuromuscular scoliosis .

generally pediatric elbow dislocations are complex traumas that are associated with fractures around elbow , medial epicondyle avulsion fractures and median nerve injuries . the purpose of this study is to present an 8-year - old patient who has open elbow dislocation without fracture accompanying with brachial artery injury . an 8-year - old boy had admitted to our emergency department after falling on his left hand with outstretched upper extremity . the patient had admitted to our clinic 30 min after trauma . in the clinical examination of the patient distal humerus and articular surface could be seen by looking through the wound ( fig . although neurological examination showed us no pathology , distal pulses could not be palpated in vascular examination . in the conventional radiographies , a posterior elbow dislocation without accompanying fracture because of the strong possibility of brachial artery injury , the patient had consulted to the vascular surgeon and an emergent surgical intervention had planned for this patient . the transverse incision had lengthened 1 cm and the buttonhole dislocation had released . following the exploration of the open wound in the elbow , open reduction was performed . brachial artery was identified in the open wound without continuity and distal pulses were still impalpable . the vascular surgeon had attended to the surgery and the brachial artery had repaired primarily . after the treatment of vascular pathology , the stability of the elbow was evaluated and the elbow was stable during 0140 of flexion and extension . due to the stability of the elbow , we decided to follow this patient with splint instead of an external fixator . the wound was closed in a standard fashion with the approval of vascular surgeon ; upper elbow splint was applied at 100. we did not observe any postoperative vascular or wound complications in the follow - up . after 4 weeks follow - up with splint , using angle adjustable hinge elbow orthotics started controlled active assistive exercises . by the end of the 12th week , the orthotics was removed and movement was completely allowed . the patient 's elbow range of motion , in supination pronation arc , has been detected as 8085 and in extension hastings class ii heterotopic ossification was detected between the anterior capsule and the brachialis muscles ( fig . posterior dislocation is the most common direction for these injuries and mostly there are associated fractures around elbow . elbow dislocation without fracture in pediatric patients is a very rare injury . according to our knowledge usually the trauma mechanism of elbow dislocation is falling on outstretched hand with elbow in approximately 30 of flexion . although this type of trauma mechanism is typical for supracondylar humerus fractures in pediatric age group , in our patient an open posterior elbow dislocation without fracture had occurred . concomitant vascular injuries and open dislocations are classified as complex dislocations that are often fracture dislocations . in these complex elbow dislocations associated pathologies like compartment syndrome , neurological injury ( 10% ) ( most often the median nerve ) , vascular injuries ( 68% ) , and complications like residual instability and heterotopic ossifications are observed in the treatment of these rare injuries . in the literature , complex open elbow dislocations without fracture have not been well described in pediatric population . in the pediatric population , sharma et al . presented 12-year - old boy who had a closed posterolateral dislocated elbow with a concurrent milch type ii lateral condyle fracture . medial epicondyle avulsion fractures may be associated with complex pediatric elbow dislocations . in some dislocated elbows the clinician should beware of possible entrapment of medial epicondyle in the joint especially after closed reduction . especially before the ossification of medial epicondyle ( under the age of 7 ) , mistaken of this pathology is more possible . pediatric elbow dislocations are rare injuries and the management of these injuries can be technically demanding due to concurrent neurovascular injuries . an open dislocation without fracture is very rare and it should be treated with immediate intervention , an effective teamwork and good rehabilitation . gkhan polat and gkhan karademir , hasan h ceylan helped in english language editing and writing the manuscript .

introductionelbow dislocations in children are rare injuries . these injuries are often in the form of complex injuries that is accompanied by the median nerve damage and medial epicondyle fracture in the pediatric age group . open elbow dislocation without fracture in the pediatric age group has been reported very rarely in the literature.presentation of casethe purpose of this study is to present an 8-year - old patient who has open elbow dislocation without fracture accompanying with brachial artery injury . in the clinical examination of the patient , there was an open wound in the transverse antecubital region . after repair of brachial artery injury , open reduction was performed under general anesthesia . in the postoperative clinical examination at 6 months , left elbow flexion was 140 , extension was full and there were no deficit in the supination and pronation of the forearm.discussionelbow dislocation without fracture in pediatric patients is a very rare injury . usually the trauma mechanism of elbow dislocation is falling on outstretched hand with elbow in approximately 30 of flexion . however our patient had fallen on outstretched hand with elbow in full extension . although this type of trauma mechanism is typical for supracondylar humerus fractures in pediatric age group , in our patient an open posterior elbow dislocation without fracture had occurred.conclusionpediatric elbow dislocations are rare injuries and the management of these injuries can be technically demanding due to concurrent neurovascular injuries . an open dislocation without fracture is very rare and it should be treated with immediate intervention , an effective teamwork and good rehabilitation .

teeth are complex anatomic structures that encounter with developmental anomaly in various aspects like defects in the structure , shape , size and number . fusion is a rare developmental anomaly with a complex morphology that can give rise to reduced esthetics , misalignment , dental caries and periodontal problems . the purpose of this article is to report a rare case of bilateral fusion of mandibular second premolar with supernumerary tooth . a 25-year - old male patient reported to the chettinad dental college hospital with complaint of pain on the left lower posterior tooth ( 35 ) which was decayed and had bizarre morphology similar to its counterpart on the right side ( 45 ) . both the teeth were larger on all aspects than the adjacent normal first premolar and had a pronounced buccal groove [ figures 1 and 2 ] . radiographic examination of 35 revealed pulpal involvement associated with a complex coronal and radicular pulpal anatomy . the orthopantomograph showed no evidence of missing tooth in the mandibular arch but presented bilateral impaction of third molars [ figure 3 ] . tooth 35 showing abnormal crown morphology and pronounced buccal groove partially erupted 45 showing abnormal crown morphology orthopantamograph showing abnormal tooth morphology in 35 and 45 along with impaction of 38 and 48 fusion is a developmental dental anomaly caused by the union of two tooth germs to form a single tooth . fused teeth are usually united by enamel and dentin , whereas the pulp chambers and pulp canals are either unified or separated . the etiology of fusion is still unknown ; however , the crowding of the tooth germs during their development can be an important factor . local metabolic disturbances or developmental aberrations of ectoderm and mesoderm during morphodifferentiation of tooth bud can be considered as etiological factors . they do not show any sex predilection but are more frequent among japanese population and american indians . fusion of teeth is more common in the deciduous dentition than the permanent dentition with a prevalence rate of 0.52.5% and 0.1% , respectively . literatures reveal the prevalence of bilateral fusion in primary teeth to be 0.010.04% and in permanent teeth as 0.05% . invariable of the type of dentition , majority of them are associated with the incisors and canine than the posterior arch . fusion of posteriors is infrequent in secondary teeth with a prevalence range from 0.08% to 0.5% . in general , the present case is a rarity as it occurred in the mandibular arch , in the posterior region with bilateral presentation , caused by the union of second premolar and a supernumerary tooth which was seldom reported . the number of teeth present is usually reduced in fusion , but is normal if the anomaly occur between a regular and supernumerary tooth . in contrast , gemination results in an apparent increase in the number of teeth , as they are caused due to the division of a single tooth germ to form two separate teeth . in these situations , even though there is no variation in the treatment plan , an attempt can be made to differentiate both the anomalies by performing a thorough clinical and radiographic examination . fusion between two teeth usually results in space gain or diastema but may not be the case when the anomaly involves a supernumerary tooth as seen in our case . the portion of the fused tooth that is formed by the supernumerary tooth is conical in morphology and is smaller when compared with other fused component that is separated by a developmental groove , whereas in gemination both the halves are mirror images of each other . the present case also revealed two disproportionate components with dissimilar morphology separated by a groove . according to which if the anomalous tooth is counted as two teeth and teeth count is normal in the arch , then it is considered as fusion . in cases where the anomalous tooth is counted as two teeth and if an extra tooth is present in the region , then it is regarded as gemination or a fusion between a normal and a supernumerary tooth . he also suggested referring teeth joined together by dentin as fused teeth . brook and winter proposed the neutral term intraoral radiographs can be considered but may not be confirmatory in the differentiation of double teeth as geminated teeth have a single large root canal , whereas fused teeth may have separate or fused root canals . in the present case , the anomalous teeth revealed an increased mesiodistal width , a single pulp chamber ; single root canal and normal teeth count in the respective quadrant as evident radiographically . . the buccal and lingual grooves may be deep and extend subgingivally favoring plaque accumulation leading to dental caries and periodontal diseases . the complex tooth morphology and pulpal anatomy , tooth position , and difficulty in rubber dam placement may negate endodontic treatment and necessitate surgical removal of the affected tooth . fusion of primary teeth may lead to hypodontia , malformation , impaction , delayed , or altered path of eruption of permanent successors . in conclusion , fusions are rare developmental anomaly and need to be recorded during routine clinical examination . the abnormal morphology demands prophylactic and early interceptive treatment in order to avoid the complicated pulpal and periodontal treatment related to these teeth . surgical extraction of the affected primary teeth can be an option to avoid its delayed exfoliation and the subsequent delayed or ectopic eruption of the successor .

fusion is the union of two normally separated tooth germs resulting in the formation of a single large tooth . the prevalence of this anomaly is less than 1% and most common in the primary dentition , in the incisor - canine region . fusions are almost always unilateral , but few cases of bilateral fusions have been reported . the purpose of this article is to report a rare case of bilateral fusion of mandibular second premolar with supernumerary tooth .

iga nephropathy ( igan ) is one of the most frequent forms of glomerulonephritis ( gn ) . polycythemia can be associated with igan , focal segmental glomerulosclerosis and membranoproliferative gn . here , we report a case of idiopathic erythrocytosis in a 31-year - old male who was incidentally detected to have hypertension during his pre - employment checkup . biochemical parameters revealed raised serum creatinine , and histological findings of the renal biopsy showed igan . a 31-year - old male was referred to nephrology in view of hypertension , renal dysfunction , proteinuria , and active urinary sediments . his clinical examination revealed evidence of bilateral pitting pedal edema and blood pressure ( bp ) of 160/110 mmhg . there was no history suggestive of polyarthralgia , rash , skin ulcers chronic cough and drug intake . complete blood count revealed hemoglobin of 19.2 g / dl , normal total count , and platelet count . serum creatinine was 2.2 mg / dl and uric acid 10.6 mg / dl . the liver function , uric acid , electrolytes , glucose , complement 3 ( c3 ) , and complement 4 ( c4 ) serum levels were normal . coagulation and the levels of antistreptolysin o and high - sensitivity c - reactive protein were also normal . antinuclear antibody profile was done , and antibodies to anti - myeloperoxidase , proteinase 3 , double - stranded dna , nucleosome and sjgren 's syndrome type a ( ss - a ) and ss - b antibodies tested negative . ultrasound revealed normal - sized kidneys with right kidney of 9.6 cm and left kidney measured 9.4 cm . radioisotope scan revealed a glomerular filtration rate ( gfr ) of 78 ml / min with a right and left differential of 33 ml / min and 35 ml / min , respectively . renal biopsy showed chronic iga nephropathy ( igan ) , with m , mesangial hypercellularity ; e , endocapillary proliferation ; s , segmental glomerulosclerosis / adhesion ; t , tubular atrophy / interstitial fibrosis ( mest ) scoring of m1 , e0 , s1 , t1 . the patient was treated with two sittings of phlebotomy , started on amlodipine 5 mg and ramipril 2.5 mg for control of hypertension along with allopurinol 100 mg and aspirin 150 mg for hyperuricemia and polycythemia , respectively . he was followed up every 2 weeks on outpatient basis . at the end of 8 weeks to the best of our knowledge , there have been 24 cases of polycythemia associated with renal disease described so far and out of them only ten have been associated with igan . the other renal lesions associated with polycythemia were focal segmental glomerulosclerosis , membranoproliferative glomerulonephritis , and rapidly progressive glomerulonephritis . furthermore , of the ten cases of polycythemia associated with igan described so far , all happen to be males . only three cases reported to have normal renal function whereas others had either moderate to severe renal dysfunction . several cytokines and growth factors play a role in the progression of renal disease in polycythemia ; one such report demonstrated abnormally upregulated mrna expression of platelet - derived growth factor and insulin - like growth factor . furthermore , the increased blood viscosity and blood volume leads to vascular microthrombi and glomerular capillary occlusion thus reducing the gfr . capillary occlusion leads to ischemia which eventually leads to chronic renal damage if not reversed on time . this is the 11 case of polycythemia associated with igan being reported and this is the first case of idiopathic erythrocytosis being associated with igan . striking similarities with other similar case reports are the preponderance in males ( all ten case reports being males ) and the presence of hyperuricemia , hypertension , and renal failure . there has been an increased presence of igan worldwide and it has become one of the leading causes of renal failure in young adults . hypertension and active urinary sediment is a common finding in most of these individuals , what makes this case report rare is the presence of idiopathic polycythemia as an additional cofactor . as only nine such reports have been made so far , it is essential to dig further into the pathogenesis of polycythemia in these individuals to know more about this causal relationship

we report a case of idiopathic erythrocytosis in a 31-year - old male who was incidentally detected to have hypertension during his preemployment checkup . urine routine showed proteinuria and hematuria . biochemical parameters revealed raised serum creatinine , and histological findings of the renal biopsy showed igan .

angioleiomyoma is a benign soft tissue neoplasm that originates from smooth muscle in the tunica media of arteries and veins . it can occur anywhere in the body , with predilection for the lower extremity , and can be found in the dermis , subcutaneous fat , or fascia . the tumor typically presents as a painful , solitary , small ( < 2 cm ) , slow growing , firm , mobile , subcutaneous nodule . angioleiomyoma of the knee is rare and only few cases have been reported so far . herein , we have described the first case report of giant angioleiomyoma of the knee with atypical presentation as a painless ulcer and have discussed the surgical management of the same . a 22-year - old male presented with a painless ulcer of 2 year 's duration on his left knee [ figure 1 ] . the lesion started as a small ulcer and gradually increased in size over the next 2 years . it was neither preceded by trauma nor associated with systemic symptoms like fever or weight loss . the ulcer was 10 8 cm in size , situated on the left knee , well - demarcated , non - tender , and was not fixed to any underlying structure . pre - operative photograph showing the giant ( 10 8 cm ) ulcerative lesion on the patient 's left knee total excision of the lesion with ligation of the feeder vessel , performed under general anesthesia , resulted in a large defect and exposure of the knee joint capsule [ figure 2 ] . the resulting defect was covered by raising an anteromedial thigh fasciocutaneous flap based on septocutaneous perforator , and the donor site defect was closed primarily [ figure 3 ] . the defect after excision of the tumor immediate post - operative photograph of reconstruction of the defect on microscopic examination , the tumor sections showed interlacing fascicles of smooth muscle cells encircling vascular lumina lined by normal - looking endothelial cells [ figure 4 ] . immunohistochemistry confirmed the diagnosis , the tumor being positive for smooth muscle actin ( sma ) , desmin , and vimentin , and negative for hmb45 . there has been no recurrence at 1 year follow - up , and the aesthetic outcome of surgery is satisfactory [ figure 5 ] . photomicrograph showing the tumor composed of numerous fascicles of smooth muscle cells encircling blood vessels h and e , 40 aesthetic outcome after 1 year of surgery the tumor usually presents in the third to fifth decade of life and has female preponderance . they have a predilection for the lower extremities , and the commonest site is the lower leg . the typical lesion is a solitary , small , slowly growing , firm , mobile , subcutaneous nodule . pain , often paroxysmal , is the most striking clinical feature of angioleiomyoma and is present in about 60% of cases . angioleiomyoma of the knee is rare , with only a few case reports in the literature . a case of intra - articular angioleiomyoma of the knee joint has also been described . our case has the following unique features as compared to the other reported cases of knee angioleiomyoma : ( 1 ) male patient ; ( 2 ) relatively younger age of presentation ; ( 3 ) giant size of angioleiomyoma of knee ; ( 4 ) presentation of angioleiomyoma as ulcerative lesion ; and ( 5 ) absence of pain . to our knowledge , this is the first case report of giant angioleiomyoma of the knee . more importantly , cases of angioleiomyoma presenting with an ulcerative lesion have never been described before . after an extensive literature search , we did not come across a single case report of angioleiomyoma of knee that presented with ulcerative lesion . finally , majority of angioleiomyomas of the lower extremity are painful , whereas , in our case , the tumor grew to a massive size but remained painless throughout . our case highlights for the first time the fact that angioleiomyomas may atypically present as an ulcerative lesion , rather than as a typical subcutaneous nodule . this is the first case report of giant angioleiomyoma of the knee , presenting uniquely as a painless ulcer . this case also highlights that angioleiomyomas may atypically present as ulcerative lesion , rather than as a typical subcutaneous nodule .

angioleiomyomas are benign tumors originating in the vascular smooth muscle . the tumor typically presents as painful , solitary , small ( < 2 cm ) , slow growing , subcutaneous nodule . angioleiomyoma of the knee is rare , and only few cases have been reported so far . we have described herein a giant angioleiomyoma of the knee presenting as a painless ulcer in a 22-year - old man . there was no intra - articular extension of the tumor , and total excision was curative . this is the first case report of giant angioleiomyoma of the knee as well as the first case report of angioleiomyoma presenting as a painless ulcerative lesion .

we present a case of a patient with cardiac tamponade , who presented with symptoms six weeks after the initial blunt trauma to his chest . a 63-year - old male presented to the emergency department , short of breath and feeling generally unwell for 2 days prior to admission . six weeks prior to admission , he had fallen off a ladder and fractured 2 ribs in the right hemi - thorax . four weeks later he was seen by his gp feeling chesty and was treated with a short course of antibiotics for a suspected lower respiratory tract infection . two days prior to admission , the patient felt increasingly unwell , with the symptoms of being cold and clammy at times . he had right lumbar and right upper quadrant pain , with the latter being more dominant . on admission to the ed , he was hypotensive ( 70/50 ) , tachycardic ( 112 ) but maintaining his oxygen saturations in air ( 100% ) and was afebrile . chest and abdominal plain film x - rays demonstrated healing rib fractures , with no consolidation , pneumo - thorax or haemo - thorax . he remained slightly tender in his right upper abdomen and lumbar regions but looked generally unwell ( sweaty , pale and restless ) . as a result of his persistent hypotension , tachycardia , partial response to resuscitation and acidosis on the arterial blood gases , a ct scan was arranged . the ct findings were a moderate to severe pericardial effusion , with a fluid rim of approximately 2 cm , around the whole pericardium ( as shown in fig . 1 ) . ct slice showing pericardial effusion the effusion was drained under ultrasound guidance . his blood pressure improved dramatically and stabilised , the patient felt immediately better , his breathing eased , and his shortness of breath settled . this case study appears to be the first report of cardiac tamponade that presented 6 weeks after the initial blunt injury . a search on pubmed ( delayed cardiac tamponade blunt trauma ) gave 28 results , of which only 4 were reports of delayed cardiac tamponade as opposed to other pathologies . of these four a second case , presenting three weeks ( 2 ) after blunt trauma , was believed to have developed secondary the commencement of an anticoagulant treatment . the remaining two cases ( 3,4 ) were at 2 weeks and eight days respectively . it is therefore clear that this presentation at six weeks is rare . in this case , other causes ( cancer , uraemia , spontaneous cardiac rupture and recent heart surgery ) were excluded . this case does demonstrate the need to have an index of suspicion of cardiac tamponade in patients presenting with symptoms and signs of being unwell , even if the insult ( blunt chest trauma ) was sustained a few weeks prior to their presentation .

cardiac tamponade is a recognised complication of blunt trauma to the chest . it usually presents at the time of the acute event but there are rare cases of delayed presentations . we present such a case where the tamponade occurred six weeks following the trauma to the chest wall .

they have a tendency to recur after initial treatment , including at sites outside the bladder such as the urethra . the urethra may be the site of recurrence after previous cystectomy but occur less frequently prior to cystectomy . patients with noninvasive disease in the bladder may prefer to keep their bladders and urethras to preserve normal voiding function . there have been a variety of treatments including urethral chemotherapy , resection , open surgery , as well as fulguration with the nd : yag or ho : yag laser to treat recurrence of uc . we describe a case wherein a distal urethral noninvasive uc was ablated using holmium laser technique ( ho : yag ) . the patient is a 83-year - old man with an history of noninvasive urothelial carcinoma 6 years prior to presentation previously treated with turbt and an induction course of intravesical of bcg therapy . he presented to the spinal cord injury unit after a cauda equina syndrome developed following lumbar spine surgery . due to his previous uc history , the initial office flexible cystoscopy demonstrated a noninvasive appearing uc covering approximately 50% of his pendulous urethra with frondular lesions . the patient was taken to the operating room for cystourethroscopy under anesthesia , biopsies and upper urinary tract evaluation . the upper urinary tracts were negative for lesions on retrograde pyelogram and bladder and urethral biopsies were obtained with similar pathology as seen on office cystoscopy . on pathology , the biopsies showed low - grade superficial urothelial carcinoma ( pta ) with a small focus of high - grade carcinoma [ figure 1 ] . surgical options were then discussed and the patient wanted a bladder sparing minimally invasive surgery . therefore , the patient was taken back to the operating room for a second look turbt and a staged fulguration of urothelial carcinoma with the holmium laser [ figures 2 and 3 ] . using holmium : yag laser vaporization through a 21-fr cystoscope , the lesions in the pendulous urethra were ablated using a 600 m fiber with settings of 8 hz and 1.0 joules ( 8 watts ) . a total time of 20 minutes was taken for the procedure with minimal discomfort postoperatively . mitomycin c was instilled ( 40 mg in 20 cc of sterile water ) for 1 hour immediately after the procedure . this was done due to his history of uc in the bladder as well as cells from the ablation may have been introduced into the bladder with the irrigation fluid . we subsequently performed the second stage of the procedure which included treatment of the other half of the lesions after one month once an office cystoscopy revealed there was no urethral stricture formation . after the second therapy he elected for bcg therapy and he has now been under surveillance for 18 months without cystoscopic evidence of a urethral recurrence and no formation of urethral stricture [ figure 4 ] . urothelial carcinoma of the pendulous urethra is exceedingly rare with minimal literature to provide accurate incidence . although a different scenario , urethral recurrence after cystectomy is approximately 9% which may give some insight into prevalence . while the primary treatment of recurrent uc is resection and or fulguration with or without the use of intravesical therapy , the efficacy in the urethra is unknown . we were concerned that resection would cause significant bleeding and the use of monopolar energy with the bugbee electrode would cause urethral stricture . therefore , we considered treatment with the holmium laser as it provides tissue destruction with a depth of penetration of about 0.5 mm but is very controlled . ho : yag laser therapy has been accepted as a means of fulguration in superficial bladder cancer . the holmium laser has also provided therapeutic use in urethral stricture disease . despite the use of holmium laser to treat lesions of the ureter , there have been no reports of diffuse fulguration of the urethra to determine if this may cause urethral stricture . previous reports suggest that using the ho : yag for ureteral fulguration of uc is effective and the stricture rate is less than 9% . the case was performed in a staged fashion and provided effective treatment without urethral recurrence .

urothelial carcinoma may occur anywhere in the urinary tract including the pendulous urethra . to prevent urethral stricture after resection and monopolor fulguration we describe the use of the holmium laser to fulgurate recurrent pta uc from the urethra . the surgical approach was staged and provided excellent long term results for management of superficial uc .

aneurysm formation after pancreatitis is well documented in the literature , caused by the digestion of arterial walls by the enzymes such as elastase released by the pancreas . rupture is generally considered to be a rare complication after pancreatitis , but the formation of aneurysms themselves is relatively common , occurring in 10% of patients following pancreatitis . endoscopic retrograde cholangiopancreatography ( ercp ) is a common procedure used both as a diagnostic and therapeutic tool in patients with biliary tree obstruction . it is considered a safe procedure and serious complications are rare ; with the most common complications being pancreatits , cholangitis , bowel perforation , and bleeding from the ampulla of vater following sphincterotomy . however , although far rarer than any of the above the following case illustrates life - threatening and therefore significant complication following ercp . we present the case of a 75 year old female admitted with acute pancreatitis , deranged liver function and gram - negative rod septicaemia . she was admitted to intensive care and magnetic resonance cholangiopancreatography carried out to determine the aetiology of the pancreatitis . this showed a 2 cm filling defect in the dilated lower end of the common bile duct , suggestive of an impacted stone . endoscopic retrograde cholangiopancreatography ( ercp ) was performed 10 days later , but showed an undilated biliary system with no filling defects ; despite trawling the common bile duct several times . subsequently the patient had several episodes of haematemesis and malaena , with the patient s haemoglobin repeatedly dropping despite transfusion . oesophogastroduodenal ( ogd ) endoscopy and computerised tomography ( ct ) scan failed to identify a source . the only positive finding on ct scan was pneumobilia , most likely due to sphincterotomy during the ercp . the haematemesis continued and emergency laparotomy was performed . on external examination of the stomach and duodenum pyloroduodenotomy identified a small healed duodenal ulcer and fresh clots in the first part of duodenum . on palpation of the third part of duodenum , a periampullary diverticulum was identified , although it was not bleeding . a second urgent ogd endoscopy showed more blood clots and fresh blood in the fundus , but once again no source for the bleeding was seen . this showed a 1 cm pseudoaneurysm of the right hepatic artery in the right lobe close to the porta hepatis . the pseudoaneurysm arose from the anterior branch of the right hepatic artery and communicated with the bile duct . the 2 part of the duodenum was opacified , showing haemorrhage from the lesion into the duodenum via the bile duct ( figure 1 ) . initially , selective embolisation of the anterior right hepatic artery was performed using small coils ( cook uk ) and small particles of grated gelatin sponge soaked in 0.5ml of 5% ethanolamine . however this proved to be ineffective ( figure 2 ) , and so the posterior branch of the right hepatic artery was embolised , successfully controlling the haemobilia . there were signs of recurrent haemorrhage such as fluctuating serum haemoglobin levels and some ongoing malaena . however , these ceased spontaneously after a few days , and the patient subsequently made an uneventful recovery . the formation of pseudoaneurysms after pancreatitis is very common , although most of these will not rupture and therefore remain asymptomatic . cases of haematemesis following spontaneous fistulation into the biliary tree after pancreatitis are described in the literature ; although often they have presented after a more severe necrotising pancreatitis . similar presentations have also been described with a different pancreatic source , such as a bleeding pancreatic pseudocyst . given that the haematemesis commenced following ercp in this case , it is likely that it was the instrumentation of the common bile duct which led to the fisulation of the aneurysm into the biliary tree . this theory is feasible as the branches of the hepatic artery and portal vein are in very close proximity to the bile duct allowing concurrent injury to occur . we hypothesize that during the ercp procedure the walls of the intra - hepatic bile duct and paralleling artery were injured during steel guide wire maneuvering , causing the false aneurysm . it is important to recognize that iatrogenic causes are the most common cause for haemobilia ( 52% ) . we also assume the bleeding in the acute recovery phase post embolisation was mainly due to the dissolving nature of the gelatin sponge . however , even though a fraction of the particles may have dissolved , the bleeding subsequently stopped as low enough perfusion pressures were maintained to stabilize the lesion . ogd endoscopy is of course the first line investigation in any patient presenting with haematemesis , but in this case was unhelpful because there was no blood discharging from the ampulla of vater at the time of investigation . ct scanning lacked the sensitivity required to detect the 1 cm pseudoaneurysm described in this case , though it has been successfully used in the diagnosis of larger aneurysms in other cases . angiography is considered the gold standard in the diagnosis of this condition , and it is important learning point to remember this as the next line of investigation of haematemesis , particularly in patients with a history of pancreatitis . in this case there was a delay before an angiogram was done , and this delay was very nearly fatal for the patient . although this case report describes a rare complication of pancreatitis , it is a complication that is readily treatable provided the diagnosis is made in time . therefore we publish it in the hope that clinicians will think of it in patients with upper gastrointestinal bleeding of unknown origin .

we present a case of pancreatitis with secondary pseudoaneurysm formation in a branch of the hepatic artery , which fistulated into the common bile duct following endoscopic retrograde cholangiopancreatography . this aneurysm manifested itself clinically with recurrent haematemesis , and was treated by embolisation of the anterior and posterior branches of the right hepatic artery .

in june 2004 , a 58-year - old asian female presented with anxiety , palpitations and breathlessness . differential leukocyte count revealed blasts , promyelocytes , myelocytes , and metamyelocytes with decreased leukocytes . liver function test ( lft ) and kidney function test ( kft ) were normal . the patient was started on imatinib 400 mg and folic acid 5 mg once daily in august 2004 . peripheral smear remission was seen in 2 months , i.e. , tlc returned to normal value and hemoglobin and platelet counts normalized . one year later , the patient presented with bilateral redness of eyes and facial swelling which was treated with antihistamines . following this , 1 month later , the patient presented with bilateral parotid swelling which was not evaluated . parotid swelling resolved of its own within a month without any treatment . in august 2005 , bone marrow biopsy revealed normocellular marrow spaces with adequate representation of all three marrow elements , i.e. , complete hematological remission . triiodothyronine ( t3 ) , thyroxine ( t4 ) , and thyroid - stimulating hormone were normal , but thyroid microsomal autoantibodies ( tma ) antibody was positive . fine needle aspiration cytology was planned , but the patient did not consent for the investigation . most common adverse events reported with imatinib are edema , nausea , muscle cramps , musculoskeletal pain , diarrhea , rash , fatigue , and headache . this case illustrates a possible occurrence of imatinib - induced autoimmune thyroiditis . in a case of thyroiditis , the differential diagnosis of thyroid pain includes acute , subacute thyroiditis , chronic thyroiditis , hemorrhage into a cyst , malignancy including lymphoma , and rarely , amiodarone - induced thyroiditis or amyloidosis . the absence of small tender asymmetric goiter , fever , dysphagia , and erythema over thyroid rules out bacterial or fungal causes of thyroiditis . the patient had bilateral parotid swelling 6 months before the presentation which resolved of its own . the absence of fever and presence of tma ruled out mumps as a cause of thyroiditis . finally , the positive response to treatment with corticosteroids also ruled out infection and malignancy as a cause of pain in thyroid . thus , only possible cause can be autoimmune thyroiditis which responded well to the treatment with corticosteroids . imatinib was started in august 2004 , and the patient presented with the thyroiditis in november 2005 establishing the temporal association of thyroiditis with imatinib . use of naranjo 's probability scale and who causalty scale assessment indicated imatinib as a possible cause of thyroiditis because the idiopathic cause of autoimmune thyroiditis can not be ruled out . sunitinib , a tk inhibitor , a drug of same class has been implicated as a cause of lymphocytic and destructive thyroiditis . a review the literature of studies of thyroid dysfunction induced by tk inhibitors showed that thyroid dysfunction is not a rare entity with the use of tk inhibitors although not so common with the use of imatinib . as there was possibility of progression of cml when drug is withheld , drug withdrawal was not done to check the possible causation of thyroiditis by imatinib . second , there was no increase in the thyroid hormone levels and the severity of thyroiditis did not mandate drug withdrawal . hence , a possibility of activation of autoimmune diseases with the use of imatinib can not be ruled out . hence , clinicians should be aware of the possibility of thyroiditis with imatinib and should have an eye not to miss the event .

here , we present a case of chronic myeloid leukemia for which imatinib therapy was initated . triiodothyronine ( t3 ) , thyroxine ( t4 ) , and thyroid - stimulating hormone was normal , and thyroid microsomal autoantibodies ( tma ) were positive and patient was diagnosed as thyroiditis treated with corticosteroids for 1 months which lead to resolution .

mucous membrane pemphigoid ( mmp ) is a putative autoimmune , chronic inflammatory , subepithelial blistering disease predominantly affecting mucous membranes . mucous membranes that may be involved include the oral cavity , conjunctiva , nasopharynx , larynx , esophagus , genitourinary tract and anal canal . a 37-year - old male patient presented with complaints of redness and pain in his left eye since two years , painful erosions in the oral cavity since six months , and ulcers over the scrotum and right knee since two months . on ocular examination , there was congestion of the left sclera mainly on the lateral side along with a fibrotic band connecting the upper palpebral conjunctiva with the bulbar conjunctiva near the lateral canthus [ figure 1 ] . on examination of the oral cavity there were widespread erosions involving the inner side of lips , gingival , and labial mucosa [ figure 2 ] . detailed examination revealed a single depigmented macule over the glans penis [ figure 3 ] , which according to the patient appeared insidiously around 8 years ago and remained static . a tzanck smear from the oral erosions , done to rule out pemphigus vulgaris , failed to show any acantholytic cells . in addition there was involvement of nasal mucosa , larynx , scrotal skin , perianal skin , and right knee [ figure 2 ] . a skin biopsy from the right ear showed subepidermal bullae and fibrosis beneath it [ figures 4 and 5 ] . the direct immunofluroscence of perilesional uninvolved skin showed strong linear deposits of igg along the basement membrane zone . detailed hematological , biochemical , and imaging studies did not reveal any evidence of malignancy . the long - standing depigmented macule over the glans penis did not show any evidence of atrophy , ruling out lichen sclerosus et atrophicus . mmp lesions affecting the left eye mmp lesions affecting the oral mucosa , scrotum and knee ( clockwise ) classical vitiligo affecting the glans penis subepidermal bulla with fibrosis beneath it , black arrow demonstrates the site of blistering ( h and e , 10 ) subepidermal bulla ( h and e , 40 ) mmp is described as a heterogeneous group of chronic , inflammatory , mucous membrane - dominated , subepithelial blistering diseases that manifest a varying constellation of oral , ocular , skin , genital , nasopharyngeal , esophageal , and laryngeal lesions . life - threatening airway obstruction and sight - threatening ocular scarring can occur in this condition , also known as cicatricial pemphigoid , benign mmp and incorrectly as ocular pemphigoid . these include bullous pemphigoid antigen 1 ( bpag1 , 230 kda ) , bullous pemphigoid antigen 2 ( bpag2 , 180kda ) , laminin 5 , laminin 6 , 6-integrin subunit , 4-integrin subunit , collagen vii , and other proteins of unknown identity and/or function . scarring is common at non - oral sites of involvement , contributing to disease - related morbidity . the choice of agents for treatment of mmp is based upon the sites of involvement , clinical severity , and disease progression . found there is an association with autoimmune diseases , both organ and non - organ - specific in a group of 34 patients with cicatricial pemphigoid suggesting a possible genetic basis for the association . autoimmune mechanisms with an underlying genetic predisposition are the most likely causes of vitiligo , although neurohumoral and autocytotoxic hypotheses are alternative theories or contributing mechanisms . previously association of vitiligo with bullous pemphigoid either alone or along with other autoimmune diseases have been reported . gaspar et al . reported a case of a 68-year - old woman with vitiligo , primary hypothyroidism and cicatricial pemphigoid with severe laryngeal involvement necessitating tracheostomy . a thorough search failed to reveal any other report of co - existence of these two conditions in the published literature . we believe that this is the first case report of vitiligo coexistent with mucous membrane pemphigoid from india . the co - existence of these two conditions is probably is related to their common autoimmune etiology .

mucous membrane pemphigoid describes a rare heterogeneous group of chronic , inflammatory , mucous membrane - dominated , subepithelial blistering diseases that manifest a varying constellation of oral , ocular , skin , genital , nasopharyngeal , esophageal , and laryngeal lesions . life - threatening airway obstruction and sight - threatening ocular scarring can occur in this condition , which is rarely reported in indian literature . vitiligo is another acquired autoimmune disorder characterized by loss of melanocytes . vitiligo is associated with a number of disorders also considered to be autoimmune . here we report a very rare coexistence of mmp and vitiligo , the first such report from india .

gastric volvulus can be acute , acute on chronic or chronic , and may be primary or secondary to anomalies that affect the fixity of the stomach like hiatus hernia , eventration of diaphragm or congenital diaphragmatic hernia ( cdh ) . the association of bronchopulmonary foregut malformations such as congenital cystic adenomatoid malformation of lung and bronchopulmonary sequestration ( bps ) with cdh has also been well described in the literature . herein , we describe a 4-year - old girl with a rare presentation of gastric volvulus and pulmonary sequestration in association with cdh . a 4-year - old girl presented to our casualty with upper abdominal pain , distension , and nonbilious vomiting for the last two days . her mother gave a history of recurrent episodes of similar symptoms every month for the last six months , and each episode lasted for 4 - 5 h. the symptoms used to diminish spontaneously . her perinatal history was unremarkable . a preliminary work up for her past symptoms in another hospital revealed that she had a suspected left sided eventration as seen on the chest x - ray [ figure 1a ] . on examination , the child was lethargic , obtunded , had cold peripheries with tachycardia , hypotension , and feeble pulse . a nasogastric tube insertion was attempted that failed initially but subsequently drained hemorrhagic gastric contents . a skiagram [ figure 1b ] at this stage revealed a gastric bubble , with a paucity of distal bowel gas and a raised left hemi diaphragm . ( a ) radiograph of the abdomen prior to presentation at our institute showing evidence of viscus in left hemithorax . note the paucity of bowel gas excluding the single stomach gas shadow suggesting the possibility of a volvulus after adequate resuscitation , the child was explored , and the intra - operative findings revealed a mesoaxial gastric volvulus . after successful reduction , it was noted that the stomach was grossly healthy except the greater curvature , which was congested . there was a large defect in the posterolateral aspect of the left hemi - diaphragm , which was covered with a sac and the diaphragm had a reasonably well - developed rim of muscle along the anterior segment [ figure 2a ] . ( a ) defect in the diaphragm is seen with well - defined anterior lip ( arrows ) . ( b ) intraoperative image of the mass seen to occupy the cranial aspect of the diaphragmatic hernia sac ( cut edges indicated by arrows ) to repair the defect the sac was excised and it was noted that the cephalic surface of the sac had a lobular 6 cm 3 cm fleshy pedicled mass flimsily attached to it [ figure 2b ] . the pedicle of the blood supply was traced and it was found to be entering the mediastinum . the repair was completed by primary interrupted sutures , and a three - point gastropexy was done to prevent recurrences . the histopathology report of the mass indicated that it was an extra - pulmonary bps . the stomach is prevented from rotating along its axis by the ligamentous supports like the gastrocolic , gastrohepatic , gastrophrenic and the gastrosplenic ligaments . the gastroesophageal junction and the pylorus are two additional fixed points that afford protection against volvulus . when there is a laxity in these supports and the stomach rotates more than 180 , a primary gastric volvulus results . other conditions like hiatus hernia and cdh predisposes to secondary volvulus . based on the two axes along which this rotation takes place it could produce either an organo - axial , mesentero - axial or a mixed volvulus . it has been noted from the literature that the secondary gastric volvulus in children usually is associated with the mesentero - axial type of volvulus as was seen in our case . an astute clinician can diagnose acute gastric volvulus by the typical complaint of sudden onset upper abdominal pain with retching and inability to pass a nasogastric tube - the borchardt 's triad . this symptom complex was also present in our case except for retching , but the radiographs endorsed our clinical suspicion . the exploration revealed a mass on the superior aspect of the sac , which looked like an extrapulmonary bronchopulmonary sequestration ( ebps ) . these are non - functional pulmonary tissues with the systemic blood supply that seldom communicate with the tracheobronchial tree . bps comprise approximately 6% of all congenital malformations , and these are associated with cdh in 30 - 40% of cases . this distinction is made depending on whether or not the visceral pleura invests the sequestered lesion , with the latter referred to as ebps . although the above associations with cdh are common but cdh presenting with acute gastric volvulus and with an underlying ebps is very rare in pediatric age group and is described only once in english literature . had reported a case similar to ours except that the child had an eventration of the diaphragm . another case report in spanish describes an adult with bochdalek 's hernia with stomach volvulus and extrapulmonary sequestration presenting as acute respiratory distress . the aim of this report is to reinforce pediatric surgeons to look for underlying secondary causes of gastric volvulus as most of them can be tackled simultaneously . an awareness of this triad of cdh , gastric volvulus , and bps can result in early detection and safe resection of bps and thus avoid future diagnostic dilemmas .

congenital diaphragmatic hernia ( cdh ) is a known cause of secondary gastric volvulus . it is also known that bronchopulmonary sequestration ( bps ) may be associated with cdh . an extremely rare case of bps associated with gastric volvulus in a girl with left sided cdh is being reported .

imatinib , a specific tyrosine kinase inhibitor has shown excellent efficacy in management of chronic myeloid leukemia ( cml ) . there may be almost complete remission hematologically in the chronic phase of management of cml due to this drug . most commonly reported adverse events are maculopapular eruptions , periorbital edema , and the less commoner ones include steven johnson syndrome - toxic epidermal necrolysis ( sjs / ten ) , acute generalized exanthematous pustulosis , hypopigmentation , lichenoid reaction , pityriasis rosea , and sweet 's syndrome . a 52-year - old male presented to the medical outpatient department with complaints of low grade fever and lump on the left side of abdomen since one month duration . bone marrow examination revealed 2% blast cells , serum lactate dehydrogenase was 1134 iu / l , and an ultrasound of the abdomen revealed splenomegaly with span of 19 cm . the case was diagnosed as cml in chronic phase and patient was prescribed imatinib mesylate 400 mg once daily . ten days later , he developed redness and scaling involving dorsum of both hands which increased to involve his entire body over a period of 20 days . there was no history of any other drug intake , or history of fever , jaundice , chest pain , palpitation and dyspnoea on exertion . imatinib was continued and the rash worsened . when the patient was referred to us , he had generalized skin rash of 40 days duration . general physical examination was unremarkable while systemic examination revealed splenomegaly , 3 cm below costal margin . dermatological examination revealed generalized involvement of the body in form of dusky , blanchable erythema , and diffuse fine scaling . lichenification was noted in flexures viz groin , axillae and neck [ figures 1 and 2 ] . investigations revealed hemoglobin of 14.5 gm% , total leukocyte count 11,500/mm , and differential count was polymorphs- 50 , lymphocytes- 8 , monocytes- 2 , and eosinophils 40 . urine examination , blood sugar , renal , and liver function tests were within normal limits . extensive scaling of ( a ) neck ( b ) axillae and upper chest involvement of ( a ) lower limbs and ( b ) both forearms imatinib was immediately stopped and patient was prescribed on tablet prednisolone 40 mg / day , which was maintained at full dose for two weeks , tapered by 10 mg every week , and stopped after a total of five weeks . supportive measures including high protein diet , appropriate temperature control , fluid , and electrolyte balance were provided . improvement was noted with decreased erythema on fifth day and significant reduction in scaling by day 11 . the patient was changed to an alternative anti - cml medication ( cyclophosphamide based ) and rechallenge or desensitization was not resorted to , as erythroderma is considered a severe form of drug reaction . despite common occurrence of cutaneous adverse event with imatinib , severe adverse cutaneous drug reactions are uncommon and seen in 5% of cases . acute generalized exanthematous pustulosis , epidermal necrolysis , and steven johnson syndrome have been reported previously . other rare reactions mentioned include mycosis fungoides like eruption , follicular mucinosis , porphyria cutanea tarda , neutrophilic eccrine hidradenitis , eccrine squamous syringometaplasia , and panniculitis . there are only six probable cases of exfoliative dermatitis due to imatinib reported in literature . exfoliative dermatitis generally occurs 1 - 3 wks after starting therapy on initial exposure and within hours to days on rechallenge . mechanism of development of rash after imatinib administration is not known , however , hypersensitivity reaction as a mechanism has been postulated . in this case causality assessment using naranjo scale showed that imatinib was the probable cause for the adrs ( score 7 ) . most of the rashes due to imatinib are self limiting and do not require discontinuation of treatment . desensitization therapy can be used by administering increasing doses of drug in cases of mild reactions . erythroderma necessitating stoppage of therapy is considered as grade 4 toxicity according to national cancer institute / national institutes of health ( nci / nih ) common toxicity criteria . it is important to recognize the cutaneous adverse effects of this drug , so that severe and potentially life threatening adverse reaction can be identified early and remedial action can be taken early . it is equally important to continue therapy in minor cutaneous adverse events so that patient can get benefit from treatment with this gold standard drug for cml .

imatinib , a specific tyrosine kinase inhibitor is a newer anticancer agent , which has shown excellent efficacy in managing chronic myeloid leukemia . it is generally well tolerated with few side effects . most commonly reported adverse events are maculopapular eruptions and periorbital edema . severe adverse reactions are seen in 5% of patients . exfoliative dermatitis has been very rarely reported with this drug . we report a case of a 52-year - old male who initially presented with a maculopapular rash and developed erythroderma on continuation of the drug .

criss - cross heart ( cch ) , or superoinferior ventricles , is a complex congenital rotational abnormality in which the systemic and pulmonary venous streams cross at the atrioventricular ( av ) level without mixing . its frequency is less than 8/1000000 and accounts for < 0.1% of congenital heart defects ( chd ) . in the normal heart , av structures are parallel to each other when viewed from the front , whereas in cch the av structures are not parallel but angulated by as much as 90 degrees.14 the atrium connects with the contralateral ventricle and the ventricular chambers are arranged in a superoinferior fashion , with the right ventricle ( rv ) superiorly and the left ventricle ( lv ) inferiorly located , regardless of whether the av connection is concordant or discordant . the diagnosis is made by using 2-dimensional and color doppler echocardiography.35 here , we report a rare case of cch with concordant atrioventricular connections with dorv which was diagnosed by echocardiography . a 2 month old female infant , weighing 3.7 kg presented with history of breathlessness , feeding difficulty and cyanosis . she was a second product of non - consanguineous marriage and born by normal vaginal delivery . respectively . blood pressure was recorded at 75/35 mmhg and oxygen saturation 85% in room air . cardiac auscultation revealed normal first and second heart sound along with a grade 3/6 ejection systolic murmur audible best at the left upper parasternal area . the two - dimensional echocardiography , subcostal coronal views demonstrated the connection of the left - sided left atrium and the right - sided left ventricle through the mitral valve and the right - sided right atrium to be connected to the left - sided right ventricle through the tricuspid valve by anterior angulation of the transducer . it also shows the two great arteries arising from the right ventricle ( figure 1 ) . subcostal coronal views shows the connection of the right - sided ra to be connected to the left - sided rv through the tricuspid valve , inlet vsd and the two great arteries arising from the rv . short - axis views shows the right ventricle was superior and left ventricle was inferior with horizontal position of the ventricular septum ( figure 2 ) . short - axis views shows the superior ( rv ) and inferior ( lv ) ventricle with horizontal position of the ventricular septum . the standard 4-chamber view was not showing simultaneously all four chambers and both atrioventricular valves ( figure 3 ) . a large size inlet type of ventricular septal defect ( bidirectional shunt ) and a moderate size ostium secundum atrial septal defect ( bidirectional shunt ) were present . cch is a complex congenital anomaly produced by the rotation of ventricular mass along its long axis . this positional anomaly can coexist with a horizontal displacement of the ventricular mass along the horizontal plane of long axis , which produces superior - inferior ventricles . in 1961 , lev and rowlatt described unusual arrangement of the cardiac inlets that is ventricular chambers arranged in a superoinferior fashion , with the rv superiorly and the lv inferiorly.2 in 1974 , anderson et al used the term cch for cardiac anomaly producing the illusion of crossing of the systemic and pulmonary venous stream without mixing at the av level.3 in the later years , other cases was described with situs solitus or situs inversus or isomerism , mostly with av concordance.45 the physiology is determined by the concordant or discordant atrioventricular ( av ) and ventriculoarterial ( va ) connection and the associated cardiac defects.67 cch may be seen in three forms , that is complete transposition , corrected transposition , and normal hearts . in our case , there was av and va concordance with malposed great arteries . a review of the medical literature does not reveal any isolated presentation of cch cases . most patients with cch has other anomalies such as vsd , transposition of great vessels , right ventricular hypoplasia , subpulmonary stenosis , straddling av and others.78 the diagnosis should be suspected by echocardiography when the parallel arrangement of the av valves and ventricular inlets can not be achieved , and the two valves are not easily visualized simultaneously on apical 4 chamber view . color flow mapping can help in assessing the av connection , visualization of the direction of intracardiac blood flows and recognition of the crossover of the inflow streams.910 yang yl et al11 reported that the failure to obtain a characteristic 4-chamber view was diagnostic for recognition of the cch . the echocardiographic features suggested by yang yl et al11 were present in our case . surgical options vary according to the exact sequential segmental analysis and associated abnormalities . in conclusion , cch is a rare cardiac anomaly than can be diagnosed by a transthoracic echocardiography by an alert echocardiographer to determine the relationships of the cardiac chambers and associated cardiac anomalies .

crisscross heart ( cch ) is a rare cardiac malformation characterized by crossing of the inflow streams of the two ventricles due to an apparent twisting of the heart about its long axis . the developmental mechanisms and causes of cch are remaining unknown . neonates mainly presents with cyanosis and a systolic murmur . we herein present a case of cch with concordant atrioventriculo connections with double outlet right ventricle ( dorv ) which was diagnosed by echocardiography .

while abdominal pain is a common presentation in patients with sickle cell disease , its progression to an acute surgical abdomen is relatively rare . only a few case reports describing ischemic bowel secondary to sickle cell crisis have been published . ischemic bowel secondary to sickle cell disease has been recognized with increasing frequency [ 1 , 2 , 3 ] . most patients with ischemic bowel are elderly and often have a past medical history significant for atherosclerotic disease manifesting with congestive heart failure or cardiac dysrhythmias . in contrast , patients with sickle cell disease are considerably younger , often with no history of cardiovascular disease . a 42-year - old african - american man with a past medical history of hypertension , end - stage renal disease , and mechanical heart valve was admitted to the hospital in sickle cell crisis with complaints of chest and bilateral upper extremity digit pain . cardiac work - up showed an ekg with st depression in the left lateral wall leads . cardiac catheterization was performed , but demonstrated no significant coronary artery lesions and required no further cardiac intervention . the patient had an elevated amylase and lipase of 456 and 334 u / l , respectively . electrolytes were within normal limits except for a total bilirubin of 2.1 mg / dl . as such , the patient was managed conservatively and eventually started on oral intake . once the patient was started on a regular diet , he developed acute generalized abdominal pain which was similar in his estimation to previously experienced pain associated with sickle cell crisis . upon examination , nevertheless , his examination revealed a tense , distended abdomen which revealed diffuse rebound tenderness and guarding . complete blood cell count demonstrated a white blood cell count of 16,200 with 80% neutrophils . an upright abdominal film demonstrated pneumoperitoneum . the patient was immediately given intravenous piperacillin / tazobactam , hydrated aggressively , and taken to the operating room for exploration . intraoperatively , evaluation of the small bowel demonstrated 3 lesions that were grossly infarcted and leaking bile . the ischemic regions and infarcted / perforated portions of duodenum and jejunum were resected with primary anastomosis . the patient 's operative course was uneventful and he was transferred to the intensive care unit for recovery . histologic sections of the duodenal and jejunal specimens of infarcted bowel demonstrated transmural infarction with necrotizing acute inflammatory cell exudates along with submucosal edema and necrotizing acute serositis consistent with ischemic bowel ( fig . 1 , fig . its etiology has been attributed to a myriad of sources including mesenteric and retroperitoneal adenopathy , infarction in vertebral bodies , hepatobiliary disease and splenic infarction . while the above may at some point in the natural history of the disease contribute to abdominal pain , it is more likely that intermittent ischemia , stemming from transient low - flow states due to red blood cell deformity , is the primary source of the abdominal pain . at the capillary level , red blood cell deformability is the major determinant of viscosity with the capillary diameter at which red blood cell deformation inhibits normal blood flow being < 10 m . increased membrane rigidity resulting in decreased deformability may be the primary contributor in transient vaso- occlusion in capillaries that often range in diameter from 3060 m . one classic study by boley et al . reproduced ischemic colitis pathology in dogs after injection of microspheres of 30100 m into the mesenteric circulation [ 6 , 7 ] . we believe that the bowel injury found in our particular patient likely represents an extreme manifestation of what is likely to be a spectrum of pathology that ranges from minor abdominal pain secondary to transient ischemia to full - blown transmural bowel infarction . most case reports and reviews emphasize that the majority of patients with abdominal pain will not have clinically significant bowel injury and thus can be managed conservatively with bowel rest , aggressive hydration , and intravenous analgesics . however , our case highlights the fact that the far end of the clinical spectrum , i.e. transmural bowel injury with pneumoperitoneum requiring operative intervention , does indeed occur and should be kept in mind when managing and following sickle cell patients with abdominal pain .

we report a case of small bowel ischemia secondary to sickle cell disease . acute bowel ischemia is an uncommon presentation of patients with sickle cell disease . historically , only a handful of cases have been reported . we also provide a summary of the literature relevant to sickle cell patients with acute bowel ischemia .

olanzapine is an atypical antipsychotic with efficacy in schizophrenia slightly higher than others.1 ) the extrapyramidal adverse effects are much lower than typical antipsychotics , though it has a higher metabolic adverse effect profile.2 ) orthostatic hypotension is common with first - generation antipsychotics with rates up to 77% compared to 15% with placebo.3 ) among atypical antipsychotics , clozapine , risperidone and quetiapine have higher rates of orthostatic hypotension.3 ) we report a case where olanzapine , in a dose dependent fashion , caused transient postural hypotension . a 22-year - old female was diagnosed with paranoid schizophrenia 16 months back and was started on olanzapine , which was increased up to 15 mg / day over two weeks . she responded well to it , resuming her higher studies as well as a part - time job of a school teacher . however , after two months , she had sudden falls on five occasions over one week , seriously injuring herself with bruises and lacerations . vitals were checked on all the occasions which showed pulse rate ranging from 7090 beats per minute ( bpm ) and blood pressure ranging from 110130/ 7080 mmhg . however , in all the occasions the examinations could be done after the patient had recovered and gained consciousness . investigations including complete blood count , fasting and post prandial plasma glucose , lipid profile and liver function test were normal . her body weight has been maintained at 52 kg over the past one year , her menstrual cycles were regular and abdominal ultrasonography revealed no abnormalities . however , on current assessment both cortisol ( 9.31 g / dl ) and adrenocorticotropic hormone ( 14.5 pg / ml ) levels were within normal range with respect to age , sex and time ( morning / evening ) of sampling . she was advised rest at home , dose of olanzapine was reduced to 10 mg / day and a round - the - clock nurse was appointed for observation and monitoring . thereafter , the frequency of fall reduced , and there was only one such event when she was dizzy and about to fall but prevented by the attending nurse . her pulse rate was 140 bpm and blood pressure was 76/40 mmhg in supine position , and three minutes later pulse rate was 80 bpm and blood pressure was 110/70 mmhg in standing position , which was suggestive of orthostatic hypotension . she recovered from her dizziness and was fully oriented within five minutes of the event . subsequently , olanzapine was cross - tapered with aripiprazole up to 15 mg / day , on which she is maintaining well without further recurrence of adverse effects during follow up at two months . our patient developed orthostatic hypotension with olanzapine 15 mg per day that subsided following discontinuation of medication . there are few reports of hypotension associated with olanzapine,4,5 ) one in an elderly individual , and the other with intramuscular administration . the summary of product characteristics for olanzapine reports 5% subjects develop orthostatic hypotension as compared to 2% in placebo,6 ) which is much less as compared to the rates observed with first generation antipsychotics.3 ) nevertheless , hypotension and bradycardia has been reported with olanzapine as low as 5 mg per day.7 ) most commonly orthostatic hypotension is an incidental finding during routine examinations , and symptomatic hypotension is not usually observed except in the elderly patients . in contrast , our patient , a young female , developed symptomatic orthostatic hypotension with olanzapine that resulted in several episodes of falls , which required treatment discontinuation . interestingly , nourian et al.8 ) had shown through animal experiments that olanzapine , along with ziprasidone and aripiprazole has the least propensity to cause postural hypotension . several other putative mechanisms such as calcium blockade , inhibition of centrally mediated pressor reflexes , and negative inotropic effects , have also been proposed for this adverse effect.9 ) sedative effect of olanzapine may additionally contribute to the falls , though such mechanism appeared unlikely in our case . although the rates of orthostatic hypotension appear lower with olanzapine , an exercise of caution is definitely warranted in the light of the current case .

olanzapine is an atypical antipsychotic which is efficacious in the treatment of schizophrenia . the adverse effect profile for olanzapine is benign except for higher rates of metabolic events . orthostatic hypotension is less commonly reported with olanzapine as compared to first - generation and few atypical antipsychotics . we report a case where olanzapine , in a dose dependent fashion , caused transient postural hypotension .

a 15-year - old female patient has been referred to the dental hospital in madurai , tamil nadu , india , with a chief complaint of missing maxillary central incisor . past dental history revealed that the patient had earlier visited a dental clinic where she had been diagnosed with a congenitally missing central incisor and was advised to wear a removable partial denture . the patient was wearing the removable partial denture for the past 3 years . for a more definitive treatment , clinical examination revealed orthognathic facial profile and presence of good facial balance in all proportions . an intraoral examination revealed the presence of all permanent teeth except for the right upper central incisor . panoramic ( orthopantomogram or opg ) , periapical radiographs were taken to establish a good idea about the position and morphology of unerupted right permanent central incisor in maxilla . tooth was bulging in the labial sulcus at the mucogingival junction [ figure 1 ] . its position was very high up in the alveolar bone with a thick layer of soft tissue covering the crown in a vertical direction . the largest width of the crown of erupted permanent left central incisor was 8 mm . it was decided to do surgical exposure of impacted tooth and then bond a bracket on the labial surface of the tooth and bring down to its normal position . begg brackets were bonded on permanent maxillary left central incisor , lateral incisor , and left canine and right lateral incisor and right canine . after the crown of the impacted incisor was surgically exposed [ figure 2 ] , a begg bracket was bonded to the exposed incisor and 0.010 a. j. wilcock wire of supreme grade was used to align the right central incisor [ figure 3 ] . the patient was pleased with the esthetic results [ figure 4 ] . at 6-month follow - up , the left maxillary incisor remained vital and responded normally to percussion and mobility and sensitivity testing with good width of attached gingiva . an anomaly in the eruption of anterior teeth can affect facial esthetics and may cause psychological problems . if the impacted tooth is extracted , loss of alveolar bone is anticipated . following the healing period , the alveolar ridge becomes thinner and deficient , with these disadvantages in mind , facilitating eruption of the natural tooth and maintaining natural appearance become the goals of orthodontic treatment . as a result , several reports have indicated an impacted tooth can be brought into proper alignment in the dental arch . the following factors are used to determine whether successful alignment of an impacted tooth can take place : ( 1 ) the position and direction of the impacted tooth , ( 2 ) the degree of root completion , ( 3 ) the degree of dilacerations , and ( 4 ) the presence of space for the impacted tooth.[27 ] orthodontic and surgical intervention should not be delayed to avoid unnecessary difficulties in aligning the tooth in the arch . two of the most commonly used surgical exposure techniques for labial impacted teeth are : ( 1 ) exposure of the entire labial aspect to the anatomic crown with total excision of all keratinized tissue ( the window approach ) and ( 2 ) a technique which exposes only 45 mm of the most superficial portion of the labial aspect of the cusp tip while maintaining 23 mm of keratinized tissues . in this case , the available space for tooth alignment was sufficient and tooth was brought into right anatomical position in the dental arch . approach causes statistically significant loss of attachment , recession and gingival inflammation occur on maxillary canines after surgical exposure . therefore , a part of keratinized gingiva must be preserved or an apical flap should be used . it is important for a tooth to erupt through attached gingival , and not through alveolar mucosa.[911 ] if the impacted tooth is diagnosed with its root completely formed or if present in an unfavorable position , combination of surgical and orthodontic treatment has to be carried out . the treatment of an unerupted tooth will depend on its state , position , and presence of enough space in the dental arch to accommodate . if eruption is delayed , the permanent tooth should be exposed because it is important to allow the tooth to erupt into correct position as soon as possible . impaction of maxillary permanent incisors is not a frequent case in dental practice , but its treatment is challenging because of the importance of these teeth in facial esthetics .

impaction of maxillary permanent central incisor is not a frequently reported case in dental practice , but its treatment is challenging because of its importance to facial esthetics . early detection of such teeth is most important if complications are to be avoided . we report a case of a 14-year - old female with an impacted central incisor tooth in the maxillary anterior region . the impacted supernumerary tooth which was preventing the eruption of permanent incisor was surgically removed . combined approach with surgical exposure and the application of an orthodontic force brought the impacted left maxillary central incisor down to its proper position in the dental arch .

a 70-year - old woman was admitted to hospital because of bilateral pleural effusion , and referred to our neurology department due to a 2-year history of gait disturbance that occasionally resulted in falling down , before which she had been well . six months after the first episode of falling down , her family noticed that she had a masked face , monotonous speech , and bradykinesia in both arms and legs . during the subsequent year she fell down more frequently , and she could not walk without assistance . she had been taking vitamin d and a calcium preparation to treat a 10-year history of idiopathic hypoparathyroidism and recurrent compression fracture of the thoracic and lumbar vertebral bodies . , she was oriented to place and people , but had difficulty copying , calculating , and maintaining attention . a neuropsychological investigation showed a severe impairment in memory function , reduced psychomotor speed , and visuospatial dysfunction ( table 1 ) . she had a decreased blink rate , a paucity of movement of the face , and mild dysarthria . horizontal voluntary saccades were slightly limited and hypometric , and vertical upward and downward saccades were extremely limited and slow . she had a full range of eye movement in the doll 's head maneuver , demonstrating that her gaze palsy was caused by a supranuclear problem . all four limbs exhibited bradykinesia and rigidity . sitting up in a bed resulted in spontaneous retropulsion . she stood on a narrow base , and her stride was reduced with decreased arm swing bilaterally . the deep tendon reflexes were normoactive in all four limbs . however , there was no dystonia , pyramidal signs , or autonomic impairment . laboratory studies at admission showed the following values : 6.6 mg / dl serum total calcium ( normal : 8.6 to 10.0 mg / dl ) , 4.99 mg / dl phosphate ( normal : 2.7 to 4.5 mg / dl ) , 52 u / l alkaline phosphatase ( normal : 25 to 100 u / l ) , and 1.44 pg / ml intact - parathyroid hormone ( normal : 13.0 to 54.0 pg / ml ) . brain computed tomography ( ct ) demonstrated extensive , bilateral calcification of the basal ganglia , centrum semiovale , and bilateral dentate nuclei of the cerebellum ( fig . there were mixed high - and low - intensity signals compatible with the distribution of calcification on ct . 1-b ) . single - photon - emission computed tomography using tc - hexamethylpropylene amineoxime showed no abnormal perfusion defects in the brain . levodopa was started at 100 mg twice daily , and the dosage was slowly increased to 1000 mg per day . however , this did not produce any clinical improvement . we experienced a case of striopallidodentate calcification associated with idiopathic hypoparathyroidism presenting with prominent oculomotor disturbances . the prominent oculomotor disturbances , in combination with these extrapyramidal features and dementia , suggested a clinical diagnosis of psp.13 the origin of the parkinsonian symptoms and oculomotor disturbances of this patient is difficult to establish . one possibility is that the basal ganglia calcification observed in brain ct was an incidental finding , because asymptomatic basal ganglia calcifications may be seen at autopsy , especially in the elderly.14 however , the probability of such a coincidence is extremely low given the reported prevalence.15,16 in addition , it has been suggested that a metabolic disturbance such as low calcium or a high phosphorous plasma level may cause dopaminergic dysfunction in the substantia nigra pars compacta.17 f - deoxyglucose and positron - emission tomography have shown that glucose metabolism in the basal ganglia and the frontal brain is massively reduced in fahr 's disease.9,18 this abnormality possibly results from a disruption of frontostriatal circuits , presumably at the level of the basal ganglia.9 the precise mechanism of oculomotor disturbance in our patient was unknown . in view of the co - occurrence of oculomotor abnormalities in other progressive basal ganglia disorders such as parkinson 's disease , psp , and huntington 's disease , the significant impairment of eye movement in our patient may have been attributable to dysfunction of the basal ganglia in the generation of voluntary saccades.19 in conclusion , we have presented a rare case with idiopathic hypoparathyroidism complicated by oculomotor disturbances and parkinsonism with a psp - like phenotype .

we present a 77-year - old woman with levodopa - nonresponsive parkinsonism , dementia , and supranuclear gaze palsy on vertical and horizontal gaze . laboratory findings were consistent with idiopathic hypoparathyroidism , and brain computed tomography showed extensive bilateral calcifications of the basal ganglia , centrum semiovale , dentate nuclei , and cerebellar white matter . these results illustrate that striopallidodentate calcification due to hypoparathyroidism may present with symptoms mimicking progressive supranuclear palsy .

what was different in pci between the two merged analysis described above?14,31 we may argue that patients treated with dess had more three - vessel disease ( 87.3% vs. 36.1% with bms ) and perhaps a higher syntax score than those treated with bms ( in fact , we will never know which was the syntax score for them ) . however , on the other hand , bms pooled data also included diabetic population ( 18.1% vs. 0% with dess ) and more frequently compromise of proximal lad stenosis ( 90% vs. 59% with dess).14,31 the fact that first - generation dess were used in syntax trial could be one of the most attractive explanations ; in fact , syntax patients with definitive set ( 6.8% ) had 35.4% occurrences of cardiac death.24 however , that was not the case with ees used in the best trial , which significantly improved safety compared to the first designs . therefore , perhaps , stent design itself is not solely the reason for the poorer long - term outcome data of these two rcts . if we analyze the trial methods,21,30 in spite of different des designs , both studies share similar pci strategies , meaning that the goal was to achieve complete anatomic revascularization defined by authors as not any residual stenosis 50% in any major coronary artery or their large branches30 ; consequently , we can assume that many intermediate lesions were stented and that concurs with the similarity of stent length in both studies . when we analyze completeness of revascularization with pci,1 we should take into account several different situations such as the amount of myocardium at risk or stenosis severity of the lesions not included in the revascularization strategy . prognosis in patients with incomplete revascularization should be different if the not - attempted vessels had a complete chronic closure with collateral circulation ( fig . 1a ) , high - degree steno - sis in a large vessel ( fig . differences in the amount of completeness of revascularization with both , pci and cabg , was present in all rcts since the first study was performed ; however , in the past , such differences were not associated with poorer outcome4,14 ( 53.2% with pci vs. 82.7% with cabg , p = 0.0003 , see table 1 ) . furthermore , the fact that only 56.7% and 50.9% of syntax and best patients , respectively , in the pci arm achieved the goal of complete revascularization demonstrated how difficult it is for pci to achieve such aim.31 therefore , improved des design is only one face of the problem , while changing pci strategies using a more conservative policy during des implantation would be the other . the eraci iv study,32,33 with a patient population of multiple vessel disease and left main stenosis , used a second - generation des and a conservative pci strategy , defined as stenting only severe lesions ( visually 70% ) in large vessels . intermediate ( > 50 to < 70 ) lesions in small or large vessels or severe lesions in small vessels ( < 2.0 mm ) were not included . this pci strategy of not scoring lesions not included in the revascularization strategy allowed to build a new scoring system where low syntax score was found in 54% of patients and only 17.2% of patients persisted with high syntax score.34 investigators of eraci iv , at more than two years of follow - up , reported remarkable low rates of adverse events including death / mi / stroke of 3.9% , unplanned new revascularization of only 4% , and death / mi / stroke / target vessel revascularization ( tvr ) of 6.7% . it is important to note that there was no major penalty for this conservative policy as it was reflected by the low tvr rate in the not - stented intermediate lesions ( 1.3% ) . we do not know whether these results will remain at a five - year follow - up , although at the present time , the low rate of events in the not - stented lesions supports the pci strategy of this study . in agreement , fame investigators largely demonstrated that nonischemic lesions had better outcome when they were not treated with dess.35,36 we have to take into account that , even in the era of safer dess , neoatherosclerosis as a consequence of des implantation has not disappeared.28 to recap , interventional cardiologists have been doing a lot of work during this 25-year journey trying to close the safety / efficacy gap between pci and cabg in patients with multiple vessel disease and , looking at long - term results from rcts with the old and new dess , we may conclude that improved stent design alone is not enough to narrow the gap between pci and cabg.37 new revascularization strategies during pci and the search for a more functional revascularization avoiding unnecessary des implantation should be the new goal for future randomized comparisons between pci and cabg .

randomized clinical trials ( rcts ) with first- and second - generation drug - eluting stents ( dess ) confirmed the superiority of coronary artery bypass surgery ( cabg ) in patients with multiple vessel disease . in spite of different des designs , investigators in these trials used similar percutaneous coronary intervention ( pci ) strategies hoping to achieve complete revascularization , meaning that all intermediate lesions would be stented . one of these studies also included small vessels in the revascularization policy . on this revision , authors searched for a potential explanation of these intriguing findings and also for solutions to this problem , not seen years ago when other rcts compared cabg with pci in the previous des era . after they revised old and new scientific data , they concluded that improved des design is not itself enough to narrow the gap between pci and cabg and that in the future rcts we should institute more conservative strategies avoiding unnecessary multiple des implantation .

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we present a case of endometriosis presenting as large bowel obstruction in a woman of childbearing age . she had no previous symptoms to suggest endometriosis and on presentation urgent surgery was required . no cases of endometriosis confined to this sigmoid colon without pelvic involvement were noted in the literature . when women of childbearing age seek medical attention for signs and symptoms of intestinal obstruction , and there is no obvious etiology , intestinal endometriosis should be considered as a differential diagnosis . a 37 year - old housewife and mother of a 10 year - old by caesarian section , presented with a history of generalized , colicky abdominal pain and constipation for 6 days , with bilious vomiting and distention for 2 days . she had normal , regular menses with no history of dyspareunia , dysmenorrhea , abdominal pain , constipation , diarrhea or rectal bleeding . on examination , she was in painful distress , ill looking and mildly dehydrated , with a tachycardia of 108/min . the abdomen was distended and tympanitic with generalized mild tenderness but no peritonism or palpable mass . ct - scan showed grossly distended large intestine from the caecum to the sigmoid colon , with no air in the rectum ( see fig . the complete blood count , liver function test and carcino - embryonic antigen were all within normal limits . at surgery , the large intestine was grossly distended from the caecum to sigmoid colon where there was a palpable solid tumor in the wall of the bowel . the pelvis , ovaries , tubes and uterus were grossly normal and there were no other intra - abdominal abnormalities . with an operative diagnosis of carcinoma , sigmoid colectomy with primary anastomosis the cut specimen revealed an obstructing tumor on the mesenteric border , within the wall of the sigmoid colon without involvement of the mucosa ( see fig . she was subsequently referred to the gynecologist for treatment and remains asymptomatic 15 months later . intestinal involvement by endometriosis occurs in 5% of premenopausal women . of these , 70% present with large bowel obstruction . however , the vast majority of these patients reported are known cases of endometriosis , having complaints of pelvic pain , dyspareunia and/or dysmenorrhea . many of them also have a history of infertility , for which they were subjected to investigation , such as laparoscopy , hence , presenting with a previous diagnosis of endometriosis . our patient had no history to suggest the condition with no visible endometrioma , ovarian or pelvic abnormality indicative of endometriosis . the pathophysiology of endometriosis has been explained by various theories ; direct myometrial extension , coelomic metaplasia , lymphatic and hematogenous metastasis , reverse menstruation and implantation during salpingography or due to operative spillage . isolated endometriosis within the muscular layer of the bowel with no involvement of mucosa or any pelvic organ remains unclear . although seat belt trauma had , in one case , been suggested as an etiologic factor , no clear evidence was provided for this . in our patient when the initial presentation of endometriosis is intestinal obstruction with no previous history and no suspicious findings at surgery , the diagnosis is unlikely to be made preoperatively . in most reported cases , the patients had a known history of endometriosis or the surgical findings were very suggestive of it . both these observations were absent in our patient . in the non - obstructed case , colonoscopy , endoscopic we believe that one should always maintain a high level of suspicion of endometriosis , when a woman of childbearing age presents with intestinal obstruction and there is no other obvious cause . if the diagnosis is made preoperatively , surgery may be avoided in the non - obstructed case and only a limited resection done in the event of obstruction . when a woman of childbearing age presents with large bowel obstruction , one should always entertain a possible diagnosis of endometriosis whether or not the patient has other evidence of the disease . 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 . all the authors of this article contributed to the study design , data collection , data analysis and writing .

introductionisolated endometriosis of the intestine causing large bowel obstruction is rare.presentation of casewe present a case of endometriosis presenting as large bowel obstruction in a woman of childbearing age . she had no previous symptoms to suggest endometriosis and on presentation urgent surgery was required . the diagnosis of endometriosis was made only after pathological evaluation of the specimen.discussionno cases of endometriosis confined to this sigmoid colon without pelvic involvement were noted in the literature.conclusionthe diagnosis of endometriosis should be entertained when women of childbearing age presents with large bowel obstruction , whether or not the patient has other evidence of the disease .

an 85-year - old man presented with an 8-month history of slowly increasing diffuse yellow skin lesions on the torso and upper arms . he had been unaware of the discoloration until his internist noted it and referred him to dermatology . the patient had been followed by a hematologist for paraproteinemia that had not been treated . the examination showed well - demarcated yellow patches and plaques covering large portions of his upper trunk with some islands of sparing ( figure 1 , 2 ) . the dermoscopic evaluation showed a reticular pattern of yellow amorphous homogeneous structures with overlying branched and linear vessels ( figure 3 ) . a skin biopsy showed scattered foamy histiocytes within the reticular dermis , which were consistent with a plane xanthoma ( figures 4 , 5 , 6 ) . a complete blood count , comprehensive metabolic panel , fasting lipid panel were within normal range . diffuse normolipemic plane xanthoma ( dnpx ) was first described by altman and winkelmann in 1962 . dnpx is characterized by xanthelasma palpebrarum ; diffuse plane xanthoma of the head , neck , trunk , and extremities ; and normal plasma lipid levels . xanthelasma typically appears first , followed by involvement of the lateral parts of the neck and upper trunk . clinically , the dermatosis is characterized by the presence of symmetric yellowish - orange plaques that favor the neck , upper trunk , flexural folds and periorbital region . histologically , foam cells ( macrophages that have engulfed lipid droplets ) and variable numbers of touton giant cells , lymphocytes , and foamy histiocytes can be seen . while not all cutaneous xanthomas are associated with systemic diseases , dnpx has been associated with systemic diseases , particularly multiple myeloma and monoclonal gammopathy . however , other malignant hematological or lymphoproliferative disorders associated with dnpx include acute monoblastic leukemia , chronic myelomonocytic leukemia , chronic myloid leukemia , chronic lymphatic leukemia , non - hodgkin s lymphoma , adult t - cell lymphoma / leukemia , sezary syndrome , waldenstrom s macroglobulinemia , cryoglobulineimia and castleman s disease [ 2,3,512 ] . the pathogenesis of dnpx has not been fully elucidated , however , in gammopathy - associated dnpx , monoclonal igg is thought to bind to circulating ldl , rendering the antibody ldl complex more susceptible to phagocytosis by macrophages . dnpx can precede such disorders by several years ; therefore close follow - up with periodic laboratory tests for myeloproliferative disorders should be performed . the erbium : yag laser has been used successfully to treat facial xanthomas in one patient . in this patient , currently there are no associated systemic symptoms . , this case shows that dermatological lesions can be the first manifestation of important hematological diseases and so physicians should be familiarized with this rare entity .

diffuse normolipemic plane xanthoma ( dnpx ) was first described by altman and winkelmann in 1962 . it is a rare and non - inherited form of xanthomatosis . clinically , the dermatosis is characterized by the presence of symmetric yellowish - orange plaques that favor the neck , upper trunk , flexural folds and periorbital region . it has been recognized to be associated with hematological diseases , especially with multiple myeloma and monoclonal gammopathy . we present a patient with diffuse plane xanthoma , normal lipid level , and monoclonal gammopathy .

in recent years the problem of " cutaneous ulcers " ( venous , arterial , diabetic , and pressure sores ) has become increasingly important , in particular because of the progressive increase in the elderly population and , therefore , of chronic disorders ( 1 ) . chronic cutaneous ulcers of the comorbid elderly patient tend to resist on standard therapy and therefore the management is difficult , time - consuming and expensive ( 2 ) . optimally , a short period of advanced therapy to stimulate healing can result in the transition of an intractable wound to a treatable one , which can then be treated with less expensive , standard care ( 3 ) . platelet - rich plasma ( prp ) is an autologous preparation of platelets in concentrated plasma and contains various growth factors . due to the presence of high concentrations of these growth factors , prp has been used in a wide variety of surgical procedures and clinical treatments ( 4 ) . we herein report a comorbid patient with refractory cutaneous ulcer which did not respond to formal therapeutic methods . the patient was treated with autologous prp and could achieve complete response of the skin lesion within 3 months . on november 25 , 2011 , a 94 yr - old woman was referred for the management of erythematous to violaceous patches with bullae on dorsum of left foot . she repeatedly hit her left foot in which intravenous line was situated with the opposite side for days . she had a various underlying disease including alzheimer 's dementia , angina pectoris , type 2 diabetes , hypertension , chronic kidney disease ( stage iv ) and was in a bed ridden state . because of the poor oral intake and noncompliance to the levin - tube , the percutaneous endoscopic gastrostomy tube had been inserted on her abdomen and only minimal amount of nutrition was maintained . despite of the daily simple dressing and intermittent debridement about 2 weeks , the status of the wound was deteriorated and advanced to painful ulcerative patches with eschar and granulation tissues ( fig . autologous prp were prepared for the alternative management of her worsening wound . 12 ml peripheral autologous blood was obtained and collected into tubes containing acid - citrate - dextrose solution formula ( acd - a ) anticoagulant . subsequently , the yellow plasma ( containing buffy coat with platelets ) was separated from other components . the upper 3 to 4 ml plasma called platelet poor plasma was removed by micropipette . the patient underwent a total of 7 prp treatments over a span of 8 weeks ( performed twice a week until 5th treatment and then weekly until 7th treatment ) . as described above , these treatments involved topically application of activated prp as well as packing of the wound with aseptic film for the sustained effect of growth factors . the film was removed on next day and the top layer was covered with hydrofoam dressing material until subsequent prp treatment . over the course of the prp treatment , the results of the changes in wound surface area and depth are shown in fig . the wound was essentially filled in with granulation tissue after seven prp treatments with only subtle epithelialization of the new tissue remaining . there were neither signs of infection and allergic reaction nor discomfort of the patient during the treatment . after 2 months from the last ( 7th ) treatment , the complete epithelialization was seen and no further simple dressing was required for the wound . the complete response of the recalcitrant cutaneous ulcer using prp therapies implies that this stimulation therapy could be an appropriate alternative modality when the degree of healing with conventional treatment appears to be unsatisfactory . the ulcer bed more rapidly demonstrated margin in - growth , granulation tissue development , vascularization and epithelialization ( 5 , 8) . first of all , the growth factors and cytokines released from prp are able to stimulate the proliferation , migration and differentiation of dermal fibroblasts and endothelial cells . prp also increased type i collagen and mmps gene expression , suggesting that prp also have the potential to promote the tissue remodeling of aged skin ( 4 ) . the second component is the mesh of fibrin that constitutes the " structure " of the platelet gel : this mesh forms a biological scaffold that helps and guides the migration of the mesenchymal cells , derived from populations of resident stem cells or circulating precursors , from the base and the margins of the wound . the third component is the antibacterial properties of the factors released by the platelets and , probably , by the leucocytes contained in the prp ( 1 ) . on the basis of these molecular understandings , prp therapy has been successfully used in the treatment of various dermatological defect including pressure ulcer ( 1 , 5 ) , diabetic ulcer ( 1 , 2 , 6 ) vascular ulcers , post - traumatic ulcers ( 1 ) and lipodermatosclerosis ( 7 ) . however , a few studies have reported in the treatment of severe cutaneous ulcers associated with old age , hypomobility and many underlying diseases ( 2 , 9 ) . although further studies are required for better understandings of the mechanism , efficacy , and safety of autologous prp therapy , it could be considered as an effective alternative option for the recalcitrant cutaneous ulcer with various comorbidities .

the platelet - rich plasma ( prp ) has been advocated as a way to introduce increased concentrations of growth factors and other bioactive molecules to injured tissues in an attempt to optimize the local healing environment . a 94-yr - old woman with various comorbidities presented with a two - week history of severe cutaneous ulcer on the left dorsum of foot . it was caused by recurrent mechanical trauma and did not respond to several wound debridement and simple dressings . however , after she was completed on seven times of autologous prp treatments , we observed complete healing of the skin lesion within 3 months . herein , we report a case of recalcitrant cutaneous ulcer with various comorbidities and discuss about the promising possibility of autologous prp as an effective alternative therapeutic modality .

morvan 's syndrome is a rare autoimmune disorder characterized by peripheral nerve hyperexcitability , autonomic dysfunction , and central nervous system symptoms . the syndrome of muscle twitching , dysautonomia , insomnia , and delirium was first reported by morvan by the name of la choree fibrillare in 1890 . we report a rare case of contactin - associated protein - like 2 ( caspr2 ) , a subtype of voltage - gated potassium channel ( vgkc ) complex antibody positive morvan 's syndrome , with syndrome of inappropriate antidiuretic hormone secretion ( siadh ) . a 45-year - old male presented with 4-month duration of nonradiating mild back pain , followed a month later by burning sensation in palms and soles with nocturnal exacerbations . he became aggressive , over - talkative , and insomniac over 15 days before presentation . electromyography showed spontaneous activity including myokymic discharges [ figure 1 ] , doublets , and triplets in both upper and lower limb muscles . the patient had positive serum anti - caspr2 antibody , a subtype of vgkc complex detected by immunofluorescence method . his cerebrospinal fluid examination showed raised proteins 76 mg / dl ( normal : 2040 mg / dl ) , with normal cell count ( cells : 3/mm , all lymphocytes ) . computed tomography ( ct ) of the chest showed no evidence of thymoma . the patient was diagnosed as morvan 's syndrome with positive anti - caspr2 vgkc antibody . spontaneous activity in right tibialis anterior showing myokymic discharges ( sweep speed : 0.1 ms and sensitivity : 50 v ) there was persistent low serum sodium in the range of 125130 meq / l , for which patient was evaluated . his urinary osmolarity was raised ( 216.36 mosm / kg , normal < 100 mosm / kg ) and random urinary sodium was increased ( 42 mmol / l , normal < 30 mmol / l ) . the serum osmolarity was decreased ( 271.5 mosm / kg ) and urinary specific gravity was 1.010 . the patient was treated with intravenous immunoglobulin ( iv ig ) 2 g / kg in 5 divided doses . he was given phenytoin at dose of 100 mg three times a day for symptomatic relief for twitching , which acts as membrane stabilizer . the patient was started on oral prednisone ( 1 mg / kg ) and fluid restriction was advised . he had marked improvement in muscle twitching and was able to sleep properly with immunotherapy . electromyography done 2 weeks after the course of iv ig showed decrease in spontaneous activity ; occasional fasciculations were seen . his hyponatremia was also corrected . on follow - up , after 3 months , the patient was completely normal and electromyography showed no spontaneous activity . oral prednisone was given 1 mg / kg for 3 months and later tapered gradually over next 2 months . morvan 's syndrome is characterized by myokymia associated with muscle pain , excessive sweating , weight loss , hallucinations , sleep disorders , and behavioral abnormality . the basic mechanism for both presentations is same , that is anti - vgkc antibody , acts at different levels of neuraxis , both at central and peripheral level . caspr2 antibodies positive cases but common in anti - leucine - rich glioma inactivated-1 ( lgi-1 ) antibodies positive cases . lgi-1 antibodies are usually associated with hyponatremia , and caspr2 antibodies are usually associated with thymomas which carry poor prognosis . caspr2 antibodies mostly bind the neuropil , whereas antibodies to lgi-1 bound to neuronal cell bodies including the antidiuretic hormone - secreting and orexin - secreting hypothalamic neurons present in hypothalamus , raphe nucleus , and locus coeruleus . hyponatremia is not commonly reported in morvan 's syndrome although it is present in half of the patient in lgi-1 antibodies positive limbic encephalitis . in addition , fasciculation , doublets , triplets , multiplets , and positive sharp waves are also present . this is a rare case report of morvan 's syndrome with siadh having anti - caspr2vgkc antibodies that responded to immunosuppression .

morvan 's syndrome is a rare autoimmune disorder characterized by triad of peripheral nerve hyperexcitability , autonomic dysfunction , and central nervous system symptoms . antibodies against contactin - associated protein - like 2 ( caspr2 ) , a subtype of voltage - gated potassium channel ( vgkc ) complex , are found in a significant proportion of patients with morvan 's syndrome and are thought to play a key role in peripheral as well as central clinical manifestations . we report a patient of morvan 's syndrome with positive caspr2anti - vgkc antibody having syndrome of inappropriate antidiuretic hormone as a cause of persistent hyponatremia .

the use of invasive mechanical ventilation by endotracheal intubation in acute respiratory failure is long known.1 noninvasive ventilation ( niv ) began to be considered as an alternative to invasive mechanical ventilation in acute respiratory failure caused from exacerbations of chronic obstructive pulmonary disease ( copd ) in the 1980s , and its use gradually rose worldwide . in 1998 , an international prospective survey reported that about one - third of patients initially treated with niv had to undergo endotracheal intubation,2 but 6 years later another large international prospective study reported a better outcome.3 also , niv effectiveness is supported by results of systematic reviews,4 and this has led to an increase in the application of this treatment , even in complicated situations such as a do - not - intubate order in elderly patients.5 however , in 2010 , manuel et al reasoned that there is little convincing evidence for the use of niv in severe , but stable copd and that what is less clear , however , is the quality of how niv is delivered to patients.6 indeed , a recent study focused its attention on the kind of niv setup , which can be based on high inflation pressure and high backup rate ( high - intensity niv ) or high pressure and low backup rate ( high - pressure niv ) . the authors reported that the pivotal role in managing by niv hypercapnic respiratory failure in copd patients is the high - pressure component.7 seventy - eight patients ( 57 males , mean age 78.3 9.2 years ) undergoing niv were evaluated . of them , 48 ( 62.3% ) had acute hypercapnic respiratory failure because of a copd exacerbation and the remaining 30 had acute hypercapnic respiratory failure from other causes , mainly cardiac failure . all patients were treated by niv using the bi - level positive airway pressure set up at high pressure / high backup rate . niv was successful in 67 subjects ( 85.9% ) and the patients were discharged , 57 of whom continued niv at home and ten were in a state of spontaneous breathing . niv was unsuccessful in eleven patients , ten of whom died and one was successfully treated by invasive mechanical ventilation . the mean age of successfully treated ( 77.7 9.4 years ) and unsuccessfully treated ( 82.1 8.1 years ) patients was not significantly different . significant differences were detected for a higher basal glasgow coma scale score in successfully treated patients ( p = 0.007 ) , a higher basal score of the acute physiology and chronic health evaluation score in unsuccessfully treated patients ( p = 0.004 ) , and a lower ph after 1 hour in unsuccessfully treated patients ( p = 0.015 ) . among the risk factors for failure of niv , the ph value seems particularly important,8 which corresponds with the current data . also , the acute physiology and chronic health evaluation confirmed the predictive value of a score higher than 29 , which was detected in patients with a negative outcome . overall , the current data , obtained in a pneumology unit , show that niv is able to manage the large majority of patients ( 86% ) with acute respiratory failure from copd exacerbations and also from other causes . a randomized study found that the use of niv as rescue therapy was associated with a lower number of patients meeting the endotracheal intubation criteria and with a lower mortality rate compared with endotracheal intubation.9 the factors underlying a negative outcome of niv warrant investigation in studies in large populations of patients . a recent study reported that patients with copd and obesity hypoventilation syndrome treated with niv because of acute hypercapnic respiratory failure had a response to treatment similar to patients with only copd.10 moreover , a low rate of endotracheal intubation need was recently reported in patients with acute respiratory failure from heart failure treated with niv,11 and this observation is pertinent for the patients in the current study . the findings show a very high rate of success of niv in patients with acute hypercapnic respiratory failure from copd as well as from other causes , including cardiac failure . this suggests that the use of invasive mechanical ventilation may be further reduced , with a decrease in its known complications as well .

noninvasive ventilation ( niv ) was introduced as an alternative to invasive mechanical ventilation for acute respiratory failure caused from exacerbations of chronic obstructive pulmonary disease in the 1980s , and its use gradually rose worldwide . seventy - eight patients ( 57 males , mean age 78.3 9.2 years ) undergoing niv were evaluated . of them , 48 ( 62.3% ) had acute hypercapnic respiratory failure because of a chronic obstructive pulmonary disease exacerbation , and the remaining 30 had acute hypercapnic respiratory failure from other causes , mainly cardiac failure . all patients were treated by niv using the bi - level positive airway pressure set up at high pressure / high backup rate . niv was successful in 67 subjects ( 85.9% ) and the patients were discharged , 57 of whom continued niv at home and ten had spontaneous breathing . niv was unsuccessful in eleven patients , ten of whom died and one was successfully treated by invasive mechanical ventilation . significant differences were detected for a higher basal glasgow coma scale score in successfully treated patients ( p = 0.007 ) , a higher basal acute physiology and chronic health evaluation score in unsuccessfully treated patients ( p = 0.004 ) , and a lower ph after 1 hour in unsuccessfully treated patients ( p = 0.015 ) . these findings show a very high rate of success of niv in patients with acute hypercapnic respiratory failure not only from chronic obstructive pulmonary disease but also from cardiac failure . this suggests that the use of invasive mechanical ventilation may be further reduced , with a decrease in its known complications as well .

a 35-year - old male , diagnosed with obsessive - compulsive disorder ( ocd ) for the past 5 years was prescribed on fluoxetine up to 80 mg / day . he was receiving this treatment for the past 1 year with partial improvement of symptoms despite adequate compliance . cognitive behavior therapy was not possible due to transportation inconvenience . due to the persistence of symptoms , despite a high dose of fluoxetine , augmentation with clomipramine ( up to 100 mg / day ) after few days of the addition of clomipramine , the patient had reported dark pigmentation over face localized to both malar eminences . the dermatological consultation was sought for this hyperpigmentation ; a diagnosis of melasma was made . his routine hemogram , thyroid function test , and serum cortisol levels were within normal limits . due to poor tolerance to clomipramine ( excessive sedation and severe constipation ) , dose of clomipramine was reduced to 50 mg / day . due to the persistence of ocd symptoms , this resulted in improvement in ocd symptoms over next 3 months ; however , the skin pigmentation and severe constipation persisted . clomipramine dose was reduced to 25 mg / day for next 2 months and persistence of constipation resulted in its stoppage . assessment score on naranjo adverse drug reaction ( adr ) probability scale was six , which was suggestive of probable adr . assessment on the world health organization - uppsala monitoring centre ( who - umc ) system for standardized case causality assessment also suggestive of probable adr with clomipramine . in our patient , the pigmentation is confined to the malar eminences of the face , with sparing of rest sun - exposed areas of the body . this adverse effect of clomipramine is not life - threatening , but can be a reason of concern from cosmetic sense . in our patient , clomipramine was not acceptable due to constipation and excessive sedation , although he was worried about melasma . stoppage of clomipramine resulted in improvement of sedation , constipation as well as melasma . causality assessment is an important evaluation to establish the link between an adverse event with a particular drug . causality assessment has been done on naranjo adr probability scale and the who - umc system , which revealed probable adr ( i.e. , the adverse event followed exposure to drug and disappeared following drug discontinuation , it is unlikely attributable to disease or other drugs ) . in a study , it was found that the prevalence of severe adr being 1.4% in general ; higher with the tricyclic antidepressant ( tca ) group and lower with monoamine oxidase inhibitors and selective serotonin reuptake inhibitors . among the tcas allergic exanthematous cutaneous reactions are known with tcas . however , there is description of a single case of 60-year - old female suffering from depressive episode , who developed pigmentation over light exposed areas of the body with the use of clomipramine . there are few case reports of such pigmentation with the use of imipramine , a congener of clomipramine . this is probably the second case study , reporting the rare side effect of clomipramine .

melasma is a hyperpigmented dermatological condition common in females . drugs such as steroids , cosmetics , and photosensitizing agents are known to cause melasma . we report here a case of an adult male with obsessive - compulsive disorder , receiving clomipramine , who developed melasma .

in the previous issue of critical care nathani and colleagues have assessed kl-6 , a specific marker of type 2 alveolar epithelial cell injury , as a biomarker in acute lung injury ( ali ) . biomarkers allow identification of patients at risk of developing disease or can be used as surrogate measures for clinical outcomes . additionally , measuring biological markers may be a valuable tool in understanding disease pathogenesis . in ali , the alveolar capillary barrier is disrupted and the alveolar epithelial cell function is critical to the recovery from ali / acute respiratory distress syndrome ( ards ) . this knowledge provides a rationale for measurement of alveolar epithelial cell injury using surrogate biochemical measures such as kl-6 , as a biomarker of ali . in the study of nathani and colleagues , plasma and bronchoalveolar lavage samples were collected following inclusion and on day 4 from 30 ventilated ali patients , from 12 patients at risk of developing ali and from 10 nonsmoking volunteers free of respiratory disease the study therefore had the benefit of allowing the investigators to look at kl-6 both in physiological and pathological states . the important findings from the study are that plasma kl-6 levels are increased in patients with ali , plasma kl-6 correlates with the severity of lung injury and plasma kl-6 is significantly elevated in nonsurvivors compared with survivors . furthermore bronchoalveolar lavage kl-6 is elevated in patients with ali and is higher in nonsurvivors . these findings extend the previous data showing kl-6 is elevated in plasma and epithelial lining fluid in ali . in relation to kl-6 in ali , questions that still remain to be answered include the specificity of the type 2 epithelial cell as the source of kl-6 as well as a need to confirm whether kl-6 elevation reflects epithelial cell injury , regeneration or secretion in response to inflammatory mediators . in addition , mechanical ventilation is known to cause epithelial injury , and an important area in which biomarkers may be valuable is in the assessment of ventilator - associated lung injury . increased surfactant protein d is associated with injurious ventilation strategies , and it would be interesting to know the effects of mechanical ventilation on kl-6 . regardless of these outstanding questions , nathani and colleagues ' work together with other data showing that elevated surfactant protein d , a type 2 epithelial cell product , is associated with a worse clinical outcome in ali / ards and that the receptor for advanced glycation end - products , an alveolar type 1 epithelial cell - associated protein , is increased in patients with ali implicates epithelial cell damage as an important determinant of outcome and implies the potential for alveolar epithelial cell biomarkers to predict outcome in ali . further , these data support the central role of epithelial injury in the pathogenesis of ali . notable limitations of all these surrogate biomarkers exist ; they do not directly measure epithelial function , and they require laboratory analysis and therefore can not be performed by a clinician at the bedside . additionally , there is no biomarker of epithelial function that reliably identifies patients at risk of ali who will develop ali . the resolution of pulmonary oedema from the alveolar space , which is dependent on alveolar fluid clearance , is critical to the recovery from ali / ards . although this can not be measured at the alveolus , the potential difference can be measured readily across the nasal epithelium . measuring nasal potential difference is a simple noninvasive measurement easily undertaken repeatedly at the bedside . in an animal model , measurement of nasal potential difference correlated with alveolar fluid clearance . this observation supports the hypothesis that nasal potential difference measurement may be a surrogate marker for alveolar epithelial function . furthermore , premature infants with pulmonary oedema and patients susceptible to high - altitude pulmonary oedema have reduced nasal transepithelial sodium resorption , as measured by the baseline nasal potential difference , indicating that nasal potential difference measurement may be able to identify patients at risk of developing ali . although further work validating such functional measures of epithelial activity is required , it is probable that as well as biochemical measures such as kl-6 , as demonstrated by nathani and colleagues , future biomarkers in ali will combine both biochemical and functional measures .

studies have indicated that measuring biochemical measures of epithelial injury in plasma and alveolar fluid may be useful in predicting outcome in acute lung injury . the present commentary briefly reviews the evidence supporting the use of these biochemical biomarkers of epithelial injury in acute lung injury , and in particular kl-6 , as well as their limitations . the article additionally proposes the need for physiological markers of epithelial function to complement current biochemical biomarkers .

we review 4 patients with severe gbs and one patient with miller fisher syndrome ( mfs ) admitted to our institution from 2012 to 2013 . all patients received very high - dose ivig ( total dose of at least 4 g / kg , equivalent to 2 standard treatment courses [ 2 g / kg ] ) administered over fewer than 14 days . all patients with gbs did not respond initially , prompting a second course of treatment ( table ) . as this was a retrospective review of a small case series , institutional review board approval was not required . all of the patients with non - o blood types ( 3/5 ) treated with very high - dose ivig developed clinically significant anemia requiring blood transfusion . when tested ( 2/3 cases ) , there was clear evidence of hemolysis ( positive direct antiglobulin test , low haptoglobin , high lactate dehydrogenase / bilirubin ) . in addition , there were no other medical comorbidities or medications commonly implicated in causing autoimmune hemolytic anemia ( table ) . patient 4 developed hematuria prior to discharge with normal hemoglobin and returned with massive hemolysis leading to pigmentary nephropathy and requiring hemodialysis . patients with o blood type did develop anemia , but it was mild and clinically asymptomatic . in addition , the patients with o blood type did not have evidence of hemolysis . antibodies to blood group antigens ( a and b ) found in ivig due to pooling from donors with o type blood are proposed as the hemolytic mechanism in many prior observations . the current industry standard antibody titers are < 1:64 for anti - a and < 1:32 for anti - b . privigen , a commonly used liquid ivig formulation , currently has anti - a < 1:32 and anti - b < 1:16 . however , the titers of anti - a and anti - b may vary within lots . therefore , patients who are given higher doses of ivig will be exposed to higher levels of anti - a and anti - b antibodies . although our patients received privigen , ha has been reported with use of many liquid ivig formulations . antibodies to blood group antigens ( a and b ) found in ivig due to pooling from donors with o type blood are proposed as the hemolytic mechanism in many prior observations . the current industry standard antibody titers are < 1:64 for anti - a and < 1:32 for anti - b . privigen , a commonly used liquid ivig formulation , currently has anti - a < 1:32 and anti - b < 1:16 . however , the titers of anti - a and anti - b may vary within lots . therefore , patients who are given higher doses of ivig will be exposed to higher levels of anti - a and anti - b antibodies . although our patients received privigen , ha has been reported with use of many liquid ivig formulations . we propose that risk of ivig - related ha is increased with high doses , significant in non - o blood types , and more likely to occur when administered over a short interval ( i.e. , < 2 weeks ) . ha has not been frequently observed in patients receiving maintenance ivig , likely due to the lower dose , longer time intervals between infusions ( typically 34 weeks ) , and 23 week half - life of anti - a / b igg . although all of our patients had gbs / mfs , ha has been reported in many other conditions treated with high - dose ivig such as postpolio syndrome , chronic inflammatory demyelinating polyneuropathy , myasthenia gravis , renal transplantation , and kawasaki disease . the applicability of our findings is limited by a small number of cases , retrospective analysis , and hemolysis testing in only 2 of the 3 patients . however , our findings raise relevant , novel considerations such as a possible maximum tolerated dose of ivig in a short time frame . in addition , practitioners should exercise caution if considering escalating ivig dosages to > 2 g / kg in < 14 days . hemoglobin and creatinine levels should be monitored closely during and after treatment with high - dose ivig ( up to 12 weeks ) to detect this untoward complication . in the literature , nadir hemoglobin level seemed to occur within 2 weeks of treatment , commonly within 45 days . if anemia occurs , testing should include direct antiglobulin test , peripheral blood smear , bilirubin , haptoglobin , lactate dehydrogenase , and reticulocyte count . manufacturers should label anti - a and anti - b titers on their product and consider more stringent industry standards . we propose that there is a maximal tolerated dose of ivig over a 2-week period , which is dependent on patient abo blood group and ivig 's anti - a / anti - b titers .

objective : we describe an underrecognized side effect of high - dose iv immunoglobulin ( ivig ) , hemolytic anemia.background:there are no established guidelines on treating patients with guillain - barr syndrome ( gbs ) who relapse or do not improve after a standard course of treatment ( ivig or plasma exchange ) . some centers will opt for a second course of the initial treatment . there is an ongoing trial of a second course of ivig in patients with severe gbs.methods:we retrospectively reviewed 4 patients with severe gbs who received high - dose ivig . one patient inadvertently received a high dose of ivig for miller fisher syndrome . all patients received a total of at least 2 courses of the standard dose of ivig ( total > 4 g / kg ) . we review their clinical course and side effects.results:all patients with non - o blood types developed clinically significant hemolytic anemia requiring blood transfusion.conclusion:hemolytic anemia may limit doses of ivig for treatment of severe gbs in patients with non - o blood types .

sertoli - leydig cell tumours ( sclts ) are rare sex - cord stromal tumors of the ovary . retiform sclt 's are rare histological variant of sclt with an average age of presentation being 16 years . to the best of our knowledge the retiform variant of sertoli leydig tumors reported so far are seen in less than 10 years of age . we present a rare case of retiform variant of sclt 's presenting in a 42-year - old female . a 42-year - old female presented with painless lump in the lower abdomen for 7 years . largest measured 14 12 cm , smallest measured 3 3 cm . on cutting fleshy , firm tissue was found within the cyst wall [ figure 1 ] . microscopic examination showed ovarian tumor with tubular , cord like pattern lined by bland cuboidal to columnar cells . also seen is retiform pattern at few places and scattered cells with abundant eosinophilic cytoplasm suggestive of leydig cells . leydig cells showed focal positivity for vimentin and negativity for leydig cells [ figures 7 and 8 ] . histological diagnosis of retiform variant of sertoli leydig cell tumor of ovary stage 1 ( t1n0m0 ) was made . the patient was followed up to 2 years , she did not have any complaints . gross appearance of sertoli leydig cell tumor sertoli cells arranged in cords , sheets and aggregates ( he 100 ) retiform pattern in serrtoli- leydig tumor ( he 100 ) sertoliform cells with clear cytoplasm ( he 400 ) leydig cells showing eosinophilic cytoplasm ( he 100 ) sertoli cells showing atypia ( he 400 ) cytokeratin positivity in sertoli component of sertoli leydig cell tumor of ovary ( 100 ) vimentin positivity in leydig component of sertoli leydig cell tumor of ovary ( he 100 ) however , retiform sertoli - leydig cell tumors present in the average age of 16-year - old . clinically , non - virilised patients present with non - specific symptoms like abdominal mass , pelvic pain . it is seen that 80% of the patients with ovarian slcts and virilising manifestations present with elevated serum levels of testosterone and androstenedione . however , sclts are unilateral and are confined to ovary at the time of diagnosis . computerised tomography ( ct ) , magnetic resonance imaging ( mri ) , and positron imaging tomography ( pet ) scans can provide us a better visualisation of the extra - ovarian or metastatic spread of the tumor . well - differentiated slct have an average diameter of 5 cm whereas intermediate type and poorly differentiated types have an average diameter of 15 cm . microscopically , they are divided into following categories : well - differentiated or meyer type 1 : they constitute 11% of slct.intermediate type or meyer type 2 : they constitute 54% of slct.poorly differentiated ( sarcomatoid or undifferentiated or meyer type 3 ] : they constitute 13% of slct.sclt with heterologous elements like skeletal muscle , cartilage.retiform type ( 15% ) : they resemble rete of ovary or testes . staging wise t1 means tumor limited to the ovaries , t2 means tumor involves one or both ovaries with pelvic extention , t3 means involvement of one or both ovaries with microscopically confirmed peritoneal metastasis outside the pelvis . intermediate type or meyer type 2 : they constitute 54% of slct . poorly differentiated ( sarcomatoid or undifferentiated or meyer type 3 ] : they constitute 13% of slct . staging wise t1 means tumor limited to the ovaries , t2 means tumor involves one or both ovaries with pelvic extention , t3 means involvement of one or both ovaries with microscopically confirmed peritoneal metastasis outside the pelvis . a total abdominal hysterectomy is considered as the treatment of choice in patients with unfavourable findings like rupture , extra - ovarian spread , poorly differentiated neoplasm , heterologous mesenchymal differentiation . post - operative chemotherapy , radiotherapy or a combination of both , may also be considered in the patients with above mentioned poor prognostic factors . similarly the 5 year survival rates for stage 1 tumor is 95% and that for stage iii and stage iv is zero percent .

sertoli - leydig cell tumors are the uncommon sex - cord stromal tumors of the ovary . we report a case of 42-year - old female with retiform variant of sertoli - leydig cell tumour . she presented with the complaint of mass in abdomen for 7 years . ultrasound revealed bilateral ovarian mass suggestive of malignancy . bilateral salpingo - oopherectomy with surgical staging was done . the tumor was diagnosed as stage i retiform variant of sertoli - leydig cell tumor on histopathology and immunohistochemistry .

solid pseudopapillary tumor ( spt ) of the pancreas has been variously designated as solid and cystic tumors , solid and papillary epithelial neoplasms , papillary cystic neoplasms , papillary cystic tumors , and frantz 's tumors . the world health organization renamed this tumor as spt in the international histological classification of tumors . spt represents a rare tumor that occurs most frequently in young women ( 90% ) . although spt is considered an indolent lesion with a low malignant potential and a favorable prognosis after surgical resection , some cases of locally infiltrating and metastatic varieties , or recurrences after surgery , have been reported . a 74-year - old woman was admitted to the hospital for fever and general weakness . on the computed tomography scan , there was large abscess cavity showing partially septated cystic lesion with peripheral inflammatory hyperemia in the right hepatic lobe . incidentally , a lobulated , 5 3.2 cm , heterogeneous pancreatic mass containing several intratumoral calcification and cystic change on the tail of the pancreas and a 6-cm , well - defined mass with heterogeneous contrast enhancement in the left lobe of the liver were detected ( fig . 1 ) . two months after conservative treatment for hepatic abscess , the abscess was completely resolved . however , the mass in the left lobe of the liver remained unchanged and then ultrasound - guided liver biopsy was performed . the tumor consisted of papillary neoplasm showing monomorphic polyhedral cells with hyalinized fibrovascular stalks , which was best classified as metastatic spt of the pancreas . immunohistochemistry was positive for vimentin , antitrypsin , and neuron specific enolase . on the presumption of an spt of the pancreas with hepatic metastasis , the patient underwent radical antegrade modular pancreatosplenectomy with hepatic resection . on operation field encapsulated masses , 6 5 cm and 5 4.5 cm in size , were found in the segment of the left liver and in the tail of the pancreas , respectively . microscopically , the growth pattern of the pancreas tumor was heterogeneous , with a combination of solid and pseudocystic structures in varying proportions . the tumor was composed of monomorphic polyhedral cells with hyalinized fibrovascular cores , thereby leading to the characteristic pseudopapillary appearance ( fig . . however , perineural invasion and infiltration into the peripancreatic fat tissue could be seen . the microscopic findings of the hepatic tumor were similar to the findings of the pancreatic tumor . a final diagnosis of spt of the pancreas with metastasis to the liver was made . spt is a rare neoplasm of the pancreas accounting for only 1 to 2% of all exocrine pancreatic tumors . although spt occurs in young females and is generally considered to be low malignant potential , local recurrence or distant metastases can be found in a significant number of patients . 14.7% ( 43 of 292 ) were evaluated as malignant due to metastasis ( 22 of hepatic , lymph node metastasis or peritoneal dissemination ) or invasion into adjacent organs . the prognosis for spts is excellent after curative resection ( more than 90% survival at 5 years ) . metastasis develops in less than 15% of cases and among them hepatic metastasis is most common . suggested that complete resection was associated with long - term survival even in the presence of metastatic disease . in case report series , even patients with local recurrence as well as liver and peritoneal metastasis have experienced long - term survival [ 4 - 8 ] . one study suggested that patient age tended to be older and the tumor size tended to be larger in metastasizing spt than in non - metastasizing spt . invasion of blood vessels , perineural clefts and adjacent organs , a high degree of cellular pleomorphism and an elevated mitotic rate are considered to associated with metastasis . nishihara et al . compared the histological features of metastasizing spt and non - metastasizing spt and reported that venous invasion , nuclear grade , and prominent necrobiotic nests are useful as histologic indicators for the malignant potential of spt . the present patient was very old age and had an incidental detection of the disease . microscopic findings showed focally - infiltrative and perineural invasion , while without lymph node metastasis and less than 5% of ki-67 positivity . in conclusion , we report a case of pancreatic spt with synchronous hepatic metastasis , which appeared later in life . preoperative diagnostic work - up is a cornerstone for surgical approach and complete resection should be considered for this potentially curative disease if operation is possible . the literatures reported up to date , as well as our case , demonstrate an aggressive approach to this rare low - grade malignant tumor can result in long - term survival even in patients with local invasion and distant metastasis .

solid pseudopapillary tumor of the pancreas is a rare tumor that affects young females with low malignant potential and good prognosis with more than 90% survival at 5 years . metastasis is very rare . we report the case of a 74-year - old female who had pancreatic solid - pseudopapillary tumor and synchronous hepatic metastasis .

vaginismus is defined according to dsm iv - tr as recurrent or persistent involuntary spasm of the musculature of the outer third of the vagina , which interferes with coitus and causes distress and interpersonal difficulty . the inclusion of spasm in the definition has been questioned as it has not been consistently documented . it has been classified as primary when the woman has never experienced non - painful penetrative sexual intercourse and as secondary when she has experienced non - painful penetrative vaginal intercourse in the past . vaginismus is comorbid with dyspareunia ; sensitivity and specificity of the distinction between the two , however , has been doubtful . pain due to inflammation , infections , trauma , radiotherapy , muscular and neurological causes may contribute to psychological causes . classical psychoanalytic theory conceptualized vaginismus as a conversion disorder caused by unresolved psychosexual conflicts in early childhood . vaginismic women have been characterized as fixated or regressed to the pre - oedipal or oedipal stages . vaginismus is believed to be a psycho - physiologic disorder due to fear from actual or imagined negative experiences with penetration and/or organic pathology . women with vaginismus have also been noted to have a lack of sex education . in this case report , we discuss the successful treatment of vaginismus with sex therapy based on a model proposed by keith hawton . a 25-year - old lady sought consultation with the psychiatry outpatient services for sexual dysfunction . she reported of tightness of vagina and introital pain while attempting sex with her husband . patient 's husband disclosed that few months back when they got engaged , she had expressed apprehensions about having painful sexual intercourse . after the wedding , patient had postponed attempts at penetrative intercourse for 10 days . whenever penetration was attempted , she would not part her legs and begin to cry . she had initially consulted gynecologists who diagnosed the condition as primary vaginismus . on their advice , after about 6 weeks , the couple consulted psychiatry services on advice by the gynecologist . the patient reported of sadness , ideas of worthlessness and depressive symptoms over the past 56 weeks . the family of origin was religious ; sex was not openly discussed in keeping with local cultural norms . her mental status examination revealed depressed affect , ideas of hopelessness and worries about non - consummation of marriage . the patient underwent five sessions ( weekly ) of sex therapy with the first author being the primary therapist . the sessions included the husband and the couple initially participated jointly in educative sessions with the primary therapist . the exercises were carried out at home by the patient and her husband . in the first session , normal reproductive anatomy and physiology of the sexual act were explained . the patient was made comfortable with her genitals by asking her to look at the area in the mirror . kegel 's exercises help control the pubococcygeus muscle which surrounds the entrance to the vagina . in the next couple of sessions , she was advised to insert her fingers into her vagina and move them around , initially one finger , later two fingers . only after the patient became comfortable with these over three sessions , vaginal containment with lubrication and local anesthesia provided by 5% lignocaine jelly was advised . vaginal containment involved the patient in female superior position , guiding penile penetration with her hands and the couple remaining still , concentrating on the pleasant sensations they experience . after a month of initiating therapy , the patient was able to indulge in normal sexual intercourse without the need for local anesthesia . james marion sims first coined the term vaginismus in 1862 at an address to the obstetrical society of london . vaginismus is thought to be one of the most common female psychosexual dysfunctions but the exact prevalence rate among the general population is not known . however , in sexual dysfunction clinics , the rates vary from 5 to 17% . this condition can result in significant interpersonal problems and marital discord . botulinum toxin injection and hypnosis are the other approaches that have been tried . there are no randomized controlled trials available to garner evidence for treatment of vaginismus . discussed here , an eclectic approach was used involving education , graded insertion of fingers , kegel 's exercises and usage of anesthesia with vaginal containment . the use of ssri escitalopram may also have aided the therapy by providing anxiolysis and relief from depression . the report demonstrates a successful approach toward managing vaginismus in a clinical setting . there is a need for randomized controlled trials to establish efficacy and bolster these approaches .

vaginismus is defined as recurrent or persistent involuntary spasm of the musculature of the outer third of the vagina , which interferes with coitus and causes distress and interpersonal difficulty . in this report , we describe the successful treatment of vaginismus in a 25-year - old lady based on a model proposed by keith hawton . the eclectic approach involved education , graded insertion of fingers , kegel 's exercises and usage of local anesthesia with vaginal containment along with the prescription of escitalopram .

she was diagnosed with subfoveal cnv secondary to age - related macular degeneration ( amd ) and the best - corrected visual acuity of the right eye was 20/50 . pdt with verteporfin was performed according to the standard protocol of the treatment of age - related macular degeneration with photodynamic therapy investigation and repeated after 5 months.13 the visual acuity of the involved eye did not change . fifteen months after the second pdt , fluorescein angiography of the affected eye revealed persistent subfoveal cnv and the best corrected visual acuity remained 20/50 ( fig . 1a , 2a ) . combined pdt according to the same standard protocol and 1.25 mg intravitreal bevacizumab injection was performed on the same day . one day after the treatment , the patient 's vision had decreased to counting fingers . fundus examination and optical coherence tomography ( oct ) showed serous retinal detachment involving the macula ( fig . , the patient was initiated on an oral steroid ( triamcinolone 24 mg # 1 ) to complete a two week course . one week into steroid therapy , the visual acuity remained the same , but oct findings of the right eye showed decreased serous retinal detachment ( fig . 2c ) . the vision slightly improved to 20/200 upon completion of the two week steroid course and fluorescein angiography showed decreased leakage of fluorescein dye ( fig . there have been several reports about serous retinal detachment after pdt treatment.7,9,14 mennel et al.7 quantified transient serous retinal detachment in cnv after pdt using oct . kim et al.9 also suggested that serous retinal detachment might develop following pdt but might also resolve spontaneously several days following treatment . however , there is neither a published report of serous retinal detachment after the combined use of pdt and intravitreal bevacizumab injection nor following intravitreal bevacizumab injection alone . several mechanisms may be responsible for subretinal fluid accumulation after combined treatment with pdt and intravitreal bevacizumab injection . the pdt may induce fluid leakage from the cnv itself or damage of retinal pigment epithelium causing an alteration of the blood - retinal barrier.7,9 bevacizumab may cause instability of the intraretinal and subretinal fluid and subsequent serous retinal detachment by rapidly modulating the permeability and activity of the cnv.10,12 in our patient , there was no evidence of increased subretinal fluid after the first two treatments of pdt alone and the pdt protocol was the same for all three treatments . occurrence of the serous retinal detachment just after combined pdt and intravitreal bevacizumab injection suggests the interaction of these two modalities as a possible causal role . however , the definitive causation of serous retinal detachment in pdt alone , intravitreal bevacizumab injection alone or combined treatment can not be determined . a two week course of oral steroids was used to manage the serous retinal detachment after the combined treatment of pdt and intravitreal bevacizumab injection . kim et al.9 injected triamcinolone into the vitreous in a patient with serous retinal detachment following pdt , whereas , we elected to use a more conservative management with an oral steroid . in conclusion , combined pdt and intravitreal bevacizumab injection can be associated with serous retinal detachment which may cause sudden visual loss . additional studies are needed to establish the standard protocol , safety and complications regarding the combined treatment of pdt and intravitreal bevacizumab injection .

we report a case of serous retinal detachment following combined photodynamic therapy ( pdt ) and intravitreal bevacizumab injection in subfoveal choroidal neovascularization ( cnv).a 53-year - old woman was diagnosed with subfoveal cnv secondary to age - related macular degeneration ( amd ) and treated with combined pdt and intravitreal bevacizumab injection . one day after treatment , the patient experienced a sudden decline of vision and optical coherence tomography ( oct ) showed serous retinal detachment involving the macula . she was managed conservatively with an oral steroid beginning on the second day of the combined treatment and the subretinal fluid started to decrease one week following the initiation of steroids.this case suggests that combined pdt and intravitreal injection of bevacizumab can be associated with serous retinal detachment . additional studies are needed to establish the safety and complications following this treatment regimen .

the prospective investigation conducted by fleischhackl and coworkers , reported in the previous issue of critical care , sets out to determine whether young students have the physical and cognitive skills to implement cardiopulmonary resuscitation ( cpr ) . in this investigation , the average time from the last class of cpr instruction to the evaluation session was 120 days . it is not clear whether such a large gap in time between initial instruction and skills testing may have affected testing performance , except that good performance could indicate good retention . students tested also included those with special learning needs , which may skew the examination results in an unfavorable direction . nevertheless , the outcomes were generally very good . as the investigators demonstrated , students as young as 9 years are able to effectively learn cpr skills , including automated external defibrillator deployment , correct recovery position , and emergency calling . as in adults , physical strength may have limited the depth of chest compressions and ventilation volumes , but perhaps the key finding was that skills retention was good . these findings are consistent with other studies in which none of the students aged 9 to 10 years could compress the chest to the depth recommend by the guidelines , but 45% of students aged 13 to 14 years old could . studies also have found that with retraining , cpr performance can improve in school - aged children . although the study by fleischhackl and coworkers did not specifically address this retraining , it would have been interesting to know how well these students would have retained their learned skills several months later , because it is well known that cpr skills rapidly deteriorate after initial training . intuitively , the higher the number of persons trained in cpr skills in a given community , the more frequently it is performed . for example , in los angeles , where the relative percentage of citizens trained in cpr is estimated to be relatively low , only a small percentage of bystanders performed cpr and just 1.4% of out - of - hospital cardiac arrest victims survived . however , 10-fold improvements in survival rates ( > 15% ) have been reported in seattle , where the frequency of bystander - performed cpr is one of the highest in the nation . in essence , one of the most effective means of improving surviving for out - of - hospital cardiac arrest is to attempt to train all individuals across a given community , and experience has shown that having captive audiences for training , such as in school systems , is a major component . when closely examining such numbers , the survival improvements are not linear but may actually be logarithmic . young children between the ages of 10 and 14 years only account for approximately 15% of the population in the usa , and they are not in the main target age group for cpr except for perhaps drowning incidents . however , there are multiple benefits from teaching school - aged children cpr beside the concept of capturing entire generations of cpr - trained citizens . children older than 10 years of age are teachable and capable of abstract thought , and most have the coordination and physical strength to perform cpr . the cpr kits that are currently being promoted by the american heart association are designed to train individuals who are as young as 8 years old in less than a half hour . it has been demonstrated that using such a kit that contains a self - instructional video and an inflatable manikin not only promotes retention but also has a multiplier effect . one study found that distributing cpr training kits to students aged 12 to 14 years resulted in another 2.5 persons trained per student . furthermore , early training can lay the foundation for a sense of social obligation and reinforce cpr knowledge and follow - up training , so that by the time a student graduates from high school cpr skills are well engrained and can easily called upon in an emergency situation . if government agencies enforced mandatory age - appropriate cpr and first aid training in schools , similar to how schools practice evacuation and file drills , then one could only imagine the impact this could have on the number of individuals capable of intervening in out - of - hospital cardiac arrest and , in turn , the number of lives saved .

the usefulness of basic cardiopulmonary resuscitation ( cpr ) training in school systems has been questioned , considering that young students may not have the physical or cognitive skills required to perform complex tasks correctly . in the study conducted by fleishhackl and coworkers , students as young as 9 years were able to successfully and effectively learn basic cpr skills , including automated external defibrillator deployment , correct recovery position , and emergency calling . as in adults , physical strength may limit the depth of chest compressions and ventilation volumes given by younger individuals with low body mass index ; however , skill retention is good . training all persons across an entire community in cpr may have a logarithmic improvement in survival rates for out - of - hospital cardiac arrest because bystanders , usually family members , are more likely to know cpr and can perform it immediately , when it is physiologically most effective . training captured audiences of trainees , such as the entire work - force of the community or the local school system , are excellent mechanisms to help achieve that goal . in addition to better retention with new half hour training kits , a multiplier effect can be achieved through school children . in addition , early training not only sets the stage for subsequent training and better retention , but it also reinforces the concept of a social obligation to help others .

neuroleptic malignant syndrome ( nms ) is a rare but life - threatening complication to antipsychotic agents . it was initially reported in 1960s and since then many cases have been recognized worldwide . nms is associated with a significant mortality rate and requires early recognition and prompt treatment . early diagnosis of nms can be a clinical challenge to family physicians who treat patients with both medical and psychiatric conditions . with increasing the use of antipsychotic agents in primary and secondary care setting , nms can be missed if not suspected in patients on antipsychotics presenting in general practice with hyperthermia and muscle rigidity . we report a case of nms diagnosed in a patient with poorly controlled diabetes in the urban health center at vellore , tamil nadu , india . a 34-year - old male with history of type 2 diabetes mellitus on oral hypoglycemic agents was hospitalized twice with symptoms of ketosis . poor drug compliance , poor motivation regarding diabetes care , and psychomotor agitation were noted during hospitalization . one week after starting on antipsychotics , he presented with extrapyramidal symptoms of dystonia , parkinsonian gait , fine tremors , high spiking fever , altered sensorium , and muscle rigidity . blood counts , urine microscopy , and renal function were normal except for low sodium [ table 1 ] . creatine phosphokinase ( cpk ) was ordered in view of muscle rigidity that was very high ( 1543 ) . a diagnosis of nms was made according to diagnostic and statistical manual of mental disorders ( dsm - iv ) criteria . laboratory abnormalities in our patient risperidone was stopped immediately , and he was treated with lorazepam , trihexyphenidyl , paracetamol , and intravenous fluids in consultation with psychiatrist . within 48 h of hospitalization , family was informed of the diagnosis and the need for close monitoring of his glycemic control . nms is a rare condition as reported in the two studies done in neurology and psychiatric units of teaching hospitals in india with an incidence of 1.401.41/1000 cases treated with antipsychotics . among the risk factors , some studies report older age as high - risk due to the associated medical morbidities , nutritional deficiencies , dehydration and electrolyte abnormalities , male gender , and genetic predisposition . other studies report more cases in the age group of 2050 years associated with high antipsychotic dosage as seen in our patient . the antidopaminergic activity of antipsychotic drugs is associated with the symptoms of muscle rigidity , hyperthermia , autonomic dysfunction , and mental status change . these symptoms are recognized as diagnostic according to the american psychiatric associations dsm - iv . the signs and symptoms develop during a 2472 h period following the administration of antipsychotic drug ; however , it can develop later as seen in our patient . newer antipsychotic agents such as risperidone block serotonin receptors more than dopamine receptors ; however , nms has been reported with their use as in our case study . anti - emetics such as metoclopramide and droperidol have been linked to nms by their dopaminergic blocking activity . laboratory abnormalities may include leukocytosis , electrolyte disturbances , and elevated cpk secondary to muscle damage . diagnostic tests for fever may include urine analysis , chest radiography , and lumbar puncture . imaging studies of brain are not diagnostic of nms , however , may rule out other causes of altered mental status . possible complications include dehydration from poor oral intake , renal failure secondary to rhabdomyolysis , and coagulation abnormalities . mortality in nms has decreased from 76% to between 10% and 20% , however , complete recovery is noted in most patients . mortality is caused by complications such as respiratory failure , cardiovascular collapse , renal failure , arrhythmias , and thromboembolism . patients who are hemodynamically unstable are to be transferred to higher centers for intensive monitoring . mild cases can be managed at the secondary care setting in consultation with a psychiatrist . supportive therapy involves discontinuation of antipsychotic agents , correction of electrolyte imbalances , nutritional deficiencies and monitoring of airway , breathing , and circulation . our patient was managed at the urban health center by the team of family physicians . specific dopaminergic agents such as bromocriptine , dantrolene , and electroconvulsive therapy as an option are considered for more severe cases by psychiatrists . symptoms may last for a month in patients who were on depot preparations . restarting antipsychotics in patients with history of nms if needed is done on consultation with psychiatrist . depot preparations are generally not recommended , however , a 2 weeks interval is to be considered between recovery and restarting antipsychotic agents . early detection and management of side effects caused by neuroleptic agents are of particular consideration to family physicians who attend to the early symptoms . decreasing risk factors that aggravate rigidity include avoiding dehydration , minimal use of restraints and intramuscular injections , adequate nutrition , screening for history of nms in the patient , or other family members .

neuroleptic malignant syndrome ( nms ) is a life - threatening emergency that is often seen as a complication of antipsychotic agents . it is characterized by a tetrad of motor , behavioral , autonomic , and laboratory abnormalities . we report a case of a 34-year - old man with a history of newly diagnosed type 2 diabetes mellitus , mental retardation , and behavioral abnormalities who developed nms after starting on antipsychotic agents . he presented with high temperature , muscle rigidity , tachycardia , and elevated blood pressure . after a week of hospital treatment in the general ward of a secondary care unit , he was discharged in a hemodynamically and mentally stable state .

dense , vision - obscuring calcification on the posterior aspect of silicone intraocular lenses ( iols ) is often not amenable to neodymium : yttrium - aluminum - garnet capsulotomy , and , in prior reports , has required iol exchange . we report the successful removal of dense calcium deposition on the posterior surface of a three - piece silicone lens using pars plana vitrectomy ( ppv ) . a light pipe was used to retroilluminate the iol , and a dense fibrous tissue setting with a low cut - rate and high aspiration rate was able to clear the visual axis of the dystrophic calcification without damaging the iol optic . small - gauge ppv may be utilized to remove dense dystrophic calcium deposits on the lens surface in lieu of iol exchange . dystrophic calcification of the silicone intraocular lenses ( iols ) is a rare complication that can diminish visual acuity and contrast sensitivity . dystrophic calcification has been reported with the use of silicone iols after vitreous hemorrhage or in patients with asteroid hyalosis , and it has been primarily treated with lens explantation , as neodymium : yttrium - aluminum - garnet ( nd : yag ) laser treatment often can not remove dense deposits.1,2 the first report was published in 2004 by wackernagel et al describing the composition of deposits on an explanted silicone lens 4 years after implantation.3 recently , mamalis et al reported the presence of calcium and phosphate in 16 explanted opacified silicone lenses from various manufacturers ; 86.4% of those lenses were in patients with confirmed asteroid hyalosis . concurrently , the authors analyzed 111 calcified hydrophilic acrylic lenses ; none were associated with a history of asteroid hyalosis.4 previous treatments of lens opacification have relied on the lens explantation alone with or without subsequent reimplantation of a new lens.14 this requires a large incision and risks refractive change . herein , we describe a novel approach to treating patients with vision - obscuring dense calcification of silicone lenses without explantation of the iol . a 78-year - old woman underwent bilateral phacoemulsification and three - piece clariflex silicone iol insertion ( abbott medical optics inc , santa ana , ca , usa ) . she had no significant past medical history and a past ocular history of asteroid hyalosis , greater in the right eye , and wet macular degeneration in the left eye with a central scar . also , 9 years later , she described decreased vision in her right eye . visual acuity was 20/100 in the right eye and count - fingers in the left eye . examination revealed dystrophic calcification on the posterior surface of the right intraocular lens ( figure 1 ) . at that time , a nd : yag capsulotomy was unable to clear the calcification . to access the dystrophic calcification , a pars plana approach was taken using a stellaris pc 23-gauge vitrectomy system ( bausch and lomb incorporated , bridgewater , nj , usa ) . after performing a core and anterior vitrectomy , a dense fibrous tissue setting was used with a high aspiration rate of 600 and a variable cut rate less than 1,500 cuts / minute to slowly remove the calcified material from the posterior aspect of the iol , along with the posterior capsule . after clearing the central optic , her visual acuity was 20/25 without correction on postoperative day one , and the central optic was clear of dystrophic material ( figure 2 ) . at 1 year , no material had reaccumulated on the central optic , and her vision remained stable at 20/25 ( figure 3 ) . calcification of the posterior surface of silicone iols in patients with asteroid hyalosis is a rare but well - described late complication after cataract surgery.2 prior reports in the treatment of this dystrophic calcification have required iol explantation or exchange . a case series studying 22 iol exchanges identified complications in 36.4% of procedures , including corneal decompensation , loss of capsular support , vitreous prolapse , and an uncertain refractive outcome.57 in comparison , the united kingdom national ophthalmology database study of vitreoretinal surgery found only 7.8% of pars plana vitrectomies ( ppv ) had an intraoperative complication , the most common of which were iatrogenic retinal breaks ( 3.2%).8 the use of a high - speed , small - gauge vitrectomy system to clear calcification with lasting results via a posterior approach allows use of the existing iol with a more rapid surgical and visual recovery compared to iol exchange . patients maintain the same intraocular optics achieved immediately after cataract surgery and the chance of refractive alteration associated with iol exchange is minimized . in theory , removing the asteroid bodies during vitrectomy decreases the risk of future calcium deposition on the silicone iol optic . in the interest of saving a patient from the risks and refractive uncertainty of an iol exchange , small - gauge ppv may be utilized to remove dense dystrophic calcium deposits from the lens surface .

purposedense , vision - obscuring calcification on the posterior aspect of silicone intraocular lenses ( iols ) is often not amenable to neodymium : yttrium - aluminum - garnet capsulotomy , and , in prior reports , has required iol exchange . we report the successful removal of dense calcium deposition on the posterior surface of a three - piece silicone lens using pars plana vitrectomy ( ppv).materials and methodsa 23-gauge ppv was performed using the stellaris vitrectomy system . a light pipe was used to retroilluminate the iol , and a dense fibrous tissue setting with a low cut - rate and high aspiration rate was able to clear the visual axis of the dystrophic calcification without damaging the iol optic.resultsvisual acuity improved from 20/100 to 20/25.conclusionsmall - gauge ppv may be utilized to remove dense dystrophic calcium deposits on the lens surface in lieu of iol exchange .

hearts , isolated from wildtype zebrafish embryos ( 24hpf to 72hpf ) were stained with the transmembrane potential - sensitive dye di-8-anepps ( invitrogen ) or the calcium - sensitive ratiometric dye fura-2 , am ( invitrogen ) for the measurements of action potentials and calcium transients , respectively . fluorescence intensities were recorded with a high - speed ccd camera ( redshirt imaging ) . propagation velocities of depolarizing waves were estimated as previously described , with some modifications ( see full description in the online methods ) . hearts , isolated from 72hpf zebrafish embryos were fixed in prefer fixative ( anatech ltd . ) and stained with primary antibodies : rabbit anti - connexin43 ( sigma ) 1:50 , mouse anti--catenin ( bd biosciences ) 1:200 , mouse anti - zn8 ( dshb ) 1:50 ; secondary antibodies : donkey anti - rabbit or mouse alexa - fluor-488 or -546 conjugated ( invitrogen ) 1:1000 . wnt11:cfp fusion protein was cloned downstream of cmlc2 promoter into the tol2kit expression system using gateway technology ( invitrogen ) . 10 g/l or 25 g/l of dna was co - injected with 25 g/l capped tol2 transposase mrna into 1 cell stage embryos . scientific ) was added to 30hpf old zebrafish embryos in egg water buffered with 5 mm hepes . isolated zebrafish hearts were incubated in a mix of thapsigargin 10 m ( sigma ) and caffeine 10 mm ( sigma ) in tyrode s solution for 45 min prior to optical imaging . hearts , isolated from wildtype zebrafish embryos ( 24hpf to 72hpf ) were stained with the transmembrane potential - sensitive dye di-8-anepps ( invitrogen ) or the calcium - sensitive ratiometric dye fura-2 , am ( invitrogen ) for the measurements of action potentials and calcium transients , respectively . fluorescence intensities were recorded with a high - speed ccd camera ( redshirt imaging ) . propagation velocities of depolarizing waves were estimated as previously described , with some modifications ( see full description in the online methods ) . hearts , isolated from 72hpf zebrafish embryos were fixed in prefer fixative ( anatech ltd . ) and stained with primary antibodies : rabbit anti - connexin43 ( sigma ) 1:50 , mouse anti--catenin ( bd biosciences ) 1:200 , mouse anti - zn8 ( dshb ) 1:50 ; secondary antibodies : donkey anti - rabbit or mouse alexa - fluor-488 or -546 conjugated ( invitrogen ) 1:1000 . wnt11:cfp fusion protein was cloned downstream of cmlc2 promoter into the tol2kit expression system using gateway technology ( invitrogen ) . 10 g/l or 25 g/l of dna was co - injected with 25 g/l capped tol2 transposase mrna into 1 cell stage embryos . scientific ) was added to 30hpf old zebrafish embryos in egg water buffered with 5 mm hepes . isolated zebrafish hearts were incubated in a mix of thapsigargin 10 m ( sigma ) and caffeine 10 mm ( sigma ) in tyrode s solution for 45 min prior to optical imaging .

electrical gradients are critical for many biological processes , including the normal function of excitable tissues , left - right patterning , organogenesis , and wound healing14 . the fundamental mechanisms that regulate the establishment and maintenance of such electrical polarities are poorly understood . using high - speed optical mapping of transmembrane potentials and calcium concentrations in the zebrafish heart , we have identified a gradient of electrical coupling across the developing ventricular myocardium . we excluded a role for cellular excitability , connexin localization , tissue geometry and mechanical inputs , but in contrast we were able to demonstrate that non - canonical wnt11 signals are required for the genesis of this myocardial electrical gradient . importantly , though the traditional planar cell polarity pathway is not involved , we obtained evidence that wnt11 acts to set up this gradient of electrical coupling through effects on transmembrane ca2 + conductance mediated via the l - type calcium channel . these data reveal a previously unrecognized role for wnt / ca2 + signaling in establishing an electrical gradient in the plane of developing cardiac epithelium through modulation of ion channel function . the regulation of cellular coupling through such mechanisms may be a general property of non - canonical wnt signals .

a 77-year old female was referred to our clinic with a prolonged period of delayed healing following the uneventful extraction of the lower right second molar tooth in may 2007 . at this point the patient had been taking alendronic acid 70 mg orally once per week for seven years . clinical examination revealed only mild erythema overlying the socket , with no evidence of any osteonecrotic process , either clinically of radiographically . the patient attended the following year in november 2008 with right sided facial pain following recent curettage of the affected socket by her dentist . the patient was discharged following an extended course of penicillin v and metronidazole accompanied with chlorhexidine mouthwashes . over three years later in june 2012 the patient re - attended with a repeat occurrence of her symptoms . clinically there was no evidence of infection or discharge but radiographically there remained a clearly demarcated radiolucency over the right posterior mandible ( fig . the histopathology findings confirmed bone necrosis consistent with mronj , and ruled out dysplasia or malignancy . the patient s most recent attendance was in october 2013 where she attended with increasing trismus and some swelling over the right posterior mandible . radiographic examination revealed a much more extensive area of necrosis at the right body and ramus of the mandible spreading to involve the coronoid process and the appearance of a pathological fracture ( fig . 2 ) . a ct image was taken to assess the extent of the necrosis . exploration of the right side of the mandible with removal of the fractured coronoid process and debridement of necrotic bone was carried out at the royal surrey county hospital in december 2013 . an incision was made to explore the body , ascending ramus , and coronoid process of the mandible . the coronoid process was gripped firmly to prevent the temporalis pulling the fragment superiorly , and gently removed ( fig . once the coronoid process was removed , gentle curettage of the necrotic bone was carried out using a slow hand piece and saline irrigation . necrotic bone was removed leaving a margin of bleeding bone with normal appearance thus indicating sufficient metabolic potential for healing to take place ( fig . 8) [ 36 ] . the patient was admitted for two nights following the procedure where she received intravenous metronidazole , and was discharged with oral antibiotics and chlorhexidine mouthwashes four times per day for two weeks . this case demonstrates the unpredictable nature of mronj and how the disease can progress to cause significant morbidity . in this case extensive surgery was required to remove the necrotic fragments of bone with no guarantee that the necrosis will stop spreading . at present , the incidence of mronj in patients taking alendronic acid for osteoporosis is 1:10001:1,700 however , this incidence increases with the duration of treatment [ 79 ] . our case illustrates the extent of the damage that can be caused when straightforward dental procedures are carried out in low risk patients taking oral bisphosphonates . it is important to realise that osteonecrosis may remain asymptomatic for long periods in the absence of infection . it seems a matter of great importance that national evidence based guidelines are published on the dental treatment of patients taking bisphosphonates , and that further information regarding the possible consequences of bisphosphonate use is given to general medical practitioners . there are no conflicts of interest . written informed consent for publication in print and electronic form , and publication of radiographic and photographic images written informed consent for publication in print and electronic form , and publication of radiographic and photographic images was granted by the patient . authorsconception and design of study / review / case seriesacquisition of data : laboratory or clinical/ literature searchanalysis and interpretation of data collecteddrafting of article and/or critical revisionfinal approval and guarantor of manuscriptadam jowett clinical/ literature searchanwer abdullakutty clinical/ literature searchmalcolm bailey clinical/ literature search

highlightsthis case reports the first case to our knowledge of pathological fracture of the coronoid process of the mandible secondary to long term use of alendronic acid.demonstrates the unpredictable nature of symptoms associated with medication related osteonecrosis and its management within the hospital environment.demonstrates the rapidly progressing and unpredictable nature of the spread of the necrotic process in medication related osteonecrosis.clear clinical photographs of the surgical procedure involved in the removal of necrotic bone and curettage of the surgical site in medication related osteonecrosis .

chronic subdural hematoma ( csdh ) is a common clinical entity treated by neurosurgeons with very good cure rates . recurrence of the hematoma , infection , seizure , cerebral edema , tension pneumocephalus and failure of the brain to re - expand are well - recognized complications following surgery for csdh . an intracerebral or intraventricular hemorrhage following such an evacuation is very rarely observed ( incidence ~0.7 - 5% ) , albeit with devastating consequences . we report the finding of a subependymal hemorrhage after evacuation of a csdh and discuss the probable causative mechanisms underlying this rare occurrence and the prevention strategies to avoid it . past history revealed a minor fall with head injury 1 month ago for which no medical attention was sought . examination was unremarkable , except for the presence of pronator drift on the left side . ct ( computed tomography ) of the head showed a right csdh , measuring 15 mm in thickness and causing a 7 mm midline shift to the left [ figure 1a and b ] . after baseline investigations which documented a normal coagulation profile , the patient was taken for right frontal and parietal burr holes and drainage of the subdural hematoma under mac ( monitored anesthesia care ) with conscious sedation . after making the burr holes , the dura was coagulated with bipolar cautery and incised in a cruciate manner . the cut edges of the dura were further cauterized to shrink the dura and keep the dural opening patent . the subdural collection was allowed to drain without any negative pressure being applied in the subdural cavity . after the subdural hematoma had drained on its own accord ( owing to the pressure differential ) , the cavity was gently irrigated with normal saline till the returning fluid was clear . ct head done immediately post - surgery revealed a subependymal bleed in the right lateral ventricle , extending laterally into the thalamus and medially into the body of the lateral ventricle [ figure 1c and d ] . in spite of these findings in the post - operative ct , the patient did well in the post - operative period with complete resolution of headache and improvement of the subtle pyramidal deficit on the left side . ( a and b ) pre - operative ct head showing right chronic subdural hematoma . ( c and d ) post - operative ct head showing subependymal bleed in the right thalamus with extension into the lateral ventricle as mentioned above , do tend to jeopardize the good outcome normally expected in these patients ; but none are as devastating as the rare complication of intracerebral hemorrhage ( ich ) after evacuation of csdh . several causative mechanisms have been postulated to explain this complication following a simple surgical procedure . beneath the csdh , focal cerebral edema due to impeded venous drainage chronic dilatation of small arterial vessels and loss of carbon dioxide reactivity in the ischemic hemisphere could also contribute to the pathogenesis . possible pathogenic mechanisms , for ich in the thus compromised brain , include hemorrhage into previously undetected areas of contusion , damage to cerebral vasculature secondary to rapid peri - operative parenchymal shift , and sudden increase in cerebral blood flow ( post - decompression ) combined with focal disruption of autoregulation . compression by extra - axial collection decreases cerebral blood flow on the affected hemisphere and alters its vascular self - adjustment . some authors hypothesize that the rapid increase in cerebral blood flow ( after drainage ) in areas of the brain with altered vascular self - adjustment may be the most likely precipitating mechanism of intracerebral hemorrhage after surgical evacuation of csdhs . , as the compressed brain is more toward the surface , this mechanism is likely to explain superficial or lobar hematomas . we hypothesize that after sudden decompression of the brain , the differential expansile qualities of the solid ( brain ) and liquid ( csf ) components of the cranium may result in mechanical stress at the interface , which in turn , may cause rupture of the engorged subependymal veins ( due to prolonged compression ) . chronic sdh being a benign entity , neurosurgical outcome have to be optimized for this entity . considering that intracerebral hemorrhage inadvertently portends a poor outcome for the affected patients , preventive steps are imperative . these include gradual and graded decompression of the chronic sdh ( especially for large collections ) , use of closed drainage system which facilitates slow re - expansion of the brain , and careful control of blood pressure lability in the peri - operative period . the gradual and graded decompression can be achieved by covering the dural aperture with a cottonoid after draining around 15 to 20 ml of the subdural hematoma . after waiting for approximately 5 min , this procedure can be continued till the subdural pressures equalize with the atmospheric pressures , and at this juncture , saline washes can be instituted . this especially holds true for very large subdural hematomas , presenting with raised intra - cranial pressure . our patient was fortunate to have a good outcome as the bleed was moderate in size , however , we advocate taking steps to prevent such complications altogether .

chronic subdural hematoma ( csdh ) is a common clinical entity treated by neurosurgeons with good cure rates . recurrence of the hematoma and failure of the brain to re - expand are commonly observed complications following surgery for csdh . an intracerebral or intraventricular hemorrhage following evacuation of csdh is very rarely observed , albeit with devastating consequences . we report the finding of a subependymal hemorrhage after evacuation of a csdh and discuss the probable causative mechanisms underlying this rare occurrence and the prevention strategies to avoid it .

both f-18 fluorodeoxyglucose ( fdg ) and c-11 methionine pet / ct studies were done in a treated case of glioblastoma multiforme who was sent for evaluation of recurrence . apart from recurrence at the primary site a subependymal deposit was noted in the trigone of the lateral ventricle which was well demonstrated on the c-11 methionine study as compared to the f-18 fdg study . this case therefore highlights the important role that c-11 methionine can play for evaluating not only recurrence but also metastatic deposits which are a possibility in such high grade tumors . a 10-year - boy post - operative , post - radiotherapy case of left temporal glioblastoma multiforme ( gbm ) was referred for f-18 flurodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) to rule out residual / recurrent disease 6 months following completion of therapy . the fdg scan 3 months following therapy had not shown evidence of viable residual or metastatic disease . 185 mbq of f-18 fdg was injected intravenously and the patient was rested for one hour followed by the pet / ct acquisition on a discovery ste 16 camera ( ge ) . low dose ct was followed by 3d pet emission scan of the brain for 15 minutes . images were reconstructed by 3d vue algoritm ( ge ) and viewed on a xeleris workstation ( ge ) using the volumetrix protocol . a tiny focus of abnormal fdg accumulation was noted in the region of the trigone of the left lateral ventricle which was best appreciated on the plain pet image [ figure 1-arrow ] . coronal , saggital and transaxial fused f-18 fdg pet / ct images a correlative c-11 methionine study was done the next day 20 minutes following intravenous injection of 740 mbq of the tracer . a well - defined focus of abnormal tracer accumulation in the region of the left trigone [ figure 2-arrow ] was well - appreciated on the mip , plain pet and fused pet / ct methionine images . f-18 fdg uptake can vary greatly but uptake is high in high grade tumors and their metastases . however , because of the high rate of physiologic glucose metabolism in normal brain tissue the detectability of lesions is restricted . amino acid pet tracers like c-11 methionine in contrast have high uptake in tumor tissue and low uptake in the normal brain , thus giving better lesion to background ratios . they are transported into the cell via carrier mediated transport processes and transport is upregulated following malignant transformation . in this case because the subependymal deposit was away from the grey matter it could be visualised on f-18 fdg pet , however this deposit was clearly delineated on the c-11 methionine study and correlated well with the cect and mri findings [ figure 3 ] . gbm is the most aggressive type of brain tumor and has a very poor prognosis . the tumor may extend into the meninges or ventricular wall and malignant cells carried in csf may spread to the spinal cord or cause meningeal gliomatosis . though various studies in literature have compared f-18 fdg with c-11 methionine for evaluation of gliomas and recurrent brain tumors.[68 ] their comparison in metastases such as this subependymal deposit has not been reported . the possibility of subependymal metastases should be kept in mind while reporting the f-18 fdg brain study in gbm and these deposits can be well delineated with amino acid tracer such as c-11 methionine study . coronal , saggital and transaxial fused c-11 methionine pet / ct images ( a ) axial cect done subsequently showing the mildly enhancing subependymal nodule and ( b ) axial flair mr sequence showing the hyperintense subependymal nodule in the trigone of the left lateral ventricle

a 10-year - boy post - operative , post - radiotherapy case of left temporal glioblastoma multiforme ( gbm ) was referred for f-18 flurodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) to rule out residual / recurrent disease 6 months following completion of therapy . the fdg scan 3 months following therapy had not shown evidence of viable residual or metastatic disease . the present scan showed a tiny focus of abnormal fdg accumulation in the region of the trigone of the left lateral ventricle which was best appreciated on the plain pet image . a correlative c-11 methionine study showed a well defined focus of abnormal tracer accumulation in the region of the left trigone . cect and mri done subsequently proved it to be a subependymal deposit . this case therefore demonstrates the possibility of subependymal deposits in gbm and the need for this possibility to be entertained during interpretation of the fdg study . it also highlights the advantage of labelled amino acids like c-11 methionine for clearly delineating subependymal deposits apart from the advantage for unequivocal interpretation of the pet study in recurrent brain tumors .

the mind is never passive ; it is a perpetual activity , delicate , receptive , responsive to stimulus . you can not postpone its life until you have sharpened it . whatever interest attaches to your subject - matter must be evoked here and now ; whatever powers you are strengthening in the pupil , must be exercised here and now ; whatever possibilities of mental life your teaching should impart , must be exhibited here and now . that is the golden rule of education , and a very difficult rule to follow . ( alfred north whitehead , presidential address to the mathematical association , january 1916 ) peets and colleagues report on a strategy for selecting content for inclusion in a critical care curriculum for residents . the authors constructed a three - domain classification of common clinical problems and asked resident trainees and attendings to score each problem according to the threat to life , to frequency and to reversibility . the scales were organized to give greatest weight to greater life - threat , higher frequency and ease of reversibility . the authors report strong concurrence between the product of domain scores of resident trainees and of their supervising attending physicians . in their conclusion , the authors assert that their process is widely applicable and ' can facilitate creation of a reliable and valid curriculum ' . it is unsurprising that residents and their teaching staff should have similar assessments of the three objective features listed . for example , brain death which appears at the bottom of the priority list is irreversible by definition . if any resident or attending scored brain death as anything other than not reversible , it would be at once surprising and problematic . similarly , the frequency of the condition of brain death in the intensive care unit ( icu ) studied and the degree to which brain death threatens life are not matters for debate . what is of greater concern , however , is that the methodology advanced by the authors results in brain death being placed at the very bottom of the needs assessment . the authors state in their key messages that their tool ' will provide content validity for any curriculum ' . herein lies the greatest problem with the methodology : it overvalues those curricular elements that focus on reversible conditions . the unfortunate fact is that many patients are admitted to the icu with conditions that either will not respond to critical care or are terminal , or both . curricula that fail to forthrightly confront this reality perpetuate costly and quixotic efforts to cure where care would be more appropriately directed towards comfort and dignity . the same scoring logic relegates end - of - life decision - making , arguably one of the most important activities in any icu , to a level of importance below that of obstetrical complications . the critical question left unaddressed in the methodology proposed for selecting curricular elements is whether the topics most highly ranked can be uniquely and best learned in the icu . for example , topics such as shock , seizure and drug overdose are highly ranked , but are also frequently encountered in the emergency department . in contrast , the management of acute and fulminant hepatic failure , and the strategies that reverse those conditions or else indicate the need for transplantation , can only be learned in the icu . yet acute and fulminant hepatic failure ranks just above brain death in the needs assessment listing . in summary , the authors should be complimented for conducting a needs assessment and also for reporting strong concurrence between trainees and supervisors . the report should serve as a basis for ensuring that acute and critical care topics are covered within a comprehensive curriculum spanning the entire training period . the fact that a condition might not be reversible should not diminish its importance in the icu curriculum . on the contrary , one might reasonably argue that brain death , fulminant organ failure and end - of - life decision - making ought to be pushed near the top of the priority list . if these topics are not explored during the icu rotation of the trainees , then where ?

curricula for residents on rotations through intensive care units are necessarily abbreviated . the selection ( and omission ) of topics can be informed by assessment of perceived needs . a curriculum can not , however , be formed exclusively from the top - scoring needs . topics that are encountered exclusively in the critical care unit ( such as brain death ) must be included .

monilethrix is an autosomal dominant disorder of hair shaft with regular thinning of hair shaft with fracture of the hair shaft at constricted points , which is commonly caused due to human hair keratin hhb6 gene located on long arm of chromosome 12 . patients have short and fragile hair not requiring haircut since childhood commonly over the occipital and temporal scalp , but may also involve eyebrows , eyelashes , axillary , pubic or body hair . holt - oram syndrome is a syndrome characterized by limb defects associated with cardiovascular anomalies . it is an autosomal dominant disorder caused due to mutations of tbx5 gene located on a long arm of chromosome 12 . the cardiovascular defects are usually in the form of septal defects commonly atrial septal defect . limb defects range from a hypoplastic thumb , metacarpals , carpals , radius to an absent limb . a 2-month - old female child , first born of second degree consanguineous marriage was brought with complaints of absence of hair over the scalp , eyebrows and body since birth . examination revealed absence of hair from the scalp , eyebrows and body with presence of hair over eyelashes [ figure 1 ] . other examination findings included a hypoplastic thumb on the right side with normal nails [ figure 2 ] . clinical photograph showing absence of hair over scalp and eyebrows clinical photograph showing hypoplastic thumb dermoscopic examination revealed presence of numerous empty follicles , small broken hairs , black dots and hair with uniform nodal dilatations with intermittent constrictions at which there was shaft breakage . dermoscopy of scalp showing hairs with monilethrix microscopic hair shaft examination could not be carried out as hair were too short for plucking . echocardiography showing atrial septal defect thus , a diagnosis of holt - oram syndrome with monilethrix was arrived at . it can be transmitted as an isolated condition , which has an autosomal dominant inheritance . it can also be associated with papular atrichia , keratosis pilaris , vitamin d resistant rickets , ectodermal dysplasia , moynahan 's syndrome , hypotrichosis simplex , marie - unna hereditary hypotrichosis , pseudo - thalidomide syndrome , amino acid metabolism and hair shaft disorders . the common hair shaft disorders , which may present as hypotrichosis include monilethrix , pili torti and pili annulati . monilethrix ( omim # 158000 ) is an uncommon hair shaft defect , which can manifest as congenital hypotrichosis . monilethtix is associated with trichorrhexis nodosa , nail and teeth defects , retarded growth and juvenile cataracts . the conditions where a hypoplastic thumb can be associated with atrial septal defect include holt - oram syndrome and vacterl association . holt - oram syndrome ( omim # 142900 ) is associated with limb defects such as hypoplastic thumb , metacarpals , carpals or radius defects and cardiovascular anomalies especially septal defects . vacterl association includes vertebral anomalies , anal atresia , cardiovascular anomalies , especially septal defects , trachea - esophageal fistula , renal defects and limb defects like hypoplastic thumb , syndactyly , polydactyly and forearm bony defects like radial aplasia . the presentation of monilethrix as congenital hypotrichosis is not very common . in this patient , the association of holt - oram syndrome with monilethrix , in spite of the causative genetic mutations being on the same chromosome , has not been reported to the best of our knowledge .

congenital hypotrichosis may be due to a number of causes and may have multiple systemic associations . a child born of second - degree consanguineous marriage was found to have monilethrix as the cause of congenital hypotrichosis . a detailed systemic evaluation in the child revealed atrial septal defect and a hypoplastic right thumb leading to a diagnosis of coexisting holt - oram syndrome .

2005 after a detailed polyphasic study and reclassification on the genus thermoactinomyces . at present the genus laceyella has four recognized species namely ; laceyella putida , l. sacchari , type species of the genus laceyella , laceyella sediminis and laceyella tengchongensis . cells are aerobic , non - acid - fast , produce endospores and chemo - organotrophic . l. sacchari strain gs 1 - 1 , was isolated from hot spring of chumathang , leh , ladakh , india , ( n 32 58 e78 15 ) , at the height of 4600 m above sea level . genomic dna was extracted from 36 h old culture using zr fungal / bacterial dna miniprep as per manufacturer 's instructions . the genome of l. sacchari strain gs 1 - 1 was sequenced using the illumina - hiseq 1000 paired - end technology that produced a total of 40,874,820 paired - end reads ( paired distance ( insert size ) ~ 330 bp ) of 101 bp . clc bio workbench v6.0.2 ( clc bio , denmark ) was employed for preprocessing the data so as to trim and remove low quality sequences . a total of 40,668,128 high quality , vector filtered reads ( ~ 1194 times coverage ) were used for assembly with clc bio workbench ( at word size of 45 and bubble size of 98 ) . the final assembly contains 42 contigs of total size 3,324,316 bp with n50 contig length of 249,341 bp ; the largest contig assembled measures 698,403 bp . this draft genome comprising 3,324,316 bp was annotated with the help of rast ( rapid annotation using subsystem technology ) system server . a total of 3429 predicted coding regions ( cdss ) , 6 rrnas and 69 trnas were predicted . rast indicates that strain geobacillus thermodenitrificans strain ng80 - 2 ( score 502 ) , geobacillus kaustophilus strain hta426 ( score 471 ) and geobacillus sp . strain g11mc16 ( score 436 ) are the closest neighbors of the strain gs 1 - 1 . the strain gs 1 - 1 contains the genes for glycolysis and gluconeogenesis , tca cycle and pentose phosphate pathway . genes of alkaline phosphatase ( ec 3.1.3.1 ) , ferroxidase ( ec 1.16.3.1 ) , manganese superoxide dismutase ( ec 1.15.1.1 ) , shikimate kinase i ( ec 2.7.1.7.1 ) , chorismate synthase ( ec 4.2.3.5 ) , alcohol dehydrogenase ( 1.1.1.1 ) and superoxide dismutase [ fe ] ( ec 1.15.1.1 ) and pathogenicity islands are also present in the genome annotation of strain gs 1 - 1 . we have mapped all predicted 3429 cdss to kegg pathways with the help of kass server . the genome has all the essential pathways for dna , rna metabolism , iron , sulfur and phosphorus acquisition and metabolism pathways ( fig . the l. sacchari strain gs 1 - 1 whole genome shot gun ( wgs ) project has been deposited at ddbj / embl / genbank under the project accession aszu00000000 of the project ( 01 ) that has the accession numbers aszu00000000 and consists of sequences aszu01000001aszu01000042 . the authors declare that there is no conflict of interest on any work published in this paper .

we report the 3.3-mb draft genome of laceyella sacchari strain gs 1 - 1 , isolated from hot spring water sample , chumathang , leh , india . draft genome of strain gs 1 - 1 consists of 3 , 324 , 316 bp with a g + c content of 48.8% and 3429 predicted protein coding genes and 75 rnas . geobacillus thermodenitrificans strain ng80 - 2 , geobacillus kaustophilus strain hta426 and geobacillus sp . strain g11mc16 are the closest neighbors of the strain gs 1 - 1 .

the author receives funding from the swedish research council , the ragnar sderbergs stiftelse , the novonordisk foundation and lund university . the author is also part of the following strategic research consortia in diabetes : the linnaeus lund university diabetes center ( ludc ) funded by the swedish research council and the excellence of diabetes research in sweden ( exodiab ) supported by the swedish government . the author was responsible for the conception , design and drafting of the manuscript and approved the final version for publication . this article is distributed under the terms of the creative commons attribution noncommercial license which permits any noncommercial use , distribution , and reproduction in any medium , provided the original author(s ) and the source are credited .

common genetic variations in the gene encoding transcription factor 7-like 2 ( tcf7l2 ) reveal the strongest association with type 2-diabetes known to date . these lead to impaired insulin production and output , but the mechanisms of disease remain incompletely known . in this issue of diabetologia , two publications provide new insights into tcf7l2-dependent diabetes .