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uterine artery embolization ( uae ) is a safe and effective procedure for the control of postpartum hemorrhage ( 1 ) . detailed knowledge of uterine vascularization and ovarian artery supply to the uterus is necessary for the management of postpartum hemorrhage . the ovarian arteries usually arise from the anterior circumference of the abdominal aorta at the level of the renal arteries ( 2 ) . anatomic variations of the ovarian arteries are relatively common ( 2 ) , and awareness regarding these variants is essential for successful uae . few studies have reported the abnormal origin or course of ovarian arteries ( 2 , 3 ) . here , we present a very rare case of an aberrant ovarian artery arising from the common iliac artery . a 46-year - old pregnant woman at 31 weeks gestation was referred to the gynecology department of our hospital for premature membrane rupture with uterine atony and placenta accreta . after an emergency caesarean section , the patient presented severe postpartum hemorrhage secondary to uterine atony and was referred to our department ( interventional radiology ) . embolization was performed using the single femoral artery approach with insertion of a 5-fr introducer sheath ( terumo , tokyo , japan ) . at the beginning of the procedure , flush aortography ( fig . 1a ) with a 5-fr pigtail catheter ( cook , bloomington , in ) positioned at the level of the celiac trunk showed a single aberrant artery arising from the right common iliac artery . selective angiography of the aberrant artery using a 5-fr yashiro catheter ( terumo , tokyo , japan ) revealed that it had a sinuous course and was tortuous ( fig . this artery showed the typical ovarian blush and abundant collateral perfusion to the markedly enlarged uterine fundus ( fig . both ovarian arteries could not be observed on pelvic aortography , and anastomosis with the uterine artery was not clearly defined . the diameter of the aberrant artery was larger than the diameters of both uterine arteries . selective angiography of both the internal iliac arteries and both uterine arteries showed markedly increased uterus vascularity . 1e , f ) were embolized with 1 1 mm gelfoam pledgets ( johnson & johnson , skipton , uk ) by using a 2.7-fr microcatheter ( progreat , terumo , tokyo , japan ) . three days after embolization , the patient underwent a contrast - enhanced abdominal - pelvic computed tomography ( ct ) scan , and no associated unusual findings such as anomalies of the kidney or renal arteries were noted . the patient 's condition rapidly stabilized after the embolization procedure , and she was discharged in a good condition . anatomic variations in the origin of ovarian arteries are relatively common ( 2 ) , and awareness regarding these variants is essential to ensure successful uae . few cases of an aberrant ovarian artery originating from the inferior mesenteric artery or renal artery have been reported ( 2 , 3 ) . in the present report we suggested that this aberrant artery was an ovarian artery because of its characteristic sinuous course and tortuous shape . this artery supplied the right adnexa and a large portion of the enlarged uterine fundus . we believe that the enlargement of the aberrant ovarian artery was a response to pregnancy . one of the reasons underlying embolization failure is non - embolized aberrant branches continuing to perfuse the uterus ( 4 - 6 ) . if vaginal bleeding persists after uae , aberrant arteries or collateral blood supply must be checked for and these arteries should also be embolized . in conclusion , we described a very rare case of an aberrant ovarian artery arising from the common iliac artery supplying a large portion of the uterus . currently , ovarian artery embolization is frequently performed , ever since the advent of uae for treating postpartum hemorrhage . therefore , precise knowledge of the anatomic variations of the ovarian artery is important for embolization to be successful .

a 46-year - old vietnamese woman received embolization therapy in order to control postpartum hemorrhage . angiography revealed an aberrant ovarian artery arising from the right common iliac artery . superselective catheterization and subsequent embolization of the aberrant ovarian artery and bilateral uterine arteries were performed . precise knowledge of the anatomic variations of the ovarian artery is important for successful embolization .

we present a case of postpartum female with hemolysis , elevated hepatic enzymes , low platelets ( hellp ) with deranged renal function , and cerebral venous thrombosis , without a history of hypertension and proteinuria . plasma exchange therapy was successfully used in reversal of organ dysfunction and resolution of hemolysis in this patient . a 20-year - old primigravida ( 36 weeks gestation ) woman was admitted in the semi - urban private hospital in north india with generalized tonic clonic seizure ( gtcs ) . magnesium sulfate iv 4 g and 0.5 g / hour infusion . an emergency lower segment cesarean section ( lscs ) was done and she delivered a 2.5 kg healthy male baby . after 6 hours of surgery , she became drowsy , blood pressure ( bp ) dropped to 70/38 mm hg and had decreased urine output ( 20 ml for the last 2 hours ) . she was started on dopamine infusion and transferred to a higher level tertiary care hospital , 36 hours after the lscs . in our emergency department , she got repeat gtcs , was given 4 mg lorazepam and loaded with inj . her bp was 100/74 mm of hg on vasopressors ( dopamine 12 g / kg / min and norepinephrine 15 g / min ) and she had anuria for 6 hours . her abdomen was distended , tense to palpation and there was per vaginal blood - stained discharge . the records of antenatal visits to urban health center were reviewed and there was no evidence of hypertension or proteinuria . her arterial blood gas ( abg ) showed severe metabolic acidosis , peripheral smear showed 3 + fragmented red blood cells ( rbcs ) , reticulocyte count 3.4% , direct and indirect coomb 's tests were negative besides other investigations [ table 1 ] . eclampsia with differential diagnosis of thrombotic thrombocytopenic purpura ( ttp ) versus hellp syndrome was made . laboratory tests results she was given four packed rbcs and platelets in view of active bleeding , 10 mg i.v . her abdominal distention further increased and her hemoglobin ( hb ) was 5.2 g / dl , platelet count ( pc ) was 53 109/l , peripheral smear showed 3 + fragmented rbc and lactate dehydrogenase ( ldh ) was 4580 u / l . in view of persistent hemolysis and worsening platelets and multiorgan dysfunction , therapeutic plasma exchange was started with 100% volume replacement with fresh frozen plasma ( ffp ) . on the next day , she was disoriented and not moving her limbs after stopping sedation . her non - contrast computerized tomography ( ncct ) scan head revealed bilateral parasaggital region and centrum semiovale ischemic infarct . she was continued on plasmapheresis with 80% volume replacement with ffp for five cycles over 5 days . on day 8 , her magnetic resonance imaging ( mri ) brain showed bilateral parasaggital , occipitoparietal , high frontal infarcts and magnetic resonance venogram ( mrv ) revealed cerebral venous thrombosis including inferior saggital , left transverse , sigmoid sinuses and cortical veins thrombosis [ figures 13 ] . in view of altered mental status and anticipated prolonged ventilation , percutaneous tracheostomy was done on day 9 . her renal functions improved and she was started on enoxaparin 60 mg subcutaneous ( s.c . ) brain ( t2 flair ) magnetic resonance venogram ( mrv ) neck and brain patient 's neurological condition gradually improved ; she started responding to verbal commands . she was then switched to warfarin on day 19 to target international normalized ratio ( inr ) 2.02.5 and enoxaparin was stopped . her tracheostomy was decannulated on day 28 and she was discharged on the next day . on follow - up after 28 days , her weakness had further improved and she was ambulated with support and warfarin was continued for 1 year to target inr 2.03.0.the patient and her family were counseled regarding the possibility of recurrence of symptoms during pregnancy . the hellp syndrome is usually associated with hypertension and proteinuria ; however , it can present without preeclampsia in 1020% patients . there are two main diagnostic criteria [ table 2 ] depending on the platelet counts , ldh and aspartate aminotransferase ( ast ) levels . the differential diagnosis of hellp syndrome includes idiopathic thrombocytopenic purpura ( itp ) , acute fatty liver of pregnancy ( aflp ) , and ttp . the management of these life - threatening microangiopathic disorders differs ; therefore , an accurate diagnosis is required . diagnostic criteria for hellp syndrome ttp shares pathophysiological characteristics of the hellp syndrome and differentiation between the two is sometimes difficult . we started dexamethasone as recommended by sibai et al . , but the patient 's condition continued to deteriorate with conservative measures . there are few case reports of use of plasmapheresis or plasma exchange in postpartum hellp syndrome . in our case , there was evidence of persistent hemolysis ( fragmented rbcs , ldh > 600 u / l and anemia ) , thrombocytopenia and deranged ast / alanine aminotransferase ( alt ) . our case thus fulfills the diagnostic criteria of hellp syndrome , but due to the absence of clear history of hypertension and proteinuria and non availability of adamts 13 test , we had difficulty deciding whether it was hellp or ttp . life - threatening neurological complications of the hellp syndrome are rare ; there are few case reports of cerebral infarction after delivery . this is the first case to our knowledge , where we found cerebral venous thrombosis with non - hemorrhagic cerebral venous infarct . in our case , we used low molecular weight heparin , enoxaparin after resolution of hemolysis and thrombocytopenia , and switched her on warfarin to target inr 2.03.0 for 12 months as recommended by american college of chest physician ( accp ) 2008 guidelines . the distinction between preeclampsia - eclampsia , hellp and ttp may not always be possible due to the various overlapping clinical and laboratory findings . the therapeutic plasma exchange therapy should be considered in persistent , life - threatening microangiopathy that is refractory to conservative measures .

the differential diagnosis of life - threatening microangiopathic disorders in a postpartum female includes severe preeclampsia eclampsia , hemolysis , elevated liver functions tests , low platelets syndrome and thrombotic thrombocytopenic purpura . there is considerable overlapping in the clinical and laboratory findings between these conditions , and hence an exact diagnosis may not be always possible . however , there is considerable maternal mortality and morbidity associated with these disorders . this case underlines the complexity of pregnancy - related microangiopathies regarding their differential diagnosis , multiple organ dysfunction and role of therapeutic plasma exchange in their management .

we present the case of a 60-year - old female with a history of polycythemia , chronic obstructive pulmonary disease ( copd ) on home oxygen , and obesity who presented to the emergency room complaining of mild dyspnea on exertion that had been experienced for 3 days . she denied any medical history , including autoimmune disease , connective tissue disorders , or vasculitis . as part of her general workup in the emergency department , she was found to have elevated troponin levels at 0.182 ng / ml ( 0.00.045 ng / ml ) . ecg revealed sinus tachycardia ; there were no st segment changes suggestive of ischemia ( fig . the patient was admitted to the floor and managed as a possible case of copd exacerbation . twelve - lead ekg at the time of emergency department presentation with sinus tachycardia and no acute st segment changes . a nuclear stress test was performed that revealed a large inferior wall reversible defect with associated inferior wall hypokinesis . coronary angiography of the right coronary showed a spontaneous dissection throughout the mid - right coronary artery ( fig . due to poor distal thrombolysis in myocardial infarction ( timi ) 1 flow , it was decided to stent the lesion . a prowater wire was steered toward the mid - right coronary artery which initially went into the false lumen ; we then pulled the wire back and redirected it to the true lumen which was confirmed by the ability to advance the wire into the distal vessel . follow - up angiography showed excellent results with complete tacking of the dissection plane and excellent timi 3 flow at the end of the procedure ( fig . 3 ) . coronary angiography showing a dissection plane throughout the mid - right coronary artery . follow - up angiography showing complete tacking of the dissection plane and timi 3 flow . while a few of the reported cases have been called idiopathic , a predisposed arterial disease or underlying cause has been identified in most of the cases ( 3 ) . intimal tear or bleeding of vasa vasorum with intramedial hemorrhage has been proposed ( 4 ) . younger females in the pregnancy and peri - partum periods are thought to be at a higher risk than the general population which is believed to be secondary to increased hemodynamics during labor and delivery , and possibly hormonal effect on the arterial walls affecting the tunica media ( 5 , 6 ) . it is unclear whether this inflammatory reaction is reactive or causative of dissection by weakening the coronary architecture ( 5 ) . connective tissue disorders such as ehler danlos type iv and marfans have also been implicated in scad ( 8 , 9 ) . our patient did not have any clinical features usually associated with such connective tissue diseases . she is an elderly female presenting with symptoms initially thought to be suggestive of a copd exacerbation with a troponin elevation secondary to a supply demand mismatch . this presentation serves as a reminder to include scad in the differential diagnosis even among elderly patients with comorbidities presenting with unexplained troponin elevation . other management strategies have been proposed in the literature ranging from percutaneous coronary interventions to heart transplantation . revascularization in scad is challenging and should be considered in patients with ongoing ischemia ( 10 ) . intravenous ultrasound ( ivus ) may be helpful in confirming the diagnosis . in our patient , ivus was not used due to her narrow lumen coronaries with a concern of possible worsening of the dissection with the bulky ivus catheter . none of the authors have any financial or personal bias that would inappropriately compromise the publication of this work . the authors have not received any funding or benefits from industry or elsewhere to conduct this study .

spontaneous coronary artery dissection is a poorly understood phenomenon that usually affects women during pregnancy or the immediate post - partum period . we present the case of a 60-year - old female with chronic obstructive pulmonary disease who presented with vague complaints of shortness of breath , dizziness , and weakness with a mildly elevated troponin . she denied any anginal symptoms . as part of her initial workup , a nuclear stress test revealed inferior wall reversible changes . coronary angiography revealed spontaneous right coronary artery dissection which was treated with a drug - eluting stent .

emphysematous cystitis ( ec ) is a rare infection of the urinary tract that results in gas production in the bladder . it is more common in diabetic and female patients , and can be associated with more serious complications , including pyelonephritis . we describe a case of recurrent bacterial cystitis caused by escherichia coli ( e. coli ) . an incidental finding in our patient of pneumaturia on computed tomography ( ct ) scan prompted further work - up . differential diagnoses for pneumaturia include infection , trauma , and fistula , most commonly colovesicular . the patient history ruled out trauma and ct scanning ruled out a fistula ; culture of the urine then showed a bacterial load greater than 100,000 e. coli / ml . the literature was scarce when it came to diagnoses of ec based on bacterial load . we present this case to increase health care providers ' awareness of recurrent ec with a urine culture bacterial load greater than 100,000 e. coli / ml . emphysematous cystitis ( ec ) is a rare urinary tract infection caused by gas - producing bacteria colonizing the urinary bladder . the initial treatment typically includes parenteral antibiotic administration and possibly percutaneous catheter drainage . in the most extreme cases , where the kidney is involved , nephrectomy can be performed to be curative . we present the case of a diabetic female patient diagnosed with ec and a bacterial load greater than 100,000 escherichia coli ( e. coli)/ml on four different occasions . a 67-year - old woman presented to the emergency department with right hip pain secondary to a fall . the patient 's vitals on presentation were as follows : blood pressure ( bp ) 152/93 mmhg , heart rate ( hr ) 88 bpm , respiratory rate ( rr ) 20 breaths / min , temp . the patient had a past medical history of chronic ec , diabetes mellitus ( dm ) , peripheral neuropathy , arthritis , coronary artery disease , and asthma . the patient underwent a right lower extremity computed tomography ( ct ) without contrast and was found to have a subcapital fracture with mild dorsal angulation . an incidental finding on the ct was pneumaturia , which was probably due to a case of ec [ figure 1 ] . because of the finding of pneumaturia , pelvic cts with and without contrast were ordered to rule out a colovesicular fistula , and one midstream catch sample and one catheterized sample of urine were taken and cultured . the ct scans ruled out any fistulas , and both urine samples were found to contain greater than 100,000 e. coli / ml . the patient was then treated with ceftriaxone 1,000 mg for 5 days , and was released in stable medical condition . on further examination of the patient 's other medical records , three prior hospital admissions with the e. coli cystitis were noted , each with approximately the same bacterial load . each can present with dysuria , abdominal pain , burning sensations during urination , and hematuria . while not specific to ec , pneumaturia will only occur in ec if present and can help the clinician decide between a diagnosis of ec and pyelonephritis or cystitis . ec is found predominantly in females over 60 years of age , with 60 - 70% of cases being among diabetic patients . the most sensitive measure to diagnose ec is the presence of gas pockets surrounding the urinary bladder on ct . a urinalysis is nonspecific and closely resembles that of pyelonephritis with the presence of red blood cells , white blood cells , and numerous bacteria . one theory postulates that gas formation occurs when bacteria such as e. coli ferment the glucose in the urine . in nondiabetic patients , local inflammation and impaired circulation have been postulated as mechanisms of pathogenesis . the presentation is similar to acute pyelonephritis , with symptoms including abdominal flank pain , leukocytosis , fever , chills , nausea , and vomiting . the treatment includes systemic antibiotics and open drainage or , in severe cases , emergent nephrectomy . our patient 's bacterial load was over 100,000 e. coli / ml . we present this case to increase health care providers ' awareness of recurrent ec in diabetic women .

context : emphysematous cystitis ( ec ) is a rare infection of the urinary tract that results in gas production in the bladder . it is more common in diabetic and female patients , and can be associated with more serious complications , including pyelonephritis.case report : we describe a case of recurrent bacterial cystitis caused by escherichia coli ( e. coli ) . an incidental finding in our patient of pneumaturia on computed tomography ( ct ) scan prompted further work - up . differential diagnoses for pneumaturia include infection , trauma , and fistula , most commonly colovesicular . the patient history ruled out trauma and ct scanning ruled out a fistula ; culture of the urine then showed a bacterial load greater than 100,000 e. coli / ml . the patient was then diagnosed with ec . she was treated with ceftriaxone and released in stable condition.conclusion:the literature was scarce when it came to diagnoses of ec based on bacterial load . we present this case to increase health care providers ' awareness of recurrent ec with a urine culture bacterial load greater than 100,000 e. coli / ml .

a 12-year - old male presented with the chief complaint of a 1-month - history of decreased visual acuity in his right eye . at the time of admission , the best corrected visual acuity was 0.5 in the right eye and 1.0 in the left eye . based on fundoscopy , the serous detachment had a well - defined margin of 1 disc diameter and was observed in the posterior pole ( fig . fluorescein angiography ( fag ) identified the point - like hyperfluorescent findings of the early stage ( fig . the fluorescent substance was found to slightly spread from the leakage point with time ( fig . there was serous neurosensory detachment on optical coherence tomography ( oct ; stratus oct , carl zeiss meditec , dublin , ca , usa ) ( fig . there were also no notable finding upon hematologic assessment performed in the department of endocrine pediatrics . the patient was diagnosed with idiopathic csc , whose clinical course was monitored without any specific treatments . however , recurrence was detected by oct five months after a monitoring regimen , and the corrected visual acuity was decreased to 0.6 . there was no improvement of the symptoms over the course of a two month period . , san francisco , ca , usa ) was then injected into the vitreous cavity , which gradually improved the visual acuity . a complete resolution of the subretinal fluid was shown eight months later , and the corrected visual acuity had improved to 1.0 . csc is a disease that can show serous retinal pigment epithelial detachment or neurosensory retinal detachment in the macular region due to the accumulation of serous fluid in the posterior pole . risk factors for developing csc include male sex , mental stress , type a personality , the use of steroids , and pregnancy [ 1 - 5 ] . the age of csc onset has been reported to be between 20 and 50 years of age . fine and owens reported a case of central serous retinopathy occurring in a 7-year - old girl in 1980 , which was not primary but secondary to focal chorioditis . there were no cases in which the typical symptoms of csc had manifested without other causes . in the current case , the patient was of healthy status and had no past history of any specific disease . the patient made no complaints regarding any specific systemic diseases when he was referred to the department of pediatrics . the patient also had no past history of steroid use or use of psychiatric medicinal products . furthermore , the patient had no underlying stress - related events and was not attributed a type a personality . according to fag and oct , acute csc has a relatively good prognosis in regard to visual acuity , and it has also been known to result in the spontaneous loss of retinal detachment and improvement of visual acuity without any specific treatment . it has recently been considered as a chronic disease that can produce decreased visual acuity . active treatments have been attempted , and the effectiveness of laser therapy and photodynamic therapy ( pdt ) have been demonstrated . , choroidal hyperpermeability has been assumed to be the cause of csc based upon indocyanine green angiography and has been considered a basis of pdt treatment . vascular endothelial growth factor ( vegf ) an intra - ocular injection of anti - vegf bevacizumab may improve the symptoms of csc by blocking the activity of vegf . the effectiveness of this treatment method has been described . to date , however , no studies have demonstrated the correlation between hyperpermeability of the choroidal vessel and vegf . also , in the current study , due to the lack of improvement of symptoms following the recurrence , bevacizumab was injected into the vitreous cavity . eight months following this procedure , a complete resolution of the subretinal fluid was shown . it remains unclear , however , whether the improvement of visual acuity and the resolution of the subretinal fluid originated from the treatment effect of the bevacizumab or as a result of spontaneous recovery .

a 12-year - old male presented with a chief complaint of a 1-month - history of decreased visual acuity in his right eye . the patient had no past history of steroid use or other systemic diseases . on fundus examination , which included fluorescent angiography ( fag ) and optical coherence tomography ( oct ) , the patient was diagnosed with idiopathic central chorioretinopathy , whose clinical course was monitored without any specific treatments . at the time of admission , the best - corrected visual acuity was 0.5 in the right eye and 1.0 in the left eye . on fundoscopy , a serous detachment of 1 disc diameter in size was observed in the posterior pole . according to the fag and the oct , serous neurosensory detachment was present . after two months of monitoring the clinical course , the best corrected visual acuity in the right eye was 0.8 , and there was improvement of neurosensory retinal detachment . however , oct detected recurrence after five months , and the corrected visual acuity was decreased to 0.6 . bevacizumab was then injected into the vitreous cavity . complete resolution of the subretinal fluid resulted eight months later , and the corrected visual acuity improved to 1.0 .

chronic myeloid leukemia ( cml ) is a clonal malignant disorder of a pluripotent hematopoietic stem cell characterized by the presence of the reciprocal translocation t(9;22)(q34;q11 ) , which generates the philadelphia ( ph ) chromosome . frequency of additional chromosomal abnormalities has an incidence of 7% in chronic phase and increases to 4070% in advanced disease / blast crisis . progression from chronic phase to accelerated phase or blast crisis is often associated with secondary chromosomal aberrations such as trisomy 8 , trisomy 19 , duplication of the ph chromosome , isochromosome 17q ( leading to the loss of p53 gene on 17p ) , acquisition of t(1;21 ) or translocations and inversions associated with aml / myelodysplasia , which translates a genomic instability of cml cells and the appearance of bcr - abl1 kinase mutations , both of which can confer resistance to tyrosine kinase inhibitors ( tkis ) . herein we report an exceptional case of cml diagnosed in lymphoblastic blast crisis which subsequently suffered a progression to myeloblastic blast crisis with rare cytogenetic abnormalities . a 65-year - old woman presented with profuse sweating and weakness for 3 months and a leukocytosis of 20010e9/l with more than 80% blasts in peripheral blood . a bone marrow aspirate was performed showing 50% lymphoid blasts with aberrant myeloid markers : cd34 + cd45+w dr+ cd38 + ctdt+ ccd79a+ cd19 + cd10 + cd20 cd24 + cigm sig cd7 + cd13 + cd33+/. genetic testing for philadelphia chromosome was done by fluorescence in situ hybridization ( fish ) and conventional cytogenetic analysis ( karyotyping ) . bcr - abl rearrangement was detected by fish in 96% of the bone marrow cell population and monosomy 7 in 71% of them ( fig . the patient received chemotherapy based on anthracycline , vincristine and steroids and imatinib at doses of 600 mg daily with intrathecal chemotherapy , achieving complete remission of acute leukemia and chronic phase regression . immediately after treatment , a new fish assay in peripheral blood was performed and showed bcr - abl rearrangement in 92% of cells , while monosomy 7 was not detected ( fig . afterwards , the patient received consolidation therapy with vincristine and daunomycin plus imatinib , which was tapered to 400 mg daily due to dyspnea and marked palpebral and ankle edema . overall , she showed a good clinical outcome with 3.3% bcr - abl transcripts three months after diagnosis . nevertheless , one week after this last determination , the patient returned to consultation due to headache and b symptoms . a new bone marrow had infiltration by 75% blasts of myeloid lineage consistent with myeloid blast crisis of cml . dr+ cd38 + cd13 + cd33 + cd11b cd64 cd56 cd7+/ cd9+d cd123 + showing the following karyotypic changes : 46,xx , t(1;6)(q22;q21),del(4)(p14),t(9;22)(q34;q11.2),der(11)add(11)(p14)add(11)(q23),add(17)(q12~21 ) ( fig . the patient also developed a t315i bcr - abl mutation detected by dna sequencing . in spite of treatment with steroids , she suffered a seizure and a parenchymal hematoma was observed in a cranial tc - scan , and subsequently died . in the current study we report on a patient with cml diagnosed with lymphoblastic blast crisis with monosomy 7 in 71% of bone marrow cells ; karyotype was 45,xx,-7,t(9;22)(q34;q11.2 ) at diagnosis . after treatment , monosomy 7 was not detected and a t(1;6 ) was observed upon regression into chronic phase . afterwards , a subsequent myeloid blast crisis was associated with the following karyotype : 46,xx , t(1;6)(q22;q21),del(4)(p14),t(9;22)(q34;q11.2),der(11)add(11)(p14)add(11)(q23),add(17)(q1221 ) . these findings would suggest the presence of a leukemic stem cell carrying the t(9;22 ) as the primary event in the onset of the disease . a second hit would be the acquisition of a monosomy 7 in a cell committed to lymphoid lineage , which might confer a proliferative advantage giving rise to the lymphoid blast crisis , when diagnose was made . at this time point , it was also observed the presence of a small fraction of leukemic cells , which would be committed to myeloid lineage , carrying a t(1;6 ) in addition to the t(9;22 ) , which latter on gave rise to the myeloid blast crisis . at this time , the emergence of several additional cytogenetic abnormalities was observed and t315i mutation was found , as an indicative fact of refractoriness to treatment . thus , treatment for acute lymphoblastic leukemia based on chemotherapy and imatinib successfully erradicated the clone involved in lymphoblastic blast crisis while allowing a clonal selection and subsequent expansion of myeloid cells carrying the t(1;6 ) . very few cases of lineage switch in cml have been reported in the literature before [ 69 ] and during the imatinib era . furthermore , to our knowledge , this is the first report describing sequential lymphoid and myeloid blast crisis with differentiated cytogenetic abnormalities . in summary , this is an interesting case depicting the underlying clonal heterogeneity of cml in evolution as well as the clonal selection under combined treatment with chemotherapy plus tkis .

frequency of additional chromosomal abnormalities in chronic myeloid leukemia ( cml ) is estimated to be 7% in chronic phase and increases to 4070% in advanced disease . progression of cml from chronic phase to accelerated phase or blast crisis is often associated with secondary chromosomal aberrations . we report an exceptional case of cml as debut in lymphoblastic blast crisis and a subsequent progression in myeloblastic blast crisis with rare cytogenetic abnormalities .

a 38-year - old woman , in her 24th week of pregnancy , had a sudden cardiac arrest . ventricular fibrillation ( vf ) was documented , and electrical defibrillation was performed by an emergency unit to restore the sinus rhythm . the electrocardiograph ( ecg ) was normal , as was the cardiac magnetic resonance imaging . the examinations that were carried out showed only a mitral valve prolapse with a mild mitral regurgitation . an implantable cardioverter defibrillation ( icd ) therapy , for secondary prevention of sudden death , was scheduled ; the procedure was guided by the non - fluoroscopy electro - anatomical mapping system ensite velocity ( st . a single deflectable quadripolar electrophysiology catheter was used for catheter manipulation and geometric reconstruction of the right atrium ( ra ) and of the right ventricle ( rv ) . after the creation of the icd pocket , a linox smart s dx lead ( biotronik se & co , berlin , germany ) was inserted through the left subclavian venous sheath . this is a single - coil screw - in lead capable of atrial bipolar sensing , thanks to two electrode rings mounted 150 mm from the tip . the ventricular pace - sense and the atrial sense dipoles were connected to the ensite velocity system junction box , in order to simultaneously display the lead tip and the atrial dipole in the electro - anatomical map ( see fig . 1 ) . the lead was then moved to the rv apex . after a satisfy measurement of the electrical parameters the fixing of the tip to the myocardial tissue was confirmed by the decrease and subsequent stabilization of the pacing ventricular impedance , after the tightening of the screw ( mean value 620 ) . the pacing threshold was 0.5 v at 0.4 ms , the mean ventricular and atrial sensing were 5.4 and 4.4 mv , respectively . the visualization of the atrial dipole , in the electroanatomical map at the level of the high right atrium , confirmed an appropriate bend of the lead shaft . the procedure lasted 2 hours ; a quick fluoroscopy , lasting only 4 seconds , was performed , in order to confirm that the screw had been extracted properly . the patient was discharged 2 days after the implant , and she completed the pregnancy uneventfully . the non - fluoroscopy technique may have an important impact during the implantation of cardiac resynchronization therapy ( crt ) devices , where a longer radiation exposure is expected . nevertheless , there are a few special cases where the x ray exposure has to be avoided , even if it is expected to be limited . abello et al . used transesophageal echocardiography , but this technique is not in wide use by most electrophysiologists . non - fluoroscopy guidance via electro - anatomical mapping was successfully used in some reported cases of icd implantation , , . the most challenging part of the procedure was assessing that lead shaft had bent correctly , even when a dual - coil lead was used . this is due to the fact that the mapping system can display the geometrical center of the proximal coil , but not its entire dimension . as previously speculated in a study , where single - lead pacemakers were implanted without the use of fluoroscopy , the use of an atrioventricular single lead ( for vdd mode of pacing ) makes the procedure easier . this is because the atrial pair of electrodes allows the system to display the proximal part of the lead , which implies being able to correctly assume its trajectory using the two dipoles , so the course of the lead is deductible from the two dipoles . we report a case of a single - lead icd with a floating atrial sensing dipole implant , without the use of fluoroscopy . this system , that has proven reliable for atrial sensing , is convenient for the proper positioning of the lead , thanks to the simultaneous display of both atrial and ventricular dipoles on the electro - anatomical map . this technique may be taken into consideration for the few rare cases where fluoroscopy can not be performed .

in this case report , we look into the implant procedure of a single - lead icd with floating atrial sensing dipole in a pregnant woman , without using fluoroscopy . this system benefits the proper positioning of the lead . this is possible thanks to the simultaneous display of both the atrial and ventricular dipoles on the electro - anatomical mapping system . this technique may be taken into consideration for the few rare cases where fluoroscopy is absolutely contraindicated .

massive hemoptysis is a life - threatening medical emergency with mortality rates ranging as high as 75% . transcatheter embolization of bronchial arteries has been regarded as an effective and safe method for the management of massive hemoptysis . chronic pulmonary thromboembolism ( pte ) is one of these causes , though its exact incidence remains unknown . compensatory hypertrophy of bronchial arteries is known to occur in chronic pte and pulmonary hypertension . however , the exact mechanism of hemoptysis in these patients is unclear . bleeding due to the rupture of smaller vessels or bleeding from hypertrophied bronchial arteries though bronchial artery embolization has been proposed to be effective in the management of hemoptysis , the ideal treatment for hemoptysis in patients with chronic pte remains uncertain . we report one case of hemoptysis in chronic pte with pulmonary arterial hypertension ( pah ) treated successfully with bronchial artery embolization . a 30-year - old male patient presented to the emergency department of our institute with recurrent bouts of hemoptysis for 1 week , along with shortness of breath . , he was found to have tachycardia ( pulse rate : 100 bpm ) and tachypnea ( respiratory rate : 30 breaths / min ) with decreased oxygen saturation ( 85% in room air ) . the patient underwent computed tomography ( ct ) and bronchial angiography that showed two bronchial arteries on the right side , which were hypertrophied and tortuous ( measuring ~2.6 mm and 2.4 mm , respectively , in maximum caliber ) [ figure 1a ] . in addition , there was a thrombus in the right pulmonary artery , extending from its origin till the subsegmental branches [ figure 1b ] . the right pulmonary artery was reduced in caliber with the dilated main and left pulmonary arteries . two - dimensional echocardiogram ( 2d echo ) was done for the patient that showed severe pah with dilated right atrium and ventricle . on bronchoscopy , active bleeding with clots was seen in the segmental bronchus of the right lower lobe . ( a , b ) coronal maximum intensity projection ( mip ) images of ct bronchial angiography showing hypertrophied right bronchial arteries ( arrows in a ) . one of the bronchial arteries was arising from the intercostobronchial trunk from the descending aorta ( not shown here ) . origin of the second right bronchial artery was , however , not clear on ct angiography . a thrombus was seen in the right pulmonary artery ( arrow in b ) extending till the subsegmental branches the patient was taken up for bronchial angiography . the second bronchial artery was being reconstituted by the internal mammary artery and was hypertrophied [ figure 3 ] . both the bronchial arteries were embolized with polyvinyl alcohol ( pva ) ( 500 - 700 m ) and gelfoam slurry . no other significant systemic collateral was identified on diagnostic runs of the bilateral subclavian arteries . digital subtraction angiogram of the right intercostobronchial trunk ( anteroposterior projection ) showing hypertrophied and tortuous bronchial artery . postembolization angiogram showed no opacification of the distal bronchial artery ( image on the right side ) digital subtraction angiogram of the right internal mammary artery showed hypertrophied second right bronchial artery arising from the internal mammary artery . chronic pte is associated with compensatory hypertrophy of the bronchial arteries and bronchopulmonary collaterals . increased incidence of bronchial artery hypertrophy on ct angiography has been reported in chronic pte and pah . though hemoptysis can occur due to hypertrophied bronchial arteries secondary to any cause , its exact incidence in chronic pte is unknown . one systemic review of the literature reported chronic pte as a cause of moderate - to - massive hemoptysis in 0.1% of cases . we report one case in which bronchial artery embolization was successful in managing hemoptysis in a patient with chronic pte with pah . one patient was successfully treated while the other patient continued to have active hemoptysis due to collaterals from the subclavian artery . reembolization was carried out for this patient but the patient died due to right ventricular failure . it was suggested that due to the requirement of anticoagulation for pte , even mild hemoptysis in these patients should be offered immediate intervention . however , another study reported the failure of bronchial artery embolization to prevent hemoptysis in a patient with chronic thromboembolic pah . in chronic pte , there is a propensity for the formation of thromboemboli . at the same time even mild hemoptysis in these patients should be given prompt treatment due to the underlying risk of progression to massive hemoptysis due to the ongoing anticoagulation therapy . however , very limited literature is available on the management of hemoptysis in chronic pte . no study has been carried out to evaluate the long - term effect of bronchial artery embolization in preventing hemoptysis . also , the precise mechanism of hemoptysis in chronic pte is still not clear . thus , further studies are required to establish the role of bronchial artery embolization in chronic pte .

bronchial artery embolization is the treatment of choice for the management of life - threatening massive hemoptysis . chronic pulmonary thromboembolism ( pte ) is one of the rare causes of hemoptysis . management of hemoptysis in chronic pte is a point of debate . in this article , we have reported one case of hemoptysis in chronic pte managed successfully with bronchial artery embolization .

erythema elevatum diutinum ( eed ) is a chronic and rare dermatosis that is considered to be a variant of leukocytoclastic vasculitis . clinically , it is characterized by symmetrical , persistent nodules , and plaques over the extensor aspects of both upper and lower extremities with a predilection for the joints particularly hands , feet , elbows , and knees . the condition was first described by hutchinson and bury in the 1880s , and the condition was later named in 1894 by radcliffe - crocker and williams . there have been several recent reports of the disease in association with human immunodeficiency virus ( hiv ) . the nodular lesions are rare variants and such lesions develop most often in hiv positive patients and they are confused with kaposi 's sarcoma and bacillary angiomatosis . early lesions of eed show leukocytoclastic vasculitis with fibrinoid necrosis of the vessel walls with abundant neutrophilic dust and late lesions show most frequent fibrosis and areas of granulation tissue with no proliferation of spindle cells . we report a case of nodular eed in a hiv infected woman , who has been on anti - retroviral therapy ( art ) for the past seven years . a 52-year - old married hiv infected woman , who had been on art for the last seven years ( zidovudine + lamivudine + nevirapine ) , was referred for evaluation of skin lesions that had appeared nine months ago . there was no history of any opportunistic infection before the onset of skin nodules . except arthralgia and mild constitutional symptoms , her general condition was fairly good and all her vitals were within normal limits . on examination , there were multiple , dusky red , large nodules and plaques over the feet , ankles , soles , shins , knees , and elbows . majority of the lesions were non tender and symmetrically distributed [ figures 1 and 2 ] . clinically , kaposi 's sarcoma or bacillary angiomatosis was suspected and the case was evaluated . multiple , dusky red , large nodules and plaques on the legs multiple , yellowish nodules on right elbow laboratory tests disclosed the following values : hemoglobin , 10.4 gm / dl ; total leukocyte count , 3600 cells / mm ; total red blood cell count , 2.70 million / mm ; erythrocyte sedimentation rate , 80 mm/1 hour ; reticulocyte count , 2.5% ; packed cell volume , 29.4% ; mean corpuscular volume , 108.9 fl ; mean corpuscular hemoglobin , 38.5 pg ; platelet count , 3 , 21,000/mm , differential count 59% neutrophils , 32% lymphocytes , 7% eosinophils , 2% monocytes ; serum glutamic oxalotransferase ( sgot ) , 48u / l ; serum glutamic phosphotransferase ( sgpt ) , 96 u / l ; serum alkaline phosphatase , 102 iu / l ; cd4 + count , 164/mm ; hiv rna pcr ( viral load ) , 666 iu / ml . mantoux test , serological tests for syphilis ( vdrl , tpha ) , hepatitis b and c , and anti - nuclear antibodies ( ana ) were negative . both specimens showed areas of ribbon - like thickening of collagen and areas with infiltrates of neutrophils and abundant neutrophilic nuclear dust . based on histopathology , a diagnosis of eed was arrived at and the patient 's blood was sent for estimation of serum iga level which was 11.3 after four weeks , colchicine 0.5 mg twice daily was given in place of dapsone . the response was slow and smaller lesions regressed , while larger lesions are in the process of regression . as the patient has low cd4 + count inspite of being on art since seven years , she was referred to the nearest art plus centre for second line therapy . the second line therapy ( atazanavir - based ) ribbon like thickening of collagen and several interstitially scattered neutrophils and few eosinophils ( h and e , 100 ) neutrophils and neutrophilic nuclear dust , few eosinophils surrounding affected blood vessels with surrounding ribbon like collagen ( h and e , 400 ) it is mediated by immune complexes ( arthus type reaction ) and has been associated with auto immune , neoplastic and infectious processes . in addition , eed has been associated with hypergammaglobulinemia , iga monoclonal gammopathies , as well as with myelodysplasia , pyoderma gangrenosum , and relapsing polychondritis . the nodular variant of eed is even rarer , but it evolves most often in hiv - positive individuals . the nodular lesions closely resemble kaposi 's sarcoma and bacillary angiomatosis and careful histopathological examination would differentiate these conditions from one another . a thorough literature search revealed an association of 20 - 25 cases of eed with hiv disease . to the best of our knowledge , our case is the first case of nodular eed simulating kaposi 's sarcoma associated with hiv infection in indian literature . it is now recognized as one of the defined reactive dermatosis associated with hiv . in majority of cases including ours , eed lesions were seen in patients with low cd4 + counts ( < 200 ) . but , in some cases , eed was reported as a first clinical manifestation of hiv infection . recently , a case of erosive and bullous eed in hiv - positive patient was also reported . a rare case of eed with polyclonal gamma - globulinemia with hiv-2 infection the association of eed with hiv infection is not clearly known , but it may be due to either hiv - related antigen - antibody complexes directly damaging the small blood vessels or the hiv - induced immunosuppressed state allowing other infectious agents to serve as an antigenic stimulus . in addition , eed has been associated with iga hypergammopathy , which is also a common finding in the hiv infection . since , in our case also iga levels were elevated , we presume that iga hypergammopathy may act as a trigger and promote chronic vasculitis with hiv background . although dapsone is the drug of choice , it is not very effective in older fibrotic nodular lesions in hiv - related eed . intralesional and topical steroids , colchicine , chloroquine , tetracycline , and niacinamide also may be tried . the course of the eed is chronic and benign and the lesions persist for a long time . may belong to a wide spectrum of tumours , ranging from the common appearance of kaposi 's sarcoma , bacillary angiomatosis , xanthomas , lymphomas to the very rare occurrence of benign nodular eed . our case raises the necessity of histopathological confirmation for all cases of suspected kaposi 's sarcoma and other tumours in patients with hiv background .

erythema elevatum diutinum ( eed ) has been emerging as a specific human immunodeficiency virus ( hiv ) associated dermatosis in recent times . it is an extremely rare chronic disease of unknown origin and part of the spectrum of leukocytoclastic vasculitis . we describe a case of eed simulating kaposi 's sarcoma in a 52-year - old hiv infected female patient with no previous opportunistic infections and cd4 + count of 164/mm3 . therapy with oral dapsone ( 100 mg / day ) for two weeks resulted in resolution of some lesions .

both anomalous origin of the right coronary artery ( rca ) from the left coronary cusp or congenital long qt syndrome ( lqts ) are an important cause of sudden cardiac death , especially in young and healthy individuals . however , sudden cardiac death in a patient with these two disease entities has not yet been reported . here , we report the case of a young female with repeated aborted cardiac death accompanied by anomalous origin of the rca and congenital lqts . a 15-year - old female with a prior history of aborted cardiac death and surgical correction of anomalous origin of the rca was referred to the cardiology department due to repeated aborted sudden cardiac death after physical exertion with boxing . 1 ) . biphasic 200 joules defibrillation restored the heart to sinus rhythm and cardiopulmonary function recovered without neurologic sequelae . two years prior to this event , she experienced chest discomfort followed by aborted sudden cardiac death after heavy exercise for the first time . she was successfully resuscitated after basic life support maneuvers and fully recovered with hypothermic treatment after being admitted to the emergency department . her family history of structural heart disease , syncope or sudden cardiac death was unremarkable . cardiac computed tomography revealed anomalous origin of the rca from the left coronary cusp coursing between the pulmonary artery and the aorta ( fig . her follow - up ecgs after stabilization showed a sinus rhythm with an inverted t wave and a prolonged qt interval which was suggestive of congenital lqts ( fig . she was not taking any medications which can prolong the qt interval and laboratory findings did not show electrolyte abnormalities . but this finding was overlooked and anomalous origin of the rca was provisionally considered as the cause of the aborted cardiac arrest . she underwent surgery to re - implant the anomalous rca from the left to the right sinus of valsalva . after receiving surgical correction of anomalous origin of the rca , she had been doing well before the second event of aborted cardiac death developed . coronary angiogram with a provocation test using ergonovine was done after the second event , and it revealed no significant abnormalities and the re - implantation site of the rca ostium was intact . an electrophysiologic study was performed to rule out possible causes of other arrhythmias leading to cardiac arrest . however no other arrhythmia was induced and also polylmorphic ventricular tachycardia was not inducible with programmed stimulation . the ecg after restoring the heart to sinus rhythm showed still significant prolongation of the qt interval , resulting in the diagnosis of lqts , probably type i , regarding her clinical presentation , even though the ecg pattern showed a prolonged qt interval and a notched t wave suggesting lqts type 2 . medication with a -blocker ( atenolol ) was started and the dose was titrated up to 1.3 mg / kg ( 50 mg bid , regarding her body weight ; 78 kg ) . a follow - up exercise tolerance test revealed blunting of the heart rate response during maximal exertion and she was instructed to avoid heavy exercise . she remained free from ventricular arrhythmias while she was on a -blocker medication during the follow - up of more than 6 months . her serial follow - up ecgs showed significant qtc prolongation of > 500 ms consistently . we report the case of a young female with repeated cardiac arrest accompanied by anomalous origin of the rca and congenital lqts . surgical correction of the coronary anomaly was performed and the diagnosis of lqts was made after the second episode of aborted cardiac death . congenital lqts is a genetic disorder characterized by a prolonged qt interval and polymorphic ventricular tachycardia . prolongation of the repolarization phase acts as a primary step for the generation of early after depolarizations ( eads ) and prolonged repolarization is associated with increased spatial dispersion of repolarization in the lqts . the focal ead - induced triggered beats can infringe on the underlying substrate of inhomogeneous repolarization to initiate polymorphic reentrant ventricular arrhythmia.1 ) the features of qt prolongation on ecg can be difficult to recognize or sometimes overlooked . although genetic testing may provide additional information , the rationale is often debatable and the diagnosis of lqts remains a challenge . the schwartz scoring system is widely used in clinical practice and the high probability of a clinical diagnosis gives the yield of positive genetic testing in 72%.2 ) we could not confirm the genotype in this patient ; however , the scoring system was suggestive of a high probability of lqts . coronary artery anomalies of ectopic rca originating from the left sinus were found in approximately 0.92% in a recent prospective study.3 ) anomalous origin of the rca from the left coronary cusp which courses between the pulmonary artery and aorta can cause a malignant arrhythmia due to the potential for rca ischemia , particularly during exertion or stress . physical exertion can precipitate a ventricular arrhythmia in both conditions , even though the mechanisms are different . also , it is possible that both conditions can interact with each other to increase the susceptibility to develop a ventricular arrhythmia . recent studies have shown that only a long qt interval is not sufficient to provoke polymorphic ventricular tachycardia.4 ) heterogeneity of repolarization throughout the transmural regions of the ventricle , which results in transmural dispersion of repolarization , is a marker of electrical instability . this enhanced transmural dispersion of repolarization allows for the propagation of multiple waves of reentry , which is responsible for polymorphic ventricular tachycardia by serving as a functional underlying reentrant substrate.5 ) as previously reported , the qt interval can be prolonged during the early post - infarction period.6 ) furthermore , the qt interval was significantly more prolonged in patients with extensive transmural infarction.7 ) thus , significant myocardial ischemia due to extrinsic compression or spasm of the rca might exacerbate transmural dispersion of repolarization and play a role in triggering ventricular arrhythmia during the first episode of aborted sudden cardiac death in our patient with qt prolongation . however , lqts might be solely responsible for the second episode of polymorphic ventricular tachycardia because she received definite treatment for the coronary anomaly . implantation of a defibrillator was considered because of repeated aborted cardiac death ; however , treatment for lqts had not been attempted before . -blocker is relatively effective in congenital lqts type i ; therefore , a defibrillator was not placed in the patient and sympathetic denervation was not performed in this patient , who was carefully monitored instead . in conclusion , both anomalous origin of the rca from the left coronary cusp and lqts can coexist and interact to induce a ventricular arrhythmia . we should not overlook qt prolongation even in a patient with a diagnosis of anomalous origin of the rca from the left coronary cusp .

a 15-year - old female with a prior history of aborted cardiac death and surgical correction of anomalous origin of the right coronary artery ( rca ) presented with polymorphic ventricular tachycardia . her electrocardiogram after defibrillation was suggestive of congenital long qt syndrome ( lqts ) . the patient was treated with a -blocker and remained free from ventricular arrhythmia during the follow - up of more than 6 months . here , we present the case of a young female with repeated aborted cardiac death accompanied by anomalous origin of the rca and congenital lqts for the first time .

vpa is a substrate for the mitochondrial beta - oxidation pathway due to its structural resemblance to simple fatty acid . methylmalonic aciduria ( mma ) is a rare inborn error of metabolism caused due to the impaired isomerization of l - methylmalonyl - coa to succinyl - coa . it usually presents in the infantile age with global developmental delay , seizures , movement disorder and failure to thrive . we present a case of intermediate phenotype of mma who got aggravated by valproate therapy . an 11-year - old boy , product of a nonconsanguineous marriage , with uneventful antenatal and perinatal period , presented with delayed motor milestones after the age of 6 months and clusters of generalized seizures precipitated with fever , since the age of 6 years . he was initially managed with thereafter , he started experiencing myoclonic jerks , involving both upper limbs with a frequency of about 1 per week , initially but increased subsequently to several per day . at the age of 8 years , he developed abnormal behavior in the form of recurrent clapping , hyperactivity , echolalia and fidgety nature with no visual or hearing impairment . his electroencephalogram ( eeg ) and magnetic resonance imaging ( mri ) brain studies were normal . , he was receiving oral valproate ( 600 mg / day ) for the control of seizures ( myoclonus and generalized tonic clonic ) later on , clobazam ( 5 mg / day ) and levetiracetam ( 500 mg / day ) were also added . over 4 months prior to admission , he developed progressive weakness in both legs , distal more than proximal , without any sensory or sphincter involvement . after another 2 months he became progressively dull , less communicative , lethargic , irritable , had nausea , occasional vomiting and refused oral intake . neurological examination revealed severely affected comprehension and presence of spasticity in both lower limbs with brisk tendon jerks and extensor planters . his serum ammonia ( 56.9 g / dl , normal , 3086 g / dl ) and serum valproate levels ( 76.7 mcg / ml , normal , 40100 mcg / ml ) were in normal range but serum lactate levels were raised ( 27.5 mg / dl , normal , 4.819.8 mg / dl ) . his urine analysis revealed markedly elevated levels of methyl malonic acid ( 7190.30 mmol / mol of creatinine , normal , < 209 mmol / mol of creatinine ) . eeg showed multifocal spike and wave discharges with generalized background delta slowing [ figure 1a ] . ( a ) multifocal spikes in t3 , t6 and o2 ; ( b ) after 3 months of treatment , disappearance of multi focal spikes ( a - d ) axial t2-weighted image and coronal fluid attenuated inversion recovery images show periventricular white matter hyper intensities with accompanying cortical atrophy with the diagnosis of methyl malonic acidemia , sodium valproate was changed to oral lacosamide ( 100 mg / day ) . he was also started on oral metronidazole ( 600 mg / day ) for 4 weeks , l - carnitine ( 1000 mg / day ) , injection hydroxocobalamin 1000 mcg / day and low protein diet ( 10 g / day ) . after about a month of treatment , he was able to speak sentences of four to five words , tremors lessened , lower limb spasticity also reduced . urine mma levels were in normal range ( 54 mmol / mol of creatinine ) . at 6 month of follow - up , he had no tremors , markedly less spasticity in lower limbs , could walk with little assistance , well communicative , seizure free and with no multifocal spikes in eeg [ figure 1b ] . , a wide range of adverse effects have also been reported like gastrointestinal disturbances , tremor , weight gain , teratogenicity , hepatotoxicity , fatal hemorrhagic pancreatitis , bone marrow suppression and hyperammonemic encephalopathy . valproate impairs mitochondrial function by the induction of carnitine deficiency , depression of beta - oxidation of fatty acids , and inhibition of oxidative phosphorylation . vpa usage has been reported to exacerbate the genetic impairment of the underlying metabolic error in patients with epilepsy . three variants of mma have been described ; infantile , intermediate , and atypical or benign variant . infantile , vitamin b12 nonresponsive type , is the most common . in this form , infants are normal at birth , and in the neonatal period develop lethargy , vomiting , dehydration , hepatomegaly , hypotonia , and encephalopathy . intermediate , b12-responsive phenotype , usually presents in the first months or years of life . affected children exhibit anorexia , failure to thrive , hypotonia , and developmental delay , and sometimes have protein aversion or vomiting and lethargy after protein intake . six biochemical and genetic forms of methylmalonic acidemia have been defined : two - partial ( mut ) or complete ( mut 0 ) loss of enzymatic function in the mutase apoenzyme , and four ( cbl a , cbl b , cbl c , and cbl d ) resulting from deficient adenosylcobalamin synthesis . our patient , an intermediate , b12 responsive , mma phenotype , became symptomatic by 6 months of age , had slowly progressive cognitive decline , delayed motor milestones , intractable epilepsy , spastic paraparesis and postural tremors in hands . although , the genetic test could not be performed , considering his vitamin b12 responsiveness , he could be one of the adenosylcobalamin deficient genotype . his worsening after institution of valproate could be due to the exacerbation of the mitochondrial dysfunction . further the existence of an underlying intermediate form of mma had delayed the development of clinical severity . our patient , on mri showed generalized brain atrophy with bilateral , symmetrical , fronto - parietal periventricular white matter hyperintensities on fluid attenuated inversion recovery and t2-weighted images as reported with mma . our patient had multifocal discharges in eeg , which disappeared after withdrawal of valproate and institution of b12 therapy pointing to the underlying cortical irritability as their causative factor . vitamin b12 and carnitine supplementation ; antibiotics such as neomycin or metronidazole to reduce propionate production from gut flora plays an important part in the management . thus , our case is unique to have valproate induced exacerbation of the b12 responsive intermediate mma . hence , one should suspect and evaluate for underlying metabolic disorder if , a patient clinically deteriorates while on valproate therapy .

a , 11-year - old male , with delayed milestones after the age of 6 months with recurrent myoclonus and generalized tonic - clonic seizures had clinical worsening after institution of valproate , was detected to have elevated serum lactate level and marked methyl malonic aciduria . patient had remarkable improvement following withdrawal of valproate and substitution of hydroxocobalamin .

firstly , my congratulations to those at the irish critical care trials group on their paper detailing the epidemiology and ventilatory management of ali / ards across ireland ; the first publication from this recently established clinical research group . the authors also report hypothesis - generating data on the implications of statin use in this population . the present commentary reviews aspects of this work , with particular attention to the implementation of low - tidal - volume / lung - protective ventilatory strategies in ali / ards . the advantages of multicenter collaborative research are clear to all , but does the influence of such groups extend beyond the individual studies undertaken ? although individual organizations often have differing infrastructures and approaches to study development , planning and funding , they invariably share a cohesive spirit and a mission to improve the care and outcomes of those with critical illness . as a result , they surely provide fertile environments not only for ongoing education and training in critical care medicine and research methodology , but also a forum for the discussion and advancement of clinical critical care practice . the data regarding statin use are tantalizing , and add to the growing debate and interest regarding these agents in the critically ill patient . as the authors rightly conclude , the small numbers involved and the modest data collection in this field , require that these data be considered hypothesis generating albeit in conjunction with existing data supportive of further study and potentially , a therapeutic trial . the incidence of ali / ards in the study by the irish critical care trials group , 27% of those patients receiving mechanical ventilation ( 19% of all intensive care unit admissions ) , is comparable with the literature from recent large surveys from both europe and north america . the mortality figures , albeit only the intensive care unit mortality is quoted , seem favourable in comparison with historical data in europe . such figures alone , however , require the provision of significant healthcare resources in terms of hospital and critical care services , and the figures mask , or at best make no reference to , the longer - term morbidity attributable to this condition , with its significant direct and indirect healthcare , social and personal costs . acknowledging this burden of disease , the authors rightly identify the importance of accurate , up - to - date epidemiological data to inform the design and feasibility of future clinical trials . amongst the physiological data presented , the irish critical care trials group report no association of the tidal volume or plateau pressure with mortality . cognizant of some deficiencies in their data collection , they suggest that this is at least in part due to ' relatively good adherence to lung protective ventilation ' , reasoning that only 5% of patients received a tidal volume > 12 ml / kg predicted body weight ( pbw ) and only 12% of patients received a plateau pressure > 30 cmh2o . the mean tidal volume was ~8.4 ml / kg pbw , however , suggesting that a significant number of patients received higher tidal volumes than the accepted upper limit of a low - tidal - volume approach in the acute respiratory distress syndrome network arma study ( 8 ml / kg pbw ) . is this truly lung protective , or is this just as far as clinicians were prepared to go ? the publication of the acute respiratory distress syndrome network ( ardsnet ) arma study in 2000 , with its 22% relative reduction in mortality with low - tidal - volume ventilation in patients with ali / ards , was generally heralded as a landmark advance in the care of the critically ill patient . poor translation of research into clinical practice is not a problem specific to critical care , or indeed to lung protective ventilation , with potential causes and solutions well documented . furthermore , even where clinicians are willing , significant organizational and clinical barriers exist in implementing what is a complex , multidisciplinary process at the bedside . translation of evidence to clinical practice may be improved by the adoption of protocols to drive clinical care , although , this may not be as simple as it might first appear . but what of the subsequent concerns voiced about the study design and the aspects of patient safety ? the arma study was criticized for comparing two extremes of ventilatory practice ( 6 ml / kg pbw versus 12 ml / kg pbw ) rather than employing more common and potentially safer practice as a control ( 8 to 10 ml / kg ) . critics argued that as a result participants were subjected to higher risks , and that the investigators were led to erroneously conclude that ventilation with the lowest tidal volumes is superior . in response , the ardsnet investigators challenged the methodology of their critic 's meta - analysis and reiterated their belief that , at the time the study was instigated , no such standard of ventilatory care existed . in addition , they subsequently reported further data from the arma study , detailing benefits of tidal volume and plateau pressure reduction across the range of disease severity and baseline plateau pressures , arguing against the u - shaped relationship for tidal volume and mortality alluded to by their critics . as the reader is undoubtedly aware , the subsequent repercussions from these events have been significant , and robust debate continues . it seems highly probable that this perceived uncertainty has impacted on the degree to which low - tidal - volume ventilation has been pursued by practicing clinicians . clinical education and process management undoubtedly have a role to play , but at what target are clinicians aiming ? on reading the current article by the irish critical care trials group , some will suggest that , although there has been improvement , there is some way to go before we achieve the evidence - based standard of ventilatory practice in ali / ards . others , meanwhile , will argue that based on existing evidence we may be close to arriving at that standard , and to go lower requires further investigation . under such circumstances , it would not be surprising if we see relatively little change in the delivered tidal volume in the near future . ali = acute lung injury ; ards = acute respiratory distress syndrome ; pbw = predicted body weight .

acute lung injury ( ali ) and the acute respiratory distress syndrome ( ards ) remain important causes of morbidity and mortality in the critically ill patient , with far - reaching short - term and long - term implications for individual patients and for healthcare providers . it is well accepted that mechanical ventilation can worsen lung injury , potentially worsening systemic organ function , and can thus impact on mortality in acute lung injury ( ali)/ards . unfortunately , although the concept of minimizing such damage via lung - protective ventilatory strategies is widely acknowledged , effective integration of such an approach into clinical practice remains more elusive . the study by the irish critical care trials group published in the previous edition of critical care describes a 10-week real - life survey of all intensive care unit admissions across ireland , detailing for the first time the epidemiology of ali / ards in this population and clinician 's attempts to deliver lung - protective ventilation . the authors also report hypothesis - generating data on the implications of statin use in this population . the present commentary reviews aspects of this work , with particular attention to the implementation of low - tidal - volume / lung - protective ventilatory strategies in ali / ards .

a 29-year - old black woman presented with a complaint of a photo - distributed dermatitis that had erupted each spring or summer for many years . an initial physical examination revealed several dark brown patches bilaterally on her forearms with interspersed 1- to 2-mm monomorphic lichenoid papules . a punch biopsy specimen of her left forearm revealed a lichenoid infiltrate , compact orthokeratosis , and absence of hypergranulosis ( figures 1 and 2 ) . three years later , she presented with scattered hyperpigmented macules with oval pink / violaceous plaques on both forearms ( figure 3 ) , as well as a 3-cm plaque on her lower neck posteriorly . figure 1low power magnification of lesion biopsied on initial presentation figure 3clinical presentation on follow - up visit . clinical presentation on follow - up visit . the histopathologic examination of the punch biopsy specimen revealed a lichenoid lymphohistiocytic infiltrate and abundant melanophages , expanding the papillary dermis , mimicking the classic ball - in - claw histology of lichen nitidus . the epidermis displayed slight hyperplasia , compact orthokeratosis with abundant civatte bodies , and a few foci of parakeratosis . the patient was educated regarding sun protection and prescribed a class i topical corticosteroid to use intermittently for flare - ups , which improved this photosensitive eruption . the first description of aln was reported in india in 1978 by bedi , who described 25 patients with hypopigmented pinpoint papules in sun - exposed areas and termed this photosensitive eruption summertime actinic lichenoid eruption ( sale ) . in 1981 , isaacson et al . reported a similar case of an african - american female with pinpoint papules and annular plaques . the report by isaacson et al . expanded sale to include other types of lichenoid eruptions such as hyperpigmented plaques , pinpoint papules , and lichen planus ( lp)-like papules , considering them part of a spectrum of actinic lichen planus ( alp ) , but preferring the term sale . in a later report of nine patients with aln , hussein argued against using the broader term sale . he reported the co - occurrance of alp and aln in two of the nine patients , which would appear to be the case in our patient with a diagnosis of aln and who later presented with oval violaceous plaques on her neck and forearms , a clinical picture more consistent with alp . aln and alp present mostly in children and young adults with darkly pigmented skin , following significant sun exposure . aln , a rare photo - induced eruption , shares the same histological features as lichen nitidus including parakeratosis , absent granular layer , basal cell liquefaction , and a ball - in - claw arrangement of lymphocytic infiltrate . in addition , aln usually presents with pinpoint lichenoid papules , restricted to sun - exposed areas . in contrast , typical alp lesions are oval or annular plaques with hypopigmented , palpable borders , and hyperpigmented centers that appear depressed or atrophic . the histopathology of alp includes hyperkeratosis , wedge - shaped hypergranulosis , and necrotic keratinocytes with a band - like lymphocytic infiltrate , similar to classic lp . . the term aln should describe miliary papules with lichen nitidus - like histopathology , and alp should refer to annular lesions with lp - like histological features . patients with both types of lesions can be considered to have related diagnoses or diagnoses that lie on a continuum , which is comparable to the co - occurrence of classic lp and lichen nitidus . the histopathologic examination of the punch biopsy specimen revealed a lichenoid lymphohistiocytic infiltrate and abundant melanophages , expanding the papillary dermis , mimicking the classic ball - in - claw histology of lichen nitidus . the epidermis displayed slight hyperplasia , compact orthokeratosis with abundant civatte bodies , and a few foci of parakeratosis . the patient was educated regarding sun protection and prescribed a class i topical corticosteroid to use intermittently for flare - ups , which improved this photosensitive eruption . the first description of aln was reported in india in 1978 by bedi , who described 25 patients with hypopigmented pinpoint papules in sun - exposed areas and termed this photosensitive eruption summertime actinic lichenoid eruption ( sale ) . in 1981 , isaacson et al . reported a similar case of an african - american female with pinpoint papules and annular plaques . the report by isaacson et al . expanded sale to include other types of lichenoid eruptions such as hyperpigmented plaques , pinpoint papules , and lichen planus ( lp)-like papules , considering them part of a spectrum of actinic lichen planus ( alp ) , but preferring the term sale . in a later report of nine patients with aln , hussein argued against using the broader term sale . he reported the co - occurrance of alp and aln in two of the nine patients , which would appear to be the case in our patient with a diagnosis of aln and who later presented with oval violaceous plaques on her neck and forearms , a clinical picture more consistent with alp . aln and alp present mostly in children and young adults with darkly pigmented skin , following significant sun exposure . aln , a rare photo - induced eruption , shares the same histological features as lichen nitidus including parakeratosis , absent granular layer , basal cell liquefaction , and a ball - in - claw arrangement of lymphocytic infiltrate . in addition , aln usually presents with pinpoint lichenoid papules , restricted to sun - exposed areas . in contrast , typical alp lesions are oval or annular plaques with hypopigmented , palpable borders , and hyperpigmented centers that appear depressed or atrophic . the histopathology of alp includes hyperkeratosis , wedge - shaped hypergranulosis , and necrotic keratinocytes with a band - like lymphocytic infiltrate , similar to classic lp . the term aln should describe miliary papules with lichen nitidus - like histopathology , and alp should refer to annular lesions with lp - like histological features . patients with both types of lesions can be considered to have related diagnoses or diagnoses that lie on a continuum , which is comparable to the co - occurrence of classic lp and lichen nitidus .

we present the case of a 29-year - old black female with an initial clinical and histopathologic diagnosis of actinic lichen nitidus . three years later , she presented with scattered hyperpigmented macules with oval pink / violaceous plaques bilaterally on her forearms and on her neck , clinically consistent with actinic lichen planus . she was treated with topical steroids at each visit , with subsequent resolution of her lesions . in this report , we discuss the spectrum of actinic lichenoid dermatoses and of disease that presents even in the same patient .

chondroblastoma is a rare , benign bone tumor that represents 12% of all primary bone tumors and approximately 5% of benign bone tumors 1 , 2 . it most commonly occurs in the femur , humerus , and tibia 3 , 4 , 5 but has also been described in flat bones such as the scapula , patella , sternum , and the bones of the skull . while the tumor is characterized by the presence of compact areas of chondroblasts , microscopic examination also readily demonstrates an abundance of osteoclasttype giant cells . these giant cells have been examined in depth in relation to giant cell tumor of bone . there is evidence that these cells are responsible for the osteolytic nature of these tumors due to their osteoclastic nature 6 , 7 . denosumab , a monoclonal antibody that targets the receptor activator of nfb ligand ( rankl ) responsible for the recruitment and activation of giant cells , has been demonstrated to inhibit the growth of giant cell tumors 8 . yuang et al . 8 found that rankl was also expressed in chondroblastoma and theorized that this was responsible for the aggressive nature of the tumor . to our knowledge , denosumab has not been used for the treatment or prevention of recurrence of chondroblastoma . we report a case of recurrent chondroblastoma of the squamous temporal bone that is currently suppressed with denosumab . a 64yearold gentleman presented to our service with a chondroblastoma of the squamous temporal bone . , he presented to an ear , nose , and throat surgeon with a painful swelling around his right temporomandibular joint , which had been present for 3 weeks with only pain prior to this for 2 months . a computerized tomography scan of the area demonstrated a lobulated , solid mass measuring 33 25 24 mm within the squamous portion of the temporal bone . surrounding edema was noted in the overlying temporalis muscle , extending into the surrounding lateral pterygoid and adjacent temporomandibular joint on magnetic resonance imaging ( fig . 1 ) . postgadolinium magnetic resonance imaging demonstrating an expansile mass on the inferolateral surface of the right squamous temporal bone . biopsy of the lesion demonstrated a giant cellrich neoplasm , which was supported by the radiographic findings . the gentleman subsequently underwent a resection of the tumor , with histopathology demonstrating there to be contaminated margins . ten months following resection , the patient developed nocturnal pain in the right side of the jaw . mri and ct demonstrated an expansile soft tissue mass in the greater sphenoid wing , representing the anteroinferior margin of the previous resection ( fig . 3 ) and demonstrating tumor present at the margins of the specimen . postgadolinium magnetic resonance imaging demonstrating a lobulated mass at the inferomedial border of the previous resection . 400 magnification . following a review of the literature and discussion with the patient 's treating medical oncologist , the decision was made to commence the patient on denosumab therapy to prevent recurrence of the tumor . the patient underwent treatment with denosumab from august 2012 to december 2013 . during this time , followup scans did not demonstrate any local recurrence of the tumor . in december 2013 repeat resection of the tumor was performed in february 2015 with histopathology demonstrating clear surgical margins . denosumab was recommenced in february 2015 , and as of january 2016 , repeat imaging has not demonstrated any recurrence of the malignancy . while undergoing denosumab therapy , the patient did not experience any known adverse effects of the medication . 9 demonstrated that the cartilagesignaling molecules indian hedgehog / parathyroid hormonerelated protein and fibroblast growth factor were both active in the disease suggesting that the tumor is of mesenchymal cell origin that is committed to chondrogenesis via active growth plate signaling pathways . the current mainstay of treatment for chondroblastoma is surgical resection with no widely accepted methods for the prevention of recurrence . radiotherapy has been described in case reports for the prevention of recurrence , but it is not currently accepted as the standard of care . hence , a treatment modality that can minimize recurrence risk is needed . in a 2003 paper , huang et al . 10 demonstrated that rankl was expressed in chondroblastoma tumor cells ; however , it was not expressed in chondrosarcoma . rankl is critical in the recruitment of osteoclastic giant cells and has been extensively studied in relation to giant cell tumors . interim results of phase 2 studies 11 , 12 have demonstrated that the human monoclonal antibody that targets rankl , denosumab , may be a safe and effective treatment that slows the progression of giant cell tumors of bone , which typically are very aggressively lytic . as the agent is not tumoricidal , prolonged treatment with denosumab to prevent bone lysis there have been no studies or trials that have looked at the responsiveness of chondroblastoma to denosumab . we believe that this is the first reported case in which it has been used for the prevention of recurrence of chondroblastoma . the patient described initially had recurrence of his tumor within 10 months after initial resection . during the 16 months that the patient was on denosumab , there was no identifiable tumor recurrence and it was not until the agent was ceased that there was return of the tumor . the decision to cease treatment with the agent was made by the treating physician due to lack on recurrence on followup scans and the patient due to significant outofpocket expense incurred with offlabel use of the medication . while a single case is insufficient to draw any definite conclusions , we believe that this patient 's disease free period following intralesional resection and in the context of a previous quickly recurrent tumor suggests that denosumab may be a potential therapy for chondroblastoma and may warrant further study . dw : contributed to the initial conception of the article , drafting of the article , and final approval of the article . nc : contributed to the data collection , interpretation , and drafting of the article .

key clinical messagechondroblastoma is a rare , benign bone tumor that represents 12% of all primary bone tumors . denosumab , a monoclonal antibody , has been demonstrated to inhibit the growth of giant cell tumors . we report a case of recurrent chondroblastoma of the squamous temporal bone that is currently suppressed with denosumab .

pancreatic solid pseudopapillary tumor ( spt ) is a rare neoplasm , representing ~2% of pancreatic tumors . the histological origin of the tumor is poorly understood , showing remarkable variability in their presentation , biological behavior and prognosis . our literature search has identified only 16 previously reported cases of spt at an ectopic site [ 25 ] . we report a case of 33-year - old male presenting with spt arising in the root of small bowel mesentery with lymph - node metastasis . the site as well as the presence of lymph - node metastasis makes our case report unusual . a 33-year male presented with a 1-year history of epigastric and right upper abdominal pain associated with episodes of upper abdominal fullness relieved after vomiting . initial laboratory values revealed a white blood cell count of 8400/mm ( 80% neutrophils , 20% lymphocytes ) , hemoglobin 12.5 g / dl , total bilirubin 2.4 mg / dl , conjugated bilirubin 1.8 mg / dl , alanine aminotransferase 33 u / l ( normal 545 u / l ) , aspartate aminotransferase 41 u / l ( normal 545 u / l ) and alkaline phosphatase 27 u / l ( normal 42128 u / l ) , albumin 3.9 mg / dl and total protein 6.7 mg / dl . abdominal ultrasound ( us ) examination revealed well - defined thick and irregular - walled cystic lesion of size 6 5 cm below the body of the pancreas . contrast - enhanced computed tomography ( ct ) scan showed a round cystic lesion of size 5.2 4 cm with irregular enhancing thick wall , below the body of the pancreas ( fig . us - guided fine needle aspiration cytology showed clusters of ductal epithelial cells and occasional acinar cells . with the diagnosis of a cystic neoplasm of the pancreas , patient underwent exploratory laparotomy which revealed a cyst of size 6 5 cm found to be arising from the root of small bowel mesentery , along with a lymph node of size 2 cm 1.5 cm adjacent to it and medial to the duodenojejunal flexure ( fig . the cut surface showed a cystic lesion containing hemorrhagic fluid with areas of solid components and necrosis . figure 1:photomicrograph of the arterial phase ct showing well - circumscribed low attenuated lesion ( arrow).figure 2:a photograph during laparotomy showing the origin of the lesion ( arrow ) at the root of mesentery medial to the duodenojenunal flexure . photomicrograph of the arterial phase ct showing well - circumscribed low attenuated lesion ( arrow ) . a photograph during laparotomy showing the origin of the lesion ( arrow ) at the root of mesentery medial to the duodenojenunal flexure . histopathological examination revealed tumor cells arranged in organized nests , few trabecular and pseudopapillae formation . the slide shows few polygonal and elongated cells with round to oval vesicular nucleus , inconspicuous nucleoli and moderate to abundant faint eosinophilic granular cytoplasm ( fig . cytoplasm was positive for vimentin , cd-10 and nse ( neuron - specific enolase ) ; granularity of cytoplasm was positive for alpha-1 antitrypsin and alpha-1 antichymotrypsin . figure 3:photomicrograph indicating the tumor composed of irregular pseudopapillary structures ; prominent myxoid changes can be seen in the stroma . photomicrograph indicating the tumor composed of irregular pseudopapillary structures ; prominent myxoid changes can be seen in the stroma . he received six cycles of gemcitabine ( 1 g / m ) . till the end of 10 months spts of the pancreas are rare exocrine pancreatic tumor , well known for indolent biologic behavior . frequently , it is named as solid and cystic tumor , solid and papillary epithelial neoplasm , papillary - cystic neoplasm , papillary - cystic epithelial neoplasm , papillary - cystic tumor or franz tumor . in 1996 , the world health organization renamed this tumor as spt for the international histological classification of tumors of the exocrine pancreas . it commonly occurs in the head or tail region of the pancreas , usually in females in second to fourth decade of life . the present case was a 33-year - old male who had presented with an epigastric mass . laparotomy revealed the lesion to be arising from the root of small bowel mesentery medial to the duodenojejunal flexure , with a nearby lymph node ; the lesion being separate from the normal pancreas . histopathological examination showed that the lesion was an spt of pancreatic origin and had also metastasized to the lymph node . spt occurring in an ectopic pancreas is a rare phenomenon , with only 16 previously reported cases , at various sites such as the mesocolon , ovary , retroperitoneum , liver , stomach and duodenum [ 25 ] . the root of small bowel mesentery is till date an unreported site for spt ; this being the first such case described . the presence of tumor in male and the lymph - node metastasis is also a rare occurrence . the clinical presentation of the tumor is usually nonspecific , like vague abdominal pain , gradually enlarging mass or compressive signs . some asymptomatic patients are detected incidentally during routine physical examination or by imaging studies . in some rare instances , acute manifestations such as pancreatitis or hemoperitoneum caused by rupture of the tumor ultrasonographically , the tumor is well encapsulated , homogeneous or heterogeneous , and composed of solid echogenic and hypoechogenic cystic components . ct scan usually demonstrates a well - encapsulated and circumscribed retroperitoneal mass , hypodense , with various solid and cystic components owing to hemorrhagic degeneration . magnetic resonance imaging is better than ct for distinguishing certain tissue characteristics , such as hemorrhage , cystic degeneration or the presence of a capsule . the pathologic diagnosis of spt on light microscopy depends upon the presence of solid areas alternating with pseudopapillary formations ; evidence of cellular degeneration , cholesterol clefts , nuclear grooves , aggregates of foamy histiocytes and hyaline cytoplasmic globules . immunohistochemical staining for specific cell lineage markers is of marginal utility in proving the diagnosis of spt . vimentin is consistently expressed , and tumor cells are focally positive for cytokeratin and synaptophysin , and few positive for s-100 protein , cd-56 and cd-10 [ 7 , 12 ] . the management of spt localized to the pancreas is straightforward and surgical removal with or without pancreatic resection is the treatment of choice . similarly , local invasion and metastasis should not be the contraindication for resection [ 7 , 12 ] . there have been reports of using various chemotherapy regimens based upon cisplatinum and 5-flururacil , gemcitabine alone or combination of ifosfamide , cisplatin and vp-16 . spt remains a distinct entity and has favorable prognosis , though histogenesis is still controversial .

abstractpancreatic solid pseudopapillary tumor is a rare neoplasm . very rarely , it may arise from an ectopic site . solid pseudopapillary tumor occurring in the root of mesentery has not been described in the literature . this report summarizes a case of an adult male having the tumor arising from the mesenteric root . he underwent complete resection of the tumor followed by six cycles of adjuvant chemotherapy and remains asymptomatic till date .

snp array revealed a 195 kb homozygous deletion encompassing the entire slc34a2 gene , not reported previously in pam . our findings underline that deletions of this gene should be analyzed in patients with a clinical diagnosis of pam . a 20yearold woman without a relevant medical history was referred to the hospital for analysis after her chest xray suggested the presence of interstitial lung disease . physical examination revealed no clubbing and no lymphadenopathy fine crackles were heard over the basal lung fields . she did not have a history of smoking and none of her relatives had a history of pulmonary disease . she had been vaccinated with bcg and had , to her knowledge , not been in contact with people with tuberculosis . pulmonary function testing showed a restrictive pattern [ tiffenaeu index 89% ] with a forced vital capacity ( fvc ) of 2.12 l ( 56% ) , a total lung capacity ( tlc ) of 3.59 l ( 70)% , and a diffusing capacity of carbon monoxide divided by the alveolar volume ( dlco / va ) of 1.91 mmol/(min * kpa*l ) ( 103% ) the lung . highresolution computed tomography ( hrct ) showed septal and subpleural linear calcifications leading to thickening of the interlobular septa with calcified intralobular nodules , most profound in the lower fields ( fig . a bronchoalveolar lavage showed intracellular calcification and a transbronchial peripheral lung biopsy revealed lung tissue with numerous calcified concrements in the alveolar space . her parents and siblings have no medical complaints and chest xrays of her parents were normal . ( a ) highresolution computed tomography ( hrct ) in lung window showing thickened interlobular septal lines with fine centrilobular calcifications leading to diffuse increased density of the lung parenchyma . ( b ) hrct in bone window highlighting the microlith deposition ; most pronounced in the middle lobe and mediobasal segment of the right lower lobe . many calcified concrements are seen in some areas surrounded by mild fibrotic reaction or giant cell response . total genomic dna was extracted from blood using standard protocols and sent to the acibadem laboratory in instanbul to test for mutations in the slc34a2 gene . the relative dna copy numbers at the cnv loci were determined by comparison of the normalized array signal intensity data for the proband 's dna sample against the hapmap270 reference file provided by affymetrix , using nexus software ( from biodiscovery , hawthorne , california , usa ) 1 . slc34a2 , the causative gene for pam , was located in a large region of homozygosity on chromosome 4 . the log2 ratio of the cnv probes covering the slc34a2 gene indicated a homozygous deletion with a maximal homozygously deleted region spanning ~195 kb and encompassed the entire slc34a2 gene . homozygous deletion at 4p15.2 ( double red bar ) encompassing the entire slc34a2 gene ; breakpoints deletion : arr[hg19 ] 4p15.2(25,553,88225,748,658)x0 . the log2 ratio of 1,9 ( yaxis of upper plot)is indicative for a homozygous deletion . the random noisy pattern of the snp probes in the lower plot confirm the deletion of both alleles . pam is a rare disorder in which numerous fragments ( microliths ) consisting of calcium phosphate gradually accumulate in the alveoli throughout the lungs 2 , 3 , 4 . pam is an autosomal recessive disorder caused by loss of function mutations in the gene encoding type iib sodiumphosphate cotransporter , scl34a2 . the gene has 13 exons , the first of which is noncoding . the protein is involved in phosphate homeostasis 3 , 5 . homozygous loss of function mutations result in reduced phosphate reuptake by type iib sodium phosphate transporter in the apical membrane of type ii alveolar cells , resulting in calcium phosphate chelation and microlith formation resulting in a typical radiological appearance . most patients are asymptomatic for years or even for decades after the diagnosis , which is often based on the incidental finding of a the potentially lethal disease often follows a longterm progressive course resulting in slowly deteriorating pulmonary functions . the age at clinical onset in an extensive study of 300 individuals was highly variable ( 541 years ) with a great discrepancy between radiological findings and clinical symptoms 2 . . currently , no clearly defined treatment options are available with exception of lung transplantation . in addition , clinical and radiological improvements were reported in two patients with pam who were treated with bisphosphonates 6 . the reported mutations in the scl34a2gene most commonly occur in one of the exons with a predicted protein truncating effect 7 . only five different homozygous deletions and one deletion plus insertion mutation have been described so far 5 , 7 , 8 . the previously reported deletions were three different single nucleotide deletions , one deletion of 186 nucleotides , and a 5.5 kb long deletion 5 , 8 . this finding confirmed the clinical diagnosis of pam in the proband and enabled genetic counseling and ( presymptomatic ) genetic testing in her relatives . our study confirms the occurrence of homozygous scl34a2 deletions associated with pam , adds to the mutation spectrum by presenting the first reported homozygous whole gene deletion and underlines the importance of including a snp array when considering this rare disease , especially in a consanguineous pedigree .

key clinical messagewith around 500 cases published worldwide , pulmonary alveolar microlithiasis is a rare disorder with an autosomal recessive pattern of inheritance . we show for the first time that homozygous deletions encompassing the entire scl34a2 can be associated with this rare genetic pulmonary disease .

sjgren - larsson syndrome ( sls ) is an autosomal recessive neurocutaneous disorder characterized by clinical triad of congenital ichthyosis , diplegia or tetraplegia , and learning disability / mental retardation . sls is caused by mutation in the gene for fatty aldehyde dehydrogenase ( faldh ) which catalyzes oxidation of long chain aliphatic alcohols to corresponding fatty acids . this leads to accumulation of aldehyde - modified lipids or fatty alcohol which probably disrupts the barrier function of skin and white matter of brain . we describe a series of six children with sls seen over a period of 1 year to highlight their varied clinical presentation . the study comprised six children , ranging from 1 to 12 years with an equal gender ratio diagnosed as sls seen during the period january 2012 to december 2012 , referred from pediatric neurology department . all the children had ichthyosis since birth and were associated with delayed milestones and speech abnormalities [ table 1 ] . there was generalized ichthyosis in all patients with relative sparing of face [ figure 1 ] . type of scales varied from fine [ figures 2 and 3 ] to lamellar type [ figures 4 and 5 ] . spastic diplegia was present in four cases and two children had spastic quadriplegia [ figure 6 ] . skin biopsy was done in all children ; features showed hyperkeratosis , parakeratosis , and normal to thinned out granular layer with mild perivascular lymphocytic infiltrate in dermis consistent with ichthyosis [ figure 7 ] . brain magnetic resonance imaging ( mri ) in all children revealed delayed myelination with signal changes in periventricular region predominantly involving occipital region [ figure 8 ] . clinical summary of patients case 1-fine scaling over extremities with sparing of face in a 1.5-year - old female child case 2-x - linked type of ichthyosis over extremities case 3-fine scaling over extremities similar to ichthyosis vulgaris case 4-three - year - old male child with lamellar type of scales over the extremities case 5-lamellar type of scaling over extremities case 6-generalized ichthyosis ( lamellar type scales ) with spastic quadriplegia epidermis shows hyperkeratosis , parakeratosis , thinned out granular layer with mild perivascular lymphocytic infiltrate in dermis ( h and e , 40 ) t2-weighted magnetic resonance imaging ( mri ) showing signal changes in bilateral peritrigonal regions in 1957 , sjgren and larsson described the disorder affecting 28 patients from a highly consanguineous population in a remote area of sweden with clinical triad of mental retardation , spastic diplegia , and congenital ichthyosis . the incidence of sls is 1 in 200,000 births in sweden , but it occurs more rarely worldwide . sls is caused due to recessive mutations in the fatty aldehyde dehydrogenase ( faldh ) gene faldh3a2 on the short arm of chromosome 17p11 . the disorder presents at birth or in the neonatal period with varying degrees of erythema and ichthyosis , but a collodion membrane is rarely seen . although erythema may be present at birth , it is usually not evident after a year of age . the morphology of the scaling may be fine , dandruff - like similar to ichthyosis vulgaris or x - linked recessive ichthyosis or lamellar similar to autosomal recessive congenital ichthyosis . the ichthyosis is generalized , but sites more predominantly affected are lower abdomen , sides and nape of neck , and flexures . the diagnosis of sls is delayed until the onset of neurological symptoms , because only cutaneous manifestations are present at birth . neuroimaging studies of the cerebral white matter and corticospinal tracts shows white matter disease , delayed myelination , periventricular gliosis , and a permanent myelin deficit . one - third of patients present with perifoveal glistening dots in the ocular fundus which appear after several years of age . the histopathological features are nonspecific and include hyperkeratosis and acanthosis with a normal granular layer . the diagnosis of sls can be confirmed by measurement of enzyme activity in cultured skin fibroblasts or leucocytes . sequence analysis of faldh3a2 gene causing mutations is highly sensitive and also detects possible carrier . multidisciplinary approach includes team of dermatologist , neurologist , ophthalmologist , orthopaedician , and physiotherapist . topical emollients , keratolytics , calcipotriol , and oral retinoids can be used to improve the cutaneous symptoms . cutaneous symptoms improved after treatment . in our case series , one child had congenital heart disease and cardiomegaly that has not been described in earlier reports . due to the limited availability of diagnostic laboratories , enzyme analysis was not done in our study . any case of congenital ichthyosis with spastic paresis and mental retardation should be evaluated for sls .

sjgren - larsson syndrome ( sls ) is a rare autosomal recessive disorder characterized by triad of congenital ichthyosis , spastic paresis , and mental retardation . it is an inborn error of lipid metabolism caused by deficiency of the enzyme fatty aldehyde dehydrogenase . we report our observations of six children with sls .

hypophysitis is an inflammatory condition of the pituitary gland that is often mistaken for other pituitary mass lesions . granulomatous hypophysitis is a rare inflammatory disorder , accounting for < 1% of cases involving panhypopituitarism or visual field defects with headaches . granulomatous hypophysitis commonly presents with enlargement of the pituitary gland that mimics an adenoma , making it difficult to confirm the disease until the time of surgery . in korea , only one case has been previously reported . we herein report a 31-year - old woman who had normal brain magnetic resonance imaging ( mri ) findings 4 months prior to admission and was diagnosed with granulomatous hypophysitis at the time of surgery . a 31-year - old woman was admitted to our hospital with severe headache , nausea , and vomiting . four months before admission to our hospital , she had visited the department of neurology due to a bilateral temporal headache . neurologic examination and 1 ) , so she was treated pharmacologically with a nonsteroidal anti - inflammatory drug . due to nausea and vomiting for 2 weeks , she was brought to the emergency room . her blood pressure was 110/70 mmhg , pulse rate was 58 beat / min , respiratory rate was 16 breath / min , and body temperature was 36.6. a neurologic examination revealed bitemporal hemianopsia . initial complete blood analysis revealed a white blood cell count of 4,860/mm , a hemoglobin level of 12.4 g / dl , and a platelet count of 241,000/mm . the biochemical test results were as follows : protein , 7.2 g / dl ; albumin , 4.2 g / dl ; aspartate aminotransferase , 16 iu / l ; alanine aminotransferase , 6 iu / l ; blood urea nitrogen , 5.9 mg / dl ; and creatinine , 0.5 mg / dl . the electrolyte test results were as follows : sodium , 113 meq / l ; potassium , 4.3 meq / l ; and chloride , 82 meq / l . the thyroid function test results were as follows : serum thyroid - stimulating hormone ( tsh ) level , 0.56 iu / ml ( reference range , 0.27 to 4.2 ) ; free t4 , 0.91 ng / dl ( 0.93 to 1.7 ) ; and t3 , 0.89 ng / ml ( 0.8 to 2.0 ) . we performed a combined pituitary stimulation function test and rapid adrenocorticotropic hormone ( acth ) stimulation test for further assessment . we injected regular insulin ( 0.1 /kg ) , thyrotropin - releasing hormone ( 200 g ) , and luteinizing hormone ( lh)-releasing hormone ( 100 g ) ; 2 hours later , the patient 's blood glucose level fell to 60 mg / dl and she complained of hypoglycemic symptoms . the combined pituitary function stimulation test showed no increase in serum growth hormone , acth , or tsh level . serum levels of lh and follicle - stimulating hormone were normal over time , and mild hyperprolactinemia was present with normal increments over time ( table 2 ) . t1- and t2-weighted mri showed an 18 10-mm round mass with isosignal intensity in the sella . 2 ) . prednisolone and levothyroxine were prescribed and the mass was removed using a transsphenoidal approach . the acid - fast bacilli stain , tuberculosis polymerase chain reaction , angiotensinogen - converting enzyme , and venereal disease tests yielded no abnormal finding . the patient was finally diagnosed with idiopathic granulomatous hypophysitis , and estradiol and progesterone were added to the prednisolone and levothyroxine for maintenance therapy . inflammatory pituitary lesions are extremely rare and can be classified into three clinicopathologic entities : lymphocytic , xanthomatous , and granulomatous hypophysitis . granulomatous hypophysitis , a rare chronic inflammation of the pituitary gland that creates granulomas , has been reported in < 1% of sellar lesions based on surgical findings with the transsphenoidal approach . lymphocytic hypophysitis is distinguished from granulomatous hypophysitis by the absence of nodular aggregations of epithelioid histiocytes and multinucleated giant cells , but these two diseases show ultrastructural similarities . the two entities may have the same pathogenetic background or even represent different stages of the same disease , and some authors have suggested that they represent an autoimmune spectrum from a purely lymphocytic form , constituting the predominant early lesion , to a granulomatous form appearing later . granulomatous hypophysitis , however , differs from lymphocytic hypophysitis in certain epidemiological features , which are mostly associated with pregnancy and autoimmune diseases . imaging studies can help to distinguish granulomatous hypophysitis from pituitary adenoma . in patients with granulomatous hypophysitis , mri usually shows symmetric enlargement of the pituitary gland , pre - contrast homogeneity of the pituitary mass , an intact sellar floor , and rapid enhancement of the pituitary stalk after gadolinium administration . however , these findings are not specific and are quite indistinguishable from other neoplastic or inflammatory hypophyseal processes on imaging . patients may also present with progressive chiasmal compression , hypopituitarism , amenorrhea - galactorrhea , hyperprolactinemia , fatigue , diabetes insipidus , a sudden onset of aseptic meningitis , and/or blurred vision . granulomatous hypophysitis is difficult to differentiate from a simple adenoma on the basis of clinical manifestations or imaging findings . secondary causes of granulomatous hypophysitis include infections ( tuberculosis , syphilis , and fungal ) , systemic inflammation ( sarcoidosis , wegener 's granulomatosis , crohn 's disease , and histiocytosis x ) , and foreign body reactions ( ruptured rathke 's cysts ) . hypopituitarism resolves after surgery in some cases , but many patients remain on full hormone replacement therapy , as in our case . lymphocytic hypophysitis was first presumed in the present case because of the rapidly growing sellar lesion observed on mri and the patient 's history of delivery 6 months prior to admission . however , the postoperative biopsy revealed granulomatous hypophysitis , and other examinations ruled out secondary causes of hypophysitis . in summary , idiopathic granulomatous hypophysitis is a very rare pituitary condition that is difficult to diagnose preoperatively . the treatment of this disease is surgery , which helps in diagnosis and treatment , and hormone replacement .

granulomatous hypophysitis is a rare pituitary condition that commonly presents with enlargement of the pituitary gland . a 31-year - old woman was admitted to the hospital with a severe headache and bitemporal hemianopsia . magnetic resonance imaging ( mri ) showed an 18 10-mm sellar mass with suprasellar extension and compression of the optic chiasm . interestingly , brain mri had shown no abnormal finding 4 months previously . on hormonal examination , hypopituitarism with mild hyperprolactinemia was noted . the biopsy revealed granulomatous changes with multinucleated giant cells . we herein report this rare case and discuss the relevant literature .

a man presented with clinical and radiographic evidence consistent with an acute appendicitis . after an ileocolic resection he was found to have intestinal spirochetosis of the caecum . a 50-year old- male presented to our institution in november with a three - day history of right lower quadrant abdominal pain associated with nausea . upon assessment in the emergency department , he did not appear ill and was afebrile . his white blood cell count was 12.0 x 10 cells / l . computed tomography abdomen and pelvis revealed a markedly dilated appendix measuring 1.3 cm with fat stranding and enlarged lymph nodes . the clinical impression at the time was consistent with an acute appendicitis with a potential caecal neoplasm . computed tomography images obtained preoperatively . the patient was given intravenous antibiotics and taken to the operating room within 5 hours of his initial presentation . a transverse right lower quadrant incision of the abdomen revealed an enlarged , firm appendix adherent to the lateral peritoneum was identified . the area of caecum surrounding the appendiceal base was firm and adjacent mesentery was oedematous . a neoplasm could not be adequately ruled out in the setting of an emergency operation with difficult anatomical visualization . an ileocolic resection was therefore performed with creation of a standard functional end - to - end ileocolic anastomosis using gastrointestinal staplers . the patient was also placed on a 10-day course of antibiotics ( ciprofloxacin and metronidazole ) postoperatively as the intra - operative impression was highly suspicious for a contained perforated viscus . at follow - up 10 days post - discharge the patient complained of occasional loose bowel movements but was otherwise feeling well . ( h&e staining , light microscopy , magnification x 200 ) ( b ) : caecum . note false brush border ( arrow ) composed of intestinal spirochetosis adhering to caecal mucosal epithelium . ( h&e staining , light microscopy , magnification x 400 ) the patient returned for follow up one month after surgery and was clinically well . at a subsequent visit 3 months later , he had a colonoscopy showing few diveritcula and a significant spasm . to our knowledge , he continues to remain asymptomatic . intestinal spirochetosis ( is ) was first discovered in 1719 by van leeuwenhoek who analyzed his own stool and identified small mobile spiral organisms.(1 ) is is a rare disease wherein gram negative bacteria belonging to the spirochetes species infect the gastrointestinal tract . spirochete organisms are classified into 3 groups : spirochaetaceae , leptospiraceae and brachyspiraceae ( 2 ) . two distinct groups have been identified to predominantly colonize human colorectum : brachyspira aalborgi and brachyspira pilosicoli . morphologically , is is characterized by the presence of microorganisms attached to colorectal epithelial cells in a perpendicular fashion . histologically they form a diffuse blue fringe along the border of the epithelial layer ( with hematoxylin - eosin staining ) . although invasion beyond the mucosal border is rare , mast cell and ige plasma cell reactions have been reported . it is suspected that loss of integrity of the mucosal surface may play a role in the mechanism of invasion.(1 ) the epidemiology of is was first described in the 20 century . a report published in 1923 in chicago revealed a 28% prevalence in stools of healthy individuals ( 3 ) . since then , other reports have demonstrated a direct relationship between the incidence of spirochetosis and rapidly growing industrial towns , homosexual men and patients infected with the human immunodeficiency virus ( hiv ) ( 2 ) . prevalence ranges from 1 - 5% in healthy individuals and within developed countries ( 4 ) to 64.3% in healthy individuals in a village in india ( 5 ) . difficulty with measuring prevalence resides in the sensitivities of methods of detection , which over the years has encompassed histology , microscopy , pcr , and in - situ - hybridization from fecal and/or tissue specimens . a retrospective study examined the colonoscopic descriptions of 15 patients with biopsies consistent with is . appearance , an erythematous mucosa was noted in one patient , a lesion was noted in one patient and the remaining 6 patients had normal mucosa ( 6 ) . is has been described to involve the mucosa of the colon , rectum and appendix . the severity of clinical manifestation has been correlated with the degree of histological invasion as evidenced by intestinal epithelial injury . however , it must be noted that presence of fecal spirochetes does not coincide with symptomatology . associated symptoms described include blood tinged feces , abdominal pain , constipation and most commonly diarrhea ( 1 ) . a little over ten cases of systemic spirochetemia ( mainly secondary to b. pilosicoli ) have been described in critically ill patients ( 7 ) . few reports in the literature ( amounting to less than 30 patients ) have described patients with intestinal spirochetosis who presented with a clinical and histological picture of acute appendicitis ( 8,9 ) . in 1984 , henrik - nielson and colleagues studied 681 appendiceal specimens and found is to be associated with 12.3% of clinically suspicious appendicitis that proved to be histologically normal whereas only 0.7% of is cases had a co - existing confirmed appendicitis on histology ( 8) . intestinal spirochetosis in the appendix , therefore , has a spectrum of presentation ranging from asymptomatic to fulminant histologically proven appendicitis . no consensus guidelines for the treatment of intestinal spirochetosis exists as only successful case reports of trial antibiotic regimens have been described . generally , a trial of antibiotic therapy is warranted , especially when the patient is symptomatic and/or histology shows signs of invasion . treatment with metronidazole administered at 500 mg q.i.d . for 10 days has been reported to eradicate symptoms and may lead to negative fecal cultures and improvement in histological features ( 4 ) . at our institution it is estimated that the prevalence of is in the hiv and in the homosexual population is as high as 54% ( 1 ) . most of these patients present with a chronic diarrhea and have a delayed diagnosis secondary to prolonged culture growth and low suspicion of this aetiology . in about two thirds of cases , patients with hiv diagnosed with is suffer from severe underlying disease and/or immunosuppression leading to death ( 10 ) . awareness of this disease entity should be heightened among surgeons especially those who treat patients with hiv .

acute appendicitis is a frequent pathology encountered by general surgeons . on the differential of aetiologies that can lead to this diagnosis lies the spectrum of infectious disease . we present a case report of a patient who presented to our institution as a classic acute appendicitis . upon histological analysis it became evident that he had spirochetosis of the cecum and appendix . this paper reviews the literature on this rare infectious aetiology . a synopsis of the microbiology , epidemiology , clinical manifestations and treatments for intestinal spirochetosis is described . at large , the aim of this paper is to heighten awareness of this disease amongst clinicians and specifically surgeons .

zollinger - ellison syndrome ( zes ) is caused by ectopic secretion of gastrin resulting in damage to gastrointestinal ( gi ) mucosa . zes may be sporadic or occur in association with multiple endocrine neoplasia type 1 ( men1 ) . zes is usually diagnosed in fifth decade of life . in one - third of patients with gastrinoma , laboratory analysis showed hemoglobin 11.5 g / dl , total protein 5.2 g / dl and albumin 2.5 g / dl . serum gastrin level was significantly elevated 9,100 pg / ml ( normal < 100 pg / ml ) . mri abdomen , somatostatin receptor scintigraphy and endoscopic ultrasound failed to reveal primary site of neuroendocrine tumor . gastrinomas are located in the duodenum ( 60 - 80% ) or pancreas ( 10 - 40% ) . most of gastrinomas ( 80% ) are sporadic but 20 - 30% occur in association with men1 . although gastrinomas are one of the most common pancreatic neuroendocrine tumors ( nets ) , only 30% arise in pancreas . abdominal pain ( 75% ) and diarrhea ( 73% ) are the most common clinical features of zes . diarrhea is caused by gastric acid hypersecretion and decreased absorption of sodium and water due to hypergastrinemia . fasting serum gastrin levels more than 10 times the upper limit of normal ( > 1,000 pg / ml ) in the presence of low gastric ph ( < 2 ) is diagnostic of zes . secondary hypergastrinemia can be seen in atrophic gastritis , renal failure and proton - pump inhibitor ( ppi ) use . more than 90% of the patients with zes have peptic ulcers and hyperplastic gastric folds . once diagnosis of zes is established , gastrinoma can be identified with a ct , mri or somatostatin receptor scintigraphy . medical therapy with high - dose ppis is the standard of care for zes patients with men1 syndrome . patients with zes should be started on high - dose ppis ( omeprazole 60 mg or pantoprazole 120 mg daily ) . patients with a sporadic gastrinoma and without evidence of metastases can be treated with surgery . hepatic artery embolization can be performed to control symptoms in patients with diffuse liver metastases . all authors have directly participated in the execution in a manner substantial enough to take responsibility for it .

zollinger - ellison syndrome ( zes ) is caused by hypersecretion of gastrin from duodenal or pancreatic gastrinomas . we report a case of a 57-year - old female who presented with chronic diarrhea . ct abdomen showed multiple liver masses . liver biopsy suggested metastatic well - differentiated neuroendocrine tumor . serum gastrin level was markedly elevated . mri abdomen , somatostatin receptor scintigraphy and endoscopic ultrasound failed to reveal primary site of the tumor . upper gastrointestinal endoscopy showed hyperplastic gastric folds and multiple duodenal ulcers consistent with zes . patient was started on high - dose omeprazole and octreotide resulting in improvement in diarrhea .

vasculitis of medium- and large - sized arteries is an uncommon disease with an estimated annual incidence rate of 1.2 - 2.6 cases per million . systemic symptoms may manifest in the form of fever , arthralgia , night sweats and weight loss . pulmonary sequestration consists of a nonfunctioning mass of normal lung tissue that lacks normal communication with the tracheobronchial tree , and that receives its arterial blood supply from the systemic circulation . it is estimated to comprise 0.15 to 6.4 percent of all congenital pulmonary malformations , making it an extremely rare disorder . a 25-year - old female presented with complaints of malaise , weight loss , and diffuse abdominal pain , more inthe umbilical region since 1 - 2 months . on examination , her blood pressure was 160/92 mm hg in right arm and 158/90 in left arm ; lower limb blood pressure was 126/80 in right lower limb and 128/82 in left lower limb . pulse rate was 90 per minute , regular , good volume with no radiofemoral delay . complete blood count , liver function tests , kidney function tests , and urine routine microscopy were within normal limits . erythrocyte sedimentation rate ( esr ) was raised to 35 mm / hour and c - reactive protein ( crp ) was more than 5 serology for hiv 1 and 2 , hepatitis b surface antigen ( hbsag ) , anti hepatitis c virus ( anti - hcv ) , venereal disease research laboratory ( vdrl ) test , anti - nuclear antibodies ( ana ) , cytoplasmic antineutrophil cytoplasmic antibodies ( c - anca ) and perinuclear antineutrophil cytoplasmic antibodies ( p - anca ) were negative . contrast enhanced computed tomography ( ct ) scan of the thorax and abdomen was done , which revealed thickening of the descending aorta upto bilateral renal artery origin with thickening of the superior mesenteric artery . ct angiography was done , and it revealed wall thickening of the descending aorta with narrowing of lumen . there was also thickening of the superior mesenteric artery with narrowing of lumen . there was evidence of a heterogeneous , hyperdense region in the left medial basal segment deriving its arterial supply separately from the aorta . there was no direct communication with the tracheobronchial tree , and the heterogeneous lung segment had no separate pleural covering . the above - mentioned findings were suggestive of an intralobar pulmonary sequestration [ figure 1 ] . bilateral renal artery showed stenosis with involvement of the ostia and proximal segment with post - stenotic dilatation [ figure 2 ] . axial computed tomography sections of the thorax showing heterogeneous segment ( black arrow ) in the medial basal segment of the left lung deriving its arterial supply from the descending aorta ( white arrow ) saggital contrast enhanced computed tomography image showing thickened wall of the descending aorta with narrowing of the caliber ( large arrow ) . volume - rendered angiographic images shows narrowing of the superior mesenteric artery ( small arrow ) with stenosis of bilateral renal arteries involving the ostia and proximal segments ( double arrows ) with post - stenoticdilatation . the arterial branch to the sequestered lung segment from the thoracic aorta is well seen in the central angiographic image vasculitis of medium- and large - sized arteries is an uncommon disease with an estimated annual incidence rate of 1.2 - 2.6 cases per million . the generalized symptoms include malaise , fever , night sweats , arthralgias , anorexia , and weight loss , which may occur months before vessel involvement is apparent . thickening of the vessel wall is the earliest manifestation of the disease before stenosis and dilatation occur . color doppler may be useful in identifying circumferential vessel wall thickening and thereby in evaluating and monitoring the disease in the aorta and its branches . conventional or digital subtraction angiography is the standard imaging tool for diagnosis and evaluation of vasculitis . but it only demonstrates the appearance of the lumen ; it may be normal in the initial stages of the disease with only diffuse mural thickening without any luminal narrowing . in these situations , noninvasive multidetector ct angiography is helpful in demonstrating mural thickening as well as luminal narrowing . use of contrast material may reveal enhancing inflammatory lesions in the early stage of the disease , prior to the development of stenosis . magnetic resonance ( mr ) angiography can also demonstrate thickening of the vessel wall , along with better soft tissue differentiation and other signs of inflammation like mural edema and increased mural vascularity . two types of pulmonary sequestration are recognized , depending on whether the abnormal lung tissue possesses its own pleural covering . in the intralobar pulmonary sequestration , the malformation is incorporated in the normal pulmonary parenchyma of a lobe with the venous drainage via the pulmonary veins . the extralobar pulmonary sequestration consists of pulmonary parenchyma separated from the rest of the lung by its own pleural envelope with its venous drainage via the systemic veins into the right atrium . pulmonary sequestration remains a rare congenital anomaly comprising 0.15 6.4% of all congenital pulmonary malformations and 1.1 1.8% of all pulmonary resections . numerous reports have described serious complications arising from pulmonary sequestrations such as fungal infection , tuberculosis , fatal hemoptysis , massive hemothorax , cardiovascular problems , benign tumors , and even malignant degeneration . distinguishing pulmonary sequestration from other pulmonary disorders , such as congenital cystic adenomatoid malformation , pulmonary emphysema , intrapulmonary abscess , bronchiectasis , or malignant tumor is not always possible on chest x - ray as on plain radiograph , the sequestered segment shows heterogeneous opacity with some cystic areas within . three - dimensional ( 3d ) reconstructions with multidetector ct depict the anomalous arterial supply and venous drainage . angiography is not a part of the routine investigation but is the gold standard in determining arterial supply . our patient presented with symptoms of intestinal angina , which included postprandial pain and weight loss , but appetite was intact . the superior mesenteric artery narrowing led to intestinal ischemia , which worsened on food intake due to mismatch of blood supply and demand . the stenosis of renal arteries explained the pathogenesis of hypertension , which was renovascular in origin . however , her chest x - ray was normal , and tuberculin test was also negative . moreover , her clinical profile did not meet the american college of rheumatology ( acr ) criteria for takayasu 's arteritis . to the best of our knowledge , this is the first ever case reported to have an asymptomatic intralobar pulmonary sequestration in association with medium and large vessel vasculitis .

vasculitis of medium- and large - sized arteries is an inflammatory and stenotic disease characterized by a strong predilection for the aortic arch and its branches . it presents with symptoms and signs as per the vessels and organs involved . pulmonary sequestration is a rare abnormality characterized by a mass of nonfunctioning lung tissue that receives its vascular supply from a systemic artery and is separated from the normal tracheobronchial tree . the following is a rare case report showing the presence of pulmonary sequestration in a patient with recently diagnosed hypertension and intestinal angina due to medium and large vessel vasculitis .

she underwent total abdominal hysterectomy with bilateral salpingo - oophorectomy followed by pelvic radiation in 2004 . , she presented with a gradually enlarging mass on the pubic area extending to the mons pubis for the past 2 months . she had a history of loss of appetite and weight loss of 3 kg within 2 months . clinical examination revealed a large well - defined firm violaceous plaque , 12 25 cm in size , and several bluish nodules on the mons pubis and labia majora , in the shape of a pair of panties ( fig . 2a ) , composed of multiple cords and strands of atypical cells with little mitosis , some forming irregular dissecting anastomosing lumen and papillary projection into the lumen ( fig . in our patient , the large tumour could not be resected ; chemotherapy was started with paclitaxel 70 mg / m every 2 weeks . after completing the fifth cycle of paclitaxel , the lesion was markedly decreased in size and the symptoms previously described were also completely resolved . angiosarcoma is a rare aggressive malignant mesenchymal tumour with endothelial differentiation that accounts for 2% of all soft tissue sarcomas . the majority of patients develop angiosarcoma at advanced age on sun - exposed skin , with predilection for the scalp . the diagnostic criteria for radiation - induced sarcomas include previous history of radiotherapy with a latency period of more than 34 years , development of sarcoma within a previously irradiated field or in the tissues adjacent to the field , and histologic confirmation . the most frequent clinical setting is the treatment of breast carcinoma , predominantly after radiotherapy in breast conservative therapy , followed by gynaecological malignancies such as ovarian , endometrial or cervical carcinoma . rare reasons for radiation treatment include acne , ringworm , graft radiation of a renal transplant , congenital hemangioma , hodgkin 's disease , multiple myeloma and melanoma . the median age at presentation is 68 years ; the median radiation dose is 4060 gy . the latency between radiation and diagnosis of raas is approximately 56 years , ranging from 1 to 40 years . the latency period is shorter in patients treated for breast carcinoma than in patients receiving radiation for other reasons ( median latency 5.5 and 30 years , respectively ) . the clinical presentation is quite variable , including violaceous or erythematous patches , plaques , nodules or bruise - like discolouration , overlying a painless area of induration . most are multifocal lesions and the size varies from a few millimetres to more than 20 cm ( median 7.5 cm ) . the histologic features range from well to poorly differentiated and can vary within a single case . well - differentiated angiosarcoma consists of irregular interanastomosing channels that infiltrate surrounding tissue , diffusely dissecting the dermal collagen . the atypical endothelial cells are usually multilayered , with enlarged and hyperchromatic nuclei and sometimes nucleoli . blood - filled spaces are a frequent feature in these less well - differentiated lesions . areas of necrosis are frequently present and the mitotic index is high . in poorly differentiated ones , it shows positive staining with antibodies against endothelial markers including cd31 , cd34 , fviii - related antigen and von willebrand factor . d2 - 40 ( podoplanin ) , known as a lymphatic marker , is positive focally less frequently in angiosarcoma . unfortunately , the treatment options are relatively limited , including surgery , radiation , chemotherapy and combinations thereof . however , when surgery is not possible , re - irradiation and chemotherapy have also proved to be beneficial . anthracyclines alone or in conjunction with ifosfamide have led to disease control after several months , always in combination with previous surgery . over the past few years , paclitaxel has been used in advanced - stage and/or metastatic angiosarcoma . the 5-year survival rates vary from 28 to 54% , and the overall 5-year local recurrence - free interval is 32% . . found that tumour size and age are the prognostic factors for local recurrence and that adding re - irradiation to surgery improves local control . however , some studies suggest a more indolent behaviour of raas compared with the conventional or lymphedema - associated variants [ 4 , 11 ] . raas of the breast has a significantly better prognosis more than that in other locations ( overall survival > 60 months and 7 months , respectively ) . raas is an angiosarcoma developing within a previously irradiated field , and the latency period is approximately 56 years . most of the patients incurred raas after the treatment of breast carcinoma , followed by gynaecological malignancies . the clinical presentations are multiple , rarely solitary large violaceous or erythematous patches , plaques , nodules or bruise - like discolouration .

we present a case of radiation - associated angiosarcoma . a 67-year - old thai woman was diagnosed with endometrium carcinoma stage ic and was treated with surgery and radiations . ten years later , she presented with a gradually enlarging mass on the pubic area , in the shape of a pair of panties . skin biopsy of lesions confirmed angiosarcoma . the diagnosis was radiation - associated angiosarcoma . she was treated with chemotherapy due to unresectable tumour . the chemotherapy was started with paclitaxel 70 mg / m2 every 2 weeks . after completing the fifth cycle of paclitaxel , the lesion was markedly decreased in size and the symptoms previously described were also completely resolved .

a 58-year - old female patient with relapse - remitting multiple sclerosis since 1992 had been treated from 1999 until 2007 with interferon beta-1a at two different doses . because of leukopenia and increasing relapses , immunomodulatory therapy was switched to natalizumab ( tysabri ) in 2007 . after only one relapse while on this treatment , she presented to an external neurological department in august 2010 with a reading disorder , deterioration in visual acuity , and weakness and spasm in her right leg . after intravenous treatment with methylprednisolone 1000 mg per day for 5 days , her clinical neurological symptoms partly recovered , but an ophthalmological deficit characterized by visual loss and visual field defects was unresponsive to this treatment ( see figure 1 ) . although ataxia and weakness on the right side increased , a lumbar puncture was negative for john cunningham virus ( by polymerase chain reaction [ pcr ] ) at that time . in october 2010 , the patient was referred to the neurological department of the ludwig - maximilians university in munich with suspicion of progressive multifocal leukoencephalopathy ( pml ) because the john cunningham virus pcr was now positive in the cerebrospinal fluid ( 468 copies / ml ) . the ophthalmologic examination at our clinic in october 2010 showed a best corrected visual acuity of 20/100 snellen equivalent in the right eye and in the left eye . the visual field ( octopus 101 , 30 , haag streit , germany ) displayed a homonymous hemianopia on the left side ( figure 2 ) . cranial t1-weighted magnetic resonance imaging revealed a nodular contrast enhancement with subcortical diffuse restriction in the right parieto - occipital region , consistent with the diagnosis of pml , in addition to white matter lesions typical of multiple sclerosis ( figure 1 ) . lumbar puncture showed normal white blood cell count , glucose of 52 mg / dl and protein of 60 mg / dl in the spinal fluid . a pcr for john cunningham virus was positive ( 431 copies / ml ) and confirmed the diagnosis of pml . within one month , general ataxia , reduction of free walking distance to 165 m , and weakness in the left leg worsened ( expanded disability score 5.5 ) , whereas the findings on visual field testing remained stable . follow - up magnetic resonance imaging revealed new subcortical temporo - occipital lesions with hyperintense signals in native t1-weighted images with diffuse restriction and contract enhancement at a size of 1.3 cm . the patient then underwent combination therapy of immunoadsorption ( five times ) , mirtazapine 45 mg / day , mefloquine 250 mg for 7 days , and immunoglobulins 25 mg / day on two consecutive days , starting in october 2010 . cyclosporin 150 mg / day was added in order to suppress potential immune reconstitution inflammatory syndrome , which has been described in almost all patients with natalizumab - associated pml so far . immune reconstitution inflammatory syndrome refers to clinical worsening in patients with opportunistic infections due to recovery of the immune system . despite one month of therapy and repeat high - dose methylprednisolone , the patients clinical and neurological status steadily worsened . as a result , the ophthalmological parameters , including visual acuity and visual field , were not evaluable due to the poor clinical condition of the patient . according to the patients subjective symptoms and enlarging pml lesions on magnetic resonance imaging , it can be assumed that these parameters were further deteriorating . a biopsy was performed demonstrating that the pml was the leading pathological finding in this patient and not immune reconstitution inflammatory syndrome.1 this case demonstrates the difficulty in diagnosis and treatment of natalizumab - associated pml due to the subtle clinical signs in different functional systems . pml is primarily known to occur in association with immunodeficiency disorders , such as acquired immunodeficiency syndrome , treatment with highly immunosuppressive agents such as rituximab , or rare congenital disorders like wiskott - aldrich syndrome.2,3 however , pml is gaining importance in association with the increasing use and duration of natalizumab therapy in patients with multiple sclerosis . natalizumab is generally well tolerated and has sustained safety and efficacy in patients with active multiple sclerosis.4 on the other hand , the expected benefits of natalizumab have to be weighed against the risk of pml , and patients need to be selected and monitored carefully.5 with regard to multiple sclerosis , most of the ophthalmological symptoms can be explained by optic nerve head disturbances . however , cortical involvement has to be taken into consideration in patients with nonspecific neurological signs and progression of the disease . the ophthalmologist plays a crucial role in being able to detect any deterioration clinically without too much effort even at the bedside . visual acuity and visual field examination are possible even in a very immunocompromised patient , and are completely noninvasive and cost - effective clinical measures . in this case , the main features leading to diagnosis of pml were detection of reactivated john cunningham virus in the spinal fluid , in combination with deteriorating clinical symptoms and findings on magnetic resonance imaging . there are data showing that natalizumab - associated pml and consecutive immune reconstitution inflammatory syndrome can be well managed in some cases.5 however , recent publications on this topic emphasize the critical importance of the time interval between diagnosis of pml , natalizumab cessation , and implementation of treatment for pml , resulting in a more favorable clinical course for this devastating disease.6 this case demonstrates that highly vigilant ophthalmological observation of patients with multiple sclerosis on natalizumab can contribute to a fast diagnosis of natalizumab - associated pml.79 we suggest that visual acuity and visual field testing should be performed every 3 months in patients treated with natalizumab for multiple sclerosis for longer than one year . in close cooperation , neurologists and ophthalmologists could thus prevent an unnecessary delay in diagnosing natalizumab - associated pml and initiate early treatment of this severe complication . atypical clinical signs and unusual morphological findings on magnetic resonance imaging should raise the suspicion of pml in a natalizumab - treated patient with multiple sclerosis .

this case describes typical ophthalmic findings as a key feature for diagnosis of progressive multifocal leukoencephalopathy ( pml ) and its possible differential diagnosis . a 58-year - old female patient with relapsing - remitting multiple sclerosis on immunotherapy with natalizumab developed visual disturbance , reading problems , and visual field defects due to pml . pml is a reactivation of latent infection with the john cunningham virus , which is a type of polyomavirus acquired in childhood or adolescence and is quite common in the general population . pml so far has been mostly associated with other immunodeficiency disorders , such as acquired immunodeficiency syndrome , but is also gaining importance in association with the increasing use and duration of treatment with natalizumab in patients suffering from multiple sclerosis . natalizumab is a highly specific 4-integrin antagonist approved for treatment of patients with active relapsing - remitting multiple sclerosis .

tuberculosis ( tb ) is the most widespread human infection worldwide affecting over one billion people . amongst the extrapulmonary sites , breast and bones are infrequent sites of tb , with less than 1 percent of reported cases . it is more frequently encountered in developing countries like africa and asia where the disease is found to be endemic . however the disease is assuming significance even in developed countries due to preponderance of aids . primary mycobacterial infection of the sternum , leading to osteomyelitis , is an extremely rare condition . tuberculosis of bones and joints accounts for 1 - 3% of patients with the disease . however , no case of tuberculous osteomyelitis of sternum following primary tb mastitis has been reported in literature so far to the best of our knowledge . a 40-year old married female presented with lump in the upper and inner aspect of left breast present since one month . there was associated low - grade fever with night sweats and evening rise of temperature . on examination there was a soft fluctuant swelling in the upper inner quadrant of breast . fine needle aspiration cytology ( fnac ) of the swelling revealed it to be a tubercular cold abscess ( figure 1 ) . the pus sent for ziel nielsen ( zn ) staining was positive for acid fast bacilli ( afb ) . nondependent aspiration of the abscess was carried out and patient was started on four drug first line anti - kochs therapy ( akt ) viz . : isoniazid ( h ) , ethambutol ( e ) , rifampicin ( r ) and pyrazinamide ( z ) . the patient presented a month later with a sinus and swelling over the anterior chest wall overlying the sternum . x - ray chest with contrast injected through the sinus ( sinogram ) showed osteomyelitic changes in the sternum with a cavity ( figure 2 ) . incision along with debridement and curettage of osteomyelitic bone was performed leaving an ulcer ( figure 3 ) . the ulcer healed with daily dressings by secondary intention . patient completed 12 months of akt , which included 3 months of herz and 9 months of hr along with pyridoxine and antacids . it usually results either as an extension from hilar lymph nodes or as part of hematogenous or lymphatic dissemination of the disease from other sites . sternal tuberculosis has also been reported after bacillus calmette - gurin vaccination in the pediatric age group . sir astley cooper in 1829 first described breast tb as a scrofulous swelling at the bosom of young women suffering from enlargement of cervical glands . the incidence of tuberculosis of breast is less than 1% . the resistance to survival and multiplication of mycobacterium tuberculosis offered by the breast tissue might be a contributory factor in low incidence of tb mastitis reported in literature . it comprises of two types , primary being confined only to the breast , and secondary with coexisting tuberculous lesion elsewhere in the body . recently it has been reclassified into three categories namely nodular , disseminated and abscess varieties . it occurs more frequently in women predominantly in the reproductive age group ( 17 - 42 years ) . various ways of manifestation include painless unilateral breast mass , generalized breast edema and localized abscess with or without axillary involvement . tenderness and erythema may also be present . although any area of breast can be involved but due to proximity of axillary nodes , upper outer quadrant is the most frequently site involved . very rarely the primary foci of tuberculosis from the breast may invade the pectoral fascia and involve the sternum leading to sternal tubercular osteomyelitis as seen in the present case report . sternal tuberculosis presents with an insidious swelling and pain over the sternum and constitutional symptoms are usually fewer , whereas patients with pyogenic sternal infections will have a fulminant clinical course with severe systemic upset . fnac or trephine biopsy with histological and microbiological examination of sternal tissue for caseating granulomas and acid - fast bacilli are the methods of choice for a definite diagnosis . fnac can diagnose tb osteomyelitis on the basis of caseating granulomas of epitheloid histiocytes and multinucleate giant cells . khanna et al . reported 100% success rate of fnac , while kakkar et al . reported a success rate of 73 percent . in a study by fadari - agrahi et al no patient had positive stain for afb . however , pus was positive for afb in the present study . computed tomography scans can be used to determine anatomical localization , osseous destruction , and soft tissue abnormalities . early drainage and complete debridement of necrotic material from the lesions along with multi - drug anti - tubercular therapy has been seen to hasten recovery . rotational tissue flaps can be employed to cover the chest wall defect due to the extensive loss of soft tissue and bone integrity after debridement . . however , with the resurgence of the disease , more virulent strains are seen invading this fascia giving rise to such unusual presentations .

sternal tuberculosis secondary to tuberculous mastitis is uncommon . the invasion of the sternum following a primary focus in the breast has not been reported . this may be due to the resistance offered by pectoral fascia and periosteum to the spread of infection into the bone . we present a case of tubercular sternal osteomtyelitis following tubercular mastitis in a 40-year old female . a brief case report and a review of literature are presented .

the introduction of osseointegration and the use of endosseous implants provide alternative treatment options to clinicians for all indications of edentulism.1 implant - supported , fixed restorations are usually classified as screw- or cement - retained.2,3 the advantage of screw - retained restorations is the combination of a rigid connection between the restoration - abutment complex and its retrievability . however , these restorations are usually more expensive than cement - retained restorations because of the use of extra components and laboratory costs.4 cement - retained restorations were introduced to compensate for problems of screw loosening and the lack of esthetics of screw - retained restorations.4 the lack of fastening screws in cement - retained restorations reduces the possibility of preload stress and screw loosening.5 the major advantages of cement - retained restorations are the passive fit of frameworks , enhanced esthetics resulting from lack of screw access holes , and reduced complexity of laboratory procedures and chair - side time.6,7,8 the disadvantages of cement - retained restorations include the requirement for extra time for cementation , removal of residual excess cement , limited design possibilities for superstructure , and the reduced possibility for modifying treatment in case of periimplantitis.9 the existence of residual excess cement in peri - implant sulcus is a common complication of cement - retained implant prostheses.10,11 if there is excess cement located in the soft tissue deeper than 3 mm , it might be difficult to observe and remove . insufficient removal of excess cement may result in swelling , soreness , exudation or bleeding on probing , and can initiate a local inflammatory process , which is evidence of peri - implant disease and can ultimately lead to implant failure.12,13,14 moreover , removal of excess cement may cause scratching and gouging on the implant surfaces when plastic and metal scalers are used.15 several authors have reported on techniques regarding procedures used to assist in minimizing residual excess cement extrusion.16,17,18 this article describes a method of controlling cement flow , using stock or custom implant abutments , when cement - retained implant - supported restorations are utilized . the method can be used easily and quickly at chair - side by the use of daily restorative and laboratory materials . the use of die spacers results in a uniform space between the crown restoration and the implant abutment . check the marginal fit of the crown restoration to the implant analog on the dental model . 1a ) according to the manufacturer 's recommendations ( siladent die spacer 12 m gold , dr . bhme & schps gmbh , germany ) . repeat applying the die spacer until the desired cement film thickness is achieved ( application of the die spacer 3 - 4 times forms a film thickness of approximately 45 - 50 m ) . completely fill the crown restoration with a bis - acrylic temporary restorative material ( luxatemp plus , dmg chemisch - pharmazeutische fabrik gmbh , germany ) and put a retention pin ( bredent gmbh & co. kg , germany ) with a smaller diameter tip into the uncured material to form a handle ( fig . 1d ) and secure the retention pin until the bis - acrylic material is cured . remove the crown restoration and check any discrepancies between the implant abutment and the bis - acrylic abutment . check that there are no voids on the duplicate abutment , and that the finish line has been duplicated accurately ( fig . 1f ) . clean the intaglio surface of the crown restoration with air and check any residual die spacer . mix a desired luting agent ( temp bond ne , kerrhawe s.a . , switzerland ) and apply to the intaglio surface of the crown restoration ( fig . 2b ) place the crown gently onto the bis - acrylic abutment and wipe off the excess cement with a cotton swab ( fig . if there is a lack of cement layer , line the intaglio surface of the crown restoration with a thin layer of extra luting agent . this article presents a method of minimizing the excess cement around implant - retained restorations . the advantage of the technique is allowing the control of cement flow by using a custom - made duplicate abutment that can be fabricated quickly , easily , and economically at the time of implant abutment / crown insertion.16 the major benefit of extraoral cementation is to allow the indirect removal of excess cement around the margins . this clinical procedure is extremely important for avoiding the potential of peri - implant disease caused by residual cement left in the gingival sulcus . it is important not to form an oversized cement space when duplicating the implant abutment . the use of a die spacer provides a space of approximately 50 m , which represents the ideal cement space , and may be used for both custom and prefabricated abutments . the disadvantage of the technique is that it is a time - consuming procedure for routine clinical processes . dumbrigue et al.16 stated that when the extraoral cementation technique is preferred , the luting agent must have a longer working time . when a custom abutment is to be used , the dental laboratory may be instructed to make an abutment analog using an acrylic resin , but this is time consuming for the technician and involves additional laboratory costs.18

the major drawback of cement - retained restorations is the extrusion of the excess cement into the peri - implant sulcus , with subsequent complications . insufficient removal of the excess cement may initiate a local inflammatory process , which may lead to implant failure . this article presents a method of controlling cement flow on implant abutments , minimizing the excess cement around implant - retained restorations .

some cutaneous manifestations serve as a warning sign for high likelihood of early onset coronary artery disease ( cad ) in an individual . these include xanthomas , xanthelasma , arcus juvenilis , and premature graying and balding in smokers ; markers of insulin resistance like skin tags , acanthosis nigricans , and central obesity ; hyperpigmented hands in betel quid sellers , cutaneous signs of gout , rheumatoid arthritis , psoriasis . we report here the case of a young male with familial xanthelasma and gynoid obesity who presented with acute st elevation inferior wall myocardial infarction . we present the case to highlight the familial xanthelasma with dyslipidemia which predisposed our patient to acquire early onset cad . a 35-year - old male presented to the medical emergency with complaints of retrosternal discomfort , uneasiness , and sweating for 1 hour prior to presentation . the patient had a similar episode 2 days back for which he was observed for a few hours and sent back home after serial electrocardiograms came out to be normal . the patient was a nonsmoker and there was no history of use of smokeless tobacco either . his father had died of heart attack at around 40 years of age . the patient had developed light - colored patches around the eyes over the past 10 years ; the same were also present in his mother and were also developing in his younger brother [ figures 1a c ] . ( a ) patient with prominent bilateral xanthelasma , ( b ) mother with prominent xanthelasma at medial and lateral canthi , and ( c ) brother with xanthelasma at medial and lateral canthi . on examination , the patient was conscious , oriented , and alert with a blood pressure of 112/76 mmhg . the pulse rate was 74/min with all peripheral pulses being palpable and no radio - radial or radio - femoral delay . the patient 's bmi was 32 kg / m with a waist to hip ratio of 100 cm/101 cm . ecg showed q waves and t wave inversions in leads ii , iii , and avf and tall the patient was thrombolyzed with streptokinase on the basis of ecg findings and the discomfort subsided following thrombolysis . post - stk ecg showed q - waves with t - inversion in the inferior leads . his fasting blood sugar was 97 mg / dl and 2 hour postprandial blood sugar was 104 mg / dl . the patient 's carotid intima media thickness was 0.056 on the right side and 0.058 cm on the left side with no plaque ( normal : 0.100 ) . the presence of xanthelasma in family members prompted us to get the lipid profile for the mother and younger brother also . the lipid profile of the three is shown in table 1 . as it turned out , all three were dyslipidemic with even the 26-year - old , nonobese younger brother qualifying for therapeutic intervention for dyslipidemia . the dutch lipid network criteria score for the index case was 5 in the absence of genetic analysis and was consistent with possible heterozygous familial hypercholesterolemia . as per the simon broome criteria also the patient is having possible heterozygous familial hypercholesterolemia . these are the most common cutaneous xanthomas , seen as yellow plaques over one or both lids . they are frequently symmetrical with the inner canthi involved more commonly than the outer ones and the upper eyelids more commonly than the lower . the incidence of xanthelasma in the general population has been variously reported between 0.3% and 1.5% . the age of onset ranges from 15 to 73 years with a peak in the 4 and 5 decades . histologically xp lesions consist of foamy histiocytes laden with fat , predominantly in the upper reticular dermis . in addition to being a cosmetic problem , xp draws great attention because of its controversial relationship with hyperlipidemia , atherosclerosis , ischemic heart disease , diabetes , and hypothyroidism . different studies have shown a varying incidence of dyslipidemia in individuals with xanthelasma ranging from as low as 9.1% to as high as 67.9%.[46 ] of these , a family history of xp can be expected in around 10% of patients . the lipids research clinics program prevalence study showed both xanthelasma and corneal arcus to be associated with increased levels of serum cholesterol and ldl - cholesterol , especially in young males . also , the odds ratio for ischemic heart disease was found to be higher in these individuals . the possible risk for atherosclerosis in normolipidemic patients with xanthelasma is more obscure because of the relative paucity of epidemiologic data and the conflicting results in some of the lipid studies . the cause in these patients could possibly be attributed to abnormal apolipoprotein levels or phenotype . thus xp should be considered as a marker for accelerated atherosclerosis even independent of cholesterol levels . interestingly our patient seemed to fit into probable heterozygous hypercholesterolemia . in conclude , one must be wary of the tendency to dismiss familial xanthelasma as just another family trait and look for underlying dyslipidemia and evidence of asymptomatic coronary artery disease in these individuals .

some external features serve as a warning sign for accelerated atherosclerosis . their early recognition may help in early detection and primary prevention / preemptive treatment of coronary artery disease ( cad ) . a 35-year - old nonsmoker , nonalcoholic , nonhypertensive , nondiabetic male presented with chest pain and was diagnosed to have acute st elevation inferior wall myocardial infarction . his father had died of cad at 40 years of age . the patient had bilateral extensive xanthelasma and gynoid obesity . his mother and younger brother also had evidence of bilateral xanthelasma ; both turned out to be dyslipidemic the younger brother qualifying for therapeutic intervention for dyslipidemia at 26 years of age . this case highlights the importance of familial xanthelasma as a cutaneous marker for underlying dyslipidemia and accelerated atherosclerosis in the young .

biliary colic is a common clinical presentation , with the majority of cases being related to gallstone disease . however , rarely , patients may present with biliary symptoms without evidence of gallbladder stones referred to as acalculous gallstone disease . this case report details a rare case of chronic biliary colic associated with ketamine abuse . a 24-year - old caucasian female presented to the emergency department with a history of intermittent right upper quadrant pain associated with nausea and malaise . she had experienced bouts of similar symptoms three times a year for the past 4 years . various investigations had been conducted during her multiple admissions , which showed possible dilatation of the common bile duct , with no evidence of gallstones . this requires considerable investigation , with an emphasis on drug history , especially with the current rise of recreational hallucinogenic drug abuse . rarely , patients may present with biliary symptoms without evidence of gallbladder stones ; commonly referred to as acalculous gallstone disease . this has been described in association with a wide variety of clinical conditions,1 the majority being related to infections and hematological disorders ( table 1 ) . there have been recent reports of increasing use of ketamine for recreational purposes ( street ketamine).2 ketamine was originally developed for use in anesthesia , especially within pediatrics . street ketamine is impure containing 30%90% ketamine , the reminder being composed of cutting agents such as paracetamol , caffeine , and flour . ketamine has a fast - acting nature and prolonged high with a limited hangover effect , which makes it a popular nightclub drug . ketamine abuse at moderate - to - high doses can cause sedation , immobility , and amnesia ; it increases the risk of serious central nervous system and cardiovascular effects , including respiratory distress or arrest , resulting in death.3 the physical side effects of ketamine when used for ecstasy is well researched and case reports show that ketamine may be associated with chronic epigastric pain , nonobstructive biliary dilatation , abnormal liver function tests , and urinary bladder dysfunction.4 here , we present a case of a young caucasian female who had a 4-year history of ketamine abuse and presented with multiple episodes of right upper quadrant ( ruq ) pains and symptoms suggestive of biliary colic . investigations showed a dilated common bile duct ( cbd ) with no evidence of gallstone disease . this study was deemed exempt from full review according to the southend university hospital ethical committee , uk as this was a retrospective , non - comparative study . written informed consent was obtained from the patient for publication of this case report and any accompanying images . a 24-year - old caucasian female presented to the emergency department with a history of intermittent ruq pain associated with nausea and malaise . she reported similar episodes of her symptoms three times a year for the past 4 years . she did not have any significant past medical history and did not take any regular medicines . on examination , she was hemodynamically stable ; there was no fever or tachycardia and her blood pressure was within normal limits . abdominal examination showed no evidence of peritonitis but revealed tenderness in the ruq and epigastric regions . full blood count and serum biochemistry showed a normal white cell count of 5.810/l with normal renal and liver function tests . the initial differential diagnoses by the admitting causality officer included biliary colic and peptic ulcer disease . she was admitted and started on opiate analgesics and antispasmodics and her symptoms gradually improved . ultrasound scan of the abdomen demonstrated a normal liver and gallbladder with no evidence of gallstones but with a dilated cbd of 8.5 mm . two days following admission , she underwent magnetic resonance cholangiopancreaticography , which again showed no gallstones , but also conversely no evidence of intra- or extrahepatic biliary dilatation with a cbd of normal diameter . on review of our patient s previous hospital admissions , we noted that 60 days prior , she had presented with similar episodes of ruq pain ; ultrasound scan of the abdomen and an esophagogastroduodenoscopy were normal . two years prior to that , she had presented to a different unit with similar symptoms and at the time radiological investigations revealed a dilated cbd of 11 mm with no other abnormality . on further enquiry into her social history , the patient admitted recreational use of oral ketamine over the past 4 years , all episodes of which coincided with her intermittent abdominal symptoms . ketamine is an n - methyl - d - aspartate receptor antagonist developed in 1962 for use in anesthesia.5 however , street ketamine , which is a close analog of ketamine , has become a commonly used drug for ecstasy . unlike the other it takes effect within about 10 minutes , while its hallucinogenic effects last up to 2 hours when ingested orally , making it a popular club drug parties.6 ketamine is metabolized by hepatic microsomal enzymes and has a half - life of 2.5 hours . it is excreted mainly through urine ( 90% ) , with the remainder through bile.7 wide variety of symptoms is associated with ketamine abuse . a large number of abusers present with impaired level of consciousness , dizziness , abdominal pain , and lower urinary tract symptoms . the most common physical signs in patients with ketamine abuse are hypertension , tachycardia , and abdominal tenderness.8 severe urinary bladder dysfunction and recurrent episodes of epigastric pain associated with dilated cbd in the absence of gallstones in ketamine abusers has also been reported.7 a study by ng et al8 investigated the clinical presentations of ketamine abusers in 233 cases . abdominal pain was the presenting complaint in 21% of cases while abdominal tenderness was noted in 18% of patients . two out of 35 patients who had radiological investigations had confirmed dilated bile ducts in the absence of gallstone disease.9 a case series published by wong et al10 also presented similar findings . in our case based on previous studies and case series , we believe that the ketamine abuse is the most likely cause for this . although the exact pathophysiology of ketamine - induced dilated biliary system is unknown , it is postulated that ketamine increases the flow of resistance across the sphincter of oddi.11 in conclusion , this case report highlights the importance of drug history taking in patients presenting with abdominal pain and specifically biliary colic .

introductionbiliary colic is a common clinical presentation , with the majority of cases being related to gallstone disease . however , rarely , patients may present with biliary symptoms without evidence of gallbladder stones referred to as acalculous gallstone disease . this case report details a rare case of chronic biliary colic associated with ketamine abuse.case presentationa 24-year - old caucasian female presented to the emergency department with a history of intermittent right upper quadrant pain associated with nausea and malaise . she had experienced bouts of similar symptoms three times a year for the past 4 years . various investigations had been conducted during her multiple admissions , which showed possible dilatation of the common bile duct , with no evidence of gallstones.conclusionpatients can present with a dilated common bile duct and an acalculous cholecystitis . this requires considerable investigation , with an emphasis on drug history , especially with the current rise of recreational hallucinogenic drug abuse .

we investigated 111 st1 gbs isolates collected by the toronto invasive bacterial diseases network during 20092015 . this network is a population - based surveillance system for invasive bacterial diseases in metropolitan toronto and peel region , ontario , canada ( total population under surveillance 5.5 million ) that includes all hospitals ( n = 28 ) providing care to and all laboratories ( n = 25 ) processing sterile site cultures from residents of the population area . most ( 103 ) of these st1 strains were serotype v , but 8 st1 isolates were found to be serotype ib , ii , iv , vi , vii , or viii ( table ) . these 8 strains represent an opportunity to investigate in more detail the molecular underpinnings of capsular switching in gbs . to this end , we sequenced the genomes of all 8 strains . we extracted genomic dna by using the qiagen dna minikit ( qiagen , toronto , ontario , canada ) and prepared genomic libraries by using nextera xt kits ( illumina , san diego , ca , usa ) . we sequenced libraries as paired - end reads with illumina hiseq ( 101 bp + 101 bp ) or miseq ( 150 bp + 150 bp ) instruments and deposited whole - genome sequencing data in the sequence read archive of the national center for biotechnology information ( table ) . we next performed de novo genome assemblies using the a5 pipeline ( 10 ) and assessed the cps loci of the 8 strains . in all cases , blastn ( http://blast.ncbi.nlm.nih.gov ) comparisons identified the cps locus expected for the serotype identified by latex agglutination . to characterize recombination leading to capsular switching , we first used the variant ascertainment algorithm vaal ( 11 ) to identify polymorphisms in each strain relative to the genome of serotype v st1 strain ss1 ( genbank accession no . strains of serotypes ib , ii , and iv had a relatively small number of polymorphisms , including single - nucleotide polymorphisms and small insertion / deletions , relative to the serotype v st1 reference . the serotype ib strain had 1,437 polymorphisms , the 2 serotype ii strains had 1,115 and 816 , respectively , whereas the serotype iv strain had 256 . however , strains of the other 3 serotypes had a substantially higher number of polymorphisms relative to the genome of the serotype v st1 strain ss1 . the 2 serotype vi strains had 12,703 and 9,406 polymorphisms , respectively , the serotype vii strain had 4,117 , and the serotype viii strain had 3,471 . by using custom scripts and the r software environment ( https://www.r-project.org ) , we created plots of polymorphism distribution relative to the genome of the reference strain with a sliding window of 5 kbp . we next plotted the polymorphisms identified in the strains against the genome of serotype v reference strain ss1 by using brig ( 12 ) and assessed recombination by using bratnextgen ( run with 20 iterations and 100 replicates at p = 0.05 ) ( 13 ) . the analysis showed that serotype ib , ii , and iv strains had a genome background similar to the st1 serotype v reference strain but that each had experienced horizontal transfer of genome sequences of differing sizes , all of which included the cps locus . namely , serotype ib strain ngbs217 had exchanged a dna region of 200 kb and acquired a cpsib locus ( figure , panel a ) . serotype ii strains ngbs814 and ngbs748 had exchanged dna regions of 189 kb and 152 kb , respectively , and gained a cpsii locus ( figure , panel b ) . recombination in the serotype iv strain ngbs1098 was less extensive ( 79 kb ) but resulted in acquisition of a cpsiv locus ( figure , panel c ) . thus , in strains of these 3 serotypes , capsular switching most likely resulted from a single recombination event that replaced the original cpsv locus of the receiving strains with those of the donor strains but left the rest of the genome of the receiving strains unchanged . we speculate that these recombination events most likely occurred by conjugation and that dna exchange took place in the human gut or urogenital tract . serotype v sequence type ( st ) 1 group b streptococcus isolates collected by the toronto invasive bacterial diseases network , ontario , canada , 20092015 . polymorphisms identified in non serotype v st1 strains ( illustrated in different colors , with each line representing a polymorphism ) are plotted against the genome sequence of reference serotype v st1 strain ss1 ( innermost circle ) . genome landmarks , such as genes used in the multilocus sequence typing scheme ( adhp , atr , tkt , glck , sdha , glna , and phes ) are shown in light blue . polymorphisms mapping to mobile genetic elements identified in the various non serotype v st1 strains were not included in the analysis . in contrast with those results , the genomes of strains of serotypes vi , vii , and viii differed extensively from the reference serotype v strain . indeed , recombination detected by bratnextgen involved > 50% of the genomes of these isolates , even though they were found to be st1 by mlst ( figure , panels d f ) . based on the available data , it is difficult to ascertain if those strains were originally st1 strains that acquired a cps locus and many other genomic regions by recombination in apparently multiple recombination events or , alternatively , if the isolates under investigation originally possessed the cps locus corresponding to their serotype and acquired by recombination from unknown st1 donors the different portions of the genome that contain all genes used in the mlst scheme . in the latter scenario , and in particular for serotype vi strains , the increased number of polymorphisms in st1-like areas of the genome suggests that the putative st1 donor was genetically not closely related to the serotype v st1 reference strain ss1 . our data suggest that extensive recombination might be a key contributor to clonal diversification and emergence of gbs serotypes vi , vii , and viii , which are less often identified in north america but have substantial regional dominance in other geographic areas ( 15 ) . mlst is a useful approach to begin to examine the genetic relationships of gbs strains . in some cases , particularly when temporally and geographically related isolates are evaluated , this approach also permits initial prediction of recombination events ( 3,58 ) . whole - genome sequencing extends mlst capabilities and enables precise identification and characterization of genetic variation attributable to extensive recombination in this opportunistic pathogen . although the polysaccharide - based , trivalent conjugate gbs vaccine under development offers great promise , frequent capsular switching suggests that subunit vaccines based on antigens expressed by all gbs serotypes might offer enhanced protection . results of a sliding - window analysis of the distribution of single - nucleotide polymorphisms identified in the genomes of 8 non serotype v sequence type 1 group b streptococcus isolates collected by the toronto invasive bacterial diseases network , 20092015 , relative to the genome of serotype v sequence type 1 reference strain ss1 .

we report several cases of recombination events leading to capsular switching among sequence type ( st ) 1 group b streptococcus strains . these strains otherwise shared a common genome backbone with serotype v st1 strains . however , the genomes of st1 serotype v strains and those of serotypes vi , vii , and viii strains differed substantially .

air - blowing equipment is often used in dentistry and oral surgery , and there are many reports of subcutaneous or mediastinal emphysema with the use of these machines . furthermore , emphysema has been reported with jaw corrective surgery , injuries and alternating washes in root - canal therapy . however , there are no previous reports of subcutaneous emphysema after the maxillary sinus - lift procedure . here an 80-year - old man with a history of en bloc resection of squamous cell carcinoma of the hard palate ( t4an0m0 ) complained of mastication difficulties due to the maxillary defect , and water and air leakage into the maxillary sinus . maxillary segmentectomy included resection of the alveolar ridge from the left central incisor to the right pterygoid process of the sphenoid bone ( fig . 1 ) . the resection site was reconstructed with a forearm flap , and the maxillary sinus was completely closed . however , these teeth were not strong enough to support a large jaw prosthesis that would cover the right maxillary defect , and residual teeth were missing . however , computed tomography ( ct ) revealed that the bone mass of the left molar region was inadequate for implantation of these fixtures ( fig . 2 ) . the soft tissue from the canine region to the molar tooth region was incised and elevated to expose the underlying lateral wall of the left maxillary sinus . a 12 5 mm window was created in the bone of the maxillary sinus anterior wall with a piezoelectric instrument to expose the underlying schneiderian membrane , which is the lining of the maxillary sinus cavity . through careful instrumentation , the membrane was carefully peeled from the inner aspect of the sinus cavity ( fig . the newly formed space within the bony cavity of the sinus inferior to the intact membrane was grafted with artificial bone , and artificial bone was placed on the alveolar crest to raise the alveolar crest ( fig . figure 2:the bone mass in the left molar region was inadequate for implantation of fixtures . a 12 5 mm section of bone has been removed at the maxillary sinus anterior wall with a piezoelectric instrument . ( b ) artificial bone has been grafted in the formed sinus cavity and placed on the alveolar bone . a 12 5 mm section of bone has been removed at the maxillary sinus anterior wall with a piezoelectric instrument . ( b ) artificial bone has been grafted in the formed sinus cavity and placed on the alveolar bone . two hours after the surgery , the patient complained of sudden malar swelling ( fig . marked swelling was present from the left infraorbital region to the buccal region , and we suspected postoperative bleeding . there were a few hematomas in the wound , but an apparent bleeding point was absent . the ct images revealed that the artificial bone grafted inferior to the membrane had not leaked into the maxillary sinus , and there was no apparent damage of the maxillary sinus membrane . however , the marked malar swelling was associated with air pockets at the alar base and in the angulus oculi medialis region and subcutaneous malar tissue , and no hematoma was evident ( fig . we made a diagnosis of subcutaneous emphysema and administered an intravenous drip containing antibiotics to the patient for 3 days . we confirmed emphysematous improvement and the graft situation of the artificial bone on the ct images at 12 days postoperatively . figure 4:after sinus - lift surgery , marked swelling is obvious from the left buccal region to the angulus oculi medialis . emphysema ( indicated by arrows ) is visible from the left buccal region to the angulus oculi medialis . after sinus - lift surgery , marked swelling is obvious from the left buccal region to the angulus oculi medialis . emphysema ( indicated by arrows ) is visible from the left buccal region to the angulus oculi medialis . emphysema of the maxillofacial region caused by dental treatment , oral surgical procedures or trauma is frequently reported . therefore , the mucous membrane of the maxillary sinus might have had a small hole , and air might have entered the subcutaneous tissue via the bone window when the air pressure in the maxillary sinus increased with nose blowing . one of the complications of the sinus lift is damage of the maxillary sinus membrane , and perforation of the sinus membrane occurs in 1035% of sinus - lift procedures , although membrane perforation is reduced to 7% when osteotomy is performed using the piezoelectric technique . the thickness of the sinus membrane is < 1 mm , so the membrane is easily damaged , and a small hole may not be apparent during the operation . the maxillary sinus is sometimes perforated by the extraction of teeth in contact with the maxillary sinus floor . perforation is common with the presence of a maxillary sinus septum or a history of surgery in the sinus region . rhymes reported that if the vertical section of the attached gingiva reaches the infraversion , then , during tooth extraction , it is easy to cause emphysema because air readily invades the loose tissue . furthermore , an inflow of air from the gap is reported to occur easily because of the texture when the periosteum or fascia is torn . , surgeons should take care not to tear the periosteum during periosteum detachment in mucoperiosteal flap creation . emphysema with sinus lift has not previously been reported and may not be anticipated with this procedure . patients are usually requested not to blow their noses after extraction of teeth in contact with the maxillary sinus or palatal closure of a fistula . however , we did not consider this precaution necessary with the sinus - lift procedure . it is important to advise patients to avoid increasing the intraoral pressure , for example , by blowing the nose vigorously or playing musical instruments , as these activities could cause introduction of more air . therefore , it is important to warn patients about nose blowing , coughing , sneezing and mucous plugging during the postinjury and postoperative periods [ 8 , 9 ] . special attention should be paid to postoperative supportive care to avoid increased subcutaneous air diffusion in patients who have undergone open surgery . however , it is necessary for oral surgeons to consider the possibility of postoperative subcutaneous emphysema after a maxillary sinus lift and to inform patients accordingly .

an 80-year - old man with a history of en bloc resection of squamous cell carcinoma of the hard palate ( t4an0m0 ) was performed a lateral - window sinus lift of the edentulous area of the left maxillary molar region to facilitate future placement of dental implants.two hours after the surgery , the patient complained of sudden malar swelling . marked swelling was present from the left infraorbital region to the buccal region . the swelling was associated with air pockets at the alar base and in the angulus oculi medialis region and subcutaneous malar tissue . emphysema appeared after the patient blew his nose . therefore , the mucous membrane of the maxillary sinus might have had a small hole , and air might have entered the subcutaneous tissue via the bone window when the air pressure in the maxillary sinus increased with nose blowing . it is important to advise patients to avoid increasing the intraoral pressure after sinus - lift procedure .

the latter enters the cranium via the mastoid foramen to supply the dura of the posterior fossa . it also participates in anastomoses with other vessels , most important one being the vertebral artery and branches of anterior inferior cerebellar artery . the transmastoid artery is an important feeder to posterior fossa vascular malformations and tumors and can be the only prominent supply occasionally . superselective microcatheterization of this artery , however , can be difficult due to extreme tortuosity and the difficulty in navigating through the narrow mastoid foramen . a 45-year - old woman presented with headache since 2 years and gradually deteriorating vision since 3 months . she had bilateral papilledema ( left more than right ) with decreased visual acuity ; however , other neurological examinations were normal . a magnetic resonance imaging ( mri ) scan showed a large left petrous - based meningioma , bright on t2w images and enhancing brilliantly on post - gadolinium scans . considering these to be indicative of hypervascular nature of the tumor , a preoperative embolization was undertaken . during the procedure , the tumor was found to be hypervascular and with a single feeding artery , that being the transmastoid branch of the left oa [ figure 1a ] . however , this artery proved difficult to catheterize due to its tortuosity , small size , and a recurrent course of the ostial and proximal portions . various microcatheters ( including semi and true flow - guided catheters ) and microguidewire systems were used without benefit . an attempt was then made to induce spasm in the parent artery distal to the feeding vessel origin by repeated passages and torquing the wire . however , this also turned futile as the spasm inductions lasted short [ figure 1b ] . ( a ) left external carotid artery injection showing the hypervascular posterior fossa tumor fed primarily by the transmastoid foraminal branch of occipital artery . ( b ) microcatheter injection of the left occipital artery near the mastoid branch showing spasm of the occipital artery with no forward flow . ( c ) post coil - occlusion of the occipital artery ; the contrast flow is entirely via the artery of interest now . we then decided to sacrifice the parent artery and used a guglielmi detachable coil ( gdc ) for the same . the artery was sacrificed just distal to the origin of the mastoid branch [ figure 1c ] . after occlusion , embolization was done with polyvinyl alcohol ( pva ) particles , injections being made from the oa trunk but still outside the cranium . care was taken to inject slowly , allowing the pulsations carry forward the contrast - mixed particles , and not allow any reflux . post procedure angiogram showed near complete occlusion of the tumor bed [ figure 1d ] . the patient was operated on after 2 days , with complete excision of the tumor and minimal blood loss . the transmastoid artery ( 1 - 3 in number ) is a major and important branch of the oa ( with the stylomastoid branch ) passing through the mastoid emissary foramen and supplying the dura of the posterior fossa.[13 ] it is a prominent supply to vascular malformations and tumors of the posterior fossa . it enters the mastoid foramen soon after origin and may have a recurrent proximal course . the mastoid foramen is often small and often does not allow the passage of microcatheter of the available sizes . the narrow - caliber ( mean diameter 0.6 mm ) and the tortuosity of the artery does not make things any easier . the oa provides arterial supply to most of the muscles of the posterior and lateral upper portion of the neck , occipital muscles , posterior half of the scalp , and multiple neuronal branches to facial , spinal accessory , hypoglossal , occipital nerve of arnold and greater occipital nerve . its terminal branches form an anastomotic rete with branches of the contralateral oa , posterior auricular artery , and superficial temporal artery.[13 ] hence , knocking off the main trunk does not have any major side effects . in fact , for the same reason , it is a favored artery for bypass surgeries and can be ligated / occluded during surgeries or for treating aneurysms / pseudoaneurysms . however , unwanted embolization into the distal vascular bed can lead to scalp necrosis and neuronal ischemia , and hence we must prevent the same . in our case , we occluded the arterial trunk just after the mastoid branch had originated , so as to enable injection of pva particles without entering the mastoid foramen . another important point to be remembered is the common occurrence of occipital to vertebral artery anastomosis . hence , mixing of contrast agent with the pva is compulsory and so is a slow injection , with the surgeon and assistants on an active lookout for any reflux . here we describe a case of petrous meningioma with a single feeder from the mastoid branch of the oa , which could not be superselectively catheterized ; hence , the occipital trunk was sacrificed and pva particles injected without entering the cranial cavity .

the transmastoid branch of the occipital artery is an important supply to posterior fossa vascular malformations and tumors and is often difficult to catheterize due to tortuosity and a transforaminal course . in very difficult situations , we can try to induce spasm of the occipital artery just beyond the origin of the mastoid branch by repeated passages of the microcatheter / wire . this induces a temporary ligation like effect so that the microcatheter can then be manipulated into the mastoid branch via the mastoid foramen . rarely , the occipital artery has to be sacrificed if spasm can not be induced or is short living , following which particles can be injected from a distance without entering the mastoid foramen . occluding the occipital artery proximally has no effect on distal perfusion , as collaterals and anastomoses from superficial temporal artery , posterior auricular artery , and opposite occipital artery take over the supply .

the world health organisation estimates that each year more than 8 million new cases of tb occur and approximately 3 million people die of the disease worldwide . recently , the increase in the incidence of drug resistant tb has further worsened the problem . oral tb is rare and accounts for less than 1% of all cases of tb . with the increasing number of tb cases , unusual forms of the disease in the oral cavity a 7-year - old boy presented with difficulty in swallowing solid foods , low - grade fever and loss of weight for 11/2 month . there was no history of cough , abdominal pain , vomiting , diarrhea , or urinary complaints . on admission , his heart rate was 104/min , the respiratory rate was 28/min and blood pressure was 98/60 mmhg . his weight was 13 kg , and height was 104 cm ( both below the fifth percentile for age and sex ) . oral cavity examination showed perforation of the hard palate , 3 3 cm with an irregular , undermined margin , and a necrotic base [ figure 1 ] . investigations showed : hemoglobin 8.8 g / dl , total leucocyte count 9000/cumm ( neutrophils 40% , lymphocytes 60% ) , erythrocyte sedimentation rate 80 mm at the end of 1 h. his liver and renal functions tests were normal . computed tomography scan of the oral cavity showed erosion and scalloping of the posterior part of the hard palate on the right - side [ figure 2 ] . high power view of papanicolou stain of fine - needle aspiration of the lymph nodes showed granular caseous necrosis and granuloma composed of epitheliod cells and histiocytes [ figure 3 ] . however , in view of the clinical presentation and evidence of pulmonary and lymph node tb , the palatal perforation was most likely due to tb . he was started on antituberculous therapy ( isoniazid , rifampicin , pyrazinamide , and ethambutol ) and is on regular follow - up with the oral surgeons for repair of the palatal perforation . oral cavity examination showing perforation of the hard palate , 3 3 cm in size with an irregular , undermined margin , and a necrotic base plain axial computed tomography scan of midface with 3d reconstruction showing erosion and scalloping of the posterior part of the hard palate on the right side high power view of papanicolaou stain of fine needle aspiration of the cervical lymph nodes showing granular caseous necrosis and granuloma composed of epithelioid cells and histiocytes oral tb may either be primary , or more often , secondary to pulmonary tb . in secondary oral tb , the bacilli reach the oral mucosa by hematogenous or lymphatic spread . in primary oral tb there is direct inoculation of the mycobacterium due to break or loss of the natural barrier resulting from trauma , inflammatory conditions , leukoplakia , tooth extraction , or poor oral hygiene . other local predisposing factors include dental cysts , periapical granulomas , dental abscess , periodontitis , and jaw fractures.[24 ] abbot et al . were able to isolate the tubercle bacilli from mouth washings of 44.9% of the patients with active pulmonary lesions and thus highlighting the importance of an intact mucosal epithelium in resisting oral tb infection . the systemic factors that favor the chances of oral infection in tb includes lowered host resistance and increased virulence of the organisms . tuberculous involvement of oral cavity is rarely seen even in populations with high incidence of the pulmonary disease . factors that attribute to relative resistance of oral cavity to tb are protective effect of saliva , presence of saprophytes , resistance of striated muscles to bacterial invasion , and thickness of protective epithelial covering . other sites include the soft palate , hard palate , lip , cheek , tonsils , gingiva , floor of mouth , uvula , and alveolar mucosa . table 1 highlights some of the reported cases of oral tb in the literature.[2369 ] oral tb lesions usually manifest as single , non - healing ulcers with an indurated , irregular , undermined margin , and a necrotic base . oral lesions can also present as nodules , fissures , plaques , vesicles , tuberculomas , or granulomas . the palatal lesion of tb may be seen as granulomas , ulceration , or perforation and are usually more common in the hard palate than in the soft palate . according to baruah , et al . palatal involvement of tb resulting in perforation is usually seen in patients with strong immune responses and considered to be due to hypersensitivity to acid - fast bacilli that causes tissue destruction . reported cases of oral tuberculosis in addition to tb , the causes of palatal perforation include infection ( syphilis , leprosy , leishmaniasis , or fungal infection ) , wegener 's granulomatosis , sarcoidosis , neoplasms ( salivary or squamous cell ) , drug abuse ( cocaine ) , and midline lethal granuloma . in our case , the above differentials were unlikely in view of the clinical presentation , evidence of pulmonary and lymph node tb and the clinical improvement seen with antituberculous therapy on follow - up . the treatment of palatal tb should follow the general guidelines established for the treatment of extrapulmonary tb . tb of the palate is relatively rare and should be included in the differential diagnosis of palatal perforation . also , a search for the primary site should be done extensively in all the cases of palatal tb .

tuberculosis ( tb ) of the hard palate is rare and usually secondary to pulmonary tb . we present a case of a 7-year - old boy who presented with difficulty in swallowing solid foods , low - grade fever and loss of weight . oral cavity examination showed perforation of the hard palate with an irregular , undermined margin and a necrotic base . chest x - ray showed homogeneous opacity in the right upper zone . fine - needle aspiration of the cervical lymph nodes showed granular caseous necrosis and granuloma composed of epitheliod cells and histiocytes . in view of the clinical presentation and evidence of pulmonary and lymph node tb , the palatal perforation was most likely due to tb . patient was started on antituberculous therapy and is on regular follow - up .

on august 5 , 2011 , a community hospital laboratory contacted the minnesota department of health to submit for identification a bacillus sp . cdc typed the isolate as gt59 using multiple - locus variable - number tandem repeat analysis specific for 8 loci ( 8) . the isolate was related to strains with genotypes generally associated with imported animal products and most closely related to a strain obtained from a 1965 investigation of a case of cutaneous anthrax in a worker at a new jersey gelatin factory , which used bone imported from india ( 9,10 ) . the patient , a 61-year - old florida resident , had begun a 3-week trip with his wife on july 11 , 2011 . they drove through north dakota , montana , wyoming , and south dakota , where animal anthrax is sporadic or enzootic ( figure ) . while traveling , they walked in national parks , collected loose rocks , and purchased elk antlers . on july 29 , they drove through herds of bison and burros , frequently stopping while animals surrounded their vehicle . the man was hospitalized on august 2 , following onset of illness while he was en route to minnesota . his condition was treated with antimicrobial drugs , according to published recommendations , supplemented with anthrax immune globulin , and he fully recovered ( 11 ) . route traveled by anthrax case - patient in the united states , july 11 , 2011 to august 2 , 2011 . the couple had no contact with dead animals , but reported dusty conditions while driving through the herds . the patient had not traveled abroad during the past year or been exposed to tanneries , wool or goat hair mills , bone meal , african drums , or illicit drugs . he crafted metal and stone jewelry and knives with elk - antler handles in a home workshop . one month before illness onset , he had constructed fishing flies using hair from a healthy elk he hunted in kentucky 8 months previously . because his wife may have experienced the same exposure , postexposure prophylaxis was provided , including a 60-day course of oral antimicrobial drugs and a 3-dose series of anthrax vaccine adsorbed ( emergent biosolutions , lansing , mi , usa ) , under an investigational new drug protocol . symptoms of anthrax did not develop ; however , she was unable to provide blood for serologic testing before starting postexposure prophylaxis . environmental sampling of the entire trip route was not feasible because the travel route involved several thousand miles over 3 weeks , specific suspected exposure locations were lacking , and recovering b. anthracis from environmental samples ( 12 ) , particularly soil ( 13 ) , gives variable results and is inefficient . to identify an exposure source and focus the environmental investigation , investigators obtained targeted samples from the vehicle and personal items where spores were likely to be found . from the patient s vehicle and contents , 47 environmental samples were collected , and 18 environmental samples were obtained from his home workshop and garage ( table ) , following procedures of the national institute for occupational safety and health and the environmental protection agency ( 14 ) . all samples were processed and cultured by using cdc s laboratory response network culture and pcr methods at the minnesota department of health and the florida department of health . multiple agencies , including cdc , state public and animal health departments of minnesota , north dakota , montana , wyoming , south dakota , and florida , and other federal and state partners , conducted enhanced retrospective surveillance to identify other possible human or animal anthrax cases during june 1august 31 , 2011 . infection preventionists , medical examiners , and coroners were asked if they were aware of cases of unexplained death or fulminant illness potentially caused by anthrax . the us department of agriculture veterinary services and wildlife services / animal and plant health inspection service , the southeastern cooperative wildlife disease study , the us geological survey national wildlife health center , the national park service , and state wildlife and veterinary agencies were asked to report unexplained animal die - offs and animal deaths consistent with anthrax . in addition , 450 laboratory response network and 7 national animal health laboratory network laboratories in the participating states , the national veterinary services laboratories , and other veterinary and wildlife laboratories were asked to review records for non - hemolytic bacillus spp . a single bovine case was reported in aurora county , south dakota , in august 2011 ; however , the isolate was determined to be of an unrelated genotype ( gt3 ) . no other anthrax - associated die - offs in wildlife or domestic animals were identified , and no other suspected or confirmed human cases of anthrax were identified or reported during this period . we did not find a specific exposure associated with anthrax infection for this case - patient and no other human or related animal cases . the clinical isolate genotype and sequence closely matched several previous environmental sample isolates from north america , however , no epidemiologic links were identified . the case - patient was exposed to airborne dust while traveling through areas where anthrax was enzootic ; however , testing of vehicle air filters in which the dust was concentrated was negative for b. anthracis . nonetheless , the patient may have been exposed through contact with an unidentified contaminated item . this investigation was limited by the poor sensitivity expected for soil sampling and lack of data to guide additional focused environmental sampling , precluding widespread random sample collection and testing along the route traveled . it is unusual for inhalation anthrax case - patients to have no identified exposure source ( 5,9,15 ) . inhalation of spore - contaminated soil has been suggested as a possible source of infection for bison in anthrax outbreaks in canada ( 16 ) . a heavy equipment operator acquired inhalation anthrax during a bison outbreak in canada , where he dragged carcasses to burial sites and was exposed to airborne dust during operations ( 2 ) . chronic pulmonary disease or immunosuppression may increase a person s susceptibility to inhalation anthrax ( 9,15 ) . this case - patient had a decades - long history of chemical pneumonitis , and although he reported no respiratory difficulties , perhaps his risk was increased . he also had a history of mild diabetes ; diabetes has been observed in other anthrax patients ( 9 ) . this report highlights the challenges of investigating cases of anthrax when no specific suspected source exists . anthrax , either naturally - occurring or bioterrorism - related , is a major public health concern , and timely recognition is critical . clinicians and public health professionals should be cognizant that naturally acquired anthrax can occur in the united states and take appropriate steps to rapidly diagnose and investigate such cases .

inhalation anthrax occurred in a man who vacationed in 4 us states where anthrax is enzootic . despite an extensive multi - agency investigation , the specific source was not detected , and no additional related human or animal cases were found . although rare , inhalation anthrax can occur naturally in the united states .

myrtle ( myrtus communis l. ) is an evergreen shrub belonging to the family of mirtaceae that grows spontaneously throughout the mediterranean area . in italy it grows along the coast and in the inner hills , and it is spread especially in the islands , where it is one of the most characteristic species . myrtus communis had history in the popular and traditional medicine : the essential oil obtained from leaves and , sometimes , flowers and berries has been used for its tonic and balsamic properties , and it is used in flavour and fragrance industries . in sardinia natural formations are still the main source for the production of a traditional liqueur , that every year reaches 3 million bottles . essential oils are gaining remarkable interest for their potential multipurpose use as antioxidant , antibacterial , and antiseptic agent [ 14 ] ; the essential oil obtained from the leaves was used in the past for the treatment of lung disorders . the isolation of essential oils from myrtus communis leaves is usually obtained by hydrodistillation method with a clevenger - type apparatus , according to the italian official pharmacopoeia . the chemical composition of the essential oils , analysed by gas / cromatography ( g / c ) , generally exhibits -pinene , 11% ; 1,8-cineole , 16% ; linalool , 12% ; -terpineol , 7% ; and limonene , 5% . the sardinian myrtle oil is characterized by the lack of myrtenyl acetate and by a higher content of limonene . . showed the biological activities of tannins , including anticancer and antioxidant . in our previous studies ( data not published ) the antimicrobial properties of myrtus essential oil against several clinical strains and in particular against helicobacter pylori were studied , and we obtained encouraging results . considering these results , in this study , we have used this essential oil towards strains of m. tuberculosis and strains of mycobacterium avium subsp . in this study we investigated the antimicrobial properties of the essential oil of myrtus communis against two reference strains : m. tuberculosis h37rv ( virulent strain ) and m. tuberculosis h37ra ( avirulent strain ) and 8 clinical isolates of m. tuberculosis resistant to one or more drugs , collected in the department of biomedical sciences , microbiology of the university of sassari , italy . we also studied the antimicrobial properties of the essential oil towards two human strains of mycobacterium avium subsp . the antibacterial activity of the oil was assessed by the proportional method used for mycobacterium tuberculosis as described in nccls - national committee for clinical laboratory standards . briefly , isolate suspensions of m. tuberculosis in 7h9 broth were adjusted to an optical density of 1 mcfarland , and two dilutions , 10 and 10 , were plated onto 7h10 agar with a different concentration of essential oil ( 16% , 14% , 12% , 10% , 8% , 4% , 2% , 1% , and 0.17% v / v ) . we also investigated the antimicrobial properties of some components of m. communis and , in particular , limonene , 1 - 8 cineole and -pinene , using the proportional method . in table 1 we report the data obtained with the essential oil in toto . towards all the strains of m. tuberculosis , including the extensively drug - resistant ( xdr ) , the oil of m. communis tested showed an mic of 0.17% ( v / v ) , whereas against the two m. paratuberculosis strains showed an mic of 2% ( v / v ) . subsequently we tested each different compound ( limonene , 1 - 8 cineole , -pinene ) to see if it had different antimicrobial properties . as far as limonene concerned , it showed towards all the strains an mic of 2% ( v / v ) ; about 1 - 8 cineole , for 4 strains , including h37rv , the mic was of 2% ( v / v ) , while the mic shown for other 4 strains was of 16% ( v / v ) ; -pinene showed an mic of 1% ( v / v ) for 3 strains , and for one strain an mic of 2% ( v / v ) , for 3 others an mic of 8% ( v / v ) , and for only one mic was of 16% ( v / v ) ( table 2 ) . limonene and -pinene are monoterpenic that , according to the literature , are used as expectorant , antalgic , revulsive , antitussive , mucolitic , and decongestant ; about 1 - 8 cineole it is an oxide used as expectorant , mucolitic , and decongestant . we compared the activity of our essential oil with four standard antitubercular drugs : streptomycin , isoniazid , rifampin , and ethambutol , performing according to international protocols . two clinical strains were rifampicin resistant , three strains were resistant to 2 drugs ( one streptomicin and rifampicin resistant , one streptomicin and isoniazid resistant , and one isoniazid and rifampicin resistant ) , one was resistant to 3 drugs ( streptomicin , isoniazid , and ethambutol ) . the essential oils screened in toto have a better antimicrobial activity than each single compound against all mycobacteria tested . the results presented here may contribute to the knowledge of the antimicrobial properties of myrtle and our aim is to carry on further studies . the chemical composition of the essential oil of m. communis exhibited qualitative differences that depended on different geographical areas and from the season in which the leaves were picked up . the essential oil that we used for antimicrobical in vitro assay contained a high quantity of monoterpenic and oxide that , according to literature , do not have antimicrobical activity . from our studies emerged that the essential oil in toto might have a good activity towards m. tuberculosis , although the individual compounds ( except -pinene ) showed in all the strains a higher mic . the results from the myrtle oil in toto showed a good activity towards m. tuberculosis but not toward m. paratuberculosis . the mic registered against m. tuberculosis was 0.17% ( v / v ) in comparison with an mic of 2% ( v / v ) observed toward m. paratuberculosis . a limit of our study is the small amount of the essential oil that did not allow to perform the mic towards all the strains used . testing and large clinical studies are necessary to verify the potential use of the essential oils of myrtle as antitubercular drug . given the excellent results that we obtained in this study , we would expand the research with further studies , to value the possible cytotoxic effects , and eventually to perform tests using in vivo mouse model .

mycobacterium tuberculosis is the etiological agent of tuberculosis . the world health organization has estimated that 8 million of people develop active tb every year and the situation is complicated by an increase of mycobacterium tuberculosis strains resistant to drugs used in antitubercular therapy : mdr and xdr - tb . myrtle leaf extracts , used as an antiseptic in sardinian traditional medicine , have strong antibacterial activity as several investigations showed . in this study we investigated the antimicrobial properties of the essential oil of myrtus communis against clinical strains of m. tuberculosis and m. paratuberculosis .

hepatitis b virus ( hbv ) is a partially double spiral type dna membrane virus . globally , approximately over 400 million individuals are infected with hepatitis b. hbv is known as one of the most important carcinogens . every year , over one million individuals die due to hbv - related causes . primary objective in hepatitis b treatment is to improve clinical and histological progression and to provide virus eradication . for many years , interferon , lamivudine , adefovir , telbevudine , entecavir and tenofovir the treatment with these drugs leads to normalization in liver enzymes , improvement in liver histology , hbsag and hbeag loss and undetectable hbv dna levels.[24 ] elevation of the decreased hbv dna during treatment is attributed to drug resistance or noncompliance . a 30-years - old man , a marketer by profession , had been diagnosed with hepatitis b infection for 10 years . his laboratory values were hbsag ( + ) , hbeag ( - ) , anti - hbe ( + ) , hbv - dna : 123.000.000 iu / ml , alt:160 hepatitis activity index was 13 and fibrosis score was 5 . according to those findings , entacavir 0.5 mg 11/ day was initiated . in the third month of the treatment , / l , ast was 48u / l . in the ninth month of the treatment , hbv dna levels were increased to 26,000 iu / ml , alt was 61 u / l . in this period it was observed that the patient had taken the drugs regularly in recommended dose and duration . entecavir resistance can be investigated by molecular methods . in this patient , it was investigated by determination of surface and polymerase gene mutations method . the patient was requested to inform how and under which conditions he has taken the medication and where the drugs were preserved . the patient was a marketer by profession and used to spend 2/3 part of the day in his car . to avoid missing the medication dose , the atmospheric temperature was high ( 4050c ) and therefore the drug was degenerated and lost its activity as a result of inappropriate preservation . after three months of following this recommendation , patient 's hbv dna levels were regressed to 200 iu / ml alt : 26 u / l and ast:26 u / l [ table 1 ] . in some cases treated with hepatitis b treatment even though alt level are found to be normal , resistance is considered if one log elevation is observed in hbv dna levels . hence , treated cases were recommended to be followed up with estimation of hbv dna . if one log elevation is observed in hbv dna levels , this should be confirmed by repeating the test . in those cases , if treatment compliance was found and the doses were appropriate , anti - viral resistance should be examined . in this case , we could detect neither noncompliance nor drug resistance . despite this , there were elevations in the hbv dna level and in alt , ast levels . because of the negative results of the compliance and resistance tests , alternative etiologies were searched . proper preservation of the drugs is as important as taking them in suitable doses and at appropriate time in terms of treatment outcome . according to the manufacturing firms , the drug requires to be preserved under 35c , away from excess heat , light and moisture . , heat levels inside waiting cars probably exceed the prescribed limits for preservation and therefore the drug degenerates . entecavir should not be preserved at very low or high temperature and should be avoided from exposure to moisture and direct sun light . the author therefore suggests that when the response to antiviral treatment is not obtained , patient compliance as well as drug preservation conditions should be examined , and patient should be counseled to preserve the drug at requisite temperatures at all times .

if virologic breakthrough is observed during chronic hepatitis b treatment , drug resistance or compliance problem should be considered . but in some cases , breakthrough depends on drug preservation conditions . we report the case of a 30-years - old man , who experienced viral breakthrough due to wrong preservation conditions of the drug .

nasal and pharyngeal swab specimens were collected from the dogs , and swab eluate extracts were prepared . pooled extracts were added to monolayer cultures of canine a72 cells ( american type culture collection , crl-1542 ) . after 3 passages in culture , cells in some of the flasks showed subtle cytopathic changes . after continued passage , small foci of rounded cells developed , and rapid and progressive cell death throughout the flask ensued in a pattern uncharacteristic of the viruses commonly isolated from dogs . testing these cultures with a panel of diagnostic reagents specific for common canine respiratory agents failed to identify a known virus . immunofluorescence assays ( ifas ) ultimately detected 13 positive cultures over a 4-month period when a monoclonal antibody ( mab ) pool against human respiratory syncytial virus ( rsv ) ( vp - r151 , vector laboratories , burlingame , ca , usa ) was used . the staining pattern included filamentous membrane - bound and free - floating virions and cytoplasmic inclusions , typical of the pattern in rsv - infected cells . after obtaining ifa results , we attempted to amplify a fragment of the nucleocapsid gene ( n ) from the virus by using pcr primers designed on the basis of an alignment of human , bovine , and ovine rsv sequences . stocks of the individual mabs in the anti - rsv pool and their specificities were obtained from the manufacturer and used for ifa ( figure ) . mab 2g122 ( p protein specific ) stained primarily inclusions and gave a relatively uniform membrane - associated signal . recognition of the canine virus by only 2 of 4 mabs and the inability to amplify a conserved region of the rsv genome suggested that it was related to , but unlikely to be , rsv . slides showing immunofluorescence of a72 cells with human respiratory syncytial virus monoclonal antibodies ( mabs ) . primary mab stocks were used as obtained from the manufacturer at a dilution of 1:100 . , we used a consensus - degenerate hybrid oligonucleotide primer algorithm to design degenerate pcr primers based on highly conserved amino acid sequences within multiple sequence alignments of all viruses in the subfamily pneumovirinae ( 3 ) . specific regions within the l ( polymerase ) and n genes were targeted ( table ) . sequencing of the reaction products and blast ( www.ncbi.nlm.nih.gov/blast/blast.cgi ) analysis showed the virus to be closely related to murine pneumovirus ( mpv ) , traditionally known as pneumonia virus of mice . two l - gene pcr products were found to be 96%97% identical to mpv , and an n gene fragment was 96% identical ( table ) . * mpv , murine pneumovirus ; nt , nucleotide ; aa , amino acid ; hrsv , human respiratory syncytial virus ; hmpv , human metapneumovirus ; n , nucleocapsid ; r , reverse primer ; f , forward primer l , polymerase gu247050 ; l gene fragments nos . 1 and 2 are entered under genbank accession no . mpv is 1 of only 3 virus species classified in the family paramyxoviridae , subfamily pneumovirinae , genus pneumovirus . human rsv is the type species and is closely related to bovine rsv ; mpv is more distantly related . for example , the n protein nucleotide sequences of human and bovine rsv are 94% identical to each other but only 60% identical to those of mpv . only 2 strains of mpv , j3666 and strain 15 , have been fully sequenced , and they are 99.7% identical at the nucleotide level . the nucleotide identity between virus isolated from the dogs and mpv was consistently > 95% throughout the gene regions examined . because the degenerate primers used were designed to cover regions that are generally highly conserved , regions of the genome that are typically more variable in other pneumoviruses may show greater differences between the canine pneumovirus and mpv . of the conserved regions sequenced , amino acid identities were 70% ( n ) , 55% ( l fragment no . 2 ) when aligned with the same regions of human rsv , so finding cross - reactivity between human rsv mabs and the newly isolated virus is not completely unexpected . ( 4 ) did not find recognition of mpv when they used 2 anti human rsv mabs , but they did observe recognition of mpv n protein when they used mouse anti human rsv serum in immunoblot assays . ling and pringle ( 5 ) showed cross - reactivity of n proteins with polyclonal serum and cross - reactivity with a mab that recognized p in immunoblot assays . although the mabs used in this study had been extensively tested for reactivity against a panel of 13 viruses , including several members of the family paramyxoviridae ( 6,7 ) , they were probably not tested for cross - reactivity against mpv because it is not a known human pathogen . the finding that reagents used to identify human rsv showed strong recognition of a closely related but distinct virus highlights the need for caution when interpreting research studies and conducting diagnostic evaluations . however , comparison with mpv leads to speculation that the virus isolated in this study may have pathogenic potential . mpv is commonly known to infect laboratory rodent colonies , and serologic evidence points to infection of several wild rodent species . however , little is known about mpv epidemiology , such as whether mpv has multiple natural hosts or whether closely related viruses are circulating in other species . neutralizing antibodies to mpv in other mammalian species , including humans , were first reported in the 1940s ( 8,9 ) , and more recently , high prevalence of neutralizing antibodies but low ( 3%4% ) association with clinical disease in humans has been reported ( 10 ) . sequelae to experimental infection in laboratory mice can vary from asymptomatic to severe disease with high morbidity and mortality rates . pathogenic strains , including j3666 and strain 15 , can produce severe pneumonia and death in 610 days after mouse inoculation with a low dose ( 11,12 ) . the pathogenicity or lack thereof may depend on the virus and on the mouse strain ( 13 ) . questions remain as to whether this newly isolated virus commonly infects dogs and , if so , why it has not been previously isolated . perhaps the strain that was circulating in these particular animal shelters is more easily isolated in culture . or , because the initial cytopathic changes observed with these isolates were subtle , they could easily have been missed . as anticipated , other viruses , primarily canine influenza and parainfluenza viruses , were isolated during the study , often from the same animals that carried the pneumovirus . work is ongoing to further determine pneumovirus prevalence among dogs and its involvement in acute respiratory disease of dogs .

to determine which respiratory viruses circulate among confined dogs , we analyzed nasal and pharyngeal swab specimens from shelter dogs with acute respiratory disease . an unknown virus was isolated . monoclonal antibody testing indicated that it was probably a pneumovirus . pcr and sequence analysis indicated that it was closely related to murine pneumovirus .

a 56-year - old man with a history of prior myocardial infarction and severe left ventricular dysfunction underwent an implantable cardioverter defibrillator ( icd ) implantation for primary prevention of sudden cardiac death . since the patient had normal sinus rhythm and normal atrio - ventricular conduction , a single lead , biotronik , lumax 640 vr - t dx icd was selected . a dx system consists of a single lead , which provides atrial as well as ventricular electrograms , and enhances atrial arrhythmia detection . the patient was followed - up in the clinic and via the home monitoring service . three months later , several atrial monitoring episodes , classified as atrial tachycardia ( fig . unusual high frequency artifacts were seen on the far field ( rv coil to can ) and the atrial channels , whereas the bipolar right ventricular channel was unaffected . due to the unusual combination of the far field and atrial channel artifacts , normal lead parameters ( p=2.0 mv , r=11.4 mv , rv threshold=0.6 v , and impedance=464 ) , and due to the unique high frequent crescendo decrescendo artifacts pattern lead fracture was very unlikely . the patient was telephoned immediately ; he confirmed using an electrical disk saw ( fig . however , it was unclear why the artifacts were seen on the atrial channel but not on the right ventricular . the probable explanation was that the floating atrial dipole sensor was amplified 4-times , and the programming of the atrial sensitivity was higher than the ventricular sensitivity . the relatively novel lumax dx system consists of a single lead which provides both atrial and ventricular signals . the atrial signals are sensed by a floating atrial pole , and are amplified by the lumax 640 vr - t dx device . this system along with the home monitoring was developed to decrease the risk of inappropriate icd shocks by appropriately detecting atrial arrhythmias and enabling physicians to intervene earlier . however , in the present case , electromagnetic interference was detected by the atrial lead and falsely classified as atrial arrhythmia . physicians should thus be aware of the unique dx atrial sensing mechanism , and carefully assess each of the intra - cardiac electrogram recorded by the device in order to avoid an inacurate diagnosis of a lead fracture or atrial arrhythmia .

a 56-year - old man underwent a single lead lumax 640 dx implantable cardioverter defibrillator implantation for primary prevention of sudden cardiac death . a dx system consists of a single lead , which provides atrial as well as ventricular electrograms , and enhances atrial arrhythmia detection . three months after the implantation , high - frequency episodes were detected on the far field and the atrial channels , but not on the bipolar right ventricular channel ; these were classified as atrial tachycardia . in the present report , we discussed the unusual pattern of the artifacts that was related to an electromagnetic interference detected by the novel dx system .

hypercalcemia , syndrome of inappropriate secretion of antidiuretic hormone , cushing 's syndrome , and skeletal - connective tissue syndromes are common in patients with lung cancer , especially those with small cell lung cancer ( sclc ) . approximately two thirds of sclc patients present with metastatic stage at the time of diagnosis . lambert - eaton syndrome occurs in 3%percnt ; of sclc patients as neurological paraneoplastic syndrome . however , the association of malignancy and guillain - barr syndrome ( gbs ) is not well known . it is a rare disease that occurs at a rate of 1.11 cases per 100,000 person - years . however , once infected , up to 20%percnt ; of patients develop severe disability , and approximately 5%percnt ; die . there have been reports of paraneoplastic gbs in different cancers . to the best of the present authors knowledge , there are 6 published reports of gbs in sclc [ 2 , 3 , 4 , 5 , 6 , 7 ] . here , we report a case of a 52-year - old man who was diagnosed with gbs in sclc with chemotherapy . he was diagnosed with sclc , limited disease , at another hospital 5 years ago . he had been recommended concurrent chemoradiotherapy at that time but had wanted to be treated with chemotherapy only . after 6 cycles , complete remission was noted , and prophylactic cranial irradiation was given . a year later , he had relocated residence and visited our hospital , presenting with relapsed lung cancer but refusing treatment . after 7 months , further disease progression and pancreatic metastasis was noted on computed tomography ( ct ) scanning . meanwhile , superior vena cava syndrome occurred , and radiation therapy was delivered in the middle of chemotherapy . a ct scan for the response showed disease progression . he was started on cisplatin , doxorubicin , and cyclophosphamide ( cap ) and also received palliative brain radiotherapy due to brain metastasis during the chemotherapy . however , in his fifth year of cancer diagnosis , he presented with lower - limb weakness and was admitted . he noticed weakness in both legs 2 weeks prior to admission and developed numbness throughout the lower extremities . a neurological examination revealed no nuchal rigidity or kernig 's sign as well as absence of deep tendon reflexes . magnetic resonance imaging ( mri ) of the brain showed some unidentified bright objects ( fig . however , there was little possibility of brain metastasis or embolic infarction when comparing the patient 's symptoms and physical examination with the mri lesions . to evaluate neurologic problems , it revealed a fracture of the second lumbar vertebra that seemed to be benign ( fig . , the ascending paralysis , from the lower extremities to the upper extremities , did not correlate with the cord compression lesion either . nerve conduction studies showed decreased amplitudes and slow velocities of compound muscle action potential and sensory nerve action potential in the extremities . a lumbar puncture yielded cerebrospinal fluid protein 94 mg / dl , albumin 61.3 mg / dl , glucose 173 mg / dl , and white blood cells 1/l . these were assessed as being due to albuminocytological dissociation , and there were no signs of meningitis . the patient was diagnosed with gbs and was recommended treatment with intravenous immunoglobulin ( ivig ) , which he refused . it could be caused by adverse effects of chemotherapy or by other underlying diseases that have nothing to do with malignancies , such as diabetes . however , these sensorimotor neuropathies almost always result in mild to moderate dysfunction and usually do not affect patients survival . it is characterized by both limbs and muscles innervated by cranial nerves being weakened . approximately 70%percnt ; of gbs cases a progressive bilateral , symmetric weakness of the extremities manifests over a period of 12 h to 28 days from disease onset . the nerve conduction study result was consistent with acute peripheral neuropathy , a cerebrospinal fluid analysis demonstrating an elevated protein level and absence of leukocytosis due to albuminocytological dissociation will be helpful to confirm the gbs diagnosis . gbs should take into account various diseases , such as brain or leptomeningeal metastasis from cancer , and spinal cord compression . described neuropathy occurring after treatment with a combination of oxaliplatin and cisplatin ; nardone et al . in addition , when patients with gbs are not able to walk independently , immunotherapy is required . in immunotherapy , either ivig or plasmapheresis can be used . these treatments have been observed to be effective when started within the first 2 weeks after disease onset . gbs occurred in a patient receiving long - term antineoplastic therapy , whereas gbs had occurred at the initial point of presentation in the 6 other cases [ 2 , 3 , 4 , 5 , 6 , 7 ] . this is the first reported case of paraneoplastic gbs that developed in an sclc patient with long - term therapy . to the present authors knowledge , in the 5 cases of gbs with sclc , there was no relationship between chemotherapy and gbs . in 4 of the 5 reports , patients received chemotherapy ; however , in all 4 of these reports , the patients underwent chemotherapy after gbs had been diagnosed and ivig had been given . in addition , in 3 of these 4 reports , a combination of etoposide and carboplatin was used , whereas the other report did not indicate the chemotherapy regimen [ 2 , 3 , 4 , 5 , 6 ] . immunotherapy was recommended at the time of diagnosis , but he refused the treatment because of his advanced cancer stage and poor general condition . patients with cancer are vulnerable to infection and can have problems with the immune system . as illustrated by this report , paraneoplastic gbs in sclc is a life - threatening disease . thus , it should be monitored and treated when acute progressive peripheral neuropathy occurs in patients with sclc . the authors submitted a waiver from their institutional review board stating that this case report does not require institutional review board approval or oversight .

guillain - barr syndrome ( gbs ) is defined as an acute , autoimmune polyradiculoneuropathy . it is a rare disease that occurs at a rate of 1.11 cases per 100,000 person - years . however , once infected , up to 20%percnt ; of patients develop severe disability , and approximately 5%percnt ; die . there have been reports of gbs in different cancers . among them , there are 6 previous reports of gbs in small cell lung cancer . here , we report a case of a 52-year - old man who was diagnosed with gbs in the setting of small cell lung cancer with chemotherapy .

a 48-year - old man presented with visual dimness in his right eye , which had developed over the two weeks prior to the visit to the ophthalmology clinic . his medical , family , and social history was unremarkable . his visual acuity ( va ) was 20/50 in the right eye ( od ) and 20/20 in the left eye ( os ) . dilated fundus examination showed few vitreous cells and numerous yellow , placoid lesions in both eyes ( ou ) ( fig . 1 a and b ) . fluorescein angiography demonstrated early irregular hypofluorescence with late staining in the areas of the yellow placoid lesions ( fig . , va decreased to 20/30 and yellow , placoid lesions increased to involve the macula . he was started on a regimen of 60 mg ( 1.2 mg/ kg of his body weight ) of prednisone daily . one week after prednisone treatment , his right va increased to 20/30 and the retinal lesions improved . however , the left va deteriorated to 20/40 , and the placoid lesions were aggravated . we discovered that the patient did not follow our recommendation for serologic work - up . the serologic tests were ordered again , and his blood sample was sent for laboratory work - up . prednisone was tapered down to 50 mg daily , and he returned 1 week later with improved va of 20/20 and completely resolved chorioretinal lesions in the od . serologic testing was positive for venereal disease research laboratory antigen and negative for hiv titers . he had no other syphilitic manifestations . in order to avoid a possible rebound reaction to prednisone withdrawal , prednisone was rapidly tapered to 30 mg daily , and the patient was referred to an infectious disease specialist for antibiotic therapy . he returned 1 week later with improved va of 20/20 in the od and 20/30 in the os . a serologic fluorescent treponemal antigen absorption test was positive for ig g and ig m. however , the patient was not taking any antibiotics yet , because he refused to follow the internist 's recommendation of admission to the hospital and commencement of an antibiotic prescription . prednisone was tapered to 10 mg , and he was sent for medical consultation again . however , new multiple focal chorioretinal lesions were noted outside the inferior retinal vascular arcades of the os . after medical examination , including a cerebrospinal fluid ( csf ) study , he was started on ceftriaxone , 2.0 g intravenously for 14 days , and returned with resolution of the focal chorioretinitis . the most common posterior segment involvement of ocular syphilis is chorioretinitis.3 to denote a variation of posterior segment involvement , gass1 coined the term " syphilitic posterior placoid chorioretinitis " . the findings show large , placoid , yellowish lesions with faded centers at the level of the retinal pigment epithelium in the macular and juxtapapillary areas , which show similar angiographic patterns , specifically early hypofluorescence and late staining . it has been supposed that this particular finding might be a direct result of the modification of the host immune response to syphilis by the hiv virus . tran et al.4 suggested that syphilitic uveitis in hiv - infected patients seems to have a more severe course . moreover , zamani and garfinkel2 reported the relationship between oral prednisone and the appearance of asppc . in their case , they suggested that its clinical appearance might be modulated by the immune status of the patient and that if corticosteroids are given without systemic treatment for syphilitic manifestations at the same time or shortly thereafter , the number of treponemes may increase and new lesions may appear . however , the results from our case do not support their suggestions . in our case , the patient took only oral prednisone without simultaneous coverage by the proper antibiotics , because he refused to follow the internist 's recommendation . at initial presentation , his right eye had improved during the prednisone therapy , while the left eye was aggravated . this implies that his chorioretinitis had a clinical course unresponsive to the use of prednisone . instead , its clinical course was more like that of apmppe . chorioretinitis recurred after tapering the dosage of prednisone but was resolved after treatment with antibiotics . however , chorioretinitis at different stages had the same clinical course ( onset - aggravation - resolution ) , regardless of treatment with prednisone . it suggests that asppc has its own clinical course and is not influenced by systemic steroid use . another possibility is that the anti - inflammatory effect of prednisone had appeared late in treatment because the chorioretinitis of both eyes resolved at the end of the prednisone treatment . however , the latter is less likely because one eye improved and the other was aggravated during the same treatment period . our case had an atypical course for this disease , and it was not consistent with the hypothesis that a particular fundus finding of asppc may be the direct result of a patient 's altered immune response to syphilis , especially induced by corticosteroid therapy started before the initiation of antimicrobial therapy .

a 48-year - old man presented with visual dimness in the right eye that had developed 2 weeks previously . dilated fundus examination showed few vitreous cells and numerous yellow , placoid lesions in both eyes . his right eye had more severe serous retinal detachment involving the macula . fluorescein angiography demonstrated early irregular hypofluorescence with late staining in the areas of the yellow placoid lesions . he started a regimen of 60 mg of oral prednisone daily . two weeks later , a serologic fluorescent treponemal antigen absorption test was positive for ig g and ig m. he was referred to an infectious disease specialist for antibiotic therapy . a week later , he returned , having stayed on prednisone only and not having taken the internist 's antibiotic prescription . meanwhile , the chorioretinitis in his right eye , which had initially been at a more advanced stage , was resolved with the use of steroids . the chorioretinitis in his left eye , which was aggravated at an earlier stage , ultimately recovered . our case had atypical courses such that one eye improved and the other worsened during the same steroid treatment period . this result was inconsistent with that of previous reports showing that oral steroid influences the clinical course of acute syphilitic posterior placoid chorioretinitis .

there are many poets and writers in persian literature whom are called scientist ( 1 - 10 ) . it is because they knew all current knowledge such as medicine , mathematics , and literature ( 11 - 18 ) . we try to find this feature and illustrate it to the young physician . each poet such as ghanjavi has poems about medicine and religious . physician s role and position in the practice of islam physicians in the islamic society are valuable and prestigious profession of religion and those figures of speech that are involved in this business have been commendable . the three who are : scholar , the reign of amir or submit to his command , trusted medicine . the reign of amir or submit to his command , the narratives of infallible imams contents of these can be seen . three eyes that did not burn in the fire : an eye for protecting the borders of the islamic nation . relationship with god , and effectiveness of physicians in the diagnosis and treatment of patients trust on god the story of king and slave a physician could not cure slave disease but king depend on god and slave get her health . if doctor trust on god in diagnosis , they become success . in kharazmshahi , writer recommend trust on god to the doctors confirmation of the divine friendly doctor should be allowed by god to guide the treatment of incurable diseases . they should know , if god help , they can diagnose disease . having a good faith physician must have faith to the god commands . doctors should have a firm belief to god rules . once upon a time , emam abobekr daghag wanted to treat patient . after 3 ays he could not , he read fatehat- ol- ketab and could treat him . he tried each drugs , he understood he had a bad relation with his father . doctors communicate with others and the importance of trust in fellow patient cure she is very precious to god and trust in the entourage is recommended when patients go to visit . integrity and honesty respect the integrity and honesty in all good , but in the medical profession is very important . clinical studies have shown the presence of the patient , to love him ; underestimated disease health hypnosis is very effective in the recovery of patients . visiting example of prophet muhammad in order to appease the spirit and enhance the muslims who had been sick in bed . imam said muslim : your illness has benefits for you - a ) think about god ; b ) prayer is answered at this time ; c ) your disease atone sins . persian grandee such as maulana said doctor is a grand person that tries to cure sick people . a good doctor is a person that knows everything about man and tries curing mental and physical disease . our past worthiness about our medical science history is reason for this claim many poets and writers were doctors too . in persian textbook we have some subject about patient right and rules of doctors treatment . matching medical treatment with prayer shows main source treatment and cause curing sooner and better . combing medical science with ethic shows that this subject is very important in profession ( 8 - 19 ) . if a doctor accept medical ethic , he will be more successful . some subjects that comes in the affidavit such as secrecy , shows their importance for doctors , governors and people . now we have medical ethic major in shari beheshti university . knowing persian literature can help young doctors .

content of persian literature comes from innocents , has special effect on social and individual people life . we understand worth of medical science , physician and sick people relationship in society and impact of religion behavior in treatment . persian literature contains some recommendation that improve belief , trust , and secretary in medical society . in this article we chose some persian literature about medicinal behavior and discuss trough : physician relationship with god and its impact on treatment , and sick people with others . we hope by studying this article physician can work better and sick people obey their orders more .

it refers to abnormal hearing of sound heard during metabolism and not from an external sound source . the most common cause of vibratory tinnitus is intracranial vascular disease , such as aneurysm and arteriovenous malformation.1 ) the authors performed excisional biopsy on pulsatile lip masses of 46 year old male . a 46 year old male patient experienced vibratory tinnitus on the right side for 10 years . he had pulsatile mass on the right side of his lip and did not undergo any treatment . since 1 month before diagnosis , the level of tinnitus increased along with the size of the mass . upon physical examination , there were no symptoms of tenderness , but 2 pulsatile masses were found on the right upper and right lateral side lip . the size of the former mass was 11 cm and the latter was 1.51.0 cm ( fig . 1 ) . there were no other special abnormalities in the oral cavity , oropharynx and the other head and neck area . the tinnitus decreased when pressure was applied to the left tympanic membrane area and increased when leaning forward . the tympanic membrane was in sound condition and hearing tests revealed both ears to be normal . the frequency was 2 khz , and loudness was between 60 and 70 decibels and there were no residual inhibitions . arteriovenous malformation from the right facial artery was found resulting from computed tomography of the facial bone ( fig . 2 ) . ct with angiography was not conducted due to hypotension and tachycardia after the injection of the contrast media . it was diagnosed as resulting from an abnormality in vessel and excisional biopsy was conducted through oral access after general anesthesia ( fig . operation revealed the superior labial artery and facial artery after incising the oral mucosa in the mass and the area showing evidence of vascular malformation was ligated . the patient did not complain of any tinnitus symptoms after the operation and shows no sign of recurrence up until now , 1 year after the operation . tinnitus can be classified as objective or subjective tinnitus , depending on whether the examiner can hear the sound that the patient is complaining about . from a radiologic point of view non - vascular causes include the abnormal patency of the eustachian tube , temporomandibular joint disorder , arteriovenous anastomosis , myoclonus of the oropharyngeal , stapedius , and tensor tympani muscles.2 ) depending on the type of condition , pulsatile tinnitus shows varying levels of strength , duration and the size of the tinnitus , all of which are characteristically in line with the regular rhythm of the patient 's heart beat . sometimes true pulsatile and pseudo - pulsatile tinnitus is categorized by whether the tinnitus is in line with the rhythmic beating of the heart . patients diagnosed with objective tinnitus mostly show vascular disease representing a 7.6% of all tinnitus patients.3 ) finding the cause of the tinnitus raises the possibility of cure , and tinnitus can sometimes be the tell - tale sign of a more serious condition . clinical symptoms differ depending on the location of the lesion and the structure of the anatomy . if the lesion is close to the skull base or scalp vessel , symptoms such as pulsatile mass , palpable thrill or murmur may show and pulsatile tinnitus.4,5 ) the possible venous cause for pulsatile tinnitus is benign intracranial hypertension and intracranial venous malformation . benign intracranial hypertension usually shows in young obese females , and symptoms include pulsatile tinnitus , headaches , blurred vision among other non - characteristic symptoms . otologic symptoms , such as earfullness , low frequency hearing difficulty and dizziness may also follow . symptoms worsen during situations where the cranial blood pressure increases , such as coughing and leaning forwards and thus , are at its peak during the morning . pulsatile tinnitus arising from venous malformation can be due to sigmoid sinus diverticulum , jugular bulb diverticulum , dehiscent high jugular bulb , dehiscent sigmoid sinus and abnormal emissary vein . patients with pulsatile tinnitus must be examined thoroughly , including neurological and radiological examination , along with physical examination and past history . in cases with vessel malformation other neoplasms include middle ear adenoma , hemangioma , hemangiopericytoma , plasmacytoma , giant cell tumor , neuroendocrine carcinoma , and etc . in this example , the location of the lesion was relatively easier to access , and due to the abnormal reaction , the ct - angiography was not conducted . for a radiological examination of pulsatile tinnitus , enhanced temporal bone - hrct , enhanced brain ct , ct angiography , mr angiography / venography , four - vessel angiography and the contrast enhancement are used to observe the head and neck vessel in detail . this is because the diagnostic sensitivity and the specificity of each method differ , and as there is no specified standard in determining the level of the finding to be considered abnormal . however , to propose some rules to the method of examination , firstly , a method utilizing the contrast enhancement must be used , and secondly , a method checking the abnormalities in angiography in the cervical and head , not just in the temporal bone , must be used . thirdly , if there are past history in the head trauma and there is a possibility of an arterial malformation and no abnormalities are found upon initial exam , four - vessel angiography must be considered.6 ) external and internal access was considered before the surgery , however due to the young age of the patient and for cosmetic reasons , the area was accessed through the mouth . it is considered that this instance of tinnitus arising from the facial vessel is very abnormal . the authors successfully cured tinnitus after removal of arteriovenous malformation near the lips and have compiled the findings in this thesis .

tinnitus is one of the most common symptoms in an audiologic field . it can be classified as either as subjective or objective ; former referring to the sensation heard by both patient and examiner . pulsatile tinnitus is perceived as sounds that vary in frequency , intensity and duration . the cause of pulsatile tinnitus include high jugular bulb , benign intracranial hypertension , glomus tumors , carotid artery stenosis , vascular lesions of the temporal bone , arteriovenous malformation , aneurysms , and arnold - chiari malformation . vascular tinnitus is most common ( 7.6% ) . recently , the authors experienced one case of the pulsatile tinnitus caused by atherosclerosis and atheroma in superior labial artery & facial artery . after surgery , the symptom had disappeared . we report a unique case regarding the objective tinnitus with the literature review .

after myxomas and lipomas , cardiac papillary fibroelastomas ( cpf ) are the most common benign primary tumors of the heart ( 7.9%).1 ) pathologically , these tumors grossly appear like a " sea anemone " , because they consist of multiple frond - like projections . histologically , they are avascular tumors derived from normal components of the endocardium.2 ) cpf are usually discovered incidentally during a routine transthoracic echocardiography ( tte ) . clinical neurologic , embolic and coronary ischemic symptoms have been reported in association with cpf . surgical excision of the tumor is recommended for all patients who develop symptoms , but the treatment of asymptomatic patients with and echocardiographically identified cpf is still controversial.3)4 ) we present a case of multiple cpf in left ventricle and aortic valve with the left atrial thrombus , coincidentally discovered on echocardiography , and removed by a surgical resection . a 68-year - old male with hypertension and atrial fibrillation , was admitted with progressive worsening dyspnea and chest pain . physical examination at the time of the first admission revealed a temperature of 36.5 ; respiration 20/min ; pulse , 100/min and irregular ; and blood pressure , 120/80 mmhg . tte revealed a large left atrium of 7.22 cm diameter , severe mitral stenosis , mild mitral regurgitation , moderate aortic regurgitation , and presence of a multiple oscillating variable sized masses in the left ventricle and aortic valve , non - mobile 3.5 4.4 cm sized mass in the left atrium . the masses in the left ventricle were 0.31 0.92 cm , and 0.54 0.98 cm in size , oscillating heterogeneous echogenic material attached to the interventricular septum basal to mid level and 1.46 1.64 cm , 0.47 1.07 cm in size , mobile oval shaped mass , which had some echolucent area attached to the posterolateral papillary muscle and aortic valve ( fig . 1 ) . the mass of the left atrium was 3.5 4.4 cm in size , non - mobile echogenic mass in the left atrium posterior wall . cardiac magnetic resonance imaging demonstrated non - enhanced masses in the left atrium between the orifice of the right superior and inferior pulmonary vein and ventricle , which were heterogeneous in its signal intensity in t2 image ( fig . . based upon the findings as above , a differential diagnosis was made , which included thrombus , myxoma , fibroelastoma and inflammatory mass . in view of the possibility of embolism , unknown nature of the pathology and multiple valve diseases with symptoms , histopathology examination of the resected masses in the left ventricle and aortic valve revealed a papillary proliferation , including an avascular connective tissue core lined by a single layer of the endothelial cells , which was sufficient for a diagnosis of cpf ( fig . the left atrial mass was composed of fibrin and red cells with a variable platelet and leukocyte component , revealed to thrombus . the postoperative course was uncomplicated and the patient was discharged in a satisfactory condition on the 12th day . cpf predominate in adults and are particularly frequent between the 4th and 8th decades of life . most cases are probably acquired , however the etiology is unknown.5 ) cpf are more frequently located on the aortic valve ( 40% ) , tricuspid valve ( 17% ) , mitral valve ( 14% ) , pulmonary valve ( 13% ) , left atrium ( 7% ) , right atrium ( 2% ) , right ventricle ( 2% ) , and left ventricle papillary muscle ( 1%).6 ) left ventricular cpf is rare , only reported via case reports.7 ) although it is found incidentally , it can result in life - threatening complications , such as coronary and cerebral embolism , acute valvular dysfunction and sudden death.8 ) the most common clinical presentations are stroke , syncope , mesenteric ischemia , pulmonary emboli and sudden death.5 ) the clinical presentation is determined by location , size , and mobility of the tumor and when they arise from the left sided heart , systemic embolism is frequent . the treatment of choice of cpf is surgical excision , which is safe without causing significant morbidity or mortality . when valvular involvement is present , excision with valve repair or replacement is curative . asymptomatic non - mobile or right side cpf could be followed - up closely.4)5 ) the diagnostic method of choice for cpf is tte or transesophageal echocardiography ( tee ) , although the ultimate diagnosis of cpf is based on histopathology . the most characteristic echocardiographic features that identify a tumor as a cpf are small size ( usually < 1.5 cm ) , pedical or stalk attachment to endocardium , with high mobility , and refractive appearance and areas of echolucency within the tumor.2 ) although no extensive studies have yet quantified the diagnostic yield of tee for cpf compared with tte , tee is considered to be more accurate in diagnosing cpf . for tumors with a diameter < 0.2 cm , the sensitivity of tte was only 61.9% and of tee was 76.6% . in contrast , the sensitivity and specificity of tte for cpf with a diameter > 0.2 cm are 88.9% and 87.8% , respectively.9 ) however , it is impossible to differentiate cpf from myxomas or thrombi , using tte or tee alone . magnetic resonance imaging ( mri ) may be more helpful than tee in detecting the extent of the lesion invading the myocardium . mri typically demonstrates a cpf mass on a valve leaflet or on the endocardial surface of the affected cardiac chamber and increase accuracy of diagnosis by showing the differential enhacement with respect to the surrounding normal cardiac structures.10 ) histopathologically , cpf are composed of a central stalk with radiating villus - like projections . the papillae are avascular structures , which contains a core of dense collagen fibers admixed with varying amounts of reticulin and elastin fibers . the cells lining the elongated papillae are hyperplastic endothelial cells , occasionally bulging from the surface.11 ) the lining epithelium is contiguous with the rest of endocardium . this case demonstrates a surgical management of cpf and thrombus , diagnosed by echocardiography and cardiac mri . this case of cpf is unusual with respect to the site of origin showing multiple involvement including the papillary muscle ( its prevalence in the literature is only about 1% in cpf ) and the large amount of thrombus in the left atrium .

cardiac papillary fibroelastomas ( cpf ) are benign cardiac tumors and usually discovered incidentally during echocardiography . this report describes the case of a 68-year - old man , referred to cardiology for multiple masses of the left ventricle and left atrium . the transthoracic echocardiography revealed multiple oscillating masses in the left ventricle and aortic valve , non - mobile mass in the left atrium with severe mitral stenosis and moderate aortic regurgitation . the patient underwent surgical resection of the masses with valve replacements . histopathologic examination confirmed the diagnosis of cpf in the left ventricle and aortic valve , thrombus in the left atrium .

case 1 : a 31-year - old woman , with a gestational history of g9 , p1 , ab8 and l1 was admitted to mirza koochak khan hospital , tehran , iran in june 2001 with the symptoms and signs of acute abdomen . she had 5 years secondary infertility of unknown reason following her last abortion and gave the history of a missed menstrual period as well as 50 days of spotting . no abnormal finding was detected in uterus . in view of an adnexal mass along with positive pregnancy test , a possibility of ectopic pregnancy was considered . gross examination of right ovary revealed a 774.5 cm mass with a hemorrhagic cut surface . following thorough sampling of the ovarian mass ( 1 section per cm of the greatest dimension of the tumor ) , microscopic examination revealed characteristic histological features of choriocarcinoma , composed of bilami - nar structures of cytotrophoblasts and syncyti - otrophoblasts with severe nuclear atypia and mitotic figures admixed with necrotic tissue . a corpus luteum of pregnancy ( figure 1 ) with multiple colloid bodies and calcifications was also observed ( figures 2 and 3).the patient received etoposide/ metotrexate / actinomycin d/ cisplatin/ etoposide ( ema - ce ) regimen for treatment and serum -hcg level decreased to 70 miu / ml . after the surgery , she took 4 courses of chemotherapy with ema - ce regimen . corpus luteum of pregnancy adjacent to the tumor ( 40 x ) calcifications in the corpus luteum of pregnancy ( 40 x ) hyaline droplets in the corpus luteum of pregnancy ( 400 x ) case 2 : a 32-year - old woman with a gestational history of g3 , p2 , ab1 and l2 was admitted to mirza koochak khan hospital , tehran , iran in september 2003 with nausea , vomiting and vaginal spotting . serum -hcg level was 5500 miu / ml and afp level was within the normal range . a large necrotic mass in left ovary with extension to the posterior aspect of uterus was identified in laparotomy . total abdominal hysterectomy and bilateral salpingo - oophorectomy , tumor debulkation and infracolic omentectomy were performed . on gross examination , there was a left ovarian mass measuring 131110 cm with lobulated and hemorrhagic - necrotic cut surface . microscopic examination of the tumor demonstrated clusters of atypical cytotrophoblasts and syncytiotrophoblasts with mitotic figures ( figure 4 ) as well as a corpus luteum of pregnancy . no tumoral involvement was identified in endomyometrium . computed tomography of chest , abdomen and brain showed no abnormal findings . since the initial impression of the clinician was more in favor of nongestational choriocarcinoma , the patient at first received 3 courses of bleomycin/ etoposide/ platinum ( bep ) regimen for 3 months . after 4 courses of new treatment , the serum -hcg level reached to less than 5 miu / ml . choriocarcinoma of the ovary is a rare and aggressive tumor arising from germ cells , constituting less than 5% of all ovarian malignancies in western countries.1 ovarian choriocarcinoma can be divided into three groups : as a metastatic gestational choriocarcinoma due to a regressed or occult primary gestational choriocarcinoma in other parts of the genital tract ( mostly uterine corpus ) ; as a primary gestational choriocarcinoma arising from an ectopic ovarian pregnancy , and as a non - gestational germ cell tumor differentiating to trophoblastic components.2 all types secret -hcg . however , -hcg levels are usually lower in nongestational variants in comparison to gestational types.7 monitoring of serum -hcg can be useful method in evaluating response to therapy . serum -hcg elevation leads to isosexsual pseudopuberty in premenarchal patients , while patients in reproductive age usually present with menstrual abnormalities ( mainly amenorrhea).1 pure nongestational ovarian choriocarcinomas are extremely rare and highly malignant tumors frequently metastasizing through the lymphatics with intra - abdominal spread.89 non - gestational type choriocarcinoma involves the patients with average age of 13 and is largely confined to females under 20 . the presence of a well developed corpus luteum of pregnancy adjacent to the tumor may be indicative of a gestational origin.8 however , a search for paternal dna in tumor allows a definite distinction between gestational and nongestational types . tumors with gestational origin have paternal genomic structure while nongestational tumors have genomes of only maternal origin without any alleles from paternal origin.2 since dna polymorphism analysis is not a generally available laboratory technique , we could n't perform it on our cases . gestational ovarian choriocarcinomas have a better prognosis than their nongestational counterparts.1011 however some studies suggest that surgical stage of pure ovarian choriocarcinoma may be more important determinant of clinical outcome compared to being of gestational or nongestational type.12 the two presented patients were in the forth decade of life and showed no elements of other germ cell tumors but well developed corpus luteum of pregnancy with hyaline globules and calcifications adjacent to tumor . however the possibility of pronounced corpus luteum due to high levels of -hcg can not be excluded . ze and fy did the clinical diagnosis , treatment and follow - up of patients .

pure primary ovarian choriocarcinoma is an extremely rare condition of gestational or nongestational origin . the possibility of gestational origin can be suspected by the presence of a corpus luteum of pregnancy but definite diagnosis would be based on genetic analysis . here , we present two cases of pure ovarian choriocarcinoma in the forth decade of life with the possibility of gestational origin .

traumatic cerebral aneurysms with carotid cavernous fistula ( ccf ) are rare and difficult to treat by surgery . if left untreated mortality rate of 50% is reported in these cases . hence , the presence of these lesions should be diagnosed and treated at the earliest in order to prevent the untoward effects due to their poor prognosis . a 40-year - old man presented to the emergency room following a road traffic accident . computed tomography scan of the brain ( plain ) revealed frontal hematoma , intraventricular hemorrhage , and subarachnoid hemorrhage with depressed skull fracture . on the 4 day of hospitalization , he developed increasing proptosis and conjunctival congestion [ figure 1a ] . magnetic resonance ( mr ) imaging showed hyperintense signals in the basal cisterns suggestive of subarachnoid hemorrhage on t1-weighted sequence . time of flight mr angiogram maximum intensity projection image showed abnormal communication between internal carotid artery and cavernous sinus . however , the precise location of the rent was not identified [ figure 1b and c ] . digital subtraction angiogram of the left internal carotid artery showed intradural aneurysm ( caused by the trauma ) , which was seen communicating inferiorly with cavernous sinus , basillar plexus , and petrosal sinus [ figure 2a and b ] . balloon occlusion test was not tolerated and hence we chose to occlude the aneurysm and the fistulous communication ( preserving the parent artery ) over parent vessel occlusion . 40-year - old male accident victim with skull fracture presented with increasing left - sided proptosis which was diagnosed as carotid cavernous fistula due to rupture of intradural aneurysm . ( b ) t1-weighted magnetic resonance ( mr ) imaging done shows hyperintensity in the basal cisterns suggestive of subarachnoid hemorrhage ( arrow ) . ( c ) time of flight mr angiogram shows the fistulous communication on the left side ( arrow ) . digital subtraction angiograms of left internal carotid artery in the ( a ) left oblique and ( b ) right oblique views show intradural aneurysm ( white arrow ) and the fistulous communication into the cavernous sinus and petrosal sinus ( black arrows in a ) . ( c ) road map image with stable position of the micro catheter achieved distally with gentle maneuver ( arrow ) . under general anesthesia , 6 french guiding catheter was placed in the left internal carotid artery . a gentle steam shaping of the micro catheter tip was done to facilitate the navigation of the distal end of the micro catheter into the sinus [ figure 2c ] . selective micro - catheter angiogram showed high flow through the aneurysm into the cavernous sinus and inferior petrosal sinus . after achieving stable position of the micro - catheter distally , thereafter , the aneurysm was completely packed with multiple coils and complete occlusion was achieved with no flow into the fistulous communication . post - coiling angiogram showed complete obliteration of the fistula and exclusion of the aneurysm from the circulation [ figure 3a c ] . long - term follow - up to assess the coil compaction and recanalization is waiting to be done . post - coiling left internal carotid angiogram in ( a ) right oblique and ( b ) left oblique projections show complete occlusion of the aneurysm and the fistulous communication ( arrows ) . ( c ) townes view ( arrow ) shows the patent internal carotid artery with good flow in all the distal branches and no remnant fistula . a recent history of trauma with skull base fracture and presentation of ccf with no clear aneurysm and vasospasm on angiogram confirms the diagnosis of the traumatic cerebral aneurysm . in the present case , the traumatic internal carotid artery injury caused pseudoaneurysm dilatation , which ruptured into the venous sinus and caused the presentation of ccf with an opthalmological manifestation . supraclinoid segment aneurysms presenting as ccf is unusual and very few reports of these intradural aneurysms are known . this entity is difficult to access surgically and the presence of the fragile wall of the aneurysm increases the risk of complications during surgery . , described the use of coil occlusion in treating these aneurysms . today , the treatment of choice for direct ccf is balloon occlusion of the rent of the internal carotid artery . this treatment option could not be used in the present case , since the inflated balloon could cause rupture of the aneurysm and worsen the condition of the patient . parent vessel occlusion could not be done due to the distal aneurysm and also because of the absence of cross - flow from the right internal carotid artery . selective exclusion of the fistula and the aneurysm while preserving the parent artery is the treatment of choice in this type of pathology . due to high flow from the aneurysm into the fistula , initially this communication needs to be occluded , for which a stable positioning of the micro - catheter in the sinus is required . cho et al . , in their study have reported a case of the traumatic supraclinoid aneurysm with ccf where aneurysm with fistula was partially occluded with coils and glue was used in the second session to cure the remnant fistula . zhao et al . , reported a similar case which was treated by endovascular coils and onyx embolization . in their case , they first coiled the aneurysm in spite of which there was continuous opacification of the cavernous sinus . hence , they used onyx 34 to occlude the residual fistula in the same session . in our case , we initially placed the pre - shaped micro catheter distally into the communicating portion and completely packed the same with micro coils and then coiled the aneurysm after obliterating the fistula . hence , we decided not to use any liquid embolic agents and only coils were placed to obliterate both the fistula and the aneurysm . one should be extremely careful while negotiating the micro catheter through the traumatic aneurysm into the fistulous site to prevent the complication of hemorrhage due to the rupture of the aneurysm . endovascular treatment is the treatment of choice in such cases and should be considered as an emergency procedure due to the high mortality rates encountered in these cases .

carotid cavernous fistulae ( ccf ) are abnormal communication between cavernous segment of the internal carotid artery and cavernous sinus . these entities are usually encountered in 0.2 - 0.8% of patients with traumatic skull base fractures . traumatic cerebral aneurysms are rare and account for less than 1% of intracranial aneurysms . ccf due to ruptured intradural traumatic aneurysm is very rare and difficult to treat by surgical methods . we present one such case of a 40-year - old man with post - traumatic ccf due to a ruptured intradural aneurysm successfully treated with endovascular embolization .

aquired progressive kinking of the hair ( apkh ) and whisker hair are relatively rare conditions . to our knowledge , this blond , 23-year - old man referred that the hair around his ears and occipital area had turned darker , shorter , and curlier during the last 3 years . he also mentioned that the hair in that area was more scarce and fragile and was rather concerned about it . he had not taken any medications in the previous years nor had undergone any cosmetic procedures . clinical examination showed his normal blond , straight long hair on the frontal and parietal areas of the scalp , whereas the hair on temporal and occipital areas was brown , lusterless , short , and curly [ figures 1 and 2 ] . curly , dark , and short hair on the temporal areas in contrast to the patient 's normal , blond , straight hair on the parietal and frontal areas . right temporal area curly , dark , and short hair on the temporal areas in contrast to the patient 's normal , blond , straight hair on the parietal and frontal areas . left temporal area apkh presents in young adults or children who show a change in texture and shape of the hair mainly on their frontotemporal areas . he reported a case of a patient with acquired short , curly hair , similar to that of beard on temporal and occipital areas . whisker hair was included as a subtype of apkh by mortimer et al . and tosti et al . differential diagnosis includes , on the one hand , familial woolly hair syndromes , woolly hair nevus and uncombable hair syndrome which are congenital and , on the other hand , diffuse partial woolly hair which is acquired . in the latter , cases of apkh and whisker hair in males have shown to be related to rapidly progressive androgenetic alopecia . even if five of them were under treatment with topical minoxidil , developed androgenetic alopecia along the follow - up period ( mean 4.5 years ) . higher concentrations of dihydrotestosterone in the scalp as well as miniaturization of the hair follicle and a decreased anagen to telogen ratio have been reported in these patients . moreover , norwood proposed that patients with whisker hair might not only develop normal androgenetic alopecia but would also loose the hair around his ears and occipital area , where the curly hair was previously . as in our case , patients with these conditions might suffer from psychological stress secondary to the change in the appearance of their hair . therefore , it is important that dermatologist recognizes these entities so that they can warn the patients about the higher risk of developing androgenetic alopecia . at the time of the report of most of the cases of apkh and whisker hair , currently , finasteride 1 mg is considered an effective drug for preventing androgenetic alopecia , and patients suffering from apkh / whisker hair should be informed about it .

acquired progressive kinking of the hair ( apkh ) and whisker hair are relatively rare conditions . to our knowledge , fewer than 25 cases have been reported in the english literature . we present the case of a 23-year - old man whose hair on the parietal and occipital areas changed and turned curlier and shorter . patients suffering from apkh have higher risk of developing androgenetic alopecia and therefore finasteride 1 mg daily is proposed as an adequate treatment for these patients .

a problem that has frequently emerged as a critical issue in patients with venoarterial ( va ) extracorporeal membranous oxygenation ( ecmo ) is a dilatation of the left heart due to volume overload of the left ventricle . conventionally , a venting cannula is placed in the left atrium via the right upper pulmonary vein or the la auricle with a sternotomy or a lateral thoracotomy . however , these approaches are risky because of significant complications such as bleeding and scarring . the procedure chosen for percutaneous left heart decompression varies from insertion of the la venting cannula to placement of an atrial septal stent . recent reports showed that there was no significant difference between these procedures . here , we describe a successful percutaneous balloon atrial septostomy and an la venting cannula insertion during va ecmo in a patient with severe acute myocarditis . a 38-month - old , 11.7 kg boy he had suffered from intraventricular hemorrhage and intracranial hemorrhage caused by neonatal asphyxia , and he had been kept on antiepileptic medication due to infantile spasm . the patient was transferred to the intensive care unit with very poor hemodynamics and subsequent supraventricular tachycardia ( heart rate > 200/min ) , which did not respond to intravenous amiodarone or adenosine . despite conventional therapy including inotropes , his condition continued to deteriorate with tachycardia . echocardiography revealed a marked decrease of 20% in the ejection fraction , and we therefore decided to support him with va ecmo . arterial cannulation was performed via the right carotid artery with an 8 fr percutaneous arterial cannula ( rmi ; edwards lifesciences , irvine , ca , usa ) , and a 14 fr percutaneous venous cannula ( rmi , edwards lifesciences ) was placed in the right internal jugular vein . ecmo flow was initially 1,300 ml / min and was maintained between 700 to 800 ml / min . after ecmo insertion , the patient s vital signs stabilized and his chest x - ray improved ( fig . however , he began to produce a large amount of frothy , bloody endotracheal secretions and his pulse pressure disappeared twelve hours later . follow - up echocardiography revealed marked left heart distention and a left ventricular ejection fraction lower than 10% . therefore , fourteen hours after ecmo insertion , the patient was taken to the cardiac catheter laboratory for an atrial balloon septostomy and la venting cannula insertion . the procedure was accomplished with a percutaneous approach through the right femoral vein without complications and an 8.5 fr la venting cannula ( mullins sheath ; cook inc . 2 ) . after placement of the la venting cannula , the pulse pressure appeared again , with a 15 mmhg gap between the systolic and diastolic pressure . his follow - up chest x - ray improved ( fig . four days later , we observed recovery of left ventricular function and improvement of the chest x - ray ( fig . follow - up echocardiography showed improved lv systolic function and an ejection fraction of 65% . since its introduction as a major support for severe respiratory distress , ecmo has become a well - established therapy in pediatric patients , particularly those with severe but reversible neonatal respiratory failure , cardiac failure , or cardiorespiratory failure . in particular , va ecmo has been used in pediatric patients with cardiac dysfunction , such as myocarditis , cardiac arrest , and cardiomyopathy . left heart distension during va ecmo may develop , leading to progressive left heart deterioration , pulmonary edema , and impairment of myocardial oxygenation . despite its clinical importance , the management of left heart decompression in a patient with ecmo is rarely discussed . only sporadic case reports and a few articles have shown the efficacy of la decompression on functional recovery of the left heart [ 57 ] . conventionally , la decompression is achieved by insertion of an la venting cannula via sternotomy or thoracotomy . however , sternotomy or thoracotomy themselves are risky and have several complications , such as bleeding and significant scarring . la decompression with a percutaneous cardiac catheterization - based technique including septostomy using a blade or radiofrequency ablation , balloon dilatation , and la venting cannula insertion has been effective [ 2,46,8 ] . several institutions with la vents have identified technical issues related to management of the indwelling catheters , such as kinking , poor flow , movement of the catheter with required patient care , and ongoing concern for thrombosis . therefore , a recent study showed a shift in preference from la vent insertion to balloon dilation alone . however , balloon atrial septostomy alone is not always successful because of varying degrees of atrial septal thickness . we believe that using an la venting cannula after balloon dilation offers several advantages over balloon dilatation alone . first , the placement of an la venting cannula potentially allows for controlled decompression of the left heart by adjusting flow rates on the ecmo circuit or clamping the cannula . third , the size of the cannula can be tailored to each patient , so this procedure can be adjusted for use in smaller patients . for patients with left heart dysfunction causing pulmonary edema during va ecmo , percutaneous balloon atrial septostomy with la venting cannula insertion is a treatment option that carries a low risk of other complications such as bleeding .

patients with venoarterial extracorporeal membrane oxygenation ( ecmo ) frequently suffer from pulmonary edema due to left ventricular dysfunction that accompanies left heart dilatation , which is caused by left atrial hypertension . the problem can be resolved by left atrium ( la ) decompression . we performed a successful percutaneous la decompression with an atrial septostomy and placement of an la venting cannula in a 38-month - old child treated with venoarterial ecmo for acute myocarditis .

primary cardiac lymphoma ( pcl ) is a rare malignancy.1)2 ) furthermore , bi - atrial involvement is extremely rare , and most of the previous cases have been b - cell lymphomas.3 ) we report here a case of pcl with involvement of both atria and the atrial septum . a 63 year old female without any previous medical history presented with an acute onset of dyspnea and facial swelling for 1 month duration . physical examination showed that jugular venous engorgement was worse in the right decubitus position and we detected a cardiac systolic murmur ( grade 3/6 ) and a third gallop . tenderness on the right upper quadrant was noted and there were decreased breathing sounds in both lungs . the electrocardiography showed sinus tachycardia and a normal qrs axis . on laboratory findings , lactate dehydrogenase transthoracic echocardiography ( tte ) and transesophageal echocardiography ( tee ) revealed bi - atrial masses that were protruding into the ventricles with cardiac motion ( fig . the chest computed tomography ( ct ) scan showed right inferior pulmonary artery thromboembolism and both pleural effusion and huge biatrial masses . cardiac ct showed huge bi - atrial masses and invasion into the atrial septal wall , myocardium and pericardium . the preoperative coronary angiography showed no stenotic lesions and there were feeding vessels from the right coronary artery and left circumflex artery to the biatrial masses ( fig . a median sternotomy was made , but surgical resection was not done except for biopsies because the myocardium was rigid due to tumor invasion to the pericardium and myocardium . the pathologic examination showed that the tumor was cd20 ( + ) , cd79a ( + ) , cd3 ( - ) and cd5 ( - ) , and this was consistent with a diffuse large b cell lymphoma ( fig . 3 ) . positron emission tomography - ct ( pet - ct ) showed a markedly increased fluorodeoxyglucose ( fdg ) uptake in the heart and pericardium as well as mediastinal and right supraclavicular lymph nodes invasion ( fig . 2c ) . she was treated with rituximab , cyclophosphamide , adriamycin , vincristine and prednisone ( r - chop ) . follow - up tte and tee after the six rounds of chemotherapy revealed remnant , 1 cm sized , small , bi - atrial masses and reduced right and left heart failure ( fig . 4 ) . however , follow - up pet - ct after the end of r - chop chemotherapy showed no fdg uptake in the heart except for fdg uptake of resection scar and a lung abscess that occurred during chemotherapy ( fig . the currently accepted definition of pcl is non - hodgkin 's lymphoma involving only the heart or with the bulk of the tumor located in the heart . pcl is a rare malignancy in immunocompetent patients.1 - 4 ) its incidence has been increasing in immunocompromised hosts,5 ) and the main sites of involvement of pcl are reported to be the right atrium , right ventricle , myocardium and pericardium,1 - 4 ) and less often the left atrium and left ventricle.6 ) furthermore , biatrial involvement is extremely rare.7)11 - 17 ) right - sided involvement predominates in pcl in contrast to left - sided predominance in atrial myxoma , which is a major primary benign cardiac tumor . we report here a case of pcl that involved both atria in an immunocompetent patient . ikea et al.6 ) reviewed the cardiac lymphomas that had been reported in the literature between 1995 and 2002 . here , we review cases of pcl with biatrial involvement reported between 1995 and 2008 ( table 1 ) . in the present case , the patient presented with right heart failure and symptoms like a superior vena cava syndrome . in the literature , the majority of pcl patients showed various and nonspecific symptoms such as dyspnea , edema , arrhythmia , cardiac tamponade , palpitation , and congestive heart failure . this appears to be correlated to a certain extent with the location of the neoplasm within the heart . most of the patients described in the literature were older patients with a nearly equal gender distribution . diagnosis of a cardiac tumor was made on examination of the surgically resected specimen , and in earlier reports the diagnosis was also usually made postmortem . pathology - based diagnosis of malignant lymphoma is essential in order to administer appropriate therapy . most pcls in the literature were b - cell neoplasms and of the diffuse large cell type.1 - 4)7 - 9 ) our patient was also diagnosed with diffuse large b cell lymphoma and this was confirmed by immunohistology . modern imaging modalities helped make an early diagnosis and determine the extent of tumor involvement in the heart . in the present case , the mass had a butterfly shape with a bigger right " wing " ( fig . 2d ) . in conclusion , we reviewed the present case and seven patients with pcl with involvement of both atria reported between 1995 and 2008 . pcl with involvement of both atria is due to a massive cardiac tumor and has a relatively poor prognosis as complete remission was obtained in only three of the eight patients .

we described here a patient who presented with symptoms of heart failure who was found to have severe bilateral impairment of atrioventricular inflow . primary cardiac lymphoma ( pcl ) with extensive involvement of the two atria , pericardium and myocardium is an extremely rare tumor in immunocompetent patients . we report here a case of pcl in an immunocompetent patient with involvement of both atria and the atrial septum . the tumor had a butterfly shape . we could not do surgical excision because of the massive pericardiac invasion . the diagnosis was b - cell lymphoma and this was confirmed by the pericardiac biopsy .

spinal muscular atrophy ( sma ) is an autosomal recessive disease characterized by degeneration of anterior horn cells of the spinal cord leading to progressive symmetrical weakness and atrophy of the proximal muscles . in addition to classical sma types ( type 1 , 2 and 3 ) a new form of sma called type 0 with intrauterine onset and hypotonia , a progressive and early fatal course has been described . here in we report one such case of sma type 0 in combination with a dandy - walker variant . baby s was a second born male infant to non - consanguineous parents , 28-year - old father and 22-year - old mother . the pregnancy was unremarkable but the mother reported decreased fetal movements from 30 weeks of gestation . antenatal ultrasound revealed cisterna magna communicating with the fourth ventricle through the cerebellar hemisphere : findings consistent with a dandy - walker variant . baby was born by spontaneous vaginal delivery at 39 weeks of gestation , weighing 2.75 kg . he was noted to be hypotonic with poor spontaneous efforts and was intubated and shifted to nicu and mechanically ventilated . no seizures were noted and there was no clinical or lab evidence suggestive of hypoxic - ischemic injury to other organ systems . the alert look was in contrast to the marked hypotonia and absent spontaneous muscle activity . a diagnosis of sma was considered and confirmed on molecular genetic analysis which revealed the homozygous deletion of exon 7 of smn1 gene . a post natal ct scan revealed partial vermian hypoplasia with partial obstruction to the fourth ventricle but no enlargement of posterior fossa : findings consistent with dandy - walker variant [ figure 1 ] . the baby died immediately after the mechanical ventilation was discontinued on day 19 of life at the request of the parents . ct scan showing partial vermian hypoplasia with partial obstruction to 4 ventricle but no posterior fossa enlargement : findings suggestive of dandy walker variant very severe sma ( type 0 ) reportedly presents with reduced fetal movements in utero , profound hypotonia , severe asphyxia and respiratory insufficiency at birth warranting resuscitation and mechanical ventilatory support . all reported cases have been ventilator dependent and died in the neonatal period after the support was withdrawn , generally on the request of the parents . genetic studies have revealed homozygous deletions of exon 7 and 8 of the smn gene in these infants making the diagnosis of sma possible . the index case too had an antenatal onset with history of decreased fetal movements from 30 weeks of gestation , severe asphyxia needing resuscitation and mechanical ventilation , profound hypotonia and a striking alert look of the baby . like other cases reported till date our infant was also ventilator dependent and died on day 19 after the ventilator support was withdrawn at the request of the parents once the diagnosis was confirmed by molecular analysis showing homozygous deletion of exon 7 of smn1 gene . the most severe type of sma presents itself at birth or in the early days of life which may be difficult for primary care providers or pediatricians to diagnose . proper diagnosis of this disorder needs a high index of suspicion and understanding of clinical signs and symptoms . sma should be kept in mind in the differential diagnosis for unexplained severe generalized hypotonia and severe respiratory distress immediately after birth in the neonates , notably in patients with a bright expression and alert look . to date , it is not known with certainty whether this subgroup represents a distinct entity or is merely the severe end of the classical sma type 1 . dubowitz explains that from a classification point of view , the more severe cases with prenatal onset and intrauterine death or with severe asphyxia at birth and early neonatal death fit into the category of very severe sma ( type 0 ) as an extension to previous severe sma type 1 . there are several reports of sma with additional features ( sma plus ) , including pontocerebellar hypoplasia , diaphragmatic paralysis and dandy - walker complex . the report on sma in combination with dandy - walker complex was in two siblings aged 23 and 25 years who were symptomatic since the age of 10 years with progressive , symmetrical distal muscle weakness and bilateral anterior polar cataracts diagnosed at the age of 9 - 11 months . the molecular genetic analysis revealed homozygous deletions of exon 7 and 8 of the smn gene . in the present case sma a dandy - walker variant is a less severe posterior fossa anomaly than the classical dandy - walker malformation and is considered being on the lesser end of the disease spectrum in the dandy - walker continuum . there is usually partial vermian hypoplasia and partial obstruction of the fourth ventricle but without enlargement of the posterior fossa : as observed in the present case . to conclude , our case represents an unusual combination of severe spinal muscular atrophy ( type 0 ) and dandy - walker variant hitherto not reported in the literature .

spinal muscular atrophy ( sma ) is an autosomal recessive neuromuscular disease characterized by degeneration of alpha motor neurons in the spinal cord , resulting in progressive proximal muscle weakness and paralysis . in addition to the three classical sma types , a new form known as type 0 with intrauterine onset , profound hypotonia and a progressive and early fatal course has been described . herein we report a case of type 0 sma with a dandy walker variant anomaly , which has not hitherto been reported in the world literature .

brucella endocarditis ( be ) is a rare but severe and potentially lethal manifestation of brucellosis . pre - existing valves lesions and prosthetic valves ( pv ) are favorable for be . we represent the case of a 46-year - old man who was treated at the clinic for infectious diseases , clinical center of sarajevo university , as blood culture positive ( brucella melitensis ) mitral and aortic pv endocarditis . be occurs in 0.5%-2% of all brucella cases , causing 80% of deaths ( 1 ) . its characteristic features , such as valve ulceres and destruction , myocardial abscess and aneurysm , besides verucca , make it different from other infective endocarditis ( ie ) . the major cause of death in be is congestive heart failure due to destruction of valve and paravalve structures ( 2 ) . since 1967 , when the first case of valve replacement due to be was reported ( 3 ) , combination of medical and surgical therapy is considered as treatment of choice ( 4 , 5 ) . we present here the case of a 46-old man , a stockbreeder from the central bosnia canton who was treated for pv endocarditis due to brucella during 2007 . in 1998 , he underwent an aortic and mitral mechanical valve replacement surgery due to juvenile rheumatic fever . he was doing well , being work - engaged until 2006 , when he started to complain about night sweating , chills , exhaustion , dyspnea , palpitations and chest oppression . at that time after more than one year of over repeated hospitalizations at the department of internal medicine of regional hospital due to symptoms of heart failure , he was referred to the clinic for infectious diseases , clinical center of sarajevo university . upon confirmation of the disease , based on positive blood cultures ( b.melitensis ) taken during everyday subfebrile temperatures , formerly unrecognized , and on transthoracic echocardiography ( tte ) indicative of endocarditis , the patient was transferred to clinic for infectious diseases in sarajevo . upon admission , he had cardiac and extra - cardiac manifestations of endocarditis ( systolic - diastolic murmur with absolute arrhythmia , splinter haemorrhages , and laboratory criteria for glomerulonephritis ) and symptoms of severe heart failure ( class iii / iv ) according to the new york heart association ( nyha ) criteria . standard chest radiography and abdominal ultrasound revealed cardiomegaly , hepatosplenomegaly and stasis in the lungs and liver . coxiella burnetii coinfection was confirmed by elisa ( enzyme linked immunosorbent assey ) test ( igg f2 + , igg f1 + , iga f1 + ) . transesophageal echocardiography ( tee ) confirmed the presence of restenosis , multiple mobile vegetations ( 5 - 8 mm ) attached to mitral and aortic valve ( mv , av ) and signs of severe pulmonary hypertension . he did not respond to quadruple antimicrobial and cardiac therapy . a follow - up tee performed at the end of the third therapeutic week confirmed progression of the disease : persistent multiple mobile vegetations at both valves 7 - 10 mm , left atrial thrombus 7x10 mm . according to cardiac surgeons , surgical intervention had to be postponed due to cardiac instability . the patient was discharged with recommendation to continue conservative treatment and need for follow - up by cardiologist , cardiac surgeon and infectious diseases ( i d ) specialist . the case of a double prosthesis valves be ( mv , av ) is presented here . prosthetic valve be occurs in 8.3% of all cases but literature lacks reports on double prosthetic valves be . the first case report dated 1980 ( 6 ) , with only two cases since then , in 2006 and 2009 ( 2 , 7 ) . diagnosis of be is confirmed late in this case , after more than a year , despite a clearly positive epidemiological survey and the fact that the patient was under continuous observation by general practitioner , and hospitalized several times . low - grade suspicion by physician and incomplete epidemiological survey are the reasons for laterecognition of the disease ( 8 , 9 ) . etiological diagnosis of be is made on the basis of positive blood cultures , even today be is classified as negative - blood cultures endocarditis due to a long course of the disease and irrational antibiotic treatment ( 10 , 11 ) . abscess is a common complication of be ( 12 ) , but it is not the case here . the patient met all the criteria for urgent surgical treatment ( 13 ) , but pv replacement was postponed due to cardiac surgeons opinion of a high risk cardiac instability . conservative treatment with four antibiotics ( 2 , 14 , 15 ) led to slight recovery in the sense of reduction of symptoms of general infective syndrome , while pv damage progressed . despite reported cases of successful treatment of prosthetic valve be with medications ( 8,15 ) , most authors agree that combined antimicrobial and surgical treatment is the only effective form of therapy ( 2 ) . there is no consensus on optimal therapeutic approach to be but it is proved that the outcome of treatment is in function of time that passes from the onset of the disease to the administration of the causal therapy . high grade suspicion by physician significantly shortens that time and contributes to the successfulness of treatment . prolonged febrile state in patients with pv and clearly positive epidemiological survey is considered equivalent to be .

summaryconflict of interest : none declared.introductionbrucella endocarditis ( be ) is a rare but severe and potentially lethal manifestation of brucellosis . pre - existing valves lesions and prosthetic valves ( pv ) are favorable for be.case reportwe represent the case of a 46-year - old man who was treated at the clinic for infectious diseases , clinical center of sarajevo university , as blood culture positive ( brucella melitensis ) mitral and aortic pv endocarditis . he was treated with combined anti - brucella and cardiac therapy . surgical intervention was postponed due to cardiac instability . four months later he passed away . surgery was not performed .

traumatic duodenal injuries with significant tissue loss are rarely seen but are a potentially challenging problem when they occur in either children or adults [ 1 - 3 ] . the incidence of duodenal injury in the literature ranges from 3.7% to 5.0% and it is often accompanied by other abdominal injuries because of its close anatomic relationship with the liver , pancreas , gallbladder , and ampulla of vater . in uncomplicated cases , the duodenal defect can simply be closed primarily . however , more complex injuries require pyloric exclusion with a gastrojejunostomy or a pancreaticoduodenectomy . several experimental studies have also examined whether other approaches could reduce the morbidity and mortality that is associated with the management of complicated large duodenal defects . several flaps have been used successfully to repair abdominal wall defects , including the rectus femoris muscle , antero - lateral thigh fasciocutaneous , and sartorius muscle or myocutaneous flaps . however , the reconstruction of an abdominal wall defect that is associated with a large duodenal disruption is clinically challenging and technically demanding . in the present report , we describe an alternative technique for closing an abdominal wall defect that is associated with a large duodenal disruption : a free flap was used with the help of the temporary placement of a covered expandable metallic stent . to our knowledge , this is the first time this approach has been reported . he had intra - abdominal exsanguinating bleeding , duodenal disruption , and multiple small bowel perforation . duodenojejunostomy , the resection of about 150 cm of small bowel followed by anastomosis , and bleeding control were performed . despite of repeated reanastomosis due to anastomosis failure , he developed sepsis and multiorgan failure . a surgical reexploration was performed and it revealed duodenojejunal disruption and multiple enteroenteric anastomosis failure ; there was a large amount of bilestained fluid in the abdomen . since less than 1 m of the small bowel remained , pyloric exclusion or pancreaticoduodenectomy could no longer be considered . therefore , we performed enteroenterostomy and duodenal primary repair , and placed a feeding tube distal to the perforated duodenum . fortunately , after intensive care , the patient could be transferred to the general ward after ventilator weaning . however , due to severe retraction of the duodenum , a duodenal disruption gap that was approximately 5 cm long developed . however , because of persistent leakage of gastric , bile , and pancreatic juice through the duodenal defect , the abdominal wall defect could not be repaired directly with a free flap . therefore , the bile and pancreatic juices were diverted first by placing separate pancreatic duct and bile duct drainage tubes by endoscopic retrograde cholangiopancreaticography . thereafter , the interventional radiologist placed a 16-mm diameter , 90-mm long covered expandable metallic stent ( s&g biotech , seongnam , korea ) from the duodenal bulb to the jejunum . bile and pancreatic juices were diverted through endoscopic nasobiliary drainage and endoscopic nasopancreatic drainage completely . seven days after stent placement , the patient underwent debridement and closure of the abdominal wall defect with a free flap . duodenostomy was performed by placing a drainage tube that decompressed the duodenum and prevented infection . endoscopic images obtained 3 months after stent placement revealed a widely patent stent , although granulation tissue had formed above the stent . the stent was electively removed 112 days after placement by using a stent removal set . the pancreatic duct and bile duct drainage tubes were removed 13 days after stent removal . on the 234 th postoperative day , an upper gastrointestinal water - soluble contrast study was performed and showed good passage of contrast with a small enterocutaneous fistula and no significant obstruction ( fig . various treatment options have been reported to be effective for the surgical management of large duodenal defects . these include duodenal resection with end - to - end anastomosis , roux - en - y duodenojejunostomy , diverticulization , polytetrafluoroethylene patching , omental plug closure , jejunal serosal patching , and pedicled grafts . in the present report , a free flap was used with the help of a temporary stent to close a large duodenal defect and an abdominal wall defect . in this case , the patient had less than 1 m of small bowel left due to several previous small bowel resections . the main advantage of the alternative technique described in the present report is that it did not involve intestinal resection . it also prevented the possible risk of gastrointestinal contamination , peritoneal soiling , and organ injury . in our case , severe retraction of the duodenum led to a duodenal disruption that was approximately 5 cm long . contact between the free flap and the bowel appeared to be an important factor in duodenal prefabrication . an expandable metallic stent successfully reestablished duodenal continuity until the abdominal wall defect had been repaired . the stent was removed 112 days after its placement because of granulation tissue formation above the stent . a postoperative upper gastrointestinal water - soluble contrast study revealed good repair of the abdominal wall defect and the large duodenal disruption . however , the repaired duodenal wall is surrounded with a free flap , and we remain concerned that a fibrotic stricture could occur . in recent years , expandable metallic stents have been used in many hospitals for the palliative treatment of inoperable malignant gastroduodenal strictures . however , intestinal stent implantation remains potentially controversial because these stents can not always be removed easily after placement . stents used to treat a benign gastrointestinal stricture should be designed to be retrievable to prevent long - term complications such as granulation tissue formation and stent migration . in this patient with large duodenal disruption and abdominal wall defect , we placed a fully covered expandable stent to prevent leakage of bile juice and to establish duodenal continuity . we could remove the stent through per - oral route after repair of abdominal wall defect . although further clinical trials and extended follow - up studies are needed , the present case indicates that combining a free flap with a covered expandable metallic stent can effectively and successfully repair an abdominal wall defect that is associated with a large duodenal disruption .

abdominal wall defect with large duodenal disruption after penetrating abdominal injury is a rare emergency situation that can result in life - threatening complications . we report on a 64-year - old man who had abdominal wall defect with large duodenal disruption after penetrating abdominal injury . the patient presented with intra - abdominal exsanguinating bleeding , duodenal disruption , and multiple small bowel perforation . the rarity of this complex injury and its initial presentation as a posttraumatic large duodenal disruption with abdominal wall defect warrant its description . the present case indicates that combining a free tissue flap with a covered expandable metallic stent can effectively and successfully repair an abdominal wall defect that is associated with a large duodenal disruption .

in maxillary osteotomy le fort i type the detachment of the nasal mucosa should be done carefully , because mucosa tears can produce postoperative bleeding after restoration of blood pressure . piezoelectric surgery contributed much to increase the safety of osteotomies , despite the initial advantage of minimizing the risk of injury in nervous tissue mainly in bilateral sagittal split osteotomy ; we use the piezoelectric device for the initial detachment of the nasal mucosa in the maxillary osteotomy . after exposure of the nasal mucosa [ figure 1 ] , we use the tips that were originally developed for sinus lift ( mectron piezosurgery ) and completed the detachment of the nasal mucosa [ figure 2 ] using a conventional form . we recommend that you experiment and different that was found in the literature in our cases when the piezosurgery device is used to complete the maxillary le fort i osteotomy , we find increase around 20% in total operative time . nasal mucosa after the maxillary mucoperiosteal degloving nasal mucosa detached from nasal cavity using the ultrasonic tip

in maxillary le fort i type osteotomy the detachment of the nasal mucosa should be done carefully . piezoelectric surgery contributed much to increase the safety of osteotomies , despite the initial advantage of minimizing the risk of injury in nervous tissue , mainly in bilateral sagittal split osteotomy ; we use the piezoelectric device for the initial detachment of the nasal mucosa in the maxillary osteotomy .

in august 2004 , a previously healthy 31-year - old male was referred to the emergency department due to a 5-day history of progressing abdominal pain . physical examination revealed diffuse tenderness of the abdomen , without significant rebound or guarding . an emergency non - enhanced computed tomography ( ct ) of the liver ( fig . 1a , c , e ) showed a high luminal density within the main portal vein and splenic vein , which was suggestive of the formation of fresh thrombus ; a contrast - enhanced ct scan at the portal phase ( fig . 1b , d , f ) demonstrated that the diameter of the main portal vein was distended to nearly 3-fold that of the aorta , and a few fine collateral vessels developed around the thrombosed portal trunk in the liver hilum . to recanalize the thrombosed portal vein and prevent thrombus extension , a 48-h continuous infusion of urokinase into the superior mesenteric artery subsequently , heparin was administered intravenously at a starting dose of 1,000 u / h for 5 days . the patient 's thrombophilia screen results were normal , including antithrombin iii deficiency , protein c and protein s deficiency , factor v leiden and antiphospholipid antibody . , a color doppler ultrasound examination showed that the main portal vein was replaced by numerous collateral veins in the liver hilum . in november 2009 , the patient presented with repeated abdominal distension and a recent episode of variceal hemorrhage that was controlled by medical therapy . at admission , physical examination revealed splenomegaly and abdominal bloating . he had an impaired liver function with a child - pugh class c and a meld score of 16.8 points . a low platelet count of 69,000 mm and a low hemoglobin concentration of 108 g / l were also observed . indirect portography via superior mesenteric artery revealed numerous hepatopetal collateral vessels in the liver hilum ( fig . subsequently , a transjugular intrahepatic portosystemic shunt insertion via a trans - splenic approach was attempted to treat complications of portal hypertension , as previously described . however , the operation failed , because a guidewire and catheter could not access the portal vein ( fig . if acute thrombus extends into mesenteric venous arches , severe complications occur , such as intestinal infarction and even peritonitis . if the portal vein obstruction is not timely recanalized , cavernous transformation of the portal vein develops and pre - hepatic portal hypertension might cause complications , such as variceal bleeding and ascites . accordingly , both early differential diagnosis and intervention are very critical in the emergency situation . ct has been considered a mainstay diagnostic modality of pvt by visualization of its associated abnormalities . the ct imaging features that are specific to recent pvt include a high - density material in the portal vein on non - enhanced ct scans and enlargement of the obstructed portal vein on contrast - enhanced ct scans . on non - enhanced ct scans , a fresh thrombus usually appears as a hyperattenuating material in the portal vein , because the protein fraction of hemoglobin in the clot results in the elevated ct attenuation value . dilation of the portal vein is also regarded as a ct feature at the early stage of pvt . however , to our knowledge , such a great dilation of the main portal vein as in our patient has rarely been reported . additionally , it should be noted that this rare ct feature might lead to an erroneous diagnosis of the pancreatic mass ( fig . 1d , f ) , because the enlarged portal vein was anatomically adjacent to the pancreatic head . in the current practice guidelines , anticoagulation was considered as the first - line therapy for acute pvt in non - malignant and non - cirrhotic patients [ 6 , 7 ] . however , a recent prospective cohort study demonstrated that approximately 70% of patients would not achieve portal vein recanalization , despite anticoagulation therapy was immediately given . if so , prehepatic portal hypertension would occur in a high proportion of patients with pvt . patients with extrahepatic pvt who have portal hypertension but do not bleed from the varices should be observed , because bleeding may not occur for many years [ 9 , 10 ] . however , considering our case , the wait and see attitude appears to be not completely appropriate . this consideration mainly originates from the fact that the long - term outcome was poor in our case , as portal venous recanalization was not achieved by thrombolysis and anticoagulation . we hypothesized that if an early aggressive interventional treatment was carried out in the patient , portal venous recanalization rate would be elevated and portal hypertension might be avoided , thereby improving the long - term survival .

acute portal vein thrombosis ( pvt ) is rarely encountered by clinicians . the most common manifestation of acute pvt is sudden onset of abdominal pain . a computed tomography scan without contrast often shows a high - density material in the portal vein . after injection of contrast agents , absence of luminal enhancement and enlargement of the obstructed portal vein are shown . in this case report , we demonstrated a rare computed tomography finding in which the diameter of the main portal vein was enormously distended to 3-fold that of the aorta in a patient with recent pvt . despite thrombolysis and anticoagulation were immediately given , portal venous recanalization was not achieved in the patient . after 5 years , variceal bleeding and ascites occurred and liver function had persistently deteriorated . finally , he died of progressive liver failure . considering this case , we suggest that an early decision for invasive interventional treatment might be necessary to both increase the rate of portal venous recanalization and improve prognosis , as anticoagulation and thrombolysis therapy failed to recanalize recent pvt .

during october 1 , 2014june 9 , 2015 , princeton house , a psychiatric facility in suburban new jersey with an active opioid detoxification program , instituted a new hcv screening program . as part of the standard of care , patients admitted for heroin detoxification were tested for hiv , hcv , and hepatitis b virus infections . follow - up visits at princeton house before discharge were performed by one of the authors ( k.h.s . or r.g.n . ) to counsel patients on results and the disease and to link patients to care by providing directions and appointments to hcv caregivers near patients homes . a total of 861 unique patients from 10 of 21 new jersey counties were tested for hcv antibody ; 374 ( 43.4% ) were positive . of those , 237 ( 41.4% ) were hcv antibody positive . from this population , 187 patients were further evaluated ; 50 patients refused evaluation or were discharged before evaluation . races and ethnicities were 173 non - hispanic white , 2 non - hispanic black , 4 hispanic , and 8 other . hcv viral load was obtained for 172 ( 92.0% ) of the 187 patients ; 15 patients were missed or not properly collected . for 32 ( 18.6% ) patients , hcv gts were obtained from 102 patients : 64 ( 62.7% ) were gt1a , 3 ( 2.9% ) were gt1b , 8 ( 7.8% ) were gt1 undefined , 1 ( 1.0% ) was gt2 , and 26 ( 25.5% ) were gt3 . of the 187 patients , 16 ( 8.6% ) had outpatient follow - up appointments , and 3 ( 1.6% ) started oral , direct - acting antiviral treatment . two other patients returned for treatment but were denied prescriptions by insurance ; all others failed to return for continued care . our study indicates that hcv was highly prevalent in young suburban heroin users attending an acute detoxification program that serves a wide geographic area , suggesting that new jersey is participating in the second wave of hcv infection . our study highlights the challenges of linking young pwid in suburban areas to care despite the effort of 2 clinicians with extensive hcv experience to engage patients in the care cascade . that most patients were women ( 52.4% ) and non - hispanic white ( 92.5% ) probably reflects the demographic of persons seeking detoxification from heroin and coincides with demographics of other reports of young nonurban pwid in the united sates ( 2 ) . the 25.5% ( 95% ci 17%34% ) prevalence of gt3 among this population of young suburban heroin users is more than twice the national average of 12% ( 6 ) . this pattern of distribution suggests a closed network of injection drug users engaging in risky behavior that leads to hcv transmission . although hcv screening was easily attainable in princeton house , linking patients to care was a challenge ( figure ) . even with encouragement , only 16 ( 8.6% ) patients returned for in - office follow - up visits , and 2 started treatment . patient follow - up after patients left princeton house was a logistical challenge because of patient relocation and availability of transportation and communication . the program of acute detoxification at princeton house simply withdrew patients from heroin under direct observation . long - term patient management required additional treatment for patient addiction upon discharge , which was difficult to achieve . we were also limited by the absence of a care coordinator to assist with the linkage to care effort . finally , some patients who came for outpatient treatment were denied treatment by payers because of the requirement of a clean drug test before treatment initiation or because patients did not have advanced liver disease , defined as stage 3 or 4 . because we did not formally assess patients psychosocial circumstances , our considerations for the reasons for failure to link to care are speculative and are currently being studied . we believe that difficulty in linkage to care represents an area where greater support will be critical , such as through the use of case management , as was done with hiv care under the ryan white program ( 7 ) . cascade of care for suburban heroin users 1735 years of age , new jersey , october 1 , 2014june 9 , 2015 . also noteworthy is the absence of hiv in a population with a high percentage of injection drug use in a state with a high prevalence of hiv . indiana s recent outbreak of hiv among young heroin users increases concern for the establishment of hiv in this network ( 8) . to achieve success similar to that of hiv treatment in pwid , a coordinated program that includes committed case management services to help pwid navigate the complexity of accessing and maintaining treatment is likely to be needed . further study to explore this and the ability to successfully treat this population is crucial to address the national hcv infection epidemic . finally , a misconception exists that pwid are poor candidates for treatment because of ongoing drug use , possible reinfection , and possible concomitant psychiatric or medical disorders ( 9 ) . however , recent reports suggest that such patients can be successfully treated with newer therapies ( 10 ) . success in reducing hiv transmission among pwid during the past decade provides evidence that infected drug users can achieve adherence levels similar to persons who do not use drugs ( 9,11 ) . the potential for treatment as prevention was discussed by hellard et al . , who calculated the minimum number of patients within a network needed to be treated to reduce or eliminate transmission ( 10 ) . given the availability of easy - to - use therapy that is curative , linkage to care and treatment of hcv - infected pwid may be an important public health effort to prevent the continued spread of hcv .

we identified a 41.4% prevalence of hepatitis c virus , absence of hiv , and unexpectedly high frequency of hepatitis c virus genotype 3 among suburban new jersey heroin users 1735 years of age during 20142015 . despite 2 clinicians prepared to engage these users , few were successfully linked to care and treated .

common variable immunodeficiency disorders ( cvids ) are a heterogenous group of immune deficiencies characterized by hypogammaglobulinemia . the diagnostic criteria are : history of recurrent infections , decreased igg levels ( less than two standard deviations below the lowest normal level ) , one more immunoglobulin deficiency , and failure to mount sufficient antibody response to antigen challenge ( with pneumococcal or hib vaccines ) . a 42-year - old man presented to our center with high - grade fever , chills , productive cough since six weeks , weight loss ( 6 kg over four weeks ) , and diarrhea since two weeks . in mid - july , a chest radiograph ( cxr ) showed right middle zone consolidation . antibiotics were started ( levofloxacin , amikacin , metronidazole , fluconazole ) without response , at another center . when there was no response , he was referred to our hospital ( 13/9/09 ) . he was asymptomatic until 5 years ago after which he had recurrent upper respiratory tract infections ( rtis ) since 2005 : pneumonia ( 2006 ) , and was treated for pulmonary tuberculosis twice ( 2005 , 2007 ) . since 2007 , he had monthly episodes of watery diarrhea . examination revealed fever , tachycardia , tachypnea ( respiratory rate 36/min ) , blood pressure of 110/60 , and bilateral crepitations on chest auscultation . he was admitted to the intensive care unit ( icu ) where cxr showed right middle and lower zone consolidation . non - resolving pneumonia ( nrp ) was diagnosed and meropenem and levofloxacin were started , att being discontinued . antibiotics were changed ( targocid , colistin ) as per sensitivity reports , and empiric amphotericin - b was added . intravenous immunoglobulin ( ivig ) was given ( 2 g / kg on 21/9/09 ) , over five days for the hypogammaglobulinemia [ table 1 ] . by day 2 of ivig administration , he was ambulatory on discharge ( december ) and follows up ( irregularly , due to cost constraints ) for replacement ivig and chest physiotherapy . serum immunoglobulin level measurement done as a part of the workup for nrp [ table 1 ] showed hypogammaglobulinemia . there was also history of recurrent rtis and diarrhea , beginning 5 years prior to presentation . there was infection with two organisms ( klebsiella pneumonia and pseudomonas aeruginosa ) at presentation . these points suggested an underlying adult - onset pid with antibody deficiency , and possibly , t lymphocyte dysfunction . high - dose ivig was commenced ( 2 g / kg loading dose , over 5 days ) , as serum igg levels were undetectable , and significant improvement followed , within 72 hours . although theoretically this could be attributed to the effect of ivig on systemic inflammatory response in sepsis , repeat igg levels , after 8 and 12 weeks of loading dose , continued to decline [ table 2 ] , improving only after replacement doses ( 0.4 g / kg ) were given , thus confirming the diagnosis of a cvid . igg levels on follow - up recurrent rtis are the most common manifestation in cvids , complicated with sequelae like chronic sinusitis , lung fibrosis , and bronchiectasis ( 12.2% ) . treatment of hypogammaglobulinemia is replacement with a monthly dose of 0.4 g / kg , even on outpatient basis . cvids can present at any age , with onset peaking in the first and third decades . our patient became symptomatic in the fourth decade ( age 37 years ) , and was diagnosed in the fifth decade ( age 42 years ) . he could therefore fall into the late - onset common immune deficiency category , as per the defi study . pids may easily be missed as a cause of nrps , as other causes ( malnutrition , hiv infection , tuberculosis , inappropriate use of antibiotics ) are common , while the exact incidence of cvids is unknown , although it is the most common pid encountered in clinical practice . the pneumonia in our patient deteriorated despite appropriate antibiotics , mechanical ventilation , and inotropes , improving only when his underlying pid was diagnosed and treated . the learning point that emerges is , cvids are an important cause of severe pneumonia even in adults . correct diagnosis and prompt treatment of the underlying cvids , even in patients on maximum life support , can reduce the mortality of severe pneumonia .

common variable immunodeficiency disorders ( cvids ) , a heterogeneous group of primary immune deficiencies , can present at all age . our patient with a hitherto undiagnosed cvid , symptomatic since middle ages , presented with severe pneumonia . specific management of his cvid in addition to standard therapy was life - saving .

lumbar intraspinal synovial cysts can be a cause of low back pain or lumbar radiculopathy12 ) , but uncommon10 ) . the frequency of diagnosis may be on the rise due to the aging population and high diagnostic yield of magnetic resonance imaging ( mri ) scans6 ) . however , the case of bilateral new lumbar intraspinal synovial cysts after laminectomy is rarely reported . the leg pain was bilateral and aggravated recently , but muscle power and sensation were intact . we diagnosed the spinal stenosis and grade i spondylolisthesis at the l4/5 level via the lumbar mri ( fig . after 2 months , he revisited due to both legs pain for several days . on neurologic examination , he decreased muscle strength in plantar flexor muscles on both sides . on emergency lumbar mri , 2 well - defined extradural cystic mass the cysts were connected to facet joints and compressing the thecal sac and both l5 nerve roots . the histopathologic examination revealed 0.5- to 1-cm cyst of fibrous connective tissue covered with synovial lining ( fig . in 1885 , baker first described the formation of synovial cysts adjacent joints to a joint . however , in the last decade , improving imaging modalities has resulted in increased reporting . several authors reviewed spine mr images and computed tomographic images and reported that the incidence of synovial cyst was from 0.5% to 7.3%238 ) . walcott and coumans11 ) reported 10 cases of cyst formation after laminectomy for lumbar spinal stneosis . they could not estimate the incidence of postlaminectomy cyst formation because they did not systematically perform the mri scan on all patients after surgery11 ) . they did not mention whether the cysts were unilateral or bilateral . and they performed reoperation for the cysts and confirmed pathologic results in 5 patients . on the other cases , they treated with the conservative management such as observation or epidural steroid injections and could not confirm whether the cysts were pathologically true synovial cysts11 ) . howington et al.4 ) also reported 5 patients with a prior surgery and subsequent development of intraspinal synovial cyst . they reviewed the literature and found only 2 cases of bilateral intraspinal synovial cysts in 19994 ) . and lyon et al.9 ) reported 194 patients with lumbar intraspinal synovial cysts who were surgically treated . eleven percent of the patients had undergone previous lumbar spine surgery and eight of 194 patients were presented with bilateral synovial cysts9 ) . however , they did not mention how many patients develop bilateral cysts after a prior lumbar spine surgery9 ) . beside some debate remained , traumatic injury and segmental instability are the most often cited potential causative factors for the development of synovial cysts9 ) . this could cause the migration of synovium epidurally and the subsequent formation of the cyst11 ) . conservative treatment modalities include no treatment , bed rest , oral analgesics , physical therapy , orthopedic corsets , cyst aspiration and intra - articular injection and cyst puncture5 ) . some series have found that intra - articular injections provide acceptable long - term results , but these conservative treatments often show poor results57 ) . surgical excision can achieve a complete resolution of symptoms with a very low incidence of complications and recurrence7 ) . bydon et al.1 ) reviewed large literatures and reported that same - level cyst recurrence rate after surgical resection without fusion was 1.8% . but , there was no recurrence of the cyst after surgical resection with fusion1 ) . they also reported that back and leg pain recurrence rate were 21.9% and 12.7% , and 6.2% of patients required reoperation1 ) . the majority of reoperation required fusion for correction of spinal instability and mechanical back pain1 ) . they concluded that decompression and excision of lumbar intraspinal synovial cyst is safe and effect , but fusion of involved motion segment must be considered because of no recurrence of the cyst and back pain1 ) . semiconstrained ( or dynamic ) spinal instrumentations such as transpedicular dynamic systems or interspinous spacers may be applied , but studies evaluating the follow - up of these techniques for treatment of intraspinal synovial cysts are still lacking in the literature7 ) . we reported a rare case of postlaminectomy bilateral lumbar intraspinal synovial cysts . in most cases , however , the solid fusion after cyst excision must be considered if preoperative mechanical instability is identified or lumbar intraspinal synovial cyst recurs repeatedly .

lumbar intraspinal synovial cysts are included in the difference diagnosis of lumbar radiculopathy . developing imaging modalities has result in increased reporting about these lesions . however , the case of bilateral new lumbar intraspinal synovial cysts after laminectomy has been rarely reported . we report of a rare case with bilateral lumbar intraspinal synovial cysts after laminectomy , requiring surgical excision .

a 52-year - old man was referred to our hospital with a liver mass that had been incidentally detected on a liver ct at an outside hospital . the results of his laboratory tests , including the peripheral eosinophil count , serum alkaline phosphatase , serum bilirubin , aspartate aminotransferase and alanine aminotransferase , were all in the normal ranges . the levels of tumor markers , including -fetoprotein , ca 19 - 9 and carcinoembryonic antigen ( cea ) , were normal . his past medical history was unremarkable except for laparoscopic cholecystectomy that was performed due to a gall stone six years previously . in our hospital , this patient underwent two - phase liver dynamic ct because the ct images taken outside our hospital were not available . the ct showed a 4-cm , illmargined , lobulated , low - attenuation mass in the right posteroinferior segment of the liver ( figs . the mass had heterogeneous , peripheral contrast enhancement during the hepatic arterial phase and then more central enhancement was seen from the peripheral portion of the mass during the portal venous phase ( figs . a transient hepatic attenuation difference that surrounded the mass was noted during the hepatic arterial phase . there was a small portion of poorly enhancing , low attenuation in the peripheral area of the mass , which represented a necrotic portion . . there was an approximately 1.5-cm sized lymph node in the aortocaval space ( fig . the patient did not undergo percutaneous biopsy because he had little clinical evidence of a hepatic abscess . the patient underwent segmentectomy of the liver and resection of the lymphadenopathy in the aortocaval space . pathologic examination showed an ill - defined , lobulated mass measuring 765 cm ( fig . 1e ) , the mass consisted of inflammatory cells such as lymphocytes , neutrophils and macrophages , and also granulation tissue . one year after his discharge , the follow - up ct showed no unusual findings except for segmentectomy of the liver . although the incidence of human clonorchiasis infections has gradually decreased , it is still estimated that about 15 million people worldwide are currently infected ( 1 ) . the clinical manifestations depend on the number of flukes , the period of infestation and the complications such as pericholangitic abscess , recurrent pyogenic cholangitis , bile duct stones and cholangiocarcinoma ( 1 ) . on ct , the typical findings of clonorchiasis are uniform , minimal or mild dilatation of the intrahepatic bile ducts without dilatation of the extrahepatic bile ducts or any focal obstructive lesions ( 1 - 4 ) . the peripheral intrahepatic bile ducts are obstructed by numerous parasites that measure 8 - 15 mm in length and 1.5 - 4 mm in width , and so the intrahepatic bile ducts become dilated . however , the extrahepatic bile duct does n't become dilated because it is not easily obstructed by the parasites . there are many other conditions that can occlude the intrahepatic bile ducts , including adenomatous hyperplasia , mucus , periductal fibrosis and stricture ( 4 ) . several investigators ( 2 , 3 , 5 ) have reported that there was no appreciable dilatation of the intrahepatic bile ducts on the ct scans of clonorchiasis patients , but they did n't present any reasons for the absence of dilation . the intrahepatic bile ducts in our patient were not dilated on ct scans , making the preoperative diagnosis of clonorchiasis very difficult . although our patient did not recall any history of raw or partially cooked freshwater fish , we presumed that acute infestation of clonorchiasis caused no appreciable dilatation of the intrahepatic bile ducts on ct . choi et al . ( 3 ) have reported a case of multiple small pyogenic abscesses with diffuse dilatation of the intrahepatic bile ducts . ( 6 ) have also described acute clonorchiasis in a child who had presented with multiple hepatic abscesses and mild dilatation of the biliary tree . to the best of our knowledge , this is the first case in the english medical literature of a hepatic parasitic abscess caused by clonorchiasis without any perceptible dilatation of the intrahepatic bile ducts , as seen on ct scans . seong et al . ( 7 ) reported that the following ct findings favor hepatic abscess over the mass - forming type intrahepatic cholangiocarcinoma : multilayered enhancement , a sharp margin , inner air - density , a cluster sign , an air - biliary gram , a lobulated configuration , atelectasis of the lower lungs , pleural effusion and transient hepatic attenuation difference . in daily practice , however , such differentiation poses difficulty given the considerable overlap of the ct findings . in the present case , the ct findings suggestive of cholangiocarcinoma ( i.e. peripheral enhancement of the mass and lymphadenopathy ) and the absence of any clinical or radiological findings of a hepatic abscess caused great difficulty in making a preoperative diagnosis of hepatic abscess . in summary , we report here on a rare case of a hepatic parasitic abscess that was caused by clonorchiasis without dilatation of the intrahepatic ducts , and this malady mimicked cholangiocarcinoma on the ct images .

clonorchiasis is caused by a chronic infestation of liver flukes , clonorchis sinensis , and these reside mainly in the medium- and small - sized intrahepatic bile ducts . therefore , diffuse , uniform , minimal or mild dilatation of these bile ducts , particularly in the periphery , without dilatation of the extrahepatic bile duct is the typical finding on several imaging modalities . we report here on the ct findings of an unusual case of hepatic parasitic abscess that was caused by clonorchiasis ; this malady mimicked cholangiocarcinoma , and there was no dilatation of the intrahepatic bile ducts .

nonlinear terahertz ( thz ) radiation is the electromagnetic spectrum which ranges from 30 thz to 100 ghz . formerly , bulky and expensive equipment such as free electron lasers or the alternative employment of thermal sources produced weak , incoherent radiation . thz radiation has also been used in tissue with differentiating abilities.1 there is an increasing tendency towards thz technology for the next wave of noninvasive biomedical instruments.2,3 thz pulse has many properties that could encourage the use of thz pulsed imaging ( tpi ) as a medical imaging tool . moreover , thz waves are useful for the analysis of histopathological diagnosis , without any staining process.4 rayleigh scattering of electromagnetic radiation increases with the inverse of the wavelength to the fourth power . however , there is no ionization hazard for biological tissue.5 tpi is a new technique based on broadband pulses of electromagnetic radiation of thz frequencies.6,7 contrast images can be obtained for different degrees of thz wave absorption for normal tissues , such as muscles , fatty tissue and cartilage , as well as cancer tissue . we can obtain thz images using a microring resonator ( mrr ) in a wide range of wavelengths.8,9 the thz pulse interacts with the sample in reflection or transmission modes . the spectra information from thz pulses has been used to distinguish different types of soft tissues , such as muscle , fat , and kidney tissues.10,11 in order to achieve a wide band frequency carrier , we propose a novel system consisting of mrrs for many communication applications such as a wireless thz communication system and faster data transfer , which requires higher carrier frequencies.12 in this paper , we use mrrs made of ingaasp / inp material to enhance the channels of the frequency band for implementation in medical imaging.13 the transfer function of the system uses gaussian beam , described by equation 1.14 here a1 is the amplitude of the optical field and t is the time for phase shift with frequency shift of 0 . the optical outputs from the first and second ring resonators are given by equations 2 and 3.15,16 here is the coupling coefficient , and k represents the wave number in vacuum . is the fractional coupling intensity loss . l1 and l2 are circumferences of the first and second rings . exp(l/2 ) shows the roundtrip loss coefficient and is the waveguide loss . for the panda system , the output optical fields from right and left rings are expressed by equations 4 and 5 . here , er and el are the outputs from the right and left rings of the panda system . the interior output fields of e1 , e2 , e3 , and e4 are shown in equations 69 . therefore , the final equations for drop port and throughput power are given in equations 1013 . where pt and pd represent the output powers of the throughput and drop port , respectively.14,15 in this work we use a gaussian beam as an input power to the proposed system . the new design of the system is illustrated in figure 1 . in this case the gaussian pulse with a center wavelength of 1.3 m , pulse width of 20 ns , and power of 1 w , is an input into the system as shown in figure 2a . the parameters used are r1 = 5 m ( radius of first ring ) , r2 = 3 m ( radius of second ring ) , rl = 1 m ( radius of left ring of panda ) , rr = 1 m ( radius of right ring of panda ) , r = 3 m ( radius of centered ring of panda ) , aeff = 0.100.25 m.2 some fixed parameters such as nonlinear refractive index , n0 = 3.34 ( ingaasp / inp)17 and intensity attenuation coefficient , = 0.2 dbmm have been selected for this system . the input pulse is sliced to a smaller signal through the spectrum shown in figure 2b and c. the light pulse can be chopped into discrete signals and amplified in the first ring , where more signal amplification is obtained by the second ring ( smaller ring ) . output signals of the panda system are simulated and shown in figure 3a f . figure 3e and f show the best region of frequency , which can be seen at the range of 4050 thz . in practice , the output signals from the panda system are inputs to the add / drop filter system in order to cancel out noisy chaotic signals . to retrieve the signals from the chaotic noise , we suggest the use of add / drop filters with proper parameters . two complementary optical circuits of the mrr add / drop filter can be illustrated by equations 14 and 15.17 where et1 and et2 represent the optical fields of the throughput and drop port , respectively . = kneff is the propagation constant , neff is the effective refractive index of the waveguide , l = 2r is the circumference of the ring , and r is the radius of the ring . for simplification , chaotic noise cancellation and required signals can be obtained by using the particular parameters of the add / drop device . 1 and 2 are coupling coefficients of the add / drop filters , n = 2/ is the wave propagation number for a vacuum , where the waveguide loss is = 0.2 db / mm . the fractional coupler intensity loss is = 0.1 . for the add / drop filter device , the nonlinear refractive index is neglected . we used an add / drop filter system with a radius of 10 m to determine the free spectral range , the number of channels and bandwidth as shown in figure 4 . figure 5 shows the simulation results where figure 5a and b represent the throughput and drop port outputs of the system , respectively . as demonstrated in figure 6 , generated dense wavelength - division multiplexing from the proposed configuration of ring resonators are involved in the thz region , which provides a reliable frequency band for medical applications , especially for thz imaging . the main advantage of thz imaging is its diagnostic capabilities.11 these obtained pulses are also useful for analysis of the histopathological diagnosis , without any staining process.6 to date , imaging spectroscopy is used only for small areas.3,4 thz imaging exactly reflects the tissue situation , for instance tumor , nontumor tissues , tissue degeneration , and fibrosis . the thz image shows significantly reduced absorption of thz radiation in this region compared with normal tissue , which suggests its usefulness for detecting tumors ( figure 7 ) . contrast images associated with different degrees of absorption of thz waves have been obtained for normal tissues , such as muscles , fatty tissue , and cartilage , as well as for cancer tissue . we could obtain thz images of large areas from an mrr in a wide range of wavelengths.8,10,11 in this study , a new mrr design has been introduced , which provides the best frequency band when the input gaussian pulse is used . this system consists of a series of mrrs connected to a panda system . using the proposed system , multifrequency bands can be generated and simultaneously linked to an add / drop filter where it is suitable for analysis of the histopathological diagnosis . this range provides a reliable frequency band for medical purposes , especially in thz imaging .

in this study , we have generated terahertz ( thz ) frequency by a novel design of microring resonators for medical applications . the dense wavelength - division multiplexing can be generated and obtained by using a gaussian pulse propagating within a modified panda ring resonator and an add / drop filter system . our results show that the thz frequency region can be obtained between 4050 thz . this area of frequency provides a reliable frequency band for thz pulsed imaging .

congenital mesenchymal hamartoma of the chest wall ( mh ) , synonymous and partly incorrectly named as congenital mesenchymoma , is a rare benign lesion which usually presents antenatally or during early infancy . however large thoracic wall resections in the growing child subsequently result in deformations of the thoracic cage and spine . to avoid these long term sequels conservative management of patients has been suggested . however this option may be of limited value in infants with extended cystic and enlarging mh . after an uncomplicated pregnancy antenatal routine obstetrical ultrasonography of the fetus at 39 weeks of age had revealed an extensive chest mass . intrauterine magnetic resonance imaging ( mri ) depicted the presence of a large heterogeneous mass of 7 x 5.3 cm occupying the fetus right thorax . the baby was delivered by caesarean section with a weight of 3170 g. apgar scores were 5/7/8 and postnatal oxygen supplementation was applied . the baby was in a stable condition , however , a protrusion of the right chest wall was obvious ( figure 1 ( fig . 1 ) ) . chest x - ray showed a large cloudy calcificated mass extending within the right hemithorax shifting the mediastinum to the left . the lesion destructed parts of the 6 to 9 rib and displaced the right lung , vessels and the mediastinum to the contralateral side . histopathology revealed cartilage with expression of protein s100 and a low proliferation rate ( mib-1 ) . furthermore , proliferating spindle cells , osteoclast - like giant cells , newly generated fibrous ossifications as well as ectatic vascular spaces were found ( figure 2 ( fig . 2 ) ) . postnatal mri showed a multicystic mass of 6.7 x 5.8 x 5.6 cm arising from the thoracic wall and consisting of large fluid contained cavities . histopathology results and imaging both were consistent with the diagnosis of a congenital mesenchymal chest wall hamartoma . as the baby was in a fair condition a conservative treatment was favored . at three weeks of age in the course of the follow up examination at 5 weeks of age we noticed an anemia with the necessity of a transfusion ( hemoglobin 6.8 g / dl ) . the mri disclosed an increase in size to 9.3 x 8.1 x 7 cm due to hemorrhage . this enlargement was accompanied by shifting the large vessels to the left side and compressing the inferior vena cava . sedimentation levels within the large cystic spaces of the hamartoma indicated intralesional bleeding ( figure 3 ( fig . an extended resection of the thoracic wall encompassing the ribs 7 through 9 were nearly completely as well as parts of the 6 and 10 rib . chest wall reconstruction was facilitated by preservation of the outer musculature and the use of a bovine pericardium patch . she showed a slight deformity of the right thoracic wall due to a bony defects of the ribs without concomitant deformation of the spine . the mass arises from the central part of the ribs . in the recent literature nearly 80 cases usually the mass presents in early infancy although some cases with presentation in adults were observed . diagnosis is based on imaging ( chest x - ray , mri ) and histopathological findings . typically the lesion is large and well delineated but compresses the surrounding organs and results in a deformity of the thoracic wall . following an initial period of rapid growth between the 28 and 36 week of gestation discontinuity of enlargement or spontaneous regression different therapeutic approaches have been reported in the literature : the majority of the hamartomas has been excised by primary intuition or secondary due to respiratory distress , cardiovascular compression or neurological symptoms . however a few authors described conservative management and a spontaneous regression supporting the concept to be appropriate for children with minor symptoms , . the mh was left in place and follow up at the age of 6 years revealed some reduction in size after conservative management . shimotake et al . described a newborn with a large mh occupying the left hemithorax resulting in severe respiratory insufficiency . as conclusion the authors advocate conservative management after confirmation of the diagnosis . only cameron et al . reported a case comparable to our experience . initially this infant was managed conservatively but in the age of 5 month the tumor increased in size . intralesional bleeding lead to increasing compression of the lung and excisison of the mh . based on the suggestions of these case reports we initially favored a conservative management in the presented case with avoiding the risks of a destructive en - bloc resection and the risk of long term complications as impaired thoracic growth and postsurgical scoliosis . different signal intensities of the cyst content with fluid - fluid levels are a well known feature of mh in mri and ct . this bleeding can lead to an enlargement of the tumor size and compression of the lung as reported above . one patient underwent partial tumor resection because of profuse bleeding in the course of open biopsy . in the presented infant the intralesional bleeding resulted in repeated severe anemia with worsening of the patient s condition . after stabilization we performed the resection of this lesion . in conclusion conservative management of mh requires close follow - up examinations of the patient to recognize potential life threatening complications .

we report a case with prenatally diagnosed large cystic - solid mesenchymal chest wall hamartoma . an attempt of conservative management was made however repeated intralesional hemorrhage led to enlargement and severe anemia which required urgent resection at the age of 8 weeks . the infant had an unimpaired development over a follow - up of 4 years .

an individual , as a consumer researcher , wants to buy a refrigerator . with appliances of most sorts , people regard best practice decision making as consulting trustworthy comparison websites and magazines , ones that go beyond expressing opinions , or recording likes , to numerically rate the alternative products on a set of attributes or criteria . they want these decision support tools to give them ratings that can be trusted because they are produced free of any conflict of interest or other biases the consumer does not know , and does not want to know , why this refrigerator is given a 4*/80% rating on reliability , and a 3*/60% rating on environmental impact , and another one the opposite ratings . feeling justified in assuming a common sense , lay understanding of the terms reliability and environmental impact , they do not have neither the time nor motivation to find out more about what these concepts mean , in terms of the mechanical functioning of the refrigerator , the quality of its components , the emissions it produces or whatever else contributes to these ratings made by the expert assessors some may wish to establish whether consumers have made an informed decision by seeing how well they score on a test of refrigerator knowledge . giving considerable and fixed weight to knowledge in their measures of decision quality , consumers decisions might be regarded as poor quality , because their knowledge sub - score is low . in contrast , consumers may regard themselves as having made good decisions , indeed the best possible decisions they could make , given the time and cognitive effort they are willing to devote to research into the decision - making process including accessing and accumulating knowledge deemed important , even essential , by others . but surely health care decisions are different from buying refrigerators ? choosing between surgery and medical options for newly - diagnosed cancer , or pain management for chronic osteoarthritis , is not like buying a household appliance , is it ? in fact , nothing really changes for the individual , whom we now conceptualize as a researcher conducting a continuing , informal n - of-1 study into his / her health . the affective and emotional differences between the two situations may well produce differences in the decision - making process , but the patient accepts that this will not necessarily enhance the quality of the decision as he / she defines it . patients may become interested in finding out more about their medical condition than they would about refrigerators , and actually do so . but unless it leads to a change in a performance rating for an available option on one of their criteria especially the bean for a criterion they weigh heavily the additional information they now possess is decision - neutral . people - as - researchers may feel better informed in some sense , but they realize they will not necessarily be in a position to make a better decision and therefore have not ended up more decisionally empowered . they may even simply have become more anxious and regretful about the opportunity costs of acquiring the information , in the form of the foregone benefits from other activities in which they could have engaged . informed decision ? according to themselves and us , absolutely , since they have consulted a transparent set of option performance ratings on relevant criteria , originating from a source that they have decided is the most trustworthy . their decision quality score may well be low according to an instrument that weights highly the knowledge that they are assumed by others to need to make an the growing number of condition - specific decision quality instruments being developed , notably by karen sepucha and colleagues , all give very heavy weight to a knowledge subcomponent . there could be no clearer confirmation of the issue at stake here than the title of one of the background papers to these projects : how does feeling informed relate to being informed? trust is crucial here . in either shared or unshared decision making , trust relates to the inputs into decision making , since we have left behind the notion of an agency relationship , previously dominant in conceptualising medical practice . trust is always a matter of degree , rather than a binary all or nothing , whether it relates to the beans provided by the clinician , or by a decision support tool . even if there is only one , dubious , source , it will be the most trustworthy . unless , that is , the person rates his / her own estimates as more trustworthy than the best source , since so we envisage an individual regarding the respected consumer magazine s beans on refrigerators as the most trustworthy in relation to that purchase decision . people s task in health care decisions , given a restricted willingness to devote time and energy to processing information , is to assess the trustworthiness of the available sources of beans for the outcomes and other criteria important to them . they would expect a clinician , or a team developing the ratings for a decision aid , to be highly trustworthy and to be provided with evidence for this , especially in the case of an aid . the key information the person - as - researcher requires is labelling that ensures he / she will get what it says on the tin when they open an aid . with this meta - information , they can make an informed choice about which tins of what size to open . the other major problem with any imposed information requirement is that it condemns many on the continuum of health literacy , and especially health numeracy , to receiving little or no help . we fully support attempts to reduce health illiteracy and innumeracy , especially their decision - focused forms . however , it is too much to expect of a decision support tool or a clinician to overcome the limitations of previous education and socialization in these respects . moreover , it is important to accept that even if aid users are able to register and report the relative numbers of sad and smiley faces in frequency diagrams , or repeat back 1 in x statements about which there is considerable doubt this does not in any way ensure that they can meaningfully incorporate the numerical probabilities they have correctly registered ( say 10% and .05% , or 1 in 10 and 1 in 2000 ) , into their decisions . this is not to say that a decision aid should not contain help in this respect , including guidance on how the person can best avail themselves of what it offers , and information on the bases of that offering . it is to suggest that much of this should be provided on an opt - in basis . nothing in what we have said is intended to imply that the community is not entitled to apply community - level criteria and weights to what it provides , or allows to be provided , to whom , under what conditions , and at what cost , in the pursuit of goals such as efficiency , equity and justice . formal laws and regulations ( including those on informed consent and clinician liability ) and resource allocation policies ( including reimbursement decisions ) will be the context in which the individual decision is made , and they will frequently be in conflict with what an individual sees as best for him / herself , given personal criteria and weights . external consequences for others may trump individuals preferences , as in the case of infectious diseases . trickier are the issues of social responsibility or morality which are not dealt with formally . apart from issues of environmental and social impact ( such as those arising from hormonal treatments and opioids ) , there are all those that arise in resource - constrained and interdependent systems simply as a result of those constraints and interdependencies . in these cases , we say two things . first , it is not the function of individual decision support tools to mandate the inclusion or exclusion of social criteria in an individual s set , such as concern for others health , or insist that these be given specific weights . second , that in order to be regarded as having made a high - quality decision , the individual should not be required to be informed about the social criteria they do select , other than having the processed beans available to them from a trustworthy source . normative checklists for decision support tools , such as those constructed in accordance with the guidelines of the ipdasi collaboration , are clearly intended to promote person - as - patient empowerment . but most decision aids that comply with these guidelines are designed for use only within the context of shared decision making , in which the person is assigned the status of patient . in many cases , the support can be accessed only within the clinical encounter , or with provider permission . they all perpetuate the idea that only a decision informed in a particular way and to a particular extent can be a good decision . we do not need the concept of an informed decision , only that of a good , better or best possible decision . for none of these will there be a definition that is not multi - dimensional and therefore preference - sensitive . there can only be one answer : the patient s or the person s if they are not a patient .

most guidelines for clinical practice , and especially those for the construction of decision support tools , assume that the individual person ( the patient ) needs to be in possession of information of particular sorts and amount in order to qualify as having made an informed decision. this often implicitly segues into the patient having made a good decision. in person - centred health care , whether , in what form , and with what weight , information is included as a criterion of decision quality is a matter for the person involved , to decide in the light of their own values , preferences , and time and resource constraints .

the european nucleotide archive ( ena ) operates as a public archive for nucleotide sequence data . by bringing together databases for raw sequence data , assembly information and functional annotation , the ena provides a comprehensive and integrated resource for this fundamental source of biological information . central to the ena is the provision of submission services , including interactive and programmatic submission tools , search services , including text and sequence similarity search tools and data presentation and retrieval services . the ena works closely together with ncbi ( 1 ) and ddbj ( 2 ) as partners in the international nucleotide sequence database collaboration ( 3 ) . the principal policy of insdc is to provide free and unrestricted permanent access to all archived nucleotide data . all primary data in the insdc belongs to the submitters and can only be updated with submitter consent . for full policy details please refer to : http://www.insdc.org/policy.html . in october 2010 , the ena contained 500 billion raw and assembled sequences consisting of 50 trillion base pairs . in the last 3 years , the next - generation sequence reads stored in the sequence read archive ( sra ) have become the largest and fastest growing source of new data accounting now for 95% of all base pairs made available by ena . at the same time , the number of completed genome sequences has risen to over 1400 for cellular organisms and 3000 for viruses and phages ( http://www.ebi.ac.uk/genomes/ ) . new submitters should contact datasubs@ebi.ac.uk for the creation of a submission account and a secure data upload area . submitters first upload data files into the secure data - upload area in one of the supported data formats , then prepare and submit study , sample , experiment , run and submission xml files to sra . we have extended the sra submission service to support submissions of authorized access data , typically clinical samples that have been sequenced under a confidentiality and consent agreement . authorized access data can now be submitted through the sra submission service into the european genome - phenome archive ( ega ; http://www.ebi.ac.uk/ega ) . data submitted to ega are not part of the public sra database and are excluded from the insdc data exchange . permission to view and retrieve authorised access data can only be granted by the external data access committee ( dac ) responsible for the data concerned . a secure data upload area is required to submit authorised access data through the sra submission service . it is also possible to submit ega s policy , dataset and dac objects through sra . sra will shortly accept sequence read submissions in binary alignment / map ( bam ) format ( 4 ) . a bam file is a binary compressed representation of the sequence alignment / map ( sam ) format . with sequence read alignments becoming an increasingly common intermediate in primary analysis , bam format is emerging as a popular choice for storing sequence reads with alignments . the sra is currently finalizing an archive bam specification which will standardize the use of bam files for primary data archival purposes . once completed , bam submissions to sra archives will be required to follow this specification . embl - bank is a comprehensive public database of nucleotide sequences , associated biological annotation and bibliographic information . it contains a large diversity of data from patent , expressed sequence tag , whole genome shotgun and other high - throughput sequences , through genomic assemblies and richly annotated sequence fragments to whole replicons ( 5 ) . we have extended the web - based embl - bank submission service in a number of ways . for providers of genome - scale data , we have added functionality that allows data submissions in embl - bank flat file format . for smaller scale submissions each template focuses on a particular commonly occurring type of sequence and annotation data and collects required information from the submitters using a web form or spreadsheet upload . new templates are available for unannotated wgs submissions with only source organism annotation , and for protein coding and phylogenetic - marker regions . the template mechanism , introduced in 2009 , has been well received and attracts now up to half of all web - based embl - bank submissions . ena data can be browsed and retrieved in xml , html , fasta , fastq and flat file formats using the ena browser which can be used both interactively and programmatically through rest urls . in 2010 , we extended the ena browser to cover embl - bank and trace archive records and introduced several improvements including a graphical embl - bank annotation and assembly viewer and intuitive navigation between different ena data classes . for full details of the ena browser url syntax please refer to : http://www.ebi.ac.uk/ena/about/page.php?page=browser . for example , the following url returns the complete mitochondrial genome for ursus spelaeus ( cave bear ) ( 6 ) : http://www.ebi.ac.uk/ena/data/view/fm177760 ( figure 1 ) . data can be queried using the eb - eye free text search functionality available in the header section of all ebi web pages ( 7 ) . ena results are available under the nucleotide sequences category and linked to the ena browser . figure 1.the complete mitochondrial genome for ursus spelaeus ( cave bear ) from the max planck institute for evolutionary anthropology submitted to embl - bank in 2010 . the complete mitochondrial genome for ursus spelaeus ( cave bear ) from the max planck institute for evolutionary anthropology submitted to embl - bank in 2010 . rapid and comprehensive sequence similarity searches against ena data are supported through a new service based on exonerate ( 8) technology : http://www.ebi.ac.uk/ena/search/ ( goodgame , n. , manuscript in preparation ) . all nucleotide sequences archived by the insdc and made available as part of embl - bank are covered by our service . experimental search support for a limited number of raw reads is provided through de - bruijn servers based on velvet ( 9 ) , using the exonerate client - server protocol and being fully integrated with our search service . the embl - bank sequence search service is currently being expanded for more specific purposes according to community requests . bulk download of embl - bank data is supported through ftp at ftp://ftp.ebi.ac.uk/pub/databases/embl/ , and sra and trace archive data through ftp at ftp://ftp.sra.ebi.ac.uk/ and aspera through fasp.sra.ebi.ac.uk . the ena team welcomes feedback and suggestions relating to all of our services at datasubs@ebi.ac.uk . we are always interested in hearing from potential collaborators who have an interest in working with and integrating our services . the ena is funded by the european molecular biology laboratory , european commission and the wellcome trust . funding for open access charge : european molecular biology laboratory . conflict of interest statement .

the european nucleotide archive ( ena ; http://www.ebi.ac.uk/ena ) is europe s primary nucleotide - sequence repository . the ena consists of three main databases : the sequence read archive ( sra ) , the trace archive and embl - bank . the objective of ena is to support and promote the use of nucleotide sequencing as an experimental research platform by providing data submission , archive , search and download services . in this article , we outline these services and describe major changes and improvements introduced during 2010 . these include extended embl - bank and sra - data submission services , extended ena browser functionality , support for submitting data to the european genome - phenome archive ( ega ) through sra , and the launch of a new sequence similarity search service .

trichoepithelioma is a benign hamartomatous tumor originating from the pilosebaceous follicle that usually appears in childhood or early adolescence . trichoepithelioma is a benign hamartomatous tumor of the pilosebaceous follicle occuring either as a nonhereditary solitary lesion or as multiple lesions that are often dominantly inherited . with an unknown prevalence multiple trichoepitheliomas ( mts ) emerge as multiple skin colored to pink , firm , and papulonodular lesions , which are sited mainly on the face or occasionally on the scalp , neck , or upper trunk , gradually increase in number and size . malignant transformation to basal cell carcinoma ( bcc ) is unusual and occurs late in the course of the disease . mts are usually inherited as an autosomal dominant disease ; we report a sporadic case of nonfamilial mts in a 50-year - old female . a far - reaching search of english literature did not reveal any nonfamilial case of mts . a 50-year - old female presented with asymptomatic multiple skin colored raised papulonodular lesions over the face , upper back , and extremities . the lesions started from the age of 3 years and continued to appear until date . multiple discrete to coalescing skin to yellow coloured waxy dome shaped papules and nodules of varying sizes were present over face including eyelids and eyebrows , external ear , scalp , neck , chest , back and lower limb [ figures 1 and 2 ] . few of the nodules over nose and cheeks were crusted and surrounded by a zone of telengiectasia . skin biopsy was performed from two different sites viz a waxy nodule and from a crusted lesion over the face . both biopsies revealed tumour nodule in the dermis comprised of lobules of small dark blue staining cells , at places shows peripheral palisading and follicular differentiation . mitotic activity of the nodule and the crusted lesion was insignificant [ figure 4 ] . multiple discrete to coalescing skin to yellow colored waxy dome shaped papules and nodules of varying sizes present over face dome shaped papules and nodules involving the external ear beaded papules over eyelids hematoxylin and eosin staining of low power ( 10 ) presents with tumor nodule in the dermis comprising of lobules of small dark blue staining cells , at places showing peripheral palisading and follicular differentiation no treatment was offered , as surgical excision will lead to multiple scars and the effective nonsurgical treatment is currently not available in our center . trichoepithelioma is a benign hamartomatous tumour originationg from the pilosebaceous follicle that usually appears in childhood or early adolescence . originally , it was described by brooke in 1892 as epithelioma adenoides cysticum and fordyce coined the term multiple benign cystic epithelioma . copious trichoepitheliomas occur in an autosomal dominantly inherited genodermatosis called multiple familial trichoepithelioma ( mft ) or familial trichoepithelioma ( ft ) . johnson et al . reported mft in two brothers with linkage and mutational analyses in support of ft . trichoepitheliomas predominatly occurs on face , nasolabial folds , nose , upper lip , forehead , and eyelids , occasionally , on the scalp , neck , or upper trunk . trichoepithelomas begin to develop as multiple skin coloured to pink , firm , rounded , transluscent , shiny , well demarcarted papulonodular lesions , centre may be slightly depressed . although trichoepitheliomas are unassociated with malignant conditions , rare alliance with bcc has been reported by various authors . if needed , genetic studies may be used to detect the abnormalities in band 9p21 . mts form a part of rare syndromes which include rombo 's syndrome ( vermicular atrophoderma , milia , trichoepithelioma , hypotrichosis , bccs , and peripheral vasodilation with cyanosis ) and basex syndrome ( follicular atrophoderma , trichoepithelioma , hypotrichosis , bccs , and hypohydrosis ) . differential diagnosis of trichoepithelioma includes bcc , syringoma , eccrine poroma , eccrine nevus , commedonal nevus and other appendeceal tumors . histopathology typically shows horn cysts , tumor islands composed of basophilic cells of basaloid appearance arranged in peripheral palisading pattern are the major characteristic components of multiple trichoepithelioma . these basophilic cells lack atypia and mitosis . whereas the keratinization is abrupt and complete as opposed to horn pearls seen in squamous cell carcinoma . the presence of beaded waxy papules on the upper lid margin in our case highlights the possibility of lipoid protenosis ( lip ) . however , the absence of hoarseness of voice , pox like and acneform scars along with infiltration of skin and mucous membranes excludes the possibility of lipoid proteinosis . moreover , lip histologically is characterized by disruption of the basement membrane and deposition of hyaline material at the level of the basement membrane ( resulting in its thickening at the dermo - epidermal junction ) , papillary dermis , surrounding capillaries , and around adnexal epithelia , especially sweat coils . various treatment modalities are been practised which include surgical excision , chemical cautrization , dermabrasion , cryosurgery , and laser resurfacing with promising results . in a single case report an 11-year - old girl was treated with topical imiquimod cream and tretinoin 1% gel over 3 years period which showed remarkable reduction of lesions without scars . however in another report , isotretinoin for 12 weeks failed to affect the trichoepitheliomas of a patient with concurrent cystic acne . mts is a relatively an uncommon disease and diagnosis is usually based on its clinical appearance with a strong family history . the uniqueness of our case lies in the finding that besides being an autosomal dominant disease mts can occur as sporadic disorder , after an extensive search of english literature no case until date has been reported as multiple nonfamilial trichoepitheliomas . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the cases so far reported of multiple trichoepitheliomas are of familial type but this is of nonfamilial variety , not accounted till date . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the cases so far reported of multiple trichoepitheliomas are of familial type but this is of nonfamilial variety , not accounted till date . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the cases so far reported of multiple trichoepitheliomas are of familial type but this is of nonfamilial variety , not accounted till date .

trichoepithelioma is a rare benign adnexal tumor which may be of solitary nonfamilial type or multiple familial trichoepitheliomas . here , we describe a rare presentation of nonfamilial case of trichoepithelioma in a 50-year - old female with multiple skin colored facial papules and nodules over the face , upper back , and extremities .

the syndrome of multiple enchondromas and subcutaneous hemangiomas , known as maffucci syndrome , is very rare , accounting for about 200 cases during the last 140 years . the presence of visceral hemangiomas in this disease is exceedingly rare ; for this reason we believe it to be of interest to report a case of hemangioma localized in the liver in a patient with maffucci syndrome . to our knowledge , this is the first description of the presence of a hemangioma localized in the liver in a syndrome of multiple enchondromas and subcutaneous hemangiomas . b.m . is a 51-year - old female affected by maffucci syndrome with skeletal deformities involving both arms , the left scapula and chest and having variable sized deep bluish nodules on the fingers of both hands . the first lesion localized on the left arm was noted at the age of 6 years . at the age of 30 years she underwent surgery for the rupture of the vascular lesion localized at the 5th left finger ; the histology of the surgical specimen showed multiple blood - filled cavities lined with a single layer of endothelial cells . the patient was also operated at the age of 40 years because of a chondrosarcoma of the left humerus . she is also affected by hypertension , for which she is under pharmacological treatment with beta blockers and diuretics . the patient was admitted to our hospital 15 days after an ultrasound examination performed as a routine follow - up for her disease . this examination showed a hepatic lesion which had never been identified in previous follow - ups . on admission , the patient was asymptomatic and clinical examination did not reveal any alterations apart from those compatible with her disease , namely limitation of movement and chest abnormalities . her blood pressure was 130/80 mm hg and no alterations were observed in the electrocardiogram . routine blood analysis revealed an increase of ggt ( 124 u / l , normal values < 50 u / l ) and of alkaline phosphatase concentrations ( 330 u / l , normal values 98280 u / l ) . chest x - ray showed postsurgical alterations of the left shoulder and irregularly shaped , radiolucent areas with stippled calcification within the right humerus ; costal dyschondroplasias were also found . ultrasonographic examination confirmed a dishomogenous neoformation having a size of 11 10 cm localized at the iv hepatic segment with hyperisoechogenic pattern and several anechogenic areolae ( fig . 1 ) ; ultrasonographic color doppler examination of this lesion showed peripheral arterial vascular signals . an ultrasonographic real - time perfusion imaging study was performed with an esatune equipment ( esaote s.p.a . , genoa , italy ) using a convex probe of 3.5 mhz in order to better define the hepatic lesion . the esatune was coupled with the cnti ( contrast tuned imaging ) technique , which is a combined hardware and software product for imaging enhancement . a second generation of contrast agent ( sonovue , bracco international b.v . , amsterdam , the netherlands ) was also used : a bolus of 2.5 ml of sonovue was rapidly injected i.v . in the left antecubital vein using a 20 g needle ; this bolus was immediately followed by i.v . this examination showed , during the arterial phase , a globular enhancement at the periphery of the lesion with progressive centripetal fill - in in the portal and parenchymal phase ( fig . 2 ) . because the patient had an internal orthopedic device , we did not perform a mri ; in order to confirm the ultrasonographic findings , a spiral computed tomography with i.v . the computed tomography examination showed an area with a centripetally advancing border of enhancement ; this area then became progressively and homogeneously hyperdense . the patient was discharged from our department with the diagnosis of hepatic hemangioma and one month later a new ultrasonographic examination showed that the dimension of the hepatic hemangioma was unchanged . this syndrome appears to be unrelated to race and sex and patients are usually normal at birth . the presenting symptoms may be skeletal or vascular and usually occur in infancy as in our patient . the extent of skeletal involvement is variable in enchondromatosis and may include dysplasia which is not directly attributable to enchondromas . enchondromas are usually in close proximity to , or in continuity with , growth plate cartilage . consequently , they may result from abnormal regulation of proliferation and terminal differentiation of chondrocytes in the adjoining growth plate . maffucci syndrome may be associated with three types of vascular lesions : cavernous hemangiomas , phlebectasias and lymphangiectasias - lymphangiomas . clinical problems caused by enchondromas include skeletal deformity and the potential for malignant change reported in approximately 30% of reported cases , with chondrosarcomas being the most common : our patient developed this latter complication at the age of 40 years when she was operated on for a chondrosarcoma localized in the left humerus . however , involvement of the digestive system and the abdominal organs has rarely been reported in patients with maffucci syndrome : until now hemangiomas of the tongue have been described in one patient , in the oral cavity in a second patient , in both the oral cavity and the colon in a third patient , and in the spleen in a fourth patient . the case we report is the first description of a hepatic hemangioma in a patient with maffucci syndrome . another characteristic of our case is that the diagnosis of hemangioma of the liver was made using a real - time perfusion imaging ultrasound with a contrast agent , whereas mri was carried out to diagnose the hemangioma of the spleen in the case reported by ahmed et al . . we believe that real - time perfusion imaging with an ultrasound contrast agent is another possible technique in diagnosing hemangioma localized in abdominal parenchymal organs in patients with maffucci syndrome , especially in those patients in whom mri can not be carried out .

the presence of visceral hemangiomas in the syndrome of multiple enchondromas and subcutaneous hemangiomas , also named maffucci syndrome , is exceedingly rare ; until now noncutaneous hemangiomas have been described in 4 patients : they were found in the tongue in one patient , in the oral cavity in the second patient , in both the oral cavity and the colon in the third patient , and in the spleen in the fourth patient . we report the first case of hemangioma localized in the liver in a patient with maffucci syndrome ; furthermore , due to the impossibility of carrying out an mri to define the hepatic lesion , an ultrasonographic real - time perfusion imaging study with a contrast agent was performed .

paravalvular leaks ( pvls ) are common complications of patients who had a history of valve replacement . it can be seen in 2% to 10% of prosthetic valve in the aortic position . redo valve surgery is the gold standard therapy , but carries high risks for the patients . morbidity - mortality rates are high after a surgical reoperation especially on the vast majority of elderly patients . with the advancement of device technology and imaging modalities , we report our experience with percutaneous transcatheter closure of pvl in a patient who had a history of recurrent hospitalizations diagnosed as decompensated heart failure . a 57-year - old male patient who presented with new york heart association class iii iv dyspnea and massive ascites , was admitted to our clinic . he had a history of mitral valve replacement for serious mitral insufficiency and aortic valve replacement for severe calcific aortic stenosis 3 years ago . he had repetitive hospitalizations due to decompensated heart failure for a period of 3 months . on examination , he had a regular pulse rate of 82 beats / min , and blood pressure of 120/70 mmhg . the cardiopulmonary examination revealed a grade 2/6 early diastolic decrescendo murmur , clear prosthetic click and bilateral crackles in the lung bases . the transthoracic and transesophageal echocardiography ( tee ) showed mildly decreased left ventricular systolic function with left ventricular hypertrophy and aortic metallic prosthetic valve with severe paravalvuler insufficiency at the edge of the sewing ring [ figures 1 and 2 ] . he denied the options of redo operation and percutaneous closure of the defect were discussed in every detail . warfarin was stopped before the procedure and his international normalized ratio at the time of the procedure was 1 , 9 . severe paravalvuler insufficiency at the edge of the sewing ring diagnosed with tee releasing of adoii device by observing the disappearence of leak the procedure was performed under tee guidance using general anesthesia . right femoral artery puncture was performed , and paravalvular aortic regurgitation was evaluated by aortography . leak was passed by a hydrophilic terumo 0.35 inch guide wire and 5 f delivery catheter was placed into left ventricle . paravalvular aortic leak diameter was calculated using tee and 4 mm amplatzer duct occluder ( ado ) ii was chosen . ado ii was loaded into the delivery system and left ventricle side was opened first , and aortic side was opened secondly . during the opening of aortic side , disappearance of leak was observed , and device was released [ figure 3 ] . there was not any complication , and the patient was discharged from the hospital after 2 days . , echocardiography has demonstrated more and more prosthetic valves leakage and hence , the cardiologists can recognize such problem easily . . however , 1 - 5% of patients with prosthetic valves present with clinically significant pvl . symptoms may be due to regurgitation , with reduced effective cardiac output , or congestive failure , resulting in decreased exercise tolerance and dyspnea . nevertheless , the symptoms may be due to hemolytic anemia as a result of red cell fragmentation in the high shear stress regurgitant jet . in the literature , there are large number of cases with pvl which where closed with different approaches and devices . however , it is important to minimize processing with fluoroscopy since radiation causes serious health problems . a recent published study with ado device utilized electrocardiography - gated computed tomographic angiography with 2de or 3de tee reconstruction for patients with aortic pvls . in this large trial , the procedure was successful and 6 , 12 , 18-month survival rates were quite good . smaller trials used other devices such as atrial septal occluders and patent ductus arteriosus occluders and coils were used with only 2d dimensional tee and obtained reasonable degree of technical success and clinical improvement but long flouroscopy times were the biggest problems . in another trial with five patients , different approaches ( transapical - mitral , retrograde - aortic ) and different device ( vascular plug iii ) were used with three - dimensional tee guidance and results were good . the present report is an example of a successful repair of paravalvular aortic prosthesis leakage using ado ii . paravalvular aortic insufficiency is a complication of surgical aortic valve replacement and it is associated with poor prognosis . as in our case , paravalvular aortic leakage causes the persistence of congestive heart failure , and percutaneous aortic leakage repair is a good alternative to the redo surgery .

paravalvular leaks ( pvls ) are a well - recognized complication of prosthetic valve replacement . perivalvular prosthetic regurgitation causes significant morbidity and is associated with high perioperative mortality if open surgical repair is required . pvls manifest with symptoms of congestive heart failure , hemolysis , or in most cases , the combination of both . in recent years , the development of imaging and device techniques significantly shortened the duration of fluoroscopy and procedure success was achieved . percutaneous transcatheter closure of pvls with a specific device causes symptomatic improvement . we present a case of transcatheter closure of aortic paravalvular insufficiency with amplatzer duct occluder 2 device .

in an observational study published in the previous issue of critical care , elseviers and colleagues report that the mortality of critically ill patients treated with renal replacement therapy ( rrt ) for acute kidney injury ( aki ) is much higher than that of those treated by a conservative strategy ( that is , without rrt ) . rrt remains an independent factor associated with a higher mortality after adjustments for acute disease severity ( risk ratio [ rr ] 1.73 , 95% confidence interval [ ci ] 1.4 to 2.2 ) based on the stuivenberg hospital acute renal failure ( sharf ) score as well as other corrections for usually well - established prognostic factors ( age , sex , sequential organ failure assessment [ sofa ] score , type of aki , delayed admission , and clinical conditions ) . this observation might have two alternative explanations : first , rrt per se could worsen the prognosis of acutely ill patients experiencing aki ; second , aki of patients treated by rrt was more severe and this greater severity is not fully reflected by the severity scores and adjustment factors used in the multivariable models of the study . in fact , the role of aki , as an independent factor for mortality , is currently well documented , regardless of the severity of aki . the subpopulation requiring rrt in the intensive care unit represents the more severe population , and the need for rrt appears to be an independent risk factor for death . the specific role of rrt was first proposed by guerin and colleagues in their french epidemiological study . indeed , they reported , in a multiple logistic regression analysis , that the absence of hemodialysis in their severe aki population ( serum creatinine [ scr ] of greater than 300 mol / l , urine output of less than 500 ml/24 hours , or the need for hemodialysis ) was a significant predictor of survival ( odds ratio 1.78 , 95% ci 1.05 to 3.04 ; p 0.032 ) . this finding is of paramount importance given the current trend to initiate rrt early in the course of aki . moreover , recent epidemiological or prospective controlled studies show that the main criteria for rrt initiation are based on low urine output prior to a marked increase in scr or serum urea level . rrt remains associated with a high mortality , and given the lack of survival improvement using continuous rrt or augmented delivered dose , early initiation of rrt might be promising . actually , numerous retrospective studies report a better outcome with earlier initiation , but conflicting results are reported by other studies . finally , a recent meta - analysis shows a barely significant decrease in mortality using early initiation of rrt in prospective studies ( rr 0.64 , 95% ci 0.40 to 1.05 ; p < 0.08 ) and a significant decrease in mortality using early initiation of rrt in observational studies ( rr 0.72 , 95% ci 0.64 to 0.82 ; p < 0.001 ) . this new strategy seems attractive , but regarding the lack of strong data in favor of any beneficial effect , we should pay heed to the potential adverse effects . unfortunately , the study by elseviers and colleagues presents many shortcomings , which hamper any definitive conclusion . first , the study is an observational trial and no prespecified criteria regarding rrt indications and the timing of initiation were provided in the dierent centers . this shortcoming may explain the heterogeneity in the rate of patients treated with rrt between the different centers and the associated mortality . second , adjustment criteria did not take into account specific aki characteristics , like oliguria , or specific prognostic scores based on metabolic disturbances ( that is , rifle [ risk , injury , failure , loss , and end - stage kidney disease ] or akin [ acute kidney injury network ] ) . we can guess that intensivists were prompt to initiate rrt in patients with oliguria or severe metabolic abnormalities and thereby to select a more severe population . the higher mortality could be linked to the aki severity itself rather than to a specific effect of rrt . the real problem in clinical practice is the early detection of aki patients for whom rrt will be mandatory . in this population , we could probably start rrt early , whereas in the others , we could delay the initiation . as we wait for a large prospective study to test the benefit of early initiation of rrt , it seems reasonable , in the absence of a current clinical or biological marker of rrt requirement , to keep in mind that widespread use of early initiation may lead to an excess risk of complications and perhaps to a higher mortality than expected . aki : acute kidney injury ; ci : confidence interval ; rr : risk ratio ; rrt : renal replacement therapy ; scr : serum creatinine .

in an observational multicenter study , elseviers and colleagues report that renal replacement therapy ( rrt ) in acutely ill patients treated for acute kidney injury is an independent risk factor for death . this result may question the benefit of the current practice of early rrt initiation .

the first us case associated with the epidemic in hispaniola was laboratory confirmed on november 15 , 2010 , in a us resident who had traveled to haiti and returned to florida . the first case in a patient with history of travel to dominican republic was laboratory confirmed on january 29 , 2011 . as of april 4 , 2011 , a total of 23 cholera cases associated with the hispaniola epidemic had been confirmed ( figure 1 ) . patients resided in florida ( 10 ) , massachusetts ( 4 ) , new york city ( 4 ) , kansas ( 1 ) , michigan ( 1 ) , north carolina ( 1 ) , virginia ( 1 ) , and texas ( 1 ) ( figure 2 ) . illness onset dates ranged from october 23 , 2010 , to february 2 , 2011 . median age was 38 years ( range 984 years ) , and 43% were female patients . confirmed cholera cases ( n = 23 ) , by onset date and travel history , united states , october 21 , 2010february 4 , 2011 . geographic distribution of cholera cases in the united states associated with hispaniola , october 21 , 2010april 4 , 2011 . all patients were treated with antimicrobial agents , rehydration , or both ; 9 ( 39% ) were hospitalized , 6 ( 30% ) sought care at an emergency department , and none died . six patients had illness onset before returning to the united states , 5 had illness onset on the day of return , and 12 had illness onset 111 days after return ( typical incubation period for cholera is 18 hours5 days ) ( 5 ) . all 20 isolates matched the haiti isolate outbreak pattern by pulsed - field gel electrophoresis . susceptibility results for antimicrobial drug tested showed that all isolates were resistant to trimethoprim / sulfamethoxazole , furazolidone , nalidixic acid , sulfisoxazole , and streptomycin , and 18 isolates showed intermediate resistance to chloramphenicol , ampicillin , or amoxicillin / clavulanic acid . thirteen patients reported recent travel to haiti ( median length of stay 7 days , range 254 days ) and 9 to dominican republic ( median length of stay 4 days , range 29 days ) . one patient reported no recent travel but consumed cooked conch brought to the united states from haiti by relatives . travel was reported to the following departments in haiti : artibonite ( 2 ) , ouest ( 7 ) , centre ( 1 ) , nord ( 1 ) , and sud ( 1 ) . one case - patient traveled to 2 departments , and 2 did not specify a destination . all case - patients associated with the dominican republic had attended a wedding in la romana province on january 22 , 2010 ; an investigation conducted by the dominican republic ministry of health is ongoing . aside from 2 case - patients who traveled to this wedding together , no other case - patients reported traveling together . visiting friends or relatives was the main reason for travel to haiti ( table 1 ) . four patients traveled to haiti to participate in relief activities , 2 as medical volunteers , 1 on a mission trip , and 1 to distribute canned foods . a wide range of exposures was reported ( table 2 ) ; 5 patients were exposed to persons with cholera or cholera - like illness and to other risk factors for cholera acquisition . one volunteer reported no apparent lapses in safe water and food practices , although detailed information about food preparation was not available . water exposures include local lake and stream of water running down a street in haiti ; a pool in the dominican republic ; ocean ( dominican republic ) ; unspecified location in port - au - prince , haiti ; and a tank at a medical center in haiti . sources included newspaper articles ( 4 ) , friends ( 4 ) , cdc traveler s hotline ( 1 ) , and the world health organization website ( 1 ) ; 2 patients reported > 1 source . two patients reported receiving a travel health alert notice upon arrival in the united states ( m. selent , unpub . six months after the hispaniola cholera epidemic started in haiti , 23 associated cases were recognized in the united states . all cases were associated with recent travel to hispaniola or with consumption of seafood from haiti . the risk for cholera transmission in the united states is low because of improved water and sanitation , and there is no evidence of secondary transmission . florida , new york , and massachusetts have the highest populations of persons of haitian or dominican ancestry ( 6 ) . most cases were reported from florida , the state with the largest haitian population . however , case - patients also resided in states with small haitian and dominican populations . travel between the united states and haiti is straightforward ; 4 us airports offer daily direct flights from florida and new york to port - au - prince . many persons , including many of haitian descent , traveled from the united states to haiti to help with the response to the january 2010 earthquake in port - au - prince . person - to - person transmission of cholera has only rarely been reported ; cases in medical workers are almost always attributable to consumption of contaminated food or water . person - to - person transmission is not clearly supported for either of the cases we report in medical workers , although it can not be ruled out . continued surveillance and detailed investigation of cases in medical workers is warranted to further define the risk , if any , of person - to - person transmission . echoing the latin american cholera epidemic in the 1990s , travelers to cholera - affected areas should be aware of the risk and should follow prevention measures to avoid infection . in particular , travelers visiting friends or relatives may be at higher risk for travel - associated infection ( 7 ) . few case - patients had received cholera prevention education ( educational materials available at www.cdc.gov/cholera/index.html ) ; no cholera vaccine is licensed in the united states . until cholera in haiti and dominican republic resolves , clinicians , microbiologists , and public health workers in the united states should be prepared for more cases in travelers returning from hispaniola .

cholera is rare in the united states ( annual average 6 cases ) . since epidemic cholera began in hispaniola in 2010 , a total of 23 cholera cases caused by toxigenic vibrio cholerae o1 have been confirmed in the united states . twenty - two case - patients reported travel to hispaniola and 1 reported consumption of seafood from haiti .

ticks were sampled in the villages of diiso and el - humow and at the livestock market and abattoirs in garissa district , north eastern province of kenya , during april may 2008 ( figure ) . garissa district is in a semi - arid to arid ecologic zone that receives sporadic rainfall from march to may ; vegetation consists primarily of acacia - commiphora bushes . its population is largely composed of nomadic herders who travel between districts in northern kenya in search of water and pasture ( 8) . location of garissa district ( a , box ) in north eastern province , kenya , and tick collection sites ( b ) . ticks were picked by hand from infested livestock , stored in labeled sterile vials , and transported in liquid nitrogen to the kenya medical research institute laboratory . in the laboratory , ticks were washed in sterile water , rinsed first with 70% ethanol , and then rinsed with minimum essential medium containing antimicrobial agents ( 100 u / ml penicillin , 100 g / ml streptomycin , and 1 l / ml amphotericin b ) . they were identified to species by using taxonomic keys ( 9,10 ) and pooled in groups of 2 to 10 by species , sex , collection date and site , and host . the tick pools were homogenized by using 90-mesh alundum sand in a prechilled , sterile mortar and pestle with 1.6 ml2 ml ice - cold bovine albumin 1 medium ( 1 medium 199 with earle salts , 1% bovine albumin , 100 u / ml penicillin , 100 g / ml streptomycin , and 1 l / ml amphotericin b ) under high containment . the homogenates were clarified by centrifugation at 1,500 rpm for 15 min at 4c , and supernatants were stored at 80c . viral rna was extracted from tick homogenates by using trizol - ls ( invitrogen , carlsbad , ca , usa ) reagent , according to the manufacturer s instructions . rna was screened by reverse transcription pcr ( 11 ) to amplify a 536-bp fragment of the gene encoding for the nucleocapsid protein in the small ( s ) segment of the cchfv genome by using the following primers ( 12 ) : cchf f2 ( 5-tggacaccttcacaaactc-3 ) and r3 ( 5-gacaaattccctgcacca-3 ) , positions 135153 and 653- 670 , respectively , on the reference strain cchfv 10200 . electrophoresis of the pcr products was performed by using 1% agarose gels in tris - acetate - edta buffer containing ethidium bromide ; product bands were visualized and documented with the canon uvp photodoc - it gel imaging system ( uvp , llc , upland , ca , usa ) mounted with a digital camera . the pcr products of a subset of 4 of the cchfv - positive homogenates were purified by using the qiaquick pcr purification kit ( qiagen sciences , germantown , md , usa ) , according to the manufacturer s instructions , and sequenced by using the bigdye terminator version 3.1 cycle sequencing kit ( applied biosystems , foster city , ca , usa ) and the abi 3730 and automated 3130xl genetic analyzer ( applied biosystems ) . the sequences were analyzed by using the basic local alignment search tool ( blast ; http://blast.ncbi.nlm.nih.gov/blast.cgi ) and the genbank database to confirm the identity of the virus . data ( including tick species , collection site , animal host , and virologic test results ) were entered into an excel database ( microsoft corp . , redmond , wa , usa ) and analyzed by using pivot tables . a total of 8,600 ticks , of 3 genera and 8 species , were sampled primarily from camels , cattle , goats , and sheep , principally hyalomma rufipes and hy . ticks of the genus hyalomma were sampled 3 more frequently in diiso than in el - humow ( table ) . rufipes ( 3 from cattle and 1 from a camel ) , 18 pools of hy . truncatum ( 14 from cattle and 4 from camels ) , and 1 unidentified hyalomma species ( table ) in which single dna bands corresponding to the predicted 536-bp pcr product were detected . * cchfv , congo - crimean hemorrhagic fever virus . the detection of cchfv in pools of hyalomma spp . ticks from diiso village and the garissa district slaughterhouse provides strong evidence of cchfv presence in northeastern kenya and indicates that cchfv circulation in kenya is underestimated . livestock play a role in the amplification of the virus because the animals become viremic for 7 days ( 2,3 ) , during which time they can infect more ticks . our findings indicate that cchfv circulates in northeastern kenya with substantial involvement of camels and cattle . the detection of cchfv in ticks from camels at the slaughterhouse also suggests the potential of exposure for abattoir workers . the presence of cchfv among hyalommid ticks in northern kenya highlights the risk to the resident population and requires the assessment of human exposure . health care workers must therefore help create awareness among the population and take steps to prepare for and prevent outbreaks .

as part of ongoing arbovirus surveillance , we screened ticks obtained from livestock in northeastern kenya in 2008 to assess the risk for human exposure to tick - borne viruses . of 1,144 pools of 8,600 hyalomma spp . ticks screened for congo - crimean hemorrhagic fever virus by reverse transcription pcr , 23 pools were infected , demonstrating a potential for human exposure .

in family medicine , the chest x - ray is a common tool in several diseases , and unexpected findings discovered in this modality are a frequent challenge . the unexpected findings in a chest x - ray need more complex methods of evaluation and reference to other medical specialties . in pulmonology and thoracic surgery , nevertheless , a pulmonary hilum nodule may be a granuloma , primary malignant lymph node neoplasms , bronchus tumors , or metastatic tumors to lymph nodes . however , to our best knowledge , there are not previous pictorial essays regarding anatomical changes of descending aorta associated with aging that caused misdiagnosis of pulmonary hilum nodules in chest x - ray . an 84-year - old woman came with the diagnosis of a right pulmonary hilum nodule . she had a long - lasting history of obesity ( body mass index of 30.3 kg / m ) and chronic obstructive pulmonary disease treated with oxygen , bronchodilators , and a beta agonist . during an evaluation , in a chest x - ray , her family physician discovered a nodule in her right pulmonary hilum [ figure 1 , panel a ] . her physical exam was not relevant for signs of malignancy , and the chest x - ray was not conclusive . she was sent to our hospital , where a noncontrast thoracic computed tomography ( ct ) scan documented the right pulmonary hilar mass [ figure 1 , topogram , panel b ] ; however , in a coronal scan , the nodule was the tortuous descending aorta that made an angle [ figure 1 , panel c ] . with this finding , c , coronal reconstruction of computed tomography scan , showing an edge formed by descending thoracic aorta that created the false impression of a hilar lump this case illustrates how aortic changes associated with vascular aging may cause this exceptional misdiagnosis in chest x - ray . it is important to remember , that even with modern technological devices diagnostic pitfalls may occur ; for instance , with transesophageal echocardiography or positron emission tomography . it is well -known ; the descending thoracic aorta could suffer an expanding and unfolding process with aging . in addition , aortic tortuosity may occur with obesity , atherosclerotic disease , and hypertension . in our case a careful history and a diligent physical examination are the first step in identifying the underlying etiology of any symptom . because symptoms may be a presenting complaint for diverse entities , all patients should have other diagnostic tests to confirm our impression . in our case , a chest radiograph was not useful , and a coronal ct identified the underlying problem : the tortuous descending aorta . it is documented that ct scan is a suitable instrument in thoracic diseases evaluation , with its own limitations as all diagnostic procedures . this case is significant ; there are not previous pictorial assays that reported about tortuosity of descending thoracic aorta may cause a misdiagnosis in chest x - ray .

chest x - ray is a usual tool for family physicians ; however , unexpected findings in chest x - ray are a frequent challenge . we present a rare case of pulmonary hilar nodule misdiagnosis in a chest x - ray . an 84-year - old woman was sent with a diagnosis of a right pulmonary hilum nodule . she had a history of chronic obstructive pulmonary disease ; so in a chest x - ray , her family physician discovered a nodule in her right lung hilum . her physical exam was not relevant . in our hospital , a thoracic computed tomography ( ct ) scan verified the mass in the right pulmonary hilum ; nevertheless , in a coronal ct scan , the hilum lump was the tortuous descending aorta that created an angle . this case illustrates how anatomical changes associated with vascular aging may cause this exceptional pitfall in chest x - ray .

diabetic ketoacidosis ( dka ) is an acute complication of diabetes mellitus and is characterized by excessive production of betahydroxybutyric and acetoacetic acids leading to low blood ph . an arterial ph of less than 7.3 is required in order to diagnose dka . rarely , because of other complicating factors , blood ph may be in the alkalemic range and the term diabetic ketoalkalosis has been coined to describe this condition . currently , less than 30 such cases have been reported in the literature . a 34-year - old woman was admitted to the emergency department of nemazee hospital , shiraz university of medical sciences , because of polyuria and polydipsia . one year prior to admission she had underwent pancreas transplantation with pancreatoduodenal anastomosis because of repeated episodes of hypoglycemia , diabetic ketoacidosis , and poor diabetic control . after transplantation , she was on immunosuppressant drugs such as mycophenolate mofetil ( cellcept ) and tacrolimus ( prograf ) and had normal blood sugar . she discontinued her immunosuppressant drugs from 2 weeks prior to admission and gradually developed polyuria and polydipsia . at the time of admission to the emergency room her laboratory data were as follows : blood sugar : 385 mg / dl , blood ph : 7.41 , bicarbonate : 22 meq / l , bun : 28 mg / dl , creatinine : 1.1 ng / ml , k : 3.9 meq / l , na : 138 meq / l , negative urine ketone , and 3 + glucosuria . her immunosuppressant drugs were restarted and she received one pulse of 1000 mg methylprednisolone . during the next 72 hours she received an intravenous infusion of 4 units regular insulin per hour . however , her blood sugar remained high and she had repeated episodes of vomiting and had diffuse abdominal pain and extremity weakness . because of her deteriorating condition , she was transferred to the intensive care unit ( icu ) . at the time of her icu admission , her vital signs were as follows : temp : 36.5c orally , blood pressure : 100/70 mmhg , pr : 110/min , and rr : 34/min . her initial laboratory data showed : hb:13.5 g / dl , wbc : 18500/ml , 80% pmn , blood sugar : 385 mg / dl , bun : 32 mg / dl , creatinine : 1.3 ng / ml , na : 144 meq / l , k : 2.5 meq / l , blood ph : 7.50 , paco2 : 32 mmhg , bicarbonate : 25 meq / l , chloride : 92 meq / l , serum albumin : 4.2 g / dl , globulin : 2.1 gd / l , calcium : 9.2mg / dl , and magnesium : 1.6mg / dl . the patient was diagnosed as having dka and mixed metabolic acidosis and metabolic and respiratory alkalosis . because of her hypokalemia , she received 40 meq potassium chloride and normal saline during the first hour of treatment . the routine treatment of dka was started with 10 units of regular insulin per hour . during the first 4 hours of treatment , her alkalosis progressed to a ph of 7.64 . her nausea , vomiting , and abdominal pain subsided after 5 hours of treatment and her serum ketone became negative after 8 hours . she was able to eat after 14 hrs and 2 days later she was discharged on insulin ( twice daily ) . because of her undetectable c - peptide level , she was diagnosed as a case of pancreas transplant failure and her immunosuppressant drugs were discontinued . our patient had strongly positive serum ketone , but at the same time her blood ph was in the alkalemic range of 7.5 . this alkalemic ph in our patient can be explained by the presence of mixed acid - base disturbance . if the patient had pure metabolic acidosis , the serum bicarbonate was expected to drop to 11 meq / l . the serum bicarbonate in our patient had failed to decrease which signifies the presence of concomitant metabolic alkalosis . in our patients , repeated vomiting and the effect of a high dose of methylprednisolone were two causes for metabolic alkalosis . moreover , the expected arterial paco2 is 40 mmhg , but our patient had an arterial paco2 of 32 mmhg , reflecting the presence of respiratory alkalosis . pain and anxiety can be the causes of respiratory alkalosis in this patient . as expected , treatment of dka led to the progression of alkalosis , but with therepletion of water and electrolytes , plasma ph gradually returned normal . in most previously reported cases the main causes of dka were hypovlemia because of vomiting and use of diuretics , and alcohol ingestion . use of diuretics and repeated vomiting result in electrolyte depletion and hypovolemia , leading to bicarbonate reabsorption and alkalosis . two cases of endogenous cushing s syndrome because of adrenal adenoma and ectopic adrenocorticotropin ( acth ) production with dka have also been reported . excess endogenous or exogenous glucocorticoids can promote h excretion from the kidneys by their effect on mineralocorticoid receptor and contribute to alkalosis . respiratory alkalosis , as in our patient , has also been implicated as a contributing factor . vomiting and hypercortisolemia caused by steroid pulse therapy were the causes of dka in our patient . we conclude that a normal or even alkalemic blood ph does not rule out the presence of dka . in order to prevent delayed diagnosis and treat this potentially fatal condition , attention should be paid to the changes in plasma anion gap and bicarbonate and the presence of ketonemia .

diabetic ketoacidosis ( dka ) is characterized by excessive production of organic acids leading to a low blood ph . rarely , because of other complicating factors blood ph may be in the alkalemic range and the term diabetic ketoalkalosis has been coined to describe this condition . so far , less than 30 such cases have been reported in the literature . we report a 34-year - old woman who received methylprednisolone pulse therapy for the treatment of pancreas transplant rejection . thereafter , she developed vomiting and abdominal pain . her laboratory data showed high blood sugar , hypokalemia , alkalemic ph , elevated plasma anion gap , and significant ketonemia . she responded well to the treatment of dka . it was concluded that an alkalemic ph does not rule out the presence of ongoing dka . in suspected cases , changes in plasma anion gap and bicarbonate and the presence of ketonemia should be noted .

recombinant atxa , atnl and its quadruple h28y / l31v / n33g / s49d mutant lw were prepared by in vitro refolding following expression in e. coli as described . all other chemicals were from sigma aldrich or gibco life technologies . adult male balb / c mice ( 2025 g ) were maintained and humanely killed according to the guiding principles in the use of animals in toxicology ( society of toxicology , 1999 , guidelines are available at www.toxicology.org ) and the animals ( scientific procedures ) act 1986 . hemidiaphragms and accompanying phrenic nerves were dissected and placed into 10 ml tissue baths containing krebs solution ( 118.4 mm nacl , 4.7 mm kcl , 25 mm nahco3 , 1.2 mm kh2po4 , 1.4 mm mgso4 and 2.5 mm cacl2 ) , maintained at 37c and oxygenated with a mixture 95% o2 and 5% co2 . muscle contractions were evoked indirectly or directly . in the case of indirect neurally - evoked twitches , the attached phrenic nerve was stimulated at a frequency of 0.2 hz with rectangular pulses of 0.2 ms duration and a supramaximal voltage ( < 15 v ) from a grass force displacement transducer . muscle twitches were recorded on a grass polygraph model 79 ( grass instruments ) . in order to reveal the triphasic effect of recombinant proteins on nm transmission , in some experiments the preparation was partially paralyzed ( to 1520% of control ) by reducing the concentration of ca to 0.380.50 mm and allowing the tissues to equilibrate for 3045 min . in these experiments recombinant proteins were added to the tissue bath and changes in the amplitude of twitch tension responses were followed continuously . the functionality of the muscle in the preparation at the end of the recordings was checked directly by acetylcholine stimulation or indirectly by 2.5 mm ca stimulation . in experiments with directly - evoked twitches the preparation was set up as described for the phrenic nerve - hemidiaphragm for recording neurally - evoked twitches . in addition , one end of a bipolar stimulating electrode was sutured into the diaphragm muscle near the costal margin and the other one was attached to the base of the hemidiaphragm . the directly - evoked twitches were recorded by supramaximal voltage stimulation ( 50 v ) at a frequency of 0.2 hz and 2 ms duration . to eliminate nm transmission in the preparation , d - tubocurarine ( 10 m ) , an antagonist of the nicotinic acetylcholine receptors , was added into the organ bath 5 min prior to the beginning of direct stimulation . positive controls for the neurotoxic triphasic effect were performed by adding taipoxin ( 0.51 g / ml ) after reducing the concentration of ca in order to partially paralyze the nm preparation as described above . recombinant atxa , atnl and its quadruple h28y / l31v / n33g / s49d mutant lw were prepared by in vitro refolding following expression in e. coli as described . all other chemicals were from sigma aldrich or gibco life technologies . adult male balb / c mice ( 2025 g ) were maintained and humanely killed according to the guiding principles in the use of animals in toxicology ( society of toxicology , 1999 , guidelines are available at www.toxicology.org ) and the animals ( scientific procedures ) act 1986 . hemidiaphragms and accompanying phrenic nerves were dissected and placed into 10 ml tissue baths containing krebs solution ( 118.4 mm nacl , 4.7 mm kcl , 25 mm nahco3 , 1.2 mm kh2po4 , 1.4 mm mgso4 and 2.5 mm cacl2 ) , maintained at 37c and oxygenated with a mixture 95% o2 and 5% co2 . muscle contractions were evoked indirectly or directly . in the case of indirect neurally - evoked twitches , the attached phrenic nerve was stimulated at a frequency of 0.2 hz with rectangular pulses of 0.2 ms duration and a supramaximal voltage ( < 15 v ) from a grass force displacement transducer . muscle twitches were recorded on a grass polygraph model 79 ( grass instruments ) . in order to reveal the triphasic effect of recombinant proteins on nm transmission , in some experiments the preparation was partially paralyzed ( to 1520% of control ) by reducing the concentration of ca to 0.380.50 mm and allowing the tissues to equilibrate for 3045 min . in these experiments recombinant proteins were added to the tissue bath and changes in the amplitude of twitch tension responses were followed continuously . the functionality of the muscle in the preparation at the end of the recordings was checked directly by acetylcholine stimulation or indirectly by 2.5 mm ca stimulation . in experiments with directly - evoked twitches the preparation was set up as described for the phrenic nerve - hemidiaphragm for recording neurally - evoked twitches . in addition , one end of a bipolar stimulating electrode was sutured into the diaphragm muscle near the costal margin and the other one was attached to the base of the hemidiaphragm . the directly - evoked twitches were recorded by supramaximal voltage stimulation ( 50 v ) at a frequency of 0.2 hz and 2 ms duration . to eliminate nm transmission in the preparation , d - tubocurarine ( 10 m ) , an antagonist of the nicotinic acetylcholine receptors , was added into the organ bath 5 min prior to the beginning of direct stimulation . positive controls for the neurotoxic triphasic effect were performed by adding taipoxin ( 0.51 g / ml ) after reducing the concentration of ca in order to partially paralyze the nm preparation as described above .

the molecular mechanism of action of presynaptically neurotoxic secreted phospholipases a2 ( spla2s ) has not been fully elucidated . we have recently proposed a model to explain one of the hallmarks of their action the reduction in endocytosis leading to synaptic vesicle depletion in nerve terminals . our results speak strongly in favor of a mechanism in which both specific protein - protein interactions and enzymatic activity of the neurotoxic spla2 ammodytoxin a ( atxa ) are necessary for impairment of clathrin - dependent endocytosis in yeast cells . the reduction of endocytosis was strictly dependent on the enzymatic activity of spla2s expressed ectopically in our yeast model cells and was not observed with the catalytically inactive , non - neurotoxic atxa - homolog , ammodytin l ( atnl ) . here we confirm the validity of the model in mammalian cells also , by demonstrating that the enzymatically active mutant of atnl , shown to inhibit endocytosis in yeast , acts as a presynaptically neurotoxic spla2 at the mammalian neuromuscular junction .

alopecia ( hair loss ) is a psychologically and emotionally distressing side effect of cancer chemotherapeutic drugs . chemotherapy , especially cytotoxic chemotherapy - induced alopecia , can result in anxiety , depression , a negative body image , lowered self - esteem , and a reduced sense of well - being [ 1 , 2 ] . in particular , female cancer patients consider hair loss to be the most traumatic aspect of chemotherapy , and 8% would decline treatment due to fear of hair loss [ 1 , 2 , 3 , 4 ] . the incidence and severity of chemotherapy - induced alopecia are variable and vary between particular chemotherapeutic protocols . however , alopecia has generally been uncommon in patients treated with molecular targeted agents . here , we report a case of alopecia that developed gradually over 2 months after initiation of alectinib , a second - generation anaplastic lymphoma kinase ( alk ) inhibitor , for the treatment of alk - positive non - small cell lung cancer ( nsclc ) . a 54-year - old female , diagnosed with advanced alk - positive nsclc , was admitted to our hospital because of appetite loss and vomiting . she had been treated with cisplatin + pemetrexed as first - line therapy and was subsequently treated with crizotinib . whole - brain radiotherapy was planned , but she developed dyspnea and orthopnea , resulting in cardiac tamponade before this could be performed . pericardiocentesis with echocardiographic guidance was performed , and cytological examination revealed alk - positive nsclc . the patient was successfully treated , and chest radiographs showed disappearance of pericardial effusion and significant tumor reduction in the primary lesion . in addition , multiple brain metastases also disappeared after initiation of alectinib treatment without brain radiation therapy . alectinib therapy was continued without any toxicity , but the patient became aware of hair loss 2 months after initiation of the therapy . although the severity of the adverse event was grade 1 according to the common toxicity criteria for adverse events ver . 4 , she complained of no psychological or emotional distress except the alopecia and did not report any changes in lifestyle . in addition , there have been no cutaneous adverse events or abnormalities in laboratory data during alectinib treatment . alectinib is a second - generation alk inhibitor with potent in vitro activity against both wild - type and mutated alk , including mutations that confer resistance to crizotinib . several recent clinical trials showed that alectinib was effective in patients resistant to crizotinib , as well as crizotinib - naive patients . there have been no previous reports of alopecia or hair loss in clinical trials of first - line settings using alectinib and crizotinib in patients with alk - positive nsclc [ 6 , 7 , 8 ] . in the profile1007 clinical trial , which showed superior efficacy of crizotinib to standard chemotherapy in previously treated advanced nsclc with alk rearrangement , the rate of alopecia was reported to be 8% with crizotinib . although the event was not reported in detail , these findings may be reflected by prior chemotherapy or whole - brain irradiation . indeed , the symptom score of alopecia was improved in the crizotinib group compared with deterioration in the chemotherapy group ( pemetrexed or docetaxel ) . the present case developed brain metastasis , but whole - brain radiation therapy was not performed . in addition , there were no other additive agents that could have contributed to alopecia . therefore , alopecia in the present case was most likely due to the alectinib therapy . there are no previous case reports of the clinical presentation of alopecia in pubmed based on a search using the terms hair loss or alopecia and crizotinib or alectinib. therefore , this is the first case report of alk - positive nsclc showing alopecia during alectinib therapy . we highlight the present case to alert physicians to the possibility of alopecia developing from the use of alectinib . alectinib has a more favorable toxicity profile compared to crizotinib [ 6 , 7 , 8 , 9 , 10 ] . alectinib was approved for use in japan in 2014 . due to the rarity of alk - positive nsclc , there has been limited clinical experience with this regimen for such cases . in particular , as the side effects of alk - targeting inhibitors are unique , strategies are required to minimize the toxicity as well as maximize the efficacy of alk inhibitors . in conclusion , although an extremely rare clinical manifestation , we should be aware of the possibility of alopecia as an adverse event in patients treated with alectinib . written informed consent was obtained from the patient for the publication of this case report and accompanying images .

alectinib , a novel alternative anaplastic lymphoma kinase ( alk ) inhibitor , is highly effective against alk - positive non - small cell lung cancer ( nsclc ) and is well tolerated . molecular targeted agents generally have little contribution to alopecia . we encountered a case of alopecia that developed gradually over 2 months after initiation of alectinib administration for the treatment of alk - positive nsclc . the patient had no history of alopecia in previous treatments of cisplatin + pemetrexed and crizotinib . the present case indicates that alopecia should be taken into consideration as toxicity during alectinib treatment , which could adversely affect the psychological and emotional condition and quality of life even in patients treated with specific molecular targeted agents .

jejunal diverticular disease is a rare clinical entity with an incidence of between 0.06 and 1.5% . the true incidence however may be higher as the majority of jejunal diverticula are asymptomatic , and thereby remain undiagnosed . in symptomatic cases , non - specific epigastric pain and bloating are the most common complaints [ 2 , 3 ] . however , life - threatening complications such as gastrointestinal ( gi ) bleeding , bowel perforation and obstruction have been reported in up to 18% of cases . it is therefore vital that the diagnosis of the condition can be made promptly to ensure optimal and timely management for the patient . we present a case of a 78-year - old male with massive gi bleeding secondary to jejunal diverticular disease who was surgically managed with good post - operative outcome . a 78-year - old male with a history of ischaemic heart disease , stage iv chronic kidney disease and chronic obstructive pulmonary disease , presented to the emergency department after a sudden collapse at home . whilst in the department , he experienced a further syncopal event with two episodes of large - volume melaena . he was pale , clammy and haemodynamically unstable with a systolic blood pressure of 75 mmhg . his blood results showed a haemoglobin ( hb ) of 66 g / l and a urea of 32.7 mmol / l . he was initially resuscitated with intravenous fluids , red blood cells ( rbcs ) , platelets , fresh frozen plasma ( ffp ) and cryoprecipitate . the patient proceeded to urgent upper gi endoscopy ( ogd ) but the procedure failed to identify any evidence of recent or active bleeding . colonoscopy showed large amounts of altered blood in the colon as well as the presence of sigmoid diverticula but without evidence of active bleeding . segmental ct - angiogram was contraindicated given patient 's poor renal function . despite vasopressor support and a proton - pump - inhibitor infusion , with on - going melaena , the patient 's hb and blood pressure remained low , requiring 19 units of rbcs in the first 48 h of admission . over the subsequent 36 h , the patient 's condition stabilized with an hb consistently over 90 g / l . however , on day 4 of admission , he produced five further episodes of melaena , and became acutely tachycardic and hypotensive . the decision was then made to proceed to exploratory laparotomy with a pre - operative plan of performing a subtotal colectomy . the presumptive diagnosis was that the bleeding was likely secondary to his known colonic diverticulosis . intra - operatively however , we found multiple diverticula clustered along the proximal and mid jejunum at the mesenteric edge . partially digested blood was seen in the portion of small bowel distal to these diverticula . we performed a small bowel resection of 80 cm length of diseased jejunum and an end - to - end anastomosis ( figs 1 and 2 ) . histology confirmed numerous true diverticula , with no evidence of malignancy . in total , since admission , the patient received 23 units of rbcs , 16 units of ffp , 4 units of platelets and 3 units of cryoprecipitate . note that the diverticula arise at the mesenteric border and are clustered around proximal jejunum . figure 2:intra - operative photography demonstrating jejunal diveritcula proximally and presence of altered blood in the small bowel distal to these diverticula . note that the diverticula arise at the mesenteric border and are clustered around proximal jejunum . intra - operative photography demonstrating jejunal diveritcula proximally and presence of altered blood in the small bowel distal to these diverticula . the patient was discharged on day 7 post - operation and followed - up in outpatients clinic 2 weeks later . there were no further reports of bleeding and he was discharged from surgical follow - up . jejunal diverticular disease is an uncommon condition that is usually asymptomatic but may rarely present with life - threatening complications including massive gi bleeding [ 2 , 3 ] . other complications such as chronic malabsorption , volvulus , diverticulitis with or without perforation or abscess occur in 1030% of patients [ 1 , 5 ] . the majority of patients who present with gi bleeding do not display previous gi symptoms and are reported to develop an acute onset haemorrhage per rectum . jejunal diverticulosis is often difficult to locate endoscopically and diagnosing jejunal diverticular bleeding remains problematic , with current imaging techniques continuing to be unreliable . whilst jejunal diverticulosis can be identified by abdominal ct and barium follow - through studies , enteroclysis remains the investigation of choice . although some reports demonstrate success with capsule endoscopy and double balloon endoscopy [ 9 , 10 ] such investigations are of limited use in emergency settings . in cases of jejunal diverticular bleeding , selective mesenteric angiography or ct angiogram can be used to localize active bleeding but may be contraindicated in patients ' with severe kidney impairment as with our patient . urgent laparotomy is indicated in the presence of acute complications of jejunal diverticula including diverticulitis , massive bleeding or bowel perforation both as a diagnostic and therapeutic measure [ 3 , 6 , 7 ] . several successful cases with complete small bowel resection with primary entero - entero anastomosis have been reported . due to low incidence , low clinical index of suspicion and unreliable diagnostic imaging in emergency situations , diagnosis of jejunal diverticular disease is challenging and therefore often delayed several days after initial presentation . this has major implications with regard to prompt and timely management , and can lead to significantly increased morbidity and mortality . whilst there are undoubtedly more common causes of gi bleeding , this case demonstrates that jejunal diverticular disease should remain on the differential diagnosis and investigations to confirm the diagnosis should be considered . however , despite investigations , the diagnosis may remain elusive and in patients with on - going bleeding , laparotomy and surgical resection is currently the treatment of choice [ 3 , 6 , 7 ] .

jejunal diverticula are rare and the condition remains mostly asymptomatic . however , they can present with vague chronic abdominal symptoms and , in some cases , acute life - threatening complications , such as gastrointestinal ( gi ) bleeding , bowel obstruction and perforation . we present a case of an adult male who presented with life - threatening gi bleeding secondary to jejunal diverticular disease . whilst there are undoubtedly more common causes of gi bleeding , this case demonstrates that jejunal diverticular disease should remain on the differential diagnosis and investigations to confirm the diagnosis should be considered . however , despite investigations , the diagnosis may remain elusive and in patients with on - going bleeding , laparotomy and surgical resection is currently the treatment of choice .

hypoparathyroidism is an uncommon metabolic disorder characterized by hypocalcemia and hyperphosphatemia due to a deficiency or absence of parathyroid hormone ( pth ) the abbreviation is correct secretion . it may occur in combination with other disorders , including autoimmune problems ( e.g. , autoimmune polyendocrinopathy - candidiasis - ectodermal dystrophy syndrome ) or developmental defects ( digeorge syndrome and velocardiofacial syndrome ) . hypoparathyroidism may also develop as an isolated entity of unknown etiology called idiopathic hypoparathyroidism ( ihp ) . , it presents as consequent paresthesias , muscle spasms ( i.e. , tetany ) and seizures . in contrast , in chronic hypoparathyroidism the onset is so gradual that the only symptom may be visual impairment from cataracts . permanent physical and mental deterioration can occur if initiation of treatment for either acute or chronic cases is delayed . these disorders include alopecia , scaling of the skin , deformities of the nails and dental abnormalities . hypoparathyroidism is diagnosed on the basis of measurements of serum calcium ( ca ) and pth levels . the treatments available for these patients are ca salts , vitamin d or its analogs and drugs that increase renal tubular resorption of ca , to obtain adequate , but low , normal serum ca levels . clinical evaluation of the patient and frequent determinations of serum ca levels are indicated in the early stages of treatment . if pth is absent or nonfunctional , its hypocalciuric action can not occur and thereby the increase in serum ca concentration may cause hypercalciuria , nephrolithiasis , or renal damage . in general , treatment is satisfactory ; however , in longstanding cases , repair of cerebral and dental changes is not likely . this case report describes atypical dental changes in a 40-year - old woman , which led to the diagnosis of primary ihp . a 40-year - old female patient reported to the department of periodontics at sdm college of dental sciences with the chief complaint of bleeding gums . she gave a medical history of hypothyroidism and complained of profuse bleeding from her gums andalso , general weakness , muscle cramps , tingling sensation over hands , feet and perioral region . a detailed dental examination revealed bleeding on probing , sub gingival calculus , generalized gingival inflammation , deep bite and periodontal pockets measuring 4 - 5 mm in depth with teeth 16 , 26 and 12 [ figure 1 ] . radiographic examination revealed abnormally short roots with respect to 16 , 14 , 15 and 26 , 25 with intact lamina dura [ figure 2 ] . the orthopantamogram showed generalized cemental hyperplasia albeit mild in nature , widened periodontal ligament space and alteration in the bone trabecular pattern [ figure 3 ] warranting further investigation . blood investigations showed decreased serum ca level ( 7.1 mg / dl ) and normal serum alkaline phosphatase level ( 126 u / l ) . thyroid hormone ( t3 , t4 and thyroid - stimulating hormone ) assay values were also normal , suggestive of euthyroid state , whereas the pth assay by chemi - luminescent immuno assay method showed subnormal parathormone values ( 7.3 pg / ml ) [ table 1 ] . the lowered pth in conjunction with lowered ca levels led to a provisional diagnosis of hypoparathyroidism . lateral skull radiographs were also taken to rule out any calcifications of the basal ganglia [ figure 4 ] . clinical presentation full mouth intraoral periapical radiographs laboratory investigations two types of primary hypoparathyroidism have been described in medical literature - pseudohypoparathyroidism and ihp . the basic difference between the two lies in their response to treatment with parathormone and dihydrotachysterol ( vitamin d ) . in pseudohypoparathyroidism the ca level returns to normal following vitamin d treatment but not following parathormone administration whereas ihp responds to both of these pharmacologic agents . according to the consulted literature , only 6 studies investigating the oral findings in ihp patients have been published since 1966 . hypoplastic enamel is the most frequent finding , although short rounded roots , hypodontia and delay or lack of tooth eruption are also cited , some of which were observed in our patient as well . however , there were some atypical findings such as generalized cemental hyperplasia , altered trabecular pattern and widening of the periodontal ligament space , which have not been reported thus far in literature . the dental findings in this patient are suggestive of reduced bone reparative capacity due to low serum ca levels . the process of root resorption involves a complex interaction of inflammatory cells , resorbing cells , hard tissue , cytokines and enzymes such as collagenase , matrix metalloproteinase and cysteine proteinases . the periodontal ligament is a specialized connective tissue , which acts as a barrier between the alveolar bone and cementum . localized damage or loss of periodontal ligament renders the denuded cementum surface chemotactic to clastic cells such as osteoclasts , macrophages and monocytes , resulting in root resorption . the presence of intact lamina dura in the intra oral periapical radiographs of the patient suggests that the short roots may be congenital in origin . non - surgical periodontal therapy resulted in resolution of gingival inflammation and shallow periodontal pockets . the serum ca level were monitored periodically for 1 year post diagnosis and was found to be 9.7 mg / dl at the end of the first year ; which was considered normal for age . a patient with a systemic disorder , such as ihp , a rare metabolic disorder , requires careful anamnesis and thorough clinical and radiographic examinations . the clinician must be alert to oral abnormalities such as cemental hyperplasia , enamel hypoplasia , widened pulp chambers , shortened roots , hypodontia which could be suggestive of a systemic disorder . a careful examination and diagnosis is fundamental to the treatment of such atypical systemic conditions .

idiopathic hypoparathyroidism ( ihp ) is a rare endocrinopathic disorder , of idiopathic nature , characterized by a deficiency of parathyroid hormone causing low serum calcium ( ca ) and high serum phosphorus concentrations . we present a case report ofa 40-year - old woman with complaint of bleeding gums and tingling sensation in fingers and toes reported to our department in march 2005 with no tangible diagnosis from previous medical examinations . oral findings included abnormally short roots , cemental hyperplasia , widening of the periodontal ligament space and root resorption . laboratory investigations revealed a low serum ca level and along with the clinical findings , a diagnosis of ihp was reached . oral prophylaxis was performed and ca supplements were prescribed . we believe that this case report is a first of its kind reporting cemental hyperplasia , altered alveolar bone patterns and periodontal ligament widening in a patient with ihp .

the majority of granular cell tumors occur in the head and neck region , with a predilection for occurrence in the tongue . tracheal lesions are by far the least common . a confirmative diagnosis for gcts should be made histopathologically and should be supported by immunohistochemical staining . the treatment of a granular cell tumor consists of wide local excision . a review of the english language literature revealed 31 reported cases of tracheal granular cell tumors , with only four reported in children8 ) . a review of the korean ent literature revealed several cases of laryngeal gcts and one case of malignant gct in the retrotracheal space1 - 3 ) . there are no reports of tracheal gct in the korean literature here , we report a case of a 20.year.old man with tracheal gct and discuss the diagnosis and management of this rare tumor . a 20-year - old man presented to our clinic with blood.tinged sputum , cough , and left pleuritic pain of 2-weeks duration . he developed a small amount of blood - tinged sputum and left pleuritic pain , and had lost 4 kg of body weight in one month . the workup of the patient included a complete blood count and blood chemistries , which were normal . a chest x - ray did not demonstrate any evidence of pulmonary disease ( figure 1 ) . the pft showed a mild restrictive pattern : fvc , 4.1 ( 76% ) ; fev1 , 3.2 ( 72% ) ; and fev1/fvc , 78% . a rigid bronchoscopy revealed a smooth , sessile , yellow lesion emanating from the right posterior terminal trachea wall ( figure 2 ) . the sections showed a benign granular cell tumor with marked granular , eosinophilic cytoplasm and small , eccentric , round nuclei . an immunohistochemical analysis showed that the tumor cells were positive for neuron - specific enolase ( nse ) , s-100 , and cd 68 ( focally ) ( figure 3 ) . a chest high - resolution computed tomography ( hrct ) was normal ( figure 5 ) . the patient was discharged from the clinic as his hemoptysis and pleuritic pain had resolved . granular cell tumors , first reported by abrikossoff in 1926 , were initially called myoblastomas , suggesting a myogenic origin5 ) . currently , most pathologists believe that the cell of origin is the schwann cell and refer to this lesion as a granular cell tumor4 ) . ninety - eight percent of granular cell tumors are benign , and only 1 to 2% of all gcts are malignant5 ) . although only 30 malignant lesions have been reported worldwide in the medical literature , none of these malignant lesions has been reported for the pediatric age group or in the trachea5 ) . more than 50% of granular cell lesions occur in the head and neck region , most commonly in the tongue . other head and neck sites for gcts include the central nervous system and cranial nerves , skin and subcutaneous tissues , middle and external ear , parotid glands , pharynx , palate , floor of the mouth , esophagus , and tonsils . a patient may remain completely asymptomatic with even 50 to 75% obstruction of the tracheal lumen . symptoms of wheezing , especially with exertion , are typically attributed to asthma and chronic bronchitis . there may be cough , hemoptysis , and a globus sensation , while pain is unusual6 ) . laryngoscopy and bronchoscopy can be performed to establish a safe airway , and to assess the extent of the lesion and perform a biopsy . a diagnosis can usually be made with hematoxylin - eosin staining , but immunohistochemistry with an s-100 protein stain and neuron.specific enolase are also helpful9 ) . benign tracheal neoplasms may include a benign fibrous histiocytoma , papilloma , neurofibroma , pleomorphic adenoma , hemangioendothelioma , neurilemmoma , and benign chondrogenic tumors4 ) . the differential diagnosis of malignant tracheal neoplasms includes a malignant fibrous histiocytoma , malignant chondrogenic neoplasia , carcinoid , rhabdomyosarcoma , fibrosarcoma , adenoid cystic carcinoma , and squamous cell carcinoma4 ) . neoplasms arising from the mediastinum , including esophageal tumors that erode the tracheal wall , should also be considered . neither radiation therapy nor chemotherapy has any role in the management of tracheal granular cell tumors . bronchoscopic excision may be adequate for tumors less than 1 cm in diameter , whereas wide excision with tracheal resection is reserved for tumors with diameters greater than 1 cm . in one study , recurrence after excision occurred in approximately 8% of the cases , with all recurrence associated with the presence of a positive margin5 ) . follow - up is based on appearance of symptoms rather than serial endoscopic examinations and imaging studies . although respiratory symptoms caused by asthma or foreign bodies are common in children , the possibility of a tracheal tumor masquerading as recalcitrant asthma or recurrent pneumonia should not be overlooked . an unusual case of a mid.tracheal gct presenting with obstructive sleep apnea and exercise.induced asthma illustrates this point . the diagnosis of a gct and other tracheal masses depends on a high index of suspicion and an efficient workup .

a 20-year - old man presented to our outpatient clinic with hemoptysis , cough , and pleuritic chest pain . his chest radiograph and pulmonary function tests ( pft ) were normal . a bronchoscopy showed a small yellowish patch with a regular surface . a direct bronchoscopic biopsy was performed . the pathologic findings showed a benign granular cell tumor . the respiratory symptoms resolved after biopsying the tumor . on follow.up , there were no signs of recurrence of the granular cell tumor after a period of 24 months .

uhl 's anomaly is characterized by the partial or complete absence of the myocardium of the right ventricle . a review in 1993 found 84 reported cases in the literature , since the beginning of 20 century . an unguarded tricuspid valve is a rare congenital anomaly described in only a few antemortem case reports . here , we report a rare case of uhl 's anomaly with unguarded tricuspid valve orifice with free tricuspid regurgitation in the infant . a 2-month - old male infant weighing 3.15 kg , presented with a history of breathlessness . he was a full - term normal vaginal delivery at home . on general examination , afebrile , tachypneic , cyanosis , and clubbing were present , and all peripheral pulses were present . his vitals were heart rate : 122/m , respiratory rate : 52/m , blood pressure : 108/88 mmhg and oxygen saturation : 80% . on auscultation , ejection systolic murmur was best heard in the upper left parasternal area . chest x - ray showed an enlarged cardiac silhouette ( cardiothoracic > 0.5 ) and decreased pulmonary vasculature . transthoracic two - dimensional echocardiography reveals situs solitus , enlarged right atrium and ventricle , no definitive right ventricular wall and small diverticulum of the right ventricular outflow tract . small size ( 2 mm ) perimembranous type ventricular septal defect with left to right shunt ( gradient 60 mmhg ) was present . large size ( 5 mm ) ostium secundum type of atrial septal defect with the right to left shunt was present . subcostal long axis view shows the dilatation of the right atrium with right ventricle and absent tricuspid valve . uhl 's anomaly is a rare cardiomyopathy characterized by a thin walled dilated right ventricle due to the absence of myocardium . in 1902 , osler reported a heart with a very thin wall like a parchment . subsequently , in 1949 , uhl reported the similar type of case after an autopsy of old infant and later in 1979 , fontaine et al . described a condition called arrhythmogenic right ventricular dysplasia ( arvd ) characterized by local deficiency or fibro - fatty replacement of the right ventricular myocardium . uhl 's anomaly and arvd are different diseases which has separate and different morphological entities . in uhl 's anomaly , the affected wall does not have a fat tissue , and endocardial and epicardial layer lie parallel to each other whereas in arvd , is the replacement of myocardial cell by fibrous fatty tissue . uhl 's anomaly has been reported in identical twins and has been diagnosed in utero . a failure during embryonic development of the right ventricular myocardium occurs is a possible cause . recently , the hypothesis that the right ventricular myocardium may be replaced due to a process of apoptosis of the myocardiocytes has been cogitated and supported by studies of molecular biology . some authors suggested that overexpression of vascular endothelial growth factor , mainly cardiomyocytes may be responsible for impaired development of the ventricular myocardium . it may be associated with other congenital cardiac malformation such as tricuspid valve anomalies , pulmonary atresia , patent ductus arteriosus , atrial septal defect and ebstein 's anomaly . congenitally unguarded tricuspid orifice is a rare anomaly , and few cases of antemortem diagnosis have been reported in the literature . reported the partial or complete absence of tricuspid valvar tissue diagnosed on fetal echocardiography . in uhl 's anomaly , mainly there is a lack of right ventricular contraction and hemodynamically right ventricle act as a transition zone between the right atrium and pulmonary trunk . the forward flow to the pulmonary circulation is driven by vigorous paradoxical septal motion and right atrial contraction . whereas in the unguarded tricuspid orifice , pulmonary circulation is maintained by the pumping action of the right atrium or the outflow tract because of the poor right ventricular contractile function . hemodynamically our case as having very poor right ventricular function as well as right atrium workload is increased for feeding the pulmonary vessels . the clinical finding in uhl 's anomaly is heart failure with complaints of palpitation , syncope , tachycardia , and sudden death . arrhythmia and conduction disturbances are not the most common feature o due to the absence of residual foci to transmit anomalous electrical activity . on physical examination , cyanosis , and jugular venous disten - sion with a dominant a wave is present . cardiac auscultation usually reveals a decrease in the intensity of the heart sound , especially the first heart sound with widely split second heart sound . a typical pansystolic murmur of tricuspid insufficiency may be present , but the patient may have other nonspecific murmur . echocardiography identifies a large right ventricle with an akinetic free wall , brisk paradoxical septal motion of the ventricular septum and normal tricuspid leaflets in uhl 's anomaly . the most frequent approaches were exclusion of right ventricle by closure of tricuspid valve orifice with atrial septostomy and bi - directional glenn shunt . a modification of this procedure combines with atrial septostomy and bi - directional glenn shunt with partial right ventriculotomy also called as it is unclear that the infant will benefit from bi - directional glenn shunt or one - and - a - half ventricular repair . in view of severe right

uhl 's anomaly is characterized by absence of the myocardial layer of the right ventricle , with opposition of the endocardium and epicardium . it is rarely associated with other congenital malformation . here , we reported the rare association of uhl 's anomaly with absent tricuspid valve in an infant .

incidence of transverse vaginal septum varies from l : 21000 to 1:72000 and may be present in lower , middle , and upper thirds in 19% , 35% , and 46% of patients , respectively . vertical fusion / canalization disorder of mullerian ducts and urogenital sinus result in partial / complete vaginal septum of various thickness ( 0.56 cm ) . septum may be complete but usually has laterally placed tiny hole giving an impression of vaginal vault without cervix . concomitant presence of unicornuate uterus is very rare in term pregnant woman . till date , only one case of concomitant transverse vaginal septum with unicornuate uterus has been reported in young non - pregnant girl . a 24-year - old primigravida at 38 + 5 weeks came to labor room in emergency hours with chief complaints of pain in abdomen since morning . she was an unbooked patient with no investigations except for her ultrasonography done at 26 weeks of gestation which revealed no abnormalities . liquor was less than period of gestation , uterus irritable , fetal heart sound was regular ( 144/min ) . on local examination , labia majora and minora were well - formed with abundant pubic hair . on per speculum examination , vagina was blind pouch with 2 mm opening present in centre [ figure 1 ] . urethral opening was normal . on per vaginal examination , vagina was blind - ended pouch ; but behind that small opening , a fibrous ring - like structure felt which was around two fingers loose and through which bag of members were also felt . so diagnosis of transverse vaginal septum was made and as patient was in labor with breech presentation , she was shifted for emergency lower segment cesarean section , after all necessary investigations . on exteriorizing uterus it was found that uterus was unicornuate with only one tube and ovary on left side , the right sided tube and ovary was absent [ figure 2 ] . post - operative period was uneventful and whole abdomen and pelvic ultrasonography done on 7 day of lower ( uterine ) segment cesarean section ( lscs ) revealed absent right kidney and ureter [ figure 3 ] . mother and baby were discharged in good condition and advised to come for follow - up after 6 weeks for septum resection . showing normal uretheral opening with red rubber catheter and a small aperture in the transverse vaginal septum with a small dilator gently introduced in it showing unicornuate uterus with single ovary and fallopian tube on left side with absent fallopian tube and ovary on right side empty renal fossa with absent kidney on right side mllerian ducts are primordial anlage of internal female reproductive organs and differentiate to form fallopian tubes , uterus , uterine cervix , and superior aspect of the vagina . when an interruption occurs in any of these dynamic processes of differentiation , migration , fusion , and canalization , wide spectrum of mllerian duct anomalies occur . transverse vaginal septum ( tvs ) is formed when tissue between vaginal plate and caudal aspect of fused mllerian ducts fails to reabsorb . most of these septa are located in superior vagina at putative junction between vaginal plate and caudal aspect of uterovaginal primordium ( 46% ) . it is one of most rare mllerian duct anomalies , with frequency of 1 case in 70,000 females . unlike other mllerian duct anomalies no evidence indicates that this disorder is genetically inherited , although a study of an inbred amish community suggested that hydromucocolpos due to obstructive tvs was the result of a rare autosomal disorder . in general if tvs is complete , patient commonly presents with primary amenorrhea and cyclic pelvic pain . physical examination reveals palpable central lower abdominal or pelvic mass secondary to hematocolpos , hematometra , hematosalpinx , and hemoperitoneum . however , unlike an imperforate hymen , examination of genitalia reveals no evidence of bulging at introitus . diagnosis can be made on transvaginal sonography , computed tomography , and magnetic resonance imaging . management consists of surgical correction depending on its location within vagina and its thickness . in high tvs , surgical correction is more difficult because septum is usually thick and extensive ; dissection between bladder and rectum is required . in low , mid , and thin tvs , unicornuate uterus results from abnormal / failed development of one of paired mllerian ducts accounting for 2.413% of mllerian anomalies . this group of anomalies can be further subdivided into four variants according to the american fertility society . isolated unicornuate uterus is most common , with frequency of 35% . when rudimentary horn is present , it is non - cavitary in 33% of cases , cavitary non - communicating in 22% , and cavitary communicating in 10% . anomalies of urinary tract are commonly associated and are more frequent with unicornuate uterus than other mllerian duct anomalies .

transverse vaginal septum is a result of faulty canalization of embryonic vagina . septum may be complete but usually has laterally placed tiny hole giving an impression of vaginal vault without cervix . we described a case of untreated transverse vaginal septum with small central aperture diagnosed during labor and unicornuate uterus diagnosed intraoperatively , with successful pregnancy outcome .

takotsubo cardiomyopathy ( tc ) is an increasingly reported syndrome characterized by transient apical left ventricular dysfunction in absence of low limiting coronary artery disease . prolongation of qtc interval associated with tc has been previously reported in published case series . bradycardia induced qt interval prolongation seemed to be amplified by the occurrence of tc , resulting in torsade de pointes . temporary ventricular pacing at a high rate decreased the qt interval and prevented the recurrence of torsade de pointes . we report a case of torsade de pointes associated with qt prolongation and tc . because qt prolongation and bradycardia persisted even after the resolution of tc , the patient received a permanent pacemaker . a 78-yr - old female with a medical history of hypertension was admitted to our hospital due to syncope on 20 july , 2010 . her antihypertensive medications were nifedipine ( 30 mg ) and valsartan ( 80 mg ) . upon arrival to hospital , the patient was fully conscious and alert with a pulse rate of 40 beats per minute and a blood pressure of 140/80 mm hg . electrocardiography ( ecg ) on admission showed an escape junctional rhythm of 35 beats per minute combined with t wave inversion in precordial leads and qt prolongation measuring 580 ms in the qtc interval ( fig . the serum levels of potassium , magnesium , and ionized calcium were within normal limits . the serum troponin t level was slightly elevated to 0.081 ng / ml ( 0 - 0.1 ng / ml ) . echocardiography showed akinesia of mid and apical left ventricular ( lv ) walls with the systolic ballooning of the ventricular apex ; lv ejection fraction was estimated to be 35% by a modified simpson 's method ( fig . she was diagnosed with takotsubo cardiomyopathy based on echocardiographic and coronary angiographic findings . on the second day , ecg showed severe bradycardia ( heart rate 20 beats per minute ) with marked prolongation of qt ( qtc = 720 ms ) followed by torsade de pointes ( tdp ) ( fig . after temporary ventricular pacing , which was performed at a rate of 80 beats / min , the qtc interval decreased to 480 ms . on the fourth day , the temporary pacemaker was removed . at that time , ecg showed a marked bradycardia ( heart rate of 40 beats per minute ) with qt prolongation and r on t phenomenon ( fig . 1c ) . on the 10th day , we checked follow - up echocardiography , which showed normal lv systolic function without previous wall motion abnormalities ( fig . 2c , ecg still showed a severe bradycardia with qt prolongation while we checked daily serial ecg up to the 20th day ( fig . therefore , we planned to insert a permanent pacemaker to prevent recurrent tdp , although lv systolic dysfunction of the patient was recovered . on the 21st day one and 3 months after the index procedure of permanent pacemaker insertion , ecg still showed qt prolongation ( qtc = 552 ms ) with t - wave inversion at precordial leads ( fig . tdp denotes a polymorphic ventricular tachycardia associated with long qt syndrome , and is a life - threatening arrhythmia . qt prolongation is the surface ecg manifestation of abnormal repolarization of myocardial cells due to problems with cellular ion channels . acquired qt prolongation may be associated with the following conditions : 1.electrolyte depletion ( especially potassium or magnesium ) 2 . also , the association of tc with tdp has rarely been reported ( 1 , 2 ) . patients with tc generally show st elevation , t - wave inversion , q - wave formation and qtc interval prolongation in ecg . the prevalence of qtc interval prolongation among tc patients is high , ranging from 50% to 100% according to different case series . this is probably because systolic dysfunction is associated with both tc and qt - interval prolongation ( 3 ) . although qt - interval prolongation is prevalent among tc patients and might precede tdp , the later has rarely been reported in tc patients . danney et al . ( 5 ) first reported tdp occurring in patients with tc . since then , numerous reports of tdp related to tc have been published ( 4 , 5 ) . also , bradycardia with or without av block and suspicious of congenital long qt syndrome are the risk factors of tc - associated tdt ( 4 ) . however , permanent pacemaker insertion has been performed on a few patients ( 6 , 9 ) . in general , qt interval prolongation and st - t changes occur during the acute or subacute phase of illness , and these ecg changes typically normalize within several weeks of improvement in lv wall motion ( 3 ) . in our case , qt prolongation and st - t change persisted for 3 months . inoue et al . ( 6 ) reported a patient who underwent permanent pacemaker insertion due to complete av block associated with tc . in their case , the ecg of the patient showed complete av block even after recovery from illness . ( 8) also reported 2 cases of suspicious congenital long qt syndrome associated with tc . during the previous regular medical check up of the present case , after development of her illness , qtc interval did n't improved , even after normalization of lv wall motion and cardiac enzyme level . based on her previous medical check up ecg and history , a diagnosis of acquired long qt syndrome associated with tc , we inserted a permanent pacemaker because sinus bradycardia ( 40 beats per minute ) and qt prolongation ( qtc = 550 ms ) persisted for 2 weeks even after recovery from lv dysfunction . to date , the association between tc , bradycardia and tdp is not exactly known . ( 9 ) stated that prolongation of bradycardia - induced qt interval may be augmented by the occurrence of takotsubo cardiomyopathy , resulting in tdp . in their cases , permanent pacemaker insertion was also performed because bradycardia and complete av block persisted after the recovery from lv dysfunction . although tdp has rarely been reported in patients with tc , the clear relationship between the 2 conditions has yet to be determined . however , qt prolongation and bradycardia could be persistent even after full recovery of tc , and permanent pacemaker insertion may be a treatment option of long qt syndrome related with tc .

prolongation of qtc interval associated with takotsubo cardiomyopathy ( tc ) has previously been reported in published case series . we report an unusual case of a patient who presented with tc associated with long - qt syndrome and developed cardiac arrest secondary to torsade de pointes . since qt prolongation and bradycardia persisted after the resolution of tc , the patient received permanent pacemaker . since then additional event did not occur . qt prolongation and bradycardia could be persistent even after recovery of tc , and permanent pacemaker insertion may be a treatment option of long qt syndrome related with tc .

gm1 gangliosidosis is an autosomal recessive lysosomal storage disease caused by deficiency of the lysosomal hydrolase , acid beta - galactosidase . the infantile form ( type 1 ) is characterized by progressive organomegaly , dysostosis multiplex , facial coarsening , and progressive neurologic deterioration within the first year of life . we describe an infant with gm1 gangliosidosis associated with diffuse ecchymosis and mongolian spots on the trunk and extremities with ventral and dorsal distribution . a 7-month - old girl was born to consanguineous palestinian parents ( i.e. , first cousins ) at term by cesarean section delivery due to fetal distress . at age of 2 weeks , she developed clonic seizures of upper and lower limbs . she was unable to sit , roll over , crawl , laugh or smile on social contact . she also had marked growth delay : weight 4 kg ( -4 sd ) and occipitofrontal circumference 40 cm ( -2.5 sd ) . her examination revealed generalized hypotonia , gingival hypertrophy , coarse facial features , diffuse ecchymosis and numerous diffuse mongolian spots on her trunk and extremities [ figures 13 ] . numerous , diffuse ecchymoses and mongolian spots on ventral surface of the trunk numerous , diffuse ecchymoses and mongolian spots on dorsal surface of the trunk coarse facial features the hyperpigmented skin lesions were apparent at birth and had not changed in number , position or size . a skin biopsy revealed dermal dendritic melanocytes ( consistent with mongolian spots ) and also perivascular accumulation of foamy histiocytes ( usually associated with storage disease ) . the patient lacked any history of bleeding tendency and her platelet count , prothrombin time , partial thromboplastin time , bleeding time and fibrinogen level were all normal . her electroencephalogram revealed irregular slow activity with occasional bursts and spikes of slow waves in a depressed background . clinical and biochemical evidence supported the diagnosis of gm1 gangliosidosis type 1 in our patient . she had numerous and diffusely distributed hyperpigmented lesions which is an unusual presentation for typical mongolian spots . eczematoid facial rash , truncal macular rash , angiokeratomas and generalized telangiectasia , in patients with this illness , have been sporadically described in the literature.[14 ] however , diffuse , extensive and unusual mongolian spots have been reported in increasing number of cases of gm1 gangliosidosis type 1 in recent years.[57 ] table 1 summarizes the reported cutaneous findings in these patients . this association has also been described with other lysosomal storage diseases such as hurler 's and hunter 's syndromes . reported the first case of possible chance association between gm1 gangliosidosis type 1 and extensive mongolian spots in a 5-month - old female . selsor et al . reported a 10-month - old male with gm1 gangliosidosis type 1 who also had hyperpigmented macules and patches which were most probably mongolian spots . beattie et al . described a 5-month - old female with gm1 gangliosidosis who had unusual mongolian blue spots on her dorsal and central trunk . tang et al . presented a 13-month old child with gm1 gangliosidosis who had multiple mongolian spots and further demonstrated swelling of the endothelial cells of the dermal capillaries with narrowing of the vascular lumen . the authors postulated that this may lead to weakening and dilatation of the vascular walls resulting in angiokeratoma and telangiectasia in these patients . hanson et al . described two infants with extensive dermal melanocytosis in association with gm1 gangliosidosis type 1 in one and with hurler 's syndrome in the other . they hypothesized that the accumulating metabolites in these illnesses may contribute indirectly to the arrest of the transdermal migration of melanocytes within the dermis leading to the appearance of these cutaneous findings . this may occur through interference with neural growth factor and tyrosine kinase - type receptor interactions . described seven japanese boys with hunter 's syndrome and reported extensive mongolian spots in all of them ; the authors suggested that the extent and persistence of the hyperpigmentation could allow earlier diagnosis and possible intervention before irreversible nervous system impairment develops . although a chance association of the dermal findings and gm1 gangliosidosis can not be excluded , we think our patient adds to the increasingly recognized evidence that patients with this disorder may manifest abnormal dermal pigmentary lesions , which may be present at birth thus helping physicians make an earlier diagnosis . whether the ecchymosis in our patient is incidental or part of the dermal endothelial vasculopathy associated with gm1 gangliosidosis and other lysosomal storage diseases needs to be investigated further . we are not aware of this association in the literature and its significance in the diagnosis is not yet clear .

a 7-month old girl with gm1 gangliosidosis type 1 manifested with diffuse ecchymosis and mongolian spots . the cutaneous lesions were present at birth before the appearance of the other features of the disease . we postulate that dermal pigmentation may be recognized as an early sign of gm1 gangliosidosis .

case - patients undergo a smear examination of the characteristic patch of thick gray membrane and samples are cultured for c. diphtheriae . to describe the epidemiology of diphtheria , we included cases defined as an acute febrile infection with gray - white patch in pharynx , tonsils , or fauces among residents of hyderabad admitted to fever hospital during 20032006 . we obtained data regarding age , sex , religion , month and year of occurrence , and circle ( municipal administrative subdivision ) of residence from the medical records of 2,685 diphtheria case - patients admitted during 20032006 . thirty - one case - patients died ( overall case - fatality rate 1.2% ) . diphtheria occurred throughout the year with lower incidences during july and august ( figure ) . annual incidence increased from 11/100,000 to 23/100,000 from 2003 through 2006 ( trend 152 ; p = 0.00001 ) . median age of case - patients was 17 years ( range 9 months80 years ) . attack rates were lowest among infants , increased with age , and reached a maximum among children 1014 years of age . of the 2,685 case - patients , 70% were muslim , who had rates 3 times higher than other communities . circle - specific attack rates ranged from 17/100,000 to 25/100,000 and were highest in the first 4 circles of the city where a predominantly muslim population resided ; this area accounted for 90% of cases in 20032006 . during 2006 , 81% of the cases were either smear or culture positive . we surveyed the 7 circles of the city to estimate primary vaccination coverage among children 1223 months of age , fourth diphtheria dose ( dpt ) among those 1836 months of age , and fifth diphtheria dose ( dt ) among children 5472 months of age , respectively . criteria for a completely vaccinated child were defined according to the uip vaccination schedule by age group . coverage for primary vaccination , fourth , and fifth doses was 90% ( 95% confidence interval [ ci ] 89%90% ) , 60% ( 95% ci 59%60% ) , and 33% ( 95% ci 33%34% ) , respectively . although coverage for primary vaccination did not differ among muslims and non - muslims ( coverage ratio 0.95 , 95% ci , 0.90%1.1% ) , coverage for fourth and fifth doses was lower among muslims ( coverage ratios 0.86 , 95% ci 0.75%0.99% and 0.59 , 95% ci 0.5%0.8% , respectively ) . we compared laboratory - confirmed case - patients < 10 years of age who lived in hyderabad with age- and residence - matched controls . information about educational status of parents , monthly family income , religion , and number of vaccine doses received was collected through interviews of mothers or guardians . vaccination status was ascertained from vaccination cards or the mother s history when a card was not available . all exposures were included in a stepwise conditional logistic regression by using epi info ( centers for disease control and prevention , atlanta , ga , usa ) . vaccine efficacy ( % ) was calculated by using the 1 odds ratio formula ( 6 ) . we included all children in the analysis to estimate vaccine efficacy for the first 4 doses . however , to estimate the efficacy of 5 doses of diphtheria vaccine , we restricted the analysis to children 510 years of age because the fifth dose of the vaccine is given to children > 4.5 years of age . we included 123 case - patients in the case - control study . only 20 ( 16% ) controls and 23 ( 19% ) case - patients had a vaccination card . twenty - one children ( 17% ) were younger than 5 years of age . when adjusted for religion , family income , and literacy status of parents , vaccine efficacy increased from 49% ( 95% ci 0%80% ) for 3 doses to 65% ( 95% ci 8%87% ) for 4 doses . among children > 5 years of age , efficacy for 5 doses was 91% ( 95% ci 68%98% ) ( table 2 ) . * conditional logistic regression taking into account vaccine doses , religion , family income , and parental literacy . vaccine efficacy among children > 5 y of age ; younger children could not have received that booster . our results indicate that in hyderabad , diphtheria mainly affected children 519 years of age , girls and women , and the muslim population . receiving a fourth and fifth doses of the vaccine was needed for protection against the disease . coverage of primary vaccination was adequate in the city whereas , coverage for the boosters was low . low booster coverage , especially among muslims , might have influenced herd immunity and thereby contributed to higher attack rates among this community . similar phenomena were observed in countries where diphtheria reemerged after successful control with vaccination ( 2,7 ) . several studies have reported vaccine efficacies ranging from 95% to 98% for 3 doses and from 90% to 99.9% for 5 doses ( 8,9 ) . first , misclassification may have occurred when assessing vaccination status of children that mainly relied on a mother s recollection of the child s history . second , program quality issues in vaccine supply or in cold - chain maintenance may have affected efficacy in hyderabad . however , an evaluation of the universal immunization program conducted in 2006 in hyderabad did not identify any gaps in cold - chain maintenance in the public health sector ( government of andhra pradesh , unpub . thus , we concluded that misclassification with respect to assessment of vaccination status ( using only the mother s recollection ) probably explains the low vaccine efficacy that we observed . two factors could also explain the lower booster coverage among muslims : 1 ) issues concerning the offer of vaccine by the health services or 2 ) issues concerning vaccine demand . however , primary vaccination coverage was identical among muslims and non - muslims in hyderabad . this suggests that , initially , the demand for primary vaccination was identical in all communities , but that the health system was not able to retain the same demand for boosters in the muslim community . patients with milder symptoms who might not have sought treatment at the hospital were not considered . this situation may have led to an underestimation of attack rates but would not have led to different conclusions about the persistence of the disease . second , a large proportion of children did not have vaccination cards , which may also have affected our vaccine efficacy estimates . we tried to address this factor by comparing vaccination status with the child s developmental milestones . on the basis of our study results , we propose recommendations for control of diphtheria in hyderabad . first , coverage for boosters must be improved , with special emphasis on the 4 administrative areas with high attack rates . third , because attack rates were high among adolescents , tetanus toxoid ( administered to school children at 1015 years of age ) could be replaced with a combined tetanus - diphtheria vaccine . fourth , coverage of boosters could be considered as performance indicators to improve the immunization program .

during 20032006 , diphtheria rates in hyderabad , india , were higher among persons 519 years of age , women , and muslims than among other groups . vaccine was efficacious among those who received > 4 doses . the proportion of the population receiving boosters was low , especially among muslims . we recommend increasing booster dose coverage .

idiopathic megacolon is a rare and poorly understood clinical condition that usually presents as refractory abdominal distension and constipation . it includes idiopathic megarectum , idiopathic megacolon , or a combination of these disorders . among them , idiopathic megacolon without megarectum is the most uncommon disorder . in this case , we experienced the idiopathic megacolon with bowel dilatation only proximal to the splenic flexure . one month before being referred to our hospital , abdominal distension and constipation developed and the severity of both increased with time . the patient could not defecate and the abdominal pain increased despite fasting and frequent enemas . because of the intractable symptoms , she was referred to our emergency unit for further investigation . the physical examination during the obstructive episodes revealed marked abdominal distension with hypoactive bowel sounds . the patient denied a history of metabolic , neurological , cardiovascular or pulmonary disease , previous abdominal or pelvic surgery , or a history of abdominal cancer , inflammatory disease or trauma . the laboratory testing showed a normal hemoglobin , hematocrit , white blood cell count , blood glucose , creatinine , and coagulation profile . the serum electrolytes were also normal except hypokalemia was present with a level of 2.7 mmol / l ( normal range 3.5 - 5.1 the abdominal computed tomography ( ct ) scan showed bowel distension , and the transition zone was detected at the splenic flexure ( fig . anorectal manometry showed a normal resting anal sphincter pressure , normal squeezing pressure during maximal voluntary effort , rectal sensory threshold , maximal tolerance volume ( 120 ml ) , and rectoanal inhibitory reflex was present . the colon transit time was delayed ( total 135.6 hours , right colon 66 hours , left colon 56.4 hours , and rectosigmoid colon 13.2 hours ) . based on these findings however , the abdominal distension and constipation were not improved . on the 19th day of hospitalization , a small amount of loose stool was passed and the abdominal pain was relieved . on the 22nd day of hospitalization , the frequency of loose stools increased up to 3 - 4 times / day . oral feeding was started on the 27th day of hospitalization after checking the ct endoscopy ( fig . the finding was marked dilatation of ascending and transverse colon at the level of distal transverse colon without obstructive lesion . the patient was discharged on the 44th day of hospitalization on oral mosapride , itopride , domperidone , and lactitol ( fig . after discharge , she did not have additional symptoms even after the medication was discontinued . she was admitted to a local hospital for 20 days and sigmoidoscopic irrigation and enemas were performed there severe abdominal distension was noted on physical examination and on the simple abdominal radiograph ( fig . 3a ) . the laboratory testing was normal except for alt ( 119 u / l ) and ast ( 141 u / l ) . bisacodyl / docusate was started on hospital day 2 . by hospital day 8 , loose stools were passed after a glycerin enema and the abdominal pain and distension were relieved . on hospital day 10 , a large amount of loose stool was passed and magnesium oxide , mosapride , domperidone , and colonlyte were started . stool passage was increased to 7 - 15 times / day . on hospital day 16 , oral feeding was started and the alt and ast returned to normal . on hospital day 25 , the patient was discharged ( fig . three months later , she visited the outpatient clinic and did not have abdominal distension or constipation without medication ( fig . megacolon can be observed in hirschsprung 's disease , with an infection ( chaga 's disease ) and in some disorders of the endocrine or central nervous system ( including spinal trauma and senility ) . however , in the absence of an organic cause , it is referred to as idiopathic.1,2 idiopathic megacolon is a heterogeneous disorder including idiopathic megarectum , idiopathic megacolon , or a combination of these disorders.3 idiopathic proximal hemimegacolon is defined as megacolon only proximal to the splenic flexure.4 idiopathic proximal hemimegacolon is a very rare and therefore a poorly understood clinical entity . the plausible explanations include abnormalities involving the extrinsic nerves , the enteric nerve plexus , or the intestinal smooth muscle , which could lead to gut dilatation and impaired motility.5 anal manometry is clinically useful in a few patients with chronic constipation , although it has potential value for the exclusion of hirschsprung 's disease , due to the demonstration of internal sphincter relaxation in response to rectal distension.6 the clinical features of patients with idiopathic proximal hemimegacolon are similar to those reported in idiopathic megacolon without megarectum in previous studies . patients with idiopathic megacolon are older than patients with idiopathic megarectum . in this case , the patient experienced her first episode at 44-years of age . most patients present with constipation ; however , documented bowel habits are very variable , from 5 times per day to only once every 3 months . our patient had abdominal distension and pain , normal intelligence , and no familial history . delay in the colon transit is most prominent in the right colon area . in the rectosigmoid area , it is normal.2 - 4,7,8 our patient also showed delay in right colon transit . the abnormal ast / alt levels were considered secondary to hepatocongestion induced by the megacolon . the majority of patients can be successfully managed without surgery.2 for shortening bowel transition time and symptom relief , the prokinetic drugs like mosapride and itopride could be coadministrated.9 however , medical treatment may fail to alleviate symptoms in some patients,8 may be poorly tolerated , and must be continued lifelong to prevent the recurrence of symptoms . megacolon proximal to the splenic flexure is referred to as idiopathic proximal hemimegacolon . here , we present the case of a 44-year - old woman with idiopathic proximal hemimegacolon that had 2 episodes over 4 years .

idiopathic proximal hemimegacolon is a disorder characterized by bowel dilatation proximal to the splenic flexure . it is a very rare and therefore a poorly understood clinical entity . this report describes a case of idiopathic proximal hemimegacolon in a 44-year - old woman . the patient suffered from 2 episodes of constipation and bowel dilatation over 4 years and was successfully treated by medical therapy .

laparoscopic cholecystectomy ( lc ) is the operation of choice for symptomatic gallstones , and it was performed for the first time on an outpatient basis in 1990 . several reports have demonstrated the feasibility , safety , and cost - effectiveness of this operation as an outpatient procedure . however , outpatient lc has been questioned because of difficult to control postoperative pain and nausea and because of the possibility of a higher morbidity rate . it is for these reasons that outpatient lc is not the current practice in the united kingdom . the objective of this prospective study was to assess patients ' experience and morbidity as well as unplanned admission and readmission rates following ambulatory lc at a london district hospital . from november 1999 to may 2003 , 100 well - motivated patients ( 9 males ; 91 females ; age range 18 - 69 years ; mean age 44 years ) with symptomatic cholelithiasis and an american society of anesthesiologists ( asa ) grade i or ii underwent outpatient lc . patients with acute cholecystitis , abnormal liver - function tests , asa > ii , and patients not fitting the general surgical outpatient criteria ( morbidly obese patients , patients older than 70 years , or any patient living alone ) were excluded from our study . all operations were performed in the morning at our day surgical unit by a single upper gastrointestinal consultant surgeon with an interest in laparoscopic surgery in the presence of a consultant anesthesiologist . a veress needle was used to induce pneumoperitoneum prior to performing a conventional 4-port lc with local anesthetic ( 10 ml of 0.5% bupivacaine ) infiltration of the skin incisions . all patients received a standardized anesthetic and were premedicated with 1 g acetaminophen and 400 mg ibuprofen one hour preoperatively . tramadol ( 50 mg ) was used in case of a contraindication for acetaminophen or ibuprofen . induction of anesthesia was achieved with propofol ( 2 mg / kg ) , fentanyl ( 1 mg / kg ) , and atracurium ( 0.3 mg / kg ) . anesthesia and analgesia were maintained by propofol and remifentanil as a target controlled infusion . at the conclusion of the operation , all patients in our series had adequate muscle function , and therefore we did not utilize reversal agents . patients were discharged on the same day following assessment by the operating surgeon with a contact telephone number and were followed up at 2 weeks as outpatients . the postoperative pain control regime consisted of 1 g of acetaminophen 4 times a day together with 400 mg of ibuprofen 3 times a day as a regular prescription for the first 3 days and then on an as required basis . tramadol 50 mg 4 times was used if there was a contraindication for any of the above 2 drugs . a questionnaire was mailed to all patients to assess overall experience , postoperative pain , improvement of pain , whether they would recommend the procedure to a relative or friend , and whether they would have preferred staying in the hospital overnight . there was no hospital mortality , and none of the patients required conversion to open cholecystectomy . of the 100 patients , only one required an overnight stay in the hospital following insertion of a drain that was removed the next day . thirty - five patients rated their overall experience as excellent ( 29 very good , 3 good , and 1 poor ) . seven patients experienced no pain after the operation ( 16 very mild , 8 mild , 23 moderate , and 14 severe pain ) . all patients ' right upper quadrant pain subsided or improved following surgery except for one patient who stated that it became worse . sixty - three patients ( 92.7% ) stated they would recommend outpatient lc to a friend or relative , and 14 patients ( 20.5% ) would have preferred staying in the hospital overnight . the present study demonstrates that our hospital admission rate compares favorably to that of other reported series that ranged between 1% and 39% . postoperative nausea and vomiting was the most common reason for overnight stays in the above series , but this did not appear to be a problem in our study . the length of operation has been shown to be another independent predictor for unplanned admissions . the mean length of lc in our series appears to be less than that in other series because an experienced laparoscopic surgeon performed all operations and because no concomitant procedures such as intraoperative cholangiograms were required . a preemptive antiemetic regime with cyclizine and ondansetron together with a short operation might explain our low incidence of postoperative nausea and vomiting . readmission rates after ambulatory lc range between 2% and 8% , and retained stones is the commonest morbidity after lc . only one patient in our series was readmitted for pain control , and one patient was treated with antibiotics as an outpatient for a wound infection . fourteen of the 68 ( 20.5% ) patients would have preferred spending the night in the hospital , which may , in part , be attributable to patient 's concerns about participating in a study that involves leaving the hospital on the same day , which is not actually the current practice . our experience with ambulatory lc demonstrates that this procedure can be performed safely with low morbidity and with a high satisfaction and acceptance rate in well - motivated select patients when operated upon by an experienced laparoscopic surgeon in the presence of an experienced consultant anesthesiologist .

objective : the aim of this prospective study was to evaluate patients ' experience and the outcome of outpatient laparoscopic cholecystectomy performed by a single upper gastrointestinal surgeon at a district hospital.methods:between november 1999 and may 2003 , 100 patients underwent outpatient laparoscopic cholecystectomy . patients were followed up at 2 weeks as outpatients , and a questionnaire was mailed to all patients to assess their experiences.results:none of the patients required conversion to open cholecystectomy . one patient required admission to the hospital following drain insertion , and one patient was readmitted for pain control . one patient developed an epigastric port infection that resolved with antibiotics . sixty - eight of the 100 patients completed the postal questionnaire . thirty - five patients rated their overall experience as excellent . twenty - three patients experienced very mild or no pain . all patients ' right upper quadrant pain subsided or improved following surgery except one patient who stated that it became worse . sixty - three patients ( 92.7% ) stated they would recommend outpatient laparoscopic cholecystectomy to a friend or relative.conclusion:laparoscopic cholecystectomy can be performed safely as an outpatient procedure with a high acceptance and satisfaction rate in select patients .

iatrogenic coronary artery dissections are well known complications in the era of interventional cardiology and especially with the advances in technology , devices , and operator experience that have led to the performance of more complex percutaneous interventions . management of the dissection propagating to the ascending aorta is challenging.1)2 ) we present a case of iatrogenic bidirectional dissection of the right coronary artery ( rca ) and the ascending aorta that was managed rapidly by stenting the entry point of the dissection , however , the aortic valve was involved which necessitated valve replacement surgery . a 70 year - old woman was admitted to our hospital for evaluation of recurrent angina . diagnostic catheterization through the right radial artery was performed using a 6 french ( 6 fr ) arterial sheath and revealed a heavily calcified tight stenosis in the second segment of the rca . we used a ( 6 fr ) amplatz left 0.75 ( al 0.75 ) launcher guiding catheter ( medtronic inc . , after pre - dilation with the maverick 2 2.520 mm balloon ( boston scientific company , natick , ma , usa ) , we deployed the bare metal stent tsunami 3.520 mm ( terumo , tokyo , japan ) . we postdilated with a quantum 3.520 mm balloon ( boston scientific company , natick , ma , usa ) . the first angiographic control with the guide wire 0.014 with ice hydrophilic coating ( choice , floppy ls , boston scientific , natick , ma , usa ) was good with no residual stenosis in the second segment of the rca . next , we pulled back the guide wire and the selective injection of the rca showed a permeable rca with a proximal dissection line ( fig . 1a ) and a retrograde opacification of the proximal segment of the as cending aortic wall ( fig . in addition , we found a stagnation of contrast material within several centimeters of the aortic wall and the proximal segment of the rca ( fig . we immediately advanced the guide wire 0.014 and successfully stented the rca ostia 's with another bare metal stent tsunami 3.515 mm ( terumo ) , into the entry point of the dissection ( fig . computed tomography scans performed the same day exhibited the type a ( stanford classification ) aortic dissection ( fig . 3 ) . the cardiac ultrasound taken on the same day after the procedure showed an extension of the dissection into the aortic valve leaflets with consequent massive aortic insufficiency . an isolated aortic valve replacement was performed but the post - surgical period was complicated by the incidence of uncontrollable septic shock and death occurred at day seven following the procedure . iatrogenic coronary artery dissections may be caused by direct trauma from the catheter tip or through entrapment in an atheroma plaque . other possible causes of dissection include over inflation and dilation of the balloon or stent during percutaneous coronary interventions , the dilation of a calcified plaque , the fracture of the stent strut or from aggressive handling of guide wires , specifically those that are rigid and hydrophilic . once coronary dissection occurs , it can rapidly propagate in the anterograde direction into the distal part of the coronary artery and , sometimes , in the opposite direction into the aorta.3 ) in these latter cases , if the dissection was limited to a few centimeters beyond the aortic valve , it can be treated by sealing the entry point with a coronary stent.4 ) follow up after the procedure could then be performed using a ct scan or by transesophageal echocardiography5 ) as most of these aortic dissections heal spontaneously . furthermore , life threatening situations may occur if the dissection is extensive and involves the ascending aorta which would necessitate urgent surgical intervention.3)6 ) the integrity of aortic valve should be assessed by echocardiography in all cases . in our specifc patient , the bidirectional dissection is thought to have been related to direct trauma casued by the catheter tip with a non - coaxial alignment provoked by the removal of the guide wire 0.014 after the stent implantation . the patient was hemodynamically stable and the dissection extended only 2 to 3 cm into the ascending aorta which explains the reasoning for stenting the entry point to allow the aortic dissection to heal spontaneously . the present case report suggests that if the patient 's hemodynamic conditions were stable , and the propagation of the dissection was limited to the early proximal region of the ascending aorta , sealing the coronary entry door with a coronary stent is a reasonable strategy . imaging surveillance should be considered for early detection of other complications and during the follow up examination . the surgical approach should be reserved for cases involving impairment of hemodynamic conditions or extensive involvement of the aorta .

although rare , iatrogenic aortocoronary dissection is one of the complications most dreaded by the interventional cardiologist . if not managed promptly , it can have redoubted and serious consequences . herein , we present the case of a 70 year - old woman who was treated by stenting of the second segment of the right coronary artery ( rca ) for recurrent angina but , unfortunately , the procedure was complicated by anterograde dissection of the rca with a simultaneous retrograde propagation to the proximal part of the ascending aorta . successful stenting of the entry point was able to recuperate the rca and to limit the retrograde propagation to the ascending aorta , but there was an extension of the dissection to the aortic valve leaflets resulting in a massive aortic insufficiency . therefore , an isolated surgical aortic valve replacement was performed .

a 60-year - old woman is presented who required resection of both pulmonary and adrenal metastases from cervical adenocarcinoma . the patient was diagnosed with stage ia adenocarcinoma of the cervix which was treated surgically . five years later she was diagnosed with recurrent , metastatic cervical adenocarcinoma to both lungs and her right adrenal gland . following multiple courses of chemotherapy over the next 2-years , pet - ct imaging failed to show any advancement of her disease . because of the stability of the metastatic disease and due to the fact that no futher metastases developed , a multidisciplinary team decided to consider surgical resection of her metastases . the decision was made to perform the surgery in two stages , first , a left thoracotomy for resection of the left pulmonary nodules , followed 3 months later by a right thoracotomy and a trans - diaphragmatic right adrenalectomy . this case report is unique as it involves simultaneous pulmonary and adrenal resection due to metastatic cervical cancer and that the operation required partial vena caval resection due to local spread of disease . a multi - disciplinary team approach was utilized consisting of general , thoracic and vascular surgery . the second stage of the procedure was carried out through double thoracotomy incisions a thoracotomy through the 5 intercostal space for resection of her pulmonary metastases and an additional thoracotomy through her 8 intercostal space to provide adequate exposure for retroperitoneal dissection of her right adrenal gland . following the wedge resections of her right lung metastases , dissection a firm mass was palpable prior to incising the diaphragm , and it appeared that the adrenal metastasis had invaded the diaphragm locally . a 3 cm x 3 cm portion of diaphragm was incised and dissection was carried down around the liver and the adrenal mass was excised from the caudate lobe . the cancer was noted to be invading the wall of the ivc , and therefore , a vascular surgeon was consulted to assist in completion of the resection . dissection of the caudate lobe of the liver to gain proximal and distal control of the ivc . the tumor is being retracted cephalad with a clamp . following careful dissection of the posterior ligamentous attachments of the ivc to the liver , a satinsky vascular clamp was placed and a 1 cm x 1 cm x 0.5 cm portion of the ivc was excised . the entire specimen was removed en bloc ( figure 2 ) consisting of diaphragm , right adrenal metastasis measuring 6 cm x 4 cm x 3 cm , a scant amount of normal adrenal tissue , the small patch of ivc and the right adrenal vein which was completely encased by tumor . a patch angioplasty of the ivc was then completed with a ptfe patch and sewn in with a running 5 - 0 prolene suture ( figure 3 ) . the diaphragm was closed primarily with pledgeted 1 - 0 ethibond mattressed sutures , two 28-french chest tubes were placed through separate stab incisions , and the lung was re - expanded under direct vision . the patient tolerated the procedure well and was discharged home on post - operative day 8 . the en bloc specimen with the ivc surface in the foreground and the left adrenal vein clearly visible ( arrow ) . this case is unique in that it discusses a transthoracic , transdiaphragmatic adrenalectomy for metastatic cervical adenocarcinoma and partial resection of the ivc to remove the tumor en bloc . al.(1 ) subsequently , this thoracoscopic technique has been reported in case reports , small institutional studies , and animal studies(2 ) . use of minimally invasive techniques in this case would not have been feasible or appropriate due to invasion of the ivc , but when certain criteria are met its use has been shown to be both safe and effective for what is otherwise considered to be a very morbid procedure . previously , if a patient was found to have concurrent pulmonary and adrenal lesions , both thoracotomy and laparotomy incisions would be required ; this approach is much less desirable due to reported success with the trans - diaphragmatic surgery . in 1998 , flores et . al.(3 ) described 2 cases of trans - diaphragmatic adrenalectomy with concurrent pulmonary lesions one of which consisted of a metastatic leiomyosarcoma . again in 2006 , a case report was published describing a transdiaphragmatic adrenalectomy in patients with non - small cell lung cancer and these authors(4 ) and others previously(5 ) have theorized that due to the high frequency of lung cancer with ipsilateral adrenal metastasis a direct lymphatic connection must exist between the lung and the retroperitoneum . , improved survival from combined resection of pulmonary and adrenal lesions in cases of lung carcinoma has also been shown(6 ) indicating that when this clinical situation exists simultaneous resection may be considered as a viable option . a more recent study in 2005 by mercier et . al.(7 ) , has also confirmed improved survival in patients with non - small cell lung cancer . a small case series out of bnai zion medical center in 1996 looking at 13 patients discussed using the transthoracic approach for removal of solitary adrenal masses irrespective of a concurrent pulmonary mass(8 ) . this case highlights the use of a multidisciplinary team to resect simultaneous metastases to the lung and adrenal gland from cervical adenocarcinoma through one skin incision and demonstrates the possibility of accomplishing this when the tumor is locally invasive into major structures such as the ivc . prolonged survival has been shown in small trials related to primary lung cancer with sole adrenal metastasis if both lesions are resected . simultaneous spread of cancer to the adrenal gland and to the lung should not by itself exclude complete resection . admittedly , the chance for curative resection in this patient is certainly smaller compared to her chances for cure at her initial resection , however , she had tolerated 2 years of extensive chemotherapy without any advancement in her metastases by our most sensitive screening methods . this report is meant to highlight the technical feasibility of the operation and not the rationale . the rationale for proceeding was made on an individual basis after multiple conversations between the patient and her surgeons .

we present a 60-year old woman with recurrent cervical adenocarcinoma who presented with metastasis to both lungs and to her right adrenal gland . a thoracotomy was performed for resection of her pulmonary metastasis and then the right adrenal gland was excised through a trans - diaphragmatic approach . the adrenal gland resection was more complex due to involvement of the tumor with the inferior vena cava ( ivc ) which was repaired with a ptfe patch graft . this case demonstrates both an interesting approach to surgical resection of multiple metastases as well as a safe , although more challenging , alternative to partially resect and repair the ivc .

an 18-year - old white female with a history of type 1 diabetes since age 6 years presented with a 3-year history of poorly controlled diabetes , increasing insulin requirements ( up to 3 units kg day ) and frequent hospitalizations for diabetic ketoacidosis . the patient 's diabetes had been under excellent control ( a1c 6.9% ) until she began a low - carbohydrate diet . the ensuing 50-lb ( 22.7-kg ) weight loss was exacerbated by personal stressors and the development of gastrointestinal symptoms . she reported nausea , which after dinner progressed to retching and vomiting along with the onset of abdominal pain , tachycardia , and the sudden development of severe nighttime hyperglycemia ( with capillary blood glucose levels often in the 400600 mg / dl [ 22.233.3 mmol / l ] range ) with ketonuria , which was poorly responsive to large doses of insulin ( 6080 units of insulin delivered overnight ) . associated symptoms included depression , painful sensory peripheral neuropathy , arthralgias , constipation , hair loss , dry skin , and amenorrhea . on exam , she had orthostatic changes with a baseline heart rate of 116 bpm and a blood pressure of 133/92 mmhg . additional evaluation included a urine analysis for free cortisol , dehydroepiandrosterone , transglutaminase antibodies , serum igf-1 , and glucagon levels ; a complete blood count ; and a fecal fat measurement : all were within normal levels . urine albumin levels were slightly elevated on repeated evaluations . a 6-week trial of domperidone and oral dietary supplementation along with an intensive insulin regimen failed to improve the patient 's condition , with only a marginal improvement in a1c to 10.5% . the patient was further assessed for the possibility of anorexia nervosa and insulin bulimia ( avoidance of prescribed insulin administration ) . she had previously undergone extensive gastrointestinal evaluation ( upper gi series and endoscopy ) , which had only revealed delayed transit time on gastric emptying scintigraphy . because of the suspicious presentation and negative workup , a computed tomography angiography ( cta ) was ordered that demonstrated dilatation of the first and second portions of the duodenum with normal gastric volume . compression of the third portion of the duodenum was evident , and a reduced aortomesenteric angle of 15 ( normal > 22 ) and an aortomesenteric distance of 3.3 mm ( normal > 10 mm ) were identified ( fig . an exploratory laparotomy , duodenojejunostomy , and division of the ligament of treitz were subsequently performed to relieve the mechanical obstruction . the patient 's postoperative course was uneventful : her symptoms resolved and her a1c was 8.4% at the 1-month postoperative visit and 7.0% six months later , concomitant with a 10-lb ( 4.5 kg ) weight gain . patients with poorly controlled diabetes may present with symptoms of bloating , early satiety , epigastric pain or discomfort , nausea , vomiting , postprandial fullness , and anorexia , often attributed to gastroparesis . we report a case of smas in a patient with type 1 diabetes mistakenly diagnosed as gastroparesis . this case is interesting because the most severe manifestations of smas occurred after the evening meal . they were exacerbated by the patient lying supine and were associated with ketogenesis and a significant degree of resistance to insulin therapy . smas is a term used to define the clinical signs and symptoms caused by compression of the third portion of the duodenum between the angle made by the aorta and the superior mesenteric artery ( sma ) . the compression is caused by a decrease in the aortomesenteric angle and distance , with complete obstruction observed with angles between 6 and 16 and a distance of 28 mm ( 36 ) . smas is thought to occur because of duodenal compression following the loss of the mesenteric fat pad , which maintains the angle and distance between aorta and sma . this can be seen in acute catabolic states , dietary disorders such as anorexia nervosa , chronic wasting diseases , and rapid weight loss following bariatric surgery . the importance of reporting this case lies in heightening the diagnostic index of suspicion for smas in patients with type 1 diabetes presenting with suspicious gastrointestinal symptoms and glycemic instability . patients manifesting intermittent and recurrent postprandial abdominal pain , nausea , vomiting , and anorexia associated with weight loss ( all often attributed to diabetic gastroparesis ) should be screened for smas . a variety of modalities have been utilized for diagnosis ( 7,8 ) ; most have been largely replaced by the noninvasive and more accurate helical computed tomography with oral and intravenous contrast and three - dimensional reconstruction ( 9 ) . in conclusion , patients with type 1 diabetes presenting with gastrointestinal symptoms associated with recent weight loss or low bmi should be evaluated for smas . computed tomography angiography is a quick and reliable diagnostic tool to identify the structural and anatomic abnormalities associated with this syndrome .

objective to describe and discuss a case of superior mesenteric artery syndrome ( smas ) presenting with gastrointestinal signs and symptoms mistakenly attributed to , and treated as , diabetic gastroparesis.research design and methods a case report was compiled describing the clinical presentation , including history and physical examination , evaluation , diagnosis , and treatment of a patient with type 1 diabetes presenting with gastrointestinal complications.resultsclinical suspicion combined with the appropriate radiological evaluation led to a diagnosis of smas , with classic findings of reduced aortomesenteric angle and distance . surgical intervention resulted in resolution of symptoms and recovery of glycemic control.conclusionsthe possibility of smas should be considered in patients with type 1 diabetes presenting with gastrointestinal symptomatology , especially when associated with weight loss .

alwan et al . showed that ethanolic extract of mc inhibited aryl hydrocarbon hydroxylase ( ahh ) activity and h - benzo(a)pyrene ( h - bp ) binding to rat liver microsomal protein , effectively . in the same study , no inhibitory effect was shown with aqueous extracts . in another study of the same author , different organic extracts of eight plants were tested against ahh activity and h - bp binding to dna in vitro . the obtained in vitro effects of plants are similar with the in vivo effects from the previous study , and mc showed significant inhibitory effect when n - butanol extract was used . the n - butanol extract was more effective than the extracts of chloroform and petroleum - ether , respectively . none of the aqueous extracts showed any inhibitory effects on both ahh activity and h - bp binding to dna . in a screening study performed at unlv cancer research institute at brigham young university , essential oils of various plants were tested against different cancer cell lines at 2005 . at 100 g / ml concentration , mc essential oil showed 81.4% cell line inhibition at breast cancer cell line , while at 200 g / ml concentration the inhibition percent was 67 and 95.2 for prostate and breast cancer cell lines , respectively . probably , the most exciting and hopeful result of this study is the inhibition value of essential oil of mc on 3t3 fibroblast cell line is 3.7% and 6.5% for the dosages of 100 this kind of screening studies are fast , inexpensive , and very useful for providing well - directed leads for further studies . in another study , authors tested mc for its anticancer effects on ehrlich tumor of injected cd1 mice . in this study , authors postulated that mc distilled oil has cancer preventive properties , and they determined maximum tolerance dose ( mtd ) for mc distilled oil first . at the cd1 race mice , mtd is found 2 ml / kg and the ld50 is 2.5 ml / kg . from the four group of animals into which ehrlich tumor is injected , one group is used as a control group , the other three groups are given different doses of mc l. ( doses are 0.1 , 0.2 , 0.4 ml / kg for the 2 , 3 , 4 , experiment groups , respectively ) . furthermore , we performed a similar study with erlich ascites tumor injected mice and found that mc water extract provides almost complete cure in these animals when injections for extract and ascites cells had been started to apply together . in our study , animals with erlich ascites tumor died about 19 days after injection , but extract treated animals continue to live and we terminated the study at 29 day of the experiment and showed that there is also histopathologically sumlessful cure . methanol and hot water extracts of mc were tested for their anticancer activities against two cancer cell lines ( 5637 and mcf-7 ) by yemeni researchers . although antimicrobial activity with mic values 125 g / ml against gram - positive bacteria and a significant antioxidant activity were determined for mc , ic50 values for anticancer activity test were calculated as > 50 g / ml . myrtucommulone , a nonprenylated acylphloroglucinol , is probably one of the most attracting molecules in mc . besides its other properties , recently it was shown that myrtucommulone - a induced apoptosis in cancer cell lines , with marginal cytotoxicity for non - transformed cells , via the mitochondrial cytochrome c / apaf-1/caspase-9 . in the mentioned study , authors showed that cell death had been caused via apoptosis and they found it to be much less cytotoxic for non - transformed human peripheral blood mononuclear cells ( pbmc ) or foreskin fibroblasts ( ec50 cell death = 20 - 50 m ) , and myrtucommulone up to 30 m hardly caused processing of poly(adp - ribose)polymerase ( parp ) , caspase-3 , -8 , and -9 in human pbmc . they concluded that the myrtucommulone -induced apoptosis was mediated by the intrinsic rather than the extrinsic death pathway ; hence , myrtucommulone caused loss of the mitochondrial membrane potential in mm6 cells and evoked release of cytochrome c from mitochondria . furthermore , it was found that jurkat cells deficient in caspase-9 were resistant to myrtucommulone - induced cell death and no processing of parp or caspase-8 was evident . in cell lines deficient in either cd95 ( fas , apo-1 ) signaling , fadd or caspase-8 , myrtucommulone was still able to potently induce cell death and parp cleavage . when different myrtucommulones were tested against different cancer cell lines , it was shown that aromatic phloroglucinol core is essential for the cytotoxic activity ; myrtucommulone types without phloroglucinol core do not have cytotoxic effects against some cancer cell lines while myrtucommulones with phloroglucinol core have cytotoxic effects against the same cancer cell lines . these results are so important and exciting , because all of these findings look like that sound of footsteps of the pioneer of an ideal and selective anti - cancer drug in the near future . but we need to perform new studies in order to support and improve the results for developing long - awaited cancer drug .

myrtus communis ( mc ) l. is a well - known mediterranean plant with important cultural significance in this region . in ancient times , mc was accepted as a symbol of immortality . maybe due to this belief , it is used during cemetery visits in some regions . although it is a well - known plant in cosmetics , and there is a lot of studies about its different medical properties , anticancer studies performed using its different extracts or oils are not so much , but increasing . we collected these anticancer property - related studies in this review .

prostate cancer is a common condition amongst men and its prevalence has been increasing because of the aging population worldwide . even when present , it is only seen as a late complication in patients with widespread distant metastases in various organs . here p.h . is a 56-year - old male patient with a background history of metastatic castration - resistant prostatic adenocarcinoma ( initial gleason score of 5 + 4 = 9 ) . he presented with 8 weeks history of worsening expressive dysphasia and his prostate - specific antigen ( psa ) on admission was 1,472 g / l ( normal range : initial computed tomography ( ct ) brain demonstrated a contrast - enhancing mass measuring 47 mm 21 mm 47 mm associated with calcification and extensive vasogenic edema in the left frontal lobe ( fig . 1 ) . extending from the mass lesion is a cystic structure measuring 44 mm that appears to be compressing on the genu of the corpus callosum . magnetic resonance imaging ( mri ) subsequently showed six additional metastases throughout the supratentorial compartment . preoperative ct brain with contrast in axial ( a ) , coronal ( b ) , and sagittal ( c ) section demonstrating the left frontal lesion with heterogeneous contrast enhancement , calcification and a tumor cyst compressing on the genu of corpus callosum . preoperative mri brain with gadolinium in axial ( d ) , coronal ( e ) , and sagittal ( f ) section demonstrating the same lesion . ct , computed tomography ; mri , magnetic resonance imaging . postoperative mri brain with gadolinium in axial ( a ) , coronal ( b ) , and sagittal ( c ) section demonstrating the postoperative changes following a partial debulking of the tumor and drainage of the tumor cyst with evidence of residual - enhancing lesion in the posteromedial margin . mri , magnetic resonance imaging . u.s . is a 65-year - old male patient with no known history of malignancy , presented with a left temporal soft tissue mass that had been increasing in size over the preceding 2 months . ct showed a contrast - enhancing mass measuring 91 mm 62 mm 96 mm extending from the left middle cranial fossa , through the temporal bone and into the subgaleal region ( fig . the mass effect of the lesion results in a midline shift of 11 mm and the bone within the lesion has a sunburst type appearance . staging ct demonstrated extensive sclerotic bony lesions throughout the axial skeleton and a moderately enlarged prostate associated with extensive internal and external iliac lymphadenopathy . biopsy of the extracranial portion of the soft tissue mass was performed and histology confirmed metastatic adenocarcinoma of prostatic origin . ct brain with contrast in axial ( a ) and coronal ( b ) section demonstrating the left frontotemporal mass with homogenous contrast enhancement invading through the skull and into the middle cranial fossa . the bone window of ct brain in axial ( c ) and coronal ( d ) shows the sunburst appearance of the skull . cerebral metastases are the most common intracranial neoplasms in adults , representing more than 50% of all the reported brain tumors.1 2 it is a frequent neurological complication that was reported in 10 to 40% of adults and 6 to 10% of children with malignant disease.2 3 4 with the advance of more targeted oncological treatment , and hence an improved survival and an increasing use of mri , there has been a steady rise in the incidence of cerebral metastases over the last two decades.5 6 in particular , approximately 170,000 new cases cerebral metastasis is reported annually in the united states.7 primary tumors that are commonly associated with cerebral metastasis include small cell lung cancer , nonsmall cell lung cancer , melanoma , renal cancer , breast cancer , and colorectal cancer.6 prostate cancer , however , rarely metastasizes to the brain . early autopsy study by saitoh et al reported a 1.1% rate of cerebral metastasis amongst 1,885 patients with prostate cancer.8 such rate of cerebral metastasis is noted to be even lower in clinical studies , with only 4 out of 3,732 patients with prostate cancer were found to have intracranial involvement.9 despite the rarity of this condition , cerebral metastases from prostatic carcinoma are relatively well described in the literature . several case reports have been published over the last few decades regarding prostatic metastases in the supratentorial region,10 11 posterior fossa,10 11 12 brain stem,13 and cerebellopontine angle.14 15 here , we reported a rare case of metastatic prostate adenocarcinoma presented as a cystic and solitary intraparenchymal tumor with no evidence of systemic disease . intracranial metastasis from prostate cancer is rare , and prostatic metastasis manifesting as a cystic intraparenchymal tumor is even rarer . bland et al reported the first case of a large cystic intracranial metastasis from prostate cancer in 1992.16 since then , three other similar cases were reported by a case report by behrens et al and a case series by tsai et al . in 2001.17 18 therefore , to the best of our knowledge , this is the fifth reported case of prostatic metastasis manifesting as cystic intraparenchymal tumor in the literature . our second patient was not known to have prostate cancer before his initial presentation with a soft tissue mass in the scalp . base on the radiological features of the lesion , osteosarcoma , and atypical meningioma were both considered as potential differential diagnoses . adenocarcinoma of the prostate was only diagnosed following the biopsy results of the soft tissue mass in the scalp . subsequent staging ct scan demonstrated an enlarged prostate with patchy enhancement and widespread sclerotic bony metastases throughout the axial skeleton . to the best of our knowledge , this is the first reported case of prostate adenocarcinoma with a solitary cerebral metastasis as the first manifestation . prostatic metastasis to the brain is considered a late complication and it is associated with a poor prognosis . early diagnosis and treatment of prostate cancer plays an essential role in preventing the progression of the disease to the brain . with the current lack of funding to the national health service and the lack of a reliable test to implement a screening program for prostate cancer , the incidence of cerebral metastasis secondary to prostate cancer is likely to increase in the near future .

cerebral metastasis secondary to prostatic adenocarcinoma is rare and it is usually a late complication in patients with widespread distant metastases . here , we report two unusual cases of such a rare condition . our first case presented with a large frontal contrast - enhancing lesion - associated calcification and a large tumor cyst as shown on computed tomography and magnetic resonance imaging . this is the fifth reported case of prostatic metastasis manifesting as a cystic intraparenchymal tumor in the literature . the second case presented with a large soft tissue mass in the scalp and this lesion appeared to invade through the skull and into the middle cranial fossa . he was not known to have prostate cancer before his initial presentation and it was only diagnosed following histology results of the scalp lesion .

a 61-year - old man came to the hospital complaining of bilateral flank pain . left flank pain began approximately a month earlier , followed by right flank pain 4 days later . the patient also complained of dyspnea on exertion , urinary frequency , and foamy urine . he was diagnosed with hypertension 15 years prior and had taken traditional korean herbal medicines , such as acanthopanax , phellinus , licorice root , and steamed red ginseng . one week before coming to our hospital , he visited a local ophthalmologic clinic for bilateral floater symptoms and was diagnosed with anterior uveitis and treated with systemic and topical steroids . laboratory tests conducted at the hospital showed a blood urea nitrogen of 26 mg / dl ( the upper limit of normal ) , a serum creatinine of 2.3 mg / dl , and an estimated glomerular filtration rate ( gfr ) of 30.9 ml / min ( normal , 80 to 120 ml / min ) . a kidney biopsy specimen revealed diffuse interstitial infiltration of lymphocytes and eosinophils and mild tubular atrophy with focal fibrosis of the interstitium ( fig . systemic administration of prednisolone 1 mg / kg / day was started and slowly tapered ; furosemide and carvedilol were added for blood pressure control . the patient 's visual acuity was 20 / 20 in the right eye and 20 / 25 in the left eye . a slit lamp examination showed a mild degree ( 1 + to 2 + ) of inflammatory cells in the anterior chamber of both eyes ; however , synechia of the iris was not observed . based on these findings , a diagnosis of tinu syndrome was made , and the patient was treated with a topical steroid ( pred forte ; allergan , irvine , ca , usa ) for anterior uveitis . tinu syndrome is reportedly three times more common in women than in men , with a median age at onset of 15 years ( range , 9 to 74 years ) . although diagnosis of tinu syndrome is uncommon after 61 years of age , we experienced a patient with such a case . generally , renal involvement in tinu is mild ; however , in our patient the grf decreased to well below the normal range . failure to detect the disease earlier may have been the possible cause of the renal impairment . several medications , such as antibiotics and non - steroidal anti - inflammatory drugs , have been reported as a cause of tinu syndrome . using traditional korean medicines for 9 years tinu is an uncommon disease and is primarily a diagnosis by exclusion ; symptoms are associated with both uveitis and interstitial nephritis . general symptoms with tinu include fever ( 53% ) , weight loss ( 47% ) , general weakness and malaise ( 44% ) , and anorexia ( 28% ) . ocular effects seen with uveitis are limited to the anterior chamber in approximately 80% of cases ; both eyes are affected in 77% of cases . posterior uveitis can sometimes be seen . in most cases ( 65% ) , nephritis precedes uveitis ; however , in 21% of cases , uveitis is present before nephritis , and in 15% the two conditions occur simultaneously . symptoms associated with nephritis are typical of acute interstitial nephritis , such as flank pain , pyuria ( mainly sterile ) , hematuria , and proteinuria ( which is less severe than seen with nephrotic syndrome ) an association with human leukocyte antigens has been mentioned in some reports , as well as sensitization of common antigens located both in the eye and the kidney as possible pathologic processes of tinu . in addition to hypersensitivity reactions , the causative drug may act as a hapten , inducing cytokine production or vasculitis . another possible etiology is cross reactivity of active transporters that are inhibited by carbonic anhydrase inhibitors in both the renal tubule and ciliary epithelium . eosinophilia , elevated c - reactive protein , elevated erythrocyte sedimentation rate , normocytic normochromic anemia , and abnormal liver function tests are among the possible findings . in addition , anti - neutrophil cytoplasmic antibody , anti - nuclear antibody , and rheumatoid factor may be positive , and complement levels may be decreased . in the present case , all laboratory findings were within the normal range except for kidney function . in the absence of proteinuria and pyuria , detection of beta-2 microglobulin in the urine the degree of proximal or distal tubular dysfunction may not be directly proportional to the decrease in gfr . tubulointerstitial edema and infiltration can be observed in kidney biopsy under a light microscopy , while the glomerulus and blood vessels are relatively normal ; this is consistent with the present case . infiltration with lymphocytes , plasma cells , and histiocytes may be observed , and in 34% of cases , eosinophils . immunofluorescent findings are negative for antibody or complement in 86% of cases ; similarly , no antibodies or complements were detected in our patient . the differential diagnosis of tinu includes sarcoidosis , sjogren 's syndrome , behcet 's disease , wegener 's granulomatosis , systemic lupus erythematosis , hyperthyroidism , primary hyperparathyroidism , and infectious diseases ( e.g. , tuberculosis , brucellosis , toxoplasmosis , and herpes ) . tinu is often not recognized and affected patients often fail to seek medical care , making the prevalence of the disease appear less than in actuality . topical steroids are commonly recommended as the treatment of choice of anterior uveitis . during the initial attack , topical steroids are usually sufficient to control the inflammation . based on published case reports , ocular symptoms resolved in 3 months in 44% of cases while symptoms persisted for more than 3 months in 14% of cases if the inflammation is not controlled with steroids or if steroid use is contraindicated or not tolerated , immunosuppressive agents such as azathioprine or methotrexate may be considered . a diagnosis of tinu should be considered in patients with idiopathic uveitis presenting with renal disease even in elderly patients . as tinu is diagnosed by exclusion , a kidney biopsy and a thorough ophthalmologic examination are needed . once the diagnosis of tinu is made , immediate steroid therapy is necessary together with a collaborative approach to treatment by an internist and an ophthalmologist .

tubulointerstitial nephritis and uveitis ( tinu ) syndrome is a rare disease entity usually occurring in children . in the present study a case of tinu syndrome in an elderly patient is described and relevant literature is reviewed . a 61-year - old man presented with bilateral flank pain , urinary frequency , and foamy urine . a kidney ultrasonography revealed an increase in kidney parenchyma echogenicity . following a kidney biopsy , the patient was diagnosed with acute tubulointerstitial nephritis . an ophthalmology examination initially performed for floater symptoms , revealed anterior uveitis in both eyes . acute tubulointerstitial nephritis and anterior uveitis in both eyes responded to treatment with oral prednisolone , furosemide , carvedilol , and a topical steroid . tinu syndrome can occur in the elderly and should be part of the differential diagnosis when seeing a patient who has uveitis in association with renal disease ; any therapy should be managed by both an internist and an ophthalmologist .

capsular block syndrome ( cbs ) has been known to occur as a rare complication of cataract surgery with continuous curvilinear capsulorhexis ( ccc ) and a posterior - chamber lens implant . typically , it presents with reduced vision in the early postoperative period and is characterised by a forward displacement of the posterior - chamber intra - ocular lens and an accumulation of intra - capsular opaque material . management of cbs is usually by nd : yag laser capsulotomy . we describe here a unique case of very - delayed - onset cbs with good visual acuity , occurring 8 years after surgery . a 75-year - old lady was referred to the eye department with a 6-month history of misty and fuzzy vision in her right eye . eight years earlier , she had undergone uncomplicated bilateral cataract surgery by phacoemulsification with lens implants . upon examination , her logmar scale best - corrected visual acuity ( bcva ) was 20/20 in both eyes . refractive error was 1.50 ds for the right eye and + 0.25 ds for the left eye , which suggested a myopic shift in the right eye . the left eye was normal , but there was a hazy appearance in the right eye in the retro - lenticular space between the posterior surface of the lens optic and the posterior capsule . the space was homogenously whitish and slightly opalescent , characteristic of a milky , turbid fluid ( fig . 1 ) . there were no signs of inflammatory activity . on clinical examination , the lens implant did not appear to have shifted forward and the fundus examination was normal . intra - operatively , a 30-gauge needle mounted on a 1-ml syringe was passed through the sclera and pars plana , 3.5 mm behind the limbus in the infero - temporal quadrant . the needle tip was gently passed through the posterior capsule and 0.2 ml of the turbid fluid was aspirated and then sent for microbiological culture and analysis . intra - vitreal vancomycin ( 1 mg/0.1 ml ) was administered at the end of the procedure . she was reviewed after 5 days and there were no signs of inflammation or endophthalmitis . in addition , her myopic shift had disappeared and she now had a + 0.25 ds for both eyes , both still with a bcva of 20/20 . two months afterwards , at follow - up , she was asymptomatic and reported complete resolution of her misty vision . examination showed a bcva of 20/20 , with a clear papillary axis and an intact posterior capsule and lens implant . it is mainly known to occur with ccc and phacoemulsification , but has also been reported with can - opener - type capsulorhexis , extracapsular cataract extraction and intra - ocular lens implantation in the sulcus [ 4 , 5 ] . our case did not have reduced visual acuity and only had symptoms of haziness and mistiness , which is quite unusual and , to the best of our knowledge , has not been reported in the literature before . miyake et al . divided the condition into 3 different types , depending upon the time of onset : intra - operative , early post - operative and late post - operative . they postulated that , in the late post - operative period , the cortical cells undergo metaplastic changes and proliferate in the bag , which leads to posterior capsular opacification and also causes occlusion of the capsular opening by sealing off the gap between the anterior capsule and the lens implant . these metaplastic cells also cause the release of a turbid fluid , which gets retained in the retro - lenticular space . upon electrolysis , this fluid has been found to be made of alpha - crystallin and collagen released from the necrotic and/or apoptotic autolysed cells [ 7 , 8 ] . eifrig described 3 cases with capsulorhexis - related lacteocrumenasia ( lacteo = milky and crumen = small bag or coin purse ) . the aspirated fluid was examined with electrophoresis and showed a large amount of alpha - crystallin and a small amount of albumin . the study concluded that it was unlikely to be a result of an antigen - antibody reaction in the capsular bag as there was no gamma globulin detected . there have been a few reports of late - onset cbs being linked to propionibacterium acnes [ 9 , 10 ] . carlson and koch reported a case of p. acnes - associated endophthalmitis after nd : yag laser capsulotomy . these reports led us to err on the side of caution , and we therefore opted to perform the aspiration of the fluid via the pars plana , rather than using the conventional approach of nd : yag laser capsulotomy . our case had the unique feature of a very late presentation 8 years after an uncomplicated phacoemulsification and ccc . in addition , in comparison to most of the reported literature where the visual acuity was dramatically reduced , our patient had a bcva of 20/20 and only had symptoms of misty vision with a reduction in her contrast sensitivity , which improved after the aspiration of the fluid . in summary , late - onset cbs can present after a long time and may cause significant symptoms . the usual treatment for the condition is nd : yag laser capsulotomy , but an infective aetiology could be present and this may cause a dissemination of intra - ocular infection . we have described a pars plana removal technique with simultaneous administration of intra - vitreal antibiotics , which we believe is a safe approach for this condition .

capsular block syndrome ( cbs ) has been known to occur as a rare complication of cataract surgery with continuous curvilinear capsulorhexis and a posterior - chamber lens implant . typically , it presents with reduced vision in the early postoperative period and is characterised by a forward displacement of the posterior - chamber intra - ocular lens and an accumulation of intra - capsular opaque material . management of cbs is usually by nd : yag laser capsulotomy . in this report , we describe a unique case of very - delayed - onset cbs with good visual acuity , occurring 8 years after surgery . it was treated successfully with surgical removal of the opaque material .

new records of yeasts from yokjido , korea were screened from 60 yeast strains from yokjido by searching keris , pubmed , and other fungal taxonomy databases [ 9 , 11 , 20 ] . cryptococcus uzbekistanensis yj10 - 4 and cryptococcus adeliensis yj19 - 2 , which were isolated from chrysanthemum coronarium in yokjido , korea , were finally screened as two new records of yeasts . the phylogenetic tree was constructed based on the large - subunit rdna d1/d2 domain sequence . c. adeliensis yj19 - 2 finally , we reconfirmed the two newly recorded yeasts as c. adeliensis yj19 - 2 and c. uzbekistanensis yj10 - 4 and submitted their sequences to the genbank database with accession nos . kj410348 ( c. adeliensis yj19 - 2 ) and kj410347 ( c. uzbekistanensis yj10 - 4 ) . c. uzbekistanensis was first isolated by powel et al . from an immunocompromised patient with lymphoma , and yalcyn et al . reported the molecular characterization and lipase profiling of c. uzbekistanensis isolated from environments contaminated with petroleum . sipiczki condueted a molecular taxonomic analysis of c. adeliensis isolated from verbascum flowers , and velzquez et al . and scorzetti et al . the morphological and cultural characteristics of the two newly recorded yeasts are summarized in table 1 and fig . 2 . c. uzbekistanensis yj10 - 4 was round in shape , while c. adeliensis yj19 - 2 was oval - shaped . the two newly recorded yeasts grew well in ypd medium , yeast extract - malt extract medium , and potato - dextrose broth . c. uzbekistanensis yj10 - 4 grew in vitamin - free medium , was tolerant to sugar and salt and also grew in 50% glucose and 5% nacl - ypd broth . we investigated the assimilation of various carbon sources by the two newly recorded yeasts using the api 20c aux yeast identification kit ( biomriux , marcy - i'etoile , france ) ( table 2 ) . both the yeasts utilized d - glucose , 2-keto - d - gluconate , l - arabinose , d - cellobiose , d - maltose , d - saccharose and d - raffinose . however , d - xylose , d - sorbitol , methyl--d - glucopyranoside and d - melezitose were only utilized by c. uzbekistanensis yj10 - 4 . in particular , c. adeliensis yj19 - 2 utilized only seven kinds of carbon sources that were utilized by c. uzbekistanensis yj10 - 4 . furthermore , the two newly recorded yeasts did not ferment all of the carbon sources tested , such as d - glucose , d - galactose , d - fructose , sucrose , maltose , lactose , mannose , raffinose , starch , cellobiose , and sorbitol ( data not shown ) . we compared the morphological and cultural characteristics of these new yeast strains with those of the known strains of the respective species . c. adeliensis from antarctica , which was obtained from the cbs - knaw fungal biodiversity center was very similar to c. adeliensis yj19 - 2 in that both the strains appeared globose to subglobose , measured ( 3~9 ) ( 2~7 ) m in 5% malt extract medium , and looked cream colored after being cultured for 30 days . its optimal growth temperature ranged from 25~30 and it did not grow in 50% glucose - ypd and 20% nacl - ypd medium . the first isolated c. uzbekistanensis strain was from s patient with lymphoma ; it was obtained from the cbs - knaw fungal biodiversity center , and the yeast extrac was very similar to the extract of the new yeast strain ( c. uzbekistanensis yj10 - 4 ) in terms of urease activity and growth of the yeast in 50% glucose - ypd . c. uzbekistanensis was round , approximately 5 m in diameter , and asexually reproduced by budding . the physiological functionalities of the supernatants and cell - free extracts obtained from the newly recorded yeasts were investigated ( table 3 ) . the antihypertensive ace - inhibitory activities of supernatants from c. adeliensis yj19 - 2 and c. uzbekistanensis yj10 - 4 were 71.8% and 61.4% , respectively , and the inhibitory activities of the supernatants were also higher than those of the cell - free extracts ( 37.2% and 29.5% , respectively ) . these results were higher than those of the biomass from s. cerevisiae kctc 7904 ( 42.1% ) , pichia anomala ( 31.0% ) , and its mutant ( 16.0% ) . moreover , these results were similar to those for pichia anomala kccm 11473 ( 72.0% ) and kluyveromyces fragilis kctc 7260 ( 68.9% ) but lower than that for edible mushrooms , pleurotus cornucopiae ( 78.0% ) . the antidiabetic -glucosidase - inhibitory activity of the cell - free extract from c. adeliensis yj19 - 2 was very high ( 81.2% ) . this inhibitory activity was higher than that of aspergillus oryzae n157 - 1 ( 48.3% ) , but was lower than that of pichia burtonii y257 - 7 ( 90.9% ) isolated from traditional korean fermented foods . tyrosinase - inhibitory activity in the c. uzbekistanensis yj10 - 4 supernatant was 39.2% , and the other physiological functionalities were either not detected or weak ( 15 ) . from the results described above , we conclude that antihypertensive ace - inhibitory activity ( 71.8% ) and antidiabetic -glucosidase - inhibitory activity ( 81.2% ) of a new yeast strain , c. adeliensis yj19 - 2 , were higher than those of other known yeasts and the other new yeast strain , c. uzbekistanensis yj10 - 4 . therefore , c. adeliensis yj19 - 2 would be very useful for food or medicinal applications as a potent bioactive compound - producing yeast .

two new yeast records , cryptococcus adeliensis yj19 - 2 and cryptococcus uzbekistanensis yj10 - 4 were screened from 60 yeasts strains that were isolated and identified from wild flowers in yokjido , gyeongsangnam - do , korea . the morphological and cultural characteristics of the newly recorded yeasts and the physiological functionalities of the supernatants and cell - free extracts obtained from their cultures were investigated . the two newly recorded yeasts did not form ascospores and pseudomycelia . they also grew well in yeast extract - peptone - dextrose broth . c. uzbekistanensis yj10 - 4 grew in a vitamin - free medium and was also tolerant to sugar and salt . antihypertensive angiotensin i - converting enzyme inhibitory activity of the supernatant from c. adeliensis yj19 - 2 was high ( 71.8% ) and its cell - free extract also showed very high ( 81.2% ) antidiabetic - glucosidase inhibitory activity .

sarcoidosis is a multisystem granulomatous disorder that is manifested by the formation of granulomas without central necrosis . this disease can affect any part of the body , but the lungs and mediastinal lymph nodes and the skin , heart , eyes , and joints are mostly involved in a majority of patients . chest imaging and multidetector computed tomography are usually performed to confirm the final diagnosis of this disorder . hence , taking a skin biopsy may aid in prompt diagnosis of the disease , particularly of scars or sites of trauma , such as that caused by tattooing . permanent makeup is a type of cosmetic tattoo , in which the colorants ( pigments ) are deposited in the dermis after piercing the skin using tiny solid needles . adverse effects such as local inflammation , infection , and allergic reactions on the skin and even systemic adverse effects such as sarcoidal reactions are common . the important histopathological finding of sarcoidosis is the existence of noncaseating granulomas devoid of prominent infiltrates of lymphocytes or plasma cells . sarcoidal granulomas in cosmetic tissues are a sign of cutaneous sarcoidosis and may be associated with systemic sarcoidosis . we describe a case of cutaneous sarcoidosis in a long - lasting cosmetic tattoo reaction , which led to the diagnosis of pulmonary sarcoidosis . a 45-year - old female was referred to the dermatology clinic of our hospital with a 15-month history of lesions on her tattoo . her medical and family histories were unremarkable , except for development of chronic exertional dyspnea and a mild cough 9 months before the development of the skin lesions . on examination of the tattoo , the tail of her tattoo was slightly raised with fine scaling [ figure 1 ] . the results of a skin biopsy demonstrated nonnecrotizing granulomas with few lymphocytes around the granuloma and negative results on acid - fast bacillus and fungal stains [ figure 2 ] . in addition , the chest imaging revealed perihilar lymphadenopathy and few small nodules in both lungs [ table 1 ] . interstitial markings , especially in the peripheral part of the lungs , were increased and peribronchial cuffing was detected [ figure 3 ] . cutaneous sarcoidosis lesion in the cosmetic eyebrow tattoo histopathology of skin biopsy taken from cutaneous lesion showing infiltration of few lymphocytes around the nonnecrotizing granulomas indicative of cutaneous sarcoidosis radiographic stages of pulmonary sarcoidosis chest x - ray of patient showing bilateral hilar lymphadenopathy and few diffuse reticulonodular opacities in both lung fields investigations for sarcoidosis , as well as for ophthalmologic and respiratory consultations , were conducted . blood tests revealed an abnormally high level of angiotensin - converting enzyme ( ace ) . a high - resolution computed tomographic scan revealed a diffuse increase in the interstitial markings ( stage ii pulmonary sarcoidosis ) and mediastinal adenopathy . the dose of prednisone was tapered to 10 mg / day for maintenance therapy ( for 6 weeks ) . the patient was administered a potent topical steroid for her cutaneous sarcoidosis lesion on her eyebrow for 6 weeks ; followed by pimecrolimus 1% cream as maintenance treatment ( for 6 weeks ) . on follow - up at 24 weeks cosmetic tattooing has , however , become a risk factor for several adverse reactions related to inks , procedures , and settings . these range from simple infections at the site ; transmission of infection , for example , syphilis , tuberculosis , and hepatitis ; to allergic reactions to the pigments used , usually mercury . both infectious and noninfectious adverse reactions have been observed in otherwise healthy people with significant morbidity . sarcoid reactions to pigment placement in the skin are not uncommon , but the development of tattoo granulomata as a presenting feature of sarcoidosis is rare . a tattoo may become involved after several years as a part of idiopathic sarcoidosis usually in the absence of other cutaneous or systemic lesions . in contrast , the tattoo pigment may elicit granulomatous hypersensitivity and induce systemic sarcoidosis . while the involvement of a scar or tattoo in systemic sarcoidosis is rare , tattoo sarcoidosis has been reported as a presenting sign of systemic sarcoidosis . the latency period between the act of tattooing and the development of cutaneous symptoms varies . in the current case , the patient manifested with dyspnea and a mild cough , 9 months before the development of the cutaneous sarcoidosis lesion . there was a interval of 15 years between the cosmetic tattooing of the eyebrow and the diagnosis of cutaneous and pulmonary sarcoidosis , reflecting the complexity of this association . , the present case highlights the fact that cutaneous sarcoidosis can develop in a long - standing tattoo .

cosmetic tattooing has become a risk factor for several adverse reactions related to inks , procedures , and associated clinical conditions . development of a sarcoid reaction to the inserted tattoo pigment in the skin is not common . we report a 45 year - old patient with history of dyspnea and mild cough since two years who had subsequently developed reddish , scaly lesions in her 15-year old tattoo done near her right eyebrow . skin biopsy of the tattoo lesion revealed cutaneous sarcoidosis which led to further investigations and a diagnosis of pulmonary sarcoidosis . the present case highlights the fact that cutaneous sarcoidosis can develop in a long - standing tattoo . also such a patient should be screened for systemic sarcoidosis disease .

there are only limited case reports of isolated tubercular involvement of the parietes even though tuberculosis is a rampant in developing countries and with the rapid spread of acquired immune deficiency syndrome ( aids ) it has made inroads into the developed nations as well . the common organs of involvement are the lungs , kidneys , bones , and gastrointestinal tract . the varied manifestations seen in tuberculosis are because of the difference in the number and virulence of bacilli , the routes of infection and the hosts immune status . a 62-year - old male presented to outpatient department of surgery with a painless , gradually increasing swelling over anterior abdominal wall for the last three years . there were no other symptoms and the patient did not have any other past medical history suggestive of tuberculosis . physical examination revealed a nontender swelling ( 7 cm 5 cm ) on the anterior abdominal wall to left of midline which extended from left hypochondrium above to the umbilicus below [ figure 1 ] . the swelling was soft and cystic in consistency and failed to disappear while making the anterior wall muscles taut . chest radiograph and blood chemistry including human immunodeficiency virus ( hiv ) test did not reveal any abnormality . ultrasonography and computerized tomography ( ct ) scan examination revealed two cystic masses one 3.7 5.2 cm and another 7.4 5.4 cm abdominal parietal wall mass ( predominantly cystic ) of mixed echogenicity , one in right hypochondrium and another in left hypochondrium extending to umbilical region with peripheral enhancement [ figure 2 ] . the differential diagnosis multiple hydatid cyst of anterior abdominal wall was excluded once indirect hemagglutition test was reported to be negative . fine needle aspiration cytology revealed caseating granuloma with central necrosis , lymphocytes and giant cells , consistent with tuberculosis . clinical presentation of anterior abdominal wall cold abscess ct scan of the abdomen showed two cystic collections in the anterior abdominal wall with peripheral enhancement only isolated cases of tuberculosis of skeletal muscle have been reported in the medical literature ) . the incidence of primary muscular tuberculosis was reported as 0.015% by petter . skeletal muscle involvement occurs in two forms : most commonly the tubercular abscess spreads into the muscle through extension from the neighboring structures like lymph nodes , bone , joint or tendon , etc . a tubercular abscess arising from a costochondral junction may track down , either lateral or medial to the linea semilunaris . if it extends lateral to the rectus , it spreads down between the internal oblique and transverse abdominis muscles , but if it extends medial to the linea semilunaris , it may spread into the rectus sheath and may extend downward behind the rectus muscle . in the second type this case is of interest because he seems to have a primary tubercular anterior abdominal muscular lesion without any history of contact or previous anti tubercular therapy . the possible explanation for the rarity of muscle involvement in tuberculosis may be high lactic acid content , lack of reticulo - endothelial tissue in muscle , lack of lymphatic tissue , the abundant blood supply and the highly differentiated state of muscle tissue . ultrasonography of the entity usually shows a parietal - wall mass ( predominantly cystic ) of mixed echogenicity , with irregular walls and a liquefied , necrotic center . sometimes , an evidence of posterior acoustic enhancement with focal areas of calcification within the lesion may also be demonstrated sonographically . computed scan of the abdomen usually shows a well - defined abscess in the abdominal wall . ultrasonography or ct - guided aspiration followed by cytological examination usually reveals tuberculous granulomas with areas of caseous necrosis . ziehl - neelsen ( z - n ) staining or culture of the aspirate may also help in confirming the diagnosis . surgical intervention in the form of either sonography or ct - guided aspiration or open drainage is usually reserved for patients in whom medical treatment fails . present case cautions the clinicians and radiologists about the possibility of tuberculosis in considering the differential diagnosis of any lesion even in any unlikely anatomical area , especially in those areas where tuberculosis is endemic or where the disease is rampant .

tuberculosis is considered as ubiquitous disease as it involves any organ , but primary involvement of abdominal muscles is very rare . in most cases , the muscle involvement is secondary and is caused by either hematogenous route or direct inoculation from a tuberculous abdominal lymph node or extension from underlying tubercular synovitis and osteomyelitis . autopsy studies have shown abdominal wall involvement in less than 1% of patients who died of tuberculosis . antitubercular therapy is main form of management . surgical intervention is always secondary in the form of either sonography or computerized tomography - guided aspiration or open drainage which is usually reserved for patients in whom medical treatment has failed . a case is hereby reported about primary tubercular anterior abdominal wall abscess without any evidence of pulmonary , skeletal or gastrointestinal tuberculosis in an apparently healthy individual with any past history of contact or previous antituberculosis therapy .

coronary artery spontaneous dissection is a rare and uncommon cause of sudden cardiac death and acute coronary syndrome . we report a case of a 31-year - old male admitted to our hospital with an acute coronary syndrome . a dissection of the left main coronary artery was diagnosed with a multidetector computed tomography ( ct ) scan and it was decided to perform an emergency coronary artery bypass grafting . a 31-year - old male , airline pilot , with no clinical history or cardiovascular risk factors , was admitted to the emergency room with 12 h history of retrosternal chest pain radiating to the jaw and upper extremities , during a trans - atlantic flight , which did not improve with analgesia . because of persistent pain , he came to our hospital . upon arrival to the emergency room , his vital signs were stable and he had a normal physical examination but complained of precordial pain . the cardiac enzymes were elevated and the ekg detected s - t changes consistent with mi ( fig . 1 ) . a multidetector ct coronary angiography was performed showing left main trunk dissection with 50% stenosis ( fig . figure 2:multidetector coronary angio - tomography reveals dissection at the ostium of the left main coronary artery . multidetector coronary angio - tomography reveals dissection at the ostium of the left main coronary artery . the patient underwent emergent coronary revascularization with saphenous vein grafts to the first obtuse marginal and the left anterior descending artery ( lad ) . control ct coronary angiography was performed 2 months later revealing patent aorto - coronary grafts ( fig . figure 3:ct coronary angiography reconstruction showing patent grafts to the lad and obtuse marginal arteries . in 1931 harold pretty described the first case of spontaneous dissection of a right coronary artery during the autopsy of a woman who presented with precordial chest pain . this condition is a rare cause of ischemic heart disease and affects mainly young healthy women . in 1987 , 85 cases were reported in the literature , currently more than 300 cases have been published [ 2 , 3 ] . before the coronariography era , these cases were reported during autopsies of patients having sudden cardiac deaths and its incidence may have been under - estimated . with the advent of these studies , the incidence has been reported to range between 0.07 and 1.1% . dissection of the lad artery is most common in women , whereas in men it is the right coronary artery [ 2 , 4 ] . the left main coronary artery involvement , like in our case , is rare , occurring in up to 12% of the cases , but is the most severe injury and presents as mi . hemodynamic and hormonal factors determine morphological changes in the arterial wall , and these may contribute to spontaneous dissection of the coronary arteries , with a peak incidence during the second week after birth [ 3 , 4 ] . to date , the pathophysiology is unclear [ 24 ] . in a subgroup of patients it is not possible to identify a specific condition causing spontaneous coronary dissection and is therefore classified as idiopathic . in patients with connective tissue disorders such as marfan s and ehlers danlos syndromes , predisposing to the dissection arises from medial degeneration of the coronary arteries . certain vasculitis , including systemic lupus erythematosus and polyarteritis nodosa , has been associated with the occurrence of coronary artery dissection . other factors that can cause vascular spasm and coronary dissection include intense exercise , sneezing and prolonged cocaine abuse [ 2 , 3 ] . it should show the presence of a double radiopaque lumen separated by a radiolucent intimal flap or a slow clearance of contrast from the false lumen . an intimal tear is present in only a minority of cases and a medial hematoma may not be recognized on coronary angiography as the medial hemorrhage may cause luminal narrowing or occlusion by pushing the inner media against the opposing wall . several cases in the literature have shown that ct coronary angiography provides additional information over invasive coronary angiography , with an accurate demonstration of the intimal flap and extent of the intramural hematoma . this study constitutes an emerging noninvasive alternative for diagnosis and also the follow - up of coronary artery spontaneous dissection . there are no specific guidelines for the management of spontaneous dissections of the coronary arteries [ 3 , 5 ] . coronary artery bypass surgery is indicated in patients with multivessel dissection , failed angioplasty and dissection of the trunk of the left main coronary artery [ 35 ] . although spontaneous dissection of a coronary artery is an uncommon condition , treatment should be based on the clinical status and imaging studies , including conservative and surgical means depending on the clinical presentation , location and characteristics of the dissection , we elected to proceed with surgery based on the ct findings .

spontaneous dissection of the coronary arteries is a rare disease with a wide range of clinical presentations ranging from angina to myocardial infarction ( mi ) ; its pathophysiology has not yet been fully established . in this paper , we present the case of a 31-year - old male with an acute coronary syndrome . the initial results of the electrocardiogram and cardiac enzymes were consistent with mi . however , a coronary angio - tomography revealed a dissection of the left main coronary artery and the patient underwent emergent surgery with coronary artery bypass grafting . the treatment of spontaneous dissection of the coronary arteries depends on the anatomical location and the patient 's clinical presentation . coronary revascularization is associated with good results .

a 65-year - old man with intermittent , colicky periumbilical pain which first occurred two months earlier was admitted to hospital . he had an eight - year history of congestive heart failure caused by mitral valvular regurgitation and atrial fibrillation . he was nonalcoholic , and there was no history of diarrhea , hematochezia , or melena . vital signs at admission were stable , and laboratory findings including white blood cell count , a liver function test and electrolytic balance were within the normal ranges . electrocardiography revealed atrial fibrillation , and chest radiography demonstrated cardiomegaly ( not shown ) . to exclude acute appendicitis , initial ultrasonography ( us ) was performed , and this demonstrated diffuse , segmental , concentric wall thickening of the terminal ileum just proximal to the ileocecal valve . nonspecific ileitis , crohn 's disease , intestinal tuberculosis or ischemic enteritis were suggested as possible causes of the bowel wall thickening , and in order to evaluate the terminal ileum , colonoscopic examination was performed . the ascending colon was found to be completely obstructed by a circumferential mass lesion , and the colonoscopic fiber could not be advanced further . subsequent ct scanning showed a markedly dilated small bowel and ascending colon , with concentric , hyperattenuating , focal wall thickening in the hepatic flexure of the ascending colon ( fig . in addition , the terminal ileum was dilated and showed diffuse , concentric wall thickening of its long segments with heterogeneous contrast enhancement . in the thickened wall , there appeared to be several possible diagnoses , including ischemic enterocolitis caused by thromboembolism of mesenteric vessels arising from atrial fibrillation , inflammatory bowel disease involving the ascending colon and terminal ileum , and ischemic or infectious enteritis associated with colon cancer . stool culture yielded lactose - fermenting gram - negative bacillus , urine culture yielded citrobacter freundii , and gram staining of urine revealed the presence of gram - negative rods ; no organisms were isolated from blood cultures . in addition to conservative management of congestive heart failure , the patient underwent antibiotic therapy with amoxacillin , tobramycin or aztreonam for two weeks and ciprofloxacin for several days . symptoms such as abdominal pain ameliorated , the level of bowel sound decreased , and laboratory findings continued to be within normal ranges . vital signs were stable , except for intermittent fever of up to 38.2 until 30 days after admission . at this time , , the hepatic flexure of the ascending colon was found to be completely obstructed by a hard concentric mass , and about 15 cm of the terminal ileum , just proximal to the ileocecal valve , was markedly dilated and diffusely thickened . a cut section of the thickened terminal ileum revealed marked submucosal edema to a depth of approximately 10 mm , though there was no evidence of mucosal lesion . microscopic examination also showed that the mucosal folds of thickened ileal loop were blunted by submucosal edema and there was extensive inflammatory reaction , with infiltrations of neutrophils . in some areas of the thickened bowel wall neutrophil infiltration extended into the subjacent muscular layer and even to the serosa ( fig . 1d ) . the inflammatory reactions provided no evidence of granuloma formation , and the histologic findings were consistent with phlegmonous enteritis phlegmonous enterocolitis is a rare inflammatory bowel disease with a high mortality rate of at least 60% . the association between this disease entity and a variety of liver diseases has been well documented ( 1 - 3 ) , though cases associated with lobar pneumonia , pharyngitis , infected peritoneojugular venous shunt and septicemia have been reported , and it has also occurred in healthy individuals ( 1 , 4 - 6 ) . several kinds of pathognomic bacteria have been demonstrated in culture or histologically ( 1 - 6 ) : gram staining of intestinal lesions has revealed a variety of entities such as streptococci , gram - positive cocci , and gram - negative or -positive rods . the mortality rate associated with this disease continues to be high due to delayed diagnosis ( 1 - 5 ) . thus , most cases reported in the literature were discovered at autopsy or after the examination of surgical specimens following surgery ( 1 - 6 ) . some reports have suggested that earlier diagnosis and surgical resection of diseased bowel , together with the use of broad- spectrum antibiotics , has led to a good outcome ( 1 , 6 ) . the pathogenesis of this disease entity is not clear : ito et al . first , the direct toxic effect of alcohol may affect the gastrointestinal tract , and prolonged alcohol ingestion leading to changes in the intestinal mucosa , with increased intestinal permeability ; subsequent penetration of the lamina propria by antigens ( organisms ) from the intestinal lumen results in a local antibody response . second , in chronic alcoholism , the systemic and mucosal immune mechanisms are impaired , and this may exacerbate bacterial infection . it is not known why the organisms involved in phlegmonous enteritis are confined to the submucosa . bowel wall edema associated with portal hypertension has been described in liver cirrhosis , and the loose connective tissue in the submucosa can be excellent soil for the rapid and diffuse spread of the organisms involved in an episode of bacteremia ( 5 ) . first , the patient was not alcoholic and had no evidence of liver disease or septicemia . second , his clinical course was silent for a long period , being discovered only at surgery ; previously reported cases , on the other hand , manifested an acute and serious clinical course , one which led even to sudden death . in our case , the early use of broad - spectrum antibiotics might have helped his condition to persist . the literature in english includes only one case report dealing with the radiologic findings pertaining to this disease . mooney et al . ( 6 ) reported the ct findings in one case , in which only nonspecific small bowel wall thickening with a small amount of ascites was noted . in our case , the terminal ileum showed marked wall thickening , and thumbprinting and blunted mucosal folds were revealed by ultrasonography . the hypoattenuating focal areas in this thickened wall , shown at microscopic examination to be submucosal abscesses , are , in our case , thought to provide a clue to the diagnosis of phelgmonous enteritis .

phlegmonous enteritis is a rare infective inflammatory disease of the intestine , predominantly involving the submucosal layer . it is difficult to diagnose and often fatal . its association with alcoholism and various liver diseases , although rarely reported , is well documented . we report a case of phlegmonous enteritis in a male patient with congestive heart failure and colon cancer , and describe the ultrasonographic and ct findings .

the supratentorial region is the most frequent site for meningiomas ; however , they are sometimes observed in other locations , such as the spine12 ) . however , a few of these tumors are thought to arise from the extradural component12 ) . here , we have reported our experience involving a case of extra - intradural calcified meningioma . a 49-year - old woman presented with low back and left leg pain of 5 years duration and a history of medication use for treatment of hypertension and diabetes mellitus for over 20 years . neurological examination revealed hypesthesia of the left leg and no motor deficit , with normal deep tendon reflexes . magnetic resonance imaging ( mri ) showed an extradural mass , 1.8 cm in size , with rim enhancement in the spinal canal at the t12 level ( fig . the mass extended to the midline and the left t12-l1 foramen . computed tomography ( ct ) revealed dense calcification within the mass ( fig . a linear durotomy was performed at the midline , and the intradural portion was removed using an ultrasonic surgical aspirator and a kerrison punch ( fig . the adjacent dura was severely ossified , and the left t12 nerve root sleeve was thickened . the extradural portion was not separable from the adjacent dura and the left t12 root , and it was removed by dural excision using a # 15 blade and edson forceps ( fig . the dural margin was coagulated using a bipolar coagulator , and the dural defect was repaired with artificial dura ( fig . spinal meningiomas account for 25 - 45% of all spinal tumors4 ) and 1.2 - 12.5% of all meningiomas10,12 ) . most spinal meningiomas are intradural , with only 3.3 - 21.4% of them being extradural12 ) . a previous study reported that 85% of spinal meningiomas are intradural , 7% have an extradural extension , and 8% are entirely extradural10,11 ) . spinal meningiomas generally arise from arachnoid cap cells or dural fibroblasts , and from the arachnoid in the case of cranial nerves and the choroid plexus1 ) . some investigators have suggested the possibility of islands of arachnoid tissue migrating into the extradural spaces or aberrant arachnoid islets in the extradural spaces , as seen with other extracranial meningiomas such as those of the nose or skin3,9 ) . the differential diagnosis of meningioma includes schwannoma , metastatic tumors , lymphoma , and tuberculoma2 ) . misdiagnosis can lead to an incorrect operative plan . in our patient , the mass was more likely to be a schwannoma at the point of passing the path of the left t12 nerve , but the ct finding of ossification supported the diagnosis of meningioma . intraoperative histologic examination is crucial for an optimal surgical plan and so are the preoperative imaging studies in tumors mimicking other pathology5 ) . the four common microscopic patterns of meningiomas are psammomatous , syncytial , transitional , and fibrous12 ) . of these , the most common type is the psammomatous meningioma in which calcification is comparatively more likely to be present12 ) . in our patient , the long term prognosis of extradural spinal meningiomas is not clear , but recurrence rates might depend on whether total tumor excision was performed1 ) . if a tumor is excised totally , recurrence - free survival rates are 93% , 80% , and 68% at 5 , 10 , and 15 years , respectively11,12 ) . in cases of subtotal resection , recurrence - free - survival rates are 63% , 45% , and 9% at 5 , 10 , and 15 years , respectively1 ) . other factors associated with poor outcomes are the presence of an extradural component , young age ( < 50 years ) , multiple lesions , calcification , ossification , and anterior location of the lesion2,7,8 ) . the presence of an extradural component , calcification , and ossification make total resection of spinal meningiomas more difficult . in our patient , the adjacent dura was resected , and the left t12 nerve root was sacrificed for gross total resection . although there is no consensus on the degree of total excision , nakamura et al . reported that recurrence rates are significantly different according to the simpson grade8 ) . in our patient , although the left t12 nerve root was sacrificed , the patient experienced improvement in her leg pain . there was no aggravation of pain or development of any other complication observed at the 6-month follow up . when surgical treatment is not feasible in case of recurrent , residual , or multiple lesions , radiosurgery can be considered as an option . reported 11 cases of spinal meningiomas ( who grade i ) treated with cyberknife radiosurgery ( ckrs ) . the median prescription dose was 14gy ( 95% confidence interval 13 - 15gy ) and isodose was 70%6 ) . after a median follow - up of 18 months ( range , 6 - 50 months ) , no local tumor progression was observed . in 1 of the 3 patients with pain before ckrs , the initial symptom was improved . in this report , we have presented our experience with a case of meningioma and a literature review of similar cases . extradural meningiomas are uncommon , and it is important to clearly distinguish them from other types of tumors as misdiagnosis can lead to an incorrect operative plan . the long term prognosis of extradural meningiomas is not clear but total excision is thought to be essential .

extradural spinal meningiomas are uncommon , and their pathophysiology is not entirely understood . here , we present the case of a 49-year - old woman with low back and left leg pain of 5 years duration . magnetic resonance imaging revealed a mass , 1.8-cm in size , with rim enhancement in the spinal canal at the t12 level and extending into the left t12-l1 foramen . in the surgical field , the mass presented with the characteristics of an extra - intradural spinal meningioma . the patient underwent a t12 total laminectomy . a linear durotomy was performed at the midline , and the intradural portion was removed . the extradural portion was not separable from the adjacent dura and the left t12 root , and it was removed by dural excision . pathological examination confirmed the diagnosis of psammomatous meningioma . we also conducted a literature review of similar cases . based on our experience with this case , we believe that it is important to clearly distinguish extradural meningiomas from other types of tumors as misdiagnosis can change the operative plan . the long term prognosis of extradural meningiomas is not clear but total excision is thought to be essential .

the latest study on the therapeutic value of statins in pneumonia adds further support to the association between statin use and improved patient outcomes . the role of statins in the management of sepsis has been increasingly studied but remains contentious . animal studies of statin use in sepsis have shown a significant benefit with respect to mortality but a plethora of observational studies have come to conflicting conclusions . many mechanisms have been postulated to explain why statins may influence sepsis , and it is possible some of these may be of particular benefit in pneumonia [ 6 - 8 ] . statins are taken by millions of people around the world and pneumonia represents one of the most common infections seen in clinical practice . this provides an opportunity to review a large number of cases of pneumonia that involve coincidental statin use . as would be expected for a drug targeting vascular disease and hypercholesterolaemia , statin users have different comorbidities and risk profiles to those patients that do not take statins . therefore , observational and retrospective studies are potentially confounded by both an unhealthy user effect ( a cohort often older with more coexisting cardiovascular disease ) and the ' healthy user ' effect . the recent study by nielsen and colleagues is a well conducted , large , observational study . the authors provide extensive detail on coexisting diseases in each of the cohorts in an effort to reveal hidden confounders that plague database interrogation . this paper adds another observational study to a large bank that already exists addressing the outcome from pneumonia in statin users [ 10 - 13 ] . it serves to remind us that while again suggesting a possible therapeutic role for these agents in pneumonia , it does not prove a clear cause and effect relationship . firstly , it defined ' current use ' as one prescription filled in the past 125 days . while the results suggest the improvement in pneumonia outcome was associated with current and not former statin users , a huge variation in timing of statin administration in relation to onset of infection is possible within these definitions . in addition to the timing of administration , the impact of one statin over another or drug dosage remains unclear . finally , large registry databases pose other challenges . as was highlighted recently , both the consistent use of a diagnostic category over time and the motivations for recording specific diagnosis can further confound the validity of results from large national or administrative databases . it is fortuitous that prevalence has provided the opportunity to observe statin use in pneumonia patients . these observations have provided the impetus to investigate statins as a potential adjuvant therapy for severe lung injury . two randomised controlled trials are currently underway : statins for acutely injured lungs from sepsis ( sails ) and hydroxymethylglutaryl - coa reductase inhibition with simvastatin in acute lung injury to reduce pulmonary dysfunction ( harp 2 ) . in contrast , harp-2 will compare 80 mg simvastatin to placebo . whilst these may be equipotent doses in the management of hypercholesterolaemia , it remains unknown what effects these agents at these doses will have in acute lung injury . these two drugs have pharmacokinetic differences , including lipid solubility , volume of distribution , metabolism and potency . without an explanation of how statins may benefit patients with lung injury , predicting compared to the general population , the pharmacokinetics of atorvastatin differs in critical illness , and perhaps with sepsis in particular . exploring commonly used statin pharmacology in critically ill patients with pneumonia would better define dose - response relationships on varied biological endpoints and this may provide some valuable insights into mechanisms that could explain an association with improved patient outcomes . in addition to statin use possibly being associated with a reduction in the severity of infection , a second intriguing possibility is that statins may reduce the risk of infection . a post hoc analysis of the jupiter trial suggested a modest reduction in the incidence of pneumonia in patients randomised to receive 20 mg of rosuvastatin . a nested case control study has also suggested a reduced incidence of pneumonia in statin users . the recent study by nielsen and colleagues again suggests a decrease in the risk of hospitalization for pneumonia with statin use , but only after adjustment for potential confounding variables . pneumonia represents an illness with known risk factors and the administration of statins as preventative therapy could be prospectively explored in this patient population in an effort to avoid confusion from uncontrolled or unknown factors . rather than question what can be learned from observational data while the results of randomised trials are awaited , perhaps we can use all these research methods to better inform our clinical practice . whilst observational studies to date are far from conclusive , it is hard to see how randomised controlled trials will resolve a complex debate that is still in its infancy . it is more likely that a multipronged approach with retrospective large databases , smaller biological studies and randomised trials will help complete the biological puzzle of statin use and its role in patients with pneumonia . harp 2 : hydroxymethylglutaryl - coa reductase inhibition with simvastatin in acute lung injury to reduce pulmonary dysfunction ; sails : statins for acutely injured lungs from sepsis .

several studies have investigated the use of statins as an adjunct in the treatment of pneumonia , some with conflicting conclusions . the most recent of these large observational studies again suggests statin use is associated with an improved outcome for patients with pneumonia . how best to incorporate these findings into current practice is of great interest . hidden confounders plague database interrogation and so cast doubt on the real or causal nature of observed associations . data from large , observational studies must be complemented by smaller biological studies and randomised controlled trials in an effort to complete missing pieces in the biological puzzle of the use of statins in patients with pneumonia .

choledochal cysts are congenitally malformed thin walled , dilated , intrahepatic or extrahepatic bile ducts . these cysts offer a major challenge for diagnosis and treatment during pregnancy because of the altered physiology associated with it . there has to be fine balance between the benefits as well as the risks associated with the treatment . it is secondary to raised intracystic pressure because of distal stricture or stones [ 4 , 5 ] . spontaneous rupture of the choledochal cyst during immediate post partum has rarely been reported [ 7 , 8 ] . here we describe a young female who presented to us with choledochal cyst with cholangitis during pregnancy . but unfortunately she presented in the immediate post partum with peritonitis secondary to spontaneous rupture of the cyst . the rarity of this presentation in post partum poses a diagnostic dilemma in the minds of treating clinician . this complication should be kept in mind while treating a known patient of choledochal cyst who presents to the emergency with peritonitis . a 27-year - old primigravida presented to surgical emergency with obstructive jaundice and grade ii cholangitis at 6 months of pregnancy . she was evaluated and was found to have a choledochal cyst on ultrasound and this was subsequently confirmed as type iv ( fig . 1 ) on magnetic resonance cholangiopancreatography ( mrcp ) . for these complaints , she underwent ercp and a biliary stent was placed so as to tide over the situation . an endobiliary stent could be placed and she was being managed conservatively but had pain abdomen , fever and vomiting 1 week after stenting . tachycardia ( pr : 120/min ) was present and there was generalized guarding and rigidity on abdominal examination . a contrast - enhanced ct scan was done that revealed the rupture of the suprapancreatic part of the choledochal cyst with free fluid in the abdominal cavity ( fig . 2 ) . after optimization , this patient underwent cyst excision with roux - en - y hepaticojejunostomy . histopathology of this excised cyst confirmed the choledochal cyst rupture without any evidence of dysplasia ( fig . the patient did well in the postoperative period and was discharged on the 10th postoperative day . with recent advances in medical science , choledochal cyst is diagnosed 25% of the times during adulthood and rarely during pregnancy [ 9 , 10 ] . it is associated with multiple complications during pregnancy like cholangitis , preterm labor , abortion and even rupture . the cumulative risk of spontaneous choledochal cyst rupture varies between 1.8% and 2.8% [ 11 - 13 ] . rupture is more common during childhood and rare during post partum period of the pregnancy . weakness of the dilated cyst wall with raised intracystic pressure because of stones / strictures , trauma due to multiple interventions , regurgitation of the amylase rich fluid in the bile duct and association of the anomalous pancreatobiliary junction are the commonly reported in literature [ 14 - 18 ] . multiple ercp and stent placement secondary to cholangitis causing the cbd weakness is the most probable cause of rupture in the present case . ercp is associated with increased risk of radiation exposure and subsequent malformations in the fetus . many studies have proved the efficacy of ercp during pregnancy particularly when the radiation dose to the fetus is calculated . ercp when indicated should be done with utmost care and in controlled conditions to prevent fetal damage . . however , pregnancy along with increased pressure during childbirth puts these patients at risk for cyst rupture and fetal loss . we tided over the complications of the cyst with minimally invasive techniques but repeated interventions for the same might have triggered the rupture . there is no consensus on the management of the ruptured choledochal cyst due to the rarity of the condition . friability of the cyst wall , hemodynamic instability , inflammation and edematous bowel mandate the drainage of the cbd either with a t - tube or percutaneous transhepatic drain [ 2 , 3 ] . alternatively , single stage operation of cyst excision with bilioenteric drainage can be performed provided the intraoperative conditions are favorable . this patient had normal bowel with minimal inflammation of the bile duct so single stage operation was contemplated . cyst rupture should be kept as a differential diagnosis whenever a known patient harboring choledochal cyst presents with peritonitis . pregnancy with choledochal cyst rupture puts the maternal as well as fetal life at risk . cyst rupture should be kept as a differential diagnosis whenever a known patient harboring choledochal cyst presents with peritonitis . pregnancy with choledochal cyst rupture puts the maternal as well as fetal life at risk . ag , kc and lk were involved in writing , editing and approval of the manuscript .

with the advent of newer radiological investigations , choledochal cysts are being diagnosed more often in present era . these cysts are commonly diagnosed in early childhood and infancy , although some go undetected to be diagnosed in adulthood . these malformations are associated with multiple complications like cholangitis , jaundice , pancreatitis , rupture or even malignancy . here we describe a post partum female , who was diagnosed to have choledochal cyst during sixth month of pregnancy . she presented with obstructive jaundice in cholangitis and was subjected to endoscopic retrograde cholangiopancreatography ( ercp ) with stenting . this female delivered normally at term . she again had jaundice with cholangitis during early post partum period . endoscopic stenting could be performed . she had features of peritonitis after 1 week of stenting . investigations were performed and a diagnosis of spontaneous rupture of choledochal cyst was made . she underwent laparotomy and lavage with complete excision of the choledochal cyst and roux - en - y hepaticojejunostomy .

anatomy is the background of medical teaching and it remains in this cornerstone position , despite the decrease in importance given to this discipline in recent decades . from the start of anatomical education , back during the first renaissance century , carried out in the first universities from europe , the need of anatomical knowledge has always been emphasized . even now , when new surgical and imaging techniques develop from year to year , the need for a better knowledge of basic anatomy and of surgical anatomy including anatomical variations from the average but clinically normal is obvious . the best way to study anatomy is still on the corpses or on casts reproducing the body . more recent online systems are for the moment somewhat inferior to class teaching of anatomy . comparisons of different imaging methods for organ evaluation do not always replace the anatomical assessment . hence the need to offer the students very accurate replicas of human bodies , enabling them the access to human structures , in the absence of true corpses . we present in this paper one of the most impressive didactic pieces available in the department of anatomy of the iuliu hatieganu university of medicine and pharmacy cluj - napoca , romania . it was realized by the famous sculptor constantin brancusi at the request of professor dimitrie gerota . he had and still enjoys a worldwide reputation , being one of the most expensive artists of the world . some of his sculptures were sold at different auctions for many millions of dollars , always over the estimated price . his start was modest , in a rural environment , and started his training in bucharest [ 810 ] . around 19031904 he traveled to paris via munich and continued there a brilliant career , after a short stage in the workshop of auguste rodin . he started to simplify the physical forms , including the bodies , arriving to an essence of symbolism of great elegance and expressivity . his destiny and work attracted many disciples and he is one of the most influential sculptors of the first part of the 20 century . his artistic style was so original and seminal for the artistic world of his time , that he stimulated psychoanalytical interpretations . also , one of his first works , more naturalistic , was interpreted as a pathographic representation of a neurological hereditary disease . when he left bucharest for paris , he left behind some sculptures , most of them preserved in the museum of arts from bucharest and craiova . in bucharest , he interfered with the professor dimitrie gerota and the meeting between the young artist in quest of his way and a famous surgeon and educator left traces . only 10 years older than brancusi , dr . gerota had an established reputation in bucharest when the young sculptor started his career . they met when gerota was teaching anatomy to the fine - arts school in bucharest where brancusi was a student , and maybe the fact that they came from the same province of romania , oltenia , enhanced their collaboration and friendship . during that time they decided to work on a teaching material necessary to anatomy students ( see below ) . dimitrie gerota was not only anatomist , but also surgeon , as many professors of surgery of that time , when morphological sciences preserved their supremacy . he taught at the faculty of medicine of bucharest , but used free time intervals for continuous education abroad , mainly in france and germany . he had many scientific contributions and even a known medical dictionary preserves his name , mainly in respect to the kidney fascia called also gerota fascia . dimitire gerota was very much interested in radiology , as the first morphological investigation of that time and used one of the first roentgen devices of this country . his political anti - dynastic conceptions cost him a short term emprisonment during the carlist dictature . however , during his life he became president of the romanian society of surgery , urology and gynecology , vice - president of the chira published unwonted document s like his birth certificate , his graduation diploma , drawings by him destinad to anatomical lectures , etc . it was usual at that time to observe the anatomy of plastified bodies or on sculptures mimicking the natural body , beside the dissection hours ( as one can see in many anatomical museums in europe ) . skinless bodies ( or ecorch from french ) were real - life statues representing skinless bodies . it is said that the model for the brancusi work was the greek statue of antinous . as described by the famous art historian barbu brezianu , professor dimitrie gerota asked his disciple constantin brancusi at the school of fine - arts in bucharest to help him create a sculpture realistically displaying a nude male , allowing anatomy students to learn the segments of the body and the muscles . it means that gerota had notices brancusi s talent and selected him for this task . brancusi agreed with gerota ( both were interested by drawings and modeling , also in realistic display of human structures ) . together , they created a very impressive realistic model of a skinless male , where the muscles are very obviously and correctly displayed . at that time body building the model was displayed in 1903 at the atheneum building in downtown bucharest and was admired by visitors . further , this ecorch was multiplied by the order of the ministry of public education to serve in main universities of the country . thus , one copy arrived to cluj after the world war 1 , when the medical faculty in romanian was founded . the copy in cluj has the following dimensions : 172/52/32 cm and the inscription on the support at the bottom is : worked after natural model by dr . this statue was given personally by professor gerota to the lecturer of anatomy constantin velluda , as a token of friendship , in the 30s , few years before gerota s death [ 16 . few other copies exist in bucharest , iasi , craiova and small differences exist between them . while brancusi has continued his artistic career in france and obtained celebrity in usa , romanian medical and fine art students continued to use his beginner s work to increase the anatomical knowledge . the anatomy department of cluj - napoca preserves a masterpiece of the famous sculptor constantin brancusi , created with professor dimitrie gerota , in order to serve the anatomical education .

background and aimthe study of anatomy remains the backbone of medical education in the first years . there is a constant need for educational materials that enable the assimilation of knowledge by students . the casts after human bodies have not lost the value , even in the era of virtual education . we present in this paper a museal item destined to improve the anatomy teaching.methodsgiven the existence in the department of anatomy from cluj napoca of an item of exceptional artistic and scientific value , we intensively searched pubmed and scopus , as well as by manual search of printed only documents , for all papers related to the muscle man by brancusi created for educational purposes of anatomy students.resultsthis paper presents summary data from the biography of the creators of this item , the world famous sculptor constantin brancusi and the professor of anatomy and surgery from bucharest dimitrie gerota . we also describe this item and the conditions which generated itconclusionteaching anatomy relies on the quality of the didactic support . the muscle man by brancusi is a very realistic reproduction of a man , very useful for anatomical training and teaching .

patients with foreign bodies in their gastrointesti - nal tract are a common occurrence in emergency medicine ( 1 , 2 ) . the majority of these foreign bodies transit through the gastrointestinal system without any complication , and have more than a 90% chance of passing into the small intestine if they get to the stomach ( 1 - 3 ) . it is very likely that things the size of coins would pass through the small intestine without any complication ; however , objects larger than 2 cm in diameter are unlikely to pass through the pylorus , and objects witch are more than 6 cm in length are often caught in the pylorus easily . in the end , only 1% of ingested foreign objects manage to pierce the gastrointestinal tract ( 2 ) . we present a case of a 21-year - old female who , in the month prior to admission to our institution , had been treated multiple times in emergency departments at various hospitals referring epigastric abdominal pain and constipation . the treatment she received was based on the resolution of constipation , antacids and dietary recommendations . the patient was admitted to our emergency department after 1-month of upper quad - rant abdominal pain radiating to the left upper quad - rant , additionally she cited a weight loss of 5 kg in the last month . her symptoms had become more aggravated two days prior to admission with unquantified fever , chills and night sweats . a review of the family history revealed no significant disease . a physical examination revealed vital sings with a heart rate of 102 beats per minute , respiratory rate 16 breaths per minute , temperature of 37c , blood pressure of 110/56mmhg , the abdomen was distended , the percussion was tympanic , the bowel sounds were de - creased . additionally we found a painful , not well limited , mass adhered to deep planes in the abdominal palpation without peritoneal irritation or other alterations . a work - up started with blood chemistry , cbc , and we requested a ct of the abdomen due to clinical examination findings . the preclinical showed no elevation of acute phase reactants and no electrolyte disturbance , the ct scan evidenced an irregular , small collection in the gastric antrum and behind the left rectus muscle of approximately 30x11x16 mm , which was associated with increased density of the surrounding fat . inside this area , the end of a tubular , hypodense structure ( compatible with foreign body ) was visible coming out from the gastric lumen through the wall , which was perforated ( figure 1 and 2 ) . the findings on ct were dis - cussed with the patient who was questioned again for further information to determine the origin of the foreign body . she ad - mitted having a major depressive episode 6 months ago and attempted of suicide . she had ingested a plastic stick from a candy ( chocmelo ) ( figure 3 ) . she was referred to general surgery service who decided to take the patient immediately to the surgery room and performed an exploratory laparotomy . during the procedure , they found a perforation in the anterior wall of the stomach between the gastric body and the antrum , it was associated with a great inflammatory plas - tron generating a gastrocolic ligament edema . they therefore decided to perform a subtotal gastrectomy removing 50% of the stomach and then roux - y reconstruction . perforation of the gastrointestinal tract by purposeful ingestion of foreign bodies in adults is usually secondary to accidental ingestion which is often caused by foreign bodies included in meals ( 1 , 3 ) . predisposing factors include prostheses , patients with a history of psychosis or psychiatric disease , alcohol abuse and being in prison . objects that are frequently ingested are the teeth , toothbrushes and foreign bodies from the diet such as fish and chicken bones . the most commonly affected sites are the distal ileum , sigmoid colon or rec - tum ( 1 , 2 ) . patients with perforation of the stomach , duodenum , or large intestine , usually ingest larger objects ( 2 ) . the clinical presentation of gastric perforation by a foreign body tends to be hidden , with unspecific gastrointestinal symptoms such as chronic abdominal distension , decreased appetite , and epigastric mass sensation , usually with normal laboratory results ( 1 , 3 ) . the patient in this case had multiple visits to the doctor after the foreign body ingestion , and was treated for peptic ulcer disease and constipation . important to highlight is , the patient did not mention any foreign body ingestion until she was confronted with the scan results . enhanced ct of the superior abdomen in venous phase , which demonstrates in sagittal ( a ) and coronal planes ( b ) the tubular air filled foreign body ( white arrow ) penetrating the gastric wall ( arrow heads ) , associated with peripheral fat stranding ( curved arrow ) . enhanced ct of the superior abdomen in venous phase , which demonstrates in sagittal ( a ) and axial planes ( b ) the tip of the tubular air filled foreign body ( white arrow ) in the peritoneal fat surrounded by a fluid collection with an enhancing wall ( arrow heads ) type of candy whose stick was reportedly swallowed finally , we made a search in the literature and we surprisingly found that when there is gastric or duodenal perforation by a foreign object the clinical findings are similar to our case , for instance in the vast majority there was a slow course and latency period before presenting with the acute phase . a common finding besides gastric perforation was an inflammatory plastron or abscess , and was always treated with laparotomy and in some limited cases with minimal laparotomy plus abscess drainage and suture of the primary defect in the stomach and in two cases a patch of omentum was used ( 4 , 5 ) . in the case of our patient a subtotal gastrectomy of 50% with roux - y reconstruction was performed due to severe transmural inflammation , accompanied by multiple abscesses and a severe submucosal edema . the patient had a favorable outcome without any complications and has gained some of the lost weight . after treatment , gastric perforation by a foreign object may have a slow course rather than presenting acute abdomen . gastric perforation by a foreign object the picture may have a slow course rather than presenting acute abdomen . the realization of a proper physical examination in the emergency department is key to an accurate diagnosis . all authors passed four criteria for authorship contribution based on recommendations of the international committee of medical journal editors .

this is a case report of foreign body ingestion in a suicide attempt resulting in gastric perforation and phlegmon formation during a subsequent 6 month period that eventually required surgical intervention . the patient had a prolonged course because she did not report a history of foreign body ingestion and the initial evaluating physicians had no suspicion about possible foreign body ingestion and may have missed important findings on physical examination . gastric perforation by a foreign object may have a slow course rather than presenting acute abdomen . the realization of a proper physical examination in the emergency department is key to an accurate diagnosis .

ectopic opening of nasolacrimal duct in the normal face is so rare that only one such case has been reported in the literature . congenital disruptions and anomalies of this duct system occur in oblique facial clefts as a result of clefting in this zone . other congenital anomalies are mostly obstructive in nature and may vary from partial absence of the canalicular system to alacrima . a 22-year - old male presented with pinkish looking pit lateral to the left ala of nose with history of repeated purulent discharge with intermittent conjunctivitis since birth [ figure 1 ] . a pinkish opening about 5 mm in diameter was seen inferolateral to the base of left ala , and on palpation there was no bony indentation or notching on the nasolacrimal and infraorbital region . however on moderate pressure over the lateral wall of nose there was increased discharge from the opening . injecting saline into this opening showed exit of saline from the punctum of inferior canaliculus confirming the nasolacrimal duct [ figure 2 ] . ct scan or mri was not done because of resource constrains and the diagnosis was confirmed by injecting saline into the duct . the opening of the nasolacrimal duct located inferolateral to the left ala exit of saline from the lower punctum of left eye after injecting normal saline into the ectopic opening the patient was operated under general anesthesia . the alar facial groove was marked with methylene blue and infiltrated with 1:20000 adrenaline solution . a circular incision was placed around the swelling which was extended superiorly in the alar - facial groove taking care not to injure the nasolacrimal duct with the feeding tube in place as a guide the lower third of the duct was dissected circumferentially [ figure 3 ] . the lower part of the incision was deepened till the mucosa lining the lateral wall of nose so as to reach the inferior meatus . the distal opening of the nasolacrimal duct was cut 2 mm on the medial side and sutured with the opening in the nasal mucosa with 4.0 catgut . patient was on intravenous ceftriaxone 1 g iv followed by oral cefixime for five days , gentamicin eye drop for two weeks . the cannulated aberrant nasolacrimal duct dissected circumferentially in the lower third , rerouted to the inferior meatus through an opening in the lateral wall of nose the aberrant nasolacrimal duct rerouted to inferior meatus with a stent insitu and closure of the lateral wall the alar facial groove was marked with methylene blue and infiltrated with 1:20000 adrenaline solution . a circular incision was placed around the swelling which was extended superiorly in the alar - facial groove taking care not to injure the nasolacrimal duct with the feeding tube in place as a guide the lower third of the duct was dissected circumferentially [ figure 3 ] . the lower part of the incision was deepened till the mucosa lining the lateral wall of nose so as to reach the inferior meatus . the distal opening of the nasolacrimal duct was cut 2 mm on the medial side and sutured with the opening in the nasal mucosa with 4.0 catgut . patient was on intravenous ceftriaxone 1 g iv followed by oral cefixime for five days , gentamicin eye drop for two weeks . the cannulated aberrant nasolacrimal duct dissected circumferentially in the lower third , rerouted to the inferior meatus through an opening in the lateral wall of nose the aberrant nasolacrimal duct rerouted to inferior meatus with a stent insitu and closure of the lateral wall aberration of nasolacrimal duct in oblique facial clefts are not uncommon . in these situations the embryological zone is involved and the anomaly is obviously explained . however in non - cleft patients with a normally developed face it is very difficult to explain the anomalous opening of the lower end . the only similar anomaly reported so far has been in an infant , where the nasolacrimal duct had opening into external skin of normal face . embryologically there is a zone of mesenchymal condensation in the naso - optic groove , due to the overriding of nasolacrimal process over the medially growing maxillary process , which is the analog of future nasolacrimal duct . there are conflicting views as to how this chord of mesenchyme canalizes to connect the conjunctival system with nose . either the epithelization progresses cranially starting from the caudal end or it occurs craniocaudally or due to coalescences of multiple sacs forming within the rod . from this case it appears as if the canalization occurs craniocaudally since the last part of canalization was not complete . recent embryological studies suggest that this anomaly is due to failure of complete separation and migration of the epithelial rod most distal from the surface ectoderm to the inferior meatus of the nasal cavity . this distal remnant of the nasolacrimal duct thus remains juxtaposed to the surface ectoderm of the face on the lateral aspect of the alar nares . the anomalies are obstructive in nature either due to persistence of hasner 's valve or absence of the apparatus proximal to the lacrimal sac . a study on brachycephalic cats revealed that the shape of skull had a bearing on the course of the nasolacrimal duct which had an angulated course ventral to the sac compared to a smooth curve in its mesocephalic or dolichocephalic counterparts . though obstructive symptoms are common in children a more severe anomaly is alacrima as in riley - day syndrome . this is the only second case with aberrant opening of lower end of nasolacrimal duct into a normal face being reported . this extremely rare condition should be kept in mind when one notices a congenital discharging sinus near the ala with intermittent conjunctivitis . the treatment requires dissection of the lower third of the duct and rerouting it to the inferior meatus .

congenital anomaly of nasolacrimal duct is mostly obstructive in nature or is seen in oblique facial clefts . nasolacrimal duct drain into the inferior meatus in the nose in a normally developed face . we report a rare case of aberrant opening of distal end of nasolacrimal duct into the external skin lateral to the ala of nose in an adult patient . the only case reported so far was in an infant . the patient is a 22 year young male presenting with an opening lateral to the left ala of nose with recurrent discharge and conjunctivitis of left eye . the embryology is also discussed .

joint position sense ( jps ) is defined as the ability to maintain a static limb position . this is a somatosensory function that provides inputs from muscles and joints without the assistance of vision1 . jps is an individual s ability to reproduce a previous determined position and a parameter of proprioception . in children with difficulties related to proprioception , this is associated with the integration of proprioception with other sensory information for the planning and control of movement2 . this ability is of great important for children in , for example , play . proper orientation of the upper extremity is necessary for accurate and precise hand positioning . high proprioceptive acuity in the upper extremity is necessary for accurate positioning of the hand in space . poor jps has been reported as a factor related to motor coordination in children with development coordination disorder4 . jps is measured accurately based on joint angle replication in an open or closed chain environment . many types of assessment methods for jps are used , such as simple goniometers or isokinetic dynamometers , and electromagnetic tracking devices5 . to investigate elbow jps , we used a three - dimensional ( 3-d ) motion analyzer . in measuring jps , the most commonly used variable is the absolute difference in degrees between the achieved and target positions . although the authors of several studies have claimed that they used standardized reproducible methods for measuring jps of the elbow , to our knowledge , no study has been published on elbow jps in children . the aim of this study was to investigate the difference in target angle error of elbow jps in children ( target angles , 30 and 110 ) . nineteen healthy children ( 15 boys and 4 girls ) with a mean age of 9.7 0.36 years participated in this study . prior to the study , the children and their parents were informed of the purpose of this study and the general procedures to be undertaken . the study was approved by the human health science studies committee of kaya university . to measure elbow jps , the children were blindfolded to block visual information and instructed to move at a consistent speed according to a metronome . calibration was performed with the subjects sitting on a chair , with both hands in a neutral position . we measured 30 and 110 elbow flexion by using a goniometer and showed them the angles by moving their elbows passively . after a 5-mintue break , the children were directed to repeat 30 and 110 elbow flexion in time with a metronome set at 1-second intervals . the children were then directed to repeat this motion 10 times in time with a metronome set at 1-second intervals . the right elbow was measured first , and the left was measured immediately after . each subject was instructed to relax during the testing and was told to press the response button when they thought that the limb had reached the target position previously demonstrated . their movements were recorded by using the compact measuring system ( cms ) 10 for 3-d motion analysis ( winarm software , zebris medical gmbh , germany ) . cms markers were placed at the greater tubercle and lateral epicondyle of the humerus and wrist . angles of deviation from the targeted range of flexion were analyzed by using the matlab version 2014a ( the mathworks inc . , natick , ma , usa ) . to compare the difference in jps error at 30 and 110 flexion , all data were analyzed by using the ibm spss statistics for windows software package ( ver . table 1table 1.comparison of errors according to angle ( unit : degree)30 flexion110 flexionright14.2 10.022.1 10.8*left16.1 11.322.4 9.7*p<0.05 presents the mean and sd of the angular error . the lowest error was observed for 30 right elbow flexion , while the highest error was observed for 110 left elbow flexion . we examined whether jps differs in accordance with the angle and found a significantly lower error for 30 right elbow flexion than 110 right elbow flexion ( p<0.05 ) . elbow joint movement is necessary during activities such as performing fine manipulative tasks , and jps is generally known to be part of the neuromuscular afferent detection system7 . the purpose of this study was to provide better understanding of active joint position sense ability for the elbow in children . we examined whether the error in jps differs in accordance with changes in angles and found a smaller error for 30 flexion than for 110 flexion in the right elbow . although statistically insignificant , the error for 30 flexion was smaller than that for 110 flexion in the left elbow . the more often jps is used in daily life , the more likely it is stored as body schema in the sensory and motor systems of the brain8 . movement at 30 elbow flexion is repeatedly used in daily lives , as it stabilizes eating or writing motions . the findings of the present study may have been influenced by such repetitive body movements . although we had predicted that the dominant hand would have a proprioceptive advantage over the nondominant hand , we found no significant differences in errors between the dominant and nondominant sides . jps , which is a component of the proprioceptive sense , plays a role in controlling speed and power when performing activities and a critical role in correcting the errors of movement internally or externally9 . as it is the basis on which humans learn and correct motor skill , it is essential for functional motion in daily lives . when children lack jps , which relays information to the brain regarding body positions with regard to muscles and joints , their motor abilities are reduced , and they are easily distracted10 . such children are more inclined to depend on visual information when performing simple tasks such as sitting on a chair or using a fork correctly . people with little information about their body position exert too much or too little force when performing tasks . furthermore , lack of jps leads to increased demand for energy when performing activities that require postural maintenance . despite the importance of jps , however , hence , we expect the findings of this study to be useful for the treatment of children with impaired jps . second , the proportion of boys was higher ( 15 boys and 4 girls ) . in active movement , the level of peripheral afferent information generated and received is higher11 . in addition , detection of active motion is more comparable with situations in daily life than detection of passive movements . therefore , future studies should examine the changes in jps when performing tasks in real - life situations rather than in passive movements .

[ purpose ] the purpose of this study was to investigate the difference in elbow joint position sense in children . [ subjects and methods ] nineteen healthy children volunteered as subjects for this study . joint position sense was assessed by asking the children to flex their elbows between 30 to 110 while blindfolded . the error range of elbow movement was analyzed with compact measuring system 10 for three - dimensional motion . to analyze data , descriptive statistics and paired t - test analysis were performed by using ibm spss statistics 20.0 . [ results ] a significantly lower error was found in 30 right elbow flexion than 110 right elbow flexion ( p<0.05 ) . no significant difference was found between 30 and 110 left elbow flexion . [ conclusion ] these results indicate that in children , joint position sense errors decrease as joint angles approach 30 flexion .

obesity is a major public health concern.1 more than one third of adults in the united states are classified as obese.2 the prevalence and severity of obesity continues to increase.3 obesity increases the risk for many chronic diseases , including type 2 diabetes mellitus , cardiovascular disease , hypertension , stroke , dyslipidemia , osteoarthritis , some cancers , nonalcoholic fatty liver disease , and decreases overall quality of life.2,4 if obesity is not controlled , the current generation may have a shorter life expectancy than the previous generation.2 given its lasting efficacy for improving obesity and obesity - related diseases,5 bariatric surgery is an increasingly common treatment option.6 as the implementation of the affordable care act progresses , the impending physician shortage will become more severe.7 thus there will be an even greater need for doctors specialized in the management and treatment of obese patients . the development of integrated bariatric surgery residency programs could be considered . these modified general surgery residencies would place greater focus on treatment of morbidly obese patients . by incorporating the traditional one - year minimally invasive and bariatric surgery fellowship training , the bariatric surgery residents could complete more bariatric - specific training in overall less time . the number of bariatric surgeons has increased in recent years,8 suggesting the field is of increasing interest . it is difficult to predict the exact number of new physicians this residency might interest . however , if the competitiveness9 of other integrated ( plastic , vascular , and cardiothoracic ) surgical residency programs is an indicator , integrated bariatric surgery residency programs may be highly sought after by medical school graduates . the accreditation council for graduate medical education ( acgme ) could create a primary certificate in bariatric surgery , eliminating the requirement for certification in general surgery prior to certification in bariatric surgery . because bariatric surgery includes a substantial component of general surgery , a thoughtful modification of general surgery residency program structure will have to occur to enable the expedited training without loss of core surgical competency . leaders in bariatric surgery , general surgery , and acgme can use other integrated programs that similarly combined fellowship training into residency as models for developing strong integrated bariatric surgery programs . fundamentally , the goal of such programs would be to treat the growing obesity epidemic by most efficiently producing highly trained bariatric surgeons .

obesity is a major public health concern . given its lasting efficacy for improving obesity and obesity - related diseases , bariatric surgery is an increasingly common treatment option . as the implementation of the affordable care act progresses , the impending physician shortage will become more severe . thus there will be an even greater need for doctors specialized in the management and treatment of obese patients . the development of integrated bariatric surgery residency programs could be considered and is discussed herein .

hypoglycemia is a rare but well - known paraneoplastic manifestation of patients with neoplasms , including hepatocellular carcinoma ( hcc ) , which is referred to as non - islet cell tumor - induced hypoglycemia ( nicth ) . excessive secretion of incompletely processed precursors of insulin - like growth factor - ii ( termed the the big igf - ii excessively stimulates both igf - i and the insulin receptor , inducing hypoglycemia and tumor growth . in the era of molecular - targeted therapy , agents targeting the igf signal are being developed to treat lung and pancreatic cancers . although this signal is suggested to be involved in aberrant growth of hcc , clinical trials using these agents against hcc have been initiated only recently . sorafenib is the first agent to demonstrate a survival benefit in the treatment of advanced hcc . this small molecule inhibits serine / threonine kinase raf in tumor cells and tyrosine kinases vascular endothelial growth factor receptor ( vegfr)/platelet - derived growth factor receptor ( pdgfr ) in the tumor vasculature , decreasing tumor growth and angiogenesis . in this paper , we report a case of hcc that showed severe hypoglycemia and was treated with sorafenib . since raf is one of the downstream components of the igf signal , sorafenib may be effective against tumors with an activated igf signal . although the effect was limited in the present case , emerging drugs that directly inhibit the igf signal can be expected to be highly effective in the treatment of hcc with nicth a 77-year - old male patient with hcc was referred to the authors hospital . as he had no previous episodes of liver disorders , no imaging procedures had been performed . in february 2010 , he was first admitted to another hospital due to bleeding gastric ulcers induced by non - steroidal anti - inflammatory drugs . during hospitalization , 1a , b ) . based on elevated serum afp ( 897 ng / ml ) and typical ct scan images as hcc , he was diagnosed as advanced hcc and referred to our hospital in march 2010 . administration of sorafenib was initiated at a dosage of 800 mg b.i.d . on day 7 , pleural effusion was detected and his serum potassium concentration was elevated to 5.5 meq / l . his general condition declined and he was unable to stand by day 11 . on day 14 , he was hospitalized because of hyperpotassemia ( 6.7 meq / l ) and hypoglycemia ( 27 mg / dl ) . hyperpotassemia improved by the administration of an intravenous drip infusion of glucose and furosemide , but hypoglycemia continued at a level of 40 mg / dl . although the basal levels of adrenal hormones were normal , acth and cortisol did not increase at the time of hypoglycemia . we started to administer a short - acting corticosteroid ( hydrocortone ) , and the blood glucose level increased rapidly to around 150 mg / dl . however , several days later , the patient 's morning fasting blood glucose level decreased to around 20 mg / dl . we administrated a longer - acting corticosteroid and the patient also began to have late evening snacks . although a sufficient amount of cortisol ( prednisolone 10 mg / day ) was administered , his hypoglycemia continued . we suspected that other factors were involved in the hypoglycemia , but the serum levels of insulin and igf - i were lower than the normal limits . we assayed the patient 's serum using immunoblotting with an anti - igf - ii antibody . 2 , lane 2 ) similarly to the serum of a patient with nicth ( lane 4 ) . only mature igf - ii was detected in the serum of the normal control ( lane 3 ) . lane 1 was recombinant igf - ii . by day 14 of sorafenib administration , though the number of lung metastases had increased ( fig . 1c ) and the tumor marker levels had decreased ( afp from 4,112 to 2,381 ng / ml and pivka - ii from 4,645 to 952 mau / ml ) ( fig . the dose of sorafenib was decreased to half ( 400 mg b.i.d . ) , and the patient was discharged . though the hypoglycemia improved with treatments , sadly the patient died 6 days later of respiratory failure due to advanced lung metastases . several previous reports have shown nicth as a rare paraneoplastic manifestation of advanced hcc with a poor prognosis . as far as we are aware , there are no reports describing hcc with nicth treated with this novel molecular targeted agent , sorafenib . the present case showed interesting endocrine abnormalities such as hypoglycemia and hyperpotassemia due to relative adrenal insufficiency . the possibility that sorafenib suppressed adrenal function must be considered , since there were no other factors known to affect adrenal function such as metastasis to the adrenal glands . no reports have been identified that describe adrenal insufficiency due to sorafenib , while the drug is reported to affect thyroid functions . the possibility that sorafenib played a role in adrenal insufficiency is also supported by the fact that there are some reports of adrenal dysfunction caused by a similar molecular agent , sunitinib , targeting vegfr / pdgfr . the igf signal is related to cell proliferation and tumor growth of hcc through the igf - i receptor . reported that lower plasma igf - i levels are correlated with advanced hcc and poor overall survival . big igf - ii , is one of the most frequent mechanisms of igf signal activation in hcc . expression of igf - i may be suppressed by a negative feedback of igf - ii overexpression , resulting in lower plasma igf - i levels . big igf - ii is suggested to induce hypoglycemia through igf - i and the insulin receptor . reduction of tumor volume by surgical operation , transarterial chemoembolization or systemic chemotherapy is sometimes effective . palliative treatments , including administration of hyperglycemic hormones such as corticosteroids and growth hormones , are performed , but the effects are transient and limited . when the igf - i receptor is stimulated , the downstream signaling pathways , including pi3k - akt - tor and raf - mek - erk , are activated . such drugs directly inhibit intracellular kinase activities or block the binding of igf to the receptors . in particular , use of antibodies against igf - ii will probably be selective and safe in such cases .

hypoglycemia is a rare paraneoplastic manifestation of patients with neoplasms . hypoglycemia can be induced by several causes , including an aberrant increase of hypoglycemic agents and adrenal insufficiency . sorafenib is the first agent to demonstrate a survival benefit in the treatment of advanced hepatocellular carcinoma ( hcc ) . this small molecule inhibits serine / threonine kinase raf in tumor cells and tyrosine kinases vegfr / pdgfr in tumor vasculature and decreases tumor growth and angiogenesis . in this paper , we report a case of hcc who was treated with sorafenib and showed severe hypoglycemia . this hypoglycemia might be induced by two causes , both adrenal insufficiency as an adverse effect of sorafenib and activation of the insulin - like growth factor ( igf ) signal by excessive secretion of incompletely processed precursors of igf - ii . although the igf signal is suggested to be involved in aberrant growth of hcc in some cases , there is no other report showing the influence of sorafenib on hcc with active igf signal . unfortunately , the effect of sorafenib was limited in the present case . however , emerging drugs that directly inhibit the igf signal can be expected to be highly effective in the treatment of hcc with hypoglycemia .