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tinea capitis is a fungal infection of hair and scalp that typically occurs in childhood and equally in both sexes that has recently increased in incidence in adults and the elderly . t. tonsurans infections may not be symptomatic and mostly infects children ; however , it can also cause disease in adults who are close contact with those children . thus , it is difficult to diagnose and it requires a high index of suspicion from the examination physician . tinea capitis has clinical features ranging from a non - inflammatory scaling to a severe pustular eruption with alopecia , known as kerion . t. tonsurans performs an endothrix type of hair invasion , which causes hair to break easily . in addition , it also leads to an inflammatory tinea capitis , which often leads to misdiagnoses . a 38-year - old primary school female teacher presented with a history of symptomless , patchy , slow , progressive hair loss for several years . she had been using over - the - counter ketoconazole shampoo regularly for a long time without any improvement . the patient denied history of animal contact or history of pattern hair loss in her family . bizarre - shaped patches of hair loss with surrounding hair thinning on the vertex were observed ( figure 1 ) . there was no broken hair shaft , exclamation - point hair or significant scalp inflammation surrounding the area of alopecia . potassium hydroxide ( koh ) 10% examination from hair and scalp was negative , and therefore , fungal culture was not performed . the clinician raised the possible diagnosis of this atypical , non - scarring alopecia as alopecia areata with the differential diagnosis of androgenetic alopecia , chronic telogen effluvium , and trichotillomania . betamethasone valerate scalp lotion was prescribed to apply twice daily on the affected scalp area but without any clinical improvement . two 4 mm punch biopsy specimens were taken from the peripheral edge of the alopecia for both vertical and transverse sections to evaluate the cause of non - scarring hair loss in this patient . the histopathology of the scalp biopsy specimens demonstrated an adequate number of terminal hair follicles without evidence of dermal fibrosis . there was minimal superficial perivascular and perifollicular mononuclear cell infiltrate in some of the follicles without definite peribulbar infiltration . numerous fungal hyphae and arthrospores within several hair shafts were demonstrated in both vertical and transverse sections . the epidermis remained intact with orthokeratosis of the keratin and no significant inflammation ( figure 2a and b ) . dermoscopic examination revealed broken hair shafts , comma hair sign and a large number of corkscrew hairs ( figure 3a and b ) . wood lamp s test revealed a non - fluorescent result . a fungal culture from scalp biopsy and hair demonstrated colonies of t. tonsurans . based on clinical presentation , the patient was treated with griseofulvin 500 mg twice daily in combination with ketoconazole shampoo once daily application for eight consecutive weeks . this patient was not in a common age group and partially treated with ketoconazole shampoo . thus , the symptoms could be concealed , resulting in the delay of diagnosis . generally , the diagnosis of tinea capitis must be confirmed by koh preparation of infected hairs and a fungal culture . although scalp biopsy provides high sensitivity , this procedure is invasive and unable to identify the species of pathogen . therefore , the procedure is not in routine clinical use . scalp skin biopsy is performed in order to make a differential diagnosis of unidentified alopecia or another alopecia that is recalcitrant to treatment . due to the obscure clinical presentation of tinea capitis in this patient , the dermoscope , a non - invasive handheld device , could be useful for diagnosis . the appearance of lesional hair under the dermoscope were comma shape and corkscrew hairs , regardless of dermatophyte species . tinea capitis treatment is mainly oral antifungals , but a topical antifungal shampoo is suggested as an adjuvant therapy . this patient used antifungal shampoo for several years that prolonged the disease without complete cured . using of antifungal shampoo probably caused the result to be negative in the first koh examination . additionally , this patient had been prescribed topical steroid medication , which caused a positive result in the following koh examinations . apart from treatment , physicians should consider disease prevention by screening for close - contact individuals and carriers especially in this particular case : the patient was a primary school teacher , who had contact with children . physicians could not make a diagnosis due to the unclear clinical features , until histopathologic exam of the hair shaft showed endothrix fungal infection . the patient had been using over - the - counter ketoconazole shampoo regularly for a long period of time , which caused tinea capitis signs and symptoms unclear and a negative result in the first koh test .

tinea capitis is unusual and often misdiagnosed in healthy adults . we report a case of a healthy woman with a several - year history of asymptomatic , bizarre - shaped , non - scarring alopecia . she had used over - the - counter ketoconazole shampoo regularly for a long time . an initial potassium hydroxide preparation showed negative result for fungal organism . the scalp biopsy revealed endothrix infection , and dermoscopic examination demonstrated the comma hair and corkscrew hair signs . the fungal culture showed trichophyton tonsurans . the daily use of antifungal shampoo could be the important factor to conceal clinical and laboratory findings for diagnosis of t. tonsurans tinea capitis in our case , which required high clinical suspicion and histopathology and dermoscopic examinations .

all the reagents used are of laboratory reagent grade and solvents were of analytical grade . ibuprofen and naproxen were provided by macleods pharmaceutical pvt . ltd . as gift samples . high performance liquid chromatography ( hplc ) method has been developed for the determination of purity and stability study of synthesized derivatives . naproxen ( 8) and ibuprofen ( 9 ) were coupled with tropinol ( 3 ) using ethyl dimethylamino propyl carbodimide ( edc ) and dimethylaminopyridine ( dmap ) to obtain the esters ( 4 - 5 ) as oily products . the esters ( 4 - 5 ) were quaternized with methyl iodide to obtain the desired quaternary derivatives ( 6 - 7 ) as shown in scheme 1 . hplc method has been developed for the determination of purity and stability study of the synthesized derivatives in phosphate buffer ph 7.4 and in human plasma . all synthesized derivatives shows good stability in phosphate buffer at ph 7.4 ( half life of disappearance 30 - 45 h ) but get hydrolysed fast in human plasma ( half life 8 - 12 h ) . in order to check the site specific concentration of these quaternary derivatives ( 6 - 7 ) radiolabelling was done using radioactive tc , radiolabeling of the compounds with tc was carried out by direct labeling method to form complex and study their site specific localization tendency in the inflamed tissue . the in vitro stability of radiolabeled complexes was determined in sodium chloride ( 0.9% ) and in human serum . once the complexed derivatives ( tc qa ) were found to be stable then imaging study was performed in sprague - dawley rats using a single photon emission computerized tomography gamma camera and both derivatives shows higher affinity towards cartilage than parent nsaid . naproxen ( 8) and ibuprofen ( 9 ) were coupled with tropinol ( 3 ) using ethyl dimethylamino propyl carbodimide ( edc ) and dimethylaminopyridine ( dmap ) to obtain the esters ( 4 - 5 ) as oily products . the esters ( 4 - 5 ) were quaternized with methyl iodide to obtain the desired quaternary derivatives ( 6 - 7 ) as shown in scheme 1 . hplc method has been developed for the determination of purity and stability study of the synthesized derivatives in phosphate buffer ph 7.4 and in human plasma . all synthesized derivatives shows good stability in phosphate buffer at ph 7.4 ( half life of disappearance 30 - 45 h ) but get hydrolysed fast in human plasma ( half life 8 - 12 h ) . in order to check the site specific concentration of these quaternary derivatives ( 6 - 7 ) radiolabelling was done using radioactive tc , radiolabeling of the compounds with tc was carried out by direct labeling method to form complex and study their site specific localization tendency in the inflamed tissue . the in vitro stability of radiolabeled complexes was determined in sodium chloride ( 0.9% ) and in human serum . once the complexed derivatives ( tc qa ) were found to be stable then imaging study was performed in sprague - dawley rats using a single photon emission computerized tomography gamma camera and both derivatives shows higher affinity towards cartilage than parent nsaid . cartilage targeting strategy for the conventional nsaids has been explored for reducing their local and systemic side effects the nsaids . selective concentration of these ester derivatives into the tissue in demand could also allow reduction in their doses . it could be concluded that site specific delivery of these quaternary ester derivatives could prove to be an effective strategy for long term therapy of inflammatory conditions like arthritis .

nsaids are among the most widely used prescription drugs , for the treatment of pain and inflammation , particularly arthritis . inhibitor of cox and nsaids relieve the signs and symptoms of inflammation by decreasing pg production but may cause gi and renal damage . synthesis , stability studies and y - imaging studies are carried out . cartilage targeting strategy for conventional nsaids has been explored for reducing their local and systemic side effect of the nsaids site specific delivery of quarternary ester derivatives could have to be an effective strategy for long term therapy of inflammatory conditions like arthritis .

white cottony mycelia formed on the stems of the diseased plants , which became tough and formed abundant amounts of brown spherical sclerotia on the surface of the mycelial mat . initially , stem rot disease affected only a small number of garlic plants ; however , the disease gradually spread and caused large - scale damage . the symptoms of the disease began in may , when the canopy of the garlic plant became densely covered , blocking air flow and light . in late may , relatively high air temperatures and frequent rain fall favored disease development . according to farmers who had experience with this disease , the disease becomes troublesome only when environmental conditions favor disease development . stem rot disease of garlic is a soil - borne disease , the inoculum potential of which increases in continuously mono - cropped areas . it is presumed that the debris of infected plants is main inoculum source of the disease . abundant sclerotia are formed on the stems and bulbs of infected plants , and the sclerotia are overwintered and invade the root , crown , and stem when garlic is planted ( fig . the causal organism of garlic stem rot was isolated from the sclerotia formed on the stems of the infected plants . the surfaces of the collected sclerotia were disinfected with a 1% naocl solution for 1 min , rinsed with sterilized water 3 times , and blotted with 5 layers of flame - sterilized filter papers ( 90 mm ) . the surface - sterilized sclerotia were placed on potato dextrose agar ( pda ) medium and incubated at 25 for 4 days . the fresh mycelia grew out from sclerotia on agar surface were cut with spatula and transferred to new pda and incubated at 25. the causal organism was cultivated in pda for 3 wk , and the morphology of the hyphae and clamp connection structure and sclerotia formation were examined under light microscopy ( table 1 ) . the mycelia were white and grew fast and prosperously on the surface of the pda and had a cottony appearance . 1d ) , were tan to brown in color , and were generally spherical , although some were irregularly shaped . after a 4-day incubation period on pda at 25 , a typical clamp connection structure was observed in the hyphae ( fig . the diameter of the hyphae ranged from approximately 4 to 8 m , and the optimal temperature for growth of the fungus was 30. the causal organism of stem rot disease in garlic and the associated symptoms agreed with the findings of kishi , and the mycological characteristics of the fungus agreed with those reported by mordue . the pathogenicity of the fungus was examined in a garlic plant grown in 1/5000a wagner 's pots . three bulbs of health garlic were planted in wagner 's pots ( 10 replication ) in october 2008 . the inoculum of the fungus was prepared by mixing fungal mycelia and soil 5 kg of sandy loam soil was sieved and autoclaved at 121 for 30 min . and repeatedly autoclaved 3 times at 5-day intervals . the mycelial mat of the test fungus grown on pda for 7 days was harvested . the harvested mycelial mat from 30 petri dishes ( 9 cm in diameter ) was mixed thoroughly with 5 kg of sterilized soil in a plastic container ( 56 35 13 cm ) . the mixture containing the mycelial mat and the soil was dried in the shade for 15 days and then powdered . the powdered mixture was preserved in a green house and used as inoculums ; 200 g of soil inoculum was placed on top of the wagner 's pots . after infestation of inoculum , 500 ml of tap water was added to each pot and covered with one layer of newspaper to maintain sufficient soil moisture . the inoculated pots were kept separately in a green house and were observed for disease symptoms . seven days after inoculation , white mycelia on the stems of the garlic and premodia of sclerotia were observed . as time progressed , the premodia of sclerotia developed into typical brownish sclerotia , and the white mycelia on the stem and soil surface had typical stem rot symptoms ( fig . , many diseases caused by sclerotium rolfsii in various crops have been reported [ 3 - 6 ] . the disease described in this article and the mycological characteristics of the causal organism were in line with these reports . on the basis of the results obtained in this study , the author suggested that the disease observed was stem rot of garlic caused by s. rolfsii saccardo . the isolate obtained from garlic in this study was deposited in the korean agricultural culture collection and was assigned the registration number kacc no .

stem rot disease was found in garlic ( allium sativum l. ) cultivated from 2008 to 2010 in the vegetable gardens of some farmers in geumsan - myon , jinju city , gyeongnam province in korea . the initial symptoms of the disease were typical water - soaked spots , which progressed to rotting , wilting , blighting , and eventually death . white mycelial mats had spread over the lesions near the soil line , and sclerotia had formed over the mycelial mats on the stem . the sclerotia were globoid in shape , 1~3 mm in size , and tan to brown in color . the optimum temperature for growth and sclerotia formation on potato dextrose agar ( pda ) medium was 30. the diameter of the hyphae ranged from approximately 4 to 8 m . typical clamp connection structures were observed in the hyphae of the fungus , which was grown on pda medium for 4 days . on the basis of the mycological characteristics and pathogenicity of the fungus on the host plants , the causal agent was identified as sclerotium rolfsii saccardo . this is the first report of stem rot disease in garlic caused by s. rolfsii in korea .

recently , seok et al.1 described a successful correction of post - traumatic anterior open bite via injection of botulinum toxin type a ( btx - a ) into the anterior belly of the digastric muscle ( abdm ) in a 20-year - old man with malocclusion and wound dehiscence in the left retromolar area . in the case of mandibular angle fractures , the digastric muscles depress the anterior fragment2 ; therefore , by paralyzing the musculature , seok et al.1 were able to mitigate the forces displacing the anterior mandible . a ) , that the btx - a injections were placed in bilaterally symmetrical anterior digastric musculature that was unremarkable with regard to anatomical variation . abdm is typically considered to be bilaterally symmetrical ; however , abdm muscular variants have been reported to occur in as much as 69.9% of the population3 . diverse anatomical variations of the abdm have included duplications , triplications , and quadrifications , in addition to exotic fractal and weave patterns of musculature45678 . also , abdm has been reported to cross the midline and insert contralaterally 5 . therefore , the existence of variant abdm should be taken into consideration when attempting to reduce fractures of the mandible . an example of a variant abdm that may complicate btx - a injection is shown in fig screening for anatomical variation of anterior digastric musculature with ultrasound ( us ) , computed tomography ( ct ) , and/or magnetic resonance imaging ( mri ) should be performed in order to adequately plan injection of btx - a into anterior digastric musculature . upon imaging with us , skin can be marked directly over the digastric musculature in order to identify injection sites as variant musculature is encountered . with ct and mri , bony references , such as the mandible and hyoid , can be used to calibrate measurements corresponding to the location of the musculature , which can subsequently be used to mark injection sites on the submental skin . in light of their success in the treatment of the patient who responded favorably to btx - a injection into the abdm for the treatment of anterior open bite , seok et al.1 recommended a large - scale , case control study in which btx - a injection is applied to selective open bite patients who do not respond to extensive rubber traction . while such a study might be prudent , it is important to recognize the diversity of abdm musculature when planning btx - a injection for the management of anterior open bite , in order to avoid undesired effects . botulinum toxin injected in the neck can produce weakness of the neck flexors and dysphagia9 ; however , the majority of adverse effects due to botulinum toxin injections include bruising , edema , pain at the injection site , and flulike symptoms 91011 . the more serious side effects of injection typically result from improper needle placement due to a lack of understanding of the underlying anatomy and physiology , subsequently allowing the neurotoxin to either diffuse or be inadvertently injected into adjacent musculature91011 . there are obvious differences among these imaging techniques with regard to cost , time , and invasiveness ; however , to the author 's knowledge , no reports have specifically compared these techniques with regard to proper identification or characterization of anterior digastric musculature . screening for variant abdm should be performed prior to btx - a injection into the anterior digastric musculature in order to account for muscle variations that may result in undesired forces on the anterior mandible in patients with anterior open bite and to prevent undesired adverse effects of btx - a injection .

it has recently been reported that long - standing post - traumatic open bite can be successfully corrected with botulinum toxin type a ( btx - a ) injection into the anterior belly of the digastric muscle ( abdm ) . the report documented an individual with bilaterally symmetrical and otherwise unremarkable anterior digastric musculature . however , the existence of variant anterior digastric musculature is common and may complicate the management of anterior open bite with btx - a injection . screening for variant abdm can be accomplished via ultrasound , computed tomography , and magnetic resonance imaging . screening for variant abdm should be performed prior to btx - a injection in order to account for musculature that may exert undesired forces , such as inferolateral deviation , on the anterior mandible in patients with anterior open bite .

the calcifying epithelial odontogenic tumor ( ceot ) is a rare benign tumor of the jaws first described by pindborg in 1955 and also referred as pindborg tumor . the clear cell variant of ceot was first reported by abrams and howell in 1967 . topographically two entities have been distinguished : intraosseous ( central ) and extraosseous ( peripheral ) . twelve central lesions have been reported of which only three cases have been reported in maxilla . typically , ceot has been described as slow growing , benign and locally aggressive tumor . hicks et al . , suggested that clear cell variant may show more aggressive behavior with higher recurrence ( 22% ) . clear cells are present as cellular components of the epithelial lining of lateral periodontal and gingival cysts in adults , or they may be found as clear - cell rests of the dental lamina within the connective tissue wall of these cysts . philipsen and reichart reviewed 181 cases of pindborg tumor , 15 of which are clear cell containing lesions . some authors have classified cceot as separate clinical entity . in this paper , a rare case of cceot of posterior maxilla is reported and clinical , radiographic histological features of the same are discussed . a 36-year - old indian woman reported to our department with painless , slow growing swelling of right upper jaw and loss of upper teeth due to mobility . the swelling was about 3 2 cm with normal color , intact mucosa and indentations of opposing tooth [ figure 1 ] . opg showed 3 3.5 cm mixed radiolucent - radiopaque lesion in the posterior maxilla with impacted teeth . radiographically driven snow computed tomography ( ct ) revealed radiolucent - radiopaque mass in the right maxilla invading the cortex [ figure 3 ] . intra oral photograph showing solitary swelling in right posterior maxillary region snow - driven , radiolucent - radioopaque lesion seen in right maxilla in orthopantomogram computed tomography scan showing radiolucent - radiopaque mass in the right maxilla invading the buccal cortex an incisional biopsy was taken from the lesion . hematoxylin and eosin stained sections showed fibrous connective tissue stroma with islands of polyhedral epithelial cells containing nuclei of varying form and size . extracellular eosinophilic amyloid like material was found containing concentric calcifications in the form of liesegang rings [ figure 4 ] . initial histology sections showing liesegang rings ( h&e stain , 200 ) considering the age of the patient , esthetic concern and benign nature of ceot , curettage of the lesion along with the associated tooth was performed under general anesthesia ( ga ) . intranasal biopsy was performed , revealed clear cells in the stroma of ceot confirming the diagnosis of clear cell ceot [ figure 6 ] . recurrence evident in ct photomicrograph of intranasal biopsy showing liesegang rings and clear cells ( h&e stain , 100 ) clear cell variant of ceot is a rare entity and central cceot occurring in maxilla is rarest entity . an extensive review of literature on clinical and radiologic features of cceot has been done by anavi et al . equal distribution in males and females , age range of 14 - 68 years ; and of the 12 central lesions , nine involving the mandible and three in maxilla ( 75:25 ) have been reported . the typical clinical presentation of cceot is painless swelling of the jaw causing cortical expansion . radiological features of ceot is unilocular or multilocular lesion with mixed radiolucent and radiopacity of various density . clinical features and radiological features of the case presented are consistent with that of the other cceot reported . the three maxillary lesions described were radiolucent in opg , in the presented case lesion was typically of driven snow appearance . histological sections of ceot containing islands of polyhedral epithelial cells , having nuclei of varying form and size with extracellular eosinophilic material amyloid and concentric calcifications within the connective tissue matrix in the form of liesegang rings are typical . clear cell variant consists of sheets or cords of clear cells with foamy cytoplasm in the matrix . these features were detected in the case presented confirming the diagnosis of clear cell variant . other variants of ceot described include pigmented , langerhan cells containing and non - calcifying variants . the clear cell variant tends to be more aggressive and shows higher recurrence rates than conventional ceot . the surgical management modalities in 19 reported cases were complete or partial resection in seven patients ( 37% ) , excision in seven ( 37% ) , enucleation in four ( 21% ) and curettage in one case ( 5% ) . waldron et al . , and hicks et al . , have reported that occurrence of the clear cells may prove to be a sign of increased tumor aggressiveness indicating a more radical approach . recurrence of tumor following curettage in our case strongly supports the data and enbloc resection is recommended . evidence supports that clear cell variant is a distinct entity , has more aggressive biological behavior and higher chances of recurrence . hence , it is important that presence of clear cells be included in histopathological diagnosis . the identification of clear cell directs the surgeon towards more definitive surgical excision of the lesion . maxillary lesions should be treated more aggressively because they grow faster and possess close proximity to important structures .

the calcifying epithelial odontogenic tumor ( ceot ) is a rare benign tumor of the jaws . pindborg 's tumor having clear cells is extremely rare . twelve central lesions have been reported of which only three cases have occurred in maxilla . clear cell variant is a distinct entity , has more aggressive biological behavior and higher chances of recurrence . hence it is important that presence of clear cells be included in histopathological diagnosis . here we present a rare case of clear cell ceot having aggressive behavior .

sepsis affects more than 750,000 patients each year in the united states ; it is the 10th leading cause of death and one of the leading causes for admission to the intensive care unit [ 1 - 4 ] . the estimated mortality from sepsis is 2030% , meaning that approximately 500,000 patients survive their septic episode annually in the united states alone . are they able to resume their lives and regular activities , or does sepsis have far - reaching effects that extend beyond the hospitalization ? ten years ago , quartin and colleagues were the first to show that sepsis has long - lasting effects and increases the risk of death up to 5 years after hospitalization for the septic episode . mounting evidence has since demonstrated that survivors of sepsis have a higher long - term risk of death and a lower health - related quality of life ( hrqol ) when compared with the general population [ 6 - 8 ] . in 2001 , recombinant human activated protein c ( rhapc ) was shown to significantly reduce the 28-day mortality in patients with severe sepsis . a subgroup analysis demonstrated that patients who were more severely ill , with multiple organ dysfunction or with acute physiology and chronic health evaluation ( apache ) ii scores 25 , accrued the greatest benefit from rhapc , resulting in drug approval focused on these sepsis populations . a subsequent randomized controlled trial that evaluated rhapc use in patients with severe sepsis and a low risk of death ( apache ii score < 25 ) found no survival benefit with rhapc . given the high cost of rhapc , the attendant bleeding risk associated with its use , and the lack of clinical trials confirming its efficacy , there continues to be debate and controversy regarding its appropriate use . with the increasing emphasis on patient - centered outcomes in clinical trials , we are now more frequently assessing short - term and long - term survival and , at least sometimes , hrqol . in assessing long - term outcomes among sepsis survivors from the original rhapc trial , the short - term survival benefit for more severely ill patients ( apache ii scores 25 ) treated with rhapc was also evident at 3 , 6 and 12 months , while there was no difference in survival for those less severely ill patients . in the previous issue of critical care , longo and colleagues evaluated the effect of rhapc on long - term hrqol and resource utilization in patients with severe sepsis . using an observational cohort of 100 patients with severe sepsis ( 36 patients received rhapc and 64 patients received standard care ) who survived to day 28 , the patients ' hrqol was measured using the short form 36 ( sf-36 ) at 3 , 5 and 7 months , and resource utilization was measured using patients ' self - reports recorded in a diary . patients who were treated with rhapc had significantly better physical component scores on the sf-36 throughout the follow - up period , without significant differences in other components of the sf-36 . patients treated with rhapc also had a significantly shorter hospital stay compared with patients in the standard care group , and showed statistically nonsignificant improvements in the rate of return to employment . one of the major limitations of their study is that rhapc was administered in a nonrandomized fashion , meaning the use of rhapc was left to the discretion of the attending physician . unfortunately , we are given little information about what factors the attending physicians considered when deciding to treat patients with rhapc . without the design of a randomized controlled trial , the results are subject to bias and confounding by unmeasured factors . the authors recognize these limitations and demonstrated that the two groups of patients ( rhapc vs. standard care ) were similar in illness severity ( apache ii score ) and had similar numbers of comorbidities . however , patients who received rhapc were significantly younger and more likely to have been admitted through the emergency department . further , there are no data provided about other factors that may have influenced survival and hrqol such as the type or severity of comorbities ( hiv , malignancy ) [ 13 - 15 ] , organ dysfunction , use of vasopressors or need for mechanical ventilation . these unmeasured differences may fully or partially account for the observed differences in long - term hrqol . for example , younger patients with minor comorbidities ( for example , hypertension ) and a better chance of long - term survival may have been more likely to receive rhapc than an elderly patient with metastatic cancer . other limitations include the small sample size and the limited power to detect smaller but meaningful differences in other components of the sf-36 or resource utilization . despite the limitations of the study , longo and colleagues address an important issue evaluating the long - term effects of a sepsis therapy . their findings suggest that treatment with rhapc may result in improved long - term physical functioning in patients with severe sepsis . the authors hypothesize that rhapc may reduce acute organ dysfunction , thereby diminishing the likelihood of chronic dysfunction and thus improving long - term hrqol . as noted by the authors , this study does not provide conclusive evidence that rhapc improves long - term hrqol , and nor does this study provide any insight into the mechanisms responsible for the improvement in physical functioning . instead , the study offers intriguing preliminary data that should provide the foundation for a larger , more rigorously designed clinical trial examining the long - term effects of rhapc on hrqol . certainly , if rhapc is shown to improve long - term health outcomes , including hrqol , it will open new windows for drug evaluation and provide more evidence to help us determine the optimal use of this controversial medication . apache = acute physiology and chronic health evaluation ; hrqol = health - related quality of life ; rhapc = recombinant human activated protein c ; sf-36 = short form 36 .

sepsis is one of the most common conditions encountered in the intensive care unit and is the 10th leading cause of death overall in the united states . both long - term survival and health - related quality of life are reduced in survivors of sepsis , yet there is little knowledge of the effect of sepsis - specific interventions on either long - term survival or health - related quality of life . the present article discusses the importance of studying health - related quality of life as it relates to sepsis management strategies , particularly in the context of pharmacologic therapy with recombinant human activated protein c.

in response to jev activity , as evidenced by human clinical cases or the seroconversion of sentinel pigs , adult mosquitoes were collected on badu island during 1995 , 1998 , and 2003 ( 6,8 , and this article , respectively ) . badu island is located at 1007s and 14209e in the central western region of the torres strait and is a granite island of 101 km ; its ecology has been described ( 68 ) . the mosquitoes were collected with centers for disease control ( cdc ) miniature light traps ( model 512 ; john w. hock co. , gainesville , fl , usa ) baited with either co2 alone or in combination with 1-octen-3-ol . mosquitoes were killed on dry ice and placed in liquid nitrogen dry shippers or on dry ice in insulated containers for transport to cairns for storage at 70c . mosquitoes were placed on a refrigerated table for species or taxonomic group identification before being placed in pools of < 200 mosquitoes and sent to queensland health forensic and scientific services or the university of queensland , brisbane , for jev detection . the virus isolation protocols used in 1995 and 1998 have been described ( 6,8 ) . in 2003 , virus rna was detected by using a real - time taqman reverse transcription pcr ( 9 ) . to facilitate the comparison of virus distribution on badu island , trap locations were grouped into 3 general areas : within 1.2 km of the piggery , within the area of human habitation ( the community ) , and at a rubbish dump located 1.5 km from the community and 4.0 km from the piggery ( figure 2 ) . dumps are a potential focus of jev activity because mosquitoes , feral pigs , and wading birds congregate at them ( 10 ) . maximum - likelihood estimation of mosquito infection rates with 95% confidence intervals were calculated for each of these general areas by using the pooledinfrate statistical software package ( 11 ) . aerial photograph of badu island showing the location of the community , piggery , and rubbish dump . because of the difficulty in morphologically separating the members of the cx . sitiens subgroup , data for the 3 members of the group found in australia cx . sitiens were pooled for analysis . however , pcr restriction fragment length polymorphism analysis of a subsample of 135 cx . annulirostris was the dominant member of this group on badu island and comprised 94.1% of polymorphic specimens processed ; the other 4.4% and 1.5% were identified as cx . sitiens subgroup mosquitoes were processed for detection of jev ; 2,871 , 24,592 , and 16,865 were processed from 1995 , 1998 , and 2003 , respectively ( table ) . sitiens subgroup mosquitoes ; the highest maximum - likelihood estimation of mosquito infection rate was obtained from mosquitoes collected at the dump in 1998 . despite removal of the domestic pigs , sitiens subgroup mosquitoes collected within the community in 2003 . however , the point estimates of infection rates were lower than those obtained in 1995 and 1998 , when domestic pigs were present within the community , although the slight overlap in 95% confidence intervals indicates that this difference in infection rate was not significant . * mosquito infection rates determined by maximum - likelihood estimation ; 1995 , apr 89 and 2021 , 30 trap nights ; 1998 , mar 56 , 25 trap nights ; 2003 , mar 1319 , 92 trap nights ; ci , confidence interval ; ns , mosquitoes not sampled from this location during the year of collection . japanese encephalitis virus positive pools detected by virus isolation or taqman reverse transcription pcr . we demonstrated that although removing domestic pigs from areas of human habitation may reduce contact between amplifying hosts and vectors ( 7 ) , it does not eliminate the presence of jev - infected mosquitoes . indeed , evidence for low - level virus transmission to humans is provided by hanna et al . , who found a low level ( 32% ) of natural boosting immunity in badu residents who had received an inactivated mouse brain as has been observed elsewhere in the absence of pigs ( 13 ) , mosquitoes may have become infected by feeding on viremic herons and egrets , populations of which are found on badu island ( 14 ) . analysis of host feeding patterns demonstrated that birds accounted for 23% of blood meals of cx . data ) . alternately , feral pigs in the community and at the dump are a potential source of virus for mosquitoes , although < 1% of mosquito blood meals were from swine at these locations . mosquitoes could become infected by feeding on viremic pigs at the piggery and then disperse to other areas on the island . annulirostris is 4.4 km ; some females traverse up to 12 km ( 15 ) , which is considerably farther than the 2.5 km between the piggery and the community on badu island . solomon recommends that domestic pigs be moved > 5 km from human habitation to limit jev transmission to humans ( 3 ) . indeed , if infected mosquitoes were flying from the piggery to the community , then the data from our study support this recommendation . however , we suggest that domestic pigs be removed far enough away from human habitation to encompass the flight range of the local culex vectors .

to determine whether relocating domestic pigs , the amplifying host of japanese encephalitis virus ( jev ) , decreased the risk for jev transmission to humans in northern australia , we collected mosquitoes for virus detection . detection of jev in mosquitoes after pig relocation indicates that pig relocation did not eliminate jev risk .

a 68-year - old male with a history of palpitations was referred to our hospital for further evaluation . baseline ecg showed sinus rhythm and frequent premature ventricular extrasystoles ( pves ) with a relatively narrow qrs complex ( 115 ms ) , an inferior axis ( tall r waves in leads ii , iii , and avf ) , a qr pattern in lead v1 with no transition zone , and absence of s wave in lead v5 or v6 ( figure 1(a ) ) . an electrophysiological study was then carried out , where a detailed activation mapping of the pves was performed using a 3-dimentional electroanatomical mapping system ( carto 3 , biosense webster , inc . , diamond bar , calif , usa ) . left ventricular ( lv ) mapping was performed using a 4 mm tip deflectable catheter ( navistar , biosense webster ) . the earliest local activation site of the pves ( preceding the onset of the surface qrs by 40 ms ) was identified at the aortomitral continuity of the basal lv ( figure 1(b ) ) . ( 12 of 12 leads ) with the morphology of the pves ( figure 1(c ) ) . the first radiofrequency ( rf ) energy application ( target temperature of 60c , 30 w , 60 s , and impedance drop of 510 ohms ) abolished all the pvbs arising from the aortomitral continuity ( figure 2 ) . additional rf applications ( total ablation time up to 180 s ) were delivered at this region in order to ensure a long - term success ( red dots in figure 2 ) . lv outflow tract ventricular arrhythmias originating from the aortomitral continuity , the left coronary cusp , the superior basal septum , and the epicardial left ventricular summit display common electrocardiographic and electrophysiological features , probably due to the close proximity of those locations [ 13 ] . data regarding ventricular arrhythmias arising from the aortomitral continuity are limited and conflicting [ 13 ] . have shown that pves arising from the aortomitral continuity display narrow qrs complexes , tall r waves in inferior leads ( particularly in lead ii ) , predominantly positive forces in lead i ( where superior and lateral basal sites display negative forces ) , and a qr pattern in lead v1 . have demonstrated the occurrence of a qr pattern in lead v1 and s wave in lead v5 or v6 in 50% of patients with pves arising from aortomitral continuity . the absence of s wave in lead v5 or v6 favours the diagnosis of pves originating from the left coronary cusp or the epicardial left ventricular summit . in a different study , kumagai et al . have shown that all patients with pves arising from the aortomitral continuity exhibit monophasic r waves without s waves in almost all precordial leads . these investigators failed to demonstrate a qr pattern in lead v1 or s wave in lead v6 .

left ventricular outflow tract arrhythmias originating from the aortomitral continuity , the left coronary cusp , the superior basal septum , and the epicardial left ventricular summit display common electrocardiographic and electrophysiological features , probably due to the close proximity of those locations . catheter ablation of these arrhythmias can be challenging . the case of a 68-year - old male with frequent premature ventricular extrasystoles arising from the aortomitral continuity of the basal left ventricle is described . the electrocardiographic and electrophysiologic characteristics of this arrhythmia are discussed .

most bladder squamous cell carcinomas have been described in paraplegics or in patients with spinal trauma . they are also known to occur in patients with long - term indwelling catheters and during chronic inflammatory states associated with frequent irritation and persistent infection . here we report a case study of a patient who developed a squamous cell carcinoma involving the bladder mucosa from a well - healed suprapubic cystostomy scar after 9 years . a 56-year - old male patient who had a suprapubic foley catheter for 9 years after the formation of an urethrocutaneous fistula presented with a spontaneous suprapubic urinary leak from a well - healed suprapubic cystostomy tract scar . he originally had a urethral stricture that required multiple surgical corrections for 16 years beginning at the age of 28 years . nineteen years later ( at the age of 47 years ) , a urethrocutaneous fistula occurred spontaneously and a suprapubic cystostomy tube was eventually required for urinary diversion . on the computed tomographic ( ct ) scan of the abdomen , a tumor mass surrounding the suprapubic cystostomy tract was clearly visible ( fig . histological examination of the biopsy from the bladder mass revealed squamous cell carcinoma , moderately differentiated , probably originating from the vesicocutaneous fistula tract involving the bladder mucosa ( fig . 2 ) . the patient did not want to undergo aggressive surgical excision of the complete bladder and the abdominal wall and underwent radiation therapy . although a ct scan of the abdomen after radiation therapy showed partial remission ( fig . 3 ) , the patient died of lung metastasis and pneumonia at 6 months after the start of radiation therapy . squamous cell carcinoma is a rare form of carcinoma of the bladder that accounts for only 5% of such carcinomas . it is usually caused by chronic irritation from urinary calculi , long - term indwelling catheters , chronic urinary infections , or bladder diverticula . as many as 80% of paraplegics with chronic infections or indwelling catheters experience squamous changes in the bladder , and about 5% develop squamous cell carcinoma . cigarette smoking is also significantly associated with an increased risk of bladder squamous cell carcinoma . male predominance is far less striking in squamous cell carcinoma ( male to female ratio , 1.3:1 to 1.7:1 ) . there are only four case reports of suprapubic catheter tract squamous cell carcinoma ( two of them involving the bladder ) in the english literature [ 5 - 8 ] . the case reported here is the third case of suprapubic catheter tract squamous cell carcinoma extending into the bladder . we present the published cases of suprapubic catheter tract squamous cell carcinoma in table 1 . the prolonged presence of a tube with continuous irritation of the tract site leading to metaplasia and carcinoma provides a possible mechanism for carcinoma . in the literature , a 10% incidence of squamous cell carcinoma of the bladder has been noted in patients who have had indwelling catheters for more than 10 years . . found that spinal cord injury patients who have had indwelling catheters for more than 10 years have a 20% prevalence of squamous cell carcinoma of the bladder , and those authors advocated for periodic cystoscopies combined with random bladder biopsies for this category of patients . because this type of cancer is extremely rare , insufficient experience exists to postulate the etiology in cases that present several years later in a well - healed suprapubic catheter tract site . persistent discharge is often attributed to recurrence of stricture , for which cystostomies are frequently performed , leading to missed malignancies . these lesions are usually localized and are best treated by radical excisional surgery because of the rapid death of conservatively treated patients . bladder involvement generally dictates surgical management because long - term survival benefits resulting from radiotherapy or chemotherapy have not been reported to date . however , the patient in our study chose not to undergo planned aggressive surgical excision of the complete bladder and the abdominal wall and was instead treated with radiation therapy . he died 6 months after the start of radiation therapy owing to lung metastasis and pneumonia . several reports suggest that the stage - by - stage prognosis of squamous cell carcinoma is comparable to that of transitional cell carcinoma . the prognosis of suprapubic catheter tract squamous cell carcinoma is comparatively poor because most patients have advanced disease at the time of diagnosis . in conclusion , persistent discharge from a well - healed suprapubic catheter scar site several years after a cystostomy should be grounds for suspicion of this rare clinical entity , and an excisional biopsy should always be performed , especially when no stricture or bladder pathology is detectable on cystoscopy . this case study underscores the need for close monitoring of patients with any type of long - term indwelling catheter .

herein we report a case of a squamous cell carcinoma of a well - healed suprapubic cystostomy tract scar involving the bladder mucosa in a 56-year - old man . he presented with a spontaneous suprapubic urinary leak from a suprapubic cystostomy tract scar . he had a history of urethral stricture and failed urethroplasty . preoperative cystoscopy suggested a bladder mass . transurethral biopsy of the bladder mass revealed a squamous cell carcinoma confined to the suprapubic cystostomy tract involving the bladder mucosa . the patient died 6 months after the start of radiation therapy after lung metastasis and pneumonia .

despite advances in plastic surgery , reconstruction of soft tissue defects with exposed tendons on the hand and foot remains a challenge . there are several reconstruction options , including skin grafts , local flaps , distant flaps and free flaps.1 , 2 , 3 however , the experience over the past 20 years has demonstrated that tissue reconstruction with flap would lead to adhesion of tendon and functional impairment at the recipient site.4 , 5 , 6 the posterior rectus sheath - peritoneal free flap , developed by salgado et al . they proposed that the peritoneum interposition might reduce adhesion formation in surgery of exposed extensor tendons of the hand and foot . traditionally , the peritoneal free flap was retrieved by making a paramedian incision under direct vision . considering underlying tendon excursion this flap was supplied by the peritoneal branches of the deep inferior epigastric artery . here , a 30-year - old woman was involved in a machine accident , with extensive soft tissue defects . after debridement and repair of tendons , the patient received vacuum sealing drainage ( vsd ) treatment . extensor tendons , metacarpal bones and carpus were exposed ( fig . 1 ) . the existing defect of the dorsal hand was 17 cm 12 cm . the peritoneal flap measuring 22 cm 15 cm retrieved by laparoscopic surgery was transplanted to cover the defect ( fig . 2 , fig . 3 ) . the peritoneal flap survived two weeks postoperatively and no postoperative complications were observed ( fig . the remaining wound received a skin graft and the abdominal wall demonstrated a satisfying appearance ( fig . the function of the injured hand recovered partially within the first year follow - up ( fig . after radical debridement , repair of extensor tendons and vsd treatment , the size of the defect measured 17 cm 12 cm ( fig . 1 ) . by using the laparoscopic system , pneumoperitoneum was created through an optiview trocar placed in the subumbilical position . a laparoscope was introduced through this port and two other trocars were placed in the right and left lower abdominal wall . the bilateral pedicles of the flap were divided through laparoscopic procedures using the ultrasonic - activated scissors . the bilateral deep inferior epigastric artery was carefully isolated , double clipped and then transected at its origin to maximize pedicle length and diameter . the peritoneal free flap was transplanted to cover the defect with exposed tendons , metacarpal bones and carpus . the deep inferior epigastric artery and its accompanying veins , as pedicles of the peritoneal free flap , were anastomosed to the radial artery and cephalic vein . after survival of the peritoneal flap , the skin defect was covered by a split - thickness skin graft . reconstruction of extensive soft tissue defects accompanied by exposed tendons and/or skeleton on the hand and foot continues to be a challenging issue for plastic surgeons . the skin grafts , local flaps , distant flaps and free flaps have become the most popular procedures to cover soft tissue defects . these above mentioned treatment regimens will inevitably involve severe tendon adhesion.2 , 5 , 8 although various biochemical and physical methods have been adopted to decrease adhesion formation , most of the results are unsatisfactory.9 , 10 , 11 a thin flap that easily supports a skin graft and provides a gliding surface for tendon coverage represents an ideal option . the peritoneum acts as a physiological barrier in prevention of abdominal adhesion , and thus , a similar mechanism may apply to tendon coverage . salgado et al firstly introduced the posterior rectus sheath - peritoneal free flap for reconstruction of hand and foot . the totally laparoscopic peritoneal flap with bilateral pedicles allows a larger size , across median line . a more cosmetically acceptable result was obtained in both the donor site and the recipient site . creating one - stage reconstruction without following skin grafting might be achieved by introducing the concept of a chimeric flap , which can resurface the exposed tendons and provide cutaneous wound coverage simultaneously . in conclusion , the totally laparoscopic peritoneal free flap is a good choice for reconstruction of the hand and foot with exposed tendons . however , larger clinical trials and long - term follow - up are required to support the recommendation of this flap . this work was supported by grants from the foundation of science & technology department of zhejiang province , p.r.china ( no . 2012c13020 - 3 ) and from the foundation of health & family planning commission of zhejiang province , p.r.china ( no .

management of defects on the hand and foot with exposed tendons remains a major challenge for plastic surgeons . here , we present a case of hand reconstruction with a totally laparoscopic peritoneal flap . the anterior rectus sheath was preserved in situ . the peritoneal free flap supplied by peritoneal branches of the deep inferior epigastric artery was retrieved by laparoscopy to cover the soft tissue defect of the hand . the defect of the dorsal hand was 17 cm 12 cm . the peritoneal flap measuring 22 cm 15 cm survived completely without any complications . a following split - thickness skin graft offered the successful wound closure . motor and sensory function improved gradually within the first year follow - up . the totally laparoscopic peritoneal free flap is a good choice for reconstruction of the soft tissue defects accompanied by exposed tendons on the hand and foot .

a 52-year - old male patient presented at our hospital with progressive unilateral proptosis since 2 years ago and vision loss in his left eye . ocular examination revealed 2 + relative afferent pupillary defect in the left eye . computed tomography scan of the patient showed an intraconal mass sticking to superior ophthalmic fissure [ fig . 1 ] . the procedure was done without complication , and the early postoperative vision was fine but about an hour later , patient developed sudden vision loss and became no light perception ( nlp ) . for ruling out postoperation hemorrhage , fundus examination with indirect ophthalmoscopy revealed typical view of crao with the presence of cherry - red spot on the macula , white ground - glass appearance of the retina , and optic disc edema [ fig . 2 ] . for decreasing intraocular pressure and establishing retinal reperfusion , immediate ocular massage and anterior chamber paracentesis as well as systemic therapy with mannitol were done . two hours later , it was improved to 1 m counting finger , but he complained a large central scotoma . his va improved to 20/200 the day after the surgery , and he complained of diplopia . orbital computed tomography - scan of the patient shows well - defined dumble shaped intraconal orbital mass that pushed the optic nerve superomedially and extending to the supra orbital fissure fundus photograph 3 days after the surgery showing cherry - red spot on the macula and white ground - glass appearance of the retina and also optic disc edema medical workup did not show any evidence of collagen vascular or cardiovascular , hematologic , and neurologic disorders . systemic investigations including fasting blood sugar , lipid profile , and homocysteine levels all were normal . crao is usually caused by a thrombus or embolus that resulting to reduce blood perfusion of the retina . it clinically presents as sudden painless acute unilateral or bilateral vision loss in the range of counting fingers to nlp . the rate of spontaneously recanalization of the artery is about 15% with timely intervention , the prognosis is very poor as only 61% of patients can regain a final va of 6/120 or less . one of the typical findings in crao is cherry - red spot that is found in about 90% of cases . there is a golden time of 90120 min after occlusion to perform interventions to improve vision , however there is no approved modality to be effective in the treatment of crao , but some methods such as immediate ocular massage and anterior chamber paracentesis , use of drugs such as intravenous acetazolamide and mannitol or inhalation of a mixture of 95% oxygen and 5% carbon dioxide ( carbogen ) , all aimed to reduce intraocular pressure and improving blood flow to the eye . without doing these modalities , < 10% of patients can recover meaningful vision . multiple theories for crao during surgical procedures have been proposed ; a prolonged hypotensive status and reduction in blood flow or also increasing intraocular pressure including ocular compression during certain ocular surgical procedures associated with ocular ischemia , causing ischemia of the retina and may lead to crao and vision loss after surgery . although we did not face any significant drop in blood pressure ( bp ) during intervention but regarding differences in populations may be our patient was very sensitive to even small drop in bp that could lead to crao . in another theory , krner - stiefbold explains that retrobulbar masses such as hematoma , neoplasms , and retrobulbar injections may compress central retinal artery and lead to arterial occlusion and decrease blood perfusion causing crao . a more likely explanation in that ocular vascular damage may be happened by surgical maneuvers , and it will results in activation and aggregation of platelets and activated platelets release serotonin ( 5-hydroxytryptamine ) . serotonin is a vasoconstrictor and inducing transient arterial spasm , causing transient or complete arterial occlusion leading to ischemia of retina and creating crao . we recommend that in all patients with orbital tumors that undergo tumor removal , the ocular exam should be performed immediately , and every 13 h after the surgery to rule out crao , as delay in diagnosis and intervention could eventuate to severe vision loss .

a 52-year - old male patient presented at our hospital with unilateral proptosis and vision loss in his left eye . imaging evaluations showed orbital tumor , so the patient underwent surgery . about an hour later after tumor removal , patient developed sudden vision loss and became no light perception . fundus evaluation revealed central retinal artery occlusion ( crao ) . the patient was treated immediately with ocular massage and anterior chamber paracentesis as well as systemic therapy with mannitol and intravenous administration of acetazolamide . after thirty minutes , he recovered perception to light and then hand motion and 2 h later , it was improved to 1 m counting finger . crao following orbital tumor has not been reported before . we recommend ocular examination in all patients that undergo orbital surgery immediately to 23 h after surgery .

basilar artery occlusion ( bao ) has been associated with a high morbidity and mortality , compromising posterior cerebral blood flow to the occipital lobes , medial temporal lobes , the thalami , the cerebellum , and the brain stem . clinical recovery is virtually impossible in the absence of recanalization . a medical emergency , bao calls for aggressive therapies to address clot dissolution , via intra - arterial thrombolysis ( iat ) , intravenous thrombolysis ( ivt ) , and/or endovascular removal . we report a case in which recanalization on mra occurs in the absence of aggressive therapy . a 58-year old patient with global left ventricular hypokinesis and wall motion abnormalities presented with acute ataxia , hemiparesis , and decreasing alertness . initial mri revealed critically decreased basilar arterial flow likely secondary to a tight stenosis versus complete occlusion of the vessel ( figure 1 ) , and multiple associated strokes in the posterior circulation distribution ( figure 2 ) . intervention with intra - arterial clot lysis and thrombolytic therapy were initially considered but the patient and family declined an interventional approach , thrombolytic therapy , or angiography . the patient was subsequently admitted to the icu , kept flat under close observation for several days , received unfractionated iv heparin , and subsequently was administered warfarin over multiple days . the patient had a follow - up mri / mra approximately 3.5 months later illustrating no new strokes and complete recanalization of the basilar artery ( figure 3 ) . he remains on warfarin , is ambulatory , lives independently , and has only a mild residual hemiparesis and ataxia . figure 1magnetic resonance angiography at onset of symptoms revealed basilar stenosis persistent with basilar artery occlusion ( arrow ) . magnetic resonance angiography at onset of symptoms revealed basilar stenosis persistent with basilar artery occlusion ( arrow ) . figure 2at the time of presentation , t2 weighted imaging showed infarcts in the cerebellum ( a ) and axial flair imaging showed infarcts in the brainstem ( b ) . at the time of presentation , t2 weighted imaging showed infarcts in the cerebellum ( a ) and axial flair imaging showed infarcts in the brainstem ( b ) . figure 3repeat magnetic resonance angiography 3.5 months after symptom onset revealed a complete recanalization of the previously occluded basilar artery on mra ( arrow ) . repeat magnetic resonance angiography 3.5 months after symptom onset revealed a complete recanalization of the previously occluded basilar artery on mra ( arrow ) . bao can have morbid outcomes , and mortality rate has been reportedly 8598% even with fibrinolytic , and/or endovascular therapy , if not recanalized . morbidities with thrombolysis include extracerebral hemorrhage , asymptomatic or symptomatic acute intracerebral hemorrhage , and neurological damage , most concerning of which is the locked - in state may occur if these therapies are not considered and bilateral infarction of the brainstem occurs . the outcome of acute bao depends on the length and location of occlusion , degree of recanalization , and time between onset of symptoms and treatment . hospital based factors are also important , in that there are a limited number of centers with interventional neuroradiologists capable of endovascular therapy . various studies have been carried out comparing the efficacy of iat versus ivt therapy , and/or endovascular management in the setting of bao , all resulting in remarkable rates of recanalization . the aggressive treatment options are associated with a significant improvement in occlusion ; ivt or iat results in about 4065% recanalization with 2224% favorable outcome . the lower improvement in morbidity and the serious risks of these therapies may be grounds for further investigation into conservative management via anti - coagulants potentially resulting in spontaneous recanalization . our patient s decision to elect medical therapy may be supported by the data of the basics study which failed to show any special benefit to ia therapy over iv therapies , with more aggressive therapies entailing greater risks . early spontaneous recanalization has been reported in the anterior cerebral circulation , whereas late spontaneous recanalization has been documented only in the extracerebral circulation . the mr angiography changes in our patient illustrate that the use of conservative treatment with unfractionated heparin in an acute setting of basilar thrombosis was associated with complete recanalization and restoration of blood flow on mra by an unknown mechanism within 3.5 months , with a good clinical outcome . there are limited , if any , documented reports of late spontaneous recanalization of the posterior cerebral circulation similar to our case in the english language literature . reports of this nature call for further investigation into the underlying mechanism of spontaneous recanalization .

basilar artery occlusion may be associated with a poor prognosis in the absence of recanalization . choices in aggressive treatment for this potentially fatal condition vary from intra - arterial or intravenous thrombolysis , endovascular removal , or a combination of the two , with adjunct anti - coagulation therapy . these therapies have proven to be effective in recanalization , whereas conservative management with anti - coagulants alone has had more limited success in the literature . we report a case of basilar artery occlusion managed conservatively with unfractionated heparin , resulting in complete recanalization 3.5 months after symptom onset . conservative management of basilar artery occlusion with unfractionated heparin was associated with complete recanalization long after symptom onset .

acute abdomen due to spontaneous rupture of an intra - abdominal vessel is an extremely rare clinical condition particularly in otherwise healthy subjects . we report a case of acute abdomen in a young man due to spontaneous rupture of the posterior gastric artery . a 33-year - old man came to emergency department with severe upper abdominal pain of 45 h duration . he had self - induced vomiting by using his finger to trigger his gag reflex . following this there was no history of trauma or any medical disease , and he was not on any medication . his general condition was fair with stable vital signs . his pulse was 77 beats per minute with a blood pressure of 110/70 mm hg . he was afebrile with a respiratory rate of 22 breaths per minute . on abdominal examination , there was no gross distension but there was generalized guarding , tenderness and rebound tenderness consistent with acute peritonitis . his chest x - ray showed no free gas under the domes of diaphragm and the abdominal x - ray was unremarkable with a non - specific bowel gas pattern . 12.6 g / dl , platelet 239 10 l , glucose routine bloods were normal , apart from a low potassium ( 3.1 mmol / dl ) . an ultrasound showed free fluid in his peritoneal cavity and computerized tomography ( ct ) scan of abdomen with oral and intravenous contrast reported a large well - defined soft tissue mass of about 10 cm 12 cm posterior to the stomach , anterior and above the body and tail of pancreas with loss of fat plane from upper surface of body without enhancement or any blush ( fig . repeat laboratory investigations were unremarkable without any rising trend of amylase . in view of the clinical findings strongly suggestive of acute peritonitis and the suspicion of ruptured pancreatic mass after ct his peritoneal cavity was full of free and clotted blood in the paracolic gutter , pelvis , perihepatic and perisplenic areas . on opening the lesser sac a large blood clot was found in the same region as suggested by ct abdomen . after clearing the clot a spurting vessel was seen which was found to be a bleeding posterior gastric artery ( fig . 2 ) , controlled by ligation . there was no visible or palpable abnormality in his pancreas , spleen or posterior part of stomach . after control of the bleeding , the entire peritoneal cavity explored which appeared to be normal . hemorrhage within the peritoneal cavity in itself is not uncommon , but intra - abdominal hemorrhage from a non - traumatic and non - iatrogenic cause is extremely rare . this condition has been named abdominal apoplexy or more recently , idiopathic spontaneous intraperitoneal hemorrhage ( isih ) . frederick p. ross in a case report published in 1950 has stressed that this condition is a rare occurrence and that reporting of cases is warranted . common causes of spontaneous abdominal hemorrhage are visceral ( rupture of spleen , liver or kidney ) , gynecologic ( rupture of ectopic pregnancy , or ovarian follicle ) , coagulopathy related conditions and vascular conditions . examples of spontaneously ruptured vessels which have been reported include branch of middle colic artery , short gastric artery , and hepatic artery . the etiology of spontaneous rupture of vessels may be related to hypertension , arteriosclerosis , splanchnic artery aneurysm mainly of splenic and hepatic artery and the erosion of a vessel by an adjacent neoplastic or inflammatory process . in our patient , this led to the development of acute abdomen with abrupt onset of severe abdominal pain progressing to peritonitis , which finally turned out to be due to rupture of posterior gastric artery . reports are available in the literature in which violent vomiting led to rupture of arteries supplying the stomach . there is no pathognomonic sign or symptom of abdominal apoplexy , however , cushman and kilgore have described the syndrome and believe that it can help in diagnosis . signs and symptoms of spontaneous abdominal hemorrhage depend upon the site , volume and rate of bleeding . usually it presents with sudden onset of severe abdominal pain , distension , signs of generalized peritoneal irritation and an acute drop of hematocrit level . the most important aid in the diagnosis is to maintain a high index of suspicion in ambiguous cases of acute abdomen . ultrasound abdomen can detect free fluid in the abdominal cavity and is particularly helpful in unstable cases . the availability of computerized tomography can be used to confirm the diagnosis with more accuracy in equivocal cases . the primary aim of the treatment in cases of internal bleeding is to control the hemorrhage by ligating or repairing the involved vessel . in some clinically suspected cases of abdominal apoplexy if the patient is stable with more gradual onset , ct and visceral angiography can diagnose and localize bleeding vessel preoperatively . in such clinical setting , embolization may be a useful alternative to open operation . the most important strategy in management of equivocal cases is early surgical exploration to establish the diagnosis and treat accordingly . mohammad ali al qarni : management decision , responsibilities , share in making the article as reviewed and history & examination of management details ; sarwer jawaid : wrote the references and the article design .

introductionspontaneous hemoperitoneum in healthy males is an extremely rare , life threatening emergency condition with high mortality and morbidity , if not diagnosed and managed early.presentation of casewe present a 33-year - old male who presented with hemoperitoneum following self - induced vomiting.discussionto suspect and diagnose the condition is a challenge for clinician if ambiguity in presentation prevails.conclusionthe most important strategy in management of equivocal cases is early surgical exploration to establish the diagnosis and treat accordingly .

tuberous sclerosis ( tsc ) is a multi system genetic disorder affecting skin , brain / nervous system , kidneys , heart and lung and leading to growth of non malignant tumors . a combination of symptoms may include seizures , developmental delay , behavioral problems , skin abnormalities , lung and kidney disease . tuberous sclerosis lesions infrequently cause symptoms in the endocrine , gastrointestinal ( gi ) , and lymphatic systems . a 26-year - old female presented with galactorrhea for the past 10 days and menstrual irregularities over the past six months . she denied any history of substance abuse , drug intake ( antipsychotic , anti emetic , hormonal preparations ) , hypothyroidism , chronic liver or kidney diseases , epilepsy . she was a well nourished female with mild pallor , tiny nodule on face , subungual fibroma in hands . serum prolactin was 85 ng / ml with fsh-4.66 and lh- 4.21 miu / ml . in view of the theoretical possibility of involvement of cell groups of other anterior pituitary lineage , tests for evaluation of other anterior pituitary hormones were carried out and were found to be normal . abdominal and pelvic ultrasound revealed no abnormality . computed tomography ( ct ) scan revealed multiple intracerebral calcifications . these calcified lesions / subependymal hamartomas are seen along the lateral surface of the lateral ventricles giving rise to characteristic candle dripping appearance . magnetic resonance imaging ( mri ) of the brain ruled out the presence of any pituitary mass . the combined clinical scenario along with the radiologic findings leads to the diagnosis of tsc with hyperprolactinemia . patient was prescribed cabergoline 0.5 mg twice daily , which resulted in amelioration of galactorrhea and regularization of menses . there is a growing body of evidence that tsc patients develop hormone secreting tumors involving the neuroendocrine system at higher frequency than the general population . cushing 's disease , hypoglycemia secondary to insulinomas , precocious puberty , thyrotoxicosis , hypercalcemia secondary to parathyroid adenomas , hyperprolactinemia and acromegaly have all been reported in tsc patients . the circulating prolactin of our patient may be of pituitary origin or may possibly be secreted ectopically by a hamartoma . there has been evidence linking neuroendocrine tumors to the akt / mtor / s6 kinase pathway that is regulated by the hamartin / tuberin ( tsc1/tsc2 ) complex . thus , it is beginning to appear that the occurrence of these neuroendocrine tumors ( net ) in tsc is more than random coincidence .

tuberous sclerosis is a multi system genetic disorder affecting skin , central nervous system , kidney , heart and lungs . we present a case report of a 26 year old female with tuberous sclerosis who presented with galactorrhea and menstrual irregularities due to hyperprolactinemia .

the " super scan " of bone scintigraphy is defined as a bone image that " looks too good . " its characteristics include intense activity in the bones and diminished renal parenchymal activity ( 1 ) . ( 2 ) had reported the case of f - fluorodeoxyglucose positron emission tomography ( fdg pet ) super scan involving skeletons , similar to the super scan of bone scintigraphy . a " hepatic super scan " by fdg pet has been reported in a patient with hodgkin disease who had unusually elevated hepatic radioactivity , in addition to the multiple areas of focal uptake in the chest , mediastinal , left axillary , and abdominal lymph nodes , and the adrenal glands . here we report a case of f - fdg pet / ct super scan showing extensive hypermetabolic lesions throughout the skeletons and liver . contrary to the intense hypermetabolism of skeletons and liver , the skeletal muscles of limbs , mediastinum , bowel , and especially brain showed very low fdg uptake . subsequent diagnostic ct imaging of chest and abdomen revealed 3.8 cm sized mass in left lower lung with multiple lymph nodes and hepatic metastasis . excision biopsy of supraclavicular lymph node was performed and histopathologic diagnosis was small cell lung cancer . the patient 's mental status was clear and he underwent f - fdg pet / ct ( biograph sensation 16 , siemens medical systems , usa ) for staging of lung malignancy , 1 hr after injection of 444 mbq ( 12 mci ) f - fdg . f - fdg pet / ct demonstrated focal hypermetabolic lung mass in left lower lobe with diffuse and intense hypermetabolism throughout the skeleton and liver . interestingly , skeletal muscles of limbs , mediastinum , bowel , and especially brain showed very low fdg uptake ( fig . 1 ) . this patient had no medications could disturb cerebral glucose metabolisms , such as corticosteroids and sedatives . a super scan is well - known phenomenon which is characterized by a strikingly high bone to soft tissue ratio on skeletal scintigraphy , with a uniform symmetrical increase in bone uptake and absent renal visualization ( 4 ) . the super scan is associated with various malignancies , and most commonly in carcinomas of the prostate , stomach and breast ( 5 ) . metastasis from lung cancer rarely causes a super scan , although multiple bone metastases or bone marrow involvement frequently occur in patients with lung cancer ( 6 ) . sy et al . ( 7 ) hypothesized that the increased uptake of radiopharmaceutical by diseased bone results in reduced phosphate excretion , thereby producing faint renal images in the bone scintigraphy . first , even though this is not uncommon feature in patients with extensive metastasis , intense and diffuse hypermetabolism throughout the skeleton and liver was demonstrated . the similar findings had reported in other cases of fdg pet super scan , however , this case had shown the best contrast between metastatic organs and non - metastatic organs ( 2 , 3 , 8) . second , fdg uptakes in skeletal muscles of limbs , mediastinum , bowel , and especially brain were remarkably low and this is the unique characteristic of the present case . the faint visualization of the brain , renal cortex , and soft tissue might be the result of extraordinarily high uptake of fdg by bony and hepatic metastatic lesions . low brain uptake of fdg has been reported when using corticosteroids and sedatives , and these conditions should be taken into consideration ( 9 , 10 ) . the present patient had no medications could disturb cerebral glucose metabolisms , such as corticosteroids and sedatives . because of some characteristics in common with super scan on skeletal scintigraphy , this case could be considered as ' metabolic super scan ' .

a 50-yr - old man presented with intermittent hemoptysis and was diagnosed small cell lung cancer . 18f - fdg pet / ct for staging demonstrated extensive hypermetabolic lesions throughout the skeleton and liver . interestingly , skeletal muscles of limbs , mediastinum , bowel , and especially brain showed very low fdg uptake . because of some characteristics in common with super scan on skeletal scintigraphy , this case could be considered as ' metabolic super scan ' .

the occurrence of primary sex cord - stromal tumors at extraovarian sites is extremely rare . these tumors are predominantly granulosa cell tumors . to our knowledge , this is the first case of primary mesenteric sertoli - leydig cell tumor . a new case of a 78-year - old woman who presented with bowel obstruction is reported . three years previously , she underwent bilateral salpingo - oophorectomy and total abdominal hysterectomy for bilateral mucinous cystadenoma and vaginal prolapse . ultrasonography showed a solid mesenteric tumor ; exploratory laparotomy showed a nodular solid tumor at the mesenteric border of the distal ileum , measuring 11 8 cm . macroscopically , the tumor was nodular and well circumscribed measuring 11 8 4 cm . the cut surface was composed of pale yellow - grey soft tissue with foci of haemorrhage . microscopic examination revealed cellular lobules with nests and poorly developed tubules of sertoli cells ( figure 1 ) that showed moderate atypia and mitotic figures that average 510 per high - power field ( figure 2 ) . leydig cells are found at the periphery of the cellular lobules ( figure 3 ) . by immunohistochemistry , the neoplastic cells showed positive staining for antibodies against inhibin ( figure 4 ) and vimentin . tumor cells were negative for ema ( epithelial membrane antigen ) , calretinin , synaptophysin , chromogranin a , and cd117 ( c - kit ) . the diagnosis of sertoli - leydig cell tumor with intermediate differentiation of the mesentery was established . the patient has not received any additional therapy , yet she remains free of disease after five years . the occurrence of primary sex cord - stromal tumor at extraovarian sites is extremely rare , such that in the english literature only 13 cases have been reported [ 113 ] . eight were classified as granulosa cell tumors [ 16 , 11 , 12 ] , two as thecomas [ 7 , 8 ] , two as sex cord - stromal tumors , and one as an unclassified form of stromal sex cord tumor . the sites of origin were usually within the pelvis : six arose in the broad ligament , two in the retroperitoneum [ 2 , 12 , 13 ] , one in the fallopian tube , one in an umbilical herniae sac , one in the adrenal gland , and one in the pelvic sidewall . these previous reported cases are summarized in table 1 . to our knowledge , this is the first case of a primary mesenteric sertoli - leydig cell tumor . however , in our case , no biologic assay for estrogen content of the patient 's urine or blood was performed , because the nature of the tumor was not suspected until it was removed . the histogenesis of extraovarian sex cord - stromal tumors has been reviewed in the literature . in recent years , several investigators have claimed that the sex cords may originate from the mesonephros . a dualistic theory of both coelomic epithelium and mesonephros in the origin of the pregranulosa cells has also been proposed . accordingly , the mesonephros itself or its functional influence seems to be necessary for creating the sex cords . this is consistent with gonad formation being limited to the gonadal ridge and may explain why the sites of origin of extraovarian sex cord - stromal tumors are limited to the broad ligament , retroperitoneum , mesentery , and adrenal gland , all of which differentiate close to the mesonephros and mesonephric duct . sex cord - stromal tumors can be difficult to distinguish from several other neoplasms including undifferentiated carcinoma , gastrointestinal stromal tumors ( gist ) or metastatic melanoma . the prognosis for extraovarian sex cord - stromal tumors seems to be favorable ; however , reported cases and clinical experiences are limited .

the occurrence of primary sex cord - stromal tumors at extraovarian sites is exceedingly rare . we report a new case of sertoli - leydig cell tumor in the mesentery of a 78-year - old woman who presented with occlusive syndrome and reviewed the previously reported cases of extraovarian sex cord - stromal tumors in the english literature .

a 14-year - old female patient presented to our clinic with the symptom of hollowness that had been present in both gluteal areas for 6 months . the patient , who did not have any history of topical medication , redness , pain or itching in the hollowed area and did not describe any signs , had been receiving corticosteroid injections due to urticaria for the last 6 months . the patient did not have any particularities in her personal history , and there were no persons in her family with similar symptoms . the patient 's dermatological physical examination revealed an atrophic , oval and plaque - like lesion symmetrically localized on both thighs with a diameter of approximately 3 4 cm linearly overlaid by two flesh - colored striae with a diameter of approximately 1 4 cm . the patient 's laboratory values were normal , and the deep incisional biopsy collected from the patient was found to be consistent with acquired localized lipoatrophy ( all ) . histopathological examination revealed partly necrotic - looking adipocytes embedded among fibrous septa in the subcutaneous tissue interspersed with a few inflammatory cells . the option of spontaneous healing was taken into account , and control visits at the dermatology outpatient clinic once every 3 months were recommended for our patient . lipoatrophy is characterized by a preceding inflammatory period and consequent fat loss in the subcutaneous tissue . as for lipodystrophy , it refers to the absence of subcutaneous fat tissue without any signs of inflammation . lipoatrophy may be congenital or acquired and localized or diffused depending on the area of involvement . primary and idiopathic lipoatrophy has clinical forms such as lipoatrophia semicircularis , lipodystrophia centrifugalis abdominalis infantilis and involutional lipoatrophy . secondary lipoatrophy develops due to reasons such as pyogenic abscesses , connective tissue diseases ( lupus erythematosus , morphea , dermatomyositis or overlap disease ) , neoplasm ( t - cell lymphoma ) and iatrogenic ( subcutaneous , intramuscular and dermal injections ) factors . all frequently develops secondary to dermal or intramuscular drug injections of corticosteroids as seen with our case . additionally , all has also been reported following insulin , vasopressin , recombinant growth hormone , adrenal cortex hormone and methotrexate injections [ 2 , 3 , 4 ] . lipoatrophy that develops due to subcutaneous corticosteroid injection results in two different histopathological pictures . in the first histopathological picture , there are small , retracted , slightly acidophilic or albuminous fat tissues surrounding the septal connective tissue accompanied by sparse inflammatory cells and prominent blood vessels . in the second histopathological picture , there are small , atrophic fat tissues around vascular structures . decreased adipocyte counts and small - sized lobules all diagnosis is based on the basis of clinical signs , physical examination , including deep incisional biopsy , and anamnesis . primary localized lipoatrophy is generally self - limiting and may not require treatment . if the clinical signs persist and the patient has cosmetic concerns , liposuction and localized fat transplantation can be performed in a combined fashion .

lipoatrophy is characterized by inflammation and tissue loss in fatty tissue . this disease may be congenital or acquired , primary or secondary . secondary lipoatrophy develops with infections , collagen tissue diseases , tumors and drug injections . in this report , we present the case of a 14-year - old female patient who developed lipoatrophy following intramuscular steroid injection to both buttocks .

amyotrophic lateral sclerosis ( als ) is a devastating neurodegenerative disease which affects both motor function and extramotor systems . according to the revised el escorial criteria for the diagnosis of als , the presence of signs for the affection of both upper motor neurons ( umns ) in the primary motor cortex and lower motor neurons ( lmns ) in brainstem and spinal cord is mandatory , and the disease must be progressive . because of varied clinical phenotypes of als , it is difficult to differentiate it from other als - mimicking conditions . electromyography can be used for the detection of lmn involvement in addition to the clinical examination . on the other hand , umn signs must be visible at the clinical examination while electrophysiological transcranial motor stimulation abnormalities are not included in making the diagnosis of als according to the el escorial criteria . magnetic resonance imaging ( mri ) is the radiological investigation of choice with the recent advances of diffusion tensor imaging , presenting a promising technique for early detection of alterations in the motor cortex and pyramidal tracts . herein , we report a case of a 9-year - old male who presented to the department of neurology with complaints of progressive increase in weakness of bilateral lower limb , poor grip in bilateral upper limb , and difficulty in walking since last 2 years . there was no history of any muscle pain , trauma , fever , recent vaccination , dog bite , respiratory tract or gastrointestinal tract infection . physical examination demonstrated decreased power in the bilateral lower limb ( 3/5 in the right lower limb and 2/5 in the left lower limb ) and upper limb ( 4/5 in the right hand and 4/5 in the left hand ) , no signs of dermal rashes , or hypertrophy of muscles . mri of the brain was performed using 1.5 tesla scanner ( ge signa excite , usa ) . t2-weighted imaging and additional fluid attenuating inversion recovery sequence showed hyperintense signals in the posterior limb of internal capsule [ figure 1a and b ] involving corticospinal tract ( cst ) which was found to extending up to midbrain . ( a ) t1-weighted imaging , black arrow shows hyperintensity in the posterior limb of internal capsule ( b ) t2-weighted imaging , black arrow shows hyperintensity in the posterior limb of bilateral internal capsule ( c ) t2-weighted imaging , black arrow shows hyperintensity in the posterior bilateral internal capsule and midbrain ( corticospinal tract ) with classical wine glass appearance areas of abnormal signal intensity on t1- and t2-weighted images in the cst have been previously reported in patients with als . other diseases such as ischemic processes , demyelinating diseases , and infections may involve the pyramidal tracts . however , bilateral symmetrical involvement associated with the characteristic clinical findings clinches the diagnosis of als . the degeneration of motor neurons may result in a cellular loss and axonal edema as previously shown on electronic microscopy . so far , imaging methods had been of limited use because of their unsatisfactory results . approximately , 90% of als are sporadic with 10% having familial predisposition , but majority of the cases of juvenile als ( jals ) are familial . most of the jals are autosomal recessive , though autosomal dominant inheritance patterns have been described . recessive forms of jals have been mapped to chromosome regions 2q33 and 15q12 - 21 . no effective treatment for the disease have been found till date , though thyrotropin - releasing hormone , ganglioside therapy , and plasmapheresis have been tried in adults without much benefit . jals is a diagnosis that should be suspected in a child presenting with a combination of umn and lmn signs . mri facilitates reaching the correct diagnosis by showing the typical involvement of the cst and the wine glass appearance . mri plays as an important diagnostic tool for reaching up to a diagnosis and for prognosticating the disease .

amyotrophic lateral sclerosis ( als ) , also known as lou gehrig disease , is a chronic degenerative neurologic disease and is characterized by the selective involvement of the motor system . usually , patients present with upper motor neuron ( umn ) and lower motor neuron compromise . degeneration of the umn in the cerebral cortex is one of the main pathologic changes in als . these changes usually affect corticospinal tracts leading to degeneration of the fibers which show characteristic hyperintensities along the tracts leading to the wine glass sign . patients with als usually present in the sixth decade of life ; presentation in pediatric age in the form of juvenile als being rare .

the deletion of long arm of chromosome 5 ( del(5)(q32 ) ) is a very rare event in acute lymphoblastic leukemia ( all ) and has not been analyzed extensively . the del ( 5)(q ) is associated with 20 - 30% of mds , either as the sole identifiable abnormality , or in combination with one or more additional abnormalities.[14 ] although , this particular abnormality is common to myeloid lineage associated neoplasias , nevertheless , we present a case of t - all with del(5)(q32 ) as a sole chromosomal abnormality with case history and discussed with relevant literature . a 75-year - old male patient presented to the opd of bangalore institute of oncology with a one month history of intermittent fever and loss of appetite . there was no history of bleeding tendency , diabetes mellitus , hypertension and ihd . on physical examination , his abdomen , complete blood count showed hb12.5g / dl , wbc 130.46 10/l , neutrophils 2% , lymphocytes 20% , blasts 78% , rbc 4.33 10/l , platelet count 127 10/l and ldh was high with an activity of 1949u / l . immunophenotyping performed on bone marrow aspirate with a gating on ssc / fsc showed majority of the gated cells to be strongly expressing cd45 , cd5 , cd10 , cd7 , ccd3 , cd34 , and cd13 and weakly positive or negative for cd14 , cd33 , cd19 , cd3 , hla - dr , cd117 , cd8 , cd22 , cd4 , ccd22 , mpo and tdt . the scattered parameters and antigen profile as analyzed by flow cytometry ( cyan - dako ) correlated with morphology and diagnosis of t - acute lymphoblastic leukemia ( calla + ve ) the bone marrow aspirate was cultured for direct and 24hrs in rpmi-1640 medium supplemented with 20% qualified ; heat inactivated fetal bovine serum , 100 u / ml penicillin and streptomycin , without any mitogen at 37c . the cultures were exposed to colcemid ( final concentration- 0.1 g / ml ) for 30 minutes followed by hypotonic treatment ( 0.075 m kcl ) for 20 minutes at 37c and fixed in methanol : glacial acetic acid ( 3:1 ) overnight at 4c . later , air dry slides were made and incubated at 60c overnight for aging . a total of 25 metaphases were screened , captured , karyotyped and analyzed using applied spectral imaging software ( asi ) . all 25 metaphases consistently showed a karyotype of 46 , xy , del(5)(q32 ) [ figure 1 ] . del(5)(q32 ) is a most frequent and documented recurrent chromosome abnormality with favourable prognosis in mds.[13468 ] this is also reported in acute myeloid leukemia ( aml ) transformed from mds ( 1 ) . the rarity of ( 5)(q ) deletion in all and the same abnormality in our patient persuaded us to explore the literature . 1992 ) have reported three cases : del(5)(q ) in all with biphenotypic and early progenitor phenotype as sole abnormality in the first case , as an evolutionary event in another and with ph positivity in a third case . in contrast to its presence in aml , del(5 ) ( q ) in all is not an adverse prognostic indicator , and it appears to be more frequent in children . our patient is an adult and has been diagnosed as having t - all by flow cytometry and all - l2 by morphology . the patient is undergoing treatment and will be followed up to evaluate the prognostic significance of del(5)(q ) . literature review reveals , that del(5)(q ) is also reported in chronic lymphocytic leukemia ( cll ) . however , they are rare and been reported only as karyotypic results without known prognosis . ( 2010 ) describe two cll cases with del(5)(q ) not associated with adverse prognosis and not related to induced chromosome changes . this abnormality is not only reported in leukemia but also reported in small cell neuroendocrine lung carcinoma . these are difficult to estimate by conventional cytogenetic analysis , because small differences in base pairs at deletions are beyond the sensitivity of the technique . further studies are required to elucidate the prognostic value of del(5)(q ) in more all patients and to identify candidate genes that may play a vital role in the pathogenesis of all .

del(5)(q ) is a common chromosomal abnormality with favourable prognosis in myelodysplastic syndrome ( mds ) and acute myeloid leukemia ( aml ) . however , del(5)(q ) is also seen rarely in acute lymphoblastic leukemia ( all ) and its significance remains poorly understood . we present here , a case report of diagnosis of an adult 75 year old patient of all with a cytogenetic abnormality of del(5)(q32 ) . his clinical features , morphology and immunophenotyping findings were suggestive of t - all . relevant literature has been reviewed and discussed .

there are basically three variants of primary localized cutaneous amyloidosis ( plca ) : macular , papular ( lichenoid ) and nodular forms [ 1 , 2 , 3 ] . in macular and papular plca , cytokeratins serve as the amyloid precursors , and these variants are limited to the dermis [ 1 , 2 , 3 , 4 ] . in contrast , the rare type of nodular plca originates from immunoglobulin light chains ; it may extend to the subcutaneous tissue , and blood vessel infiltration can also be observed [ 1 , 2 , 3 , 5 ] . furthermore , nodular plca can originate from systemic amyloidoses or progress to systemic disease . here , we present a rare case of nodular plca , which resembled a giant tumor on the head . a 79-year - old man presented with a large tumor on the left side of his head ( fig . the waxy tumor exhibited a sulcated , mostly yellowish and reddish surface , and in some areas hemorrhages . a punch biopsy showed amorphous eosinophilic material in the dermis , so that we suspected a giant amyloid tumor [ 1 , 2 , 4 ] . histology showed that nearly the entire dermis was filled with amorphous eosinophilic material ( fig . immunohistochemistry was negative for cytokeratin , but showed that the entire dermis and parts of the subcutis were filled with amyloid consisting of both kappa and lambda immunoglobulin light chains ( fig . , we performed fine - needle abdominal fat aspiration , and the samples showed only minimal traces of amyloid , thus no signs of systemic amyloidosis . therefore , the diagnosis was primary localized cutaneous nodular light chain amyloidosis . at 1-year follow - up the diagnosis of plca requires histological analysis of a skin specimen . in order to discriminate the amyloid precursors in plca , immunohistochemistry with antibodies against cytokeratin and immunoglobulin light chains ( lambda and kappa subtypes ) it is best to also use electron microscopic work - up of a sample fixed in karnofsky 's fixative ( glutaraldehyde ) . as amyloid fibrils are quite stable , electron microscopy can also be done from formalin - fixed samples ; however , image quality will be reduced with this standard fixative . the typical amyloid fibrils with diameters of 710 nodular plca can often be successfully treated surgically , e.g. via curettage , excision or ablative laser therapy ( co2 lasers etc . ) . patients should regularly be screened for progression to systemic disease as this may occur in 550% of cases . to do so , a full history and physical examination along with an electrocardiogram , complete blood count , serum creatinine level , serum liverassociated enzymes levels , serum protein electrophoresis and urine protein electrophoresis should be preformed . it has also been suggested that an abdominal fat biopsy ( easy access for screening ) be performed to rule out systemic disease . to our knowledge , this is the most extensive case of nodular plca reported in the literature .

a 79-year - old man presented with a large tumor on the left side of his head , which had grown over 5 years . regional lymph nodes were impalpable and computed tomography revealed no signs of bone infiltration . histology showed that the entire dermis was filled with amorphous eosinophilic material . immunohistochemistry was negative for cytokeratin , but showed that the dermis and parts of the subcutis were filled with amyloid consisting of immunoglobulin light chains . there were no signs of paraproteinemia or underlying plasmocytoma . in electron microscopy , the typical amyloid fibrils were found . the tumor was completely removed via curettage . at 1-year follow - up , the patient was doing fine with no signs of relapse or systemic disease .

pterygium is an ocular lesion that is usually benign and is frequent in sunny countries . the intervention consists of the excision of the pterygium followed by the closing of the exeresis location . excimer laser , beta irradiation , and antimitotic molecules can be associated with this surgery.1 infectious complications are rare . the authors describe a case of keratomycosis that appeared after the exeresis of a pterygium . a 48-year - old patient had been referred for a red right eye , associated with an abscess of the cornea along the ablation zone of the pterygium . surgery had been performed a month beforehand and had involved the exeresis of a grade 2 recurring nasal pterygium . the patient did not leave her house , except between days 8 and 15 post surgery where she spent some time outside in her garden . on day 25 post surgery , the increase of the secretions as well as the appearance of a white area on the surgical site led the patient to consult her ophthalmologist . the symptoms persisted and the patient was referred to the service 4 weeks post surgery . there was a 6 mm high and 4 mm wide abscess of the cornea around the ablation site of the pterygium . ulceration with raised edges of about 3 mm was at the center of the abscess . the main abscess , along with several microabscesses , was located in the temporal region . a discreet proteic tyndall was noted , as well as folds of the descemet s membrane of the nasal hemicornea . there was no hypopyon or posterior synechia and no ocular hypertonia ( figure 1 ) . a clinical aspect evoking a fungal keratitis and the revealing of several septate filaments on direct examination led the authors to establish a treatment involving ticarcillin ( 7 mg / ml ) , gentamicin ( 15 mg / ml ) , vancomycin ( 50 mg / ml ) , and amphotericin b ( 0.25% ) . the antifungal treatment was prescribed as follows : one instillation every 5 minutes for 1 hour , then every hour for 3 days , then eight times a day for 2 months , and then , finally , six times a day for 1 month . reinforced collyriums were replaced on day 8 by ciprofloxacin ( six times per day ) and this was maintained for 15 days . cycloplegic collyrium was prescribed for 4 weeks and artificial tears for 12 weeks . on day 10 , a fusarium dimerum was isolated on the sabouraud agar . after 15 days of treatment , the evolution was favorable and the size of both the ulceration and the abscess had progressively diminished . the abscess was replaced with a whitish stromal scar with a big vertical axis and a diameter of about 5 mm ( figure 2 ) . there was no more ulceration , but the cornea appeared slimmer within the ablation zone of the head of the pterygium . surgery of the pterygium is usually considered as having a low rate of complications of infectious origin . nevertheless , infectious complications can appear a few days or even a few years post surgery . it essentially concerns bacterial agents ; pseudomonas aeruginosa is the germ that is usually the cause . other infections by fungus are even more rare ; in a study by lin et al2 of 30 eyes infected after pterygium surgery , only one case of fungal origin ( cephalosporium ) was included . other than cases reported by moriarty et al3 ( complications appeared 15 years after radiotherapy ) and peponis et al4 ( complications appeared 3 weeks post surgery and following use of mitomycin c ) , to the authors knowledge , this is the first case of a keratomycosis due to f. dimerum reported that complicated the exeresis of a pterygium without the use of an adjuvant anti - healing treatment . in this case , the evolution of the first observation was good , contrary to the second observation when the extension of the infection beyond the ocular structures led to the realization of enucleation . like pterygium , keratomycosis is more frequent in tropical countries.5 the breaking of the corneal epithelium following surgery encouraged the appearance of a mycosis ; the gardening undertaken by the patient is the obvious source of a vegetal foreign body . all instances of potential risk related to foreign bodies of any nature , including vegetal , must be avoided until complete re - epithelialization of the corneal ablation site of the pterygium . although pterygium surgery is a commonly performed procedure , ophthalmologists should be aware of potential infectious complications that can have dramatic outcomes .

the authors describe a case of keratomycosis that appeared after the exeresis of a pterygium . a 48-year - old patient had been referred with a red right eye associated with an abscess of the cornea along the ablation zone of the pterygium . the surgery had been performed a month beforehand . the abscess was 6 mm high and 4 mm wide . the authors instigated a treatment that included amphotericin b ( 0.25% ) after noticing a clinical aspect evoking a fungal keratitis and finding several septate filaments on direct examination . on day 10 , a fusarium dimerum was isolated on sabouraud agar . after 15 days of treatment , the result was favorable and the size of the ulceration as well as the size of the abscess had progressively decreased . the antifungal treatment was definitively stopped at 14 weeks . infectious - related complications of the pterygium surgery are rare and are essentially caused by bacterial agents . secondary infections by fungus are rare . there have been two previous cases reported : one that appeared 15 years after radiotherapy and another that appeared at 3 weeks post surgery , consecutive to the use of mitomycin c. to the authors knowledge , this is the first case of a keratomycosis due to f. dimerum reported that complicated the exeresis of a pterygium without the use of an adjuvant antihealing treatment . pterygium surgery is a common procedure ; nevertheless , ophthalmologists need to be aware of the existence of potential infectious complications .

keratosis follicularis spinulosa decalvans ( kfsd ) is a hereditary disorder of the hair follicle which presents with scarring alopecia and follicular papules affecting the scalp and other areas of the body . it usually follows an x - linked pattern of inheritance , but sporadic and autosomal dominant cases can also be seen . our first case was an 18-year - old female who complained of progressive hair loss over the scalp since 10 years and over the eyebrows since 5 years . on examination of the scalp , she had multiple patches of cicatricial alopecia with sparse hair in the frontal , parietal , and temporal areas of the scalp . sparse hair over the eyebrows , eyelashes , axillae , and pubic area were noted [ figure 1 ] . she also had generalized dry skin and multiple follicular papules over upper and lower limbs and buttocks [ figure 2 ] . follicular papules and alopecia seen over the scalp along with sparse eyebrows and eyelashes follicular keratotic papules over dorsum of hand our second case was a 26-year - old male with decreased hair growth over scalp and body . he had no family history of similar complaints . on examination , he had diffuse alopecia of the scalp with sparse hair over eyebrows , eyelashes , and beard area . multiple keratotic papules were noted over the scalp , upper limbs , and abdomen [ figures 3 and 4 ] . sparse hair and papular lesions noted over the scalp follicular papules seen over abdomen the third case was a 23-year - old male with complaints of decreased hair over scalp and body with skin lesions over the trunk . he gave a history of similar but milder complaints being present in his younger brother suggesting x - linked inheritance . on examination , he had alopecia over the crown area of the scalp [ figure 5 ] , with sparse hair over the eyebrows and axillae , multiple follicular papules over the trunk and all four limbs . scarring alopecia seen over scalp plantar keratoderma seen over the foot scalp biopsies from all three cases showed variable amounts of acanthosis and papillomatosis and dilated hair follicles filled with keratin plugs and entrapped hair shafts [ figures 7 and 8 ] . dilated follicular infundibulum filled with keratin plug ( h and e , 40 ) entrapped hair shafts within hair follicle ( h and e , 40 ) keratosis pilaris atrophicans ( kpa ) is a group of cutaneous disorders which are characterized by follicular hyperkeratosis along with scarring . kfsd ( omim 308800 ) is a rare x - linked disorder of the hair follicle . the term was first used by siemens in 1926 when he reported the disorder in a bavarian family . the candidate gene suggested is the membrane - bound transcription factor protease site 2 ( mbtps2 ) gene which is required for cleavage of sterol regulatory element - binding proteins ( srebps ) . altered srebp cleavage impairs cholesterol and lipid homeostasis in the skin causing defective epidermal differentiation . males are more commonly and severely affected than female heterozygotes in families having an x - linked inheritance pattern . the age of onset is early in life with keratotic papules appearing initially on the face and later on the trunk and limbs . as the disease progresses , cicatricial alopecia ensues on the scalp , eyebrows , and eyelashes along with the loss of hair in axillary and pubic areas . the most important differential diagnoses include ichthyosis follicularis alopecia photophobia ( ifap ) syndrome and graham little piccardi lasseur syndrome ( glpls ) . however , the alopecia in ifap syndrome is of the nonscarring type while that in kfsd is scarring . furthermore , ocular features like photophobia and corneal dystrophy are essential for the diagnosis of ifap . glpls is a variant of lichen planopilaris which presents with scarring alopecia of the scalp , nonscarring alopecia of axillae and pubic region , and keratotic follicular spinous papules over a body . the distinction from kfsd is made mainly on histopathology which reveals changes suggestive of lichen planus including vacuolar degeneration and interface dermatitis . systemic retinoids like isotretinoin and etretinate are beneficial in the early stages of the disease as they downregulate follicular hyperkeratosis and inflammation . dapsone was found to be useful due to its inhibition of leukocyte chemotaxis and stabilization of lysosomal enzymes . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .

keratosis follicularis spinulosa decalvans is a disorder affecting the hair follicles characterized by scarring alopecia of the scalp , eyebrows , and axillae , sometimes associated with photophobia and keratoderma . being x - linked , it is more commonly seen in males but can be rarely seen in females also . we report three cases of this rare disorder including one in a female .

a 46-year - old woman presented to kyungpook national university hospital with a one - year history of chest discomfort and dyspnea , as well as a history of three operations for cardiac myxoma , including biatrial cardiac tumor resection in 1984 , followed 10 years later by the resection of a recurrent left atrial and ventricular cardiac myxoma attached to the interatrial and interventricular septa . the first surgery , in 1984 , was performed in our medical center . the myxoma and its pedicle attached to the fossa ovalis were excised via right atriotomy and left atriotomy with enclosure of the interatrial defect . the second surgery , in 1994 , the interatrial septum was nearly totally excised and the myxoid mass was identified and excised . also through aortotomy , a left ventricular myxoma on the interventricular septum below the right coronary cusp was identified and excised . sixteen years later , in 2010 , the third such operation was performed in the same center . after the dissection of severe adhesion , a myxoma and its stalk on the inferior vena cava and right atrial junction were excised through a right atriotomy . this patient also had a history of recurrent cutaneous and mucosal tumors , including forearm and nose tumors ( diagnosed as myxomas based on excisional biopsies of tumors on her right forearm and right nostril ) , breast ductal adenoma ( fibroadenomas diagnosed in her bilateral breasts through mammotome excisional biopsies ) , and a uterine myoma , as well as a basilar artery aneurysm . chest computed tomography revealed a large soft tissue mass in the right atrial and ventricular cavities and findings suspicious of pulmonary embolism ( fig . two days later , with a presumptive diagnosis of recurrent cardiac myxoma , the patient underwent cardiac surgery through a median sternotomy . aortic and venous cannulation , the latter through the superior vena cava and right femoral vein , were performed for cardiopulmonary bypass . the large gelatinous tumor was visualized after right atriotomy ; its stalk was located in the right atrium - inferior vena cava junction ( fig . moreover , the tumor involved part of the distal left pulmonary artery , leading to further pulmonary arteriotomy during surgery . postoperatively , the patient s symptoms of dyspnea and chest discomfort resolved without complications , and the patient was discharged on postoperative day 9 . after resection , cardiac myxoma , the most common benign cardiac tumor , rarely recurs , with a recurrence rate of approximately 3% observed in sporadic cases and a recurrence rate of 22% in patients with the carney complex , an uncommon autosomal dominant hereditary disorder first described by carney et al . in 1985 . the carney complex , which manifests with cardiac , endocrine , cutaneous , and neural tumors and spotty pigmentation , is diagnosed when a patient exhibits either at least two main criteria , reflecting the manifestations of the disease , or one main criterion as well as one supplemental criterion associated with familial or hereditary features ( table 1 ) . the patient presented in this report had a consistent history of cutaneous myxoma , breast ductal adenoma , and cardiac myxoma . this case of four recurrences of cardiac myxoma , the first of its kind to be reported in korea , underscores the importance of performing a detailed investigation of patients with recurrent myxoma for the possibility of the carney complex in order to facilitate better management , prognostication , and patient education regarding this condition .

although cardiac myxoma is the most commonly encountered benign cardiac tumor in cardiac surgery practice , recurrent cardiac myxoma is very rare , is most commonly related to the carney complex , and usually requires multiple cardiac operations with specific requirements in terms of perioperative management . in this report , we describe a patient who experienced the fourth recurrence of cardiac myxoma and review the diagnostic criteria of the carney complex . this is the first report of such a case in korea .

a 43-year - old woman presented with recurrent paroxysmal involuntary movements of her right upper limb and quadriparesis . she had four previous relapsing and progressive episodes presenting as right upper limb monoparesis , quadriparesis , and paresthesias during three years . the previous weakness resolved incompletely with steroid treatment . on admission , her wide right forearm - flinging movements , which were occasionally provoked by left knee flexion , lying on the bed , the attacks involved flexion of the right elbow , repeated flexion and extension of the ipsilateral wrist . her only medication was baclofen , which she had been taking for the last three years . she had a quadriparesis ( grade 34 , medical research council ) , worse on the right limbs and distal muscles . rough touch , pain , and temperature sensation were slightly decreased on all extremities , but there were no deep sensory abnormalities . laboratory studies including routine chemistries , various serological markers related with connective tissue diseases , thyroid function tests , and serum and urine copper levels were all normal . csf examination showed mild elevation of protein ( 80.5 mg / dl ) and igg index ( 0.70 ) , but oligoclonal bands and nmo - igg antibodies were negative . median and posterior tibial nerve somatosensory evoked potentials showed no n13 , n20 and p37 . mri revealed diffuse swelling and heterogeneous enhancement of spinal cord from the cervicomedullary junction to c7 ( figure 1 ) . six months after the disappearance of involuntary movement , she developed decreased visual acuity with prolonged latencies of p100 components in visual evoked potentials . the occurrence of ballism - chorea and dystonia implies development of lesions involving the basal ganglia and their afferent or efferent connections , such as thalamus , subthalamic nucleus , basal ganglia , and putamen . however , the unique feature of this patient is choreic movement without responsible brain lesions . ballism has never been reported in spinal cord lesions whereas chorea has been seen very rarely.4 it was uncertain whether she exhibited a pure ballism or chorea but presumably it is not important . while ballism and chorea are distinguishable based upon the type and distribution of movements , they may represent two different symptoms or a spectrum of the same basic disease processes.5 why a patient with a spinal cord lesion develops chorea but not ballism is unknown . ballism , a coarse proximal movement , could evolve into more distal and lower amplitude chorea . athetosis , which is considered a slow form of chorea , often evolves further into dystonia.5 why movement disorders occur in only a minority of patients with myelopathy is also unknown . one mechanism involves the posterolateral cervical cord , the projections from supraspinal centers on the ia inhibitory neurons , and other interneurons concerned with reciprocal inhibition.13 disruption of the somatosensory pathways or motor cortex to the striatum also may produce abnormal movement without sensory loss.1 for our purposes , considering paroxysmal chorea and dystonia together as involuntary movements , we can infer the effect of provocative movements in this patient from the results of previous studies . paroxysmal chorea could be easily provoked by movement , either passive or volitional , and by tactile stimulation.4 it often crosses to the contralateral trunk or limbs . the so called trigger zone was found at either the same spinal segment or below the clinically estimated segment . although this patient had relapsing - remitting weakness , it did not fulfill the conditions of definite nmo when the involuntary movements occurred . however , she must have had nmo , considering the visual symptoms afterwards . despite the lack of lesions located appropriately for the involuntary movements , however , we could not evaluate the effectiveness of such therapy because our patient was treated with steroid and clonazepam . our case suggests that ballism - chorea could present in a spinal cord lesion without involvement of striatum and deep sensory abnormalities . thus , acute exacerbation of recurrent myelopathy in nmo may be associated with transient movement disorders .

movement disorders secondary to intrinsic spinal cord disease are rare . paroxysmal chorea has not yet been reported in the neuromyelitis optica ( nmo ) . we report a 43-year - old woman with relapsing - remitting cervical myelopathy who developed paroxysmal chorea during clinical exacerbation of nmo . mri scan of the cervical spine revealed a long segmental enhancing lesion , but brain mri did not show any responsible abnormalities . acute exacerbation of recurrent myelopathy in nmo may be associated with transient movement disorder .

the development of techniques for phalloplasty has paralleled the evolution of flap development in reconstructive surgery itself . gillies and harrison , in 1948 , were amongst the first to take up the challenge . the aims of penile reconstruction are to enable urination in the standing position , to enable sexual intercourse and to reconstruct an aesthetically acceptable penis with adequate tactile , protective and ideally erogenous sensation . construction of a phallus with the above criteria has always been a formidable surgical challenge and many problems have not been solved satisfactorily , even with the introduction of free flap transfer . a 21-year - old male sustained severe electrical burns on lower abdomen , genitalia , lower and upper limbs following accidental exposure to high tension electricity ( 12,000 v ) . the lesions covered 25% of his total body surface area with 20% third degree burns and 5% second degree burns . he presented with gangrene of right upper limb in its entirety , carbonization of external genitalia with subsequent complete loss of penis and left hemi scrotum and testis [ figure 1 ] . the evolution of his general condition was very difficult as a result of haemodynamic and infection problems , which required intensive care . locally , debridements of necrotic tissue were immediately performed , followed by a tensor fascia lata flap to protect the left femoral vessels and a local transposition flap to protect the brachial vessels in the left forearm . ( b ) penile gangrene disarticulation of right upper limb at the shoulder level done with primary closure of the defect . the right hemiscrotum with testis was mobilized to cover the defect in the genital region with a perineal urethrostomy . penis was planned to be reconstructed in stages . in the first stage , the right paragenital non - hairy skin was tubularized to create a neourethra from the perineal urethrostomy site to pubic region [ figure 2 ] . six weeks later staged penile reconstruction proceeded using a right - side groin flap . with non - availability of other adjacent tissues and with the loss of right upper limb and entire left forearm being grafted with a transposition flap to protect brachial vessels , groin flap remained the option for reconstruction . a tube within a tube was created from a single folded , 17 cm wide and 16 cm long groin flap [ figure 3 ] . a 4 cm wide area along the midline of flap , without the presence of hair was tubed inward around a 16 french foley catheter to form the neourethra . the adjacent skin of about 1 cm on either side was deepithelialized to allow the flap to be rolled onto itself . the remaining 11 cm width of skin was wrapped around the inner tube portion to provide external cover . with the catheter introduced onto the pubic urethra , urethral repair and skin closure with a tumble of the groin flap done [ figure 4 ] . about 3 weeks later , an axial delay of the flap done followed by flap division in another week 's time . the flap provided a satisfactory aesthetic result and enabled urination in standing position after catheter removal at 15th day after flap division [ figure 5 ] . advancements in microsurgical techniques have expanded the number of potential penile reconstructive procedures , and there is general agreement that they represent the method of choice for penile reconstruction . in phalloplasty , in female - to - male transsexuals and penile reconstruction in male patients following loss in trauma or malignancy , its main disadvantages of too hairy skin for urethral reconstruction and donor site morbidity can not be ignored . in this very complex surgical case , with loss of entire right upper limb and left forearm being grafted in its entirety and with the loss of majority of adjacent tissues , groin flap is probably the choice . however , the major difficulty was the staged nature of procedure . the patient had a small fistula at the penoscrotal junction in the dorsal aspect which healed by conservative management . after an 18 month follow - up , the patient was very pleased with the appearance of penis and his wish of male pattern urination being fulfilled [ figure 6 ] . neither author has any financial conflict of interest with any of the content discussed in this article .

plastic surgery continues to maintain a prominent presence in the evolution of male genital reconstruction . in this case report , we are presenting a case of post - electric burn with a total loss of penis . sustaining other major injuries following an electric burn with loss of right upper limb and extensive tissue damage to left upper limb , abdomen and both thighs , this young male patient was initially managed from life - threatening problems . with many options closed following a major electric burn and its acute management , penile and urethral reconstruction was a unique and a great challenge in this patient . heeding to the patient 's wish of male pattern micturition , we had performed a successful reconstruction of urethra and entire phallus with groin flap .

lung parenchymal calcifications are uncommon , but may occur in numerous conditions so their main differential diagnosis should be known by the radiologist . we demonstrate a rare etiology for lung calcifications in a 87 year - old male that came to our emergency department complaining of dyspnea and was diagnosed with diffuse pulmonary ossification secondary to a long - standing mitral stenosis . radiographic and ct findindings of the patient are described and the most important imaging features of this pathology are here highlighted . a 87 year - old male was admitted to the emergency department of our hospital referring progressively increased dyspnea over the last two days , together with anorexia in the last months . he had a history of long known moderate to severe mitral stenosis , but due to his age was not considered for valvuloplasty . the chest radiography done at arrival ( fig.1 ) revealed the presence of bilateral micronodular opacities that were more prominent on the right lung , where they were associated with coarse calcifications . , there were exuberant confluent microcalcifications , more numerous in the lung bases . despite being bilateral , they were rather assymetric , being more conspicuous on the right side and rare on the left side . a diagnosis of diffuse pulmonary ossification secondary to mitral stenosis was suggested based on both the imaging and clinical findings . despite the medical therapy instituted , dyspnea of the patient increased over the following days , leading to his death from cardio - respiratory arrest two weeks after this exam . diffuse pulmonary ossification ( dpo ) is a rare diagnosis defined by the deposition of mature heterotopic bone in the lung parenchyma . it either can be idiopathic or secondary , most commonly due to pulmonary or cardiac pathologies , particularly mitral stenosis . the association of pulmonary ossification and mitral stenosis was firstly described by salinger in 1932 . estimates establish it to occur in about 313% of patients with long - standing mitral stenosis at autopsies , but proper medical or surgical treatment of mitral stenosis and eventual underdiagnosis of dpo make it a rarely reported diesease in the literature . a male predominance has been reported with patients being diagnosed more commonly between the 2nd to the 4th decades of life , , although there have been reports in individuals over 60 years - old . a history of repeated insults to the lung , such as recurrent pneumonia , might be present . several theories have been proposed for explaining the physiopathology of this disease , focusing on different factors : pulmonary congestion , connective tissue proliferation , hemosiderin deposition or recurrent small pulmonary hemorrhages . patients may be asymptomatic . when symptoms and physical signs are present , they are not different from the ones of mitral stenosis . dyspnea and fatigue are common and lower limb edema might also be present due to congestive cardiac failure . chest radiography is the first - line imaging technique and might reveal signs of enlarged left atrium , such as : cardiomegaly , double right heart border , prominent left atrium and increase of subcarinal angle . it also displays characteristic features such as micronodular calcified opacities that are asymmetrical , usually more conspicuous on the right lung and distributed unhomogenously , sparing the apices and being more numerous on lung bases . the chest ct , particularly with high - resolution algorithm , is more sensitive in the identification of these features . it may reveal enlarged left atrium and dilated pulmonary arteries , signs of pulmonary hypertension . it can also depict the small calcifications , in the same distribution seen in the chest radiograph , often concentrated near the pleura . pathologic findings include the presence of round or mulberry - shaped bone nodules that originally form in the alveoli but may then occupy interstitial air spaces . diffuse pulmonary ossification is commonly associated with mitral stenosis , with high - resolution chest ct being the most accurate imaging technique for this diagnosis . characteristic features include small calcified opacities more numerous on subpleural locations , especially on the right lung base and eventual signs of pulmonar hypertension . the authors wish to confirm that there are no known conflicts of interest associated with this publication and there has been no financial support for this work that could have influenced its outcome .

diffuse pulmonary ossification ( dpo ) is a rarely diagnosed entity that may present with characteristic imaging features . it is listed in the differential diagnosis of lung parenchymal calcifications and should be considered by the radiologist if the appropriate findings are identified . we report a case of dpo secondary to mitral stenosis in a patient whose severe cardiac pathology lead to death few weeks after a chest ct was done . to date , there are no specific treatments with proved benefit in this pathology .

we report a case of a female patient on aspirin and warfarin who developed a retropharyngeal haematoma following minor blunt head and neck trauma . an 84 year old woman had a mechanical fall at ground level resulting in neck hyperextension . a rigid cervical collar was applied and anterior neck and chest swelling and bruising were noted beneath this . a massive retropharyngeal haematoma was observed extending between c2 and t4 vertebral body levels with displacement and compression of the trachea ( figure 1,2 ) . there was no evidence of carotid , vertebral or internal jugular vein injury and no cervical fracture . contrast enhanced axial ct image taken at the level of the hyoid bone shows a large retropharyngeal haematoma ( star ) with active intravenous contrast extravasation ( arrow ) contrast enhanced sagittal ct image shows a large retropharyngeal haematoma ( star ) extending from the c2 vertebral level to below the level of the sternal notch with tracheal compression ( arrow ) nine hours from the time of injury the patient developed stridor . although significant subglottic swelling was evident at intubation , a 6.5 mm diameter endotracheal tube was successfully and atraumatically positioned into the distal trachea . a four vessel angiogram was then obtained which revealed an actively bleeding branch of the right inferior thyroid artery . the patient was managed non - operatively with intravenous antibiotics , steroids and inr reversal . over the coming days her neck swelling decreased and serial ct showed a reduction in size of the haematoma . dysphagia necessitated nasogastric feeding for a further week and mild dysphonia persisted for two weeks . the patient otherwise made a good recovery , returning to her previous level of function by six weeks . aetiological factors implicated in retropharyngeal haematoma include blunt head and neck trauma , cervical spine injury , anticoagulation , bleeding diatheses and tumors . most cases are thought to be due to bleeding from vessels covering the anterior longitudinal ligament.(2 ) less commonly bleeding has been reported to originate from the thyrocervical trunk ( 3 ) as was likely the situation in this case . the classic clinical presentation comprises capp s triad of airway compression , displacement of the trachea anteriorly and bruising of the neck and chest.(1 ) however , the clinical presentation is highly variable and may range from mild sore throat to hoarseness , dysphagia , odynophagia , dyspnoea or stridor.(15 ) an asymptomatic interval between time of injury and onset of stridor has been described(4 ) as in this case . the first step is to secure the airway if there is any evidence of airway obstruction . the haematoma may distort the anatomy of the upper airway making visualization of the cords difficult . a surgical airway is advocated by some authors as the preferred method because endosopic intubation may be difficult and carry a higher risk of perforating the haematoma.(3 ) we elected to trial an awake endoscopic intubation method with tracheostomy equipment available if required . this intubation method allowed direct visualization of the laryngeal anatomy with minimal manipulation of the haematoma whilst maintaining cervical spine alignment . with the airway secured , most patients are successfully managed conservatively with observation , supportive treatment and monitoring with serial ct . the haematoma usually resolves but can take four weeks or more.(5 ) surgical evacuation or transoral aspiration is generally reserved for a haematoma that is rapidly expanding , impeding mechanical ventilation or failing to resolve . although surgery may lead to an earlier extubation and recovery , it does carry an increased risk of infection . ( 1 ) we have presented a case of a large retropharyngeal haematoma in an elderly anticoagulated patient following minor blunt head and neck trauma . the patient presented insidiously with capp s triad and developed delayed airway obstruction necessitating emergency fibreoptic endoscopic intubation . conservative management may be appropriate in patients with non expanding haematomas that show improvement on serial ct .

retropharyngeal haematoma is a rare and potentially fatal cause of airway obstruction . the treatment of retropharyngeal haematoma is contentious . we report a case of an 84 year old woman on aspirin and warfarin who developed a retropharyngeal haematoma following minor blunt head and neck trauma . the patient presented insidiously with capp s triad and developed delayed airway obstruction necessitating emergency fibreoptic endoscopic intubation . both tracheostomy and surgical drainage were avoided and she recovered well .

the spect acquisition in lung perfusion scan provides higher sensitivity and specificity to detect pulmonary thromboemboli ( pte ) compared to planar imaging ( 1 , 2 ) . because of higher specificity , there are hopes that the lung perfusion spect could be interpreted without a ventilation study . the value of higher specificity is more highlighted in complicated cases with vascular abnormality and shunts . in this report , a case with tetralogy of fallot ( tf ) and multiple cardiovascular abnormalities and corrective shunts are presented underscoring the importance of spect imaging in complicated patients . a 22-year - old female patient was admitted for surgery to revise her waterston central aortopulmonary shunt . she had the history of tf , pulmonary valve stenosis , ventricular septal defect and major aortopulmonary collateral artery . her shunt was placed at the age of 5 years then complicated with stenosis for which a stent was placed and re - dilated 1 year ago . after echocardiography reported minimal aorto - pulmonary shunt flow , a decision was made to operate the patient for revision of the central shunt . midsternal incision was done and a polytetrafluoroethylene ( ptfe ) tube was inserted between the ascending aorta and right pulmonary branch , where the previous gortex was located . patient recovery and rehabilitation after surgery was optimal until the patient experienced abrupt left - sided pleuritic chest pain and dyspnea . the patient was referred to the department of nuclear medicine for perfusion and ventilation lung scanning . perfusion lung scan was done with 4 mci 99 m technetium labeled macroaggregated albumin ( total particle count 120,000 ) in planar anterior , posterior , lateral and multiple oblique projections ( figure 1 ) . additionally , the brain , renal parenchyma and the thyroid demonstrated uptake due to the right to left shunt ( figure 2 ) . ventilation scan was not performed due to logistic problems ; the available ventilation tracer in iran is 81kr , which is distributed only on wednesdays . there was a large wedge - shaped peripheral defect in the apicoposterior segment of the left upper lobe . the finding was interpreted as either acute / chronic pte or secondary to developmental / iatrogenic vascular abnormality . meanwhile , the patient underwent ct angiography reporting postoperative atelectatic changes in the bilateral lower lobes without evidence in support of pte . the iv heparin was changed to warfarin after 5 days when the symptoms improved gradually and the arterial o2 saturation increased to 92% in room atmosphere . tetralogy of fallot is a congenital cyanotic heart disease in about 3.5% of infants with congenital heart diseases . there are a wide spectrum of anatomic defects with varying severity including ventricular septal defect , overriding aorta , right ventricular outflow tract obstruction , and right ventricular hypertrophy ( 3 ) . diagnosis of pulmonary emboli is a great diagnostic challenge and it should be suspected in any patient with unexplained dyspnea , tachypnea , or chest pain ( 4 , 5 ) . lung perfusion and ventilation spect is superior to planar scan in detecting pulmonary embolism with a high sensitivity and specificity ( 6 ) . perfusion lung scan without ventilation scan has also been shown to have a high specificity of 92% and sensitivity of 87% ( 7 ) . the advantages of lung perfusion spect imaging include higher contrast for better detection of the shape and location of defects and higher sensitivity ( 6 ) . in complicated cases similar to the patient described here , this is of foremost importance . because pulmonary angiography was not performed and ct angiography was negative , pte was not confirmed in this case . ct angiography is a sensitive and optimal technique for diagnosing large pulmonary emboli , but it has a relatively low sensitivity ( below 80% ) for sub - segmental ptes ( 4 ) .

a 22-year - old woman presented with acute left - sided pleuritic chest pain and dyspnea 6 days after surgery for revision of the stenotic central aortopulmonary shunt . she had a history of tetralogy of fallot ( tof ) , pulmonary valve stenosis , ventricular septal defect and major aortopulmonary collateral artery . her waterston shunt was placed when she was 5 years old and stented and re - dilated after stenosis . acute pulmonary thromboemboli ( pte ) was suspected and pulmonary perfusion scan was performed with 4 mci 99 m technetium labeled macroaggregated albumin . the left lung was globally hypoperfused with evident uptake in the brain , renal parenchyma and thyroid . spect images revealed a segmental wedge - shaped peripheral defect in the posterior segment of the left upper lobe . the scan was interpreted as acute / chronic pte or vascular abnormality . ct angiography excluded pte ; nevertheless the patient was treated with a therapeutic dose of heparin changed to warfarin and was discharged with improvement of the symptoms . pulmonary artery angiography was not performed .

the primary complaint of most patients with this tumor is testicular swelling , which is usually painless . it is not uncommon for this tumor to be of an advanced stage at the time of the diagnosis , and the prognosis is dismal . although scrotal swelling is the most common manifestation , the clinical presentation is often nonspecific and may not suggest a neoplasm , such that the diagnosis can be delayed . to our knowledge , this is the first report in the published literature documenting incidental adenocarcinoma of the rete testis with preceding detection of unilateral hydronephrosis . the patient was a 54-year - old man with right flank pain that had started 1 month previously . the kidney - ureter - bladder and urinalysis findings were normal , but ultrasonography showed mild right hydronephrosis . therefore , abdominopelvic computed tomography ( ct ) was performed to determine the cause of the hydronephrosis . we found multiple enhanced lesions around the right obturator muscle that were compressing the right distal ureter and causing the hydronephrosis . additionally , a heterogeneous , low - density lesion with an irregular margin was found with hydrocele in the right testis ( fig . according to the patient 's medical history , the painless swelling of the right scrotum had started about 1 year previously with no other significant medical history . on the physical examination , a relatively movable iu / l ) and carcinoembryonic antigen ( cea , 40.8 ng / ml ) levels were higher than normal , but the alpha - fetoprotein and beta - human chorionic gonadotropin ( beta - hcg ) levels were normal . we decided to perform cystoscopy and retrograde pyelography with radical orchiectomy to rule out any metastatic lesions in the right ureter . a right ureteral stricture was found and a d - j ureteral catheter was inserted . microscopic examination showed conventional adenocarcinoma , which shows a glandular pattern of growth with atypical stratified cuboidal to columnar epithelial tumor cells ( fig . significant immunohistochemical reactivities for cea , muc1 , cytokeratin 20 , and cdx2 were seen ( fig . there was no reactivity for prostate - specific antigen ( psa ) , carbohydrate antigen 19 - 9 , thyroid transcription factor-1 , or s-100 protein . positron emission tomography - ct ( pet - ct ) , gastroduodenoscopy , colonoscopy , and transrectal ultrasound were performed in addition to measuring the serum psa level to rule out metastatic adenocarcinoma from another site . , we finally diagnosed a right testicular lesion as the primary adenocarcinoma in the rete testis . after the radical orchiectomy , the patient received combined chemotherapy 6 times , but the progression of the disease was unstoppable . during the course of the chemotherapy , pet - ct was done , which revealed newly developed lung metastasis . up to one quarter of these tumors have been reported in association with scrotal hydrocele , as in our case . the diagnosis is often difficult and made incidentally and is often delayed , because of the nonspecific clinical presentation , which can suggest only an inflammatory process . the time from the initial problem to presentation averages approximately 24 months . in an extensive review of reported cases , 54% of patients had metastases at diagnosis with lymphatic spread to the lumboaortic and iliac nodes and metastases to the bones , lungs , and skin . the common sites of early metastases are the lymph nodes , lungs , liver , and bones . the diagnostic criteria of nochomovitz and orenstein for adenocarcinoma of the rete testis include 1 ) tumor origin around the hilum , 2 ) no involvement of the tunica , 3 ) demonstration of transition from normal to tumor epithelium , and 4 ) no evidence of teratoma or any other primary tumor elsewhere in the body . our case fit 3 of these 4 diagnostic criteria : 1 ) , 2 ) , and 4 ) . a few cases have been examined immunohistochemically and are positive for cytokeratins and negative for beta - hcg , psa , and alpha - fetoprotein . positivity for cea may be seen , and it is helpful in excluding the diagnosis of mesothelioma . in our case , however , reliable markers that can define the origin of the adenocarcinoma as the rete testis have never been indicated . in advanced stages , adenocarcinoma of the rete testis is highly resistant to adjuvant radiotherapy and any known chemotherapy . the natural history of this tumor appears to be highly malignant , with 3- and 5-year disease - free survival rates of 49% and 13% , respectively .

primary adenocarcinoma of the rete testis is a rare malignant testicular tumor with a poor prognosis . here we report the case of a 54-year - old man with right hydronephrosis that was detected before the diagnosis of the primary testicular lesion . during the evaluation of the right hydronephrosis by use of abdominopelvic computed tomography , a painless , hard , solid lesion was found on the right testis . the patient underwent radical orchiectomy , and the pathologic examination revealed an adenocarcinoma of the rete testis . multiple metastases were present at the time of diagnosis . the patient received combined chemotherapy after the surgery but lived only 8 months after the initial diagnosis .

paraneoplastic pemphigus ( pnp ) is an extremely severe autoimmune blistering disease associated with neoplasms ; the disease is most commonly of lymphoproliferative origin and presents a high mortality 1 . follicular dendritic cell ( fdc ) sarcoma is rare and classified either as a conventionaltype or as an inflammatory pseudotumor ( ipt)like variant . iptlike fdc sarcoma is a rare neoplasm with different histologic appearance and behavior from those of the classical fdc sarcoma 2 . a review of 38 cases of iptlike fdc sarcoma reveals that this tumor occurs predominantly in females with a predilection for liver and spleen , with a strong association with epstein less than 30 cases of fdc sarcoma have been reported , in which the patients were diagnosed with myasthenia gravis ( mg ) or pnp 3 , 4 . moreover , two cases of castleman 's disease were associated with mg and pnp , suggesting a putative alternative linkage of pnp , mg , and the associated neoplasms 6 , 7 . to date , we have not found any reports on iptlike fdc sarcoma associated with mg or pnp . a 60yearold chinese woman presented a 6month history of recurrent weakness and increasing upper extremity fatigue , fluctuating diplopia , and ptosis of both eyes , finally showing dysarthria and dysphagia . during 6 months several masses had been found in her left axillary region and neck for 15 years , and the masses were resected every 4 years without radiotherapy or chemotherapy . the last surgery was 2 years prior to the study . after admission , thoracic computed tomography ( ct ) showed several masses in the left axillary and neck , and the diameter of the maximal tumor was 6.4 cm . core needle biopsy revealed spindle tumor cells arranged in a storiform pattern with scattered lymphocytes ( fig . spindle tumor cells contained vesicular chromatin , small nucleoli , and thin smooth nuclear membranes ( fig . immunohistochemical studies revealed that the tumor cells were positive for ki67 ( + 20% ) ( fig . 2a ) , vimentin , cd21 , cd35 , cd20 , egfr , cd68 , s100 ( fig . 2b ) , cd1a , cd3 , and cd79a and negative for ema , ck , cd23 , cd34 , hmb45 , cd30 , mpo , and lysozyme . however , ebvencoded rna was detected by in situ hybridization and was negative . ( a ) microphotograph showing spindle tumor cells arranged in a storiform pattern with scattered lymphocytes ( 10 ) . ( b ) spindle tumor cells contained vesicular chromatin , small nucleoli , and thin smooth nuclear membranes ( 20 ) . ( b ) some of the spindle cells are positive for s100 ( 10 ) . after admission , treatment was immediately started with pyridostigmine at a dose of 60 mg three times per day following a subtle improvement of muscle weakness . five days later , the patient developed severe dyspnea , and then , an emergency intubation and mechanical ventilation were administered . immunoglobulin ( 0.4 g / kg / day 5 days ) and steroid pulse therapy ( methylprednisolone sodium succinate , 1000 mg daily 3 days , gradually tapered off ) were given . sixteen days after treatment with immunoglobulin and steroids , the patient was successfully detached from the respirator . mechanical ventilation was again administered . after steroid and immunoglobulin treatment failure , rituximab at a dose of 500 mg ( 330 mg / m/week ) after four courses of rituximab treatment , the skin lesions and muscle weakness were almost recovered , and ventilator weaning was successful . three weeks after discharge , she was hospitalized with severe generalized blistering skin eruptions and polymorphic erythemas and mucosal ulcerations ( fig . 3 ) , which were considerably worse than those in the previous occasion but without myasthenia symptoms . then , she was treated with immunoglobulin ( 1 mg / kg per day ) without improvement of mucocutaneous lesions . we describe the extremely unusual association of an iptlike fdc sarcoma with pnp and mg . the pathogenesis is thought to be due to the production of antibodies against tumorderived antigens ; the antibodies may attack components sharing the same or a similar epitope 8 . 10 reported a case in which pnp associated with fdcs showed a good response to a treatment plan that included dexamethasone , surgery , and chemotherapy . our observations on this case indicate that rituximab could be an effective alternative therapy in the treatment of pnp and mg . however , pnp recurred catastrophically , perhaps because the standard treatment dose was not achieved and surgical resection of tumors was not performed . the patient presented multiple local recurrences without systemic dissemination for a long period , suggesting an indolent clinical course . considering the high mortality of pnp , we should pay more attention on the diagnosis and treatment of iptlike fdc sarcoma .

key clinical messageparaneoplastic pemphigus ( pnp ) is an autoimmune blistering disease associated with neoplasms . the disease is most commonly of lymphoproliferative origin and presents high mortality . we describe a patient with pnp and myasthenia gravis associated with inflammatory pseudotumorlike follicular dendritic cell sarcoma , as well as the response to rituximab .

popliteal artery stenosis or occlusive disease is a common occurrence in elderly patients , diabetics , smokers and those with cardiovascular disease . it leads to significant morbidity and mortality by reducing or completely blocking blood supply through the popliteal artery and into the lower leg . we describe an unusual presentation of a 24-year - old male with a popliteal artery stenosis masquerading as an infective foot ulcer . we believe this case illustrates the importance of a meticulous history and physical examination when assessing foot ulcers . a 24-year - old male attended the department with a painful ulcer under the third metatarsal head of his left foot . this ulcer had been present for 2 months prior to presentation following a skin laceration sustained while travelling in thailand . the patient had received antibiotic therapy and surgical debridement on two occasions while abroad ; however , the ulcer had failed to heal . on closer questioning the patient reported that he sustained the injury while walking close to coral reef in the sea in thailand . there had been no history of freshwater exposure , drug misuse or of any risk - taking behaviour . initially , the patient was treated with empirical antibiotics on instruction from our microbiology department . antibiotic therapy was altered according to these sensitivities , and the erythema and swelling began to settle over the coming weeks . unfortunately , the ulcer continued to enlarge and a mri scan of the left foot revealed increased uptake in the third toe and metatarsal head indicating underlying osteomyelitis . , it proved necessary to amputate the third toe and metatarsal head as they could not be salvaged due to infective destruction . on conclusion of the procedure , the tourniquet which had been applied to the lower limb was deflated and it was noted that the left foot was slow to reperfuse . a vascular opinion was sought . ankle brachial pressure index ( abpi ) in the left leg was 0.46 when compared with 0.82 in the right leg , indicating a significant decrease in arterial blood flow to the left foot . a ct femoral angiogram demonstrated a left popliteal short segment occlusion with reforming of the tibio - peroneal trunk with small vessels . on detailed probing , a history of intermittent claudication was revealed with a claudication distance of 100 m. subsequently , a left superficial femoral artery to popliteal artery bypass was performed by the vascular team using a reverse vein graft . within 6 weeks , all antibiotic therapy had been stopped and the wound from amputation on the left foot had completely healed . stenosis of the popliteal artery is a common condition , most often seen in the context of peripheral vascular disease where an atherosclerotic pathogenesis is accountable . however , in a cohort of younger patients , there is a much broader range of pathogenesis accounting for such stenosis . conditions such as popliteal artery entrapment syndromes ( paes ) , popliteal artery aneurysm , cystic adventitial disease , multiple hereditary exostosis , some arteritis disorders ( e.g. takayasu 's ) and some rare congenital syndromes ( e.g. cutis marmorata telangiectatica congenital ) must be considered . in each of these conditions , there is an underlying disease process that culminates in a reduction in the cross - sectional area in the popliteal artery and consequently , flow through the vessel is decreased in accordance with laplace 's law . , there is an insidious process that can be unmasked by an acute insult ( e.g. trauma and embolism ) . the presentation of congenital popliteal artery stenosis is very similar to that in peripheral vascular disease , with intermittent claudication , slow healing wounds and transitory change in skin colour and temperature . a persistent wound or ulcer in a young and otherwise fit individual with no obvious predisposing factors is a red flag and should prompt further investigation . a thorough history with targeted questioning of the above presenting symptoms is important , followed by rigorous physical examination . initial bedside assessment of vascular status should be performed with abpis and doppler flow studies . blood flow may be posturally dependent in approximately a third of paes cases ; however , this is unlikely to be the case with a fixed stenosis of the popliteal artery . ct is a useful adjunct if external compression of the artery is a possibility ; furthermore , mri is useful in adventitial cystic disease . management of vascular insufficiency in young patients depends on the underlying pathogenesis . in adventitial cystic disease , symptoms may resolve spontaneously if cysts rupture ; however , recurrence is common . in congenital popliteal artery stenosis , vascular bypass is often required as with the 24-year - old gentleman under consideration here . in conclusion , this case emphasizes the importance of maintaining a broad differential diagnosis in young individuals presenting with persistent foot ulcers . in a young person with a persistent non healing ulcer , with unusual bacteriology and normal pulses , always consider a proximal vascular lesion . a prompt diagnosis of an unusual congenital vascular insufficiency can prevent complications and long - term morbidity for the patient .

here , we present a case report of a young male who had been travelling in thailand . the patient sustained a relatively minor trauma to the soft tissue under his left third metatarsal head on a piece of coral reef . he subsequently developed an infected ulcer , which did not heal despite appropriate antibiotic therapy and surgical debridement . unfortunately , the patient required amputation of the left third toe and metatarsal head due to osteomyelitic destruction . it later transpired that the ulcer was initially ischaemic in nature due to previously undiagnosed and asymptomatic popliteal artery stenosis .

laparoscopic posterior mesh rectopexy ( lpmr ) is a good surgical option for rectal prolapse with low morbidity and mortality . specific complication like mesh erosion into the rectum following lpmr is very rare with only one such case , in which mesh got expelled via rectum , reported in literature till date . we report a case of mesh erosion into the rectum after lpmr which was managed with laparoscopic mesh removal and review the published literature . a 40-year - old man presented with occasional bleeding per rectum since 10 months with low grade intermittent fever for 1 week . he had history of lpmr for complete rectal prolapse 2 years back , carried out elsewhere . sigmoidoscopy revealed hyperemic mucosa with ulceration 15 cm from anal verge , with a part of the polypropylene mesh projecting into the rectum [ figure 1 ] . computed tomography ( ct ) scan of abdomen showed asymmetric anterior wall thickening of rectosigmoid junction with fat stranding and minimal fluid collection . review of his surgical notes revealed that a 15 cm 10 cm polypropylene mesh was used for rectopexy . since it was a large mesh fixed with multiple tacks and possible dense adhesions , transanal approach was not attempted . under general anesthesia , in lithotomy position , diagnostic laparoscopy was done . appendix was inflamed with its tip densely adherent to the mesh [ figure 2 ] . there was a small rectal perforation at the site of erosion , which was confirmed by air leak test . sigmoidoscopic appearance of mesh erosion into rectum intra - operative images of ( a ) tip of appendix adherent to the mesh and ( b ) mesh eroding into rectum laparoscopic rectopexy is presently the preferred management approach for rectal prolapse as it has better results especially in terms of less post - operative pain , shorter hospital stay , and similar recurrence rate as open rectopexy . it may present with discharge of pus or blood per rectum , even several months or years after surgery . it can be evaluated by doing a flexible sigmoidoscopy , which will usually reveal an ulcerated lesion at the site of erosion with visualization of mesh . ct scan of abdomen with contrast helps in localizing the mesh in relation to rectum as well as pelvic collection , if any . it is less common with type i mesh when compared to type ii , iii , and iv meshes due to bigger pore size with lesser chance of infection . other factors that are found significant are poorly controlled diabetes mellitus , tobacco use , prior history of pelvic irradiation , and repeat procedures , which contribute to poor wound healing and subsequent infection , erosion or extrusion . same causes can be considered as possible etiologies for mesh erosion after lpmr as well . certain surgical technical errors like unrecognized rectal injury and deeper stitches through the rectum may also contribute to mesh erosion . bigger sized mesh which can produce more folding after fixation , as in our case , may be a possible cause for erosion . another possible cause in our case could be infection of mesh due to adhesion of inflamed appendix to it . since , the incidence is very rare , no definite protocol regarding management is available in literature . we preferred laparoscopic approach in our case , as it was too big mesh to remove through transanal approach . moreover , only a part of mesh was protruding into the rectal lumen and it was fixed with multiple tacks to the sacrum . severe inflammation and friable tissues precludes primary repair of the rectal defect where diverting ileostomy or colostomy plays an important role . in summary , the predisposing factors could be mesh infection , larger mesh , and unrecognized rectal injuries . laparoscopic excision of mesh is a feasible and promising approach to manage such an unusual complication following lpmr .

laparoscopic posterior mesh rectopexy ( lpmr ) is now an accepted surgical treatment for complete rectal prolapse . it is associated with complications such as partial mucosal prolapse , fecal impaction , constipation , and rarely recurrence . erosion of the mesh into the rectum after lpmr is very rare . we report herein the case of 40-year - old man who presented with mesh erosion into the rectum and managed successfully by the laparoscopic excision of mesh . this is probably the first such case managed by the laparoscopic approach .

adenoid cystic carcinoma ( acc ) is a malignant tumor characterized by perineural invasion , slow growth , and insidious destruction of surrounding tissues . it usually arises in the major salivary gland , but also in minor salivary gland occasionally . acc arising in the pterygopalatine fossa is extremely rare , only 3 cases have been reported . in these cases , 1 patient presented visual deficit because of neural invasion . owing to its proximity to the superior orbital fissure and optic canal , neoplasms arising in the pterygopalatine fossa may cause optic neuropathy by direct invasion or oncothlipsis . current literature has described possible visual recovery following excision of anterior clinoid mucocele , but few authors reported whether the visual deficit could be resolved after the resection of the tumor arising in the pterygopalatine fossa . in current study , we described 1 patient with visual dysfunction induced by acc arising in the pterygopalatine fossa . the zhengzhou university institutional research committee approved our study , and the participant signed an informed consent agreement . a 44-year - old chinese man presented a mass in the palate for 7 months , there was no ulcer or pain . one month ago , the visual acuity of the right eye dropped gradually and then there was no vision at diagnosis . preoperative radiographs presented a huge mass in the pterygopalatine fossa and infratemporal fossa ( figure 1a ) , but we could not determine whether the optic nerve was invaded ( figure 1b ) . after explaining the possible risks including total blindness to the patient , a surgical plan was formulated . and the patient reported the visual acuity had returned to normal at 10 days after the operation . at 2 months after discharge from the hospital , there was no recurrence of the tumor ( figure 1c ) , and the patient was satisfied with the appearance and the functional results . ( a ) huge space - occupying lesion in the pterygopalatine fossa and infratemporal fossa was noted . ( b ) the tumor involved the orbital apex , but the association between the optic nerve and the tumor could not be justified clearly . ( c ) at 2 months after discharge from the hospital , there was no recurrence of the tumor . the most distinctive biological behavior of acc was perineural spread . in previous case reports , all these findings supported the above - mentioned viewpoint . in current study , optic dysfunction was also noted , but it would be contributed by external compression rather than direct tumor invasion . various surgical approaches for the pterygopalatine fossa and infratemporal fossa have been advocated . it could be divided into 3 groups inferior approach , lateral approach , and anterior approach . inferior approach could not provide adequate exposure of the retromaxillary and the skull base , and it was usually suggested for the tumor restricted in the parapharyngeal space . lateral approach was limited for tumors extending medial to the pterygomaxillary fissure and especially those stretching across the medline . the maxillary swing approach was first introduced by wei et al for recurrent nasopharyngeal carcinoma as a novel anterior approach . nowadays , the route has been the preferable approach for tumors arising in the pterygopalatine fossa and infratemporal fossa . compared to the facial translocation approach , the maxillary swing approach did not require neurorraphy and could avoid the development of a free facial graft by keeping the anterior maxilla and translocated hard palate attached to the cheek flap . in current study , possible postoperative complications such as palatal fistula , epiphora , and trismus have been reported . the most interesting finding in such case was complete recovery of visual disorder following surgical resection of the disease . suri et al have studied the visual outcome in patients with suprasellar tumors who experienced preoperative blindness , and the authors found male sex , shorter duration of blindness , operative evidence of hemorrhage in tumor , and soft tumor consistency were significantly associated with the visual outcome ; another research conducted by mathiesen et al presented early optic nerve decompression and primary tumors seemed to predict better visual prognosis . similarly , kitano et al compared postoperative improvement of visual function among different approaches in treating suprasellar meningiomas , and the authors found extended transsphenoidal approach might resulted in improvements in visual acuity . other associated factors , such as the size of mass , the pathology type , tumor consistency and , so on , have also been proven in the literature . the same principle might also be compliant to the present case . in summary , acc arising in the pterygopalatine fossa visual loss contributed by the tumor in the pterygopalatine fossa could recover in selected patients .

abstractadenoid cystic carcinoma ( acc ) arising in the pterygopalatine fossa was rare , only 3 cases have been reported . in previous literature , few authors reported whether the visual deficit could be resolved following the resection of the tumor.one patient with visual dysfunction induced by acc arising in the pterygopalatine fossa was reported.complete visual recovery was achieved following the operation . and the patient was satisfied with the appearance and the functional results in the follow-up.visual loss contributed by the tumor in the pterygopalatine fossa could recover in selected patients .

nerve sheath myxoma ( neurothekeoma ) is an uncommon benign soft tissue tumor of the peripheral nerves with fairly distinctive histological features . a recent immunohistochemical pattern enables a differentiation between myxoid neurothekeoma , melanocytic tumors , and nervous system tumors . we report a rare case of myxoid neurothekeoma in a three - year - old child . a three - year - old boy presented with a nontender , left thumb swelling of four - week duration , without any history of trauma . fine needle aspiration cytology of the mass revealed a few plump - to - oval spindle - shaped cells in a myxoid background . gross examination of the submitted specimen revealed a firm , elastic , tan - colored smooth nodule , measuring 10 8 6 mm with a solid homogeneous gray cut surface [ figure 1 ] . histopathologic examination revealed a nonencapsulated dermal tumor , composed of multiple , closely situated medium - sized nodules with scattered spindle - shaped cells in a myxoid - rich stroma [ figure 2 ] . the tumor exhibited strong positivity for s-100 immunohistochemical stain [ figure 3 ] , and focal positivity for neuron - specific enolase ( nse ) . based on the histopathologic and immunohistochemical results , the case was diagnosed as nerve sheath myxoma of classical type . neurothekeoma : cut section shows solid homogeneous soft , yellowish gray tissue neurothekeoma : tissue section shows a nonencapsulated dermal tumor , composed of multiple , medium - sized nodules composed of few oval - to - plump spindle cells , in a myxoid - rich stroma ( h and e , 10 ) neurothekeoma : the tumor cells show strong positivity for s-100 immunohistochemical stain neurothekeomas commonly present as small solitary erythematous nodules on the face and the upper limb . however , other anatomic sites have been reported in the literature , such as oral cavity , cauda equina , lower limb , shoulder , and neck . it is a tumor of the adolescent and young adult , with a female predilection , but is extremely rare in infancy . neurothekeoma is a rare benign tumor of the nerve sheath , with a distinct histological presentation . it was described initially by hakin and reed , in 1969 , as nerve sheath myxoma but the term neurothekeoma was given by gallager and helwing . there are three histologic variants of neurothekeoma : myxoid ( classical or hypocellular ) , cellular , and mixed type . the myxoid type is characterized by a high degree of myxomatous changes , low cellularity with scattered spindle cells , and is well circumscribed . there may also be multinucleated giant cells and the lesions characteristically stain positively for s-100 , collagen type iv , and nerve growth factor receptor but are negative for epithelial membrane antigen or markers of histiocytic differentiation . in contrast , the cellular types of neurothekeoma are not encapsulated and the mixed type shows areas of varied cellularity with focal myxoid regions . our case is a typical example of a myxoid type of neurothekeoma . the cell of origin of neurothekeoma is controversial with theories proposing a proliferation of schwann cells or other perineural cells . dual immunoreactivity for nse and s-100 protein in a myxoid background supports schwann cell origin , whereas the absence of s-100 protein and positive epithelial membrane antigen in the cellular type suggests perineural cell lineage . fetsch , et al . postulated a tendency of neurothekeoma to recruit histiocytic cells and to differentiate into myofibroblasts . smooth muscle actin ( sma ) positivity suggests some similarity to the epithelioid variant of leiomyoma or myofibroblastic proliferations . glial fibrillary acidic protein ( gfap ) positivity is rare in neurothekeoma ; however , its positivity further supports a peripheral nerve sheath origin , as occasional peripheral nerve sheath tumors may coexpress gfap and s-100 . nerve sheath myxoma and neurothekeoma are recently identified as different entities in gene expression profiling , although they might share some morphologic similarities . gene expression array data strongly supports that nerve sheath myxomas are of peripheral nerve sheath origin and are distinct neoplasms from neurothekeomas . furthermore , data shows many similarities in gene expression between neurothekeomas and cellular fibrous histiocytomas , supporting recent arguments that these entities may in fact be related . there is an overlapping feature with other neural tissue tumors such as schwannoma , nerve sheath myxoma , and neurofibroma , leading to difficulties in diagnosis . differential diagnosis of dermal nodule in infancy and young adulthood should include fibrous tumors , histiocytic tumors , lymphocytic tumors , melanocytic tumors , and neural tumors . reporting such a case will increase awareness about this disease , as it should be included in the differential diagnosis of dermal lesions in young children .

neurothekeomas are rare benign neoplasms , typically occurring in young patients with a remarkable predilection for the female population . patients usually present with a small nodule in different anatomical sites , commonly involving the face and the upper limb . we report a case of a three - year - old boy , who presented with a nontender nodule on the left thumb . surgical biopsy and immunostaining confirmed the diagnosis as myxoid neurothekeoma . the rarity of this unusual skin tumor in a toddler prompted the following report .

the research was conducted at 4 islands on lake victoria ( kibuogi , ngodhe , takawiri , mfangano ) and 1 shoreline community of mbita district ( ungoye ) in western kenya ( figure ) . in this area , the pfpr210 ( community p. falciparum parasite rate standardized to the 2- to 10-year age group ) although in some area of kenya malaria has decreased , its prevalence remains high in the lake victoria basin because of the lake environment ( 810 ) . in 2009 , a total of 50%70% of households owned insecticide - treated bed nets ( 11 ) , which substantially reduce the risk for transmission of malaria parasites by providing barriers against mosquitoes . june and october november ) ( 9 ) , in the study sites , it is highly endemic throughout the year . study sites for investigation of k13 propeller gene in plasmodium falciparum , mbita district , kenya , 20122013 . filter paper blood spots were collected from participants ( technical appendix table 1 ) during population - wide cross - sectional malaria surveys conducted in february and august 2012 and august 2013 at the 5 study sites . parasitic dna was extracted from the filter paper ( 12 ) , and p. falciparum dna was detected by a mitochondrial dna - based pcr ( 13 ) . sequencing of the k13 propeller gene was attempted on the diagnostic pcr - positive specimens ( online technical appendix ) . the prevalence of p. falciparum , as determined by pcr , in the rainy and dry seasons was 7.2%26.2% and 6.5%15.5% on the 3 small islands ( kibuogi , ngodhe , and takawiri ) , 47.3% and 31.4% on mfangano island , and 38.4% and 41.9%64.0% in ungoye , respectively ( table 1 ) . * nine new types of point mutations were identified among these samples ( table 2 ) . participants infected with parasites harboring a mutation on the k13 propeller gene are listed in technical appendix table 2 . the sequences reported in this study have been deposited in the ddbj / embl / genbank databases ( accession nos.ab936059ab936067 ) . we detected 4 novel nonsynonymous and 5 novel synonymous mutations in the highly conserved k13 propeller gene of p. falciparum parasites from western kenya . noted that the frequency of mutant alleles strongly correlated with the prevalence of day 3 positivity after act treatment in humans in cambodia and that those mutations reflected positive selection ( 6 ) . that study found 17 mutant alleles in the k13 propeller gene . among them , c580y , r539 t , and y493h were prevalent and strongly related to in vivo delayed parasite clearance . in our study , all the mutations found differed from those reported in cambodia , and mutant alleles were not always observed in the proceeding seasons , so some mutations could be occasionally introduced . most of those mutations are not suitable for the life cycle of parasites , and only a few suitable for survival under the conditions of artemisinin selection pressure could be selected . furthermore , only 1 type of mutation , a578s from mfangano island , was detected during 2 seasons , whereas other mutations were not observed in the next season , half a year later . any family relations were not identified among the 4 participants harboring a578s mutation in february 2012 at mfangano island . point mutations can occasionally occur on the p. falciparum k13 propeller gene as a standing variation , but most of the isolates that recently acquired the mutation may disappear because of some fitness disadvantage or the effect of a random genetic drift ( 14 ) . we detected the mutant allele a578s in the k13 propeller gene in 2 consecutive seasons on mfangano island . this mutation , which modifies amino acids from being hydrophobic to hydrophilic , is close to the prevalent single nucleotide polymorphism c580y from cambodia that is thought to be necessary in protein protein interactions , which could affect artemisinin susceptibility . the genotype analyses of the parasites from this island are critical to understanding the role of this mutation and act efficiency in this geographic area . our k13 propeller sequence analysis of p. falciparum parasites from a malaria - endemic area in kenya did not detect the predicted artemisinin - resistant genotypes , but we observed some temporal substitutions . a limitation of our study was that the sample size was insufficient to specifically provide an understanding of this result . the accumulation of data from this region and from other malaria - endemic areas will increase our understanding of the relationship between the k13 propeller gene and artemisinin resistance . monitoring these molecular markers and the efficacy of antimalarial drugs is critical for increasing understanding of artemisinin resistance and predicting its spread . demographic information about the study participants ; sequencing of the plasmodium falciparum k13 propeller gene ; and data on the participants who had parasites harboring a mutation on the k13 propeller gene .

we looked for mutations in the plasmodium falciparum k13 propeller gene of an artemisinin - resistant parasite on islands in lake victoria , kenya , where transmission in 20122013 was high . the 4 new types of nonsynonymous , and 5 of synonymous , mutations we detected among 539 samples analyzed provide clues to understanding artemisinin - resistant parasites .

facial angiofibromas seen in tuberous sclerosis are usually bilateral.unilateral facial angiofibromas are seen rarely . unilateral facial angiofibromas , however , represent a rare variant of tsc , as only few cases are reported in the literature . we describe a case of a 30-year - old male who presented with unilateral facial angiofibromas without other features of tsc . a-30-year - old unmarried male born of non - consanguinous marriage presented for evaluation of right - sided facial papules that were first noted at 15 years ago . the lesions reportedly began as flat - topped , skin - colored , and erythematous papules involving the right side of his face . he denied history of any previous treatment . during last five years , gradually increasing numbers of new and similar lesions have appeared and spread to involve the right cheek , right nasofacial sulcus , and the right side of the nose . on examination , the patient has multiple , discrete , 2 - 5 mm , flat , skin - colored and hyper - pigmented papules involving the right cheek and right side of the nose [ figure 1 ] . there were no periungual fibromas , shagreen patches , caf - au - lait macules , forehead plaques or hypopigmented patches . the patient has no evidence of mental retardation and no history of seizures , cardiopulmonary lesions , renal lesions , or retinal abnormalities . multiple skins colored to hyperpigmented papules of various size distributed unilaterally on right side of face our clinical differential diagnosis included angiofibromas , verruca plana , syringoma and trichoepitheliomas . as there were no other cutaneous signs of tsc , the patient denied further evaluation and imaging studies to rule out internal manifestations of tsc . skin biopsy specimens of the facial papules revealed characteristic findings of angiofibromas , namely telangiectases , perivascular and periadnexal concentric dermal fibrosis , and large stellate fibroblastic cells scattered in the upper dermis . [ figures 2 and 3 ] based on above observations a final diagnosis of unilateral facial angiofibromas was made . patient was advised cryotherapy for his lesions , but we lost the follow - up with patient . ( h and e , 50 ) high power view showing dense collagen thickening , dilated vessels and few melanophages ( h and e , 200 ) the major impact of the disorder is on the skin , brain , and kidneys , though it also affects the eyes and heart . a major point regarding symptoms and signs of tsc is the variable clinical expression of the disorder . the major manifestations of tsc include skin lesions in more than 95% , mental retardation in approximately 50% , autism , seizures in approximately 85% . the classic triad of mental retardation , seizures and angiofibromas occur in only 29% of patients and 6% of them lack all three manifestations . facial angiofibromas occur in 80% to 90% of patients with tsc , typically presenting after 5 years of age . they typically occur as bilateral and symmetric , small , tan to erythematous papules on the cheeks , chin , and nose . our patient has multiple angiofibromas affecting only one side of the face but no other characteristic features of tsc were present , which is a rare presentation of tsc . mcgrae and hashimoto reported a case of unilateral facial angiofibromas as a segmental form of tsc . in 1997 , anliker , et al . and in 1998 , garcia , et al . reported similar cases . in 2000 , silvestre , et al . reported two patients with similar findings as a mosaic form of tsc . trauner , et al . in 2003 , reported similar case with poliosis of occipital area in a 53-year old man . in 2006 , camprubi , et al . reported a similar case in 13-year - old male and reviewed previous cases . hall , et al . in 2007 reported a case similar to our present case without any other manifestations of tsc . in 2008 , bordel - gmez reported a 28-year - old male with unilateral angiofibromas . lopez , et al . in 2009 , reported a case of unilateral eyelid angiofibroma with complete blepharoptosis as a presenting sign of tsc . our case of unilateral facial angiofibromas and without any other manifestation represents a rare clinical presentation of tsc . however , the possibility that this case represents a distinct clinical disorder can not be ruled out completely . this case highlights a rare occurrence of unilateral multiple facial angiofibromas without any other manifestations of tuberous sclerosis .

tuberous sclerosis ( tsc ) is an autosomal dominant hereditary condition with many varied forms of clinical presentation . the most frequent cutaneous findings in tsc include multiple angiofibromas , hypopigmented macules , periungual fibromas , and shagreen patch . rarely , unilateral multiple facial angiofibromas have been reported . we report a case of unilateral multiple facial angiofibromas without any other manifestations of tsc . although rare , unilateral multiple facial angiofibromas may be a mosaic form of tsc .

retained foreign body granulomas in the intervertebral disc space due to penetrant traumas are very rare like other paraspinal ones2,3,57,10 ) . to our knowledge , all of the reported retained foreign bodies in the intervertebral disc space were from missile injuries and there was not any report involving a retained foreign body granuloma in this region from wooden penetrant trauma2,3,5,6,10 ) . a 30-year - old man sought medical care with a history of low back pain ( lbp ) that radiated to lower extremities . he reported that he had fallen from a tree and a tree branch had been penetrated into him left lower back before 17 years . that tree branch had been taken out manually and he had recovered without any complaint . in the past two months , the patient has presented a lbp radiating to his lower limbs . physical examination revealed a bad - healed scar at about level of the third lumbar vertebra on left paraspinal region ( fig . the straight - leg - raising sign was negative and there was not any neurologic deficit on lower limbs . computerized tomography ( ct ) demonstrated a hypodenselytic lesion in the l4 vertebral body surrounding a linear hyperdense structure ( fig . 2 ) . anterior part of the disc space between fourth and fifth lumbar vertebrae had disappeared with fusion of these bodies in magnetic resonance ( mr ) images . mr images also revealed a lesion that lodged in posterior part of this disc space and expanding into the corpuses of these vertebrae ( especially into l4 vertebral corpus ) . it was seen a linear horizontal area of decreased signal intensity representing the intranuclear cleft in the center of posterior part of disc space between fourth and fifth lumbar vertebrae in sagittal mri image of the stir sequence . a firm mass was found lodged in posterior part of the intervertebral disc space between the fourth and fifth lumbar vertebrae . excision of the mass revealed a tough granulation tissue surrounding a tiny piece of wood in a little cavity filled with a clear yellowish fluid ( fig . there was a fusion between anterior parts of third and fourth lumbar vertebrae and a degeneration in the discal material in the rest of intervertebral disc space . postoperatively , the pain resolved gradually and the patient was discharged without any problem 10 days after operation . he had a total symptomatic cure after two months from excision of the foreign body . biological responses of tissues to foreign bodies are influenced by the location and the nature of them17,10 ) . in a soft tissue , a wooden foreign body can cause either a mild inflammatory tissue reaction resulting in a localized granuloma or a severe reaction resulting in a significant infectious lesion with or without osseous reaction in adjacent bone1,4,6,8 ) . paraspinal disorders like ones in the intervertebral disc space may cause important complications because they may affect vital structures in and near the spinal canal . in a similar way , the majority of reported cases with foreign bodies that embedded and retained near the spinal canal presented with delayed symptoms or findings resulting from compression of neural elements due to developed granuloma or abscess as well as in creased bony growths or migration of foreign body in the spinal canal2,3,57,10,11 ) . in the literature , there are only four case reports presented with the retained foreign body granulomas in the intervertebral disc space from penetrant traumas2,5,6,8,10 ) . two of these cases presented with cauda equina syndrome resulted from compression of neural structures due to granuloma and/or bony growths while two of them presented with symptoms and findings other than neurological deficits like our case2,5,6,8,10 ) . we thought that our patient s complaints probably were due to expanding of granuloma into the corpora of adjacent vertebrae . his complaints totally disappeared after removing the granuloma that in a way supported our thought . the detection of wooden foreign bodies with plain radiographies and ct has limited value because they are radiolucent . therefore , ultrasound and mr imaging are recommended in the detection of these foreign bodies like of other nonmetallic ones9 ) . but , the identification of wooden foreign bodies may also be difficult on mr imaging when they are small and on ultrasound when they are in the deeper paraspinal tissues . thus , a wooden foreign body in paraspinal tissues like intervertebral disc space may be easily missed especially in acute cases . more over , it is very difficult to predict the natural history of a retained foreign body in the intervertebral disc space , because , a sufficient number of cases is unfortunately not exist in the literature . if a retained wooden body in intervertebral disc space is missed and it is not removed , it may be potentially hazardous and result in significant morbidity because of the nature of wood and the paraspinal location of intervertebral disc space . moreover , a missed foreign body in a paraspinal tissues may cause further damages for patients and malpractice claims for physicians . therefore , physicians and radiologist need to keep their diagnostic suspicions height in terms of retained foreign bodies during examination or follow up a patient with paraspinal wooden penetrant traumas .

we report a patient with a wooden foreign body granuloma in the intervertebral disc space being symptomatic 17 years after a paraspinal penetrant trauma . according to the our result of the search for wooden foreign body granulomas , this is the first case suffered from a wooden foreign body granuloma in the intervertebral disc space that reported in the literature . in this report , we emphasized the importance of rigorous examination and follow up in paraspinal wooden penetrant traumas .

uterine artery anatomic variants have been the subject of detailed study , since the beginning of last century , given fibromyoma role in the pathogenesis of arterial vasculature . according to various studies , there are certain classifications of uterine artery anatomical variants and paper proposes such a classification , the observations selected aspects of examinations performed angiographic fibromyoma embolization . 110 examinations were conducted in women aged between 20 and 47 years , with symptoms and clinical diagnosis of uterine fibromyoma . embolization procedure was performed by unilateral brachial vascular access , using seldinger technique with a sheath pressure mounting and placing a 4f or 5f cobra catheter tip , running through the axillary artery , subclavian artery , ( brachiocephalic arterial trunk ) descending aorta , iliac artery common internal iliac artery , uterine artery tracing is the purpose of selective catheterization of it . retrospective evaluation of imaging uterine artery arteriography allowed classification into four groups , depending on the origin of the uterine artery . 220 were observed in the uterine artery catheterization and were classified number 200 , which represents 90.1% of the total , the remaining 20 were not conclusive arteriogram . analysis arteriogram interpretation allowed a classification into 4 types , given the classifications listed in the literature as follows : - type i - the origin of the uterine artery inferior gluteal artery ram ( fig . 1 ) - type ii - the origin of the uterine artery bifurcation ram inferior gluteal artery ( fig . 2 ) - type iii - the origin of the uterine artery to the internal iliac artery trifurcation ram , with lower and upper gluteal artery ( fig . 3 ) - type iv - uterine artery proximal to the origin of the origin of the gluteal arteries top and bottom ( fig . 4 ) the origin of the uterine artery inferior gluteal artery ram the origin of the uterine artery bifurcation ram inferior gluteal artery the origin of the uterine artery to the internal iliac artery trifurcation ram , with lower and upper gluteal artery uterine artery proximal to the origin of the origin of the gluteal arteries top and bottom anatomy descriptive notes uterine artery origin from the internal iliac artery anterior trunk , with arteries bladder , rectal average obturatory artery , internal pudendal artery , sciatica , however note the variant of origin of uterine artery from a common trunk artery umbilical [ 5 - 8 ] . in fact , the origin of the uterine artery is variable , according to recent studies , which are based on angiographic examinations . currently there is a classification of the uterine artery origin : type i ( the origin of the uterine artery inferior gluteal artery ram ) in 45% , type ii ( the origin of the uterine artery bifurcation ram inferior gluteal artery ) 6% , type iii ( origin uterine artery to the internal iliac artery of the ram trifurcation , with the upper and lower gluteal artery ) 43% and 6% type iv ( proximal to the origin of the uterine artery to the origin of the upper and lower gluteal arteries ) . recent studies , which are based on angiographic examination , note the presence of two trunks of bifurcation of the internal iliac artery , anterior and posterior in 70% of cases , and uterine artery is inferior gluteal artery origin or branch of the internal iliac artery trifurcation . the present study shows different values of such a percentage , such type i is 24% of the total arteriograms classifiable and type ii is in a proportion of 10% . for type iii percentage different values are observed to specialized studies , with a much increased type iv also shows that the type iii and type i are halfway to the classification established values . this again demonstrates the variability of uterine artery origin in different percentages , but major practical importance . in addition to the above aspects of the present and the emergence of uterine artery dissection of the cadavers in department of anatomy , university of medicine craiova . this notice these issues . in fig.5 the origin of the uterine artery uterine artery dissection origin proximal to the superior and inferior gluteal arteries emerging ( arrow ) in fig.6 uterine artery arises from the internal iliac artery , making common core with superior gluteal artery and inferior gluteal . knowledge of emerging variants uterine artery has an important role in the success of fibromyoma embolization , thus avoiding damage to other vascular structures , also correct assessment of uterine artery origin is reflected in the reduction of radiation dose and shortening the time of intervention .

uterine artery embolization as a therapeutic method in fibromyoma requires a good knowledge of the origin of the uterine artery to the success of this procedure involving selective catheterization . this study presents a classification of anatomical variants of uterine artery as a retrospective review of consecutive arteriogram , complete with various aspects of the origin of the uterine artery in cadaver dissection , in the department of anatomy .

in the previous issue of critical care , joulin and colleagues describe an animal model of lipopolysaccharide ( lps)-induced impaired myocardial systolic and diastolic function . diastole is composed of two physiological phenomena : myocardial stiffness , which is energy independent , and active relaxation , which is an atp - requiring process . current echocardiographic techniques can help distinguish which of the two is the predominant pathway , although these techniques may not always be practical in critically ill patients . in some patients with sepsis , a reversible component to impaired ventricular relaxation has been demonstrated , which would imply that the energy - requiring component of diastolic function is more perturbed in certain patients . one might wonder why this is an important distinction to make , but considering that increased mortality in sepsis may be correlated with increasing fluid administration , it would seem that being able to distinguish between isolated systolic , diastolic , or combined dysfunction may prove to be life saving . this was achieved by serial transesophageal echocardiographic measurement of standard indices of systolic function , as well as analyzing diastolic mitral inflow and annular tissue doppler patterns , the current standard in echocardiographic grading of diastolic function . this study highlighted the fact that we should be cognizant that not all patients suffering from sepsis and shock should be treated uniformly , and that choices of intravenous resuscitation and vasopressor therapies need careful consideration . the study further elucidated the reversible nature of the impaired ventricular relaxation in humans , suggesting that a metabolic or molecular process may be responsible . an attractive mechanism currently thought to play a major role is that of calcium flux . systole is initiated by rapid elevation of myocyte intracellular calcium , both through influx ( through l - type calcium channels ) and calcium - mediated calcium release from the sarcoplasmic reticulum ( through the ryanodine receptor ) . re - uptake of calcium into the sarcoplasmic reticulum by sarcoplasmic reticulum ca - atpase ( serca ) allows for cardiac relaxation . the ability of serca to pump calcium back into the sarcoplasmic reticulum is governed by phospholamban , a sarcoplasmic reticulum membrane - bound modulatory protein . the rapidity at which calcium is returned to the sarcoplasmic reticulum is directly related to the rapidity of cardiac relaxation . in the unphosphorylated state signaling by protein kinase leads to phosphorylation of phospholamban , which , in turn , diminishes its inhibitory activity on serca , promoting cardiac relaxation . in a knockout mouse model lacking phospholamban , while other pathways , such as sodium - calcium exchange , are important in myocardial calcium trafficking , those are currently beyond the scope of this comment , and will not be described . joulin and colleagues , in their experiments , were able to demonstrate a serca - dependent aberration in diastolic cardiomyocyte behavior . in particular , in their experiments , they were able to demonstrate a serca - dependent aberration in diastolic cardiomyocyte behavior . the force - frequency relationship ( that is , heart - rate - dependent increase in cardiomyocyte shortening ) and frequency - dependent acceleration of relaxation are physiological phenomena that ensure maintenance of cardiac output with the decreased ejection and filling times that are consequences of higher heart rates . the group hypothesized that lps would disrupt this delicate balance by exerting an effect on the molecular workings within the cardiomyocyte principally through the inhibition of the phosphorylation of phospholamban . by utilizing echocardiographic measurements of diastole , and through western blot analyses , they were able to demonstrate a correlation between lps - induced myocardial relaxation dysfunction ( frequency - dependent acceleration of relaxation depression ) and serca function . it is important to note that this work was done in a murine model of lps infusion , a model with potentially important differences from septic patients being treated in the intensive care unit . anesthetic agents were used to regulate heart rates , and real - time alterations in physiological functioning may have been missed . the group studied only the sarcoplasmic reticulum when calcium trafficking is also linked to mitochondrial function and integrity , nitric oxide production , the beta - adrenergic response , and potential protective effects on the myocardium during prolonged sepsis . would specific targeting of intracellular calcium , or serca - related protein kinase and phosphatase result in better hemodynamics in septic shock ? there is already a body of evidence to suggest that calcium - sensitizing agents , such as levosimendan , may improve hemodynamics in sepsis . in two animal models , left ventricular relaxation was improved after treatment with levosimendan in contrast to inotropes , such as milrinone or dobutamine , and in a clinical trial , levosimendan proved useful in improving global hemodynamic measurements . the study by joulin and colleagues is certainly thought provoking , and will hopefully lead us closer to developing better strategies for dealing with sepsis - induced myocardial dysfunction both its systolic and diastolic components .

sepsis - induced myocardial dysfunction has traditionally been thought of as principally affecting systolic heart function . one of the primary reasons for this concept is that systolic dysfunction is relatively easy to conceptualize , visualize , and measure . with the advent of preload - independent measurements for diastolic function , both measurement and conceptual difficulties are being resolved , and a new realm of evidence is beginning to emerge regarding the aberrations that are found during cardiac relaxation in sepsis . a recent article in critical care brings this issue into sharper focus .

neurologic complications from mycoplasma pneumoniae have been known since the 1940s.1 encephalitis is the most common complication , and occurs most often in children . a review by lerer and kalavsky2 found that 53% of patients with central nervous system ( cns ) disease associated with m. pneumoniae were between the ages 6 years and 20 years . it is estimated that between 5% and 10% of acute childhood encephalitis in europe and north america is attributable to m. pneumoniae.3 there is a spectrum of clinical manifestations such as aseptic meningitis , peripheral neuropathy , transverse myelitis , cranial nerve palsies , cerebellar ataxia , acute transverse myelitis , and acute disseminated encephalomyelitis.4 the incidence of these manifestations has been estimated to be between 0.06% and 0.1%,5 but cns symptoms are reported in up to 7% of patients in the hospital setting with confirmed m. pneumoniae infection.6 long - term neurological problems have been noted in 20%60% of cases , with severe disease resulting in mental retardation , brain atrophy , hydrocephalus , epilepsy , visual changes , and global neurologic deficits with brainstem dysfunction and cerebellar ataxa.7 two studies have shown that between 24% and 30% of patients require intensive care treatment , with mortality rates of between 8% and 10%.8,9 the index patient presented with a 2-week history of head - aches , fevers , and dry cough . her examination revealed an ataxic gait with normal muscle power , reflexes , and skin sensation . initial computed tomography imaging of her brain showed a single hypodense white matter lesion in the left frontal lobe . a subsequent magnetic resonance imaging scan ( mri ) of the brain demonstrated a corresponding t2 and fluid - attenuated inversion recovery ( flair ) hyperintense lesion in the left frontal lobe ( fig . given the patient s age and symptoms , a diagnosis of multiple sclerosis was also considered . other investigations revealed a positive total m. pneumoniae serology immunoglobulin ( ig)m antibody titer of > 1,280 . the diagnosis of m. pneumoniae - related cns disease was made based on the patient s clinical symptoms , positive serology , and the mononuclear pleocytosis found in the csf . this is typically in the range of 10200 cells/l for m. pneumoniae - associated cns infections.10 routine csf gram stain and bacterial cultures are characteristically negative . one review of 58 cases showed a diagnosis was made in 84% of patients with positive serology , 14% with csf polymerase chain reaction ( pcr ) , and 2% with csf antibody determination.11 our laboratory was able to evaluate the csf for the open reading frame ( orf ) 521 nuclear capsid protein gene with pcr , which returned a negative result . acyclovir was ceased 3 days into hospital admission , and the patient was prescribed a course of oral doxycycline . the patient s symptoms resolved over a period of 1 week of observation in hospital . further analysis of the csf with isoelectric focusing revealed oligoclonal bands of igg in the csf that was not present in the serum . two subsequent cranial mri scans of the patient were obtained over a period of 3 months in the outpatient setting , and they showed the stable appearance of the left frontal lobe lesion . the positive oligoclonal bands in the csf are likely related to the immunological phenomena that are commonly linked to m. pneumoniae infections . it has been known since the 1970s that polyclonal b - cell activation and antibodies to brain tissue have been demonstrated in m. pneumoniae infections . 12 in 1996 , nishimura et al.13 reported three cases of m. pneumoniae encephalitis with associated anti - galactocerebroside antibodies . in this patient , a moderately positive anti - ganglioside gq1b antibody was found . this supports the hypothesis that demyelination and neurologic dysfunction in this entity could be mediated by the immune system . the pathogenesis of m. pneumoniae cns disease is unclear , but it may be related to direct invasion ( proven during brain biopsy culture15 or nucleic acid hybridization16 ) or autoimmunity ( immune complexes , cross - reacting antibodies , or a neurotoxin effect).17 this case highlights a rare cause of encephalitis in a patient presenting to a tertiary - level adult teaching hospital .

we report a rare cause of encephalitis that is not often described in the adult clinical setting . our case was a 16-year - old female who presented with a clinical picture of viral encephalitis ; however , magnetic resonance imaging showed a demyelinating lesion of the left frontal lobe . in this age group , differential diagnoses of acute demyelination encephalomyelitis and multiple sclerosis were entertained . further investigations demonstrated positive mycoplasma pneumoniae serology . as a result , a diagnosis of mycoplasma pneumoniae - associated encephalitis was made based on a process of exclusion .

intraventricular tendons was first observed 115 years ago.1 they are usually thin , fibrous or fibromuscular structures that traverse the left ventricular cavity.2 they may be single or multiple and have no connection to the valvular cusps.2 false tendons can be an isolated finding or can be associated with cardiac abnormalities.3 loukas et al4 examined 200 formalin fixed , adult hearts and divided false tendons into five categories : in type i the false tendon is located between the posteromedial papillary muscle and the ventricular septum . in type ii the false tendon is located between the two papillary muscles . in type iii the false tendon iv the false tendon is located between the ventricular septum and the free wall . in type v the false tendons appear weblike and have three or more points of insertion . after careful histological examination abdulla et al3 suggested that false tendons may be intracavitary radiations of the bundle of his and that their clinical significance may be more relevant than previously believed and merits further study . recently , various physiological effects due to a newly observed thick , subaortic muscular band have been described.57 the purpose of this study was to describe the incidence of this peculiar new type of muscular band in a cardiovascular clinic . a total of 4900 adult echocardiograms , performed between 2004 and 2010 for various clinical indications ranging from hypertension to cardiac murmurs and dyspnoea were reviewed and analyzed for the presence of a left ventricular muscular band in the subaortic position . a total of 376 adult echocardiograms clearly demonstrated the presence of a muscular structure , located between the left ventricular inferior wall and the subaortic interventricular septum ( see figure 1 and 2 and movie clip 1 and 2 ) . in all of these cases a striking local ventricular hypertrophic response was present at the site of septal implantation . in some of these cases the local hypertrophic response a peculiar muscular structure , too thick in appearance to be called a tendon , extending between the subaortic portion of the interventricular septum and the inferior left ventricular wall is identified in this study . a striking feature is a localized area of ventricular hypertrophy at the site of subaortic implantation of the muscular structure . among these 376 patients with the muscular subaortic band present , the following physiological effects have been observed : st - segment elevation of the anterior electrocardiographic leads , mimicking acute myocardial infarction,4 echocardiographical mimicking of hypertropic cardiomyopathy5 and right bundle branch block.6 it has been proposed before that this muscular , subaortic band may represent a sixth type of ventricular false tendon.8 the current clinical implication of the presence of this peculiar intracardiac structure is that it may mimic cardiac disease , such as myocardial infarction or brugada syndrome in the case of st segment elevation,5 it may lead to the erroneous diagnosis of hypertrophic cardiomyopathy if the echocardiographer is not vigilant6 and recently it has been described that a third type of right bundle branch block may result due to the presence of such a subaortic muscular band.7

intraventricular tendons are structures that was identified more than a hundred years ago . it has been suggested that they represent intracavitary radiations of the bundle of his and that they may be an isolated finding or be associated with structural cardiac abnormalities.loukas et al divided these structures into five categories and recently a sixth type have been added.various physiological disturbances have been observed due to the sixth type of tendon , such as st segment elevation and right bundle branch block . it has been noted that this peculiar structure appears too thick to be called a tendon , thus the term band.this retrospective analysis analyzed the incidence of the thick , subaortic ( type 6 ) muscular band in a cardiovascular clinic .

isolated dislocation of the radial head in adults is a rare injury with less than 30 cases reported in literature [ 1 - 9 ] . if neglected , these can cause restriction of forearm supination and pronation , secondary degenerative arthritis of the elbow and distal radio - ulnar joints later on . this important injury can easily be missed in the presence of other major associated injuries . also since with this injury there is no limitation of elbow flexion - extension and limitation of prono supination is relatively less disabling , the patient may tend to ignore the disability and present late . lastly the mushrooming of unlawful and uncontrolled bone setters can make the simple injuries complicated and problematic later on . a 26-year old man presented to us 8 weeks after injury to his right elbow with a motorbike accident . the patient complained of pain in the right elbow immediately after accident though he was able to flex and extend the elbow . thinking it to be a trivial injury he went to a bone setter for treatment , who applied wooden splints after oil massage to the elbow . however , the patient had pain in the elbow and presented to us after further 4 weeks with gross limitation of supination and pronation . there was no previous history of elbow injury or arthritis . on examination , there was minimal swelling over the elbow and tenderness over the radial head , which appeared displaced backwards . although he had good flexion and extension of the elbow , forearm pronation and supination were grossly restricted and painful . radiographs showed posterior dislocation of the radial head with no associated fractures of the radius , ulna or disruption of the distal radio - ulnar joint . immobilisation was done in flexion and supination and continued for three weeks with serial radiographs done at week 1 and 2 to make sure the superior radio - ulnar joint was in reduced position ( fig . 2 ) . elbow mobilisation was started after removal of the plaster , under supervision of the physiotherapist . the patient was followed up at 3 and 6 months . at 6 months he had no residual pain at the elbow and movements of elbow 26 year old man presenting with 8 weeks old isolated posterior dislocation of right radial head with limitation of prono - supination at right elbow . post operative radiographs showing excision of the radial head three months follow up showing good range of prono supination isolated dislocation of the radial head with no other associated fracture or dislocation of elbow is rare but not unknown injury in adults is a rare injury [ 1 - 5 ] . reduction in such cases is easily achieved by a pronation / supination manoeuvre and most authors propose immobilization of the elbow in flexion and supination in a plaster cast . the proximal radio - ulnar joint is most stable in supination : in this position , the contact between radius and ulna is maximal and the interosseous membrane , the annular ligament , and the anterior fibres of the quadrate ligament are all taut , thus drawing the radial head snugly against its notch in the ulna , cadaveric studies have shown that posterior dislocation of the radial head can not occur without the rupture of the annular ligament ; in addition , partial tear of the quadrate ligament and the proximal interosseous membrane takes place . most acute cases can be reduced closed and the functional outcome seems to be good post reduction . if missed or neglected , an open reduction has to be done with either an annular ligament reconstruction or a radial head excision deemed as the procedure of choice . in this case radial head excision the period of immobilisation was 21 days . in the presence of major distracting injuries like long bone fractures , pelvic fractures , chest and abdominal injuries , an isolated radial head dislocation can be easily missed as pain is masked by the presence of major distracting injuries and flexion and extension of the elbow is almost normal . even if it is an isolated injury patient may take it as a minor injury because of normal flexion and extension at elbow . therefore occurrence of such an injury must be kept in mind in all cases of elbow trauma and supination and pronation of the forearm must be assessed . this injury if missed can have serious repercussions later on , such as degenerative arthritis of the elbow and the distal radio - ulnar joints . there is also need for awareness amongst the public about the dangers of going to bonesetters , even for small injuries after any accident .

a 26 year old male reported with gross limitation of pronation and supination of right forearm 8 week after a vehicular accident . x - ray of the elbow showed isolated radial head dislocation without any associated fracture . it was treated with radial head excision with almost full restoration of movements postoperatively . the case is presented because of rarity of this injury and the tendency to miss this rare injury especially in the presence of other distracting injuries . however its early diagnosis and proper management is required to prevent chronic pain in future due to ensuing osteoarthritic changes and the disability of pronation and supination .

all patients in this study had previously been published as cases of meier - gorlin syndrome or fulfilled diagnostic criteria for meier - gorlin syndrome ( microtia , absent / small patellae and short stature ) . no cytogenetic abnormalites were observed on routine karyotyping of these meier - gorlin syndrome cases ( n=10 patients ) . furthermore , no significant increase of either spontaneous , or radiation - induced chromosome breaks was observed ( 2 cases versus 2 control patients , 100 metaphases assessed per patient ; p 0.7 , x - test ) . informed consent was obtained from all participating families and the studies were approved by the scottish multicentre research ethics committee ( 04:mre00/19 ) , regional committee on research involving human subjects nijmegen - arnhem ( 0006 - 0119 ) ; or university of texas southwestern medical center at dallas ( irb # 032008 - 066 ) . coding exons and exon - intron boundaries of each gene were screened by bidirectional capillary sequencing on an abi 3730 gene sequencer . sequence analysis and mutation detection performed using mutation surveyor v. 2.61 ( softgenetics llc ) . amplicons were separated by capillary electrophoresis on an abi3730 sequencer , and allele sizes obtained using genemapper ( applied biosystems ) . all patients in this study had previously been published as cases of meier - gorlin syndrome or fulfilled diagnostic criteria for meier - gorlin syndrome ( microtia , absent / small patellae and short stature ) . no cytogenetic abnormalites were observed on routine karyotyping of these meier - gorlin syndrome cases ( n=10 patients ) . furthermore , no significant increase of either spontaneous , or radiation - induced chromosome breaks was observed ( 2 cases versus 2 control patients , 100 metaphases assessed per patient ; p 0.7 , x - test ) . informed consent was obtained from all participating families and the studies were approved by the scottish multicentre research ethics committee ( 04:mre00/19 ) , regional committee on research involving human subjects nijmegen - arnhem ( 0006 - 0119 ) ; or university of texas southwestern medical center at dallas ( irb # 032008 - 066 ) . primers were designed using exonprimer ( supplementary table 2 ) . coding exons and exon - intron boundaries of each gene sequence analysis and mutation detection performed using mutation surveyor v. 2.61 ( softgenetics llc ) . amplicons were separated by capillary electrophoresis on an abi3730 sequencer , and allele sizes obtained using genemapper ( applied biosystems ) .

meier - gorlin syndrome ( ear , patella , short stature syndrome ) is an autosomal recessive primordial dwarfism syndrome characterised by absent / hypoplastic patellae and markedly small ears1 - 3 . both pre and post - natal growth are impaired in this disorder and although microcephaly is often evident , intellect is usually normal . we report here that this disorder shows marked locus heterogeneity and we identify mutations in five separate genes : orc1 , orc4 , orc6 , cdt1 and cdc6 . all encode components of the pre - replication complex , implicating defects in replication licensing as the cause of a genetic syndrome with distinct developmental abnormalities .

carcinoma primarily developing in the cystic duct is uncommon , compared to other locations in the biliary ducts . they may have better survival than malignancies located in other biliary ducts [ 2 , 3 ] . we report a 66-year - old male who is still doing well 13 years after surgical resection for ccd . a 66-year - old male was admitted to the digestive surgery service of the teaching hospital of trousseau for recurrent right upper quadrant pain in january 1995 . laparoscopic surgery was performed and peroperative cholangiography made opaque only 1 cm of the cystic duct because of an intraluminal obstruction . at laparotomy a tumor was found in the distal portion of the cystic duct suggesting a ccd . the patient underwent en - bloc resection of the gallbladder , the cystic duct with its tumor , the common bile duct and the choledochus . an extending lymph node dissection in the hepatoduodenal ligament and a roux - en - y hepaticojejunal anastomosis final histological examination of the resected specimen showed a moderately differentiated excretobiliopancreatic adenocarcinoma of the cystic duct ( fig . 2 ) there was no invasion of the gallbladder or the common bile duct and the choledochus . the patient is doing well 13 years after the resection without any sign of recurrence or metastasis . ccd is rare according to the literature [ 1 , 3 , 4 , 5 ] . however its incidence would be higher than is believed now if all the advanced stage cases were added . it is proved that inflammation of the biliary duct epithelium potentially leads to malignancy sooner or later , in consideration to the frequently associated carcinomas with pancreaticobiliary maljunction . indeed , because of its narrow lumen , the cystic duct is more often affected by gallstones during their migration from the gallbladder to the choledochus than other extrahepatic biliary ducts . therefore this carcinogenesis factor should increase the incidence of carcinoma in the cystic compared to the other biliary ducts . clinically , ccd may be completely asymptomatic or be revealed by signs suggesting other pathologies . it sometimes presents with mirrizi syndrome [ 3 , 4 , 5 ] and is diagnosed preoperatively . peroperative diagnosis is common when performing operation for acute cholecystitis such as in our patient . in consequence , any relapsing cholecystitis , particularly without gallstones , associated with uniform distended gallbladder should suggest the possibility of cystic duct tumor [ 1 , 2 ] . for us , the impossibility to catheterize the cystic duct for cholangiography or to opacify the hepaticocholedochus , such as in this case , should lead to the search for cystic duct tumor . on the treatment level , most authors recommend radical surgery [ 2 , 3 , 4 ] though , according to the definition , advanced stages of ccd are excluded . these authors propose en bloc resection of gallbladder , cystic duct , common bile duct , choledochus and an extending lymph node dissection of the hepaticoduodenal ligament . we think this attitude is too aggressive and may not be necessary even if only early - stage ccd are concerned . biliary duct resection and extending lymph node dissection should be sufficient . according to the literature , among all patients with carcinoma of the extrahepatic trees , those fulfilling the definition criteria of ccd should have better and prolonged survival than those with other locations . this better survival is justified by advanced stage exclusion . in spite of these restricted criteria , the overall published survival to our knowledge varies from 14 months to 125 months [ 1 , 2 , 3 , 4 ] with a median survival of 20 months . thirteen years 156 months ) after surgery our patient is still doing well without any recurrence or metastasis . to our knowledge this is the second case with a survival over ten years and the first one with a disease - free survival over thirteen years . beside this prolonged survival , this case report may suggest possibly curability when large en bloc resection of the biliary trees with extending lymph node dissection is performed . the incidence of ccd might be increased if advanced stage ccd were taken into account . disease - free survival over thirteen years is possible , but large resection and extending lymph node dissection are necessary .

cystic duct carcinoma is known to have a better prognosis compared to its location in other biliary ducts . only one case with a survival over ten years has been previously published . the authors report a case of survival over 13 years without recurrence or metastasis . preoperative diagnosis of cystic duct tumor was carried in a 66-year - old male . under the diagnosis of carcinoma of the cystic duct , the patient underwent en bloc resection of the gallbladder , cystic duct , hepaticocholedochus and lymph node dissection . a roux - en - y hepaticojejunostomy was performed . histological examination revealed a moderately differentiated adenocarcinoma of the cystic duct . five months later the patient underwent second look surgery for benign obstruction of the hepaticojejunal anastomosis . the patient is still doing well 13 years later without any local recurrence or metastasis . to our knowledge , this is the longest disease - free survival ever published in the literature . this case sustains that better and longer survival is possible with a real chance of potential cure if radical surgery is performed .

chronic intraventricular encapsulated hematoma is a rare entity and the exact mechanisms of capsule formation , progressive expansion and resolution have remained uncertain . here damage to any of these vascular or neural elements from direct compression , ischemia or invasion can result in a multitude of signs and symptoms . these include cranial nerve deficits , features of elevated intracranial pressure from hydrocephalus , changes in higher cognitive function or even autonomic dysregulation . computed tomography ( ct ) and magnetic resonance imaging ( mri ) are excellent diagnostic tools and are essential in surgical planning . since a neoplasm could not be ruled out preoperatively , the lesion was totally removed by right frontal craniotomy with a transcallosal approach in order to eliminate the mass effect . the general medical condition of the patient is another important consideration in defining surgical strategy . after an extensive review of the literature a 30-year - old male was admitted to our hospital in october 2008 with a 4-day history of fever , photophobia , diplopia and cervical pain . on admission , the brain ct scan showed a round - shaped homogenous mass in the lateral ventricle with a peripheral zone of high density , mild perifocal edema and a ring enhancement with mass effect . brain mri showed an extra - axial round - shaped lesion in the middle line , arising from the roof of the third ventricle with definite edges and with a significant mass effect . 1 ) . after contrast injection , ring enhancement of the lesion could be observed . a yellowish mass was found with glistering appearance and a hard wall that was difficult to be operated without micro - knifes and ultrasonic aspirator . the mass was filled with engine oil - like fluid and removed totally without difficulties because of the plane with the surrounding tissue . histological examination revealed a hematoma which was consistent with early development of granulation tissue , neovascularization , accumulation of fibroblasts , histiocytes and hemosiderin deposits ( fig . chronic encapsulated intracerebral hematoma ( ceih ) is a rare lesion which is believed to be caused by angiographically occult vascular malformations ( aovm ) such as arteriovenous malformation ( avm ) , venous angiomas , cavernous malformations , capillary telangiectasia , mixed or unclassified angiomas [ 2 , 3 ] . mri can be used to demonstrate that a lesion is only a hematoma and is valuable in follow - up and in differentiating these hematomas from neoplasms . differential diagnosis by location includes astrocytoma , colloid cyst , craniopharyngioma , teratoma , choroid plexus papilloma , cysticercosis , dermoid , choroids plexus carcinoma and avm . in our case ,

this case report is a presentation of a chronic intraventricular encapsulated hematoma in an adult who presented with gait disturbance , fever , palsy of n. iii , n. vi and n. vii , and photophobia . ophthalmoscopy revealed a bilateral papilledema . computed tomography scans and magnetic resonance imaging revealed an intraventricular mass in the lateral ventricle . preoperative diagnosis could not rule out a neoplasm . the lesion was totally removed with open surgery and was found to be a chronic intraventricular encapsulated hematoma . a chronic intraventricular encapsulated hematoma is a rare entity . after the removal of the mass , the clinical symptoms of the patient gradually resolved .

kaufman described that the mean length of the styloid process on the right and left sides was 30 mm and 29.49 mm , respectively in panographic radiographs of 484 hospitalized patients . he also reported that the mean length for elongated styloid processes was roughly 33 mm and 53 mm for left and right side . therefore , a case of an elongated styloid process over 6 cm in length is very rare . our case report presents the clinical findings , the diagnostic usefulness of 3d - computed tomography ( 3d - ct ) , and a comparison of treatment methods with reference to the existing literature , which could lead to better understanding of the pathophysiology and safer therapies for elongated styloid process . a 68-year - old japanese man complained of repeated left - sided throat pain for 10 years and hard swallowing pain since 2 weeks before . 3d - ct showed bilateral elongated styloid processes ( lengths of left and right : 7.8 cm and 8.0 cm ) . the right styloid process was longer and thicker than the left styloid process ( figure 1 ) . we performed shortening of only the left - sided styloid process ( the same side of throat pain ) using by a transcervical approach ( figure 2 ) . stimulation of the pharynx or cervical nerves from an elongated styloid process is known to cause pharyngeal and swallowing pain . some institutions reported that no statistically significant correlation was found between the presence of elongated styloid process between the genders . ghosh and corrella revealed that this disease was largest distribution of patients in age was seen in fifth decade of life . vieira indicated that 36.28% of the elongated styloid process showed both right and left sides as our case . kaufman , vieira and corrella demonstrated that there was no significant correlation of process elongation and calcification to age . chief complaints of our patient were repeated left - sided throat pain for 10 years and swallowing pain . some reported that pain in the throat was most frequently encountered as the presenting complaints , and majority of kind of complaints ( e.g. , pain in the throat , painful swallowing , foreign body sensation in the throat , ) had more than one complaint as our case . such a rare case , maik introduced a patient as having glossopharyngeal neuralgia due to elongated styloid process could lead to syncope and seizures . this cartilage consists of four highly variable parts ( tympanohyale , stylohyale , ceratohyale , hypohyale ) . long process results from the ossification of the tympanohyale and the styohyle portions during the first 8 years of life , and short process encountered from the ossification for the tympanohyale segment only while stylo- and ceratohyale portions degenerate . conventionally , postnatal growth of the styloid has been advanced as a possible mechanism for the elongated styloid process . but a correlation between an increase in styloid process length and age has not been substantiated . beder recommended that 3d - ct was the radiologic investigation of choice for elongated styloid process because of the most advanced technique available for definitively measuring the length of the styloid process . also , our elongated styloid process could be easily diagnosed by 3d - ct ( left and right lengths : 7.8 cm and 8.0 cm , respectively ) and safety treated with surgery , since it can fully visualize the anatomic relationships . the management of this disease process has been known to be non - surgical or surgical treatment . the nonsurgical treatment generally involves pharmacotherapy with anticonvulsants or antidepressants , but the results are short - lived . therefore , many hospitals stated that the first choice for treatment of elongated styloid process was surgery because of the severity of symptoms . ghosh insisted that surgical shortening of the elongated process was the only to give symptomatic relief to the patient . this surgical shortening of an elongated styloid process is often performed using an intraoral or external approach . it can often enable an intraoral approach requiring shorter surgical time and less surgical trauma without leaving visible scars on the neck . the external approach to the styloid process involves a transcervical approach to the parapharyngeal space , and wider visualization in the operative field than an intraoral approach . in our cases , recently , it reported that endoscopic - assisted styliodectomy for this disease could safety resect stylohyoid ligament with a smaller portion remaining at the skull base . in our case , the right styloid process was longer and thicker than the left styloid process . however , since the throat symptoms at swallowing were on the left side , appeared that the longer and thicker right styloid process would work as the pivot of a door and that the left styloid process had an abnormal orbit that stimulated the pharynx muscles and cervical nerves .

we experienced a case of an elongated styloid process that was 8 cm in length . the patient was a 68-year - old japanese man with frequent episodes of left - sided throat pain for 10 years . the elongated styloid process could be diagnosed by 3d - computed tomography ( left and right length : 7.8 cm and 8.0 cm , respectively ) and successfully treated with surgery , since the anatomic relationships could be fully visualized . surgical methods for shortening an elongated styloid process involve an intraoral or external approach . the external approach to the styloid process involves a trans - cervical approach to the parapharyngeal space , which enables wider visualization in the operative field than an intraoral approach . in the present case , the styloid process reached the parapharyngal space . therefore , we selected the external approach and shortened only the left - sided styloid process ( the same side as the throat pain ) . the chief complaint disappeared immediately after the operation .

initially described after treatment with flutamide , responses to withdrawal of hormonal therapies in prostate cancer patients have been documented after cessation of other antiandrogens such as nilutamide , bicalutamide , or cyproterone acetate and of megestrol acetate , diethylstilbestrol or estramustine . in the southwest oncology group trial 9426 , 21% of 210 patients had confirmed psa decreases of 50% after treatment with nonsteroidal antiandrogens with a median progression - free survival of 3 months . it has been proposed that withdrawal responses could result from mutations in the androgen receptor ( ar ) . transfection experiments revealed that ar point mutations in the hormone - binding domain allow activation by ligands other than dihydrotestosterone . mutated ar from clinical samples was shown to be activated by progesterone , estradiol , adrenal androgens , hydrocortisone or hydroxyflutamide . however , a prospective study failed to demonstrate a clear association between detectability of ar mutations and antiandrogen withdrawal responses . an improvement in overall survival has been reported with abiraterone acetate ( aa ) , a selective inhibitor of cytochrome p450c17 ( cyp17 ) , in castrate - resistant prostate cancer patients who have failed docetaxel chemotherapy . the addition of low - dose corticoids to aa is mandatory in daily practice since the combination has been shown to minimize the syndrome of secondary mineralocorticoid excess related to cyp17 inhibition . here , we report the case of a patient who had a confirmed psa decrease of 50% after stopping aa , mimicking a withdrawal syndrome . a 60-year - old patient was diagnosed with a prostate adenocarcinoma ( gleason score 8 , 4 + 4 ) with synchronous bone metastases in april 2007 . he successively received an lhrh agonist , a combined androgen blockade with lhrh agonist and bicalutamide , without subsequent response to bicalutamide withdrawal , docetaxel ( 10 cycles ) , then mitoxantrone ( 3 cycles ) and diethylstilbestrol . in april 2011 , aa was started at the standard dose of 1,000 mg daily in combination with prednisone ( 5 mg twice daily ) . psa slightly decreased to a nadir of 68 ng / ml one month after the start of treatment . in parallel , a metabolic partial response according to consensus criteria on 18-fluorodeoxyglucose positron emission tomography ( pet ) and choline pet ( suvmax = 37% ) was observed . however , aa and prednisone were stopped in august 2011 because of back pain related to vertebral metastases and concomitant psa progression ( psa 128 ng / ml ) . one month later , a confirmed psa decrease to a nadir of 62 ng / ml occurred , defining a partial biological response according to standard criteria , with a concomitant improvement in back pain . to the best of our knowledge , this is the first case ever reported of a withdrawal response with aa . currently proposed mechanisms of resistance to aa include ligandless activation of ar by constitutively active variants lacking the ligand - binding domain , cross - talk with relative signaling pathways and/or activation of amplified or promiscuous ar by nonandrogenic ligands such as corticosterone or other steroids . following the latter hypothesis , the withdrawal response observed in the present case could be related rather to prednisone than to aa itself as it was stopped at the same time .

in 2011 abiraterone acetate ( aa ) was approved for the treatment of castrate - resistant metastatic prostate cancer patients who have failed docetaxel chemotherapy . we report the case of a patient who experienced a confirmed psa decrease of 50% after stopping aa , mimicking an antiandrogen withdrawal syndrome .

it is well documented that transplants save lives and improve quality of life for patients suffering from kidney , liver , and heart failure . but in many countries , moral courage and public health policies have not kept pace with need . in a recent issue of critical care , fieux and colleagues demonstrate remarkable results obtained through a coordinated effort to obtain viable kidneys from people who suffer out - of - hospital cardiac arrest in paris . in the us , however , lack of organs results in 8,000 patients who die or become too sick to receive a transplant every year . furthermore , recent trends suggest the number of patients awaiting organs is increasing by several thousand yearly . the us institute of medicine ( iom ) suggested that the transplantation community pursue donations from non - heart beating donors in the out - of - hospital setting ( that is , uncontrolled donations after cardiac death ( udcd ) ) to meet the demand for solid organs . at the time , such conclusions were based on case series experience from the us and spain demonstrating that this pool of potential kidney donors yielded transplantation outcomes similar to that of donation after neurological determination of death ( dndd ) and controlled donations after cardiac death ( cdcd ) . although fieux and colleagues report high rates of delayed graft function compared to the madrid udcd program , it is encouraging that , even using cold perfusion techniques , they achieved similar excellent rates for graft survival . the current strategies of cdcd and living donation have practical and ethical limitations . using donors where care is withdrawn in a hospital setting ( that is , cdcd ) has raised issues about how the time and manner of death will be determined and whether the patient is actually ' dead ' . living donation raises its own set of concerns : the closer the donor is to the recipient , the more concerns emerge about coercion ; the more distant the donor , the more worries about commodification . as the recent scandal about illegally purchased organs in the us illustrates , need dictates action ; illegal , mercenary , or altruistic . current cdcd strategies still yield less than 1,000 organs annually in the us , and the number of living donor kidneys has been steadily declining since 2004 . udcd programs avoid the dangers of live donation and evade the controversy that surrounds cdcd . udcd respects the ' dead - donor rule ' and complies with the deep medical traditions of respecting life at all costs and respecting the body after death . during resuscitation , emergency medical service rescuers must focus completely on their task of restarting the heart to achieve the return of spontaneous circulation in a patient in cardiac arrest . fortunately , techniques for resuscitation have advanced and survival after out - of - hospital cardiac arrest has improved greatly in the past years ( new york city fire department , unpublished data ) . when survival is no longer a possibility , and the decision to terminate resuscitative efforts is made independently of organ donation considerations , the ethics clearly allow for the pursuit of udcd . if it is evident that udcd is an effective and ethical alternative to existing efforts ( that is , living donations , cdcd , and dndd ) , why has udcd not been more widely accepted ? in order for organs to remain viable , interventions for organ preservation must be initiated within minutes after pronouncement of death . it may be difficult to obtain necessary consent for these interventions from grieving family members . to address this challenge , countries such as france and spain have passed legislation allowing ' presumed consent ' for preservation . therefore , preservation measures may be initiated unless the patient has specifically ' opted - out ' . other societies should consider first - person consent for organ donation ( as may be indicated through organ donor consent registries or on donor cards such as drivers ' licenses ) . the latter approach is that advocated for the us in the recent work by dubois and the new york city udcd study group . currently , people who die from an out - of - hospital cardiac arrest are denied the opportunity to be organ donors except in those few locations where out - of - hospital udcd programs are active . the results reported by fieux and colleagues in this journal , and the continuing success of the madrid and barcelona out - of - hospital udcd programs , demonstrate the viability and reproducibility of such protocols . the iom conservatively estimated that in the us about 22,000 decedents could become udcd donors . it is conceivable that widespread dissemination of udcd could obviate the waiting list for kidney transplants . cdcd : controlled donations after cardiac death ; dndd : donation after neurological determination of death ; iom : us institute of medicine ; udcd : uncontrolled donations after cardiac death . the nyc udcd study group is composed of the following : new york city health and hospitals corporation , new york organ donor network , new york university school of medicine , and the fire department of the city of new york . it is supported by the us health resources and services administration ( hrsa ) grant # r380t08761 . the opinions expressed in this publication are solely those of the authors with lewis goldfrank , md , responsible for its content .

it is well documented that transplants save lives and improve quality of life for patients suffering from kidney , liver , and heart failure . uncontrolled donation after cardiac death ( udcd ) is an effective and ethical alternative to existing efforts towards increasing the available pool of organs . however , people who die from an out - of - hospital cardiac arrest are currently being denied the opportunity to be organ donors except in those few locations where out - of - hospital udcd programs are active , such as in paris , madrid , and barcelona . societies have the medical and moral obligation to develop udcd programs .

no definitive treatment has been established for bleeding vesical varices . in symptomatic vesical varices , surgical devascularization , laser sclerosis , and coagulation are often only temporarily effective . as another treatment option , percutaneous transheapatic obliteration ( 1 ) and endoscopic injection of histoacryl could be performed . we report a male patient who had a history of bladder substitution with ileal segments and had been treated by balloon - occluded percutaneous transhepatic obliteration of vesical varices that presented with recurrent gross hematuria . a 50-year - old male presented to the ed with recurrent episodes of gross hematuria . 30 years ago , he was admitted to our hospital and underwent left nephrectomy and orthotopic bladder substitution with ileal segments after radical cystectomy due to renal tuberculosis in the left kidney . this patient was readmitted in october 2009 with gross hematuria and underwent transurethral cystoscopic coagulation of varices in the urinary bladder ( fig . he was initially treated with saline bladder irrigation and blood transfusion . in view of his history of recurrent gross hematuria , a contrast enhanced computed tomography ( cect , siemens medical systems , erlangen , germany ) scan of the abdomen and pelvis was performed and showed dilated blood vessels along the right side of the bladder wall with drainage into the superior mesenteric vein ( smv ) . arterial portographies via the superior mesenteric artery showed dilated veins draining into the smv in the right pelvic area ( fig . percutaneous transhepatic portography ( ptp ) was performed by catheterization of the right anterior branch of the intrahepatic portal vein . the 10 mm diameter balloon catheter with a length of 40 mm ( cook inc . , bloomington , in , usa ) was then advanced over the wire into the smv . a retrograde venogram was obtained while the outflow tract was occluded with the balloon ( fig . a renegade microcatheter ( boston scientific corporation , natick , ma , usa ) was advanced through the balloon catheter into varices using 0.14 transcend microwire ( boston scientific corporation , natick , ma , usa ) . the balloon catheter was inflated in the smv to prevent the migration of 5% ethanolmaine oleate ( grelan pharmaceutical , tokyo , japan)-lipiodol mixture ( 1 : 1 ) . about 10 ml of the mixture was injected via the microcatheter until the entire variceal complex was opacified . after approximately 15 minutes , complete occlusion of the varices was observed during deflation of the balloon catheter ( fig . the access tract was embolized with one detachable microcoil ( interolocking detachable coil , boston scientfic corporation , natick , ma , usa ) on completion of the procedure . after 6 weeks , cect showed well obliterated vesical varices and the patient did not complain of gross hematuria ( fig . vesical varices secondary to portal hypertension are rare since the bladder wall is not the usual collateral route for venous splanchnic blood flow ( 2 ) . vesical varices may occur in patients with portal hypertension in circumstances where the normal splanchnic collaterals fail to develop due to prior obliteration from treatments such as surgery , sclerotherapy or ligation ( 3 ) . the second probability is when the anatomy of the venous drainage of the bladder is altered from surgery such as bladder augmentation with a bowel segment . in our case , he underwent left nephrectomy and orthotopic bladder substitution with ileal segments after radical cystectomy due to renal tuberculosis in the left kidney , 30 years ago . varices secondary to portal hypertension have been known to occur in ectopic areas like duodenum , jejunum , ileum and anorectal regions . these varices in the gastrointestinal tract have been treated with endoscopic injection sclerotherapy , endosopic band ligation , balloon - occluded retrograde transvernous obliteration , transjugular intrahepatic portosystemic shunts , percutaneous transhepatic obliteration , and surgical procedures such as segmental resection and ligation ( 4 ) . percutaneous transhepatic obliteration of sclerosants is a less invasive procedure when compared to surgical procedure and hence may be the only means of actively arresting bleeding vesical varices in a patient who is unfit or unsuitable for surgery . the potential disadvantage of sclerosants injection is its embolization which could lead to infarcts at distant sites or even fatality . , the possibility of vesical varices needs to be considered in the care of patients with recurrent hematuria , especially , those associated with portal hypertension . balloon - occluded percutaneous transhepatic obliteration could be a treatment option for vesical varices , especially , in a patients who is unfit or unsuitable for surgery .

gross hematuria secondary to vesical varices is an unusual presentation . we report such a case recurrent gross hematuria in a male patient who had a history of bladder substitution with ileal segments that had been treated by balloon - occluded percutaneous transhepatic obliteration of vesical varices .

our previous analysis for cis - regulatory elements was unable to elucidate the mechanism regulating the gene expression pattern in 1-cell embryos , except for the gc - rich nature of regions upstream of active genes [ 11 , 12 ] . since there is no cis - regulatory element specific to 1-cell embryos , it is possible that the chromatin structure is involved in regulating gene expression in 1-cell embryos . the chromatin structure is essentially repressive for transcription , necessitating the presence of enhancers to help transcription factors access the gene promoters . however , it has been shown by reporter gene assays that enhancers do not increase transcriptional activity in 1-cell embryos , suggesting that transcription is regulated independently of enhancers at this stage [ 2 , 14 , 15 ] . furthermore , we demonstrated that transcription occurs only via the core promoter in 1-cell embryos , but not in 2-cell embryos . suggested that enhancer - independent transcription is caused by the loosened chromatin structure in 1-cell embryos , since this type of transcription was still observed at the 2-cell stage when the embryos were treated with butyrate ( an inhibitor of histone deacetylase ) , which increases histone acetylation to loosen the chromatin structure . using fluorescence recovery after photobleaching analysis , we have recently shown that preimplantation embryos at the 1-cell stage form the loosest chromatin structure . a recent analysis of the genome - wide landscape of chromatin accessibility to dnase i demonstrated that the number of dnase i - hypersensitive sites ( dhss ) is the fewest at the 1-cell stage and increases during preimplantation development , which appears to contradict the hypothesis made by the above - mentioned reports [ 2 , 14,15,16,17 ] . however , in the analysis using dnase i , the dhss do not necessarily correspond to the sites of the genome with a loosened chromatin structure . in sites with a loosened chromatin structure , transcription factors can easily access the dna and bind to it , thereby obstructing the dnase i access to the dna . in this case , the sites of chromatin with a loosened structure are insensitive , rather than sensitive , to dnase i. furthermore , since the chromatin structure is extremely loose in 1-cell embryos , dnase i might have cleaved an enormously large number of sites to make small fragments of dna that were removed by size selection ( > 50 bp ) for high - throughput sequencing , which would underestimate the number of dhss . taken together , we propose that 1-cell embryos have a loose chromatin structure that does not require enhancers for gene expression . if this is the case , we should expect a large number of genes to be promiscuously expressed , and the variation in their expression level should be relatively low . since many transcription factors would be able to easily access the promoters , enhancers should not have a strong effect on transcription . indeed , the analysis of our rna - seq data bears out this hypothesis . when genes are classified as active or inactive based on kernel density estimation , more than 90% of genes are active in 1-cell embryos , whereas only around 70% of genes are active in embryos at other stages and in oocytes ( fig . the kernel density estimation was calculated to classify all known genes as active or inactive in oocytes , 1-cell and 2-cell embryos , and blastocysts . horizontal and vertical axes are shown as the log2 of reads per kilobase per million ( rpkm ) and gene density , respectively . reads in exons were used to calculate the rpkm values in oocytes , 2-cell embryos , and blastocysts , whereas reads in introns were used for 1-cell embryos . this analysis also reveals that the variation in expression level is much lower in 1-cell embryos than in cells of other stages ; the slope of the histogram is steeper for 1-cell embryos . therefore , we suggest that a low level of enhancer - independent transcription occurs promiscuously in a large proportion of genes , which is probably caused by a loosened chromatin structure in 1-cell embryos ( fig . 2fig . gene expression is facilitated by the enhancers , which also regulate the gene expression levels . the chromatin structure is loosened genome - wide , which allows a low level of transcription in most genes independently of enhancers . ) . the kernel density estimation was calculated to classify all known genes as active or inactive in oocytes , 1-cell and 2-cell embryos , and blastocysts . horizontal and vertical axes are shown as the log2 of reads per kilobase per million ( rpkm ) and gene density , respectively . reads in exons were used to calculate the rpkm values in oocytes , 2-cell embryos , and blastocysts , whereas reads in introns were used for 1-cell embryos . gene expression is facilitated by the enhancers , which also regulate the gene expression levels . the chromatin structure is loosened genome - wide , which allows a low level of transcription in most genes independently of enhancers . we hope that this hypothesis sheds light on the mechanism that regulates the initiation of the gene expression program in embryos .

after fertilization , the genome of zygotes is transcriptionally silent . the timing of the initiation of transcription is species - specific and occurs at the mid-1-cell stage in mice . recent analyses using high - throughput sequencing ( hts ) have identified thousands of genes transcribed at the 1-cell stage , and the pattern of expression among these genes appears to be unique . in this article , we show the result of an additional analysis using hts data from a previous study , and present the hypothesis that an extremely loose chromatin structure causes promiscuous gene expression in 1-cell embryos .

takotsubo cardiomyopathy is a syndrome with transient systolic dysfunction of the left ventricle in the absence of significant coronary artery obstruction . the contractile function of the mid and apical segments of the left ventricle is classically depressed and there is hyperkinesis of the basal segments . takotsubo cardiomyopathy is much more common in women than men and is frequently triggered by emotional or physical stress . patients present most commonly with chest pain , electrocardiographic abnormalities such as st - segment elevation or t - wave inversion and elevated cardiac biomarkers . complications include acute heart failure , tachyarrhythmias , bradyarrhythmias , mitral regurgitation , cardiogenic shock , transient left ventricular outflow tract ( lvot ) obstruction , formation of a left ventricular thrombus , cardioembolic events , left ventricular free - wall rupture and death [ 15 ] . a 76-year - old female presented to the coronary care unit with chest pain and an ecg suggestive for an inferolateral myocardial infarction ( fig . 1 ) . she had never smoked and had two brothers with coronary artery disease before the age of 60 . it started at the moment she was worrying about loss of eyesight due to macular degeneration.fig . 2left ventricular angiogram a diastolic b systolic left ventricular angiogram a diastolic b systolic a beta blocker , ace inhibitor and oral anticoagulation , to prevent apical thrombus formation , were started . in the following days the patient developed atrial fibrillation and she had recurrent chest and back pain . echocardiography revealed a pericardial effusion of up to 16 mm , with diastolic collapse of the right ventricle and > 25 % transmitral flow variation with respiration which is indicative for cardiac tamponade ( fig . the oral anticoagulation was stopped and the inr of 2.6 was antagonised with vitamin k. pericardiocentesis was performed . after draining 120 cc bloody fluid , there was an immediate improvement of the haemodynamics and diuresis.fig . 3echocardiography a parasternal long axis b transmitral flow c subcostal view echocardiography a parasternal long axis b transmitral flow c subcostal view catecholamine excess , coronary artery spasm , microvascular dysfunction , dynamic mid - cavity or lvot obstruction , inflammation and regional myocarditis may play a role [ 3 , 6 , 7 ] . an mri study showed that pericardial effusion is common ( 62 % ) in takotsubo cardiomyopathy . cardiac tamponade in takotsubo cardiomyopathy , however is very rare . in 2010 , one case was described by yeh et al . in circulation . so far this complication has only been described in a total number of three cases . treatment with anticoagulants is recommended although there are no clear guidelines whether or not to start anticoagulation in takotsubo cardiomyopathy . the incidence of left ventricular thrombus formation and cardioembolic events in takotsubo cardiomyopathy is 5 % and 1.6 % respectively . of the cardioembolic events , not all patients with cardioembolic events show a left ventricular thrombus on their first echocardiography . an ischaemic brain event may cause irreversible damage while pericardial effusion can be drained . treatment with anticoagulants should therefore be considered at the moment of presentation to the hospital , irrespective of the presence of a left ventricular thrombus . the use of anticoagulants must of course be weighed against a patient s individual bleeding risk . although takotsubo cardiomyopathy complicated by cardiac tamponade is very rare , echocardiography should be performed in case of haemodynamic deterioration to exclude pericardial effusion .

we describe a 76-year - old patient with takotsubo cardiomyopathy complicated by cardiac tamponade . pericardial effusion in takotsubo cardiomyopthy is common but a cardiac tamponade is very rare . the use of anticoagulants may increase the risk of pericardial effusion and should be considered with care .

it usually presents at , or soon after birth with hyperkeratotic verrucous dark - brown ridges . porcupine men and systematized epidermal naevus have also been used to describe these lesions . based on the pattern of distribution , five clinical variants namely brocq type , lambert type , curth - macklin type , rheydt type and bfverstedt type have been described . we report a case of curth - macklin variant of ichthyosis hystrix with the absence of family history . it has been described to run in families as an autosomal dominant trait , very few sporadic cases of the entity have been described in the literature . an 11-year - old male child presented with asymptomatic , dark - brown , spiny , hyperkeratotic , verrucous scales and ridges all over body since birth . the patient gave history that scales shed after scrubbing and reappear in 5 - 7 days . there was no history of parental consanguinity , collodion presentation at birth , blistering or erythroderma . the affected skin showed symmetrically distributed , generalized , thickened , verrucous , dark - brown cobblestone - like lesions [ figure 1a ] . there was sparing of scalp and central part of face with involvement of ear [ figure 1b ] . axillary region , dorsum of hand , popliteal fossa and antecubital fossa were less markedly affected [ figures 1a and 2a ] . ( a ) generalized dark - brown , spiny , hyperkeratotic , verrucous scales and ridges . ( b ) involvement of ear with sparing of central part of face ( a ) spiny scales with relative sparing of dorsum of hand . nails are normal ( b ) keratoderma and fissuring of sole skin biopsy ( h and e staining ) showed hyperkeratosis , acanthosis and papillomatosis with thick granular layer [ figure 3a ] . perinuclear vacuolization and coarse keratohyaline granules were seen in upper spinous layers [ figure 3b ] . ( a ) h and e staining , 40 showing hyperkeratosis , acanthosis and papillomatosis with thick granular layer ( b ) h and e staining , 100 showing hyperkeratosis with perinuclear vacuolization and coarse keratohyaline granules seen in an upper spinous layer ichthyosis hystrix is a poorly defined entity which encompasses disorders displaying striking hyperkeratotic verrucous brown ridges . it is an extremely rare disorder , reported in only a few families and sporadic cases . five clinical variants have been described : brocq type , lambert type , curth - macklin type , rheydt type and bfverstedt type . the distinctive distribution of lesions , associated abnormalities and inheritance patterns help to differentiate between these forms . in brocq bfverstedt type was described in a single case which had striking follicular hyperkeratosis and mildly affected palms . lambert type , seen in the lambert family with 11 affected members across four generations , has no blistering with sparing of face , palms and soles . two large families with autosomal dominant inheritance and very few sporadic cases have been reported since its first description in 1954 . it results from heterozygous frameshift mutation in keratin 1 gene ( krt1 ) , and a new mutation for this disease has recently been reported . recently , it has been suggested to rename the entity as ichthyosis curth - macklin and group it under keratinopathic ichthyoses , along with epidermolytic ichthyosis and superficial epidermolytic ichthyosis . there is wide variability in its time of onset and clinical presentation , which varies even within families . it is characterized by massive palmo - plantar keratoderma leading to deep , bleeding , and painful fissures . there is development of hyperkeratotic , ridged or cobblestoned plaques over the large joints and/or hyperkeratotic papules on trunk and extremities . histopathological features include hyperkeratosis , acanthosis , and papillomatosis , along with the characteristic findings of perinuclear vacuolization and binucleate keratinocytes . the clinical and histopathologic findings in our patient were consistent with those of ichthyosis curth - macklin . this can be further confirmed by electron microscopy which also reveals keratin intermediate filaments aggregated into continuous , peripheral shells , without keratin clumping typical for epidermolytic hyperkeratosis . the same could not be performed in our patient due to lack of such facilities . however , it was ruled out by absence of history of blistering at birth and presence of very thick palmoplantar hyperkeratotic scales . our case , with severe palmoplantar keratoderma , generalized involvement with relative sparing of face and scalp , along with absence of blistering , erythroderma and deafness , and no family history of ichthyosiform disease , are suggestive of sporadic type of ichthyosis curth - macklin which is a very rare entity .

ichthyosis hystrix is a rare autosomal dominant genodermatosis , characterized by persistent spiny hyperkeratotic scales which cover a significant part of the skin surface . based on the pattern of distribution , five clinical variants namely brocq type , lambert type , curth - macklin type , rheydt type and bfverstedt type have been described . we report the case of an 11-year - old male child with spiny , hyperkeratotic scales all over the body since birth with sparing of scalp and central part of the face . palmoplantar keratoderma was also present . these clinical features are suggestive of ichthyosis curth macklin , which has been typically described in families . however , family history was negative in our patient . the case is being reported on account of rarity of the disease , that too with a very rare sporadic presentation .

though the most common presentation of obstructive uropathy is urosepsis , this disorder may be associated with hyponatremia and loss of salt in the urine . the present case posed a diagnostic challenge because despite the presence of an obstructive salt losing uropathy , the urinary salt concentration was paradoxically low . this previously unreported paradox was due to the entrapment of a high concentration of salt within the obstructed upper moiety of a duplex system , the contents of which were not mixing with urine in the bladder . an afebrile 6-month - old female was transferred to our institution with lethargy , vomiting , and diarrhea . laboratory evaluation was remarkable for metabolic acidosis , hyponatremia ( 133 meq / l ) , and hypokalemia ( 3.4 meq / l ) . since the patient promptly improved and laboratory values , including urinary sodium concentration , corrected with intravenous hydration , the patient was discharged with the diagnosis of dehydration without a proper work - up for obstructive uropathy . admission laboratory work revealed metabolic acidosis , hyponatremia ( 123 meq / l ) , hyperkalemia ( 6.0 meq / l ) , and decreased serum osmolality ( 270 mosm / kg ) . the urine sodium and osmolality were also decreased , 10 meq / l and 101 mosm / kg , respectively . plasma renin was > 1800 mcu / ml ( normal 664 mcu / ml ) and aldosterone was 1621 ng / dl ( normal 799 ng / dl ) , both markedly elevated . there was no history of significant vomiting or diarrhea and the cause of the electrolyte abnormalities could not be explained . renal sonography demonstrated a duplex left collecting system ( figure 1 ) and dilation of the upper pole moiety terminating as a large ectopic ureterocele at the bladder base . the echogenicity of the trapped urine was homogeneously increased suggesting a higher concentration of debris or minerals ( figure 2 ) . voiding cystourethrography confirmed the presence of a ureterocele and was otherwise normal ( figure 3 ) . the sonographic findings and discordance between urine and serum sodium concentration suggested that the enclosed upper pole collecting system had entrapped a higher sodium concentration , not reflected in the urinalysis . prior to repair , aspirates of the bladder and ureterocele revealed discordant sodium concentration , 22 meq / l and 118 meq / l , respectively . ectopic ureterocele is associated with ureteral duplication and obstructive dilation of the ureter and its calices . though the most common presentation is urinary tract infection , some children have a more insidious course with intermittent abdominal pain or failure to thrive . rarely , the infants have salt losing obstructive uropathies . salt losing nephropathy associated with obstructive uropathy has been called pseudohypoaldosteronism and is thought to reflect tubular unresponsiveness to aldosterone . though the combination of renal tubule immaturity , obstructive uropathy , and urinary tract infection are predisposing factors , pseudohypoaldosteronism has been described with pyelonephritis independent of obstruction and with obstruction independent of infection . because of these associations , urine culture and renal sonography have been advocated in any infant with an electrolyte disturbance . ordinarily the salt concentration in a urine sample correlates with the degree of renal salt excretion . however , our case demonstrates that anomalies resulting in entrapment or diversion of the urine with high salt concentration away from the bladder create exceptions to this principle . the paradox exemplified here need not be limited to cases associated with an obstructed ectopic ureterocele terminating at the bladder base . if the ectopic ureter of an obstructed moiety enters anywhere else than the bladder , such as the seminal vesicles , vagina , or urethra , similar clinical challenges may be encountered . though the observation of echogenic fluid within a ureterocele has been made before , it is usually attributed to nonspecific debris or pus related to urinary tract infection . in our case , which was not associated with urosepsis , sonography helped explain the unusual laboratory results . normal or decreased urinary sodium concentration and hyponatremia do not exclude salt losing obstructive uropathies in patients with hyponatremia . the excess salt can be entrapped in an obstructed system resulting in a false - negative urinalysis and diversion of attention away from this surgically correctible group of disorders .

a 6-month - old hyponatremic female with failure to thrive had low urinary sodium concentration . renal sonography revealed a duplex left collecting system with obstruction of the upper moiety as a blind - ended ectopic ureterocele extending to the bladder base . the echogenicity of the urine within the upper pole system was greater than the bladder contents . we believed that low urinary sodium concentration represented a false negative test and the salt loss by the obstructed left kidney was entrapped in the upper pole collecting system . prior to ureterocele repair , intraoperative bladder and ureterocele aspirates revealed discordant sodium concentration supporting the sonographic conclusion .

our patient was a 76-year - old man with a coronary artery bypass graft due to unstable angina . during the postoperative period , a blood gas analysis showed a paco2 concentration of 45 mmhg , pao2 concentration of 63 mmhg , and a ph of 7.40 during intermittent mandatory ventilation at a rate of 12 breaths per minute , a tidal volume of 500 ml , a positive end expiratory pressure of 10-cm h2o , and fio2 of 100% . the neck was prepared with povidone iodine , surgical drapes were placed , and tracheostomy was started . after neck incision , subcutaneous tissue and pretracheal fascia dissections were performed using a monopolar electrocautery device to secure hemostasis . after the identification of tracheal rings , the second and the third rings were incised vertically along the midline by using a scalpel , and to control minor bleeding in the paratracheal area , an electrocautery device was used . however , during its use , a loud pop was heard and a flame erupted from the surgical site . initially , we attempted to control the fire by applying manual pressure with a surgical towel but were unsuccessful because of the pure - oxygen spillage through the cuff and the use of positive end - expiratory pressure . we quickly disconnected the ventilator , removed the endotracheal tube , and extinguished the flame in the surgical field by using a saline spray . a tracheostomy tube was then inserted uneventfully . the skin at the peri - tracheostomy site was deeply burnt ( fig . unfortunately , the follow - up chest x - ray showed progressive pulmonary infiltration , and the patient subsequently succumbed to a multiorgan failure after unsuccessful recovery efforts . surgical field fires are dangerous to patients and surgeons , but unfortunately , many patients undergoing tracheostomy do not tolerate low fio2 . all fires require the following three elements : an oxidizing agent , an ignition source , and fuel . during tracheostomy , the oxidizing agent is supplemental oxygen , the ignition source is the electrocautery device , and the fuel is the endotracheal tube . smith and roy reported that all 18 of the reported tracheostomy fires were started by the electrosurgical unit and that the fuel was the endotracheal tube in 7 cases ( 39% ) and the patient s drape in 4 cases ( 22% ) . some case reports have described airway fires occurring when monopolar electrosurgical devices were used during tracheostomy , although this type of injury has been reported during bronchial sleeve resection , otolaryngeal surgery , and bronchoesophageal fistula repair . during tracheostomy , when an open trachea is present , the surgical field may become oxygen - enriched . furthermore , when an airway fire occurs during tracheostomy with an endotracheal tube located just proximal to the tracheostomy opening , a flame is often seen venting from the tracheostomy opening , and this venting can cause an extensive burn injury to the distal aspect of the tracheobronchial tree . to minimize the risk of fire during tracheostomy , surgeons must maintain fio2 at the lowest possible level . on the other hand , when fio2 levels are higher than 50% , an electrosurgical device should never be used to enter the trachea . in a previous study , it was shown that endotracheal tubes can be ignited in a 25% oxygen atmosphere . bailey et al . recommended that the following minimal precautions be taken to prevent fires during elective tracheostomy : 1 ) oxygen air and/or helium mixtures ( as permitted by patient condition ) be used to reduce combustibility ; 2 ) electrocautery devices be used sparingly , and if used , the voltage be set at a low level , particularly when the trachea is exposed during surgical resection ; 3 ) a bipolar rather than a unipolar electrocautery device be used to prevent / minimize current leakage ; and 4 ) saline or water be used instead of air to inflate the endotracheal tube cuff . ho et al . reported that co2 flooding of the surgical field reduces the risk of fire in patients who can not tolerate ventilation interruption or require high inspired o2 concentrations . surgeons should be aware of the possibility of airway fire and the means of preventing its occurrence during tracheostomy . our experience cautions that electrocautery be approached carefully in patients requiring high - oxygen therapy .

tracheostomy is a relatively common surgical procedure that is performed easily in an operating room or intensive care unit . open tracheostomy is needed in patients requiring prolonged ventilation when percutaneous tracheostomy is inappropriate . sometimes , it is difficult to achieve bleeding control in the peritracheal soft tissue , and in such cases , we usually use diathermy . however , the possibility of an electrocautery - ignited surgical field fire can be overlooked during the procedure . this case report serves as a reminder that the risk of a surgical field fire during tracheostomy is real , particularly in patients requiring high - oxygen therapy .

the increasing incidence of complex percutaneous coronary intervention ( pci ) is accompanied by risks of device fracture or dislodgement . guide wire fractures during pci are very rare , but in such cases , life - threatening complications such as embolization , thrombus formation and perforation may occur . although , in most cases , percutaneous retrieval techniques of fractured guide wires are recommended , there have been several reports of fragments being left in place without complications.1 - 4 ) we present the case of a 78-year - old female patient who was diagnosed with non - st segment elevation myocardial infarction ( nstemi ) and treated with pci . the patient had remnant guide wire filaments in the left anterior descending artery ( lad ) and aorta but did not experience any serious complications during a one - year follow up period . a diagnostic coronary angiogram showed diffuse significant stenosis from the proximal to middle lad and first diagonal branch ( d1 ) ( fig . we initially treated the patient with a single paclitaxel - eluting stent ( 2.7532 mm taxus , boston scientific , natick , ma , usa ) . four days later , two coronary angioplasty 0.014 guide wires were inserted { one hi - torque balance middleweight ( bmw ) universal coronary guide wire ( abott vascular , santa clara , ca , usa ) into the lad , and one high - torque whisper coronary guide wire ( abott vascular ) into the d1}. an intravascular ultrasound after predilatation with a 2.520 mm voyager balloon ( abott vascular ) revealed a large plaque burden at both the lad and the d1 . we deployed two overlapped sirolimus - eluting stents at the proximal and middle lad : a 2.7533 mm cypher at the middle lad and a 2.7518 mm cypher at the proximal lad ( cordis corporation , miami lakes , fl , usa ) . when we exchanged the guide wires to perform kissing balloon angioplasty , a fracture occurred at the distal tip of the bmw guide wire ( fig . 1 ) . another guide wire was inserted to perform a beaded wire rotation , and distal balloon inflation retrieval was attempted , in order to remove the fractured guide wires . finally , we used a goose neck loop - snare ( microvena corporation , st . multiple forward and backward movements of the snare , combined with distal balloon inflation retrieval , successfully removed most of the fractured guide wires ( fig . 2 ) but we later observed retained filaments during echocardiography ( figs . 3 and 4 ) . the patient declined surgical intervention for removal of these stray filaments and was discharged from our hospital , with triple anti - platelet medication and no complications . the patient did not experience any thrombotic or embolic events and did not suffer from any subjective symptoms over the one year of clinical follow up . guide wire fractures during pci are very rare , occurring in approximately 0.1 - 0.2% of cases.5 ) guide wire remnants could lead to life threatening complications such as thrombosis , emboli , and perforation . therefore , in the event of failed percutaneous retrieval and persistent signs of ischemia , patients should be urgently referred for surgical intervention . there are several methods recommended for the management of fractured guide wires , including emergent surgery , loop snare removal , two- or three - wire rotation , stenting over the retained wire , and conservative treatment.6 - 8 ) surgical extraction is strongly recommended in cases of protrusion of the guide wire into the ascending aorta.9 - 11 ) however , guide wire segments retained within the coronary circulation may remain benign for a long time , particularly if they are entrapped within a distal part of the vessel and do not have accompanying total coronary occlusions.3 ) vascular endothelial cell covering over the guide wire fragments may render them immobile and non - thrombogenic . hi - torque bmw guide wires consist of a distal core and a stainless proximal shaft , facilitating treatment of multiple lesions and tortuous vessels . however , guide wire fractures may occur if the distal core and stainless proximal shaft are separated by either the trapping of the distal tip or by vascular resistance . because the fracture in the lad developed after stent deployment at the main branch , we could not determine the mechanism of fracture in this particular case . however , trapping of the distal tip of the bmw wire , or stent deployment over a severely angulated guide wire are two possible explanations . in conclusion , even though the most ideal management option for remnant guide wires is their removal , conservative treatment with the fragments left in situ may be successful in cases in which patients remain asymptomatic and hemodynamically stable . however , life - long administration of intensive anti - platelet medications and close observation are recommended for these patients .

guide wire fractures during percutaneous coronary intervention ( pci ) are very rare , but when they do occur they may lead to life - threatening complications , such as embolization , thrombus formation and perforation . in cases when percutaneous retrieval has failed , surgical extraction of the remnant fragments is recommended . we present a case of remnant guide wire filaments that remained in place without complications , over a one - year clinical follow up period .

osteomas are the most common benign tumors of the nose and paranasal sinuses , with an incidence that ranges between 0.43 - 1% ( 1 , 2 ) . they occur most commonly within the frontal sinus ( 52% ) , followed by the ethmoid ( 22.0% ) , the maxillary sinus ( 5.1% ) , and the sphenoid ( 1.7% ) ( 1 ) . it is very rare for an osteoma to arise in the nasal cavity or turbinates . only four middle turbinate ( 2 - 5 ) , one superior turbinate ( 6 ) and one inferior turbinate osteoma cases ( 7 ) have been reported in the literature to date . we report an unusual case of middle turbinate osteoma associated with left - side nasal obstruction , epiphora and post nasal discharge in a patient managed successfully by endoscopic surgery ( es ) . a 41-year - old woman was referred to the department of otorhinolaryngology at the rasool akram hospital with a two - month history of progressive left nasal obstruction , epithora , and post nasal discharge . endoscopic examination revealed a mass completely blocking the left nasal cavity of bony hard consistency . computed tomography ( ct ) scan showed a circumscribed bony mass integrated laterally to the left middle turbinate that exerts pressure on the medial maxillary wall . the osteoma was totally extirpated subsequently without damaging the surrounding structures by es under general anesthesia . the mass was released gently from its attachment to the middle turbinate using a sickle knife . although the mass measured 3.02.7 cm in diameter , it passed through the left nostril without the need for further incision ( fig . 2 ) . examination of the histologic sections revealed a dense , mature , predominantly lamellar bone , consistent with osteoma ( figs . 3 and 4 ) . while it is a common tumor of paranasal sinuses that is found more frequently in the maxillary , ethmoidal and frontal sinuses ( 1 ) , it is very rare in the nasal cavity . beside the turbinates , the nasal septum can also be the origin of nasal osteoma ( 1 ) . reported cases of turbinate osteoma in the literature the etiology of osteomas arising from the paranasal sinuses can be embryological , traumatic or infective . osteomas can also be a part of gardner 's syndrome , an autosomal dominant disease characterized by intestinal polyposis , osteomas , cutaneous and soft tissue tumors . in affected individuals , the risk of developing colon cancer approaches 100% . on average , osteomas are detected 17 years before colon polyps begin to appear ( 8 , 9 ) . in turbinate osteomas , no associated history of facial trauma or nasal surgery had been reported , and paranasal sinus infection appears to be the result of sinus meatal obstruction due to mass effect of the tumor ( 3 ) . paranasal sinus osteomas may occur at any age , but usually present between the second and fourth decade , with a slight male predominance ( 3 ) . however , in the seven reported turbinate osteomas cases , including this , six cases were found in women and only one case in man ( table 1 ) . the majority of osteomas are asymptomatic in the early stages and are diagnosed incidentally during radiological examination for the other conditions . in symptomatic cases , the most common symptoms are progressive headache and chronic inflammation of the adjacent mucosal membranes ( 10 ) . the most common symptom in turbinate osteomas is nasal obstruction . in this case , the patient presented with nasal obstruction , epithora and post nasal discharge . however , it does not give detailed information of the lesion and it is not conducive for surgical interventions . although ct scan is an effective modality for the evaluation of osteomas , capable of detecting the extent of osteoma and invasion of the adjacent structures , namely the anterior cranial fossa , cribriform plate , and the orbit that may improve surgical decision making ( 6 ) . in osteoma arising from the paranasal sinuses , surgical removal is indicated if it extends beyond the boundaries of the sinus , continues to enlarge , localizes in the region adjacent to the naso - frontal duct , or if signs of chronic sinusitis are present , regardless of size in symptomatic tumors ( 6 - 8 ) . surgical approaches are classified into external , endoscopic drill - out , and combined endoscopic and external procedures ( 10 ) . although endoscopic removal is the preferred modality , the open approach should be considered to manage tumor involvement of the cribriform plate and frontal sinus . in osteoma arising from the turbinates , the tumor is more accessible and endoscopic removal carries less risk , and is easier compared to sinus osteomas . it is therefore important to diagnose nasal osteoma when it is small in size , follow it up and resect it when it is of a size that can be resected successfully by es , as in the present case . based on the fact that es carries low risk in the management of nasal osteoma , except when the cribriform plate is involved , and it is technically easier compared to endoscopic removal of sinus osteomas and smaller tumors and leads to better cosmetic outcomes , it appears clinically relevant to remove nasal osteomas at the time of diagnosis .

osteoma is the most common benign tumor of the paranasal sinuses . turbinate osteomas are very rare and only four middle turbinate , one superior turbinate and one inferior turbinate osteoma cases have been reported . we present a rare case of osteoma of the left middle turbinate in a patient presented with unilateral nasal obstruction and epiphora that was removed endoscopically , and conduct a literature review on turbinate osteomas arising from different turbinates , their symptoms and management .

we present a patient who developed pneumomediastinum after an elective bilateral preperitoneal laparoscopic hernia repair . as newer approaches to inguinal hernia repair are performed , newer complications of these techniques are being encountered . laparoscopic approaches to inguinal hernia repair have been described with equivalent short - term recurrence rates as those of open approaches . although the role of laparoscopic hernia repair has yet to be defined , decreased postoperative patient pain and quicker return to normal activity are potential advantages associated with laparoscopic herniorrhaphy . many thoracic complications of laparoscopic surgery have been reported including pneumothorax , pneumomediastinum , pneumopericardium , and subcutaneous emphysema . below , we describe a rarely reported complication of pneumomediastinum after preperitoneal laparoscopic inguinal hernia repair . although this may be an uncommon complication , surgeons performing laparoscopic herniorrhaphy should recognize it . a 64-year - old male with no past medical or surgical history presented to our clinic with bilateral inguinal hernia and desired laparoscopic repair . he was taken electively to the operating room for a standard bilateral laparoscopic preperitoneal hernia repair . intraoperatively , a balloon dissector was used to create a preperitoneal space . with a maximum pressure of 12 mm hg , two separate but overlapping 10-cm 15-cm pieces of polypropylene mesh were placed and tacked appropriately . no obvious tears occurred in the peritoneum nor did any evidence exist of pneumoperitoneum during the procedure . before all the ports were removed , 0.25% bupivacaine hydrochloride was placed in the preperitoneal space for postoperative analgesia . no elevation of end - tidal co2 was observed during the procedure . in the recovery room , the patient was monitored with continuous pulse oximetry and cardiac monitoring overnight with no desaturation or arrhythmia noted . his chest pain resolved with 1 dose of intravenous narcotics and he then required only oral analgesics . in relatively recent years , laparoscopic inguinal hernia repair became popular because of the shorter time needed to return to work and the decreased postoperative pain . another benefit of laparoscopic inguinal herniorrhaphy is the ability to perform a bilateral repair with the same incisions used for a unilateral repair . also , the surgeon is able to relatively easily diagnose and repair asymptomatic contralateral hernia during unilateral repairs in the hope of avoiding future hernia operations . although pneumomediastinum has been reported before , the actual incidence is unknown because postoperative chest radiographs are not and should not be routine after any type of hernia repair . in the situation in which an asymptomatic patient is found to have a pneumomediastinum , deviation from routine postoperative care is not necessary . pneumomediastinum can occur via air from the esophagus , trachea , bronchi , lung , neck , abdomen , or retroperitoneal space . the most likely source in our case was the abdomen because a pneumoperitoneum is present on the chest radiograph ( figure 1 ) . in fact , after paraesophageal hernia repair with or without fundoplication , pneumomediastinum ( secondary to surgical dissection through the esophageal hiatus ) has been considered a normal postoperative finding because it is present in 18% of patients . the only 2 previous cases of pneumomediastinum after preperitoneal laparoscopic hernia repair required prolonged ventilatory support for the patient . ramia et al reported an instance of a tear in the peritoneum that was noted and closed . postoperatively , the patient developed chest pain and oxygen desaturation along with subcutaneous emphysema of the neck . after 2 hours of ventilatory support , their patient was extubated and discharged 2 days later . browne et al reported a case in which extensive subcutaneous emphysema involving the scrotum , abdomen , thorax , and neck was observed postoperatively . although these 2 patients required prolonged mechanical ventilation , this is not always necessary as demonstrated by our own case . in patients without respiratory distress or with no upper airway compromise and who are hemodynamically stable with appropriate oxygen saturations , careful monitoring is all that is necessary for isolated pneumomediastinum . of course , a chest radiograph is needed to diagnose other associated problems like pneumothorax . pneumomediastinum is a rare complication of laparoscopic preperitoneal inguinal hernia repair , which potentially prolongs hospital stay and thereby increases total costs . early diagnosis and appropriate treatment are necessary to avoid serious problems . however , as an isolated finding , pneumomediastinum may only require careful monitoring .

background : as new approaches to herniorrhaphy are popularized , new complications are being encountered . pneumomediastinum is one such complication.methods:we present a patient who developed pneumomediastinum after an elective bilateral preperitoneal laparoscopic hernia repair.results:the patient was observed and discharged 24 hours later with no permanent sequelae.conclusions:pneumomediastinum is a rare complication of laparoscopic hernia repair .

myxomas are the most common benign cardiac tumors , typically originating from the left atrium . the right atrium is the second most common site where myxomas originate , accounting for 7%12% of cases . only a few cases of myxomas arising from the right ventricle have been reported . in these cases , myxomas originating from the right ventricle usually obstruct the right ventricular outflow tract , which may cause pulmonary embolism , syncope , and sudden death . in the present case , a myxoma was found to originate from the right ventricular inlet and showed dense adhesion to the anterior and posterior tricuspid leaflets . a 59-year - old man was admitted to the hospital with a three - month history of chest discomfort . an initial laboratory work - up , including complete blood count , electrolyte level , and cardiac enzyme level , revealed all results to be within normal ranges . transthoracic echocardiography revealed a large ovoid - shaped mass in the right ventricle , approximately 4.73.3 cm in size . the mass was attached to the inlet portion of the right ventricular free wall and to the anterior leaflet of the tricuspid valve ( fig . however , no tricuspid insufficiency or stenosis was observed , and the right ventricular systolic pressure was 21 mmhg . the patient was referred to the department of thoracic and cardiovascular surgery for surgical management . the mass was attached both to the ventricular surface of the tricuspid valve , along the entire posterior leaflet and one third of the anterior leaflet , and to the right ventricular free wall ( fig . . the mass was removed , and the posterior leaflet and one third of the anterior leaflet were excised as well . an incision was made along the annulus of the tricuspid valve that anchored the remaining anterior leaflet ( fig . several compression sutures were inserted to reduce the annulus for sliding annuloplasty , and sutures were placed for ring annuloplasty . the base of the anterior leaflet was attached to the annulus with 5 - 0 prolene sutures and a tricuspid annuloplasty ring was placed ( fig . intraoperative transesophageal echocardiography revealed no remaining tumor and no evidence of tricuspid regurgitation ( fig . myxomas arising from the right ventricle are extremely rare , and usually cause symptoms that result from obstructing the right ventricular outflow tract ( rvot ) . obstruction of the rvot and the main pulmonary trunk may cause complications such as syncope , pulmonary embolism , and sudden death [ 24 ] . several previous reports have described right ventricular myxomas obstructing the rvot . however , in our case , an extremely large myxoma was located in the inlet portion of the right ventricle , with a dense adhesion to the anterior and posterior leaflets of the tricuspid valve . it is thought that this dense adhesion to the tricuspid valve and the location of the myxoma in the inlet restricted the mobility of the mass and helped to avoid rvot obstruction . similarly , hirota et al . described a case of right ventricular myxoma that injured the tricuspid valve , leading to massive tricuspid regurgitation . unlike atrial myxoma , ventricular myxoma occasionally involves the valvular or sub - valvular apparatus , which makes surgery more complex . we had three surgical options for managing a myxoma that involved the tricuspid valve : tricuspid valvectomy without prosthetic valve replacement , tricuspid valvuloplasty with partial excision of the tricuspid valve , and tricuspid valvectomy with prosthetic valve replacement . arbulu and asfaw reported excellent results after using tricuspid valvectomy alone to treat tricuspid endocarditis , although 10%30% of patients subsequently required tricuspid valve replacement . we decided to perform tricuspid valvuloplasty because the remaining valve leaflet was sufficient for repair and the patient wanted a procedure involving repair of the valve leaflet in order to avoid anticoagulation therapy . in the present case , the entire posterior leaflet and one third of the anterior leaflet was attached to the mass , and we performed sliding valvuloplasty to excise the mass and surrounding tissue . two - dimensional echocardiography is useful in the diagnosis of myxoma and in determining the site , size , mobility , and attachment of masses . the treatment of choice for myxomas is surgical excision and the reported recurrence rate is 1%3% . in conclusion , this case study reports our surgical experience treating a rare type of right ventricular myxoma .

we report a rare case of an extremely large right ventricular myxoma involving the ventricular side of the tricuspid valve . the tumor was excised along with the entire posterior leaflet and part of the anterior leaflet . the tricuspid valve was repaired by sliding valvuloplasty combined with ring annuloplasty .

sjgren 's syndrome ( ss ) is a chronic inflammatory autoimmune disease characterized by progressive lymphocytic infiltration of the exocrine glands and other extra - glandular structures . dryness of the eyes ( xerophthalmia leading to keratoconjunctivitis sicca ) and mouth ( xerostomia ) constitute the typical clinical condition of this syndrome . although patients with ss have a lifespan not significantly different from that of the normal population , these patients carry a greater risk of developing lymphoproliferative malignancy . in addition , other organ malignancies can also occur in patients with ss such as lung , breast , gastrointestinal , gynecological , renal , and skin cancers . considering all these , the diagnosis of breast cancer in patients with ss can be mistaken as cancer at an advanced stage particularly in the presence of axillary multiple lymph nodes . in this study , we report a very rare case of breast cancer associated with ss in which the cancer was overstaged and , thus , overtreated on the assumption of clinically positive axillary lymph node status . a 45-year - old woman with a 12-year history of ss presented with a complaint of a breast mass . a breast examination revealed a palpable mass , measuring 10 cm in diameter , located in the upper lateral quadrant of the right breast and right axillary multiple lymph nodes . on ultrasonography , the mass was a 4 cm solid lesion located in heterogeneous breast parenchyma , and conglomerates of multiple axillary lymphadenopathy suspicious of malignancy were also detected ( figure 1 ) . the mammography was almost normal due to the dense parenchymal pattern , except for the presence of the axillary lymphadenopathy ( figure 2 ) . a core - needle biopsy was performed on the breast lesion , and the diagnosis of ductal carcinoma in situ ( dcis ) was made preoperatively . based on the clinical suspicion of an advanced - stage breast cancer , the patient underwent a modified radical mastectomy . on postoperative histopathological assessment , the diagnosis of 8-cm high - grade dcis was established , however no axillary lymph node metastases were detected ( figure 3 ) . ss is an autoimmune disease that causes chronic inflammation and primarily affects the exocrine glands . because it is a systemic disease , ss can also exhibit a wide range of clinical manifestations . among these , benign or malignant lymphoproliferation due to infiltration by lymphocytes and plasma cells may be a prominent part of this syndrome . several studies have shown a high incidence of malignancy in ss , the most common being lymphoma . other non - lymphoid cancers including breast cancer can also occur in patients with this syndrome ; however , an increase in the incidence of these cancers has not been demonstrated . lazarus et al . reported on a retrospective cohort study of 112 patients with ss in whom 25 developed malignancies , including three cases of breast cancer . in a recent study reported by zhang et al . , malignancy was detected in 29 ( 2.2% ) of 1,320 patients with ss , including four patients with breast cancer . the preoperative diagnosis of breast cancer in the presented patient with ss was established by radiological evaluation followed by breast biopsy . lesion morphology , mammary gland density , and the lack of imaging technique skills may limit the sensitivity for detecting breast cancer . as such , mammography may not demonstrate up to 20 - 30% of malignancies in a dense breast pattern , as we encountered in the present case . although the incidence of axillary metastases in patients with dcis is small , the evidence of axillary lymph node conglomerates on both physical and radiological examinations in this case led us to a clinical impression of a metastatic or " n2 " disease in the tnm staging definition , and that is why axillary dissection , in addition to a mastectomy , was performed . we reviewed the literature and did not find any report regarding the management of such patients with ss presenting with breast cancer and an axillary clinicopathological discrepancy . we recommend that lymph node status be verified either pre- or intra - operatively by lymph node biopsy using either fine - needle , core - needle , or frozen - section procedures before establishing a definitive diagnosis and appropriate surgical treatment . in conclusion , clinicians should remain vigilant to the possibility of overstaged breast cancer due to the false clinical impression of axillary metastasis in patients with ss presenting with breast cancer . therefore , axillary node status should be verified first to correctly establish tumor stage in this very rare clinical situation .

sjgren 's syndrome ( ss ) is an autoimmune disease that chronic inflammation and lymph node proliferation . patients with ss carry a greater risk of developing lymphoproliferative malignancy . in addition to other organ cancers , breast cancer may also occur in these patients . considering these , breast cancer in patients with ss can be misdiagnosed as being in an advanced stage particularly in the presence of axillary lymphadenopathy . here , we report a rare case of a 45-year - old woman with ss who presented with a breast mass . radiology showed a 4 cm solid lesion and conglomerates of axillary lymphadonepathy . a breast biopsy revealed ductal carcinoma in situ . a modified radical mastectomy was performed ; however , no axillary metastases were detected . clinicians should remain vigilant to the possibility that a false clinical impression of axillary metastasis may occur in such patients with breast cancer . therefore , axillary node status should be verified first .

although papilledema is commonly reported with hypoparathyroidism primary or secondary , but not reported commonly with php . a 10-year - old male child presented to our outpatient service with the complaints of blurring of vision , diplopia , and associated headache . patient also had clinical signs and biochemical parameters of hypocalcemia , along with normal parathyroid hormone ( pth ) levels . in cases of chronic papilledema , the assessment of the calcium serum level is a safe and simple method to exclude hypoparathyroidism or php . pseudohypoparathyroidism ( php ) is an inherited metabolic disorder characterized by end organ resistance to the action of parathyroid hormone ( pth ) . pth maintains serum calcium levels by promoting bone resorption , enhanced distal tubular reabsorption of calcium , and increased synthesis of 1,25-dihydroxyvitamin d , thereby causing enhanced intestinal calcium absorption . chronic hypocalcemia is responsible for increase in intracranial tension , thus causing papilledema , hence visual disturbances . a 10-year - old male child presented to our outpatient service with the complaints of blurring of vision , double vision , and dimness of vision . patient also had history of cramping pain of extremities with spasms more in lower limb and calf muscles . on clinical examination patient bilateral cataract was present and fundus was showing presence of papilledema [ figure 1 ] . fundus photograph of the patient before and after treatment biochemical analysis is shown in table 1 . in presence of clinical features and biochemical analysis showing hypocalcemia , urinary calcium was 3.57 mg / dl and urinary creatinine was 3.77 mg / dl , thus indicating renal tubular calcium loss . serum biochemistry of the patient in presence of hypocalcemia , renal tubular calcium loss and normal pth and aho phenotype , patient was labeled as a case of php . features of raised intracranial tension were resolved once patient was started on treatment with oral calcium , phosphate binders along with active vitamin d3 . php is called pseudo hypoparathyroidism because the blood chemistry values ( low to normal serum calcium and high serum phosphorous ) resemble the more common disorder hypoparathyroidism in which pth is absent or low , but pth is actually normal or elevated because the kidney can not fully respond to pth . a multifactorial etiology for bilateral disc edema can be postulated , such as impaired axoplasmic conduction due to reduced calcium and stasis , impaired central nervous system ( cns ) vascular autoregulation causing venous stasis , and raised intracranial pressure due to pseudotumor cerebri . the papilledema in hypocalcemia does not behave like that due to intracranial space occupying lesion or cerebral venous thrombosis . the disc edema readily responds to calcium therapy and disappears with calcium levels higher than 8 mg / dl . our patient also improved with normal fundoscopic findings [ figure 1 ] and clinical and biochemical [ table 1 ] improvement . in cases of chronic papilledema , the assessment of the calcium serum level is a safe and simple method to exclude hypoparathyroidism or php .

introduction : visual disturbance as a presenting feature of pseudohypoparathyroidism ( php ) is uncommon . although papilledema is commonly reported with hypoparathyroidism primary or secondary , but not reported commonly with php.description of the case : a 10-year - old male child presented to our outpatient service with the complaints of blurring of vision , diplopia , and associated headache . there was no history of seizure episode . patient had rounded face with a short , stocky built . shortening of the fourth metacarpal and fifth metatarsal was present . pitted nails and bilateral cataract . patient also had clinical signs and biochemical parameters of hypocalcemia , along with normal parathyroid hormone ( pth ) levels . consistent with pseudohypopathyroidism.conclusion:in cases of chronic papilledema , the assessment of the calcium serum level is a safe and simple method to exclude hypoparathyroidism or php .

miniature punch grafting is one of the easiest and cheapest office procedure for vitiligo with excellent repigmentation and minimal complications . in spite of the rapid ongoing advancement in its treatment , vitiligo remains one of the most common difficult to handle pigmentary disorders for a dermatologist , with a high degree of psychosocial impact on the patients . a large proportion of vitiligo patients do not respond to the conventional medical modalities probably because of the total absence of melanocytic reservoir in their lesional skin . in such cases , replacement of the achromic skin with newer source of melanocytes by surgical means , holds a good promise . among the various surgical modalities , miniature punch grafting is still regarded as the easiest and cheapest office procedure . with proper procurement and placement of grafts and good postoperative care , we report a case of stable vitiligo where target - like pigmentation occurred after miniature punch grafting , which is a rare and unique observation . a 14-year - old girl presented to us with asymptomatic depigmented macules in a segmental distribution over upper back of 5 years duration . the lesions had not shown any progression for the past 3 years and there was no evidence of koebner 's phenomenon . patient did not suffer from any other autoimmune disorders and her routine investigations and thyroid function tests were normal . she was previously treated with topical steroids , tacrolimus , and topical psoralen and sunlight ( puvasol ) without much improvement . as her lesions were stable and unresponsive , we performed miniature punch grafting after taking informed consent from the patient . punch grafts of 2.5 mm were taken from the gluteal skin while 2 mm punches were used to make the recipient chambers . a total of 27 grafts were put on the vitiliginous area . after achieving hemostasis , dressing was changed after 8 days , all grafts had taken up well and she was started on topical puvasol after 3 weeks . patient came for second follow up only after 4 months when perigraft pigment spread was seen around majority excepting few grafts . most of these sites showed a target - like pigmentation [ figure 1a and b ] . at the center there was the pigmented graft in place , surrounding this was a circular hypopigmented zone ( perigraft halo ) and then the zone of pigmented annulus indicating the spread of pigment from the donor graft . the successful repigmentation of achromic skin through punch grafting in vitiligo patients has been demonstrated by many authors . various reported complications include cobble stoning , sinking pits , polka dot appearance , variegated appearance , color mismatch , static graft ( no pigment spread ) , depigmentation of graft , perigraft halo , graft dislodgement / rejection , hypertrophic scar , and keloid formation . most authors have described perigraft halo as the peripheral rim of hypo / depigmentation , which persists after skin grafting in a vitiliginous patch . first , improper placement of donor grafts , that is , far from the margin of the patch so that the repigmentation does not extend till the margin . second reason could be grafting in cases of unstable / active vitiligo . as postulated by badri et al . in active vitiligo , the autoimmune process consisting of activated t cells is maximum at the margin of the lesion . these activated cd3 + , cd4 + , and cd8 + t cells express the cutaneous lymphocyte - associated antigen ( heca-452 + ) typical of infiltrating t cells . this occurs due to the contracture of the graft when it is harvested because of the contraction of the elastin fibers . in contrast to the above mentioned perigraft halo , the halo , which we found in our patient , was around the individual grafts . a possible reason for this could be circular scarring that occurred between the graft and the normal skin . savant mentions use of donor grafts 0.5 mm larger than the recipient bed to compensate for any graft contraction and prevent perigraft circular scarring . however , malakar and rajagopal et al . reported the use of same size punches with good results . in our patient , in spite of using 0.5 mm larger graft from the donor site , a thin rim of hypopigmentation appeared around many of the grafts , which was then surrounded by a zone of pigment spread . target - like pigmentation consisting of three zones , that is , central pigmentation surrounded by a rim of hypopigmentation and a peripheral zone of hyperpigmentation . the central zone ( a ) represented the graft in situ , whereas the rim of hypopigmentation ( b ) represented the junction of donor and the recipient skin . we can consider the remaining area as the fourth zone also , which actually represents the presurgical vitiligenous skin ( d ) . thus we can assume that the melanocytes travelled from the graft to the periphery through the rim of hypopigmented zone . this rim of hypopigmentation can be considered as a thin scar between the graft and the vitiliginous skin . the above pattern of pigmentation was observed despite the use of 0.5 mm larger graft from the donor site thus debating the use of larger donor grafts for vitiligo punch grafting . a- central pigmented zone , b- rim of hypopigmentation , c- peripheral pigmented zone , d- background vitiligenous skin we observed an unusual pattern of repigmentation following minipunch grafting , which resembled target - like lesions with a perigraft halo surrounding individual grafts despite the use of larger donor grafts .

surgical treatment for vitiligo has been ever evolving . each surgical modality has its own benefits and limitations . miniature punch grafting is the most extensively performed surgery , which gives good results in stable vitiligo . herein we report an unusual type of repigmentation observed after minipunch grafting in a patient of stable vitiligo , which resembled target - like lesions with a perigraft halo surrounding individual grafts . such pigment spread occurred despite the use of 0.5 mm larger graft from the donor site .

congenital anomalies of the coronary arteries , though uncommon , are an important cause of chest pain and in some cases can produce hemodynamically significant abnormalities , and even sudden cardiac death . malignant anomalous right coronary , with an incidence of 0.03 - 0.17% of patients undergoing angiography , is a rare form of such congenital anomalies . the development of electrocardiography ( ecg ) gated multidetector row computed tomography ( mdct ) allows accurate and noninvasive detection of coronary artery anomalies . knowledge of normal ct appearances of various anomalies and an understanding of their clinical significance is essential for correct diagnosis . a 38 year old male presented with complaints of chest pain , aggravated by physical stress , of more than four months duration . ct coronary angiography was performed using a 64 slice ct scanner ( brilliance-64 , philips , the netherland ) . the following parameters were used : 120 kvp , 800 mas , rotation time : 0.4 s , pitch : 0.2 mm and collimation : 64 0.625 mm . using a dual head pressure injector ( mallinckrodt ) , 80 ml of nonionic iodinated contrast ( iopamiro 370 , bayer , germany ) was administered at the rate of 5 ml / sec , followed by 20 ml of saline . the total scan time was 8.6 seconds . ct coronary angiography showed the right coronary artery originating from the left coronary sinus , coursing between the aortic root and pulmonary artery with a narrowed proximal segment . the rest of the coronary arteries , including the left main lca , the left anterior descending and the left circumflex were normal in course . maximum intensity projection of top of heart showing both right coronary artey ( rca ) and left coronary artey ( lca ) originating from left coronary sinus . rca has a slit - like ostium and courses between pulmonary artery ( pa ) and aorta ( a ) volume rendered image of same showing anomalous , interarterial course of right coronary artey ( rca ) , between pulmonary artery ( pa ) and aorta ( a ) anomalies of coronary arteries may be found incidentally in 0.3 - 1% of healthy individuals . the coronary artery anomalies can be classified into anomalies of origin , course or termination or as hemodynamically significant or insignificant . hemodynamically significant anomalies are characterized by abnormalities of myocardial perfusion , leading to increased risk of myocardial ischemia or sudden death . these include an anomalous origin of either the lca or rca from the pulmonary artery , an anomalous course between the pulmonary artery and aorta ( inter - arterial ) of either the rca arising from the left sinus of valsalva or the lca arising from the right sinus of valsalva , and , occasionally myocardial bridging or congenital coronary artery fistula . the rca arising from the left sinus of valsalva as a separate vessel or as a branch of a single coronary artery has an incidence of 0.03 - 0.17% of patients undergoing angiography . the anomalous origin may have inter - arterial , retro - aortic , prepulmonic or septal ( subpulmonic ) course , the most common being inter - arterial . the incidence of sudden death with this anomaly is estimated at 25 - 40% and is associated with exercise in half of the reported cases . various theories have been proposed for this association , including slit like ostium , acute angulation at the origin and compression of the vessel between the aorta and pulmonary artery . the choice of treatment for this congenital anomaly is still controversial ; however , because of significant hemodynamic consequences and propensity to cause sudden death , most of the literature advocates definite surgical revascularization in all cases , and the different options available include coronary artery bypass grafting , reimplantation of the coronary ostia and unroofing of the coronary artery , the last being considered the best solution if anatomically feasible . in japan , the treatment for this condition is conservative with the patient being treated medically with beta blockers . ct coronary angiography was made possible with the introduction of mdct and development of ecg gated scanning . for several decades , the imaging of coronary arteries was done with conventional angiography . however , it has been reported that anomalies of coronary arteries detected on conventional angiography were only 35% of those detected on 16 slice mdct . although coronary artery anomalies are far less common than acquired coronary artery disease , their propensity to cause premature cardiac morbidity and mortality especially in young adults make them significant . in a study by eikart et al . , cardiac abnormality was responsible for 51% of sudden deaths in young adults , with coronary artery abnormality being the most common cardiac abnormality ( 61% ) . in conclusion , we describe a 38 year old man with an anomalous right coronary artery , arising from the left sinus of valsalva and coursing between the aortic root and pulmonary artery . this case report emphasizes the role of non - invasive ecg gated mdct coronary angiography in accurate detection of coronary artery anomalies .

we report a case of an anomalous origin of a right coronary artery from the left coronary sinus with an inter - arterial course , between the aorta and the main pulmonary artery . this variant has been called malignant because of its association with sudden death , especially in young asymptomatic athletes . although these variants are rare , knowledge of cross sectional anatomy of the coronary arteries and their variants is critical , especially because some variants are associated with sudden death .

adult t - cell leukemia ( atl ) is an aggressive lymphoid proliferation associated with the human t - lymphotropic virus type i ( htlv - i ) [ 1 , 2 ] . htlv - i causes transformation and clonal expansion of t cells , resulting in atl in approximately 14% of the estimated 1020 million infected hosts , with a mean latency period of > 50 years . the diversity in clinical features and prognosis of patients with atl has led to its subclassification into the following 4 subtypes : smoldering , chronic , acute leukemic forms , and atl lymphoma . rare cases with intraocular invasion of acute - type atl have been reported [ 4 , 5 , 6 ] . we reported previously that flow cytometric analysis of vitreous samples is useful for the diagnosis of ocular sarcoidosis . here , we report a patient with intraocular invasion of smoldering - type atl after cataract surgery , which was confirmed by flow cytometric analysis , cytology , and pcr analysis of vitreous samples . this study was performed in accordance with the tenets of the declaration of helsinki , and the procedures were approved by the institutional review board of the kyoto prefectural university of medicine hospital and tohoku university graduate school of medicine . a 75-year - old man without any systemic disease underwent uneventful cataract surgery of the right eye . the best - corrected visual acuity was 1.0 on the next day , with normal postoperative findings . on postoperative day 6 , the patient presented with blurred vision in the right eye without any specific clinical findings such as ocular pain . his best - corrected visual acuity had decreased to 0.5 , and funduscopic examination revealed diffuse vitreous opacity ( fig . 1 ) . based on the clinical course and the ocular manifestations , postoperative bacterial endophthalmitis was suspected , and a pars plana vitrectomy was performed . a vitreous specimen was obtained at the start of a conventional 25-gauge pars plana vitrectomy . dry vitrectomy without perfusion of a balanced salt solution ( alcon laboratories , inc . , usa ) was conducted at a rate of 500 cuts per min so as not to damage cells infiltrating into the vitreous . after collecting 2 ml of a pure vitreous sample , an additional vitrectomy was performed under balanced salt solution perfusion in order to collect 10 ml of the diluted vitreous sample . genomic dna for herpes virus and other ocular pathogens was assayed in vitreous fluids using 2 independent pcr assays ( a qualitative multiplex pcr assay and a quantitative real - time pcr assay ) as described previously . a high level of cd4 and cd8 double - positive t cells was detected in the vitreous fluid but not in the peripheral blood ( fig . 2a , b ) , which is rarely seen in other cases of uveitis . based on the result of flow cytometry , atl or cytological examination of the vitreous specimen revealed flower cells that infiltrated into the vitreous ( fig . the proviral dna for htlv - i ( but not for cytomegalovirus or other types of infectious organisms ) was amplified in the pure vitreous sample . monoclonal t - cell receptor chain rearrangement was also detected by pcr in the pure vitreous sample ( fig . the proviral dna for the htlv - i was amplified in the serum sample by pcr and was not found by southern blot analysis . the rate of cd4 and cd8 double - positive t cells in the peripheral blood was low . as far as we are aware , this is the first report of a patient with intraocular invasion of smoldering - type atl triggered by cataract surgery . previously , a patient with smoldering - type atl was reported with severe unilateral intraocular inflammation at the time of acute crisis during the disease process without any intraocular surgery . in the present report , we speculate that the disruption of the blood - ocular barrier after cataract surgery triggered the intraocular infiltration of atl cells in our patient with smoldering - type atl . we surmise that the atl cells might be affected by postoperative inflammation and a special ocular environment where a high amount of transforming growth factor beta ( tgf- ) is found . based on this , in the present case we thought that infiltrating atl cells gradually proliferated after the operation into the ocular fluid , where there was a high amount of tgf-. in our patient , there was no recurrence even without additional treatment after vitrectomy . although this is a rare case , lymphocyte infiltrating disorder may be considered as a differential diagnosis in cases with diffuse vitreous opacity after intraocular surgery . thorough analysis of vitreous samples is essential for vitrectomy in vitreous opacity from unknown causes . flow cytometric , cytological , and pcr analysis of vitreous samples is beneficial for determining the cause of uveitis and endophthalmitis .

adult t - cell leukemia ( atl ) is an aggressive lymphoid proliferation associated with the human t - lymphotropic virus type i ( htlv - i ) . the intraocular invasion of atl is a rare event . a 75-year - old man without any systemic disease underwent uneventful cataract surgery of the right eye . on postoperative day 6 , the patient presented with blurred vision due to severe vitreous opacity in the right eye . analysis of the vitreous fluid revealed a suspected atl infection based on the flow cytometric analysis . moreover , cytological examination of the vitreous specimen revealed flower cell infiltration , and htlv-1 dna was detected by pcr analysis of the vitreous sample . monoclonal t - cell receptor chain rearrangement was also detected by pcr . thorough analysis of a vitreous sample is essential for vitrectomy in vitreous opacity of unknown cause . flow cytometric , cytological , and pcr analysis of vitreous samples is beneficial for determining the cause of this kind of severe illness .

. there are certain prophylactic and therapeutic indications for bilateral dj stenting like bilateral calculus obstruction , following bilateral lower ureteric reimplants , in cases of malignant ureteral obstruction and after bilateral uretero - renoscopy ( urs ) . after the purpose of stents is over , patients are subjected to dj stent removal . we present the technique of removing both the dj stents in one go with stent removal forceps obviating the need for inserting the cystoscope twice . 6 patients were subjected to bilateral dj stent removal from february 2011 to november 2011 after written and informed consent . indications for bilateral dj stenting were following bilateral urs in 4 patients and bilateral extracorporeal shock wave lithotripsy for pelvic calculi in 2 patients . rigid cystoscopy was done using 19 fr sheath and 30 degree lens ( karl storz , germany ) in all patients . 5 fr stent removal forceps is passed through the channel of the cystoscope bridge into the bladder . the bladder is kept partially filled , otherwise the two crossing stents will diverge apart . the two stents appear to be crossing like a x when seen cystoscopically [ figure 1 ] . with the help of stent removal forceps , both the dj stents are grasped at the point of crossing i.e. at the mid - point of x and retrieved simultaneously along with the cystoscope [ figure 2 ] . cystoscopic appearance of two dj stents crossing like a x cystoscopic appearance of 5 fr stent removal forceps grasping both the dj stent at the mid point of x ( or at point of crossing ) and being retrieved all the patients underwent successful removal of both the dj stent in one go without any complications . mean operative time from introduction of cystoscope till complete removal of bilateral stents simultaneously was 1 min 45 secs . it was ensured by imaging studies ( x - ray and usg kub ) in all patients , prior to dj stent removal that there will not be any chance of recurrent obstruction on either side . we recommend that our technique should be practiced in calculus disease after confirmation of stone clearance . it is not recommended for simultaneous removal of bilateral dj stents in cases of bilateral lower ureteric reimplants and in situations of malignant ureteral obstruction wherein the usual practice is to remove / replace one side stent followed by another . the dual dj removal concept is more relevant when dj stent is being removed with the help of flexible cystoscope in which case the entire scope needs to be passed again to retrieve the second dj stent . other described simplified techniques for stent retrieval include a nylon thread attached to the end of the stent , which dangles from the external meatus , enabling manual withdrawal of the stent without anesthesia . this solution is for the short term only because the dangling suture some - times causes unintentional dislodgment of the stent and may also be associated with a slight degree of incontinence . also , a wire introducer with a snail head coil at its distal end has been described for blindly grasping the stent within the bladder of women only . magnetic retrieval of stents by using a magnet on the retrieval urethral catheter and attaching a magnetically attractive , stainless steel bead to the end of a stent has also been used in the past . all these methods may be applied for removal of both stents simultaneously but their simplicity appears questionable . first , we have not compared the operative time with the standard technique of removing one stent after other . secondly , some may argue that during rigid cystoscopy , stents can be removed in succession with sheath left in situ but in our opinion removing stents with endoscope alone may damage the delicate instrument . our technique holds a special value in the era of flexible cystoscopy where repeated instrument manipulation for retrieval of stents can be avoided . our technique is a simple method of removing both the dj stents simultaneously in one go and thus , avoid the need of reinserting the scope to remove the second dj stent and logically , saves operating time .

there are many urological and non - urological indications which require bilateral double j stenting . we describe a point of technique for simultaneous removal of both the double j stents . both the stents are held by stent removing forceps at a point where they cross each other and then removed in one go with the help of cystoscope . medline search did not reveal any techniques of removing two dj stents in one go .

the aberrant anatomy has been reported in up to 17% of cases with bile duct injury . one such rare anomaly is congenital absence of the common bile duct ( cbd ) . in our case , the cbd was absent and the common hepatic duct ( chd ) was opening into the gallbladder ( gb ) through which bile was draining into the duodenum through the cystic duct ( cd ) . a 36-year - old lady was presented to us with symptoms of abdominal pain ; ultrasonography ( usg ) of the upper abdomen suggested cholelithiasis and the cbd was reported as normal . dissection was performed posterior to the cd and the cbd could not be seen in the porta . in view of the unclear structural anatomy , we started mobilizing the gb by fundus - first method . during dissection , laparoscopic surgery was terminated and converted to open , and it was found that a duct was opening into the gb wall through the bed and no cbd was present . intraoperative cholangiogram was performed through the ductal opening in the gb fossa that revealed both right and left biliary ductal systems ; the anomaly was confirmed as congenital absence of the cbd , with bile draining into the gb through the chd and gb draining into the duodenum by the cd . cholecystectomy was performed and roux - en - y hepaticojejunostomy ( hj ) was performed with chd [ figure 1 ] . postoperative course of the patient was uneventful , and she was discharged on the 4 postoperative day . congenital absence of the cbd is a rare anomaly of the extrahepatic biliary tract with right and left biliary radicals directly opening into the gb . in this situation , cd is the sole pathway for the biliary drainage . the possible explanation for this condition could be the presence of persistent connections between the foetal hepatic ducts and the gb with failure of complete recanalization of the extrahepatic biliary tract . variants of interposition of the gallbladder the differential diagnosis for this clinical condition could be mirizzi 's syndrome type iii / iv and choledochal cyst . mirizzi 's syndrome can be distinguished by the presence of signs of inflammation in the calot 's and the presence of large impacted stone in the gb neck . awareness of this anomaly and performing cholangiogram at the slightest suspicion are the possible intraoperative methods of the diagnosis . if detected intraoperatively , gb resection with roux - en - y hj ( most preferred ) , partial cholecystectomy with closure of residual gb over t - tube ( cholecysto - choledochoplasty ) or end - to - end chd cholecysto - choledochoplasty could be a preferred option in type iii b but there is always a risk of recurrent stone formation in the residual gb leading to biliary obstruction and end - to - end chd if the diagnosis is known preoperatively , cholecystojejunostomy can be a possible option as well .

congenital absence of the common bile duct ( cbd ) is an extremely rare developmental anomaly with right and left hepatic ducts draining directly into the gallbladder ( gb ) . other synonyms for this clinical condition are cholecystohepatic ducts , transverse lie of the gb or interposition of the gb . the potential for iatrogenic injury is high , because of either inadvertent division or ligation of the ducts . diagnosis is mostly made intraoperatively , and needs some form of biliary reconstruction . herein , we are reporting a case of congenital absence of the cbd in a 36-year - old lady that was detected intraoperatively .

in the previous issue of critical care , meaudre and colleagues measured bedside rapid assay b - type natriuretic peptide ( bnp ) levels daily , and performed bedside echocardiography in patients admitted to hospital for subarachnoid hemorrhage ( sah ) . they found that bnp levels rose in 25/31 ( 81% ) patients , peaking at day 2 ( at a mean of 126 ng / ml ) and tapering off by day 7 . importantly , bnp levels did not correlate with left ventricular ( lv ) filling pressure as estimated by echocardiography . bnp levels did correlate with cardiac troponin i levels at day 2 and day 3 ( r = 0.63 , p < 0.001 and r = 0.44 , p = 0.05 , respectively ) . the authors therefore concluded that bnp can not estimate lv filling pressure in sah patients but does correlate with myocardial necrosis as assessed by cardiac troponin i levels in these patients without prior hypertensive or cardiac disease . what is the mechanism of bnp release in such patients ? as meaudre and colleagues mention , intramyocardial norepinephrine release , possibly resulting in myocardial necrosis , appears to be a plausible mechanism of bnp release and perhaps explains the correlation of bnp with troponin i levels . it should be noted that bnp levels , putatively correlating with intramyocardial norepinephrine levels , do not necessarily correlate with serum norepinephrine levels , which makes sense given the lack of clinically detectible myocardial injury ( lack of decrease in lv ejection fraction , lack of new wall motion abnormalities ) in these patients . there are several important caveats many of which the authors mention that must be considered when interpreting meaudre and colleagues ' data . first , all patients with known hypertensive or cardiomyopathic disease were excluded from the study . the extent of neurocardiogenic injury associated with sah in patients with known cardiovascular disease is therefore unknown and could arguably be more serious . second , although echocardiographic diagnosis of the lv filling pressure has significant precedent and is clinically useful , the diagnosis remains inferior to direct hemodynamic data from right or left heart catheterization , which was not performed in this study . third , and importantly , the 6/37 ( 16% ) patients with sah who died prior to the day 7 follow - up studies were excluded from the final analysis , and consequently it is unknown whether bnp or troponin i levels had any prognostic or mechanistic effect in these expired patients . add to the growing body of evidence that bnp ( and n - terminal pro - bnp ) are poor predictors of lv filling pressure in critically ill patients . in critical care patients with indwelling pulmonary artery catheters , it has previously been demonstrated that bnp levels have a weak correlation ( r = 0.32 ) with the pulmonary capillary wedge pressure . in another study of 40 critical care patients , both bnp ( r = 0.40 ) and n - terminal pro - bnp ( r = 0.32 ) had weak correlations with the pulmonary capillary wedge pressure and were dependent on renal function . another group has shown that , in 249 critically ill patients , those with congestive heart failure confirmed by invasive hemodynamic measurements had bnp and n - terminal pro - bnp levels comparable with patients with sepsis and without congestive heart failure . yet another study showed that bnp levels were increased in critically ill patients with severe sepsis or septic shock regardless of the presence or absence of heart failure . the body of evidence therefore indicates that bnp and n - terminal pro - bnp should not be used to diagnose systolic or diastolic heart failure in critically ill patients . is this the death knell , then , for the use of bnp in critical care ? from a diagnostic point of view , yes bnp and n - terminal pro - bnp levels do not provide useful , cardiac - specific information given that sepsis , trauma or congestive heart failure can result in similar bnp levels in critically ill patients [ 6 - 8 ] . from the prognostic point of view , however , the role for cardiac peptides could be more promising . while one study in patients with sepsis showed that bnp levels did not predict inhospital mortality or length of stay , two other studies in unselected critically ill patients showed that hospital nonsurvivors had significantly higher nt - pro - bnp values than hospital survivors . a potentially important prognostic role therefore remains for cardiac peptides in critically ill patients , an area that requires further study ; however , the diagnostic role of bnp and n - terminal pro - bnp in such patients appears to be very limited indeed . bnp : b - type natriuretic peptide ; lv : left ventricular ; sah : subarachnoid hemorrhage .

although b - type natriuretic peptide ( bnp ) has been used for the diagnosis of congestive heart failure in many clinical settings , its diagnostic role in critically ill patients remains uncertain . the body of literature suggests that bnp and n - terminal pro - bnp levels are not useful for the diagnosis of systolic or diastolic heart failure in the critically ill , including in patients with brain hemorrhage , due to poor specificity . however , these cardiac peptides may have a more promising prognostic role in this patient population .

in contrast , vasculitis of the coronary arteries has only been rarely documented [ 35 ] . here , we present images of repeated coronary angiography in a young patient with churg strauss syndrome demonstrating complete resolution of severe coronary vasculitis after 1 year of therapy with prednisone and cyclophosphamide . an 18-year - old man with a history of asthma was admitted to a local hospital because of fever , profound headache , weight loss , red eyes , blurred vision , and generalized muscle aches . all symptoms resolved spontaneously within 1 month before a classifying diagnosis could be made . five months later , the headache and blurred vision recurred , and the patient was referred to our outpatient clinic . laboratory analysis at that moment revealed only mild eosinophilia ( 11% ; 600 10/l ) . renal function was normal , and anti - neutrophilic cytoplasmatic antibodies and anti - nuclear antibodies were not present . cranial magnetic resonance imaging showed a unilateral thickening of the choroidal cerebral plexus , and analysis of the cerebrospinal fluid revealed eosinophilic meningitis . another 3 months later , the patient developed a persistent fever and a pulmonary infiltrate that did not respond to antibiotics . broncho - alveolar lavage revealed profound eosinophilia ( 30% ) . during admission , eosinophilic skin infiltrates and generalized edema developed . now , we considered the diagnosis of churg strauss syndrome , and therapy with prednisone at a dose of 1 mg / kg was initiated . subsequently , the patient developed a painful injected pharynx and chest pain , with diffuse st segment elevation on electrocardiography . intravenous gammaglobulin ( 400 mg / kg per day for 5 days ) was now administered . as the chest pain recurred and there was a rise in the plasma troponin - i concentration , a coronary angiography was performed . mri scanning excluded involvement of the aorta and its major branches . in view of the potentially fatal coronary lesions , a more powerful immune - suppressive regimen consisting of methylprednisolone and cyclophosphamide ( 750 mg and 1,250 mg i.v . per day during 3 days , followed by oral doses of 1 mg / kg / day prednisone , and 2 mg / kg / day of cyclophosphamide ) was chosen . the cyclophosphamide was tapered and stopped after 16 months . a control coronary angiography 1 year after initiation of therapy showed a complete regression of all lesions ( fig . 1left coronary angiogram in right inferior oblique view before ( a ) and after ( b ) 1 year of immunosuppressive therapy with prednisone and cyclophosphamide . before the treatment , there were two prominent aneurysms in the intermediate branch and one large aneurysm proximally in the obtuse marginal branch ( a , indicated by white arrows ) in addition to diffuse wall irregularities in all branches . after the treatment , all these lesions have disappeared completely ( b ) left coronary angiogram in right inferior oblique view before ( a ) and after ( b ) 1 year of immunosuppressive therapy with prednisone and cyclophosphamide . before the treatment , there were two prominent aneurysms in the intermediate branch and one large aneurysm proximally in the obtuse marginal branch ( a , indicated by white arrows ) in addition to diffuse wall irregularities in all branches . after the treatment , all these lesions have disappeared completely ( b ) our patient fulfilled the classification criteria of the churg strauss syndrome as established by the american college of rheumatology ( asthma , eosinophilia , pulmonary infiltrates , and a biopsy containing accumulation of eosinophils ) , although he also had signs and symptoms compatible with other vasculitis syndromes , such as kawasaki disease ( bilateral conjunctival injection , injected pharynx ) . endocardial thickening due to local infiltration of eosinophils , which can trigger intracardial thrombus formation , is the most common presentation of heart involvement in patients with the churg pericardial involvement , presenting either as an acute pericarditis with pericardial effusion , as in our patient , or as chronic constrictive pericarditis , has been reported in 832% of patients . in contrast , clinically significant vasculitis of the coronary arteries has been reported only anecdotically [ 35 ] . in general , symptomatic cardiac involvement is associated with poor prognosis in patients with medium or small vessel vasculitis and justifies a strong and immediate immunosuppressive treatment . indeed , the present case report shows that therapy with prednisone and cyclophosphamide may lead to a complete and uneventful recovery of potentially fatal coronary artery lesions .

the heart is involved in up to 50% of all patients with churg strauss syndrome , but vasculitis of the coronary arteries has only been rarely documented . we present a young patient with severe coronary aneurysms and stenotic lesions due to a churg strauss vasculitis . prompt therapy with prednisone and cyclophosphamide resulted in the complete resolution of all lesions .

angiosarcoma is a rare malignant neoplasm of endothelial cells , which represents less than 2% of all soft tissue sarcomas . despite primary angiosarcoma of spleen being extremely rare , it is the most common primary nonlymphoid malignant lesion of spleen . till date we report a case of primary angiosarcoma of spleen presenting as on and off fever and anemia . a 60-year - old male presented with complaints of on and off fever and lump in left upper abdomen of 1 month duration . physical examination revealed pallor . there was a 10 7 cm lump ; 5 cm below left costal margin in midclavicular line . laboratory evaluation showed anemia and leukocytosis ; electrolyte and liver function tests were with in normal limits . general blood picture was suggestive of hemolytic anemia with spherocytes , target cells , and howell jolly bodies . abdominal sonography and computed tomography ( ct ) showed splenomegaly with heterogeneous enlargement , subcapsular hematoma with multiple ill - defined nodules in the splenic parenchyma with minimal ascites , and left pleural effusion [ figure 1 ] . computed tomography scan showing splenic enlargement with subcapsular hematoma suspecting a splenic trauma in a congested enlarged spleen , a laparotomy with splenectomy was performed . on gross examination , spleen was enlarged in size with cut surface showing a circumscribed grey white area with diffuse hemorrhagic areas and multiple ill - defined nodules [ figure 2 ] . microscopic examination showed the replacement of whole of the spleen by a vascular neoplasm composed of anastomosing vascular channels lined by atypical endothelial cells . these papillary fronds were lined by pleomorphic endothelial cells with pleomorphic hyperchromatic nuclei showing mitotic figures [ figures 3 and 4 ] . gross specimen showing the cut surface of the spleen photomicrograph showing vascular neoplasm composed of anastomosing vascular channels ( h and e , 100 ) photomicrograph showing a vascular neoplasm composed of anastomosing vascular channels lined by pleomorphic endothelial cells ( h and e , 400 ) photomicrograph showing immunohistochemistry with cd31 showing membranous positivity primary angiosarcoma is an extremely rare neoplasm with reported incidence of 0.140.23 cases per million . it may develop at any age , however , the mean age at presentation reported in the literature is 59 years ranging from 14 months to 89 years[24 ] with a slight predominance in men . splenic rupture occurs in 13%32% of patients and presents as the most serious manifestation in most of the cases . it is the worst prognostic factor because it puts the patient at an increased risk of peritoneal dissemination . fever as an associated symptom has been observed in nearly 10% of the patients with angiosarcoma . sun et al . investigated splenectomy in 35 patients of pyrexia of unknown origin and found 1 case ( 2.9% ) of splenic angiosarcoma . ( 1981 ) , where it was present in almost all of the 4 cases . hemolytic anemia with angiosarcoma has been previously reported in a patient with vinyl chloride exposure , it was also present in 3 out of 4 cases in the series of sordillo et al . radiologically , splenic angiosarcoma presents as splenomegaly ; with solitary or multiple heterogeneous mass lesions with increased echogenecity and poor margins . associated hepatic lesions or retroperitoneal lymph node enlargement may also be seen . besides these a subcapsular hematoma was also identified on ultrasonography in our case ; however , ct scan abdomen showed a characteristic picture consisting of splenomegaly with heterogeneous contrast enhancement and subcapsular hematoma with multiple ill - defined nodules in the splenic parenchyma , suggesting a diagnosis of splenic angiosarcoma with subcapsular rupture of the spleen . radiologically , splenic angiosarcoma mimic portal hypertension at times , only 1 case of primary hemangiosarcoma associated with documented portal hypertension and functional splenic aplasia has been reported in the literature . polydrug chemotherapy , including doxorubicin , daunorubicin , and vincristine , has been proposed to offer benefit . primary angiosarcoma of spleen is a very aggressive neoplasm with high metastatic rate and poor prognosis with mean survival time 10.314.4 months . concomitant malignancies are found in 5% of the patients and include carcinoma breast ; colorectal carcinoma and non - hodgkin 's lymphoma . splenic angiosarcoma is a rare tumor of the spleen and often mimic splenic trauma and hemorrhage , high degree of suspicion , and a laparotomy with splenectomy often helps in establishing the diagnosis .

splenic angiosarcoma is a rare neoplasm that often remains asymptomatic till the onset of massive intra abdominal bleeding that require emergency splenectomy . we report here a case of 60 year old male who presented with on and off fever and a splenomegaly was found on clinical examination suggesting a lymphoproliferative disorder . a contrast enhanced computerized tomography suggested splenic trauma with intracapsular bleed . a splenectomy was carried out that revealed splenic angiosarcoma . splenomegaly and fever is a very rare presentation of angiosarcoma . though rare it should be kept in mind when investigating pyrexia of unknown origin .

the use of central venous saturation ( scvo2 ) to guide haemodynamic management is an important and evolving aspect of clinical practice . an observational study published in this issue of critical care has advanced our understanding of this form of monitoring by exploring the association between derangements in scvo2 and complication rates after major abdominal surgery . this study provides a detailed description of peri - operative trends in scvo2 and confirms the findings of previous work which suggests that reductions in scvo2 are associated with increased post - operative complication rates . although the study is relatively small , the robust multi - centre approach and consistency with previous work support the applicability of the findings . the comparative simplicity of scvo2 measurement makes this an attractive technique . with the blood gas analysis technology available in most institutions , intermittent scvo2 monitoring can be performed in any patient with a central venous catheter . however , it is not yet clear whether scvo2 measurement through intermittent blood sampling is an adequate alternative to continuous monitoring with a fibre - optic catheter . interest in scvo2 measurement is not new , and several reports have explored the physiology and clinical significance of this parameter over the past 50 years . of these these authors used a scvo2 value of 70% as a target for goal - directed haemodynamic therapy ( gdt ) in patients presenting to hospital with severe sepsis and septic shock . they demonstrated that it may be possible to achieve substantial mortality reductions without the need for complex or invasive cardiac output monitoring technology . the success of rivers ' work and several trials of peri - operative gdt indicates that the use of scvo2 as a haemodynamic goal may be equally valuable in surgical patients [ 5 - 8 ] . however , several questions must be considered before embarking on an interventional trial of scvo2-guided peri - operative gdt . the study by the collaborative study group is important because it sets out to address some of these key questions . the value of scvo2 in any given patient reflects not only oxygen delivery but also oxygen consumption . reductions in scvo2 may therefore reflect a large number of acute changes in physiology including hypoxia , shivering , anaesthesia , haemorrhage and myocardial ischaemia . the therapeutic approach to achieving the target value may need to include more than simply intravenous fluids and inotropic therapy . if a period of post - operative sedation and invasive ventilation is required to control oxygen consumption , would such an intervention be valid ? although the normal value of scvo2 is often quoted as 70% , there are in fact few published data to confirm this , either in healthy volunteers or in surgical patients . previous observational work shows that considerable variations in scvo2 may occur depending on the nature and severity of the acute physiological disturbance . it would be naive simply to accept this ' normal ' value as being optimal in every clinical situation . their findings suggest that a higher target value of 75% would be more appropriate in patients undergoing major abdominal surgery . this finding is consistent with the analysis of scvo2 data from a recent interventional trial of post - operative gdt . however , both these studies have shown that large decreases in scvo2 occur immediately after surgery . it is unclear whether such changes , which are more marked in those patients who develop complications , relate predominantly to an increase in oxygen consumption , a decrease in oxygen delivery or , more probably , a failure to increase delivery to match increased consumption . what is more , these observations raise the possibility that the most appropriate goal for scvo2 may vary during and after surgery . several recent successful gdt trials have opted for short periods of early treatment lasting between 4 and 8 hours . however , gdt has also been effective when administered for periods of up to 24 hours . as with any monitoring technology anecdotal evidence suggests that clinicians have a limited understanding of the pitfalls associated with scvo2 measurement , which may lead to a number of problems in practice . for example , the aggressive targeting of too high a value for scvo2 may be harmful , particularly in the elderly . the authors make an important point in suggesting that the targeted value for scvo2 should be modified for different patient groups . in particular , the presence of cytopathic hypoxia in septic patients may result in a high value of scvo2 despite low oxygen delivery . venous oxygen saturation differs between the superior vena cava and the right atrium , and the value of scvo2 may therefore vary according to the position of the catheter tip . despite the promising findings of this most recent work , the routine peri - operative use of scvo2-guided gdt can not be recommended until a large randomised trial has confirmed the value of this approach . gdt = goal - directed haemodynamic therapy ; scvo2 = central venous oxygen saturation ; csg = collaborative study group .

measurements of central venous oxygen saturation ( scvo2 ) have been successfully used to guide haemodynamic therapy in critical care . the efficacy of this approach in the treatment of severe sepsis and septic shock has stimulated interest in the use of scvo2 to guide management in patients undergoing major surgery . the physiological basis of scvo2 measurement is complex . a number of outstanding issues will need to be resolved before incorporating scvo2 measurement into routine practice . first , it is not yet clear which value of scvo2 should be targeted . second , there is some uncertainty as to which interventions are the most effective for achieving the desired value of scvo2 or how long this value should be maintained . the study by the collaborative study group on perioperative scvo2 monitoring published in this edition of critical care may help provide answers to some of these questions . our understanding of scvo2 measurement remains limited , however , and the routine use of peri - operative scvo2-guided goal - directed therapy can not be recommended until a large randomised trial has confirmed the value of this approach .

a 69-year - old man underwent primary prevention dual - chamber icd ( fortify assura , st jude , minn , usa ) insertion due to ischemic cardiomyopathy . the current implant was performed on the right side following optimal antibiotic treatment . both leads ( atrial lead : tendril sts pacing lead , st jude med , and ventricular lead : durata defibrillation lead , st jude med ) were active fixation . three months later , the patient presented to clinic for routine check - up and atrial lead sensing and impedance significantly deteriorated . chest radiograph showed that the atrial lead had been retracted back into the superior vena cava . rv lead tip was still on the rv mid - septum as was placed at implantation ( fig . 1 ) . the device was programmed to the vvir mode and the patient was arranged for elective surgery for atrial lead revision . the patient underwent elective atrial lead revision one month later and fluoroscopic imaging on the day of procedure revealed atrial lead coiled in the device pocket , however ; right ventricular lead remained in place ( fig . 2 , panel a ) . extracted atrial lead showed a damaged helix ( fig . 2 , panel b ) . we revised the atrial lead ( tendril sts pacing lead , st jude med ) and again secured the icd to the underlying tissue in the base of the pocket to prevent this from recurring . the reel syndrome , a recently described form of twiddler 's syndrome , occurs due to the rotation of the pacemaker generator on its transverse axis with subsequent coiling of the pacemaker lead(s ) around the pulse generator . when only one of the leads get retracted or displaced , this phenomenon is called selective reel syndrome . there are few similar cases in the literature showing atrial lead displacement only , however the rv lead remained in place , , , . the authors have explained this phenomenon with speculation that implanters subconsciously tighten the rv lead sutures more firmly than atrial lead given the fact that rv lead is the life - saving lead . other reasons to be considered are : female gender , large pocket , obesity , children , elderly people and dementia . the consequences of atrial lead displacement can be significant and could be listed as following : diaphragmatic nerve stimulation , loss of capture , inappropriate therapies due to either loss of atrial sensing and lack of better tachycardia discrimination or ventricular oversensing due to atrial lead dislodged to the ventricle , and even death . in our case , patient presented with phrenic nerve stimulation . in our case , only atrial lead was displaced , however the rv lead was completely stable . we remained uncertain whether the helix broke during traction or within the pocket during extraction .

oblivious manipulation of the device by the patient is referred to as twiddler 's syndrome . it is characterized by the coiling of the pacemaker lead due to the rotation of the pacemaker generator on its long axis . however , the rotation of the pacemaker generator on its transverse axis with subsequent coiling of the pacemaker leads around the pulse generator is called as reel syndrome. in this case , we present a 69-year - old patient with selective reel syndrome and postulate the possible explanation for damaged atrial lead .

ahei , also called postinfectious cockade purpura , finkelstein 's disease and seidlmayer 's disease is a leukocytoclastic vasculitis characterized by inflammatory acral edema and ecchymotic purpura in a target or cockade pattern usually without visceral involvement . the cutaneous findings of ahei resemble those of henoch - schnlein purpura ( hsp ) . although some have suggested considering ahei a purely cutaneous variant of hsp , most authors regard it as a separate entity amongnst cutaneous small vessel vasculitis of childhood . the recognition of ahei allows making the diagnosis of a benign entity rather than a more serious disease . an 18 month - old girl was referred from the paediatric department for purpuric skin lesions since 3 days . she had an upper respiratory infection 5 days prior to the development of the skin lesions . her mother reported seeing reddish colored spots initially on the legs which gradually increased to involve the face , legs and arms . the cutaneous examination showed many symmetrically distributed , round and oval , ecchymotic , purpuric , targetoid plaques localized on the cheeks and extremities , in particular lower limbs . some plaques showed a cockade pattern [ figure 3 ] , while others coalesced to form large purpuric lesions with polycyclic borders . moderate non - pitting edema was noted over her hands and feet [ figure 4 ] . the remainder of the physical examination was unremarkable and there were no signs of systemic involvement . urinalysis , liver and renal function test , and complete blood count were within normal limits . round to oval purpuric targetoid plaque on the face with redness and edema of right pinna violaceous plaques on both soles close up of targetoid plaque on cheek inflammatory acral oedema histopathology showing fibrinoid vessel wall necrosis with perivascular neutrophilic infiltrate and leucocytoclasis the clinical features of our case , characterized by a dramatic onset of typical symmetrical , annular purpuric plaques on the face , ears and limbs , histology showing leukocytoclastic vasculitis and a spontaneous recovery within two weeks , were consistent with the diagnosis of ahei . a clinical disorder corresponding to ahei was first described in 1913 by snow , et al . who reported a patient as purpura , urticaria and angioneurotic oedema of the hands and feet in a nursing baby . ahei is a disease that affects children between 4 and 24 months of age and is characterized by a triad of fever , large palpable purpuric skin lesions , and edema . classically , there is a contrast between the dramatic skin findings and relative absence of ill health . it starts with a sudden appearance of well demarcated , annular , medallion - like , rosette shaped purpuric plaques , almost entirely limited to extremities and face , with relative sparing of trunk . fever and painful edema of the distal extremities , ears , and eyelids are associated features of ahei . since there are no diagnostic laboratory tests , the history and physical examination provide clues to the successful recognition of the disease . its aetiology remains unknown , although a history of recent upper respiratory or urinary tract infection treated with antibiotics or immunizations is found in 75% of patients . the most reported infective agents include staphylococci , streptococci and among viruses , adenovirus , although many other agents , such as escherichia coli , or mycobacteria , have been reported . a history of drug intake before the onset of the cutaneous eruption is present in many cases of ahei . visceral involvement is rare but it has been reported involving the kidneys and intestines , causing symptoms such as hematuria , mild proteinuria , and bloody diarrhea . elevated erythrocyte sedimentation rate , leucocytosis , thrombocytosis , and eosinophilia have been seen in ahei . histologic features of ahei are consistent with small - vessel vasculitis of both capillaries and post capillary venules of the upper and the middle dermis , showing typical leukocytoclastic vasculitis with or without fibrinoid necrosis and a deep perivascular and interstitial infiltrate composed mostly of neutrophils with abundant nuclear dust . the disease runs a benign course with complete spontaneous recovery occurring in 1 - 3 weeks , although relapses have been reported rarely . treatment with oral corticosteroids has been reported , but this is unnecessary due to its spontaneous recovery . it is important to remember that purpura in infants needs complete work up to exclude more serious conditions like hsp , meningococcemia , septicemia , kawasaki disease , erythema multiforme and purpura fulminans . amitai et al . suggested that ahei can not be considered a distinctive syndrome , but a benign variant of hsp pointing out a similar pathogenesis of both diseases , but a different distribution of purpuric lesions and with predilection of the face in infants ( ahei ) and buttocks and lower extremities in older children ( hsp ) . in addition , they postulated that the proportionally larger head and face in infant with a corresponding increase in blood supply would render them more susceptible to facial purpura . cases of ahei and hsp overlap have also been reported . however , most authors consider these to be distinct clinical and pathological entities .

a patient presenting with fever and purpura often presents a diagnostic challenge for the dermatologist . acute hemorrhagic edema of infancy ( ahei ) is a rare acute cutaneous leukocytoclastic vasculitic syndrome of infants leading to this presentation . we present an 18-month - old girl with ahei who presented with fever , edema , and purpuric lesions involving the face , ears and extremities with uneventful complete recovery .

the occurrence of squamous cell carcinoma in actinic keratosis is well - known and has been documented time and again . non - melanoma skin cancer ( nmsc ) is the most common variant of cutaneous malignancy , white population being the most susceptible . photo - protective effect of eu - melanin seemed to have a role to play in determining its pattern of incidence . although its worldwide incidence is skewed toward basal cell carcinoma ( bcc ) , dark - skinned indians are less prone to develop the condition . however , in the settings of genodermatoses , comprising oculo - cutaneous albinism ( oca ) , and hermansky - pudlak syndrome ( hps ) , bcc may be a relatively common finding . reduction / complete absence of melanin may lead to photosensitivity and high - risk cutaneous signs such as freckles , actinic keratosis , and bcc . here we report an indian native of oculo - cutaneous albinism , freckling , actinic keratosis , and ulcerative bcc . a 56-year - old farmer , a known case of type ia oca , presented with the complaint of discomfort in daylight , diminished vision , and a slowly progressive asymptomatic , red , raised eruption over the back for the past 10 years . he had irregular , dark , flat lesions , initially overexposed parts , which later spread to cover the whole body . some of the flat lesions turned darker in color , hard , and raised with the increasing age . skin surface examination , was conspicuous by the absence of color in the skin ( chalky white ) , hair , and eyes . multiple irregular , brown - to - amber color branched macules , the dendritic freckles were located over exposed and covered parts of the skin . in addition , a keratotic , speckled pigmentation plaque with rolled out margins of the size of 5 - 7 cm was present over the left scapular region [ figure 1a ] . smaller lesions of similar morphology were also identified over the arms and pre - auricular area [ figure 1b ] . there was no evidence of pallor , cyanosis , icterus , lymphadenopathy , and organomegaly . ( a , b ) basel cell carcinoma occupying left scapular region of the back and pre - auricular region of the face complete hemogram , liver and renal function tests were unremarkable . hematoxylin - eosin - stained sections prepared from the biopsy taken from the plaque revealed a basaloid proliferation of cells arranged in nests , with peripheral palisading [ figure 2 ] . retraction artifact was seen separating some of these cell nests from the surrounding stroma [ figure 3 ] . excision biopsy of the keratotic plaque from the auricle revealed keratinocyte atypia , solar elastosis , and melanophages in the dermis consistent with actinic keratosis [ figure 4 ] . proliferation of basaloid cells disposed in nests in the dermis , ( h and e , 40 ) sections prepared from the biopsy from an exophytic plaque . higher magnification depicting prominent peripheral palisading , and retraction space , ( h and e , 100 ) sections prepared from the biopsy of the keratotic plaque from the auricle . note the keratinocyte atypia in the lower two - thirds of the epidermis ; and melanophages , and solar elastosis in the dermis , ( h and e , 40 ) for the darker races , where melanin confers natural photo - protection , albinism poses as an established risk attribute for all three forms of cutaneous malignancies . although squamous cell carcinoma ( scc ) is usual , basal cell carcinomas have also been reported in albino . however , recent studies have incriminated basal cells in the histo - genesis of actinic keratosis . another fascinating entity that has emerged in recent times is baso - squamous cell carcinoma , where coexisting features of both scc and bcc have been encountered . such a case has also been reported in an albino indian . in our case , the clinical picture , in the setting of albinism and cutaneous features of photo - damage suggested a diagnosis of scc , but consideration of the sites of involvement , duration and absence of metastasis suggested otherwise . our case represents the documented cohabitation of cutaneous signs of photo - damage , actinic keratosis , dendritic freckles in the perspective of oca . the occurrence of bcc , therefore , is fascinating for it is infrequently reported in these settings . oca is an established risk factor for all the three major forms of skin cancers , due to reduction and/or complete absence of melaninalthough scc is common , the presence of bcc is not that infrequent and should be suspectedirrespective of the clinical morphology , histopathology is imperativeunder the circumstances , apart from identification of pure scc / bcc , keratinizing bcc and baso - squamous carcinoma should be kept in mind . oca is an established risk factor for all the three major forms of skin cancers , due to reduction and/or complete absence of melanin although scc is common , the presence of bcc is not that infrequent and should be suspected irrespective of the clinical morphology , histopathology is imperative under the circumstances , apart from identification of pure scc / bcc , keratinizing bcc and baso - squamous carcinoma should be kept in mind . basal cells in the histogenesis of actinic keratosis has recently been incriminated , enforcing this new concept .

the report highlights the occurrence of basal cell carcinoma in a native indian with oculo - cutaneous albinism , an association not frequently encountered . the clinical and histopathological features , which assisted to form the diagnosis , are outlined . a high degree of suspicion and timely recognition of the potentially aggressive neoplasm , under this unusual circumstance , is the key to its diagnosis .

amyloidosis cutis dyschromica ( acd ) is a very rare form of primary cutaneous amyloidosis ( pca ) with very few cases reported in literature . pca is a rare chronic progressive disease with deposition of amyloid in the skin without systemic involvement . it is characterized by hyper and hypopigmented spots , prepubertal onset and amyloid deposits in papillary dermis . we report a case of acd in which the patient presented with dyschromic skin since birth and positive family history . a 26-year - old female presented with hyperpigmentation of skin since birth and progressively developed , hypo- and de - pigmented spotty macules over the extremities and trunk during childhood . she was born to parents of second - degree consanguineous marriage , with history of similar skin lesions in maternal uncle . patient did not give any history of photosensitivity , blistering , itching or developmental delay . cutaneous examination revealed diffuse hyperpigmentation with mottled spotty , well - defined , de - pigmented and hypopigmented macules ranging from pinpoint to 2 2 cm in size . multiple hypopigmented and depigmented macules over upper back with few atrophic lesions on left shoulder histopathology of hypopigmented lesion showed atrophic epidermis with loss of rete ridges . the upper dermis revealed eosinophilic extracellular nodular deposits and mild perivascular aggregates of chronic inflammatory cells , of which lymphocytes predominate [ figure 2 ] . eosinophilic globular deposits of amyloid in papillary dermis ( h and e , 100 ) deposits showing positive reaction with congo red ( 400 ) histopathology of the hyperpigmented lesion also showed eosinophilic deposits that stained brick red with congo red . acd is a very rare variant of pcd , first defined by morishima in 1970 . it is characterized by i ) mottled hyper- and hypo - pigmentation ii ) little or no itching iii ) pre - pubertal onset and iv ) amyloid deposits below epidermis . acd must be differentiated from other causes of dyschromica including dyschromatosis universalis hereditaria , xeroderma pigmentosum and poikiloderma - like amyloidosis . the latter shows a similar clinical cutaneous picture as acd but with additional features such as poikilodermic lesions , lichenoid papules , blisters , photosensitivity , short stature and palmo - plantar keratoderma . the cause of acd is unknown but genetic factors and sun exposure have been implicated . it has been proposed that genetic factors cause prolonged dna repair in keratinocytes following uv damage . the source of amyloid is unclear . a possible cause may be phagocytosis of damaged keratinocytes by histiocytes or fibroblasts that produce amyloid material in the skin . sun avoidance and protection , topical corticosteroids , keratolytics , dimethyl sulfoxide , capsaicin , and carbon dioxide laser have been tried . this case differs from most other cases reported in literature due to presence of lesions since birth and atrophic de - pigmented macules , although one case of atrophic depigmentation has been reported .

amyloidosis cutis dyschromica is a very rare form of primary cutaneous amyloidosis characterized by prepubertal onset of hyper and hypopigmented spots and amyloid deposits in the papillary dermis . we report a case of a 26 year old female with amyloidosis cutis dyschromica who presented with dyschromic skin since birth .

a 56-year - old male presented with chronic on and off pain in the left eye his systemic examination revealed multiple subcutaneous tumors in distal upper limbs and more than 6 caf - au - lait macules ; but all were < 15 mm in diameter . his vision was 20/30 in right eye and counting fingers at 3 feet in left eye . applanation tonometry readings were 13 mm hg and 50 mm hg in right and left eye , respectively . dark - room gonioscopy showed appositional closure of the drainage angle with multiple peripheral iris convexities in the right eye and 360 synechial angle closure in the left eye . ubm presented a ring - shaped solitary iridociliary cyst spanning 360 in the right and 270 , sparing nasal quadrant , in the left eye . the cysts had hyper - reflectile walls and sonolucent interiors , suggestive of primary neuroepithelial cysts [ fig ultrasound biomicroscopy showing iridociliary cyst in right ( a ) and left ( b ) eye we did nd : yag laser iridotomy , peripheral to the collarette of the right iris at 8 oclock hour where the diameter of the cyst was narrowest on ubm to avoid cyst rupture [ fig . 2 ] . post iridotomy , the angle recess was narrow , but the scleral spur was visible all around on dark - room gonioscopy in the right eye . the right optic disc was healthy , and the left had advanced glaucomatous cupping [ fig . 3 ] . magnetic resonance imaging of brain and orbits was unremarkable , except for a diffuse t2 hyperintense signal in the left optic nerve . note its atypical location at 8 oclock hour optic disc photographs of right ( a ) and left ( b ) eye ultrasound biomicroscopy of right ( a ) and left ( b ) eye . note narrow but open angle recess in a and absence of iridociliary cyst in b the left eye was painful despite maximum antiglaucoma medications . this eye underwent g - probe assisted inferior 180 contact transscleral diode cyclophotocoagulation ( 810 nm laser ; iridex corporation , ca , usa ) . post procedure , severe inflammation of the anterior chamber and neovascularization of the iris occurred , which resolved gradually with steroid and cycloplegic eye drops . the iop in the left eye remained between 40 and 48 mm hg though the patient was pain - free . on repeat ubm , the inferior ciliary processes were shrunken , but the cyst was no longer imaged [ fig . primary iridociliary cysts , in the majority , are stationary lesions located focally and rarely cause visual complications . angle closure , secondary to iris and ciliary body cysts , is related to the forward bulk of the iris cyst and occlusion of the posterior chamber and is scarcely reported.[13 ] a postulated mobility of the ring - shaped iridociliary cyst during movements of the pupil might make an additional contribution to the angle closure . absolute glaucoma , as in the left eye of our patient , is not attributed to iris or ciliary body pigment epithelial cyst(s ) in the past . except for a single report of 7 members of a family with familial aortic dissection and having pupillary margin iris pigment epithelial cysts , no relation of primary cysts of iris or ciliary body epithelium to any systemic disease has been reported . the clinical features of our patient were insufficient to meet the diagnostic criteria of neurofibromatosis ( nf)- 1 . nevertheless , iris pigment epithelium cyst is formed by separation of the 2 layers of epithelium and can not be explained by the pathogenesis of nf . laser iridocystotomy when the cyst could not be visualized in mydriasis by gonioscopy is also reported . this treatment results in pigment dispersion , and carries the potential risk of inflammation and cyst recurrence . however , laser iridotomy did suffice to relieve oppositional angle closure in the right eye of our patient [ fig . the treatment resulted in inadvertent iridociliary cyst rupture with no troublesome sequel over the follow - up . thus , cyst rupture is a potential complication of diode cyclophotocoagulation in eyes with large iridociliary cyst(s ) .

primary iridociliary cysts can induce plateau iris configuration and angle closure glaucoma . we report a patient with bilateral , primary , ring - shaped , solitary iridociliary cysts . the right eye displayed normal intraocular pressure , oppositional iridocorneal angle closure , and healthy optic nerve head . the left eye had advanced chronic angle closure glaucoma . the management strategy varied between eyes and is discussed . this , to the best of our knowledge , is the first report of transscleral diode laser application in an eye with a large iridociliary cyst .

two years previously , a mass involving the left thigh had been diagnosed after wide excision as multiple myeloma and the patient had undergone chemotherapy . after 6 months , the disappearance of serumal monoclonal protein and a free light chain in the urine indicated complete remission . ugis demonstrated a luminal protruding mass in the anterior wall of the gastric cardia and along the greater curvature of the body ( fig . 1a ) , while contrast - enhanced ct revealed marked thickening of the gastric wall , with subtle contrast enhancement ( fig . mr imaging depicted a well - defined , homogeneous mass with prominent gastric wall thickening , but no necrotic portion . as demonstrated by t1-weighted imaging sequences , the signal intensity of the mass was less than that of the liver , though t2-weighting showed it as slightly higher , and homogeneous ( fig . after the administration of contrast material , the observed homogeneous enhancement was similar to that of hepatic parenchyma ( fig . 1d ) . fiberoptic gastroscopy depicted a large , protruding , luminal mass and multiple biopsies were performed . histopathologic examination revealed dense and monotonous infiltration by both mature and immature plasma cells ( fig . the extraskeletal spread of multiple myeloma occurs more frequently than is currently recognized , but clinical involvement of the gut is rarely reported . where it does occur , the small bowel is most commonly involved , followed by the stomach , colon , and esophagus . patients with gastric myeloma have presented with nonspecific gastrointestinal symptoms such as epigastric pain , weight loss , and upper gastrointestinal bleeding ( 1 - 4 ) . pathologically , gastric myeloma is thought to originate from lymphoid follicles in the submucosa , or from plasma cells in the submucosa or mucosal lamina propria ( 5 ) . the most advanced form of gastric myeloma infiltrates the deep muscular layer or serosa , and to avoid misdiagnosis , deep endoscopic biopsy may be required ( 3 ) . gastric myeloma can manifest in various forms : nodular , infiltrative , ulcerative , and polypoid ( 5 ) , among which the nodular type is most common . in our case the infiltrative form occurred , manifesting as diffuse thickening of the anterior wall of the gastric cardia and along the greater curvature of the body . the radiologic findings of gastric myeloma have been reported in the literature ( 2 , 3 ) . ugis has indicated the presence of infiltrative and polypoid mass lesions , though to differentiate on the basis of those findings between gastric myeloma and a gastric stromal tumor , lymphoma , neuroendocrine tumor , or adenocarcinoma is difficult . yoon et al . ( 3 ) describe the ct findings in two patients with gastric myeloma : in both , homogeneous gastric wall thickening was apparent , and contrast enhancement was poor and similar in degree to that of paraspinal muscle . t1-weighted mr imaging sequences indicated that the signal intensity of the mass was less than that of the liver , while t2 weighting showed it as slightly higher . the administration of contrast material revealed no necrotic portion , and the homogeneous enhancement observed was similar to that of hepatic parenchyma . although these findings simulate those of lymphoma , neuroendocrine tumor and gastric stromal tumor ( 6 ) , differentiation is based on the fact that in lymphoma the nodes are usually bulkier , while a neuroendocrine or stromal tumor usually has a necrotic portion . in conclusion , gastric myeloma is an uncommon entity that may mimic other malignant gastric tumors and tends to present as a homogeneous solid mass without a necrotic portion . correct diagnosis depends upon a correctly interpreted biopsy combined with the immunohistochemical study of immunoglobin .

we report a case of multiple myeloma with gastric involvement occurring in a patient who underwent an upper gastrointestinal series ( ugis ) , ct and mri . ugis depicted a luminal protruding mass , while contrast - enhanced ct demonstrated marked thickening of the gastric wall , with subtle contrast enhancement . at t1- and t2-weighted mr imaging , the mass showed iso- and intermediate signal intensity , respectively . after the administration of contrast material , subtle homogeneous enhancement was apparent .

incarcerated hernia frequently requires emergency surgery since it is associated with intestinal obstruction and ischemia . however , emergency surgery for intestinal obstruction has a high mortality and morbidity.1,2 recently , we were able to perform elective laparoscopic repair of a morgagni hernia causing obstructive ileus in a very elderly patient by preoperative colonoscopic placement of a transanal decompression tube . the patient was an 88-year - old woman who had experienced vomiting and abdominal distention for three days . plain radiographs showed up an inverted u - shaped pocket of air in the lower mediastinum , and dilated bowel loops ( fig . 1 ) . abdominopelvic ct revealed an incarcerated transverse colonic loop through the diaphragm ( fig . 2 ) . emergency colonoscopy for decompression demonstrated two luminal constriction sites with convergence of mucosal folds at the transverse colon ( fig . 3 ) ; these were consistent with the diaphragmatic indentations of the incarcerated hernia seen by fluoroscopy . we were able to pass the endoscope ( gif - xq240 ; olympus optical co. , tokyo , japan ) through the constrictions with resistance and see the dilated large bowel proximal to the constrictions . however , the endoscope could not be advanced further because of the presence of a walking stick - shaped loop . transanal decompression by inserting an 18 fr levine tube over the guidewire after removing the endoscope failed since the tube could not be advanced beyond the first constriction . accordingly we placed a thinner catheter ( enbd-7-liguory , 7 fr , 250 cm ; cook medical inc . , winston - salem , nc , usa ) , which is designed for nasal biliary drainage , through the working channel of the endoscope beyond the constrictions ( fig . 4 ) . the catheter was irrigated periodically with normal saline to prevent obstruction , and the intestinal contents and gas were drained successfully . follow up abdominal radiograph the next day demonstrated marked regression of the obstructive ileus ( fig . 5 ) and the patient was able to undergo elective laparoscopic repair of the hernia after a week . laparoscopy revealed omental herniation restricted to the anteromedial portion of the left diaphragm , and integrity of the bowel . the omentum was reduced into the abdomen , the hernia sac was excised , and the defect was repaired with a 56 cm mesh patch ( proceed surgical mesh , ethicon inc . , somerville , nj , usa ) . morgagni hernia is a diaphragmatic hernia that accounts for 3% of total intestinal obstructions.3 repair can be performed either by transthoracic or transabdominal approach , and good results with laparoscopic transabdominal repair have been reported recently.3,4 colostomy ( or ileostomy ) , followed by a second operation for its repair , is performed in many cases of colonic obstruction since primary anastomosis is not always feasible.5 however , we were able to safely perform one - step elective laparoscopic repair for the patient with advanced age by endoscopic decompression ; this procedure relieved the symptoms of obstruction , led to medical recovery and a favorable bowel preparation . drainage was successful even though we could place only a thin catheter beyond the constrictions . in fact , catheters of small diameter have the great advantage that they can be positioned through the endoscope . drainage can be effective in spite of the narrow lumen of the catheter if it is prevented from causing obstruction by , for example , periodic irrigation . thus , when it is difficult to position a transanal decompression tube of large diameter by the seldinger technique , insertion of a catheter of smaller diameter through the endoscope can be a useful option . finally , this case suggests that appropriate endoscopic placement of a decompression tube for incarcerated morgagni hernia permits elective and less invasive laparoscopic repair in patients at greater risk from emergency surgery .

plain radiographs of an 88-year - old woman who had experienced vomiting and abdominal distention for 3 days revealed a severely obstructed ileus , and abdominopelvic computed tomography revealed an incarcerated morgagni hernia . the endoscope was passed through the constrictions from the diaphragmatic indentations and a thin catheter was placed for decompression . the obstructive ileus regressed markedly after the procedure ; the patient underwent elective laparoscopic repair of the hernia 1 week later . this is believed to be the first case of endoscopic preoperative decompression for an incarcerated morgagni hernia .

cutaneous leishmaniasis ( cl ) is a major public health problem in many countries . however , there are many topical and systematic treatments for cl . at present , pentavalent antimonials are still the first drug of choice for the treatment of cl ( 1 ) . systemic administration of pentavalent antimonials has been associated with such adverse reactions as musculoskeletal pain , gastrointestinal disturbances , and mild to moderate headaches . electrocardiographic qt interval prolongation and a mild to moderate increase in liver and pancreatic enzymes are other adverse effects of pentavalent antimonials nonetheless , acute urticaria in infants has never been reported to be associated with maternal use . here we report a case of an infant presenting with acute urticaria after her mother s parenteral use of meglumine antimoniate ( glucantime ) for the first time . in november 2015 , a 34-yr - old breast - feeding woman from gonbad city of iran presented with a lesion on the anterior region of her abdomen that lasted about 60 days . the lesion first appeared as a small red papule and then progressed to an ulcer . it was a firm , non - tender , well - defined lesion , which measured 4 cm 3 cm in diameter . a slit - skin smear from the edge of the ulcer with giemsa stain showed leishman bodies within the macrophages , which confirmed the diagnosis of cutaneous leishmaniasis . the patient was treated with a weekly cryotherapy regimen and intralesional injections of glucan - time . the patient was discontented with no sign of improvement with the prescribed medications and interventions after three wk . as a result , the treatment was changed to glucantime at a dose of 20 mg / kg - body weight intramuscularly for two wk . for our patient weighing 95 kg , we prescribed 3 vials per day for 14 d ( according to the national guidelines for leishmaniasis surveillance in iran-2012 ) . a day after the start of systemic therapy , the patient brought her fifteen - month - old breast - feed infant with urticaria and malaise but without eosinophilia ( 1cells / mm ) . with an elevated liver enzyme sgot level ( 46 iu / l , normal range is up to 31 iu / l ) ; the infant s vital signs were normal . the physical examination results were normal except for urticaria , which was dominant on the abdomen , back and thighs ( fig . 1 ) . both the mother and the infant had no history of exposure to any alternative causes other than glucantime . the mother was advised to discontinue breastfeeding for two week and the infant to receive a single dose of antihistamine ( 2.5 mg cetirizine per oral ) . the symptoms resolved within 24 h , during which the urticaria disappeared and malaise was alleviated . with slow but definite improvement of the lesion , urticaria , obvious on the abdomen , back and thighs of the patient ( original ) two pentavalent antimonial drugs , sodium stibogluconate ( pentostam ) and glucantime , have been the drugs of choice for the treatment of cutaneous leishmaniasis for over 50 yr . in spite of their wide use for half a century , relatively little is known about their chemical structures and mechanism of actions ( 4 ) and , up until now , new side effects have been reported as a result of their use . in our case , the occurrence of adverse drug reaction and its successful management after discontinuing the drug points to the unwanted effects of glucantime as non - pharmacological causes were not found to have generated the reaction . we could not find any report of acute urticaria in infants associated with the mother s parenteral use of glucantime . this included a search on lactmed ( a toxnet database ) which led to no records being found ( 5 ) . hence , this is the first reported case of urticaria in infants due to the mother s parenteral use of glucantime . it is recommended that mothers discontinue breastfeeding and avoid taking glucantime in systemic doses because of adverse effects on their infants . ethical issues ( including plagiarism , informed consent , misconduct , data fabrication and/or falsification , double publication and/or submission , redundancy , etc . ) have been completely observed by the authors .

pentavalent antimonials are still the first drug of choice for the treatment of cutaneous leishmaniasis ( cl ) . like other treatments , they can cause adverse reactions including musculoskeletal pain , gastrointestinal disturbances , and mild to moderate headaches . in this paper , we report the first case of an infant who developed acute urticaria after her mother s parenteral use of meglumine antimoniate ( glucantime ) .

wegener 's granulomatosis is an uncommon systemic disease characterized by necrotizing vasculitis of small blood vessels with granuloma formation . central nervous system manifestations of wg include cranial neuritis , mononeuritis multiplex , cerebral vasculitis , and meningeal involvement . subdural hematoma as a manifestation of wg is extremely rare and till date , only one such case has been reported . here a 42-year - old male presented with complaints of low - grade fever , diffuse headache , and joint pains for 8 weeks . patient gave history of pain and swelling of knees , ankle , wrist and elbow joints , not associated with morning stiffness . laboratory investigations revealed hemoglobin-14 g / dl , total leukocyte count- 13,200/cmm , and platelet count 4,53,000/cmm . brain mri revealed subdural hematoma ( subacute stage ) in left fronto - parietal region [ figures 1 and 2 ] . the neurosurgeon advised surgical evacuation of hematoma , but the patient did not give consent . non - contrast ct scan of head revealed subacute subdural hematoma in the left fronto - parietal region . on 50 day of his illness , patient developed right hemiparesis , and repeat ct scan of head showed infarct of left basal ganglia , which extended up to the parietal region . mri brain t1-weighted image showing left fronto - parietal subdural hematoma with effacement of left side gyri and sulci mri brain t2-weighted image showing left fronto - parietal subdural hematoma the patient presented to the emergency room of our hospital with weakness of right half of body for 7 days . in addition , he gave history of fever , headache , and joint pains for 3 months . there was no history suggestive of chronic upper respiratory symptoms , except for 4 - 5 episodes of epistaxis about 6 months back , which subsided without any treatment . patient was conscious and oriented , and power of right upper and lower limbs was grade 3 - 4/5 with hyper - reflexia . ultrasound abdomen revealed mild hepatomegaly with fatty liver and multiple hypoechoic lesions in the substance of spleen . contrast - enhanced ct scan of abdomen showed irregular wedge - shaped hypodense lesions having attenuation value of 6 35 hu in spleen . patient was empirically put on anti - tubercular treatment ; however , he did not respond . five weeks later , contrast - enhanced ct scan of chest revealed thin - walled cavitary lesions in both upper lobes with parenchymal mass [ figure 3 ] . cytoplasmic anti - neutrophil cytoplasmic antibody ( canca ) levels were found to be raised . blood urea was 52 mg / dl , and serum creatinine was 2.1 mg / dl . ct scan showing cavities in both upper lobes associated with parenchymal lesion a diagnosis of wg was made , and the patient was treated with 3 doses of intravenous methylprednisolone followed by oral prednisolone and oral cyclophosphamide . fever subsided within 10 days of initiation of immunosuppressants , and renal functions improved to normal level within 2 weeks . cyclophosphamide was replaced by mycophenolate mofetil after 6 months , and dose of prednisolone was tapered to 10 mg per day . mri brain repeated after 8 months of follow - up did not show any subdural hematoma . the patient is on regular follow - up and for last 1 year ; he is largely asymptomatic , except for mild residual right hemiparesis . this patient initially presented with fever , headache , and joint pains and had fronto - parietal subdural hematoma on imaging . organ systems commonly involved in wg such as paranasal sinuses , lungs , kidneys , and skin were not involved . we could find only one case report of wg presenting as subdural hematoma . in this case , mri scan was suggestive of subdural hematoma or empyema , hence subdural drainage was done , which revealed pus - like fluid . shiotani et al . reported a case that presented with fever and severe headache , and ct and mri of head revealed subdural masses . the diagnosis in this case was established by needle biopsy of nasal polyps and positive canca . it has been reported that meningeal involvement usually develops in patients with localized disease who are more likely to have destructive lesions of upper airways . in our case , the diagnosis was made after detection of characteristic finding on ct scan of chest and test for canca . in our patient , the development of cerebral infarct and subdural hematoma appear to be a manifestation of cns vasculitis due to wg . wg is a devastating disease ; hence , high index of suspicion and timely treatment is warranted . in patients with subdural hematoma without history of head injury , cns vasculitis due to wg , though very rare , may be considered as one of the possibilities .

wegener 's granulomatosis ( wg ) is a systemic vasculitis , which can involve any organ system in the body ; however , involvement of central nervous system at presentation is very uncommon . dural involvement in wg has been infrequently reported ; however , presentation with subdural hematoma as the initial manifestation is extremely rare . we present a case of wg presenting as subdural hematoma , which resolved on cytotoxic therapy without surgical evacuation .

the utilization of multiple pharmacologic agents is an essential component of modern day anesthetic practice . while there have been numerous advancements in recent years in both analgesic and amnestic medications available for an anesthesiologist to use in clinical practice , the cadre of neuromuscular blocking agents available in the united states has been stagnant . the ideal neuromuscular blocking agent is one that is rapidly acting , has minimal to no adverse effects , is independent of end organ metabolism , and allows for rapid and complete reversal of neuromuscular blockade . first , the deleterious effects of residual neuromuscular blockade in the postanesthetic care unit have been well studied and are clinically relevant . emphasis on operative efficiency and patient discharge has also been widely identified as an area for potential cost saving measures in modern healthcare settings . novel drug development has been proven to be a difficult and timely process as it has been over 20 years since a new nondepolarizing muscle relaxant has been introduced for clinical use . this paper will highlight some of the latest pharmacological advancements in the area of neuromuscular blockade . the enantiomers gantacurium and cw002 are two of the most recent neuromuscular blocking agents that have shown potential for clinical application . the appeal of these molecules is the ultra - rapid reversal of neuromuscular blockade via cysteine adduction and minimal systemic hemodynamic alterations with administration . gantacurium is an asymmetric alpha - chlorofumarate and is classified as an ultra - short acting nondepolarizing neuromuscular blocker . its pharmacologic properties have been established using both animal and human models with its ed95 found to be 0.19 mg / kg . maximum neuromuscular blockade using gantacurium was found to be within 90 s following administration of 1.5 ed95 with even faster onset at higher doses . this pharmacologic profile is comparable to that of succylincholine and could eventually serve as a replacement for a rapid depolarizing muscle relaxant . gantacurium chemical structure cw002 differs in structure from gantacurium by being symmetrical and lacking a chlorine at the fumarate double bond . these properties give cw002 a greater potency than gantacurium and an intermediate duration of action of approximately 30 min . using both animal and human models , , cw002 has minimal to no hemodynamic effects at administered doses well above its documented ed95 . the first is a slow ph - sensitive hydrolysis at the ester linkages of the molecules . this results in a t of 56 min and 495 min for gantacurium and cw002 , respectively . the second pathway for inactivation is much more rapid and has the greatest clinical implications . l - cysteine adduction results in a byproduct of extremely low potency that also subsequently undergoes hydrolysis to form inactive molecules . furthermore , of importance is that unlike conventional neuromuscular blockers , this pathway allows for complete reversal at any time after bolus administration of neuromuscular blockers . l - cysteine adduction terminates the relaxants action via inactivation and not by overcoming competitive inhibition . advancements in neuromuscular blocking agents have the potential to have significant impact on anesthetic care in the united states . the ability to rapidly and reliably induce and reverse favorable conditions for tracheal intubation and surgery can profoundly impact anesthetic care in ambulatory , inpatient , and emergent settings . all anesthesia providers will need to consider some of the advantages and potentially disadvantages of these new drugs in their practice in the future . this paper details some of the promising medications on the horizon for clinical use . continued research is needed in the most important area of neuromuscular modulation in clinical practice .

pharmacological advances in anesthesia in recent decades have resulted in safer practice and better outcomes . these advances include improvement in anesthesia drugs with regard to efficacy and safety profiles . although neuromuscular blockers were first introduced over a half century ago , few new neuromuscular blockers and reversal agents have come to market and even fewer have remained as common clinically employed medications . in recent years , newer agents have been studied and are presented in this review . with regard to nondepolarizer neuromuscular blocker agents , the enantiomers gantacurium and cw002 , which are olefinic isoquinolinium diester fumarates , have shown potential for clinical application . advantages include ultra rapid reversal of neuromuscular blockade via cysteine adduction and minimal systemic hemodynamic effects with administration .

mite demodex is an ectoparasite infesting the areas of face rich in pilo - sebaceous units and less commonly other seborrheic sites like upper and medial region of chest and back are also involved . majority of the patients are immune - compromised though immunocompetent are not spared , nevertheless atypical presention in immunocompetent individuals is a rarity . we report a case with atypical presentation which posed diagnostic dilemma due to confusing clinical picture . a 32-year - old male presented with gradual onset swelling and redness on right side of face associated with minimal itching for past six months . it started as a pea - sized red raised flat lesion , which increased in size to involve 1/3 rd of right cheek , adjoining nasolabial fold and lower eye - lid . there was modest increase in redness and pruritus of the lesions on sunexposure , however hot spicy meals , alcohol or emotional stress did not aggravate his symptoms . there was no history of cough , chest pain , fever , weight loss or any constitutional or systemic symptoms . patient reported to a dermatologist about a month back , where he was diagnosed as hansen s disease in view of equivocal hypoaesthesia and solitary indurated plaque on face . he was empirically started on multi drug treatment ( mdt ) multi bacillary for hansens disease . after about four weeks of mdt , patient reported to our hospital with no clinical improvement and persistence of symptoms . dermatological examination revealed involvement of right malar region , nasolabial fold and adjacent parts of nasal bridge and lower eyelid in the form of edema , erythema and follicular dilatation ( figure 1 ) . laboratory investigations revealed complete blood count , erythrocyte sedimentation rate ( esr ) , liver and renal function tests , serum ace levels , were normal . screening for hepatitis b , hepatitis c and human immunodeficiency virus , as well as serology for antinuclear antibodies ( ana ) were negative . chest x - ray showed no abnormality and koh mount of scrapings did not reveal any pathology . repeat skin biopsy from medial end of the swelling along nasolabial fold showed mild epidermal orthohyperkeratosis . the dermis showed intense follicular and perifollicular lymphocytic inflammatory infiltrate along with dense lichenoid lymphomononuclear infiltrate . there was follicular dilatation with presence of demodex sp.mite with surrounding homogenous eosinophilic material ( figure 2 ) . patient was managed with oral ivermectin 12 mg stat and topical metronidazole gel locally twice a day . the eryhthema and swelling started regressing within few days and after 03 weeks had near complete resolution of symptoms ( figure 3 ) . demodex are parasitic mites that live in hair follicles and pilosebaceous units , accordingly maximum density is found in seborrheic sites . besides humans they also cause significant infestation in canines and felines . human infestation is very common and varies between 23 - 100% as per different authors . this infestation is usually asymptomatic , but when and how it becomes pathogenic is poorly understood . primary or secondary suppression in immunity plays a major role in demodex proliferation and resultant dermatoses . cutaneous disorders attributed to this mite include , rosacea , pustular folliculitis , perioral dermatitis , lupus miliaris disseminatus faciei , madarosis , non - specific facial dermatitis etc . our patient presented with non - specific facial dermatitis affecting only right side of the face . unilateral , indurated , erythematous plaque on face with equivocal hypoaesthesia resulted in initial misdiagnoses . earlier it has also been reported to present with facial sclera - oedema and blepharoedema which mimicked cutaneous lymphoma . further demodicidosis should also be considered in patients presenting even with unilateral lesions in seborrheic areas .

demodicosis is a common parasitic infection of the hair follicles and the pilosebaceous unit by the demodex mites viz . demodex folliculorum and demodex brevis . infection by this parasite is common among immunocompromised and elderly . we report a case of facial demodicosis which presented like atypical rosacea with a gradually progressing swelling and redness on right side of face which was initially diagnosed as a case of hansen s disease . skin biopsy revealed follicular dilatation with presence of demodex mite along with intense perifollicular lymphomononuclear infiltrate . patient was treated with oral tab ivermectin 12 mg stat along with topical gel metronidazole twice daily to which he responded favourably .

pyrethrums are organic , environment - friendly insecticides having a knock down effect on a variety of insects . pyrethrin and pyrethroids have quick action against insects , low toxicity , repellent properties , and virtually no insect immunity . the very low potential for toxicity in human being is due to its rapid metabolism into non - toxic metabolites following ingestion or exposure . fewer than 10 deaths have been reported from the ingestion or occupational exposure of pyrethroids . we describe a case of deliberate poisoning of pyrethroid ( prallethrin ) presenting as status epilepticus . a 35-year - old male was admitted to our emergency department with giddiness as the major complaint following ingestion of an unknown quantity of an unknown poison . patient was conscious , oriented with stable cardio - respiratory parameters at the time of arrival . after sometime , despite being well oxygenated via face mask , patient started having generalized tonic - clonic seizures . initial attention to airway , breathing and circulation ( abc ) was given , followed by a bolus dose of diazepam . seizures subsided for a brief period only to recur repeatedly . on repeated enquiry , the patient 's relative brought empty bottles of all out , a commercial composition of pyrethroid used as insecticides providing a circumstantial evidence of the nature of the poisoning . patient was given a bolus dose of thiopentone sodium , and soon his trachea was intubated . this was followed by a loading dose of the anti - convulsant phenytoin to control seizures . patient was shifted to the intensive care unit for further management . on examination , he was deeply comatose . hemogram , liver and kidney function tests , x - ray chest , ecg , and arterial blood gases were normal . thereafter , thiopentone sodium and midazolam infusions were administered and titrated clinically as no bedside eeg monitoring was available . gradually , thiopentone sodium and midazolam infusions were tapered off , and patient was weaned off the ventilator to be extubated after 72 hours . the patient was discharged from the hospital on the 7 day after a psychiatry consultation . the pyrethrin insecticides were discovered in the last decade of the 19 century as pyrethrum , pyrethrin , and pyrethroid . pyrethrum is an extract from the dried flowers of the compositae family such as chrysanthemums or daisies . they are widely - used organic , environment - friendly , and multipurpose insecticides with a long safety record for both acute and chronic intoxication in human beings . they are not stored in the body fat , nor penetrate the intact skin , and are very rapidly metabolized in the body into non - toxic metabolites . pyrethrins have been established to be an allergen and are responsible for dermatitis , conjunctivitis , rhinitis - like symptoms . all - out , the commercial brand of pyrethroid insecticide , contain a chemical prallethrin . as per who norms , prallethrin is a racemic mixture of 8 isomers used as household insecticides and pesticides with very low mammalian toxicity and no carcinogenicity or mutagenicity . on the basis of their chemical structure , type -1 , which produce hyperexcitability with fine tremors , and type 11 , which produce more severe symptoms and seizures on intoxication . the poisoning syndrome includes nausea , vomiting , headache , fine tremors , hyperexitability , and paresthesia in milder cases . however , severe toxicity is characterized by dizziness , gross tremors , hypersalivation , sympathetic stimulation leading to hypotension , tachycardia , profuse sweating , and dilated pupils . patient needs to be treated symptomatically i.e. , atropine for excessive salivation and diazepam for control of seizures . paresthesias lasting for short periods do occur and are treated successfully with vitamin e. gastric lavage was deferred due to continuous convulsions but was performed later . as seizures could not be controlled with diazepam and anti - convulsants , therapeutic coma was induced with thiopentone sodium and midazolam infusions , but for a brief period , due to lack of bedside eeg monitoring . the exact mechanism of poisoning is a change at the voltage - gated sodium channels i.e. leading to repetitive discharges and synaptic disturbances . severe toxicity is believed to be due to decrease in chloride through voltage - dependent chloride channels . pyrethroids in high concentration act on gaba - activated chloride channels , leading to seizures . with ever increasing demand for more powerful insecticidal agents for agriculture and household use , the chances of intoxication after long - term exposure presents a real risk to human beings and animals . very few cases of pyrethroid poisoning have been reported with status epilepticus . in india , only 2 children of allethrin poisoning and 1 adult of pyrethroid poisoning had presented with convulsions . a similar case presenting as status epilepticus after the consumption of a large amount of pyrethroid was reported earlier . pyrethroid poisoning should be considered among the differential diagnoses for a patient of suspected poisoning presenting with seizures .

this report describes a 35 year old male who presented with seizures after consuming 4 - 5 bottles of all - out a commercial composition of pyrethroid used as insecticides . our case report supports authors reporting an association of pyrethroid poisoning with status epilepticus .

since guinto , et al . reported a case of spontaneous regression of a herniated lumbar disc in 1984 , this phenomenon in lumbar discs has been well documented and discussed [ 2 - 4 ] . however , there have been fewer reports of spontaneous regression of cervical disc herniation ( cdh ) [ 5 - 7 ] , especially ones confirmed by magnetic resonance imaging ( mri ) [ 8 - 10 ] . recently , a patient with cdh who was treated at our hospital experienced this exceptional condition after 12 months of conservative treatment . in the following report , we will present this case and discuss the condition . a 59-year - old female patient who was unable to conduct her normal activities or sleep due to intense neck pain and severe radiating pain in the right c7 dermatomal distribution was admitted to our institute a day after her symptoms developed . , she could not maintain a supine position owing to insufferable pain from the day of admission . mri at 15 days after the admission ( fig 1 ) revealed multiple degenerative changes of the cervical vertebra , as well as a disc that was significantly herniated to the right at the c6-c7 level . on physical examination , her motor power ( esp . wrist flexion ) was assessed as grade 4 + ; the spurling test was positive on the right side . details of the treatment are reported in ( table1 ) based on the standards for reporting interventions in clinical trials of acupuncture(stricta ) . the most frequently targeted local points were gv16 , bl11 , te10 , gb20 , bl10 , and gb21 , bl12 and the most frequently treated distant points were si3 , te3 , and li4 . she received analgesics , non - steroidal antiinflammatory drugs and a muscle relaxant for only four weeks . the patient 's visual analog scale ( vas ) score improved from 10 points at the time of admission to 3 points after 2 weeks of treatment ( fig . , the patient 's symptoms were completely alleviated , and no abnormal sensory , motor or spurling test findings were observed . in addition , follow - up mri conducted after 12 months revealed that the protruded disc had disappeared completely and that no root compression was present ( fig . since the first report of spontaneous regression of a herniated cervical disc by krieger and maniker in 1992 , several other authors have also reported this rare phenomenon [ 6 - 10 ] . according to the guidelines of the u.s . department of health & human services , anterior surgical nerve root decompression via anterior cervical discectomy with or without fusion in patients with cervical radiculopathy is recommended for rapid relief ( within 3 - 4 months ) of arm and neck pain , weakness , and/or sensory loss compared to physical therapy or immobilization with a cervical collar . in the absence of knowledge about the natural history of a herniated disc , doctors are apt to choose surgical treatment . published a retrospective review of complications associated with an anterior cervical discectomy and fusion in 1,015 patients . the results of their studies revealed a mortality rate of 0.1% and a morbidity rate of 19.3% , with the most common complication being development of isolated postoperative dysphasia , which was observed in 9.5% of the patients . other complications observed included postoperative hematoma ( 5.6% ) , recurrent laryngeal nerve palsy ( 3.1% ) , dural penetration ( 0.5% ) and esophageal perforation ( 0.3% ) . the possibility of such complications makes non - surgical treatment for cervical disc herniation desirable . spontaneous regression of cdh with acupuncture treatment can be regarded as a benign natural course that occurs in some patients with herniated cervical disc . the result of his study revealed 30% excellent , 35% good , and 35% fair . kim et al . used mri to confirm that spontaneous regression of a herniated cervical disc had occurred in 9 patients after traditional korean medical treatment , including acupuncture , bee venom pharmacopuncture , manipulation , and herb medicine . shin et al . reported clinical improvement made by bee venom therapy at cervical hyeopcheokhyeol in the case of upper limb disability caused by cervical herniations . many factors related to the regression process have been recognized , including the age of the patient , dehydration of the expanded nucleus pulposus , resorption of a hematoma , revascularization , penetration of herniated cervical disc fragments through the posterior longitudinal ligament ( pll ) , the size of disc herniation , and the existence of cartilage and annulus fibrosus tissue in the herniated material . resorption of a herniated nucleus pulposus is thought to occur via an inflammatory reaction in the outermost layer of the herniation , with macrophages as the major cellular population . although we report only one case of cdh , it might give clinical doctors a chance to reconsider surgery , and choose conservative treatment . here , we report a case in which a cervical disc herniation patient improved in response to acupuncture . mri conducted to evaluate the patient 12 months after treatment revealed that spontaneous regression of the herniated disc had occurred .

the spontaneous regression of herniated cervical discs is not a well - established phenomenon . however , we encountered a case of a spontaneous regression of a severe radiculopathic herniated cervical disc that was treated with acupuncture , pharmacopuncture , and herb medicine . the symptoms were improved within 12 months of treatment . magnetic resonance imaging ( mri ) conducted at that time revealed marked regression of the herniated disc . this case provides an additional example of spontaneous regression of a herniated cervical disc documented by mri following non - surgical treatment .

they have poor communication with the normal lymph systems and therefore collect lymph.[15 ] the most common location in the mouth is the dorsum of tongue , followed by lips , buccal mucosa , soft palate , and floor of the mouth . these tumors typically present in children younger than 2 years and in a significant number of cases are present at birth.[19 ] this article describes a case of lymphangioma of the tongue and its management . a 5-year - old girl reported to the opd of kothiwal dental college , moradabad with chief complaint of growth on the right lateral border of tongue , associated with biting of the tongue on mastication . her parents noticed it increasing in size along with the growth of the child . on inspection , there was a marked soft tissue growth with numerous papillary and vesicle - like projections which made it appear irregular and granular [ figure 1 ] . the mouth opening was normal and there was no restriction of functions of the tongue . on the basis of history and clinical features a provisional diagnosis of lymphangioma was made and an excisional biopsy under general anaesthesia was planned . right lateral border of tongue showing the soft tissue growth under general anesthesia , a deep suture was placed on the tip of the tongue [ figure 2 ] for retraction . single linear incision was outlined on the base of the elevated mass on the lateral surface of the tongue with methylene blue . initially , the incision was mucosal all around the elevated mass and subsequently it was deepened to excise it completely [ figure 3 ] . retraction of tongue and localization of lesion tongue after excision of the lesion following excision , the tongue was approximated in two layers [ figure 4 ] . on histopathological examination it was confirmed to be a lymphangiomatous lesion [ figure 6 ] . there was no evidence of recurrence of the lesion on follow - up of 4 years . they are believed to arise from lymph sac sequestration and enlarge due to inadequate drainage , from lack of communication with the central lymphatic channels or excessive secretion of lining cells . goetsch in 1938 noted that this sequestered lymphatic tissue forms cysts which enlarge from accumulation of lymph caused by projection of endothelial sprouts from cystic walls . the sprouts further destroy tissue and force the lesion into areas of least resistance , between muscles and vessels , invading tissue planes and causing atrophy , fibrosis and hyalinization of the engulfed tissue . lymphangiomas can be classified histopathologically as : lymphangioma simplex ( composed of small thin - walled lymphatics).cavernous lymphangioma ( comprised of dilated lymphatic vessels with surrounding adventitia).cystic lymphangioma ( consisting of huge , macroscopic lymphatic spaces surrounded by fibrovascular tissues and smooth muscles).benign lymphangioendothelioma ( lymphatic channels appear to be dissecting through dense collagenic bundles ) . cystic lymphangioma ( consisting of huge , macroscopic lymphatic spaces surrounded by fibrovascular tissues and smooth muscles ) . lymphangiomas are classified according to their clinical presentation into macrocystic ( cavities larger than about 2 cm ) , microcystic ( cavities smaller than about 2 cm ) , and mixed ( combining these two types ) . they are always present at birth , but they may go unnoticed until after dentition erupts or even after puberty . however , there have been reports of its occurrence in adults . the frequent site is the anterior two - thirds of the tongue and may enlarge to a great extent that results in macroglossia , which may impair speech and eating and may become life - threatening due to their size or secondary infection . their limited extent usually permits complete surgical excision with various techniques of tongue reduction described by a number of authors . the treatment of lymphangioma depends upon their type , size , involvement of anatomical structures and infiltration to the surrounding tissues . microcystic lesions do not respect tissue planes , are diffuse and difficult to eradicate , whereas macrocystic lesions are localized and easily excised . partial surgical excision , injection of sclerosing solutions ( ok432 ) , electrocoagulation , cryotherapy , embolization , steroid administration , radiation and laser surgery may be the other modalities of treatment of diffuse lymphangioma of the tongue.[179 ] since they do not respond to sclerosing agents , pressure therapy , radiotherapy or any known chemotherapy , they are either tolerated by the patient or treated surgically .

lymphangiomas are developmental malformations and have a marked predilection for the head and neck region . they are benign proliferation of lymphatic vessels and represents hamartomas of malformed lymphatics . the most common location in the mouth is the dorsum of tongue , followed by lips , buccal mucosa , soft palate , and floor of the mouth . in the tongue , they may present as a localized or a diffused growth which may enlarge to cause macroglossia , impaired speech and difficulty in mastication . these tumors are typically present at birth , but may go unnoticed until after dentition erupts or even after puberty . presenting here is a case of localized lymphangioma of the tongue in a five year old child and its surgical management .

in the early 1970s , this picture of thymic selection was still being colored in . immunologists were unaware that the antigens recognized by t cells were peptides bound to mhc molecules . and tcrs themselves peter doherty laid out the nobel prize winning rules of what is now known as self - mhc restriction in 1973 . they showed that antiviral t cells from an immunized mouse only recognized infected cells from other mice that shared the same type of mhc genes ( 1 ) . one for a self - mhc molecule , and the other for the viral antigen ( 2 ) . but zinkernagel and doherty preferred their own altered - self hypothesis , which proposed that the t cells bore a single receptor that recognized an mhc molecule that had been somehow altered by the viral infection . michael bevan , then a postdoctoral fellow at the salk institute ( san diego , ca ) , was intrigued by the altered - self theory . he had been studying differences in the genes encoding minor histocompatibility antigens ( mhas ) that cause graft rejection even when donors and recipients are mhc matched . he found that recognition of target cells by t cells specific for mha also followed the rules of mhc restriction . he proposed that the t cells recognized self - mhc molecules that were associated with these mhas and called it the antigen interaction hypothesis . the nature of this altering the binding of peptides to self - mhc would not be known for a few more years . bevan was able to boost confidence in the altered - self theory by disproving the two - receptor hypothesis . he found that in the progeny of mhc - a and mhc - b mice , individual t cells recognized antigen on either mhc - a or mhc - b cells but not both . in the dual recognition theory , each t cell would have had receptors for both mhc molecules and thus would have recognized both targets . as a further test of his theory , bevan designed an assay in which cells that had either the correct mhc but the wrong mha or vice versa competed with cells that had both the correct mhc and mha as targets for killing by mha - specific t cells . he found that both the correct mhc and mha had to be on the same target cells to be recognized by the t cells . an enticing explanation for these data was that the self - mhc and the antigen were somehow interacting . bevan published these results in the journal of experimental medicine in 1975 ( 3 ) . a previous hypothesis suggested that t cells were selected in the thymus ( 4 ) . bevan tested this theory by injecting t cell precursors from the progeny of mhc - a and mhc - b parents into lymphocyte - deficient mhc - a recipients . newly generated t cells from these mice recognized antigen - presenting target cells from mhc - a but not mhc - b mice . furthermore , when the thymus of the progeny mouse was replaced with an mhc - a only thymus , most of its t cells only recognized antigen bound to mhc - a cells ( 5 ) . bevan concluded that the mhc molecules in the thymus determine what mhc molecules will be recognized by the t cells . the definition of the tcr structure finally vindicated the single receptor hypothesis ( 6 ) . tcrs were later shown to recognize peptide fragments bound to mhc molecules on antigen - presenting cells ( 7 ) .

in the 1970s , michael bevan showed that t cells only recognize antigens in cells that have the same type of major histocompatibility complex ( mhc ) molecule present in the thymus where the t cells mature . his work provided the first clues to how thymic self - mhc molecules select the cells that make up the mature t cell repertoire .

coomb 's positive hemolytic anemia is a very rare cause of anemia associated with tb and only few case reports have been published . the case presented here had tuberculosis lymphadenitis associated coomb 's positive hemolytic anemia which resolved on treatment with anti tuberculous treatment . a 19-year - old girl presented with swelling in the left side of the neck of 1 year duration . laboratory findings on admission were as follows : hemoglobin 3.5 g / dl , total wbc count 4800/mm(52% neutrophils and 48% lymphocytes ) , platelet count 225,000/ mm , mcv 97 , mch 30.5 fl , and reticulocyte 12% . biochemical tests were as follows : total bilirubin 3.7 mg / dl , direct bilirubin 2.0 mg / dl , indirect bilirubin 1.7 mg / dl , ldh 720 u / l and haptoglobulin 20 mg / dl . coomb 's test done prior to transfusion was positive ( table 1 for hematological parameters before starting anti - tuberculosis therapy ) . serum levels of urea , creatinine were within normal limits and glucose 6 phosphate dehydrogenase activity was normal . serologic tests for antinuclear antibodies , human immunodeficiency virus , mycoplasma , hepatitis b and c virus were negative . left cervical lymph node biopsy showed caseating granulomatous lymphadenitis [ figure 1 ] mantoux test was positive ( 18 mm induration ) . laboratory parameters of the patient during hospital stay langerhan 's giant cells seen in tuberculosis lymphadenitis patient was started on antituberculosis treatment with isoniazid ( 300 mg qday ) , rifampicin ( 450 mg qday ) , ethambutol ( 800 mg qday ) , and pyrazinamide ( 750 mg bid ) . hemoglobin improved to 5 gm / dl after 2 weeks of treatment and coomb 's test became negative after 2 months [ table 2 ] . autoimmune hemolytic anemia ( aiha ) represents a spectrum of disorders in which antibodies against self - antigens on the erythrocyte membrane cause a shortened red blood cell ( rbc ) life span . aiha can occur as an idiopathic ( primary ) disorder or can coexist with another disease ( secondary ) . anemia in tuberculosis is most often due to nutritional deficiency , malabsorption syndromes , failure of iron utilization , and bone marrow suppression . siribaddane et al . , first reported association of autoimmune hemolytic anemia in tuberculous lymphadenitis in 1997 . a detailed search revealed only 7 cases of aiha in tuberculosis in the english literature ; 3 of them responded to anti - tuberculous treatment ( att ) alone without need for blood transfusion or steroids . mehmet turgut et al.,from turkey , reported a case of pulmonary tuberculosis with aiha responding to att alone without the need for blood transfusion . ping - hung kuo et al . from taiwan , reported an episode of coomb 's positive aiha in a patient with miliary tuberculosis resulting in a hemoglobin of 5g / dl . the hematological parameters in their case improved without the use of steroids or blood transfusion . , from india , reported a case of aiha in childhood tuberculosis ( mediastinal tuberculous lymphadenitis ) , which responded to steroids and antituberculous therapy . abdullah abba et al . , from saudi arabia , reported aiha in an indonesian housemaid suffering from intestinal tuberculosis . in their case , from india , reported about a child with abdominal tuberculosis and aiha , which responded to steroids and att . apart from autoimmune hemolytic anemia , reported a case series of 5 patients with tuberculosis and thrombocytopenia who were treated with att and other drugs like danazol , vincristine , iv immunoglobulin , and steroids . this case highlights the need for detailed evaluation of anemia in tuberculosis patients and to consider aiha in the differential diagnosis of anemia in these patients . one of the main treatments of aiha is the administration of steroids ; however , this may be potentially harmful in tuberculosis patients . this case report is to emphasize the effectiveness of att alone to correct aiha in tuberculosis patients .

anemia in tuberculosis is usually anemia of chronic disease . severe hemolytic anemia is exceedingly rare in tuberculosis patients . we report a patient diagnosed with tubercular lymphadenitis complicated by coomb 's positive hemolytic anemia . patient responded well to antituberculous treatment . hematological parameters improved after initiation of antituberculosis treatment . to the best of our knowledge , this is the first case from india of an adult patient with tuberculous lymphadenitis presenting with coomb 's positive hemolytic anemia .

pmds were obtained from lister et al . , generated from bisulfite sequencing in fibroblast cells . dnam data and hypomethylation block regions were obtained from hansen et al .. affymetrix hgu133plus2 expression data was obtained from the gene expression barcode project . idat files for 17 normal colon and 34 colon tumor samples were obtained from the tcga project . data was preprocessed and normalized using the standard illumina method , hypomethylation block finding was performed using the method in minfi . raw count tables at the exon level were obtained for 3 normal colon and 37 colon tumor samples from the tcga project . analyses were performed using bioconductor packages minfi ( v. 1.8.9 ) and epivizr ( v. 1.3.3 ) . the epiviz web application is hosted at http://epiviz.cbcb.umd.edu , the epivizr bioconductor package is available through the bioconductor project . javascript files defining exon - level expression visualization tracks are available as github gists ( http://gist.github.com/11279474 and http://gist.github.com/11279449 ) . open source code for all components is available in the epiviz project github page : http://github.com/epiviz , api descriptions and other documentation for epiviz is available online at http://epiviz.cbcb.umd.edu/help . pmds were obtained from lister et al . , generated from bisulfite sequencing in fibroblast cells . dnam data and hypomethylation block regions were obtained from hansen et al .. affymetrix hgu133plus2 expression data was obtained from the gene expression barcode project . idat files for 17 normal colon and 34 colon tumor samples were obtained from the tcga project . data was preprocessed and normalized using the standard illumina method , hypomethylation block finding was performed using the method in minfi . raw count tables at the exon level were obtained for 3 normal colon and 37 colon tumor samples from the tcga project . analyses were performed using bioconductor packages minfi ( v. 1.8.9 ) and epivizr ( v. 1.3.3 ) . the epiviz web application is hosted at http://epiviz.cbcb.umd.edu , the epivizr bioconductor package is available through the bioconductor project . javascript files defining exon - level expression visualization tracks are available as github gists ( http://gist.github.com/11279474 and http://gist.github.com/11279449 ) . open source code for all components is available in the epiviz project github page : http://github.com/epiviz , api descriptions and other documentation for epiviz is available online at http://epiviz.cbcb.umd.edu/help .

visualization is an integral aspect of genomics data analysis where the output of procedures performed in computing environments like bioconductor is often visualized . algorithmic - statistical analysis and interactive visualization are usually disjoint but are most effective when used iteratively . we introduce tools that provide this tight - knit integration : epiviz ( http://epiviz.cbcb.umd.edu ) , a web - based genome browser , and the epivizr bioconductor package allowing interactive , extensible and reproducible visualization within a state - of - the - art data analysis platform .

nevus lipomatosus cutaneous superficialis ( nlcs ) is a rare benign hamartomatous condition , which usually presents at birth or manifests in the first two decades of life . the classical presentation is with multiple painless soft pedunculated , yellowish cerebriform masses commonly located on the pelvic and gluteal region . we report here a case of nlcs with huge swellings on the back and prominent peau - d orange appearance at places . a 10-year - old boy presented with multiple humps on the upper and lower back , present for the preceding 2 years . starting as a single swelling on the upper back , it gradually increased in number and size to attain the present dimension . the surface showed fine wrinkling and peau - d orange appearance at places [ figure 2 ] . there was no evidence of ulceration or comedo - like plugs associated with the lesions . there was no underlying developmental anomaly , caf-au - lait macule , neurological abnormality or evidence of any systemic disease . histological examination showed beneath a normal epidermis , aggregates of mature adipocytes in the upper dermis [ figure 3 ] . the patient was counseled and referred to department of plastic surgery but was subsequently lost to follow - up . note the swellings over the lower back also close - up photograph showing fine wrinkling and peau - d orange appearance . besides , there are smaller satellite nodules in the surrounding skin photomicrograph showing a normal epidermis , increased melanization of the basal layer , aggregates of mature adipocytes in the upper dermis , normal vascularity and lack of infiltrate . nlcs is a rare hamartoma that is characterized by the presence of ectopic isolated adipocytes or mature adipose tissue in the dermis . morphologically , the lesions of classical nlcs are asymptomatic , soft , skin colored to yellow papulo - nodules , often coalescing to form plaques . the surface may be smooth , wrinkled , cerebriform , and associated with comedones and peau dorange texture . in our case , the lesions showed prominent peau dorange appearance . the distribution may be linear , systematized , zosteriform or along the lines of skin folds . the lesions are usually located on the pelvic girdle , lumbar area , buttocks and upper thighs , with rare instances of involvement of the scalp , face , shoulder , thorax and abdomen . the disorder may coexist with pigmentary conditions like cafe - au - lait macules , hypertrichosis , leukoderma , capillary hemangioma and trichofolliculoma . however , electron microscopic appearance of lipocytesin in close association with the capillaries suggests hamartomatous origin from the pericytes . we considered the clinical differential diagnoses of plexiform neurofibroma , smooth muscle hamartoma and leiomyoma cutis . the characteristic histopathological finding is the presence of ectopic fat cells in dermis , mostly around blood vessels . goltz syndrome is characterized by the absence of collagen and associated underlying deformities . the pedunculated appearance and later onset in life help to distinguish lipofibroma from nevus lipomatosus . our case had a few unique findings like multiple huge swellings on the back mimicking smooth muscle hamartoma and plexiform neurofibroma , presence of fine wrinkling and peau - d orange appearance at places .

nevus lipomatosus cutaneous superficialis ( nlcs ) is a benign dermatosis , histologically characterized by the presence of mature ectopic adipocytes in the dermis . we hereby report a case of a 10-year - old boy who presented with multiple huge swellings on the scapular regions and lower back . the lesions were surmounted by small papules , along with peau - d orange appearance at places . histology showed features consistent with nlcs . the case is being reported for the unusual clinical presentation .

epstein barr virus ( ebv ) is the cause of mononucleosis that is generally benign disease in children with classical features of fatigue , pharyngitis , and lymphadenopathy . ebv infections have led to subclinical infection , infectious mononucleosis , and associations with malignancies and central nervous system ( cns ) disorders . many neurological manifestations have been described such as seizures , ataxia , meningitis , encephalitis , transverse myelitis , guillain barre syndrome , autonomic dysfunction , anxiety , and depression in this disease . although neurologic complications mostly emerge during acute ebv infection , the reactivated virus may also cause neurologic complications in children . herein , we describe a patient diagnosed by acute ebv encephalitis with magnetic resonance imaging ( mri ) lesion in substantia nigra and good recovery with antiviral and steroid treatment . a 8-year - old boy was referred to our hospital for with 8 days history of fever , decreased alertness , drowsiness , vomiting , tremor , and unresponsive to verbal command . he had a history of fever , sore throat , and for this reason penicillin treatment was prescribed at another hospital before 8 days . on admission , the pupillary light reflex was evident , no neck stiffness , deep tendon reflexes of the lower and upper extremities were normal . cerebrospinal fluid ( csf ) analysis showed : 2 lymphocytes / mm , 18 mg / dl protein , 61 mg / dl glucose ( simultaneous blood glucose : 85 mg / dl ) . encephalitis was diagnosed by clinical and radiological findings and empirical antibiotics and acyclovir were administered . herpes simplex virus , cytomegalovirus , anthyroid antibodies , antinuclear antibody , anti - dsdna antibody , venereal disease research laboratory , brucella serology were negative . serologic studies revealed that weakly positive for : viral capsid antigen ( vca ) igm , vca igg , epstein magnetic resonance imaging revealed bilateral symmetrical hyperintensities in the substantia nigra normal magnetic resonance imaging finding after treatment the boy was diagnosed with acute ebv - related encephalitis based on the clinical , radiological findings , and the serological results for ebv . clinically , ebv can present with meningitis , encephalitis , acute demyelinating encephalomyelitis , cranial nerve palsies , cerebellitis , myelitis , seizures , and polyradiculitis . primary inflammation due to direct viral invasion , secondary autoimmune reaction has been postulated mechanisms of ebv encephalitis . shoji et al . speculated that the neurologic abnormalities were most likely secondary to immune - mediated mechanism . our patient 's sera sample was weakly positive for ebv antibodies but csf pcr was negative for ebv . this imaging finding together with the negative csf pcr result for ebv in our patient suggests that an autoimmune mechanism also may be involved in the pathogenesis but we could n't study autoimmune antibody for technical inadequacy . ebv can also present with a wide spectrum of imaging abnormalities . in 40% of cases with ebv encephalitis , t2-weighted or fluid - attenuated inversion recovery imaging reveals multiple focus hyperintensity in the hemispheric cortex , brainstem , bilateral thalami , basal ganglia , and/or splenium . rarely , extensive white matter lesions have been reported in patients with acute disseminated encephalomyelitis associated with chronic ebv infection . most children have a benign clinical course without neurologic sequelae like our patient , but 10% have residual persistent deficits and a mortality rate of up to 10% has been reported . prognosis was reported to be better for patients with isolated hemispheric grey or white matter involvement . sequel remains in half of patients with thalamus involvement and mortality rate is highest among patients with brainstem involvement . angelini et al . reported that permanent anomalies in the imaging may be related to glial reactions emerging after the recovery of neurological findings . substantia nigra , is an important area for the movement . in recent years , literature reported cases of ebv related substantia nigra involvement in children and adult patients . guan et al . reported a 28-year - old patient presenting to hospital with akinetic rigid syndrome . reported a 20-year - old pregnant women who who presented to hospital with acute parkinsonism with postural and kinetic limb tremor , bradykinesia , nystagmus , saccadic eye movement , and axial dystonia . the patient was diagnosed with encephalitis lethargica . in our patients , lesions were seen in substantia nigra . bilateral substantia nigra lesions are characteristic of some encephalopathies including the japanese and west nile encephalitis . use of corticosteroid can improve clinical prognosis by reducing inflammation and cranial pressure in encephalitis . acyclovir has been recommended for cns involvement with ebv but white matter lesions have been noted with acyclovir treatment in some cases . a pediatric study comprising 11 patients , reported that 40% had residual neurologic sequelae such as global impairment , autistic like behavior , and focal paresis was seen in primary infection . this case demonstrates that ebv - related brainstem encephalitis can be considered as an infectious neurological disease in children .

infectious mononucleosis due to epstein barr virus ( ebv ) is a usually benign systemic viral illness common in children . many studies described nervous system manifestations of infectious mononucleosis with a wide spectrum of neurologic deficits . neurologic complications of ebv are seen in both acute and reactivate infection . herein , we describe a patient diagnosed by acute ebv encephalitis with substantia nigra involvement and excellent clinical recovery .

genital herpes is the most common sexually transmitted disease ( std ) in hiv - seropositive individuals . the severity of herpes infection increases in the presence of immunosuppression . necrotic ulceration may appear at unusual sites and more extensive and chronic lesions may occur . chronic ulcers caused by herpes of more than 1 month duration are an aids - defining illness . a 27-year - old female patient presented with non - healing painful ulcer in the left hand of 6 months duration . an ulcer measuring 10 6 cm was present over the ulnar aspect of the dorsa of left hand which extended to the first three fingers and the palmar aspect of left hand [ figure 1 ] . liver function , renal function , and chest x - ray were within normal limits . mantoux , smear for afb , koh for fungus , and tzanck smear were negative . a biopsy of the ulcer showed neutrophilic vasculitis , necrosis , and ill - defined granulomas . oral steroids were added to the dose after 5 days as the lesions increased in size . after 15 days , her ulcer did not show any improvement but pain was aggravated . cyclosporine 100 mg was advised and after 5 days she reported with vesicles and ulcers over perianal region . on genital examination , multiple vesicles and ulcers with polycyclic border were seen in the perianal region and vesicles were present near the ulcer in the left hand . tzanck smear from perianal vesicle showed multinucleated giant cells whereas from the vesicles on the hand showed only neutrophils . pus culture showed staphylococcus and e. coli , and linezolid 600 mg twice daily was started according to sensitivity pattern . a workup for std revealed hiv 1 elisa to be positive , and result was confirmed by western blot . she had dry cough for 3 days and chest x - ray showed patchy haziness on both sides . after 5 days of famciclovir , her perianal lesions resolved and her hand ulcer showed some improvement with subsidence of pain . the ulcer over the hand healed in a span of 25 days [ figure 2 ] . atypical presentations of herpes such as hemorrhagic deep painful ulcers , vegetating plaque with ulceration , hyperkeratotic verrucous plaques , erythema multiforme , like eruption or generalized erythematous and papulovesicular rash , are found in hiv - affected individuals . in these cases tzanck herpetic infection of the hands usually presents as whitlow , erythema multiforme , or cellulitis . the probable reasons for the development of herpetic ulcer over the hand in our patient have been the transfer of herpes virus from the perianal region to the left hand and subsequent involvement of perianal region . famciclovir in hiv - infected persons with genital hsv infection results in significant reduction in symptoms associated with hsv infection and the symptomatic and asymptomatic shedding of hsv . response to acyclovir is excellent except in few patients with very low cd4 count ( < 50 ) . in such cases , recurrence rate is very high and treatment with higher doses for longer period is required . pyoderma gangrenosum presents as rapidly progressive , painful , suppurative cutaneous ulcers with edematous , blue , undermined , and necrotic borders . ulcers due to arterial or venous disease , vasculitis , cancer , infection , and trauma can be misdiagnosed as pyoderma gangrenosum . a biopsy of an ulcerative variant of pyoderma gangrenosum demonstrates central necrosis and ulceration of epidermis , superficial dermal edema , and dermo - epidermal neutrophilic infiltrate with abscess . pyoderma gangrenosum is a diagnosis of exclusion , and the misdiagnosis of pyoderma gangrenosum and its treatment can result in complications in patients who have other causes of severe cutaneous ulceration . our patient was initially diagnosed as pyoderma gangrenosum and treated with immunosuppressive which might have prompted the increase in ulcer size and appearance of perianal vesicles and gave the clue to the diagnosis of herpes . chronic non - healing large ulcer mimicking pyoderma gangrenosum needs to have a thorough workup prior to initiation of immunosuppressants . although screening for hiv was carried out , the intial omission of this investigation led to misdiagnosis of pyoderma gangrenosum in our case . close long - term follow - up and workup are recommended for all patients with suspected pyoderma gangrenosum .

retroviral infection is known to cause varied manifestation of any disease . we report a 27-year - old female who presented with non - healing painful ulcer in the left hand of 6 months duration . the patient was diagnosed as pyoderma gangrenosum and treated with immunosuppressant drugs . one month later , she developed perianal vesicles and tzanck smear from the vesicles showed multinucleated giant cells . a workup for sexually transmitted disease revealed hiv 1 elisa to be positive . diagnosis of herpetic ulcer was made and treated with famciclovir and hand ulcer gradually healed in 25 days . we report this case to highlight the occurrence of herpetic ulcer in an unusual site in a hiv patient and may lead to misdiagnosis of pyoderma gangrenosum

coronoid hyperplasia is a rare condition which is macroscopically characterized by an increase in the dimensions of the coronoid process , resulting from an abnormal bony elongation of histologically normal bone . the most obvious clinical feature of this condition is reduced mouth opening associated with facial asymmetry . in addition to restricted opening , protrusion and lateral excursions may also be affected . the cause of restricted mandibular movement is widely accepted to be an impingement of elongated coronoid process to the posterior aspect of the zygomatic bone , or to the inside of the zygomatic arch , at the mouth opening . unilateral coronoid hyperplasia is usually due to a trauma or a pathologic condition and is associated with facial asymmetry , more frequently seen in women with histologic chondromatous or neoplastic changes most similar to ostechondroma . however , others consider enlargement of the coronoid process to be a hyperplastic process rather than a true neoplasm . the primary clinical feature of coronoid enlargement is limitation of opening . shira and lister reported the appearance of bony enlargement of the zygoma on the affected side as demonstrated on a submentovertex radiograph . a thorough clinical history should include information about the onset and progression of pain and other subjective symptoms . in the case of coronoid hyperplasia , ct also allows surgical planning due to its accuracy to detect coronoid process volume and morphology . the following is a report of a case of unilateral coronoid enlargement with restricted opening as well as an obvious zygomatic asymmetry . a 15-year - old male with no medical history of interest reported to the out - patient department of government college of dentistry , indore , with the chief complaint of facial asymmetry [ figure 1 ] . preoperative clinical photograph of the patient , revealing facial asymmetry the patient mentioned asymptomatic difficulty in opening his mouth . on examination , he was found to have a reduced mouth opening , with limited lateral and protrusive jaw movements and no hypertrophy of the masseter muscles [ figure 2 ] . reduced mouth opening of the patient a ct scan was carried out with three - dimensional reconstruction ( 3d ct ) , showing unilateral hyperplasia of the coronoid process and contact between said process and the zygomatic arch , limiting mouth opening- movement [ figures 3 and 4 ] . 3d ct reconstruction of face revealing facial asymmetry on left side of face large anteroposterior extent of left mandibular coronoid process as seen in three - dimensional ct reconstruction image due to massive anteroposterior extent of coronoid , transzygomatic approach was considered . a hemicoronal incision ending in a preauricular extension up to the lobule of the ear was made through the skin , subcutaneous tissue , and galea . in the temporal region , this incision was up to superficial layer of the temporal fascia . at the root of the zygomatic arch , hemicoronal incision was taken and zygomatic arch was exposed two plates were adapted , bridging the osteotomy site on the zygomatic arch . the arch was osteotomized , temporalis muscle was stripped off the coronoid , and the process exposed adequately . an osteotomy cut was taken on the coronoid and the enlarged coronoid stump was removed [ figure 6 ] . enlarged coronoid process mass removed zygomatic arch was repositioned in its proper anatomic position and fixation was performed with titanium plates [ figure 7 ] . repositioned zygomatic arch in its proper anatomic position and fixation done with titanium plates the wound was then closed in layers . the patient reported after 6 months with adequate mouth opening and marked reduction in facial asymmetry [ figures 8 and 9 ] . marked reduction in facial asymmetry seen after 6 months postoperatively increase in mouth opening appreciated 6 months postoperatively elongation of the coronoid process of the mandible was described for the first time in 1853 by von langenbeckin . in 1899 , jacob described a synovial joint formation between an elongated coronoid process and the homolateral zygomatic bone . several approaches are advised by different authors for performing coronoidectomy , such as intraoral approach , submandibular approach , and coronal approach . since the coronoid was very large anteroposteriorly , we planned a transzygomatic approach . it can be difficult to establish the best time to administer treatment in infants or preadolescents , although most authors agree that except in patients with very severe limitation of the mouth opening , it is best to perform the operation once the growth process has finished in order to avoid recurrence , deformity , or even restricted movement . active physiotherapy is to be commenced immediately after surgery and continued for at least 6 months for satisfactory results . postoperative cases of limitation of mouth opening caused by fibrosis , secondary to incorrect reorganization of a hematoma at the site of operation , and even recurrence in the growth of the coronoid process have been described . coronoid process hyperplasia as one the causes of mandibular hypomobility is largely underdiagnosed as it is a very rare entity , but a thorough clinical and radiological examination can help to rationalize the line of management and the ultimate clinical outcome . in addition to limited mouth opening , facial asymmetry was also a clinical finding . in case of massive anteroposterior extension of the coronoid , coronoidectomy with transzygomatic approach followed by active immediate physiotherapy

coronoid hyperplasia is a rare disorder , which effectively is a mechanical problem associated with limited mouth opening . in some cases , enlarged coronoid pushes the zygoma forward causing facial asymmetry . this article looks at the case of a 15-year - old boy reporting in opd of government college of dentistry , indore , with chief complaint of facial asymmetry . transzygomatic approach was taken to get proper access to the enlarged coronoid . coronoidectomy was performed which significantly reduced facial asymmetry . postoperative healing was uneventful and recurrence was not reported .

however , patients without any comorbid anomalies or patients with a mild form of this disease can reach adulthood . no significant etiological factors have been found in patients , and thus genetic , teratogenic , and mechanical factors have been thought to be responsible . since it is rarely observed in adulthood , we present a rare case of of pulmonary agenesis in an adult . a 17-year - old female patient presented to our clinic with a complaint of shortness of breath . p / a chest radiography revealed a deviation to the left in mediastinal structures , trachea , and heart ; a narrowing between the ribs in the left hemithorax ; a widening between the ribs in the right hemithorax ; and elevation of the right hemidiaphragm . spirometry findings was consistent with restrictive disease : fvc : 3.01l ( pred % 67.2 ) , fev1 2.59l ( pred % 67 ) , and fev1/fvc : 84.9 ( pred % 102.0 ) . computed thorax tomography revealed the absence of the left lung parenchyma , deviation of mediastinal structures to the left , a compensatory enlargement of right lung into left hemithorax , and rudimentary bronchus in the left [ figure 2 ] . the patient underwent thorax ct angiography , abdominal ct , and pulmonary perfusion scintigraphy . in ct angiography , furthermore , no parenchymal tissue could be observed in the left lung area [ figure 4 ] . the image of nonexistent left pulmonary artery and rudimentary left pulmonary vein . left hemithorax ; no parenchyma was observed . the remaining lung contains twice as much alveoli as normal , but has normal bronchi . although aplasia does not have the same structures , it has a rudimentary bronchus . in our case , too , the left main bronchus was determined as rudimentary . as agenesis and aplasia are difficult to distinguish from each other clinically and pathologically , they are often used in the same sense . on the other hand , pulmonary hypoplasia is characterized by immature lung tissue or by lung tissue decreased at varying degrees . pulmonary aplasia ( agenesis ) is thought to result from the negative effects that occur on the 4th week of fetal life . although its etiology is not fully understood , vitamin a or folic acid deficiency or the use of salicylates may be responsible for it . its incidence in males and females and the occurrence of the anomaly in the right or left lung are about the same . hypoplasia and aplasia are often observed together with other malformations ( diaphragma defects , kidney anomalies , extrapulmonary sequestration , muscle , or skeleton system defects ) . although the most common one is the atrial septal defect , ventricular septal defect , patent ductus arteriosus , or aorta coarctation can also be observed . in our case , too , no pathological findings were found except scoliosis . although the patients with unilateral lung aplasia ( agenesis ) are believed to die usually in the neonatal period , there are patients who live upto the adulthood , some of whom live without any symptoms . , secretions not cleared from the rudimentary bronchus and/or comorbid bronchiectasis may partially be responsible for the risk of infection . in our case , bronchus was rudimentary and an antibiotic treatment was started to clear the symptoms of a respiratory tract infection . in some cases , a significant decrease in vital capacity and exercise intolerance can be seen . chronic respiratory failure and eventually respiratory infections can develop in adults . the restrictive pattern detected in our case was thought to be secondary to the aplasic lung disorder . in the diagnosis of this condition , methods such as contrast - enhanced ct , bronchography , bronchoscopy , pulmonary angiography , and magnetic resonance imaging are also employed . in our case , the diagnosis was made with noninvasive methods such as thorax ct - angiography and ventilation / perfusion scintigraphy . in chest radiography narrowing between the ribs and elevated hemidiaphragm on this side is also observed . in our case , right pulmonary artery was quite long , left pulmonary artery did not exist , and the bronchus was rudimentary . total atelectasia , diaphragmatic hernia , diaphragmatic eventration , pneumonia , pleural effusion , pulmonary hypoplasia , or pneumonectomy should be considered in the differential diagnosis .

pulmonary agenesis is a rare congenital anomaly , the etiology of which is not clearly known . other systemic comorbidities such as cardiovascular , gastrointestinal , musculoskeletal , and urogenital system anomalies can be observed in more than half of the patients . it is usually diagnosed during childhood . diagnosis in adulthood is very rare . we present a case of pulmonary agenesis diagnosed in an adult .

these are worsened by curettage and so a high index of suspicion is necessary for diagnosis . only two cases of hysteroscopic findings in arteriovenous malformations have been reported in literature so far . we present a case of acquired uterine arteriovenous malformation , which showed a characteristic pulsatile vascular mass on hysteroscopy . a 36-year - old lady , p2l2 ( para 2 , living 2 ) underwent medical termination of pregnancy one month ago outside for failure of contraception . she was admitted at our hospital with heavy bleeding . on admission , she was pale , pulse was 110/min , b.p . was 100/70 mm hg and vaginal examination revealed a bulky soft uterus with open os and clots in the vagina . a transvaginal ultrasound showed parenchymal inhomogeneity in the uterus in the anterior wall with several interspersed sonolucent spaces of varying size [ figure 1 ] . her cbc showed a hb of 7 gm% , serum beta hcg was 50 iu / ml . a provisional diagnosis of uterine arteriovenous malformation or organized retained products of conception was made and hysteroscopy proceed was planned . transvaginal ultrasound showing parenchymal inhomogeneity in the uterus in the anterior wall with several interspersed sonolucent spaces of varying size transvaginal ultrasound color doppler showing increased vascularity hysteroscopy revealed a clot in the uterine cavity . there was a pulsatile bluish vascular mass in the fundus extending to anterior wall of uterus . mr angiography confirmed an arteriovenous malformation at the fundus and anterior wall of uterus being fed by mainly by left uterine artery [ figure 4 ] . patient was taken for percutaneous arterial embolization and bilateral uterine artery embolization was done with polyvinyl alcohol particles . patient had cessation of bleeding after the embolization and she resumed menstrual cycle 5 weeks after the procedure with scanty menstrual flow . transvaginal scan revealed reduced vascularity with embolic particles seen in uterine myometrium [ figure 5 ] . hysteroscopy showing bluish vascular mass in anterior and right lateral wall of uterus mr angiography showing arteriovenous malformation at the fundus and anterior wall of uterus being fed by mainly by left uterine artery transvaginal scan revealed reduced vascularity with embolic particles seen in uterine myometrium six months later , patient continues to have cyclic menstrual bleeding with moderate flow . acquired arteriovenous malformations are known to occur after uterine curettage , medical abortion , gestational trophoblastic disease , cesarean section and endometrial carcinoma . they usually present with torrential bleeding requiring blood transfusion and this bleeding is worsened by uterine curettage . the differential diagnosis is retained products of conception , polyps , adenomyosis , endometrial carcinoma and gestational trophoblastic disease . abnormal uterine bleeding is an important cause of morbidity in the midlife and uterine arteriovenous malformations are a rare albeit important cause of such bleeding . transvaginal sonography with color doppler may point to the diagnosis but the condition may be confused with retained products of conception . in our case , the serum beta hcg was 50 iu / ml , which was suggestive of retained products of conception or placental polyp . hysteroscopic picture of arteriovenous malformation has been described in literature as a pulsatile vascular mass in the uterine cavity . in this case there was a clot in the uterine cavity and typical vascular mass , which showed pulsations on reducing the intrauterine pressure . recognition of this rare hysteroscopic picture is important , as arteriovenous malformations are being increasingly diagnosed and treated . hysteroscopy if often the investigation of choice for abnormal uterine bleeding and arteriovenous malformations are a rare but potentially treatable cause of abnormal uterine bleeding . transcatheter embolization of feeding vessels is an established treatment of arteriovenous malformations other being hysterectomy . there are reports of failed angiographic embolization in which definitive treatment i.e. , hysterectomy was offered . , have also been described . acquired arteriovenous malformations arise mostly after pregnancy or pregnancy related complications and they may coexist with conditions like retained placental tissue , retained products of conception and gestational trophoblastic disease . while the treatment of retained products of conception is curettage , in arteriovenous malformations it can lead to torrential hemorrhage . hysteroscopy has an important potential role in the diagnosis of arteriovenous malformation in selected patients . recognition of typical hysteroscopic picture can aid in diagnosis and the coexisting confounding problems like polyps , retained products of conception can also be tackled at the same sitting . however , sometimes even putting in a small hysteroscope can cause torrential hemorrhage in cases with a_v malformations and this should be kept in mind while tacking a suspected case . transcatheter embolization of feeding vessels is a minimally invasive treatment for such lesions and affords an opportunity to manage these cases and salvage the uterus as well .

arteriovenous malformations are a rare but important cause of abnormal uterine bleeding in the midlife . acquired uterine arteriovenous malformations are being increasingly diagnosed by transvaginal ultrasound , color doppler and magnetic resonance angiography . we present a case where the suspected diagnosis was placental polyp or retained products of conception and hysteroscopy showed typical findings of uterine arteriovenous malformation , which was later , confirmed by magnetic resonance angiography . the patient underwent arterial embolization and recovered satisfactorily with resolution of hemorrhage and resumption of normal menstrual cycles . abnormal bleeding due to arteriovenous malformations is worsened by curettage and hence its recognition is important in the management of abnormal uterine bleeding in the midlife .

mechanical ventilation is one of the most frequently employed interventions in the intensive care unit ( icu ) . although it is a life - saving measure , much time and effort is spent in trying to wean patients from the ventilator as quickly as possible , since mechanical ventilation is also a cause of numerous complications . in a recent issue of critical care , supinski and callahan report that infection is a significant risk factor for diaphragmatic weakness and failure to wean patients from mechanical ventilation . the authors employed state of the art methods ( transdiaphragmatic pressure measurements during bilateral magnetic stimulation of the phrenic nerves ) , and found that patients with evidence of infection had less than half the diaphragmatic pressure - generating ability of uninfected patients . in addition , patients with the most severe diaphragmatic weakness had a markedly worse prognosis . this consisted not only of a more prolonged need for ventilator support , but was also reflected in substantially higher mortality . indeed , diaphragmatic function appeared to be a better prognostic indicator than other more conventional indices of critical illness severity , such as the sequential organ failure assessment score . interestingly , the treating physicians in the icu dramatically underestimated ( in 90% of patients ) the degree of diaphragmatic weakness present in their mechanically ventilated patients . they point to a need for greater awareness of the very high prevalence of diaphragmatic weakness in mechanically ventilated patients . the inability to successfully wean patients from mechanical ventilation has been closely linked to an unfavorably elevated level of the respiratory muscle work load / capacity ratio . although great emphasis is appropriately placed upon reducing the numerator in this relationship through attempts at improving respiratory system mechanics , the denominator ( reflecting respiratory muscle function ) is more difficult to assess and often neglected . nevertheless , several studies have now shown that diaphragmatic weakness is common and frequently profound in mechanically ventilated patients [ 4 - 6 ] , although the precise reasons for this are not well understood . based upon the fact that even uninfected patients in the study by supinski and callahan exhibited a large decrease ( to approximately 50% of normal values ) of diaphragmatic force - generating capacity , it seems clear that a major component of the diaphragmatic weakness observed in mechanically ventilated patients must be caused by additional factors other than infection . in this regard , another recent study reported that diaphragmatic weakness was present on the very first day of admission to the icu in patients requiring mechanical ventilation for a variety of conditions , including , but not limited to , sepsis . the study by supinski and callahan did not specifically evaluate the time course for developing diaphragmatic dysfunction , either from the time of icu admission and initiation of mechanical ventilation , or from the onset of infection . when taken together , however , the above studies strongly suggest that diaphragmatic dysfunction constitutes a distinct , common , and under - recognized form of organ failure that occurs with many types of critical illness , and especially during sepsis . the results of these studies in icu patients are also consistent with a large body of data from different animal models , which have consistently demonstrated impaired diaphragmatic function during sepsis . there is limited information about the impact of mechanical ventilation upon sepsis - induced diaphragmatic dysfunction , but the interaction between the two appears to be complex . although mechanical ventilation may mitigate the adverse effects of sepsis upon diaphragmatic function and oxygen demand to the muscle very early in its course , there are several reasons to believe that mechanical ventilation will either worsen or impede recovery from sepsis - induced diaphragmatic dysfunction over the longer term . in this regard , mechanical ventilation itself leads to diaphragmatic atrophy and weakness in non - septic animals and humans , a phenomenon referred to as ventilator - induced diaphragmatic dysfunction ( vidd ) . furthermore , sepsis - induced diaphragmatic dysfunction and vidd appear to share many of the same pathogenetic mechanisms , such as increased oxidative stress and mitochondrial dysfunction within diaphragm muscle fibers . perfect storm , with mechanical ventilation either exacerbating the magnitude of diaphragmatic weakness caused by infection or slowing the subsequent recovery of diaphragmatic function once sepsis has resolved . further studies will be required to specifically address these questions , and there is a clear need for novel therapeutic approaches that can either reverse or limit the development of diaphragmatic weakness in mechanically ventilated patients . the presence of common cellular mechanisms implicated in sepsis - induced diaphragmatic dysfunction and vidd raises the possibility that pharmacologic agents directed at their shared molecular targets might be effective therapies for both conditions .

accumulating evidence indicates that diaphragmatic weakness is common and frequently severe in mechanically ventilated patients . supinski and callahan now report that infection is a major risk factor for diaphragmatic weakness in this patient population . importantly , they show that patients with the greatest levels of diaphragmatic dysfunction have a much poorer prognosis in terms of more prolonged ventilation as well as higher mortality . mechanical ventilation itself has also been found to induce diaphragmatic weakness along with cellular changes resembling those found in sepsis . future studies should be directed at understanding the interaction between sepsis and mechanical ventilation , and to developing therapeutic approaches that target their common cellular pathways implicated in diaphragmatic weakness .

department of health and human services has published statistics presenting total health spending during each year . the basic aim of these statistics , termed nhe , is to identify all goods and services that can be characterized as relating to health care in the nation , and determine the amount of money used for the purchase of these goods and services ( rice , cooper , and gibson , 1982 ) . in this article , we present nhe through an accounting structure to describe historical trends in the size , growth , and distribution of health care by type of service , such as hospital care and physician services , matched against the sources that pay the health care bill , such as private health insurance ( phi ) and government programs like medicare and medicaid . this matrix provides policymakers , researchers , and the public with a system for understanding movement of the aggregate health economy and can serve as a data base from which hypotheses about the causal factors at work can be generated . in the charts that follow , we present detailed information on our nation 's health spending , focusing on calendar year ( cy ) 1997 . the charts are organized into three primary areas : an aggregate overview , the major sources of private and public funding , and the key service areas where our nation 's health dollars were spent . included in the overview are estimates of total aggregate health spending , health expenditures as a share of gdp , the distribution of health spending by primary funding sources and services , and the basic economic factors accounting for the growth in health spending . the overview is followed by more detailed statistics on each of the primary funding sources including phi , medicare , medicaid , and individual consumer health spending , as well as each of the primary service areas , including hospitals , physicians , prescription drugs , nursing homes , and home health services . tables 4 - 13 following the charts provide detailed nhe information for selected cys by type of service and funding source . over the past several years , health care expenditures have shown remarkable moderation in growth . continued excess capacity in hospitals , efforts by employers to hold down cost increases , and the downward impact of bba on medicare costs over the next 5 years should moderate future health care expenditure growth . however , beneath the recent calm waters are potential pressures for increased spending . private insurers are seeing growth in benefit payments that exceed premiums earned and increasing demands by stockholders to improve eroding operating margins . calls for increased quality and choice continue to be debated in state legislatures and in congress , with the potential for accelerating the growth in benefit costs if enacted . at the same time , consolidation in the insurer and provider marketplaces could dampen the intense competitive pressures that have helped to keep the lid on premium price increases in recent years . additional pressures will likely come in the form of increased consumer demand ( smith et al . , recent strong economic growth has boosted employment and wages while prompting employers to offer health insurance to attract a qualified workforce . with rising incomes , the demand for new technologies and treatments will likely increase , including a continued upward trend in the fastest growing nhe category , prescription drugs . although few expect a return to the double - digit growth of the past , significant forces for accelerated growth in future aggregate health spending are emerging .

in 1997 health spending in the united states increased just 4.8 percent to $ 1.1 trillion . as a share of gross domestic product ( gdp ) , national health expenditures ( nhe ) absorbed 13.5 percent of the country 's output in 1997a share that has remained relatively constant for 5 years . despite the relative stability in recent years , signs of changing trends are emerging .

in the current issue senn and colleagues present their findings from a study aimed at validating a new version of the flotrac / vigileo monitor ( edward lifesciences , irvine , ca , usa ) , a system that measures cardiac output using the arterial pulse contour method . the method was first used commercially in the finapres ( ohmedia , englewood , co , usa ) , a device that used a pneumatic finger cuff to continuously measure blood pressure . by transforming the pressure wave into a flow wave , cardiac output was measured from the area under the flow curve . in the past few years a number of pulse contour methods based on the arterial line pressure trace have been developed . the most well known of these methods are the picco ( pulsion medical systems , munich , germany ) , the lidco ( lidco group plc , london , uk ) and the flotrac / vigileo . until recently these systems have all required prior calibration using a second method of cardiac output measurement , either thermodilution ( pulmonary artery or transpulmonary ) or lithium dye dilution . the flotrac / vigileo has recently developed a method of self - calibration based on imputing patient demographic data . the main concern with all pulse contour systems has been their failure to properly adjust to changes in circulatory dynamics , such as blood loss or peripheral vasoconstriction . this failure has lead to more favourable reviews when these systems have been validated in the relatively stable haemodynamic setting of postcardiac surgery , but to less favourable reviews when validated in septic or liver transplant patients , where the circulatory changes are more challenging . the points to note about senn and colleagues ' study are that updated software ( version 1.07 ) was evaluated , by comparing 25 patients using the old software ( version 1.03 ) and 25 patients using the new software ( version 1.07 ) . comparisons of the flotrac / vigileo were also made against pulse contour and transpulmonary cardiac outputs using the pulsion picco system . three serial changes in cardiac output were generated by head - up head - down tilting , and the trending ability was assessed . patients were kept haemodynamically stable throughout the study with propofol remifentanil sedation , a pacing wire to control the heart rate and noradrenaline infusion targeted at a mean arterial blood pressure of 70 mmhg . their data showed a marked improvement in bland and altman limits of agreement between set a data ( 37.5% ) and set b data ( 21.6% ) . there was also a 20% difference in the reference cardiac output between the sets following the head - down tilt , as the set a reference output was 6.1 l / min and the set b reference output was 5.0 l / min . the last observation is relevant because it suggests the two sets of patients were haemodynamically different in respect to tilting responses . differences in nursing management and the use of fluids , sedation and noradrenaline in the intensive care unit could have been responsible for this difference . the improvement in bland and altman limits of agreement from 37.5% to 21.6% is puzzling . judged against the 28.3% acceptable level , the new software version performed very reliably but so did the picco , with limits of 25.5% . the authors attributed this improvement to more frequent recalibration from 10 minutes to 1 minute . readings in the study were taken after 15 minutes of continuous tilt , however , so recalibration should have occurred with both software versions . furthermore , the basic algorithm to adjust for circulatory changes does not seem to have been modified . more recent software ( version 1.10 ) still did not cope with the marked changes in systemic vascular resistance seen in liver transplant patients . these impressive agreement statistics may reflect experimental design and a failure to generate sufficiently large changes in haemodynamics to properly test the device . in set b data the systemic vascular resistance only changed by 5% ( from 960 to 1,008 dynscm ) . head - up tilting to 30 should produce at least a 10% to 20% increase in peripheral resistance in healthy adults . senn and colleagues ' study does not in my opinion comprehensively test the flotrac / vigileo system as their use of tilting did not provoke sufficiently large changes in haemodynamics . little new information can therefore be gained about the reliability of the flotrac / vigileo , other than the new software only improving reliability by more frequent recalibration . the flotrac / vigileo system has yet to be shown to cope with more extreme circulatory conditions . new software versions need to be rigorously tested in the animal laboratory against a true reference standard , such as an aortic flow probe , and need to be shown to track changes in cardiac output reliably over a range of conditions . only then should human studies be performed against thermodilution , using the standard bland and altman approach . finally , one should not forget that the clinical utility of the device would still need to be shown in randomized , clinical controlled trials .

the present study was performed to test a new software version of the flotrac / vigileo using head - up head - down tilting in post - cardiac surgery patients . impressive improvements in bland and altman limits of agreement from 37.5% to 21.6% were recorded . the results , however could be attributed to a failure to produce a wide enough range of test circulatory conditions . a more rigorous test of performance is needed before any real conclusion concerning use of the flotrac / vigileo in clinical practice can be made .

type i neurofibromatosis , also referred to as von recklinghausen s disease , is an autosomal dominant disease characterized by neurofibromas and abnormal cutaneous pigmentation ( cafe - au - lait spot ) . its malignant transformation , that is malignant peripheral nerve sheath tumor ( mpnst ) , accounts for about 510 percent of all soft tissue tumors . common site of the origin for mpnst is the lower and upper extremities , trunk , head and neck . we have recently experienced a case of intrathoracic mpnst in a patient with von recklinghausen s disease . a 40-year - old man presented to chonbuk national university hospital , chonju , korea , due to one rapidly enlarging palpable mass in the right neck which was extended to the supraclavicular fossa . physical examination showed a firm , non - tender and fixed mass associated with ptosis of right eyelid , engorgement of right neck vein , facial swelling and multiple cafe - au - lait spots and pigmented nodular skin lesions on the chest wall and both arms ( figure 1 . chest radiography showed an increased homogeneous mass density at right upper lung field with well - demarcated margin and elevation of right side diaphragm ( figure 1 . magnetic resonance imaging ( mri ) scan of the thorax revealed a homogeneous , well - enhanced and well - circumscribed mass measuring 12108 cm . the central portion of the huge mass showed necrotic tissue which was high signal intensity in t-2 weighted image and low signal intensity in t-1 weighted image . the main mass was located in right upper hemithorax and was associated with multiple adjacent lymphadenopathy . the lesion compressed the trachea , esophagus , right carotid and subclavian artery , and encased the brachial plexus ( figure 1 . , it showed nodular growth pattern and conspicious necrosis in the tumor . densely cellular fascicles alternated with hypocellular zones where the parallel orientation of the cell was lacking ( figure 1 . the nuclei were wavy , buckled , irregular - shaped or occasionally comma - shaped , and the cytoplasm was lightly stained and usually indistinct ( figure 1 . immunohistochemical stain for s-100 protein showed positive reaction on the cytoplasm of tumor cells ( figure 1.(f ) ) . von recklinghausen s disease ( neurofibromatosis type i ) is an autosomal dominant disease with an incidence of about 1 in 3,000 live births . the clinical features of this disorder invariably include cafe - au - lait spots , multiple neurofibromas , lisch nodules or pigmented iris hamartomas . the schwann cell most likely is the cell of origin , but some may originate from perineurium or endoneurium . in our case , the patient showed multiple cafe - au - lait spots and pigmented nodular skin lesions over the chest wall and both arms . mpnst is a kind of malignant tranformation of type i neurofibromatosis , with an incidence recently reported at about 510% . malignant transformation usually occurs in a neurofibroma , which has been present for an extended period of time . the most common anatomic location for malignant schwanomas is the lower and upper extremities , trunk , head and neck , and miscellaneous sites in descending order . this presented case showed an increased homogeneous mass density at the right upper lung field , with well - demarcated margin and elevation of right hemidiaphragm on chest radiographies . radiologic study revealed a homogeneous , well - enhanced and well - circumscribed mass measuring 12108 cm . in our case , the mass was located in the thoracic cavity , extending toward the right supraclavicular fossa and associated with multiple adjacent lymphadenopathy . this lesion compressed the trachea , esophagus , right carotid and subclavian artery , and encased the brachial plexus that caused neurologic manifestation , such as radiating pain with mild numbness , motor weakness of right upper extremity and ptosis . in our case , the histologic examinations of the nodular skin lesion showed expansion of endoneurium by myxoid ground substance , such as neurofibroma . the histologic examinations of intrathoracic mass showed consistent with malignant peripheral nerve sheath tumor which was stained with s-100 protein . our case was not accessible to operate due to the extension of the tumor to adjacent mediastinal structure , so we started radiofrequency ablation and doxorubicin - based chemotherapy . we report a case of intrathoracic mpnst in a 40-year - old man with neurofibromatosis . review of the literature showed that intrathoracic mpnst is very rare but shows highly aggressive manifestation of malignancy and poor prognosis . so , intrathoracic mpnst should be considered in the differential diagnosis of intrathoracic mass in young men with von reckling - hausen s disease .

malignant peripheral nerve sheath tumor ( mpnst ) is defined as any malignant tumor arising from or differentiating toward the cells of the peripheral nerve sheath . mpnst accounts for about 510% of all soft tissue tumors and is often associated with neurofibromatosis type i ( nf-1 , von recklinghausen s disease ) . it is one of the malignant tumors associated with von recklinghausen s disease . its common site is the lower and upper extremities , trunk , head and neck . but intrathoracic manifestations are very rare . we report a case of a 40 year - old man with multiple neurofibromatosis who was presented with an intrathoracic malignant peripheral nerve sheath tumor .

leiomyosarcoma ( lms ) of the inferior vena cava ( ivc ) is a slowly developing tumor which arise from smooth muscle cells of the wall of ivc in 38% cases.1 this tumor reside at the level of segment i represented by sub - renal ivc in 38% cases.2 a 50-year old patient , with no previous medical history , consulted for upper quadrant pain . ultrasound studies and computed tomography ( ct ) scan ( figures 1 , 2 ) revealed a multilobulated right mass , centered by the ivc invading along 7 cm . the right renal hilum was not invaded . magnetic resonance imaging ( mri ) showed an isointense t1 mass and hyperintense t2 emerging from the ivc adjacent to the right kidney ( figure 3 ) . , there was a sub - renal ivc tumor , totally obstructive , located 1 cm from the right renal vein ostium and 5 cm from iliac vein ( figure 4 ) . a resection of the ivc along with the tumor ( figure 5 ) was performed after clamping the ivc above the right renal vein . the post - operative period was complicated by the appearance of lower extremities deep vein thromboses at day 20 which well evolved with anticoagulant therapy . the diagnosis is established pre - operatively in only 10% cases.3 in tumors affecting segment i and ii , there is usually a colicky pain which can be either hepatic or renal . lower extremities edema is a rare manifestation which is due to the slow tumor growth that gives time for venous collateral circulation to develop . in our observation , ct - scan and abdominal mri allowed to precisely locating the tumor relatively to the ivc , its vasculature and within neighboring organs . it can be indicated when there is doubt in establishing the diagnosis or neoadjuvant chemotherapy is planned . in our case , segment i is the localization best suited for resection . if the tumor is not obstructing and limited to one venous wall , a lateral resection with suture can be done . if the tumor is large , totally obstructive , as in our case , wide excision with interruption of the ivc is performed . reconstruction is rarely performed as it is not needed in most cases ; because a well developed alternative circulation usually exist in the totally obstructive tumors.4 in suprarenal ivc , a prosthetic reconstruction might be necessary . the indications for prosthetics are incomplete obstruction of the vena cava or total resection along with many collaterals . however , prosthetic replacement remain a solution but controversial because of the risk of thrombotic events and infections . invasion of the renal veins especially right renal vein is a problem that can be encountered during surgery in tumors of segment i and ii . if the right renal vein is invaded , a reimplantation of the renal vein over the prosthetics , on the portal vein or renal autotransplantation in the iliac fossa is necessary to avoid right nephrectomy . 5 adjuvant treatment of sarcoma is controversial .

vascular leiomyosarcoma ( lms ) are unique . the inferior vena cava ( ivc ) is the most affected organ ( about 38% cases ) . we report the observation of a 50-year old woman who consulted for right upper quadrant pain . imaging studies revealed a retroperitoneal mass that mimic a lms of the ivc . the patient was operated . a resection of the ivc along with the tumor was performed without reconstruction . the management of lms is surgical and depends upon the location and tumor characteristics .

a 22-year - old , right - handed , unmarried woman was admitted to the psychiatric unit due to her selective retrograde amnesia that occurred after a severe stressful event . she could remember nothing of the autobiographical events for the preceding four years , that is , her university life period . she was aware only of a hallucinatory vision of a parking lot in which she was kidnapped and violated by a masked robber . however , she could recount in detail the events of her high school life and also the events of her life before that . there were no neurological signs , no suspicion of head trauma and no epileptic discharge on the electroencephalography . her brain mri was normal . to examine the underlying brain functional disturbances associated with psychogenic amnesia the patient viewed three kinds of face photographs : recognizable faces of familiar high school friends , unrecognizable faces of familiar university friends , and unfamiliar control faces . the patient was instructed to try to recognize the faces that were shown to her . all faces were presented to the patient through a mirror located at the top of the head coil that received the face photographs from outside of the magnetic room during the fmri procedure . the t1-weighted images ( time to repeat / echo time / flip angle = 500 ms/50 ms/90 ) were obtained for anatomical localization by using a 1.5 t mri scanner ( ge medical system , horizon milwaukee , wi , usa ) . the bold - contrast fmri images were acquired from 11 slices that covered the limbic areas with using gradient - echo epi ( time to repeat / echo time / flip angle = 3000 ms/50 ms/90 ) . post - processing of images was performed with matlab ( mathworks , natick , ma , usa ) and spm99 ( statistical paramatric mapping software ; wellcom department of cognitive neurology , london , uk ) . the spm99 software was used for image realignment , normalization , smoothing and to create the statistical maps of the significant relative changes in the regional blood oxygenation level - dependent ( bold ) response . the blocked design fmri paradigm , which was preceded by three dummy scans to allow the magnetic resonance signal to reach a steady state , was comprised of seven repetitions with nine seconds of rest ( fixation cross ) , and six repetitions of a nine seconds activation state ( three kinds of photograph that were randomly comprised of three seconds of recognizable faces of high school friends , three seconds of unrecognizable faces of university friends , and three seconds of unfamiliar control faces ) . a correlation coefficient algorithm was used to correlate the time course data sets with the periodic boxcar function . three different subsets of the activation periods were independently compared with those of the rest periods . the bold signals were estimated for the comparison of whole brain signals across all the experimental conditions . specific effects were tested for by applying appropriate linear contrasts to the parameter estimates for each condition , and this resulted in a z - score for each voxel . the voxels were identified as being significant only if they passed a height threshold of z = 3.5 and they belonged to a cluster of at least 30 activated voxels . the data were analyzed for the main effects of each stimulation : the recognizable faces of high school friends , the unrecognizable faces of university friends and the unfamiliar control faces . for the recognizable condition , different patterns of brain activation were found as compared with the unrecognizable condition . the functional maps of the representative brain slices demonstrated differences for activation in the limbic areas : the amygdala , hippocampus , parahippocampal gyrus and insula . especially , the activation of the amygdala and hippocampus was significantly greater during the stimulation with recognizable faces of high school friends ( fig . 1 ) . however , the activation patterns stimulated with the unrecognizable faces of university friends and the unfamiliar control faces were similar to each other . they showed significantly less activation in the limbic area than the recognizable faces ( fig . in our case , we found significantly different patterns of activation in the limbic system , especially in the amygdala and hippocampus areas , during the stimulation with recognizable faces of high school friends and the unrecognizable faces of university friends . well - known faces have the potential to elicit retrieval of affective responses and personal episodic incidents , and they may evoke activation of the limbic system , especially the amygdala ( 7 ) . the amygdala is located in the anterior medial portion of the temporal lobe , and it is connected with the hippocampal formation and with further cortical and subcortical structures . the amygdala is primarily involved in the acquisition and expression of emotional memories ( 8) . presumably , this patient has an abnormality for the expression of emotional memories during the amnesic period . emotional overflow could have resulted in the memory impairments . for instance , traumatic stress may lead to remarkable encoding and retrieval disturbance , and this is probably due to the increased release of stress - related hormones such as glucocorticoids . the intense release of glucocorticoids may result in morphologic and functional alterations of the limbic structures , especially in mediotemporal regions . these can cause broad changes in the neurotransmitter systems of the brain . in a recent study , the relation between the traumatic stress and functional brain alterations was described in terms of " psychogenic amnesia " ( 9 ) . the hippocampal region is concerned with explicit judgments that are related to the identification of faces . the lack of activation in the hippocampal region might be related to an abnormality in the explicit components of the face judgment ( 10 ) . , this fmri study could give objective evidence and illustrate that a psychogenic amnesic patient has an abnormality in the retrieval of emotional memories during the amnesic period . these findings suggest that changes in the limbic functions are related to the symptoms of psychogenic amnesia .

we present here a case in which functional mr imaging ( fmri ) was done for a patient who developed retrograde psychogenic amnesia for a four year period of her life history after a severe stressful event . we performed the fmri study for a face recognition task using stimulation with three kinds of face photographs : recognizable familiar faces , unrecognizable friends ' faces due to the psychogenic amnesia , and unfamiliar control faces . different activation patterns between the recognizable faces and unrecognizable faces were found in the limbic area , and especially in the amygdala and hippocampus .

direct antiviral agent ( daa ) has been the standard of care for patients with hepatitis c virus ( hcv ) infection . twelve weeks of paritaprevir / ritonavir / ombitasvir plus dasabuvir ( prod ) with or without ribavirin has shown to have a sustained virological response at post - treatment 12 weeks ( svr12 ) rate of > 90% in hcv genotype 1 ( hcv-1 ) patients . it may attribute to low baseline viral loads and extraordinarily rapid suppression of hcv after treatment day1 . the finding may shed light for possible response - guided - therapy for so - called ultra - super - responders in the daa era . whether the dengue virus , the flaviviridae family as with hcv , enhanced the hcv clearance remains unclear and needs further exploration . direct antiviral agent ( daa ) has been the standard of care for patients with hepatitis c virus ( hcv ) infection . the mainstream treatment duration are 12 to 24 weeks depending on regimens and patient characteristics . paritaprevir / ritonavir / ombitasvir plus dasabuvir ( prod ) with or without ribavirin has shown to have a sustained virological response at post - treatment 12 weeks ( svr12 ) rate of > 90% in hcv genotype 1 ( hcv-1 ) patients . in addition to the development of pangenotypic , high potency daas , efforts now are made trying to abbreviate treatment duration to 4 to 8 weeks without compromising efficacy . the 81-year - old female had the history of chronic hcv infection for > 30 years and co - morbidity of depressive disorder and dementia under regular treatment with quetiapine 50 mg / day . due to fear of adverse events and underlying psychiatric disorder , she was ineligible to pegylated interferon / ribavirin , the current reimbursed regimen in taiwan . the genotype and pretreatment viral loads was hcv-1b and 17,019 iu / ml ( real - time hcv ; abbott molecular , des plaines il ; detection limit : 12 iu / ml ) . the result of the acoustic radiation force impulse ( arfi ) was 1.3 m / s , which corresponded to fibrosis stage 2 . daily fixed - dose combination of paritaprevir / ritonavir / ombitasvir ( 150 mg/100 mg/25 mg ) plus twice - daily dasabuvir ( 250 mg ) was administered for the treatment of chronic hcv infection . the hcv ribonucleic acid ( rna ) became rapidly undetectable on day 1 , followed by day 7 and day 14 after dosing . unfortunately , she was admitted due to fever , progressive thrombocytopenia , and elevated liver enzymes . dengue fever infection with dengue virus genotype 2 ( sybr green - based quantitative rt - pcr ) infection was impressed on day 25 of treatment . she received supportive treatment with antipyretics ( paracetamol 500 mg / time as needed ) and intravenous fluids supply during hospitalization . dramatically , the serial testing of serum hcv rna showed that the patient achieved svr4 , svr8 , and svr12 ( table 1 ) . to our knowledge , this is the first report of daa prescription that encountered dengue fever , and the patient benefited from hcv eradication only by 4 weeks of all oral daa regimens . the current evidence support 12 to 24 weeks of interferon - free daas in treating chc . an 8-week sofosbuvir / ledipasvir is recommended for naive , noncirrhotic hcv-1 patients with baseline viral loads < 8 million iu / ml . as observed in a phase 2b study that 8 weeks of prod plus ribavirin had very high svr rates for naive , noncirrhotic patients of hcv-1b ( 96% , 23/24 ) or with low viral loads ( < 800,000 iu / ml , 100% , 9/9 ) . the achievement of svr to only 25 days of prod in this patient may attribute to hcv-1b with low baseline viral loads and extraordinarily rapid suppression of hcv after treatment day 1 . as with interferon - based therapy , the finding may shed light for possible response - guided - therapy for so - called ultra - super - responders in the daa era whether the dengue virus , the flaviviridae family as with hcv , enhances the hcv clearance remains unclear and needs further exploration .

abstractbackground : direct antiviral agent ( daa ) has been the standard of care for patients with hepatitis c virus ( hcv ) infection . twelve weeks of paritaprevir / ritonavir / ombitasvir plus dasabuvir ( prod ) with or without ribavirin has shown to have a sustained virological response at post - treatment 12 weeks ( svr12 ) rate of > 90% in hcv genotype 1 ( hcv-1 ) patients.methods:we report a hcv-1b patient who received only 25 days of prod treatment.results:the patient early terminated treatment due to dengue fever but eventually achieved svr12 . it may attribute to low baseline viral loads and extraordinarily rapid suppression of hcv after treatment day1.conclusions:the finding may shed light for possible response - guided - therapy for so - called ultra - super - responders in the daa era . whether the dengue virus , the flaviviridae family as with hcv , enhanced the hcv clearance remains unclear and needs further exploration .

in the previous issue of critical care , protti and colleagues presented a series of patients with severe hyperlactatemia secondary to biguanide intoxication . traditionally , hyperlactatemia in critically ill patients - and particularly those in shock - was normally interpreted as a marker of secondary anaerobic metabolism due to inadequate oxygen supply inducing cellular distress . this view has recently been challenged with the demonstration that , during shock states , lactate production is , at least in part , linked to an increased aerobic glycolysis through 2 stimulation . we recently demonstrated in a rat model that this mechanism occurs not only during sepsis ( high or normal blood flow ) , but also during hemorrhagic shock ( low blood flow ) . in clinical practice , there are clearly certain situations where hyperlactatemia is predominantly a reflection of tissue hypoperfusion with subsequent anaerobic metabolism . cardiogenic shock , as demonstrated previously , is associated with hyperlactatemia with a very high lactate / pyruvate ratio . in theory , nevertheless , hemorrhagic shock , when prolonged , becomes an inflammatory shock and may therefore behave as septic shock . the problem encountered with sepsis is more complex , although at least two situations are usually accompanied with hypoxia - associated hyperlactatemia . the first situation is septic shock with catecholamine - resistant cardiocirculatory failure , especially in situations of low cardiac output . the second circumstance is septic shock pre - emptively observed prior to volumetric expansion , as illustrated in the study of rivers and colleagues in which hyperlactatemia was associated with signs of poor oxygen delivery . this leads to a decrease in the atp / adp ratio and an increase in the nadh / nad ratio . a decrease in the atp / adp ratio induces both an accumulation of pyruvate , which can not be utilized by way of phosphofructokinase stimulation , and a decrease in pyruvate utilization by inhibiting pyruvate carboxylase , which converts pyruvate into oxaloacetate . an increased nadh / nad ratio also increases pyruvate by inhibiting pyruvate dehydrogenase , and hence its conversion into acetylcoenzyme a. consequently , the increase in lactate production in an anaerobic setting is the result of an accumulation of pyruvate that is converted into lactate , which stems from alterations in the redox potential . this conversion allows for the regeneration of some nad , enabling the production of atp by anaerobic glycolysis - although the process is clearly less efficient from an energy standpoint ( two atp molecules produced versus 36 ) . it is important to consider that the modification of the redox potential induced by an increase in the nadh / nad ratio activates the transformation of pyruvate into lactate , and consequently increases the lactate / pyruvate ratio . all in all , anaerobic energy metabolism is characterized by hyperlactatemia associated with an elevated lactate / pyruvate ratio , greater glucose utilization and low energy production . recent observations have suggested that metformin , similarly to phenformin , may also inhibit mitochondrial respiration in tissues other than the liver . in the previous issue of critical care , using indirect measurement of oxygen consumption , protti and colleagues found that body oxygen consumption was markedly depressed despite a normal cardiac index evoking an inhibition of mitochondrial respiration . unfortunately , arterial lactate / pyruvate and acetoacetate/-hydroxybutyrate ratios , as reflections of cytoplasmic and mitochondrial redox states , were unavailable . interestingly , there was a clear correlation between drug clearance , correction of lactic acidosis and normalization of oxygen consumption . clearly , the inhibition of mitochondrial respiration is not the unique mechanism involved in biguanide - induced lactic acidosis , since pure inhibition of mitochondrial function during cyanide poisoning is associated with death in the absence of antidote , and , similarly , since lactic acidosis associated with the use of nucleoside analogue reverse transcriptase inhibitors is due to an impairment of mitochondrial oxidative phosphorylation and is also associated with high mortality despite prompt therapy . to conclude , when looking at the literature , pure hypoxic causes of lactic acidosis are relatively rare in clinical practice . in the case of biguanide - induced lactic acidosis , the fact that the inhibition of mitochondrial respiration is reversible should encourage the early use of dialysis in order to accelerate drug elimination

biguanide poisoning is associated with lactic acidosis . the exact mechanism of biguanide - induced lactic acidosis is not well understood . in the previous issue of critical care , protti and colleagues demonstrated that biguanide - induced lactic acidosis may be due in part to a reversible inhibition of mitochondrial respiration . thus , in the absence of an antidote , increased drug elimination through dialysis is logical .