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the risk of cardiovascular disease and cardiovascular mortality is increased in patients with inflammatory rheumatic diseases compared to the general population or to persons without these diseases . although an increased risk is most well established for patients with systemic lupus erythematosus and rheumatoid arthritis , some evidence suggests an increased risk also exists for patients with ankylosing spondylitis , psoriasis , and psoriatic arthritis [ 1 - 5 ] . although the reasons for this increased risk are not completely understood , the association between markers of inflammation , in particular , high - sensitivity c - reactive protein ( hs - crp ) , and cardiovascular risk in patients without known inflammatory disease has led to speculation that systemic inflammation itself may be etiologic [ 6 - 9 ] . evidence that cardiovascular events are reduced in patients who respond to anti - inflammatory treatment supports this view . the jupiter study ( justification for the use of statins in prevention : an intervention trial evaluating rosuvastatin ) tested whether treatment with rosuvastatin altered the risk of incident cardiovascular events compared to placebo in persons with a normal level of low - density lipoprotein ( ldl ) cholesterol ( less than 130 mg / ml ) but an elevated level of hs - crp ( 2.0 mg / l or higher ) . persons with systemic inflammatory diseases , including severe arthritis and systemic lupus erythematosus , were excluded , as were those being treated with prednisone and other immunosuppressive medications . subjects ( n = 17,802 ) were randomized to receive either 20 mg rosuvastatin daily or placebo , for a planned duration of 5 years . the study 's primary endpoint was the occurrence of a first major cardiovascular event , including myocardial infarction , stroke , hospitalization for unstable angina or arterial revascularization , or death from a cardiovascular cause . the study was stopped prematurely when an interim analysis found that cardiovascular events were significantly less frequent in the group receiving rosuvastatin . the primary endpoint was 44% less likely in the rosuvastatin group than in the placebo group , although the rates of events were low in both groups . there was also a modest reduction in all - cause mortality in the rosuvastatin group . at 12 months , the median hs - crp level was 37% lower in the rosuvastatin group compared to placebo , from a baseline level of 2.2 mg / l , and median ldl cholesterol was 50% lower in the rosuvastatin group compared to placebo , from a baseline level of 186 mg / dl . these results indicate that treatment with rosuvastatin can decrease cardiovascular events among patients with an elevated hs - crp level who do not have cholesterol levels elevated to the threshold customarily used to begin treatment . one possible implication of these results is that primary prevention strategies should be broadened to treat with statins those whose only cardiac risk factor is an elevated hs - crp level . but is this an appropriate conclusion ? although subjects were selected based on both an elevated hs - crp level and a normal ldl cholesterol level , the trial did not include a group with low hs - crp levels and , therefore , did not isolate the benefit to patients with elevated hs - crp levels specifically . nor did it test hs - crp as a screening tool to target treatment , which would have required a parallel arm of subjects who had not been tested for hs - crp and were treated without regard to hs - crp level . in the strictest interpretation , the jupiter trial expands the potential mechanisms by which statins reduce cardiovascular events to include reductions in hs - crp levels . whether this effect is completely independent of the hypocholersterolemic effect is uncertain . given the elevated cardiovascular risk experienced by patients with inflammatory rheumatic diseases , and the association of elevated crp levels with cardiovascular disease , how should the results of the jupiter trial be applied to patients with inflammatory rheumatic diseases ? if hs - crp levels in persons without inflammatory diseases reflect inflammation in atherosclerotic endothelial lesions , while hs - crp ( or crp ) levels in patients with inflammatory diseases primarily reflect inflammation outside the atherosclerotic endothelial lesions , elevated hs - crp levels would not be specific to the etiologic target in patients with inflammatory diseases . in this case , targeting treatment based on hs - crp would not be helpful , and treatment might not be expected to have the same effects . statins have multiple immunomodulatory actions , which may have a role in their cardioprotective effects . differences in activation or regulation of these immune and inflammatory pathways between patients with chronic inflammatory rheumatic diseases and people without these diseases complicate using the findings in non - rheumatic cohorts to predict effects in rheumatic disease cohorts . also to be considered is whether any benefit of statins for primary prevention would be attenuated or negated by anti - rheumatic treatments , particularly chronic corticosteroids . lastly , the long - term safety of statins and unknown risks of prolonged marked hypocholesterolemia would need to be balanced against the potential benefits . given the difficulty applying the results of the jupiter trial to patients with inflammatory diseases , it would be premature to recommend statins for primary prevention of cardiovascular events in patients with inflammatory rheumatic diseases . rather , cardiac risk factor assessment and modification should be enforced , while awaiting the results of ongoing or recently completed studies specifically investigating statins for primary cardiovascular disease prevention in systemic lupus erythematosus and rheumatoid arthritis [ 13 - 15 ] .
patients with inflammatory rheumatic diseases have an increased risk of cardiovascular disease , raising questions of whether primary prevention strategies that are more aggressive than cardiac risk factor modification alone should be implemented . recent trials demonstrating the efficacy of statins in reducing rates of cardiovascular events in healthy persons with elevated levels of c - reactive protein broaden the potential protective mechanisms of statins , but do not directly translate to primary cardiovascular disease prevention in patients with inflammatory rheumatic diseases .
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a variety of high - throughput methods have made it possible to generate detailed temporal expression data for a single gene or large numbers of genes . one challenge is the comparison of temporal expression data obtained from different growth conditions where the patterns of expression may be shifted in time . we propose the use of wavelet analysis to transform the data obtained under different growth conditions to permit comparison of expression patterns from experiments that have time shifts or delays . we demonstrate this approach using detailed temporal data for a single bacterial gene obtained under 72 different growth conditions . this general strategy can be applied in the analysis of data sets of thousands of genes under different conditions .
a variety of high - throughput methods have made it possible to generate detailed temporal expression data for a single gene or large numbers of genes . common methods for analysis of these large data sets can be problematic . one challenge is the comparison of temporal expression data obtained from different growth conditions where the patterns of expression may be shifted in time . we propose the use of wavelet analysis to transform the data obtained under different growth conditions to permit comparison of expression patterns from experiments that have time shifts or delays . we demonstrate this approach using detailed temporal data for a single bacterial gene obtained under 72 different growth conditions . this general strategy can be applied in the analysis of data sets of thousands of genes under different conditions .
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pneumatosis cystoides intestinalis ( pci ) was first described by duo vernoi during a cadaveric dissection in 1730.1 it is characterized by the presence of multiple gas - filled cysts in the intestinal wall and the submucosa and/or gastrointestinal subserosa . the overall incidence of pci in the general population has been reported to be 0.03%.2 it is usually a secondary finding caused by a wide variety of underlying gastrointestinal or extragastrointestinal diseases , but can be idiopathic in rare cases.3 clinical signs and imaging features in these situations may mimic those of true abdominal viscera perforation , so correct diagnosis is imperative because the treatment of uncomplicated pci is generally conservative . management options range from supportive care to surgical resection.4 here , we report a rare case of segmental pci probably due to repeated twisting of hypermobile mesentery . a 47-year - old man was referred to our gastroenterology department with recurrent episodes of abdominal pain over a period of 1 year associated with intermittent absolute constipation , abdominal distension , and occasional bilious vomiting . a ct scan of the abdomen suggested pci in the small bowel loops and mesentery ( fig . the mesentery and branches of the superior mesenteric artery and superior mesenteric vein were twisted ( fig . 2 , 3 ) with minimal pneumoperitoneum ( fig . intraoperatively , the patient was found to have segmental pci involving most of the ileum ( fig . pci is a rare condition characterized by the presence of air - filled cysts in the bowel wall and mesentery . a previous study reported that 20%-51.6% of all pci cases involve the small intestine , 36%-78% involve the colon , and 2%-22% include both the small intestine and the colon . when the air - filled cysts rupture they cause pneumoperitoneum , which is often benign.4 the pathogenesis of pci has been extensively discussed , and multiple theories have been proposed . three different possibilities for the origin of gas within the intestinal wall have been suggested : intraluminal gas , pulmonary gas , and gas produced by bacteria . two basic mechanical features are responsible for the intrusion of intraluminal gas into the bowel wall : mucosal injury ( the most important and prevalent feature ) and increased intraluminal pressure , or a combination of both . increased intraluminal pressure may be produced by bowel obstruction , ileus , or iatrogenically by upper or lower gastrointestinal endoscopy . damage to the mucosa may result from an inflammatory process , ischemia , a defect in the gut immune barrier , and steroid or cytotoxic medical therapy.56 pci can be divided into primary and idiopathic ( 15% ) or secondary ( 85%).3 numerous gastrointestinal diseases , including necrotizing enterocolitis , cd , uc , diverticular disease , and sigmoid volvulus may accompany pci as a secondary cause . pci has also been reported to be associated with some non - gastrointestinal diseases , such as chronic obstructive pulmonary disease and collagen tissue diseases.7 disease location can act as a good predictor of etiology , with proximal disease usually being secondary to pyloric stenosis , gastric malignancy , or ulcers , and distal disease usually being a result of diverticulitis or mesenteric ischemia.8 interestingly , segmental disease , as seen in our case , is rarely found . the pci reported in the present case was probably secondary to repeated twisting of hypermobile mesentery resulting in ischemia with disruption of the mucosal barrier in the affected segment of bowel . chronic obstructive pulmonary disease is often related to the development of pneumatosis intestinalis , but was excluded in our patient . the overall incidence of pci in the general population has been reported to be 0.03%.2 the disease is usually asymptomatic . patients may present with non - specific symptoms , such as abdominal discomfort , diarrhea , constipation , or rectal bleeding . severe complications like volvulus , intestinal obstruction , bleeding , intussusception , and intestinal perforation may be seen in 3% of patients.9 the initial priority in the management of pci is to clarify whether the underlying pathology is life threatening or benign . as seen in the present case , it is common for imaging studies such as plain radiography or ct to demonstrate features suggestive of free intraperitoneal gas ( fig . it is important to consider the possibility of a perforated viscus , and thus crucial to correlate radiographic signs with clinical findings . certain radiographic features , such as crescentic or linear gas collection , can be indicative of bowel infarction.10 in the case of a suspected life threatening condition such as bowel ischemia , surgical intervention in the form of an emergency exploratory laparotomy is mandatory.8 conservative approaches , including nasogastric decompression , intestinal rest , antibiotic therapy , and oxygen are recommended for symptomatic patients with normal biochemical parameters who are confirmed by radiological examination to have no intestinal ischemia.9 however , with high associated mortality rates of between 22%-50% , non - surgical management should be used with caution.4 here , we report a rare case of segmental pci probably due to repeated twisting of hypermobile mesentery . clinical and imaging features may mimic those of visceral perforation or bowel ischemia . pci can be a cause of severe abdominal pain , which may require surgical intervention .
pneumatosis intestinalis is a rare condition that affects 0.03% of the population . pneumatosis cystoides intestinalis ( pci ) is characterized by the presence of multiple gas - filled cysts in the intestinal wall and the submucosa and/or intestinal subserosa . it is usually a secondary finding caused by a wide variety of underlying gastrointestinal or extragastrointestinal diseases . here , we present the case of a 47-year - old man who was referred to our gastroenterology department with a history suggestive of intermittent small bowel obstruction associated with abdominal pain . abdominal computed tomography demonstrated pci of the small bowel . the mesentery and branches of the superior mesenteric artery and superior mesenteric vein were twisted with minimal pneumoperitoneum . exploratory laparotomy was performed , and demonstrated segmental small bowel pci secondary to hypermobile mesentery . the affected segment of the ileum was resected , and jejunoileal anastomosis was performed . here , we report a rare case of segmental pci probably due to repeated twisting of hypermobile mesentery . the clinical and imaging features of this disorder may mimic those of visceral perforation or bowel ischemia . pci can be a cause of severe abdominal pain that may require surgical intervention .
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sinonasal teratocarcinosarcoma ( sntcs ) is a distinctly rare tumor characterized by variegated histologic features and has combination of both epithelial and mesenchymal components . malignant tumors which consisted of carcinoma and teratoma were previously reported as teratoid carcinoma , malignant teratoma , blastomatous tumors , and blastoma . it was first described by shanmugaratnam et al . in 1983 and was aptly termed as " teratocarcinosarcoma " by heffner and hyams in 1984 . probably , this is the first case report of sntcs from eastern india . by reported accounts , a 60-year - old man presented with occasional bleeding from nose and nasal obstruction for last two months . x ray of paranasal sinus showed a mass in the posterior nasal cavity . a computed tomography ( ct ) scan revealed a solid tumor with isodensity . the mass was in the posterior nasal cavity and nasopharyngeal wall extending into left ethmoidal sinus [ figure 1 ] . diagnostic imaging suspected of sinonasal malignancies such as poorly differentiated carcinoma , adenocarcinoma , and malignant lymphoma . ct scan showing a mass involving the left paranasal sinus and ethmoid sinus the patient was operated with all antiseptic measures under general anesthesia during caldwell - luc operation . cheek was retracted and an incision was made above the canine teeth in the buccogingival sulcus . it was about 3 - 5 cm long and was placed high enough to prevent damage to the dental roots . a part of the anterior wall of left antrum was removed with a chisel and hammer to make an opening allowing an access into the antral cavity . multiple gray - white tissue bits , largest measuring 3.5 3 2.5 cm received grossly for histopathological examination . the epithelial portion of the tumor was composed of benign and malignant squamous epithelium , glandular structure , and primitive neuroectodermal / blastemal cells [ figures 2 and 3 ] . teratoid components included this " fetal appearing " clear cell squamous epithelium and primitive neuroectodermal / blastemal cells . in the mesenchymal components ; mib1 ( ki 67 ) positivity was detected in 25% of the mesenchymal components and 37% of the epithelial components of the malignant tumor , whereas the clear cell epithelia revealed mib1 ( ki67 ) positivity in 5% of cells . a whole body ct scan was done at six months interval to detect any recurrence or metastasis . follow - up examination for two years after combined surgery and radiotherapy has shown no evidence of recurrence or metastasis . photomicrograph showing fetal appearing ( clear ) squamous epithelial cells , glandular components , and primitive neuroectodermal / blastemal cells in the background . [ h and e , 100 ] photomicrograph showing malignant epithelial cells arranged in glandular pattern . malignant tumors having teratoma and carcinoma were reported previously as teratoid carcinoma , malignant teratoma , or blastematous tumors . heffner and hyams in 1984 first suggested the tumor to be called as teratocarcinosarcoma in order to describe the complex histological pattern of these neoplasms . histologically , it is different from true carcinosarcoma which consists of a single malignant epithelial and a single malignant mesenchymal component , whereas sntcs has one or many epithelial and mesenchymal components ( both benign and malignant).- variegated architecture and tissue heterogeneity are characteristics of this malignancy . fetal - type " clear cells , squamous epithelium , and immature neuroepithelium represent important histologic characteristics useful in diagnosis . though immature neuroepithelium ( primitive neuroectodermal / blastemal ) was present , differentiated neuroepithelial cells with rosette formation were not evident . despite several studies , heffner and hyams postulated that the tumor originates from olfactory membrane due to presence of neural tissue . some authors believe that sntcs probably originates from primitive embryonic tissue or immature pleuripotential cells . a histogenetic origin from a multipotential adult somatic stem cell with divergent differentiaton has been favored over a germ cell origin . this assumption has been based on the lack of germ cell elements and , until recently , the absence of demonstrable amplification of 12p in tumor cells . tumor cells showing squamous cell differentiation were characterized by desmosome - like junction and intracellular tonofilaments . some of the stromal spindle cells had actin filaments with dense patches and dense core granules . shubhada et al . , observed chemotherapy - induced neuronal maturation in sinonasal teratocarcinosarcoma . in that case , the tumor was excised after four cycles of neo - adjuvant chemotherapy . on microscopic examination this case highlighted morphologic evidence of chemotherapy - induced maturation in the neuroectodermal component within sntcs . in a study by budrukkar et al . , disease recurred in 11 out of 14 patients , with a median time to recurrence of seven months . multimodality treatment , in the form of a combination of surgery , radiation therapy , and chemotherapy , appears to be the optimal approach . a combination of radiotherapy and surgical treatment offers the best five - year survival rate ( 50% ) ; followed by only surgical treatment ( 47% ) . in the recurrent or metastasis lesion , adjuvant chemotherapy may improve the survival rate since the metastatic tissue often contains sarcomata components .
sinonasal teratocarcinosarcoma ( sntcs ) is a very rare and aggressive malignant neoplasm histologically characterized by the combination of one or many components of epithelial and mesenchymal elements . here , we report a sntcs in a 60-year - old man involving posterior nasal and nasopharyngeal wall extending into left ethmoidal sinus . the patient complained of bleeding from nose , nasal obstruction , and generalized weakness for last two months . tumor was completely removed by caldwell - luc operation and postoperative radiation therapy was given . the follow - up of the patient for two years has shown no evidence of recurrence or metastasis .
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schizophrenia and other different types of psychosis are the most frequent disorders , which may be accompanied by visual hallucinations ( 1 ) . visual hallucinations have been reported in 16% to 72% of patients with schizophrenia or schizoaffective disorders and it is one of the diagnostic criteria of schizophrenia ( 2 - 4 ) and its presence is related to the severity of illness ( 5 ) . nowadays ant dopaminergic antipsychotics and serotonin - dopamine antagonists are the only therapeutic choices and recently anti - acetyl cholinesterase agents have been suggested ( 6 ) . despite all these treatments , patients may have refractory hallucinations . transcranial magnetic stimulation ( rtms ) is a technique using magnetic field for stimulation of brain . in july 2011 , the fda introduced rtms system as class ii ( special controls ) in the federal register by the phrase of : in order to provide a reasonable assurance of safety and effectiveness of these devices ( 7 ) . rtms could be a promising therapeutic method for visual hallucinations , due to the theories which suggest cortical hyperactivity as a probable mechanism for unreal visual sensations ( 8 - 10 ) . firstly , a 52-year - old patient who lost his vision partially follows a myocardial infarction . ( 11 ) at harvard medical school reported immediate remission lasting for a week following stimulating the visual cortex ( 2 cm above the patient s inion , 1 hz , 10 minutes ) , which had not been induced by sham stimuli given previously . the researchers suggested that probably the reduction of increased cortical excitability is the mechanism of responsiveness . the second case was a 32-year - old patient with refractory paranoid schizophrenia affected by complex hallucinations involving auditory , visual and olfactory systems . ( 8) in france conducted a functional neuroimaging with electrical source reconstruction and considered occipito - temporal sulcus as the target of stimulation . in 2009 , the team reported that 10 sessions of low frequency ( 1 hz ) , suppressed hallucinations significantly leaving minimum residues including tinnitus and phosphine . the third case was a 50-year - old woman experiencing visual sensations including changing colors and lights since loss of her vision bilaterally , suggestive of the charles bonnet syndrome . functional mri showed hyperactivity in extra - striate visual area v5/mt and the fusiform gyrus ( v4 ) related to visual sensations , where meppelink et al . the premier sham stimulation on v5 could not make any change , but later session of real rtms by frequency of 1 hz at v5/mt and v1 led to almost complete suppression of a part of hallucinations for two days . the patient was a 29-year - old woman who had received psychiatric treatments since the age of 19 , following talking to herself , grandiosity and persecutory delusions , irritability and aggressiveness . the diagnosis of schizophrenia confirmed by scid - i interview based of dsm - iv - tr , when the visual hallucinations commenced without symptoms of any mood disorder . electroencephalography and brain magnetic resonance imaging did not reveal any abnormality , as well as laboratory results . the content of hallucinations was strange persons with threatening facial expressions . at the time of introduction to neurotherapy unit of rasoul - akram hospital in december 2013 , she took 400 milligram of clozapine per day , but she still had refractory visual hallucinations . she had insight that these persons are not real , but they still made her anxious . she had experienced a wide variety of classical and atypical agents with full therapeutic dosage and for enough duration that just resulted in few periods of transient partial remission . the team decided to perform inhibitory stimulation on her brain hoping to diminish hallucinations . the patient received 12 sessions of rtms , 5 times per week ( 1 hz frequency , for 20 minutes , 100% of the motor threshold , magventure ; mag machine pro 100 ) on o2 , continuing the medications as before . before the fifth session , she reported decrease in the frequency of hallucinations as low as 5 , considering 10 for description of the pretreatment situation using analog visual scale . at the end of therapeutic course , she reported that the frequency and severity limited to 2.5 . her mother and her psychiatrist also confirmed improvement in her symptoms , mood and irritability . she did not report any immediate or delayed adverse effect . at follow - up interview after three months , she was still satisfied with the outcomes and hallucinations were limited to rare appearances of humanoid features . this result supports our hypothesis about effectiveness of rtms in treatment of visual hallucinations and is compatible with the cortical hyperexcitability theory about its pathogenesis . as it can be concluded of the dr jardri s case report , functional neuroimaging may be beneficial in localizing a specific target for stimulation and may prevent wasting time and cost on targets , which are less possible to be involved in the pathogenesis ( 8) . although this combination seems to be efficacious , it may not be possible everywhere . existing evidence suggests in the absence of functional imaging that occipital area is a promising target , because of probable role of the occipital cortex in the chain of pathogenesis of visual hallucination ( 13 ) . rtms can be investigated as a method for the treatment of visual hallucinations , especially for refractory types . we suggest basic studies by functional imaging on visual hallucination ( both in refractory and responsive cases ) . priority of basic localizing studies may prevent bias in the results of studies to ineffectiveness due to targeting irrelevant points , and leads to acceleration in achievement of a consensus on efficiency of rtms on visual hallucinations .
a 29-year - old woman with schizophrenia introduced for application of repetitive transcranial magnetic stimulation for refractory visual hallucinations . following inhibitory rtms on visual cortex she reported significant reduction in severity and simplification of complexity of hallucinations , which lasted for three months . rtms can be considered as a possibly potent treatment for visual hallucinations .
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kaposi 's sarcoma ( ks ) is a rare angioproliferative tumor associated with human herpesvirus 8 ( hhv-8 ) infection . four clinical variants of ks exist : classic ( predominantly located at the lower extremities of older men from mediterranean areas ) , endemic ( in younger africans with frequent local invasive and/or visceral disease ) , iatrogenic ( in organ recipients on immuno sup ression ) , and hiv - associated ( often presenting with multifocal skin lesions and frequent visceral disease ) . the latter mainly affects hiv - positive men having sex with men ( msm ) . we herein report a hiv - negative msm with ks who does not match with these 4 clinical subgroups . a 53-year old german patient presented with a rapidly growing nodule of about 1 cm in diameter located on his left foot ( figure 1 ) . histo - pathological evaluation of the lesion revealed a strong proliferation of spindle - shaped cells outlining irregular slits and clefts , consistent with ks . the patient reported three similar lesions on his chest 6 years previously that also turned out to be ks . several hiv tests performed within the last years were negative , and re - testing at our institution was negative as well . cd4cells were 563/l ( range : 410 - 1590/l ) , cd8cells were 83/l ( range : 190 - 1140/l ) , and cd4/cd8ratio was 6.82 ( range : 0.8 - 2.0 ) . complete work - up ( including chest radiographs and abdominal and lymph node ultrasound examinations ) disclosed no signs of visceral involvement of his ks . the patient reported never having had receptive and/or insertive anal intercourse , but reported to have frequently engaged in oral sex . strong immunoreactivity of hhv-8 is present in the patient 's skin lesion ( hematoxylin , original magnification 200 ) . kaposi sarcoma in hiv - negative msm has only been reported sporadically , and most of these anecdotal cases were published about two decades ago . however , a french study from 2008 reported on the clinical features and outcome of a total of 28 hiv - negative msm with ks . it was shown that ks in these patients clinically resembles classic ks but occurs at a younger age , is limited to the skin , and is associated with a good prognosis . ks of hiv - negative msm in this study accounted for approximately 9% out of a cohort of 300 hiv - negative individuals with ks , indicating that ks in these patients is not absolutely rare . in northern europe hhv 8 seroprevalence is low in the general population ( < 5% ) , in msm a seroprevalence of up to 25% has been described . epidemiologic studies have shown that oral exposure to infectious saliva is the major risk factor for the acquisition of hhv-8 among msm . this corresponds to our patient 's sexual history , as he reported exclusively oral intercourse . interestingly , ks has also been observed in msm with long - time hiv - infection on successful antiretroviral therapy . all nine patients in this report had high cd4cells and hiv viral loads below the detection limit , and only mild cases of ks restricted to the skin were seen in these people . systemic treatment with interferon or liposomal doxorubicin is reserved for wide spread disease or systemic involvement . physicians treating msm should have in mind that ks might affect both hiv - positive and negative msm , regardless of their cellular immune status .
kaposi 's sarcoma ( ks ) is a rare angioproliferative tumor associated with human herpesvirus 8 ( hhv-8 ) infection . four clinical variants of ks have been described : classic , endemic , iatrogenic and hiv - associated . we describe a 53-year - old men who had sex with men with a rapidly growing nodule on his left foot . histologically ks was confirmed . our patient did not match the clinical subgroups as hiv infection or other immune disorders could be ruled out . ks in hiv - negative msm has only been reported sporadically . it was shown that ks in these patients clinically resembles classic ks but occurs at a younger age , is limited to the skin , and is associated with a good prognosis .
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gout is an illness characterized by the deposition of monosodium urate crystals in the joints or in soft tissues,1 2 leading to acute inflammation with associated pain , impaired movement of the affected joint , and hyperuricemia.3 the small joints of the limbs and the elbows and knees are the usually affected sites.3 4 5 the temporomandibular joint ( tmj ) is rarely involved.3 4 5 there are currently few reports in the english medical literature relating gout and tmj . the article aims to report a presentation of gout in the tmj and discuss the pertinence of the role of physical and complementary exams addressed to the case . a 66-year - old white man presented for consultation at the department of oral and maxillofacial surgery , centro mdico rio branco , in may 2011 . he reported occasional pain in the left temporal muscle region for 1 year . in this period , the patient used nonsteroidal anti - inflammatory drugs ( nsaids ) with improvement of the symptoms . there was no history of diseases , although the patient reported hyperuricemia in previous hematologic exams . extraoral physical examination showed a volumetric augmentation in the left temporal region , matching the patient report ( fig . the patient denied previous trauma in the examined regions . increased volume in the left temporal region on physical examination . due to the suspicion of a gout manifestation , the blood tests showed high glucose ( 117 mg / dl reference values : 60 to 99 mg / dl ) , high creatinine ( 1.6 mg / dl reference values : 0.4 to 1.3 mg / dl ) , and high uric acid ( 8.1 mg / dl reference value : 3.4 to 7.0 mg / dl ) . ultrasonography indicated a normal parotid gland and a left hypoechoic palpable area , with well - defined contours measuring 3.7 1.0 cm ( fig . 2 ) . according to the radiologists report , the examined region presented alterations that may correspond to accumulation of subcutaneous tissue . orthopantographic radiography revealed a change in the morphology of the left mandible condyle ( fig . 3 ) . ultrasonography revealing a well - defined hypoechoic area , measuring 3.7 the sum of anamnesis , physical examination , and laboratory tests undoubtedly indicated a gout manifestation in the tmj . the patient was informed of his condition and referred to a rheumatologic consultation in july 2011 . gouty arthritis is a metabolic illness that most often affects middle - aged men and presents with pain , swelling , local sensitivity , and , in the majority of cases , hyperuricemia.2 3 monosodium urate deposits are located mainly in some small joints of the upper and lower limbs.3 4 pain and limitations of mandibular functions may suggest some diagnoses such as arthritis , inflammatory diseases , or tumors of the tmj.5 6 the manifestation of gout should be also included in the diagnoses despite of its rare occurrence.5 6 bhattacharyya et al presented , after a clinical trial , some features of tmj gout such as limited mouth opening with mandibular deviation and the occurrence of pain , swelling , joint sounds , and impaired movement.5 suba et al also reported decreased mouth opening as a symptom of gout.3 cacioppi et al presented a unique case of malocclusion of the mouth associated with extensive involvement of gout in the whole body as well as symptoms indicating rheumatoid arthritis.1 the case presented here showed coincidental features noted by other authors . the patient was a 66-year - old man complaining of pain and limitations and swelling in the left tmj region . however , a meticulous anamnesis and a thorough physical examination are paramount for the correct diagnose.1 5 6 because our patient reported a previous blood exam showing hyperuricemia , the clinical investigation was directed to a possible gout manifestation . according to suba et al,3 the elevated concentrations of urate in the serum and in the urine or the knowledge of previous episodes of gout can help differentiate the illness from other joint diseases . because one - third of patients present with hyperuricemia , radiography , computed tomography , and biopsy of the lesions could help confirm suspected cases of gout . nevertheless , kleinman and ewbank asserted that the high levels of uric acid in the bloodstream at the time of the consultation , associated with the remission of the symptoms after drug treatment ( nsaids , glucocorticoids , and colchicine ) , suggest gout.6 according to them , it also excludes the need for a biopsy . the actual case report corroborates bhattacharyya et al5 and kleinman and ewbank,6 as it was conducted after a diligent clinical appraisal . the ultrasonography presented a well - defined swelling beneath the skin and a left condyle distortion , suggesting gout manifestation in the tmj . in the present case report , the sum of anamnesis and the physical and complementary exams pointed to the diagnosis of gout , revealing the sovereign value of the clinical approach . gout in the tmj , despite being rare , should be included as a differential diagnosis for joint disorders . a previous history of gout with improvement after the use of nsaids may favor the diagnosis of gout , leading to the right treatment . the complementary exams can be helpful pinpoint tmj cases of gout and discard some other diseases that normally affect the region .
introduction gout is an illness characterized by the deposition of monosodium urate crystals in the joints or in soft tissues . the clinical manifestation results from inflammation of limb joints and pain with a rare presentation in the temporomandibular joint ( tmj ) . case report this study describes a 66-year - old white man with a chief complaint of occasional pain in the left temporal muscle region . the case disclosed a gout manifestation in the tmj after physical , radiographic , and ultrasonographic exams , and the patient was referred to proper treatment . conclusion gout manifestation in the tmj is an unusual presentation , and few reports in the english literature address to the subject . gout in the tmj should be included as a differential diagnosis for joint disorders .
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after 26 years since the first case of hiv was detected , the rapid increase in the number of women infected with hiv worldwide forecasts an inevitable greater number of women who will develop and die of acqiured immunodeficiency syndrome ( aids ) . currently , almost half of all new hiv infections are being reported in women , of these less than 50% are aware of their infection and 84% are in reproductive age group , i.e. , 15 to 45 years . rtis / stis most commonly affect this age group . both ulcerative ( syphilis , chancroid+herpes ) and nonulcerative infections ( trichomoniasis , chlamydia and gonorrhea ) , and other nonsexually transmitted rtis that involve inflammation of the reproductive tract especially in women there are very few studies done in india to compare the rtis and gynecological morbidities in hiv seropositive and hiv seronegative women . we have carried out this study to mainly highlight the importance of a detailed gynecological examination and cervical cytology in hiv positive women . this study was conducted between may 2002 and april 2004 in the department of skin and vd , baroda medical college . a total of 83 hiv seropositive women in the age range of 15 to 35 years ( tested by 2 elisa ) were enrolled in the study . a matched control ( more or less matched for age and socioeconomic status ) of 87 hiv seronegative women attending the outpatient department were also enrolled . smear examination ( gram staining , koh smear , and wet mount ) was also carried out . all patients were subjected to pap smear ( cervical cytology ) and if suspicious , colposcopy was done by the gynecologist . the mean age for hiv positive women was 30 years and that for hiv negative women was 27 years . the major mode of transmission in hiv positive females was sexual transmission ( 70% ) . these mainly included amenorrhoea ( 12% ) , oligomenorrhoea ( 6% ) , intermenstrual bleeding ( 4% ) , and menorrhagia ( 6% ) , in the seropositive group . stis / rtis as shown in table 2 were seen in 57% of the hiv positive females compared to 35% hiv negative females . table 3 shows in detail the sti / rti profile in hiv positive and hiv negative females . an abnormal pap smear was significantly higher in the seropositive group compared to the seronegative group [ table 4 ] . menstrual irregularities sexually transmitted infections / reproductive tract infection reproductive tract infection / sexually transmitted infections profile rtis including stis and hiv / aids are being increasingly recognized as a serious public health problem . rtis are suffered by both men and women , but their consequences are far more devastating and wide spread among women . among women , it is the burden of asymptomatic disease that is responsible for the frequent and severe or long - term morbidity and in part for the persistence and spread of stis in the community . hiv infection and other stis ( both ulcerative and nonulcerative ) increase the transmission of hiv by approximately three to five fold . vaginal candidiasis may occur earlier than other conditions and may serve as an early marker of hiv infection in women in our series , as high as 34% hiv positive females had candidiasis . herpes was seen in 11% of the hiv positive females compared to 2% in hiv negative females . shedding is also high in hiv infected women hence increasing the chances of transmission and acceleration of hiv disease . hsv-2 may be associated with the most extensive ulcerative disease in hiv infected women as seen in our patient . viral warts were seen in 5% of the hiv positive females compared to none in the hiv negative group . a study done by palefsky et al . , showed evidence of hpv infection in 58% of hiv positives vs. 26% of controls . they assessed hpv within cervical vaginal lavage fluids , employing polymerase chain reaction ( pcr ) . data from wihs study demonstrated 40% prevalence of abnormal pap smear in hiv infected women vs.17% in hiv negative control . studies have implicated microbes in inflammatory smears , especially chlamydia , hsv , and trichomonas vaginalis . all patients with dysplasia were subjected to colposcopy , out of which two were diagnosed with carcinoma in situ . in 1993 , the united state centers for disease control and prevention ( cdc ) added cervical cancer to the list of aids defining conditions . vulvo - vaginal candidiasis , cervical dysplasia , cervical cancer in situ , and pid were also added to the revised classification system of hiv infection under category b. because of their inherent biological vulnerability for rti / sti , all hiv positive females must be screened periodically for evidence of genital tract involvement , so that they can be promptly and effectively treated . moreover , impact of silent stis on pregnancy can also be prevented on screening . in a resource restricted set - up , hiv positive females can be helped by basic investigations including a pap smear . there is a growing need for hiv clinicians to not only provide basic but also gender specific care to the hiv infected women as they are afflicted by the same hiv manifestation as in male in addition to female specific manifestations .
background : human immunodeficiency virus ( hiv ) infection primarily affects women during their reproductive years , and the coexistence of reproductive tract infections ( rtis ) is not surprising given the fact that hiv is mainly acquired via heterosexual contact.aim:the aim of the study was to compare the occurrence of rtis among infected and noninfected women.materials and methods : a case control study of 83 hiv positive women , tested by two enzyme linked immunosorbent assay ( elisa ) and a matched control of 87 hiv negative women were screened for rtis . they were submitted to gynecological examination and cervical cytology.results:the mean age for hiv positive women was 30 years and that for hiv negative women was 27 years . 18% hiv positive women had menstrual irregularities compared to 6% in seronegative group ( p= 0.024 ) . vaginal infections including sexually transmitted infections ( stis ) were found in 47 ( 57% ) hiv positive women and 30 ( 34% ) hiv negative women ( p= 0.0037 ) . vaginal candidiasis was the most common infection ( 34% ) in hiv positive women , followed by trichomoniasis ( 12% ) . human papilloma virus ( hpv ) infection was seen in nine hiv positive women versus none in hiv negative women . cervical cytology showed inflammation in 53 ( 64% ) hiv positive women compared to 27 ( 31% ) hiv negative women ( p= 0.000023 ) . genital neoplasia , including carcinoma in situ was observed in 2 ( 2.5% ) hiv positive women and in none of the hiv negative women.conclusion:it is seen that reproductive tract morbidities are common in hiv positive women . so it is imperative that hiv positive women have a complete gynecological evaluation including a papanicolaou ( pap ) smear with aggressive screening of stis .
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a 69-year - old woman presented with a 4-month history of diplopia , 2 months of conjuntival injection , and exophthalmos . neurological examination showed normal consciousness and no cranial nerve deficits were observed . magnetic resonance imaging ( mri ) showed bulging of left cavernous sinus with internal signal void and distension of left superior ophthalmic vein . an angiogram showed left csdavf with arterial supplies from the middle meningeal artery and sphenopalatine artery arising both external carotid arteries and meningohypophyseal trunk arising both internal carotid arteries ( icas ) . there was venous drainage from the anterior cavernous sinus into the left superior ophthalmic vein ( fig . however , angiogram did not show both the inferior petrosal sinus and facial vein , which are the typical access routes for transvenous embolization . we accessed the right common femoral artery , and a 5 fr catheter ( bern ; boston scientific , natick , ma , u.s.a . ) was placed in the left external carotid artery . afterwards , the left eye region was prepped in sterile fashion by plastic surgeon and exposed the left superior ophthalmic vein through incision ( fig . 2 ) . after directly puncturing the superior ophthalmic vein with a 22 g needle , a 4 fr yellow sheath from the micropuncture set ( fig . was placed in the superior ophthalmic vein . after connecting 4 fr yellow sheath and 3-way , it was considered that both detachable and undetachable coil embolization would be needed simultaneously and a sufficiently wide microcatheter ( prowler 18 ; cordis , miami lakes , fl , u.s.a . ) and microguidewire ( synchro 14 ; boston scientific , natick , ma , u.s.a . ) were used to approach the cavernous sinus . the microcatheter was placed into cavernous sinus and a total of 10 detachable coils ( gdc 10 ; boston scientific , natick , ma , u.s.a . ) and indetachable coils ( tornado coils ; cook , bloomington , u.s.a . ) were used to perform coil embolization ( fig . after embolization , the patient 's diplopia and exophthalmos disappeared and there were no signs of cranial nerve palsy or additional neurological deficit . carotid - cavernous sinus fistulas ( ccfs ) can be classified into two main type . direct ccfs consist of a communication between the main trunk of the ica and venous channels within the cavernous sinus . csdavf is communications between the cavernous sinus and extradural branches of internal carotid artery , external carotid artery , or both . the direct ccfs are almost caused by severe trauma or rupture of an intracavernous carotid aneurysm , and are usually characterized hemodynamically by high flow . csdavf usually become symptomatic spontaneously and may be characterized hemodynamically by high or , more often , low flow . compared to other type davf , csdavf is known to have favorable prognosis and spontaneous regression rate of 10 - 73% in the literature . consequently , an initial conservative treatment such as manual compression of ipsilateral common carotid artery can be attempted . however , in cases of rapid deterioration of eye symptoms or cortical venous drainage occurrence , urgent interventional therapy is warranted . endovascular embolization can be done transarterial or transvenous approach . because transarterial embolization is difficult to embolize the entire supplying artery and frequent relapse , it is currently used as a main method to transvenous embolization . transfemoral venous approach reaches the cavernous sinus through inferior petrosal sinus , and is the most preferred method due to its anatomically short and straight course . however , if it is inaccessible due to thrombus in the inferior petrosal sinus , treatment can be attempted through the facial vein - superior ophthalmic vein route . if the facial vein is also inaccessible , direct superior ophthalmic vein approach can be tried instead . in this case , the bilateral inferior petrosal sinus and facial vein were not opacification on angiogram , and therefore transvenous embolization through direct superior ophthalmic vein approach was performed . direct superior ophthalmic vein approach has an anatomically short distance to the cavernous sinus , making it easily accessible . in this case , a 4 fr yellow sheath was placed in the superior ophthalmic vein and after connecting 4 fr yellow sheath and 3-way , continuous saline flushing was performed through 3-way . this procedures are allowed for a stable procedure and easily adjustable control of the microcatheter and microguidewire . we used a sufficiently wide microcatheter ( prowler 18 ; cordis , miami lakes , fl , u.s.a . ) compared previous study . however , this method is reported to cause in rare instances , bleeding and infection of the access area , damage to the trochlea area and orbital sulcus , nerve damage , dural complications such as laceration and penetration , diplopia , and prolonged ocular motor dysfunction . in this case , there was no complication during or post procedure and showed completed occlusion of the csdavf . in conclusion , transvenous coil embolization through direct superior ophthalmic vein approach can be good in treating csdavf cases where accessing the inferior petrosal sinus or facial vein are difficult or impossible .
transvenous coil embolization has been successfully applied for the treatment of cavernous sinus dural arteriovenous fistula ( csdavf ) . unfortunately , the technique can not be applied in cases of poor or absent inferior petrosal sinus or facial venous access route to the fistula . recently , we experienced a successful embolization using direct superior ophthalmic vein approach in cases of csdavf which were no opacification of inferior petrosal sinus or facial vein .
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the patient was seen in 1996 at the age of 32 , with blurred vision in the right eye and headache . there was mild epithelial edema of the cornea with a few keratic precipitates and no cells seen on slit - lamp examination of the right eye . he had five episodes of pss crises over the next 14 years , with a highest iop of 60 mmhg . anterior segment inflammation and elevated iop were controlled after few weeks of treatment with antiglaucoma medication and topical corticosteroids . this was his treatment course after each pss event . at his first medical examination in 1996 , his cup / disc ratio was noted to be 0.4 in the right eye and 0.4 in the left eye . 1a and 1b ) . after his fifth attack , in 2010 , his uncorrected visual acuity was 1.0 in both eyes and a relative afferent papillary defect of right eye was observed . his right optic disc was pale and the field loss in the right eye had progressed to a superior paracentral scotoma ( fig . there was p100 delay of the right eye in the visual evoked potential test ( fig . 4 ) . there were no abnormal findings on brain magnetic resonance image ( fig . pss is a syndrome associated with recurrent anterior segment inflammation and elevated iop . after an attack , iop and facility of aqueous outflow return to normal . the etiology of pss is unknown , although abnormal vascular process , autonomic defect , allergic conditions , variation of developmental glaucoma , association of hla - bw54 , cytomegalovirus , and herpes simplex virus have been suggested [ 6 - 8 ] . the presumed mechanism causing naion was decreased perfusion to the optic disc secondary to the rise in intraocular pressure . risk factors for naion such as old age , small cup to disc ratio , and hypertension were considered when treating pss patients . this is case of optic disc pallor in a patient with recurrent anterior segment inflammation and high intraocular pressure . optic disc perfusion is directly proportional to mean arterial pressure and inversely proportional to iop . we had not found something that explained optic disc pallor excluding high iop . decreased optic nerve perfusion by recurrent high iop might induce optic disc pallor . in conclusion , pss could cause optic disc changes such as glaucomatous change and ischemic change . thoughtful attention and aggressive iop control are required especially in pss patients with prolonged periods of high intraocular pressure and recurrent attacks .
a 32-year - old man with blurred vision in the right eye and headache presented with anterior uveitis , an intraocular pressure ( iop ) of 60 mmhg , an open angle , no visual field defects , and normal optic nerve . he had a history of five previous similar attacks . in each of the previous instances , his anterior uveitis and high iop were controlled with antiglaucoma medications and topical steroids . however , at the fifth attack , his optic disc was pale and a superior paracentral visual field defect was shown . brain magnetic resonance image studies were normal . this case represents that a recurrent posner - schlossman syndrome ( pss)-induced optic disc atrophy likely due to ocular ischemia caused by a recurrent , high iop . although pss is a self - limiting syndrome , we should manage high iop and prevent ischemia of the optic nerve head by treating with ocular antihypertensive medications .
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our work is supported by the state committee for scientific research , grant numbers 6 p04a 025 - 18 and 6 p04a 016 20 . the continuous line is the dna strand replicated as the leading strand ; the dashed line is the dna strand replicated as the lagging strand ; ori , the origin of replication ; ter , the terminus of replication . ori and ter divide the chromosome into two replichores , arbitrarily called left and right . plots of the relative positions of orthologs in the helicobacter pylori j99 and h. pylori 26695 genomes . the values on the x and y axes represent the positions of genes on chromosomes , in base pairs . ( a ) the closest orthologs ( best matches ) that have not switched their positions between the leading and the lagging dna strands . ( b ) all orthologs that have switched positions between the leading and the lagging dna strands . the genome sequences and orthologs , extracted from the database of clusters of orthologous groups ( ' cogs ' ) , were obtained from the national center for biotechnology information . note that if the sense strand is lying on the leading dna strand , the direction of transcription of the gene is the same as the direction of replication - fork movement . ( a ) a symmetrical inversion encompassing the origin of replication . after the inversion , the distances to the origin and the locations of the genes do not change with respect to the leading and lagging dna strands . ( b ) the inverted region encompasses the origin but the origin is not located in the center of this region . as a result , the lengths of replichores change and the distances of the noninverted genes to the origin change , although the locations of the genes do not change with respect to the leading and lagging dna strands . the locations of genes within the inverted sequence change with respect to the leading and lagging dna strands . furthermore , genes located away from the center of the inverted region change their distance from the origin .
a response to evidence for symmetric chromosomal inversions around the replication origin in bacteria by ja eisen , jf heidelberg , o white , sl salzberg . genome biology 2000 , 1:research0011.1 - 0011.9 .
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a self - expandable metal stent ( sems ) is an efficient and established tool for solution of biliary obstruction due to both benign and malignant diseases . meanwhile , there have been some reports regarding adverse events by a sems : migration , dislocation , ulceration , perforation of the bowel and so on.1 we sometimes encounter the case with kinking of the common bile duct ( cbd ) by a sems which is caused by inappropriate length or strong axial force of a sems and necessary for appropriate coping , for example , exchange of a sems or addition of another sems . a double - pigtail plastic stent is less costly than a sems and frequently used for decompression of the bile duct of patients with poor prognosis or undergoing heavy particle radiotherapy or proton therapy . furthermore , recently , a double - pigtail plastic stent is also feasible for endoscopic ultrasonography - guided2,3 or transpapillary gallbladder drainage.2 we present a rare case with ampullary carcinoma treated by a sems and additional stenting of a double - pigtail plastic stent for correction of kinking of the cbd . a 72-year - old man with jaundice was referred to our hospital . a contrast - enhanced computed tomography ( ct ) scan showed a 15-mm , weakly enhancing mass at the ampulla of vater ( fig . 1 ) and dilatation of the bile duct . biopsy of the mucosa indicated adenocarcinoma . for biliary decompression , a transpapillary , fully covered , self - expandable metal stent ( fcsems ) ( 1050 mm bonastent ; standard sci - tech , seoul , korea ) was deployed . because stent obstruction or migration was suspected , endoscopic carbon dioxide cholangiography was performed , which revealed kinking of the cbd 1 cm above the proximal end of the fcsems ( fig . a 7-f double - pigtail plastic stent ( 100 mm ; olympus medical systems , tokyo , japan ) was therefore placed through the fcsems to correct the kink , straightening the cbd ( fig . this is the first report of a unique use of a double - pigtail plastic stent to correct cbd kinking . it is reported that placement of a metal stent across the main duodenal papilla can predispose to cholangitis4 which is mainly caused by food impaction in a stent or reflux of duodenal contents to the bile duct . in this case , however , acute cholangitis was cured after the correction of cbd kinking , which indicates that cholangitis was caused by cbd kinking , not by placing a stent across the ampulla of vater . cbd kinking can occur by inappropriate placement of a sems or a large - bore diameter plastic stent5 with strong axial force . nakai et al.6 recommends a new method of sems stenting to reduce early stent - related complications including kinking of the bile duct by longer stent placement with the center of the stent located in the center of the biliary stricture . however , the new method was impossible in this case , because the site of the biliary stricture was located at the ampulla . there has been one report by park et al.7 in which a double - pigtail plastic stent with a fcsems was used for anchoring . they focused on the pigtail shape and revealed that it could help to prevent fcsems migration . meanwhile , we expected the correction of cbd kinking from the stent shaft in addition to anchoring . a use of a double - pigtail plastic stent can correct cbd kinking without exchange or addition of another sems and can save cost .
a 72-year - old man with jaundice by ampullary adenocarcinoma was treated at our hospital . for biliary decompression , a transpapillary , fully covered , self - expandable metal stent ( fcsems ) was deployed . four days later , the patient developed acute cholangitis . endoscopic carbon dioxide cholangiography revealed kinking of the common bile duct above the proximal end of the fcsems . a 7-f double - pigtail plastic stent was therefore placed through the fcsems to correct the kink , straightening the common bile duct ( cbd ) and improving cholangitis . this is the first report of a unique use of a double - pigtail plastic stent to correct cbd kinking . the placement of a double - pigtail plastic stent can correct cbd kinking , without requiring replacement or addition of a fcsems , and can lead to cost savings .
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in the previous issue of critical care , lorente and coworkers reported the results of a prospective cohort study evaluating matrix metalloproteinases ( mmps ) and tissue inhibitors of metalloproteinases ( timps ) as potential biomarkers for the determination of sepsis severity and for the prediction of mortality in septic patients . the authors found that nonsurviving septic patients presented with lower mmp-9 levels , higher timp-1 levels and a lower mmp-9/timp-1 ratio . moreover , they showed that timp-1 levels can predict the clinical outcome of septic patients and could be useful for risk stratification of patients with sepsis . mmps have been shown to be important in the pathogenesis and development of inflammatory diseases and are intimately involved in the regulation of the activities of cytokines and cytokine receptors . pathophysiologically , a successful eradication of infection by the host requires the influx of effector cells into the infected tissue , killing of the pathogen , resolution of inflammation and , finally , remodeling of the extracellular matrix . excessive inflammation following infection may cause tissue damage , however , and mmps are implicated in causing this immunopathology . first of all , human neutrophils secrete gelatinase b ( mmp-9 ) in vivo and in vitro in response to endotoxin and proinflammatory mediators such as tnf or il-8 . secondly , timp-1 can activate normal human granulocytes , protecting them from apoptosis and blocking their transmigration during inflammation . elevated serum levels of mmps have been described in some studies in response to endotoxin and proinflammatory mediators , and mmps can be considered as markers of inflammation in various diseases . studies that investigate mmps and their inhibitors in septic diseases are rare and have involved only limited numbers of patients . mmp-9 levels have been shown to be elevated in patients with severe sepsis compared with healthy control individuals . in a small study of 20 patients with septic shock , nakamura and colleagues found elevated mmp-9 levels in nonsurvivors of severe sepsis as compared with survivors and healthy controls . unfortunately , lorente and coworkers can not confirm these results in their multicenter study ; on the contrary , these authors show lower mmp-9 and a reduced mmp-9/timp-1 ratio in nonsurviving septic patients . in accordance with a previous study , moreover , timp-1 levels were shown to have prognostic implications in severe septic patients as has been described before . lorente and coworkers define an elevated risk of death in septic patients with a cut - off value > 531 ng / ml for timp-1 according to their receiver operating curve analysis . this result is quite different to another study in septic patients , which evaluated a cut - off value > 3,200 ng / ml for timp . importantly , lorente and colleagues could show an association of mmp-9 , mmp-10 and timp-1 with the severity of sepsis and a correlation of these parameters with markers of inflammation on the time of diagnosis of sepsis , which was not demonstrated in a previous study in septic patients . a limitation of the study by lorente and colleagues , however , is the lack of serial measurements of mmps and timps over several days , which could help to confirm the association between these markers with the severity of sepsis as assessed by the sequential organ failure assessment score and acute physiology and chronic health evaluation score . recent data and cumulative analysis indicate that biomarkers improve diagnosis of sepsis and may help to predict the prognosis of septic patients . in the scene of sepsis biomarkers , c - reactive protein , il-6 and procalcitonin are the most investigated markers in clinical trials . in recent published studies , procalcitonin is of better value for diagnosis and prognosis of sepsis when compared with markers such as c - reactive protein or with proinflammatory cytokines such as il-6 [ 11 - 13 ] . there are additional new sepsis markers with so far limited clinical evidence , for example triggering receptor on myeloid cells or n - terminal pro - brain natriuretic peptide . unfortunately , bio - markers rarely alter our clinical decision - making in severe sepsis as they are often nonspecific , lack adequate sensitivity and/or are difficult to measure and to interpret accurately . metalloproteinases and their inhibitors may represent a promising new class of biomarkers for the prognosis of severe sepsis patients . the development of specific inhibitors of mmps or timps as a new class of drugs for sepsis therapy is challenging , and future clinical trials have to clarify their role within the treatment regime of septic patients . the use of biomarkers may improve early diagnosis and therapy in critically ill patients , and may consecutively influence the morbidity and mortality of these patients . the implementation of metalloproteinases and their inhibitors as new biomarkers for the severity of sepsis and for mortality in critically ill patients may provide promising decision support for the intensivist to guide the allocation of hospital resources . additional larger studies are needed , however , to determine the cellular origin and the relevance of these enzymes in sepsis . il : interleukin ; mmp : matrix metalloproteinase ; timp : tissue inhibitor of matrix metalloproteinase ; tnf : tumor necrosis factor . the present work was supported by a grant of the faculty of medicine mannheim , university of heidelberg , germany .
the multicenter study conducted by lorente and coworkers published in the previous issue of critical care demonstrates that matrix metalloproteinase ( mmp)-9 and mmp-10 and their inhibitor tissue inhibitor of matrix metalloproteinase-1 ( timp-1 ) are promising novel biomarkers to predict severity and outcome of sepsis . in recent years mmps have emerged as biomarkers in a variety of diseases , such as sepsis , coronary artery disease , cancer , heart failure , chronic lung disease and rheumatoid arthritis . mmps constitute a family of proteinases that are expressed during developmental , physiological , and pathophysiological processes , for example as a response to infection . excessive inflammation following infection may cause tissue damage , and mmps are implicated in causing this immunopathology . the activity of mmps is regulated by secretion of specific inhibitors ( timps ) . studies using mmp inhibitors and mmp knockout mice indicate that mmps play an essential role in infection and in the host response to infection . the measurement of mmp-9 and mmp-10 and their inhibitor timp-1 in the intensive care setting could be an attractive noninvasive tool for determination of outcome of septic patients .
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prone positioning is widely used to improve oxygenation of patients with acute respiratory distress syndrome ( ards ) . in the previous issue , robak and colleagues report the short - term impact of combining two positioning strategies , prone and upright positioning . although randomized controlled trials failed to demonstrate an overall mortality benefit with the prone position , a trend toward better survival was observed for patients with severe ards [ 2 - 5 ] . the use of the prone position is supported by a strong physiological rationale and a significant improvement of the oxygenation status is observed in roughly 70% of ards patients . several mechanisms have been advocated to explain this effect , but the most determinant probably involves better aeration and recruitment of the vertebra - diaphragmatic lung regions , as a consequence of the reduction in ventral chest wall ( cw ) compliance due to direct compression on the bed . interestingly , either high baseline cw compliance or a greater reduction in its magnitude with the prone position were associated with oxygenation improvement . upright positioning has also been associated with oxygenation improvement in ards patients and was proposed as an alternative to the prone position . we previously demonstrated , in a group of 16 ards patients submitted to upright positioning , a significant increase in the pao2/fio2 ratio ( 94 33 versus 142 49 mmhg , p < 0.0032 ) in comparison to the baseline value obtained with the supine position . with respect to the responders , the significant increase in the measured end - expiratory lung volume , coupled with the change in chord compliance after switching to upright positioning , suggested a time - dependent lung recruitment phenomenon . based on physiological studies , one can postulate that the oxygenation improvement is mainly due to a lung volume redistribution induced by reduced ventral cw compliance during prone positioning , while an increase of the lung volume ( recruitment ) appears more significant during upright positioning . because of their complementary mechanisms of action , combining prone and upright positioning can make sense in severe ards patients . likewise , it has already been shown that the combination of prone positioning with an adjunctive technique to increase lung volume , the recruitment maneuvers , has additive positive effects on oxygenation . in the physiological study of roback and colleagues , the upright - prone position was achieved by raising the head of the bed and lowering the foot end to obtain an angle of at least 20 ( reverse trendelenburg ) . this was associated with a significant improvement in the pao2/fio2 ratio , without any short - term adverse events . the proportion of responders increased by combining the prone with the upright position , which is particularly relevant since the prone position is usually discontinued in the absence of oxygenation improvement . this study was the first to specifically investigate the effect on gas exchange of combining prone and upright positioning . unfortunately , the study design precluded conclusions about the precise physiological mechanisms involved . measurements of the lung volumes would have been useful to better document the alteration of respiratory mechanics related to both prone and upright positioning . regarding the redistribution of aerated lung regions , directly assessing the regional changes could have added to our current understanding . since computed tomography ( ct ) scanning can not be done during upright positioning , alternative techniques , such as electrical impedance tomography , may have permitted a better delineation of the importance of ventilation redistribution from non - dependent toward dependent parts of the lung with the upright - prone position . beyond the impact on oxygenation , other beneficial effects although the level of evidence is weak , both prone and upright positions have been associated with a decrease of ventilator - associated pneumonia . an additive effect of these two strategies is plausible , but the pathophysiology of ventilator - associated pneumonia is complex and one should be prudent before making such an assumption . furthermore , head elevation of patients ventilated in the prone position was shown to improve the tolerance of enteral feeding . in summary , the use of prone and upright positioning should not be considered as opposing and mutually exclusive strategies , but more as complementary ones . even if the level of evidence is debatable , combining prone and upright positioning seems to enhance the response rate in terms of oxygenation . whenever the prone position is used , combining it with the upright position this strategy is easy to implement at no additional cost and with a low risk of major complications . specific policies should be implemented to encourage caregivers to avoid as much as possible a strictly supine position . future studies should aim at determining to what extent the addition of the upright position during , and perhaps also between , prone sessions can promote the maintenance of beneficial effects .
positional strategies have been proposed for mechanically ventilated patients with acute respiratory distress syndrome . despite different physiological mechanisms involved , oxygenation improvement has been demonstrated with both prone and upright positions . in the previous issue of critical care , robak and colleagues reported the first study evaluating the short - term effects of combining prone and upright positioning . the combined positioning enhanced the response rate in terms of oxygenation . other benefits , such as a reduction in ventilator - associated pneumonia and better enteral feeding tolerance , can potentially be expected .
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lauralyn mcintyre and colleagues have neatly used a septic shock scenario - based survey to highlight considerable variations within canadian critical care practice . they acknowledge the potential pitfalls of translating survey results into ' real life ' ; however , my own experience of the diversities within uk practice suggest this would be representative of at least one other industrialized country , albeit with some variation in the detail ( for example , use of gelatin as a plasma expander is much commoner in europe ) . they found decisions regarding treatment strategy ( choice of fluid , use of inotropes and transfusion triggers ) to be highly variable . however , they did demonstrate consistency in a continuing reliance on ' basic ' monitoring ( blood pressure , heart rate , central venous pressure , urine output , pulse oximetry ) . this was to the relative exclusion of other , more sophisticated techniques ( cardiac output , central venous saturation ) whose use has been linked with outcome improvements in specific situations , such as the scenario on which their survey was based . is this heterogeneity a triumph of uncertainty and/or natural conservatism and/or arrogance and/or sloth over heavily promoted , multiple society - endorsed guidelines based primarily on the important yet limited rivers study ? why are n't we all practising homogenized medicine ? what does it take to standardize our approach to care of the critically ill ? the institute of healthcare improvement 's surviving sepsis campaign website boldly states that a bundle a group of interventions related to a disease process " when executed together will result in better outcomes than when implemented individually " and that " the science behind the elements of a bundle is so well - established ( my italics ) that their implementation should be considered a generally accepted practice . " yet three of the major planks upon which the two sepsis bundles are based , namely the use of corticosteroids , activated protein c and early goal - directed therapy are currently being questioned via , respectively , the corticus study findings , the european agency for the evaluation of medicinal products ( emea ) , and the national institutes of health ( through their recent $ 8.4 m funding of the process study ) . these new challenges will , i believe , serve to increase uncertainty still further in the short - term and , thus , affect participation in an approach that is worthy but , in my opinion , critically flawed through a lack of prospective validation . medics are a naturally conservative bunch the avid uptake of new technologies by a rapid responder minority is rarely translated into standard practice , often because the initial enthusiasm for a drug , device or strategy fails to pass muster when more rigorously scrutinised or trialled . it was not that many years ago that we were being exhorted to use high doses of dobutamine to achieve ' supranormal ' cardiorespiratory goals in the critically ill , as an extrapolation of findings from a high - risk surgical patient cohort . when subsequent randomised trials made it painfully clear that the intensive care unit ( icu ) patient outcomes did not match up to expectation , the concept was generally discarded , even from the surgical patient population in whom the benefit was repeatedly seen . i 've yet to meet a self - confessed mediocre intensivist so we all need to take a critical and regular look at our own individual performance . we do require a healthy degree of self - confidence to support our decision - making ability , but are we ready to accept that our icu is perhaps offering an inferior level of care to the hospital down the road ? or if we do acknowledge poor performance , is this from someone / everyone / anyone else but me ? anonymised audit should , in a non - threatening manner , facilitate recognition and , hopefully , correction of our shortcomings . the dutch offer external peer review ' visitations ' that can be initiated either by the icu or their hospital administration . how widespread is this practice ? finally , it is also a deficiency of ourselves as a community that we still can not answer many fundamental questions . for an individual patient , what constitutes optimal targets , for example , for blood pressure and tissue perfusion , or ' best ' treatment , such as the optimal duration of a course of antibiotics ? altruistic , multi - centre , generalisable research addressing simple questions is evolving . the canadians and australasians have clearly led the way while european and other countries are catching up . perhaps these studies could ( should ) be better coordinated to complement each other . perhaps this spirit of cooperation could ( should ) also be extended to open access , anonymised patient databases as a means of comparing models of care and for future hypothesis generation .
why is the practice of intensive care so heterogenous ? uncertainty as to ' best practice ' , conservatism , and complacency may all contribute to our divergent management strategies . the need for further generalisable research , anonymised audit , external peer review and open access databases is discussed .
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sequencing the mrna in a cell has been used as a high - throughput method for finding genes since the early days of the human genome project . beginning in the early 1990s , the expressed sequence tag ( est ) method was used to capture fragments of thousands of human genes prior to the sequencing of the genome . est sequencing relies on the fact that eukaryotic genes are polyadenylated after transcription , and the long poly - a tract can be used to capture the transcripts via reverse transcription pcr ( rt - pcr ) . the est method was subsequently applied to many other species , and est databases ( notably dbest ) became a vital resource for genome annotation . recently , a next - gen version of est sequencing has emerged , allowing researchers to capture and sequence mrna at dramatically lower cost , and higher volume , than was ever possible with the est method . the new rna - seq methods [ 2 - 5 ] are being applied to a rapidly growing variety of species , cell types , and scientific questions , revealing far more about the transcriptomes of these species than was known just a few years ago . the field is advancing so rapidly that a brief review can not cover the work of the past 2 years ; this review is just a sampling of a few highlights . sultan et al . analyzed approximately 8 million short reads and found that rna - seq could detect 25% more genes as compared to microarrays . about one - third of transcripts in their experiments mapped to genomic regions not annotated as genes . of the 94,241 splice junctions , this result has been amplified by subsequent studies that generated even more sequences and showed even larger numbers of novel splicing events . . generated approximately 430 million paired - end reads to recover 13,692 known isoforms from mouse myoblast cells , but also detected 12,712 novel isoforms , of which 7395 contained novel splice junctions while the rest represented novel combinations of known exons . this latter study also demonstrated the power of a new algorithm capable of detecting and quantifying alternative isoforms when aligning rna - seq reads to a genome . in an rna - seq study using liver rna samples from humans , chimpanzees , and rhesus macaques , blekhman et al . found that alternative splicing events vary between closely related primates and also between the sexes within species . wang et al . generated approximately 600 million short reads from 15 cell types and found that 92 - 94% of human genes are alternatively spliced , and that many alternative splicing events are tissue - specific . rna - seq is also being used to study genetic variation among individuals ( expression quantitative trait loci , or eqtls ) . combined rna - seq data and hapmap data from 69 nigerian individuals and 63 caucasian individuals , respectively , and both groups identified variants responsible for alternative splicing as well as variation in expression levels among individuals . in single - celled organisms , rna - seq can reveal novel insights about polycistronic transcripts . in the first transcriptome analysis of trypanosoma brucei , thousands of splicing and polyadenylation sites were identified and many genes were found to be differentially expressed between the parasite 's two life - cycle stages . in prokaryotes , rna - seq can provide an extremely detailed transcription map , at the single - base level , as has been shown recently in an archaeal species , sulfolobus solfataricus , and in a pathogen bacterium , helicobacter pylori . in s. solfataricus , over 1000 transcriptional start sites were detected and 80 novel protein - coding genes were discovered . in h. pylori , hundreds of transcriptional start sites within operons were found , as well as approximately 60 novel small rna genes . the power of rna - seq stems from its ability to generate deep coverage of the entire transcriptome of a cell with just a single run of a high - throughput sequencer , such as the illumina hiseq , which can produce up to 200 billion bases in a single run . the potential to characterize all genes , to capture alternative isoforms , and to measure differential expression has already been demonstrated in dozens of studies , but hundreds of species , and countless experimental conditions , are yet to be explored . several groups have developed methods besides poly - a selection to capture all rnas in a cell , for example , random hexamer priming , which allows them to analyze prokaryotic transcriptomes or to look at noncoding rna in eukaryotes . it now appears that rna - seq will replace microarray technology in the coming years , as it appears to be not only more comprehensive but also much more accurate than microarrays , particularly for transcripts with low expression levels . as this new method becomes even more widely adopted , it should greatly expand our understanding of the complex interplay of genes in all phases of cell development .
the latest high - throughput dna sequencing technology can now be applied on a large scale to capture the complete set of mrna transcripts in a cell , using a technique called rna - seq . although rna - seq is only 2 years old , it has rapidly swept through the field of genomics , and it is now being used to analyze the transcriptomes of organisms ranging from bacteria to primates . the depth of sequencing allows researchers to quantify the level of expression of genes , to discover alternative isoforms in eukaryotic species , and even to characterize the operon structure of bacterial genomes .
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chordoma is a low - grade malignant tumor that arises from the remnants of notochord . they account for 1 - 4% of malignant bone tumors and most commonly present after the age of 30 years . majority occur in sacrum or in the clivus ( a bony surface in the posterior cranial fossa sloping upward from foramen magnum to dorsum sella ) . those located in the clivus are often associated with a chronic headache and symptoms due to compression of a cranial nerve . lateral extension can lead to a cerebellopontine angle tumor symptomatology . in case of inferior spread , nasal obstruction , bleeding , and the cytological diagnosis is possible in representative location when physaliferous cells are present and radiological findings are available . but in metastatic sites they are confused with a variety of epithelial mesenchymal tumors , especially when information about the diagnosis of primary tumor is not available . we present cytological findings of a case of metastatic nasopharyngeal chordoma in upper cervical lymph node . a 30-year - old female presented with mild headache , sore throat , gradual onset of difficulty in swallowing and speech for the last 2 years . on examination , a growth was seen behind the uvula obstructing the view of oropharynx with normal overlying mucosa . a level two right cervical lymph node ( in the parotid region ) was also present . routine hematological investigations and chest radiograph was within normal limits . computed tomography scan of the neck revealed a well - defined oval - shaped mass 67 22 mm in superior nasopharynx slightly to the right side causing pressure erosion of body and lesser wing of sphenoid bone . . magnetic resonance imaging scan of the upper cervical region revealed a well - defined lobulated space occupying lesion at the skull base near foramen magnum having extension anteriorly along the prevertebral soft tissue having hypointensity in t1 and hyperintensity in t2 and short t1 inversion recovery ( stir ) sequence . the lesion also extended intracranially along the anterior aspect of the brain stem and to extra - axial space at c1 and c2 levels involving right lateral recess . microscopic examination of the smears showed a cellular tumor composed of aggregates and dispersed single cells embedded in abundant chondromyxoid ground substance . the cells were round to oval with bland nuclear chromatin , smooth nuclear membrane , and inconspicuous nucleoli . cytological picture of chordoma showing aggregates and dispersed single cells embedded in abundant chondromyxoid ground substance which appears deep magenta . multivacuolated cells are well appreciated ( mgg , 100 ) a biopsy from the nasopharyngeal mass showed a lobular growth separated by connective tissue septa . the cells were round to oval with vacuolated to eosinophilic cytoplasm and embedded in abundant myxoid matrix . immunohistochemistry was performed that showed positivity with cytokeratin ( ck ) [ figure 2 inset ] , vimentin and s-100 , thus confirming the diagnosis of chordoma . as surgery was not possible owing to the location of the tumor , the patient underwent radiation therapy . chordomas arise from embryonic remnants of notochord and show a dual epithelial - mesenchymal differentiation . in the embryo at 11 mm , the notochord obliterates and is displaced to the cranial and caudal positions . microscopic foci remain in the vertebral bodies at the cranial and caudal ends of the embryo . malignant transformation typically occurs in the third to fourth decades of life for spheno - occipital lesions and in the fifth to sixth decades for the sacrococcygeal type . they arise from the sacrum in approximately 50 - 60% of cases and from the skull base region ( spheno - occipital / nasal ) in approximately 25 - 35% of cases . chordomas are slow - growing tumors , can invade locally , but rarely metastasize . in advanced disease , metastases to lung , bone , soft tissue , lymph node , and skin occur . the location , which usually is off the midline , and the immunohistochemical features can help distinguish between the two lesions . immunohistochemically , the cells are positive for s-100 and negative for ck and epithelial membrane antigen ( ema ) . clinically , these lesions arise in the extremities ( mostly in soft tissue ) and rarely involve the axial skeleton . however , metastatic adenocarcinoma usually lacks typical physaliferous cells , and the extracellular mucin is of the neutral epithelial type , compared with the hyaluronidase - resistant sulfated mucopolysaccharide stroma of chordoma . both tumors are s-100 positive , but myxoid liposarcoma lacks epithelial markers ema and ck . chordoma can also be misdiagnosed as pleomorphic adenoma of salivary gland , as it shows bland epithelial cells and intense fibrillary metachromatic stroma . presence of multivacuolated physaliferous cells combined with radiological findings can be helpful in this regard . in a review of the fine - needle aspiration cytological features of a case of chordoma and of 17 consecutive cases of pleomorphic adenoma , it was found that the presence of a more abundant , focally vacuolated cytoplasm favors chordoma over pleomorphic adenoma . biopsies are obtained to confirm the diagnosis . the american joint committee for cancer staging system treatment options include radiation therapy or combined radiation and surgery , and surgical excision alone . due to local invasion , many tumors ( especially skull base chordoma ) often are not amenable to complete surgical excision , and the local recurrence rate is high . five - year and 10-year survival rates are approximately 50% and 25 - 30% , respectively . some data suggest that female sex , tumor necrosis , and tumor volume of more than 70 ml are independent poor prognostic variables in skull base chordomas .
spheno - occipital chordomas can rarely present as nasopharyngeal mass . metastases occur only in advanced disease . they can pose a diagnostic dilemma when information about diagnosis of the primary tumor is not available . we present cytological findings in upper cervical lymph node of a case of nasopharyngeal chordoma and discuss possible differential in such a location .
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aav2/8 was produced by transient transfection and purification from culture supernatant by peg precipitation and cesium chloride ultracentrifugation . virus was quantified by qpcr against cmv sequences and functionally validated in vitro to confirm gene expression prior to use in vivo . antibody concentration in the serum was determined using an elisa specific for either total human igg or human igg against hiv - gp120 . humanized mice expressing antibodies were produced by adoptive transfer of expanded hupbmcs into mice previously transduced with aav vectors . hiv challenge was carried out via ip or iv injection and blood was sampled weekly to determine the ratio of cd4 to cd8 cells by flow cytometry .
despite tremendous efforts , development of an effective vaccine against hiv has proved an elusive goal . recently , however , numerous antibodies have been identified that are capable of neutralizing the vast majority of circulating hiv strains15 . these antibodies all exhibit an unusually high level of somatic mutation6 , presumably due to extensive affinity maturation over the course of continuous exposure to an evolving antigen7 . while substantial effort has focused on the design of immunogens capable of eliciting antibodies de novo that would target similar epitopes810 , it remains uncertain whether a conventional vaccine will be able to elicit analogs of the existing broadly neutralizing antibodies . as an alternative to immunization , vector - mediated gene transfer could be used to engineer secretion of the existing broadly neutralizing antibodies into the circulation . here we describe a practical implementation of this approach , vectored immunoprophylaxis ( vip ) , which in mice induces lifelong expression of these monoclonal antibodies at high concentrations from a single intramuscular injection . this is achieved using a specialized adeno - associated virus ( aav ) vector optimized for the production of full - length antibody from muscle tissue . we show that humanized mice receiving vip appear to be fully protected from hiv infection even when challenged intravenously with very high doses of replication - competent virus . our results suggest that successful translation of this approach to humans may produce effective prophylaxis against hiv .
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erythema ab igne is a reticular , telangiectatic , pigmented dermatosis which occurs following prolonged and repeated exposure to infrared radiation that is insufficient to produce a burn . it is frequently encountered in cold climates where people use various devices for heating purposes . the use of laptop computers has increased manifold in india with the availability of these at affordable prices . laptop computers are often placed in contact with the skin during use , thus exposing the skin to prolonged heat . an extensive pubmed search did not reveal any report of laptop - induced erythema ab igne from the indian subcontinent , although seven cases have been reported in the english literature.[25 ] a 20-year - old male engineering student presented with asymptomatic pigmentation on one side of the abdomen which he had noticed 4 days earlier . there was no preceding history of skin lesions and no similar lesions elsewhere on the body . further enquiry revealed that the patient had the habit of placing the laptop computer on the bare skin of his abdomen , and he had been using the laptop for at least for 2 to 3 hours per day for the last 2 years . on examination , a diffuse reticular brownish hyperpigmentation was observed on the affected site [ figure 1 ] . based on the history and clinical findings a diagnosis of erythema ab igne secondary to laptop use was made . the patient was reassured and advised to avoid placing the laptop in contact with the body . erythema ab igne also termed as toasted skin , ephelis ignealis , erythema a calore , and ephelis ab igne is a reticular , telangiectatic , pigmented dermatosis caused by prolonged and repeated exposure to infrared radiation that is insufficient to produce a burn . it is commonly encountered in cold climates where people use various heating devices . in western countries , erythema ab igne was initially described as occurring on the shins of those who work or huddle close to a stove or open fire . but with the widespread use of centralized climate control in most buildings these days , the occurrence of erythema ab igne in this location decreased . the pattern of erythema ab igne depends on the direction of the radiation and the contour of the skin . prolonged and repeated exposures to the heat source result in reticulate erythema , hyperpigmentation , telangiectasia , scaling , and atrophy . erythema ab igne has been reported following the use of various heat sources , including hot pads and electric blankets , open fires , hot stoves , chulha , space heaters , infrared lamps , steam radiators , car heaters , wood - burning stoves , furniture with inbuilt heating unit , heating blankets , frequent bathing in hot water , and sauna belts . to date , only seven cases of laptop induced erythema ab igne have been reported.[35 ] cirrhosis , malnutrition , hypothyroidism , internal malignancy , hansen disease , and anemia are some of the medical conditions that have been associated with erythema ab igne . the histopathology of erythema ab igne is nonspecific and may appear normal on hematoxylin and eosin stain or may show mild acanthosis and hyperpigmentation of the basal keratinocytes . biopsy in the early stages may show epidermal atrophy , effacement of rete ridges and pigment incontinence . dilatation and congestion of postcapillary venules and a variable perivascular infiltrate of mild to moderate intensity , consisting of lymphocytes , plasma cells , histiocytes , melanophages , mast cells , and neutrophils may be seen in the dermis . rare instances of bullous erythema ab igne , cutaneous horn with thermal keratosis , squamous cell carcinoma and merkel cell carcinoma arising in erythema ab igne lesions have reported . the various treatment modalities suggested for erythema ab igne include topical retinoids , with or without topical steroids and 5-fluorouracil cream . biopsy for histopathology was not done in our patient as the findings in such cases are usually nonspecific . the information technology revolution and the availability of laptops at affordable prices has resulted in widespread use of laptops by the younger generation . a history of laptop use must be enquired for on diagnosing a case of erythema ab igne , especially when it occurs on the thighs and abdomen among the youth and those involved in the it field .
erythema ab igne is a reticular , pigmented dermatosis caused by prolonged and repeated exposure to infrared radiation that is insufficient to produce a burn . the use of laptop computers has increased manifold in the recent past . prolonged contact of the laptop with the skin can lead to the development of erythema ab igne . we present a case of erythema ab igne secondary to laptop use in an indian student .
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diabetic patients who have recurrence of their pedal ulceration following tal need to be reassessed . several studies have noted that the strongest predictor of complete healing of a diabetic ulceration is the 4-week percentage change in wound area ( 3 , 5 , 8 , 11 ) . persistent ulcerations after surgical intervention need to be assessed for multiplanar deformities , and if noted need to be surgically addressed . 3 ) the value of strict glucose control can never be underestimated and should not be an understated aspect of our day - to - day interactions with diabetic patients . foot and ankle surgeons should be advocating strict glucose control through direct communication with patient 's primary care physician or endocrinologist . in our experience , a patient with glycosylated hemoglobin of > 8% and an average daily glucose of > 250 mg / dl will experience prolonged wound healing . the practitioner should suspect previously undiagnosed or recurrent bone or soft tissue infection , vascular compromise , non - compliance , hypoalbuminemia , hyperglycemia , boney prominence , and recurrence of equinus contracture when dealing with recurrent diabetic pedal ulcerations . a pre - operative clinical picture ( a ) showing the left foot with a multiple plantar ulcerations after a failed tendo - achilles lengthening and ray amputation . post - operative picture ( b ) showing the external fixation device and soft tissue realignment procedures to address the recurrent ulcerations . this picture demonstrates an open , frontal plane , z - lengthening of the achilles tendon . the most distal cut , to the right of the picture , is directed anterior while the proximal cut , to the left , is directed posterior . the authors have not received any funding or benefits from industry to conduct this study .
foot and ankle surgeons are frequently challenged by the devastating systemic consequences of diabetes mellitus manifested through neuropathy , integumentary and joint breakdown , delayed healing , decreased ability to fight infection , and fragile tendon / ligaments . diabetic neuropathic pedal ulcerations lead to amputations at an alarming rate and also carry a high mortality rate . this article will discuss causes of diabetic pedal ulcerations that persist or recur after tendo - achilles lengthening and will highlight areas that need to be addressed by the practitioner such as infection , vascular and nutritional status , glucose control , off - loading , biomechanics , and patient compliance .
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surgery for pulmonary metastases from colorectal cancer ( crc ) is indicated for selected patients who meet conventional criteria as follows : ( 1 ) the metastases seem to be technically resectable ; ( 2 ) the general and functional risks are tolerable ; ( 3 ) the primary tumor is controlled ; ( 4 ) no active extrathoracic lesions are detected [ 1 , 2 ] . however , the optimal criteria to identify patients for whom pulmonary metastasectomy may provide a modest survival benefit remain controversial , although a number of studies have revealed potential prognostic factors such as serum carcinoembryonic antigen ( cea ) level [ 3 , 4 ] . circulating tumor cells ( ctcs ) are potential surrogates of distant metastasis , and their presence is a novel and promising biomarker in the diagnosis and therapy of a variety of malignant tumors [ 5 , 6 , 7 , 8 ] . the ctc test using the cellsearch system ( velidex , llc , north raritan , n.j . , usa ) , an automated quantitative ctc detection system , has been approved for monitoring of blood from metastatic crc patients by the food and drug administration in the usa . here , we present the case of a woman with solitary pulmonary metastasis from crc who died of tumor recurrence within 6 months after thoracotomy ; the presence of ctcs in the peripheral blood sampled before thoracotomy was the only factor to predict such a poor prognosis . a 43-year - old japanese woman with suspicion of a solitary pulmonary metastasis presented in september 2009 , after a series of therapies for colon cancer as follows : ( 1 ) descending hemicolectomy ( mucinous adenocarcinoma ; stage iiib : pse and pn2h0m0 ) in november 2007 ; ( 2 ) adjuvant chemotherapy for 4 months : uft ( uracil / tegafur)/lv ( leucovorin ) ; ( 3 ) hepatectomy for liver metastasis in january 2009 and radiotherapy for left 8th rib metastasis in february 2009 , and systemic chemotherapy for 4 months : folfox ( oxaliplatin/5-fluorouracil / leucovorin ) + bevacizumab . a pet - ct scan revealed a solitary pulmonary nodule in the right lower lobe with suspicion of pulmonary metastasis , and no other active tumors in july 2009 ( fig . serum cea level was normal ( 2.2 ng / ml ) , and pulmonary metastasectomy was performed with the establishment of pathological diagnosis of pulmonary metastasis . a 7.5-ml sample of peripheral blood taken just before thoracotomy was used for the ctc test , which revealed the presence of ctcs ( 6 ctcs/7.5 ml ) . the present case showed a very poor prognosis of only 6 months survival after pulmonary metastasectomy due to early development of nodal and bone metastases , although the case met the traditional selection criteria for pulmonary metastasectomy [ 1 , 2 ] . in addition , the present case had a normal serum cea level , solitary pulmonary metastasis and no intrathoracic nodal metastasis , which are favorable prognostic factors revealed in previous studies [ 9 , 10 , 11 ] . thus , the only factor to predict a poor prognosis in the case was her elevated ctc count ( 6 ctcs/7.5 ml peripheral blood ) , which was consistent with a study showing that an elevated ctc count ( 3 or more ctcs/7.5 ml ) was an independent and significant prognostic factor for shorter survival . thus , the present case suggests that patients with an elevated ctc count may not be good candidates for pulmonary metastasectomy even when the case meets the conventional selection criteria . a prospective study , to assess the clinical value of the ctc test in selecting patients for pulmonary metastasectomy , is warranted . the authors declare that they do not have anything to disclose regarding conflict of interest with respect to this work .
pulmonary metastasectomy is indicated for selected patients with metastatic colorectal cancer . a 43-year - old woman presented with solitary pulmonary metastasis from descending colon cancer and pulmonary metastasectomy was performed because of absence of any other active metastasis as well as normal serum carcinoembryonic antigen value . however , she died due to early development of nodal and bone metastases within 6 months after thoracotomy . the presence of circulating tumor cells ( ctcs ) in the peripheral blood ( 6 ctcs/7.5 ml ) was the only factor to predict such a poor prognosis , suggesting that the ctc test is useful in selecting patients for pulmonary metastasectomy .
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henoch - schnlein purpura ( hsp ) , a systemic iga vascultitis , is uncommon in adults , with an incidence rate of 0.1 to 1.2 per million in adults over 20 years old . this vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients . we report a case of an older woman with hsp presenting with small bowel obstruction and vasculitic rash . we report a 67-year - old woman who presented with small bowel obstruction and skin rash . skin biopsy revealed leukocytoclastic vasculitis with + iga granular deposition within the walls of superficial dermal vessels . she continued to do well with normal kidney function at a 3-month follow - up visit . hsp , a systemic iga vasculitis , is a predominantly pediatric vasculitis and is uncommon in adults . in adults , however , gastrointestinal manifestation is less common in older patients , and bowel perforation and obstruction are rare . iga nephropathy ( igan ) is the most common primary glomerulonephritis ( gn ) in the world . there is a peak incidence of igan in the second and third decades of life . henoch - schnlein purpura ( hsp ) is uncommon in adults , with an incidence rate of 0.1 - 1.2 per million in adults over 20 years old . this vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients . a 67-year - old woman presented to the hospital with a 7-day history of severe , diffuse abdominal pain associated with nausea and vomiting . she simultaneously developed pain involving both her elbows with difficulty bending her arms and a blotchy red purpuric rash over of the lower extremities knee down [ figure 1 ] . a blotchy red purpuric rash over of the lower extremities knee down physical examination revealed an abdominal distention with increased bowel sound . initial laboratory investigations revealed no leukocytosis with normal serum creatinine of 0.7 mg / dl . urinalysis demonstrated microscopic hematuria and mild proteinuria . computed tomography ( ct ) of abdomen was performed [ figure 2 ] and demonstrated a segment of bowel edema with partial small bowel obstruction . a direct immunofluorescent technique showed + iga granular deposition within the walls of many superficial dermal vessels . she underwent kidney biopsy for evaluation for vasculitis , which confirmed the diagnosis of hsp with mild mesangial proliferative igan . she had a bowel rest and a nasogastric tube was placed for her partial small bowel obstruction . computed tomography ( ct ) of the abdomen demonstrated a segment of bowel edema with partial small bowel obstruction iga nephropathy ( igan ) is the most common primary glomerulonephritis ( gn ) in the world . recently , a meta - analysis of 6,543 patients with igan demonstrated an increase in incidence of esrd in the older group . . ninety percent of cases occur in the pediatric age group between the ages of 3 and 15 years . hsp occurs uncommonly in adults with an incidence rate of 0.1 - 1.2 per million in adults over 20 years old . in adults , however , gastrointestinal manifestation is less common in older patients , and bowel perforation and obstruction are rare .
context : henoch - schnlein purpura ( hsp ) , a systemic iga vascultitis , is uncommon in adults , with an incidence rate of 0.1 to 1.2 per million in adults over 20 years old . this vasculitic syndrome can present as an uncommon cause of intestinal obstruction in older patients . we report a case of an older woman with hsp presenting with small bowel obstruction and vasculitic rash.case report : we report a 67-year - old woman who presented with small bowel obstruction and skin rash . skin biopsy revealed leukocytoclastic vasculitis with + iga granular deposition within the walls of superficial dermal vessels . kidney biopsy confirmed the diagnosis of hsp with mild mesangial proliferative iga nephropathy . her abdominal pain and small bowel obstruction were improved with conservative treatment . she continued to do well with normal kidney function at a 3-month follow - up visit.conclusion:hsp , a systemic iga vasculitis , is a predominantly pediatric vasculitis and is uncommon in adults . in adults , the disease process is identical to that in children . however , gastrointestinal manifestation is less common in older patients , and bowel perforation and obstruction are rare . intestinal obstruction with skin rash and renal involvement should raise suspicions of hsp .
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mri images of patients were spatially normalized into a standard stereotaxic space for quantitative comparison of tumor location ( spm5 ; www.fil.ion.ucl.ac.uk/spm ) . radiologists masked to patient subtype determined the 3-dimensional location of the tumor or surgical cavity relative to predefined anatomical landmarks . the expression of mouse orthologs of key signature genes of human wnt and shh - subtype medulloblastoma ( supplemental dataset 1 and supplemental table ) were mapped in the developing mouse hindbrain using four publically accessible datasets ( see supplemental methods ) . blbp - cre , ctnnb1 , atoh1-cre and tp53 mice were bred to generate appropriate genotypes and subject to clinical surveillance for signs of tumor development . atoh1-ctnnb1 transgenic mice were generated by pro - nuclear injection . in utero electroporation and cell tracking the uterus was externalized and the dorsal brainstem of e12.5 embryos electroporated with cmv - egfp plasmid dna . . 2 10 gfp cells / well were cultured in poly - d - lysine - coated 96-well plates and challenged with mutant - ctnnb1-gfp , control gfp virus , wnt1 protein ( 50ng / ml ) or shh supernatant ( 3ug / ml ) prior to pulsing with [ methyl-3h]thymidine and scintillation counting . immunohistochemistry was performed using routine techniques and primary antibodies of the appropriate tissues as described ( supplemental methods ) . mrna expression ( geo accession number gse24628 ) and dna copy number profiles ( available at http://stjuderesearch.org/site/authors/gilbertson ) were generated from mouse and human tissues using appropriate microarray platforms as detailed ( supplemental methods ) . reverse transcriptase real time - pcr analysis and gene re - sequencing of human medulloblastomas were performed as described previously3 . mrna expression and dna microarray profiles of human and mouse medulloblastomas were integrated using established and novel bioinformatic and statistical approaches . mri images of patients were spatially normalized into a standard stereotaxic space for quantitative comparison of tumor location ( spm5 ; www.fil.ion.ucl.ac.uk/spm ) . radiologists masked to patient subtype determined the 3-dimensional location of the tumor or surgical cavity relative to predefined anatomical landmarks . the expression of mouse orthologs of key signature genes of human wnt and shh - subtype medulloblastoma ( supplemental dataset 1 and supplemental table ) were mapped in the developing mouse hindbrain using four publically accessible datasets ( see supplemental methods ) . blbp - cre , ctnnb1 , atoh1-cre and tp53 mice were bred to generate appropriate genotypes and subject to clinical surveillance for signs of tumor development . atoh1-ctnnb1 transgenic mice were generated by pro - nuclear injection . in utero electroporation and cell tracking were performed by anesthetizing pregnant mice of the appropriate genotype . the uterus was externalized and the dorsal brainstem of e12.5 embryos electroporated with cmv - egfp plasmid dna . . 2 10 gfp cells / well were cultured in poly - d - lysine - coated 96-well plates and challenged with mutant - ctnnb1-gfp , control gfp virus , wnt1 protein ( 50ng / ml ) or shh supernatant ( 3ug / ml ) prior to pulsing with [ methyl-3h]thymidine and scintillation counting . immunohistochemistry was performed using routine techniques and primary antibodies of the appropriate tissues as described ( supplemental methods ) . mrna expression ( geo accession number gse24628 ) and dna copy number profiles ( available at http://stjuderesearch.org/site/authors/gilbertson ) were generated from mouse and human tissues using appropriate microarray platforms as detailed ( supplemental methods ) . reverse transcriptase real time - pcr analysis and gene re - sequencing of human medulloblastomas were performed as described previously3 . mrna expression and dna microarray profiles of human and mouse medulloblastomas were integrated using established and novel bioinformatic and statistical approaches .
medulloblastoma encompasses a collection of clinically and molecularly diverse tumor subtypes that together comprise the most common malignant childhood brain tumor14 . these tumors are thought to arise within the cerebellum , with approximately 25% originating from granule neuron precursor cells ( gnpcs ) following aberrant activation of the sonic hedgehog pathway ( hereafter , shh - subtype)38 . the pathological processes that drive heterogeneity among the other medulloblastoma subtypes are not known , hindering the development of much needed new therapies . here , we provide evidence that a discrete subtype of medulloblastoma that contains activating mutations in the wnt pathway effector ctnnb1 ( hereafter , wnt - subtype)1,3,4 , arises outside the cerebellum from cells of the dorsal brainstem . we found that genes marking human wnt - subtype medulloblastomas are more frequently expressed in the lower rhombic lip ( lrl ) and embryonic dorsal brainstem than in the upper rhombic lip ( url ) and developing cerebellum . magnetic resonance imaging ( mri ) and intra - operative reports showed that human wnt - subtype tumors infiltrate the dorsal brainstem , while shh - subtype tumors are located within the cerebellar hemispheres . activating mutations in ctnnb1 had little impact on progenitor cell populations in the cerebellum , but caused the abnormal accumulation of cells on the embryonic dorsal brainstem that included aberrantly proliferating zic1 + precursor cells . these lesions persisted in all mutant adult mice and in 15% of cases in which tp53 was concurrently deleted , progressed to form medulloblastomas that recapitulated the anatomy and gene expression profiles of human wnt - subtype medulloblastoma . we provide the first evidence that subtypes of medulloblastoma have distinct cellular origins . our data provide an explanation for the marked molecular and clinical differences between shh and wnt - subtype medulloblastomas and have profound implications for future research and treatment of this important childhood cancer .
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a 3-month - old female child weighing 4.6 kg was referred to us with the provisional diagnosis of a ventricular septal defect in the status of congestive heart failure under medication with furosemide ( 2.8 mg three times a day ) , spironolactone ( 6.25 mg once a day ) , and digitalis ( 10 mcg twice a day ) . the echocardiogram showed a tunnel connecting the aorta and the right ventricle , called the aorto - right ventricular tunnel ( arvt ) ( fig . 1 ) . the ostium of the right coronary artery was in close proximity to the opening of the tunnel ; therefore , interventional management was not feasible . during surgery , after opening the pericardium , we could see the tunnel on the anterior aspect and on the left of the aorta ( fig . the patent ductus arteriosus was ligated , cardiopulmonary bypass was instituted , and then , the aorta was promptly cross - clamped . via a standard oblique aortotomy and distal to the anomaly , a slit - like opening was identified originating above the upper border of the right sinus of valsalva , approximately 2 mm to the left and cephalad to the right coronary ostium . the passage of a probe through the tunnel and into the right ventricular infundibulum confirmed the diagnosis . identification of the ventricular end was not possible , and the aortic orifice was closed with a patch of bovine pericardium by using multiple interrupted 7 - 0 prolene sutures ( fig . the postoperative course was uneventful , and the patient was discharged from the hospital on postoperative day 9 . at the 24-month follow - up , she was thriving well and an echocardiographic examination showed no residual abnormalities and a competent aortic valve . aorto - ventricular tunnel is a rare congenital malformation and is defined as the presence of an extracardiac channel that connects the ascending aorta above the sinotubular junction to the cavity of the left or the right ventricle . , we report the case of a successful surgical closure of arvt , in which the origin of the right coronary artery was proximal to the ostium of the tunnel . only 18 cases of arvt have been reported thus far in the english literature [ 27 ] . a differential diagnosis of this malformation includes ventricular septal defect , aortic insufficiency , aortopulmonary window , coronary artery fistula , patent ductus arteriosus , and ruptured sinus of the valsalva aneurysm . the difference between aorto - ventricular tunnel and arvt is that in the case of the former , the vascular orifice lies in the tubular aorta and not in a sinus of the aortic valve and runs outside the heart between the muscular subpulmonary infundibulum and the aortic valvar sinuses , as in our case . the possible anomalies in coronary anatomy should be recognized in order to plan a therapeutic strategy . when the entire coronary system originates from a single ostium located at the left sinus , for example , interventional closure by a device may be feasible . however , in cases where the tunnel is in close proximity to the coronary ostia or aortic valve leaflets , surgical management is imperative . during surgery , the patent ductus arteriosus should be ligated before cardiopulmonary bypass institution and an aortic cross - clamp should be applied promptly in order to prevent right ventricular distention . the aortic opening of most tunnels lies above the right coronary sinus of valsalva and rarely above the left sinus of valsalva or the intercoronary commissure . surgical repair of arvt consists of closing the tunnel in such a way that the aortic valve is supported , the coronary circulation is not compromised , and the right ventricular outflow tract obstruction is prevented . we prefer patch closure of the aortic end as it is a safer method for preventing recurrence or progressive aortic regurgitation . ventricular end closure in aorto - left ventricular tunnels is necessary to prevent blind - ending pouch formation and subsequent compression of the right ventricle . however , under the low - pressure conditions of arvts , when a ventricular orifice is not identifiable , ventricular end closure can be omitted to avoid the consequences of a right ventricular incision . when the ostium of a coronary artery is proximal to the tunnel , the patch should be deviated distally in order to maintain the perfusion from the aorta . moreover , re - attachment of the orifice or patch angioplasty is considered an alternative technique in these cases . arvt repair should be conducted as early in life as possible in order to minimize damage to the right ventricle and to avoid pulmonary vasculature obstruction .
aorto - ventricular tunnel is a rare congenital malformation , and aorto - right ventricular tunnel ( arvt ) is an even less common entity . here , we report the case of a 3-month - old female who underwent successful surgical closure of arvt . the origin of the right coronary artery was proximal to the ostium of the tunnel .
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can -blockers improve outcomes in septic patients with myocardial depression ? in the previous issue of critical care , schmittinger and colleagues discuss the results of treating myocardial depression in septic shock patients with the combination of milrinone and metoprolol . support included volume infusion , treatment with milrinone ( a phosphodiesterase inhibitor ) and rapid addition of norepinephrine then vasopressin ( 31 of 40 patients ) based on hemodynamic measurements . metoprolol , a 1-selective adrenergic antagonist , was given enterally at low doses ( 25 to 47.5 mg / day ) . over 96 hours of follow - up , inotropic and vasopressor doses decreased , heart rate decreased , and cardiac output remained stable . specifically , on metoprolol , norepinephrine and milrinone dosages had to be increased in 22.5% and 15% of patients , respectively . dialysis makes interpreting creatinine changes difficult and the use of a linear mixed effects model for analysis can lead to less accurate type 1 error rates in small sample sizes . despite these limitations , this is one of the first trials to suggest the feasibility of acutely treating myocardial depression from sepsis with adrenergic antagonists . despite advances in the care of septic patients myocardial injury and depression are common during sepsis and are likely multi - factorial in etiology . the adrenergic nervous system is activated in sepsis and pharmacological doses of -agonists are commonly utilized during goal directed therapy to support oxygen delivery and maintain perfusion pressure . there is a large body of evidence suggesting that excessive adrenergic levels can cause myocardial damage . transgenic animals with overexpression of genes involving adrenergic signaling develop myocardial apoptosis , fibrosis , contractile dysfunction and eventually left ventricular dilatation . in cell culture , catecholamines have been shown to cause myocardial cell death and induce maladaptive changes in gene expression . severe head injury causes massive adrenergic discharge resulting in myocardial necrosis and cardiac dysfunction . in chronic heart failure , activation of the adrenergic nervous system is associated with increased mortality , and inotropic therapy worsens outcomes . in general -blockers are well tolerated even in severe heart failure when started at a low dose . recent large prospective trials would mandate caution when using -blockers in acute settings of hemodynamic compromise . the commit trial in acute myocardial infarction showed that metoprolol 's benefit in reducing reinfarction and arrhythmia ( 10 per 1,000 ) was offset by an increase in cardiogenic shock ( 11 per 1,000 ) . this was most prominent in the first day of therapy in elderly patients with tachycardia and low blood pressure , a population reminiscent of the one discussed in the current series . the poise trial showed that metoprolol , started 2 to 4 hours before surgery in high risk cardiac patients , led to increased rates of death and stroke . myocardial depression with -blockers could explain the need to escalate therapy with vasoactive drugs in the current series . gore and colleagues showed that esmolol acutely reduced cardiac output by 20% in septic patients . they found that even 6.25 mg of metoprolol , given orally , acutely decreased cardiac output , stroke volume and stroke work index . after 3 months and uptitration to 50 mg bid , the administration of the drug continued to cause a decrease in cardiac output and stroke work index . the use of inotropes to bridge patients to -blockade has only been reported in small heart failure studies . based on the above safety concerns , this strategy should be considered experimental until further randomized controlled studies are conducted . the reported incidence of hypotension and decreased cardiac output is high enough that it might cancel the potential metabolic and anti - adrenergic benefits of metoprolol . the likelihood that the changes observed in hemodynamic parameters are not due to metoprolol is high . if further studies are planned , smaller doses of metoprolol should be considered in dose ranging trials . carvedilol and nebivolol have vasodilatory properties that could precipitate vascular collapse in this patient population . non - selective -blockers such as esmolol and propranolol may withdraw excessive adrenergic support and precipitate worsening myocardial function .
sepsis with myocardial dysfunction is seen commonly . beta - blockers have been used successfully to treat chronic heart failure based on the premise that chronically elevated adrenergic drive is detrimental to the myocardium . however , recent reports on the acute use of beta - blockers in situations with potential hemodynamic compromise have shown the risks associated with this approach . in critical situations , the main effect of adrenergic activation is to support cardiovascular function . caution should be exercised in designing studies to assess beta - blockers in septic patients .
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the effect of whole patient health coaching on the hospitalization rate was examined in a prospective control study . the likelihood of hospitalization ( loh ) prediction model was used to select patients at high risk of hospitalization . an assessment interview was held on the phone at the outset of the telecoaching program to assess patients ' current healthcare situation . patients were subsequently contacted by phone with a view to effecting an improvement in health in the short - term and thus preventing stays in hospital , e.g. via appointments with specialists , provision of a nurse , training on correct medication intake and regular self - control . eighteen months later , the hospitalization rate for the six chronic indications was 25.3% in the study group and 28.3% in the control group .
purposethe effect of whole patient health coaching on the hospitalization rate was examined in a prospective control study.methodsthe likelihood of hospitalization ( loh ) prediction model was used to select patients at high risk of hospitalization . six chronic conditions were identified as criteria for inclusion in the program . selected patients were randomized and allocated to a study group or control group.an assessment interview was held on the phone at the outset of the telecoaching program to assess patients ' current healthcare situation . patients were subsequently contacted by phone with a view to effecting an improvement in health in the short - term and thus preventing stays in hospital , e.g. via appointments with specialists , provision of a nurse , training on correct medication intake and regular self - control . each patient was supervised for six months.resultsthe loh model was used to select a total of 9176 patients . one thousand and eighty of these were allocated to a control group.eighteen months later , the hospitalization rate for the six chronic indications was 25.3% in the study group and 28.3% in the control group . this corresponds to a relative reduction of 10.4%.conclusionswhole patient health coaching is a suitable means of preventing hospitalization in comorbid patients .
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idiopathic thrombocytopenic purpura ( itp ) is an autoimmune disorder in which antibodies are directed against platelet surface antigens , causing a characteristic purpuric rash , or bleeding . ophthalmic involvement is exceptionally rare.1 we report a patient with itp who developed spontaneous bilateral peripapillary , subhyaloid and vitreous hemorrhage with only minor platelet deficit . a 45-year - old female with underlying itp and diabetes mellitus presented to the department of ophthalmology , hospital raja perempuan zainab ii , complaining of a sudden onset of reduced vision in both eyes , of about 3 weeks in duration . there was no history of trauma , intense aerobic exercise , heavy lifting , straining on the toilet , vomiting , coughing , or vigorous sexual activity . she had been diagnosed with itp several years before , with initial presentation of menorrhagia . examination revealed visual acuity of 6/36 and 6/60 in the right and left eyes , respectively . fundus examination showed bilateral peripapillary , subhyaloid and vitreous hemorrhage , which was more pronounced in the left eye ( figure 1 ) . the patient s platelet count was 12010/l ( normal : 15045010/l ) ; her hemoglobin count was 93 after consultation by a hematologist , she was treated with oral prednisolone , starting dose of 50 mg daily ( 1 mg / kg / day ) for 1 week . review at 6 weeks showed spontaneous partial resolving of hemorrhages ( figure 2 ) , and slight improvement of vision ( to 6/24 both eyes ) . it was recommended to the patient that she undergo left eye vitrectomy ( in view of the fact the subhyaloid hemorrhage was just adjacent to the macula ) , but the patient has not yet consented to the operation . itp is an autoimmune hematological disease wherein circulating antibodies direct activity against host platelet surface antigens causing platelet destruction , thereby resulting in reduced platelet counts and subsequent complications of bleeding . the common presentations of itp are bleeding from the skin , mucous membrane , gastrointestinal tract , central nervous system , and vagina.2,3 there are no large case series on the spectrum of findings on itp in eyes ; mostly , these are single - case reports . ophthalmic manifestations associated with itp reported include vitreous hemorrhage,4 subconjunctival hemorrhage,5 massive subretinal hemorrhage with life - threatening intracranial hemorrhage,6 and vitreoretinal hemorrhages.79 majji et al,7 okuda et al,8 and pathengay et al9 have reported similar posterior segment findings in their case reports , which are also seen in our patient . it has been reported by rubenstein et al that there is a high incidence of retinal hemorrhage in patients with severe anemia ( < 80 g / l ) and severe thrombocytopenia ( < 5010/l ) , but a much lower frequency in anemic patients without thrombocytopenia.10 however , this is not what we had observed from this patient , who had only mild thrombocytopenia ( 12010/l ) and moderate anemia ( 93 g / l ) when she presented with ocular symptoms , in contrast to severe thrombocytopenia in other case reports ( 1210/l by majji et al ; 4210/l by pathengay et al ; and < 210/l by okuda et al).79 carraro et al demonstrated that retinopathy was closely associated with the conditions of severe anemia and severe , or very severe , thrombocytopenia.11 this patient was recommended to undergo left eye vitrectomy , to evacuate the subhyaloid hemorrhage that was threatening the macula ; but , she refused any surgical intervention . similar findings were seen in majji et al s patient , in whom complete resolution of hemorrhages was noted after the patient had been managed through only systemic factors , without any invasive procedure.7 nevertheless , vitrectomy can be beneficial for massive vitreoretinal hemorrhages.8 okuda et al performed vitrectomy for a patient with itp who presented with massive bilateral vitreoretinal hemorrhage . the patient s vision ( better eye ) improved from 6/90 to 6/30 after surgery .
a 45-year - old female with underlying idiopathic thrombocytopenic purpura ( itp ) complained of acute onset of reduced vision and floaters , in both eyes , for 3 weeks . visual acuity was 6/36 and 6/60 in the right eye and left eye , respectively . ophthalmoscopy showed bilateral peripapillary , subhyaloid and vitreous hemorrhage . hematological evaluation revealed moderate anemia ( hemoglobin : 93 g / l ) and mild thrombocytopenia ( platelets : 120109/l ) . she was co - managed by a hematologist and ophthalmologists ; she was treated medically . follow - up care during the next 6 weeks revealed spontaneous , partially resolving hemorrhage , with improvement of visual acuity . the purpose of this case report is to highlight ophthalmic involvement of itp in this patient , despite her only - mild thrombocytopenia , and her spontaneous recovery , despite her receiving only medical treatment .
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multifunction filters are getting significant attention in current analog ic design due to their versatility as the same configuration can be used for various filter responses . in the literature , simo type active filters employing different active building blocks / devices are available in both current mode ( cm ) and voltage mode ( vm ) . the advantages and utility of ta type filters have been highlighted in . there are few research publications which have been published dealing with the realisation of ta type filters [ 16 ] . vdta is one of the active elements out of those introduced in . owing to its flexibility and versatility , compared to other active building blocks / devices , several vdta - based applications have been reported in the literature [ 813 ] . in , yeil et al . proposed a simple cmos realization of vdta and its application as rf filter and double tuned amplifier . , an electronically controllable explicit current - mode sinusoidal oscillator using single vdta and in an electronically controllable fully uncoupled sinusoidal oscillator using two vdtas have been presented . a universal current - mode biquad filter using single vdta has also been reported in and grounded and floating inductance circuits using single / two vdtas and one grounded capacitor have been proposed in . in this communication , we present a new configuration for realizing ta universal biquadratic filter with one input and three outputs . the proposed new circuit offers various features such as employment of only two vdtas , two grounded capacitors , simultaneous realization of lp , hp , and bp filtering responses without changing circuit topology , independent control of 0 and bw , and low active and passive sensitivities . the workability of the proposed ta filter has been verified by spice simulations with tsmc cmos 0.18 m process parameters . the symbolic notation of the vdta is shown in figure 1 , where vp and vn are input terminals and z , x , and x are output terminals , having high impedance values . the terminal equations can be described by the following set of equations : ( 1)[izix+ix]=[gm1gm1000gm200gm2][vvpvvnvz ] . the proposed ta filter configuration is shown in figure 2 . assuming ideal vdtas , a routine analysis of the circuit shown in figure 2 yields the following current transfer functions : ( 2)t1(s)|bp = io1vin=s((gm1gm2)/c1)d(s)t2(s)|lp = io2vin=(gm1gm3gm4)/c1c2d(s)t3(s)|hp = io3vin = s2gm1d(s)t4(s)|notch=(io3+io2)vin = gm1(s2+gm3gm4/c1c2)d(s)t5(s)|ap=(io3+io2+io1)iinhhhhhhh = gm1{s2s(gm2/c1)+gm3gm4/c1c2}d(s ) , where ( 3)d(s)=s2+s(gm1c1)+gm3gm4c1c2 . the 0 , bw , and quality factor ( q0 ) are given by ( 4)0=gm3gm4c1c2(5)bw = gm1c1(6)q0=gm3gm4c1(gm1)2c2 . from ( 4 ) and ( 5 ) , it is seen that 0 and bw are independently controllable , the former through gm3 or gm4 or c2 and the later through gm1 . i o1 and io2 are explicit current outputs but io3 is taken through grounded capacitor c1 . to extract this current ( io3 ) explicitly another device with its input virtually grounded will be needed due to which the capacitor c1 will not be physically connected to ground but it will still be virtually grounded . taking into account the various vdta parasitics such as the finite p - terminal parasitic impedance consisting of a resistance rp in parallel with capacitance cp , the finite n - terminal parasitic impedance consisting of a resistance rn in parallel with capacitance cn , the finite x - terminal parasitic impedance consisting of a resistance rx in parallel with capacitance cx , and the parasitic impedance at the z - terminal consisting of a resistance rz in parallel with capacitance cz , then the 0 and q0 including the influence of parasitic are given by ( 7)0=((2rzrn+1rxrz+1rz2+gm1rz+gm3gm4 ) (cz2c1c2 + 2cnc2+cxc2+czc2+c1cz + cxcz+2cncz+cz2)12rzrn)1/2q0=(2rzrn+1rxrz+1rz2+gm1rz+gm3gm4 ) (cz2c1c2 + 2cnc2+cxc2+czc2+c1cz + cxcz+2cncz+cz2)1/2 (2c2rn+c2rx+c2rz+c2gm1 + 2czrn+czrx + 2czrz+czgm1+c1rz+2cnrz+cxrz)1 . using the classical definition of the sensitivity coefficient , that is , ( 8)sxf = xffx , where f may represent the parameters of the elements and x may represent passive elements as well as the active elements with respect to which the sensitivity is to be evaluated and by taking c1 = 0.05 nf , c2 = 0.1 nf , rp = rz = , cp = cx = cz = 0 , gm1 = gm2 = gm3 = gm4 = 631.702 a / v , the sensitivities of 0 and q0 are found to be either 0 or 0.5 . to confirm theoretical analysis , the proposed configuration was simulated using cmos vdta as given in . the passive elements of the configuration were selected as c1 = 0.05 nf and c2 = 0.1 nf . the power supplies used for cmos vdta were 0.9 v. the transconductances of vdta were controlled by bias currents . figure 3 shows the simulated filter responses of lp , bp , hp , br , and ap . a new transadmittance - mode biquad filter has been proposed which employs only two vdtas and two grounded capacitors . the proposed filter can realize the second - order lp , bp , and hp responses simultaneously without changing the circuit topology and without any matching condition . the notch and ap filter responses are also obtainable through proper connections of the currents . the filter circuit offers ( i ) independent control of 0 and bw and ( ii ) low active and passive sensitivities .
a new resistorless single - input - multi - output ( simo ) universal transadmittance ( ta ) type filter employing two voltage differencing transconductance amplifiers ( vdta ) and two grounded capacitors is proposed . the proposed topology realizes simultaneously low pass ( lp ) , high pass ( hp ) , and band pass ( bp ) filter functions . band rejects ( br ) and all pass ( ap ) filters are also realizable through appropriate connections of currents . the proposed configuration also offers independent control of natural angular frequency ( 0 ) and bandwidth ( bw ) and low active and passive sensitivities . the workability of proposed configuration has been demonstrated through pspice simulations with tsmc cmos 0.18 m process parameters .
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we report a case of metastasis to the proximal phalanx of the left hand as the initial manifestation of a lung adenocarcinoma . a 61-year - old male smoker presented in december 2013 at the chu sud runion with pain in his left ring finger and in the ribs , both secondary to indirect trauma a few weeks ago . he had no other complaints and was in good general condition . on physical examination of the hand , his left ring finger measured 80 mm in circumference , while the contralateral healthy finger had a circumference of 55 mm . we performed an x - ray of the rib cage , which showed a lytic lesion of the eighth rib . an x - ray of the left hand showed a lytic bone lesion with the destruction of the bone cortex in the proximal phalanx and a swelling of the surrounding soft tissue ( fig . 2 ) . a ct scan of the chest showed several suspicious opacities , one in the left lung hilum , a stellar image at the apex of the right lung , and a lytic lesion of the anterior arc of the eighth rib ( fig . then a biopsy of the rib mass identified an infiltration of the parietal pleura and striated muscle tissue by a pulmonary adenocarcinoma , moderately differentiated ttf1 + , kras mutant , braf wild type , egfr wild type , and with no alk rearrangement . the bone scintigraphy showed multiple bone metastases on the rib cage , the spine , and the left scapula . the patient received radiotherapy for his left ring finger in march 2014 ( dose of total 30 gy/10 fractions , 5 fractions / week , 6-mv x - rays with 5-mm bolus ) . over the next month , the left ring finger became much less painful . an x - ray of the left hand done 1 month after the end of the radiotherapy showed a decrease of the soft tissue swelling and an early reossification of the proximal phalanx ( fig . chemotherapy with cisplatin , pemetrexed and bevacizumab was started at the end of the radiotherapy . peripheral bone metastases are mostly associated with disseminated disease , but in our case , it was the initial manifestation of a lung adenocarcinoma . it can be easily confused with an infection ; an x - ray can be very helpful . pathology may be necessary in case of doubt and additional exams such as chest ct and bronchoscopy should be performed to identify the primary lesion . patients mostly have to be treated with an amputation of the finger [ 7 , 8 , 9 , 10 , 11 ] . patients with bone metastases have a median survival of only 6 months , and a choice of treatment should be made considering this poor prognosis : palliative radiotherapy and non - steroidal analgesics helped our patient and improved his quality of life .
phalanx bone metastasis as the initial presenting sign of lung cancer is a rare presentation . lung cancer is known to metastasize to the bone , but rarely to the fingers . a 61-year - old male smoker presented with pain in the left ring finger . severe pain discouraged the patient from using his left hand . an x - ray of the left hand showed a lytic bone lesion . the patient was treated with finger radiotherapy . analgesics were no longer needed and the patient was able to reuse his left hand in his everyday life . palliative radiotherapy relieved our patient and improved his quality of life .
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a 72-year - old man with a history of melanoma in situ presented for his regular follow - up visit . the nodule was not present in the previous visit held six months earlier ( figures 1 , a and b ) . the dermoscopic examination revealed a nonpigmented lesion characterized by a polymorphous vascular pattern , consisting of dotted and short curved linear vessels on a red - pinkish background . the latter unspecific pattern did not allow a definite diagnosis , prompting us to excise the nodule ( figure 2 ) . on low power examination the lesion was well circumscribed and involved the reticular dermis ( figure 3a ) . at higher magnification , the extensive myxoid change was evident ; cellularity was inconspicuous and vascularity consisted of small , thin - walled vessels ( figure 3b ) . sa was initially described by carney et al . in 1985 in association with carney s complex syndrome , a rare autosomal dominant disorder characterized by neoplasia involving the heart , central nervous system , and endocrine organs . in 1998 allen et al . calonje et al . reported the clinicopathological and immunohistochemical features of sa as an independent disease . in 2014 green et al . reported the dermoscopic features of sa for the first time and described the red planet signa red , translucent , globular exophytic lesion with arborizing vessels that looks like a blood moon during recent lunar eclipses . in contrast , in our case , the patient presented with a pinkish - red nodule on the lower back , not a polypoid lesion . additionally , dermoscopy did not show the red planet sign , thus we believe that this sign may be highly unspecific or anecdotic . we did not perform an echocardiogram or other tests because our patient lacked skin pigmentation , mucosal lesion , other cutaneous tumors or family history of myxomas . although spitz nevus and amelanotic melanoma come first in the list of differential diagnoses of nonpigmented nodular lesions displaying predominantly dotted vessels under dermoscopy , several other less common tumors might exhibit a similar pattern . however , since these dermoscopic findings are highly unspecific , a nodule dermoscopically characterized by predominantly dotted vessels should be promptly excised to rule out melanoma .
superficial angiomyxomas ( sa ) of the skin are rare benign cutaneous tumors of soft tissue composed of prominent myxoid matrix and numerous blood vessels . sa are more common in males [ 1 ] and they are usually located on the trunk but can also appear on the lower limbs , head , neck and genitalia [ 2,3 ] . treatment is surgical , the total excision is curative , but local recurrence is possible [ 4 ] . herein we present a 72-year - old patient with a history of melanoma in situ , with a new lesion on the lower back .
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infective endocarditis ( ie ) is an infection of the endocardial surface of the heart . ie also produces a wide variety of systemic signs and symptoms through several mechanisms , including both sterile and infected emboli and various immunological phenomena . subacute left - sided bacterial endocarditis is a serious condition that may be overlooked due to highly variable clinical manifestations . we present the case of a 45-year - old man who presented with complaints of fullness in his abdomen and was referred to a surgeon . a 45-year - old man presented to the surgery clinic with a 30-day history of fullness in the abdomen . the patient had poor appetite and fatigue and denied any history of trauma , autoimmune disease , peptic ulcer , or any gastrointestinal ( gi ) problem , sweating , allergies , any other medical conditions or weight loss . he did not smoke or use illicit drugs or alcohol and had not traveled recently ; further , there have been no changes in his daily routine . the patient had never had any major surgery and did not consume any drug but his history demonstrated acute renal failure two months ago because of which he underwent dialysis via jugular catheter . kidney was recovered and the patient discharged in a good condition with normalisation of creatinine . on physical examination , the patient appeared asthenic , and had no respiratory distress or jaundice . his blood pressure was 130/80 mm hg , and heart rate was regular , ie , 80 bpm . the respiratory rate was 16 breaths / min , and he was afebrile , with a recorded temperature of 36.7c . the lungs were clear on auscultation , and his abdomen was soft and non - tender . but spleen was 4 cm under costal margin , and hepatomegaly or lymphadenopathy was not detected . a workup of the patient was initiated . a complete blood cell count , chest x - ray , liver enzymes examination , thyroid function test , malaria smear , kala - azar , infectious mononucleosis ab , anti - nuclear factor , coomb 's test , blood cultures , serology for hiv , hepatitis and other infections , electrocardiogram , and urinalysis all showed normal findings . abdominal sonography was performed and it was normal ( no portal hypertension , splenic or portal vein thrombosis ) except for splenomegaly . mri chest / abdomen , upper gi endoscopy was performed and was normal . a bone marrow aspiration and biopsy was done for him , which was normal . because of progressively enlarged splenomegaly , the patient underwent diagnostic splenectomy . in pathology examination , three months after splenectomy , the patient had chest pain and was referred to a cardiologist . on physical examination , the patient appeared asthenic ; he had a grade 3/6 diastolic murmur in the aortic area and conjunctival haemorrhage . two - dimensional echocardiography was done and showed oscillating hypermobile mass ( vegetation ) on aortic valve and severe aortic regurgitation [ figure 1 ] . our patient presented with one major criterion and three minor criteria , thus satisfying the duke criteria for definite endocarditis . the patient was discharged from the hospital after he recovered well from surgery . in the culture of the valve , streptococcus viridans was present [ figure 2 ] . a pleiomorphic cellular infiltrate at the vegetation border can be seen including histiocytes ( arrows ) overall , the most common cause of culture - negative ie is prior use of antibiotics such as that seen in our patient who was advised to use antibiotics after catheter insertion and splenectomy . ie remains a diagnostic and therapeutic challenge . if left untreated , ie is generally fatal . the symptoms of early subacute native valve endocarditis ( nve ) are usually subtle and nonspecific . they include low - grade fever ( absent in 3 - 15% of patients ) , anorexia , weight loss , influenza - like syndromes , polymyalgia - like syndromes , pleuritic pain , syndromes similar to rheumatic fever ( e.g. , fever , dulled sensorium such as that seen in typhoid , headaches ) , and abdominal symptoms ( e.g. , right upper quadrant pain , vomiting , postprandial distress , appendicitis - like symptoms ) . patients with splenomegaly may come to medical attention for a variety of reasons and may complain of left upper quadrant pain or fullness or of early satiety . our patient was a typical mismanaging case , because the surgeon and haematologist had not paid attention to history of catheter insertion . our patient 's cardiac examination could not confirm a murmur at initial presentation necessary to diagnose and referring patient to cardiologist . in the absence of this , clinicians are encouraged to utilise transthoracic echocardiogram as an important tool to establish the diagnosisand work up of isolated splenomegaly . this patient did not present with the common sign and symptoms associated with an ie ; hence , we recommend echocardiography in the work up of isolated splenomegaly .
sub acute left - sided bacterial endocarditis is a serious condition that may be overlooked due to highly variable clinical manifestations . we present the case of a 45-year - old man who presented with complaints of fullness in his abdomen and splenomegaly that referred to the surgery clinic . he underwent diagnostic splenectomy . 3 month after splenectomy endocarditis was diagnosed . we recommend echocardiography in the work up of isolated splenomegaly .
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sample preparation : the dna origami structure was designed by using cadnano software ( version 0.2.3 ; http://cadnano.org/legacy;7 design schematics in figure s1 ) . curvature along the length of the lever was introduced by a pattern of base deletions and insertions as described previously ( deletion pattern in figure s2).13 the 8634nucleotide singlestranded scaffold dna , derived from m13mp18 , was prepared as previously described.12 , 25 scaffold dna ( 10 nm ) was mixed with each staple strand ( 100 nm ; high purity salt free , mwg eurofins operon ) in te buffer ( tris ( 10 mm , ph 7.6 ) , edta ( 1 mm ) ) containing mgcl2 ( 18 mm ) and nacl ( 5 mm ) . the mixture was subjected to a nonlinear thermal annealing ramp from 65 to 25 c over 24 h ( see the supporting information ) . folded structures were electrophoresed on 0.7 % agarose gels containing 0.5tbe buffer ( tris ( 45 mm ) , boric acid ( 45 mm0 , edta ( 1 mm ) ) withmgcl2 ( 11 mm ) and ethidium bromide ( 0.5 g ml ) at 5.5 v cm for 2 h in an icewater bath . dna was recovered by manually squeezing the excised gel slice and collecting the liquid . tem imaging : gelpurified origami solution ( 3 l ) was adsorbed onto glowdischarged tem grids ( formvar / carbon , 300 mesh cu ; ted pella , redding , ca ) at 20 c , and then stained with aqueous uranyl formate ( 2 % ) containing sodium hydroxide ( 25 mm ) . imaging was performed at 30 000 magnification ( zoomout images in figure s5 at 12 000 magnification ) with a jem1011 transmission electron microscope ( jeol ) operated at 80 kv , equipped with a fastscanf114 camera ( tvips , gauting , germany ) . particles in tem images were picked by the interactive boxing routine ( e2boxer.py ) of eman2 software ( http://blake.bcm.edu/emanwiki/eman ; example set of particles in figure s6).26 as a service to our authors and readers , this journal provides supporting information supplied by the authors . such materials are peer reviewed and may be reorganized for online delivery , but are not copyedited or typeset . technical support issues arising from supporting information ( other than missing files ) should be addressed to the authors .
abstractthe application of commonly used force spectroscopy in biological systems is often limited by the need for an invasive tether connecting the molecules of interest to a bead or cantilever tip . here we present a dna origamibased prototype in a comparative binding assay . it has the advantage of in situ readout without any physical connection to the macroscopic world . the seesawlike structure has a lever that is able to move freely relative to its base . binding partners on each side force the structure into discrete and distinguishable conformations . model experiments with competing dna hybridisation reactions yielded a drastic shift towards the conformation with the stronger binding interaction . with reference dna duplexes of tuneable length on one side , this device can be used to measure ligand interactions in comparative assays .
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the carney complex ( cnc ) is a rare dominantly inherited syndrome characterized by skin pigment abnormalities , endocrine over activity and cardiac myxomas . about 600 patients worldwide have been reported by the national institute of health ( nih ) - mayo clinic ( usa ) and the cochin centre ( france ) by january 2008 . cardiac myxomas are rare benign tumors with an estimated incidence of 0.5 - 1 per million population per year , accounting for 45% primary cardiac tumors in adults and 15% in children . recurrence is reported in sporadic ( 4%-7% ) and familial cases ( 10%-21% ) with the interval between the formation of the new tumor of more than 4 years . although well documented in the adult population , information about cerebral embolism in the pediatric population is still limited . the following case illustrates a combination of the two rare diseases clinically presenting with a cerebral stroke and an extremely rapid recurrence of the left atrial myxoma in a teenager . an athletic , dark - haired 13-year - old girl was admitted to our hospital with an acute migraine attack with vomiting , dispnoea , abdominal pain , and left - sided body weakness . anamnesis revealed frequent migraines within the previous 2 years associated with vomiting and bulbar motion abnormalities . on admission , she was somnolent , pale , with left facial nerve paresis , left - sided body weakness , hyperactive deep tendon reflexes , and positive plantar reflex . multiple hyperpygmentated skin spots and hairy forearms and thighs were also noted without visible evidence of neurofibromas . cardiac auscultatory findings , the chest x - ray and the computed tomography ( ct ) of the brain performed on admission were normal . the repeated ct scan after 12 h demonstrated a massive right - sided fronto - parietal ischemic zone [ figure 1a ] . heart ultrasound ( echo ) showed a huge left atrial mass ( 28 37 57 mm ) on a short peduncle , arising from the roof of the left atrium protruding through the mitral valve into the left ventricle [ figure 1b ] . the mass was enucleated in toto including a piece of the underlying atrial septum via the left and right atriotomy [ figure 2a , b ] . the postoperative course was uneventful and the girl was discharged to a rehabilitation centre . her postoperative cardiac echo examination on day 15 , 2 , and 4 months after surgery showed no residual tumor . she was readmitted to our hospital six months after the initial operation with severe headache , choking and speech difficulties . the cardiac echo demonstrated a new tumorous mass ( 8 10 mm ) , hanging on a long peduncle from the midportion of the interatrial septum . the finding was confirmed by nuclear magnetic resonance imaging ( nmr ) [ figure 1c ] . more attention was paid to her hypertelorism and pigmentation but no endocrine abnormalities ( thyroid and parathyroid gland , pituitary and adrenal gland ) were detected by routine imaging and laboratory investigations . the dna samples were sent to the referent centre for carney complex ( dr stratakis , national institute of health , university of washington , and seattle ) where it was confirmed protein kinase a regulatory subunit 1a mutation ( prkar1a ) , c418_419delca het in exon 4 in our patient . the genetic investigation of the family was negative , the girl obviously being a new mutation . ( c ) nuclear magnetic resonance imaging of recurrent myxoma ( a ) intraoperative view of the tumor ( b ) the enucleated myxoma her routine follow up at 1 , 3 , 6 , 12 , and 24 months showed no new cardiac masses . intracardiac tumors in the pediatric age group are rare with an incidence of 0.17% in children . myxomas account for 6% of all pediatric cardiac tumors after rhabdomyomas ( 63% ) and fibromas ( 6% ) . recurrence of the tumor has been reported with the majority of patients being reoperated more than four years after the initial surgery . carney complex is a genetic disease inherited in an autosomal dominant manner , one of the additional criteria being a myxoma ( cutaneous , cardiac , breast or bone ) . our patient with atrial myxoma had a de novo carney complex diagnosed after an extremely short recurrence period of the cardiac tumor . the carney complex associated with myxoma of various locations is being extensively studied in connection with associated genetic abnormalities . the most interesting controversy is a rather frequent recurrence of myxoma after complete resection , the fact not observed in most benign neoplasms . in spite of low incidence of the carney complex , its diagnosis is often established after several years of neurological symptoms and abnormal and clearly visible skin pigmentation . this report supports the possibility of an early recurrence of cardiac myxoma , more likely to be attributed to its underlying genetic nature , rather than incomplete surgical resection .
we report a case of an extremely early recurrence of left atrial myxoma in a 13-year - old girl . on hospital admission , the clinical presentation was of cerebral embolism with noticeable spotty skin pigmentation and hypertelorism . the left atrial myxoma originated from the roof of the left atrium . the histology specimen showed typical finding of a myxoma . six months later a new intracardial mass was evacuated , the postoperative result showing the same type of myxomatous tissue . genetic investigations demonstrated carney complex . the genetic analysis of the child 's family was negative , demonstrating de novo mutation of this rare disorder .
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a 13-year - old male child presented at this tertiary care center with gradual onset loss of vision over a period of 3 years . there was no history of pain , redness , watering , floaters , photopsia , ocular or head trauma , prior ocular surgery , systemic illness / chronic drug therapy , headache , vomiting , or seizures . there was history of frequent fractures in the past with minimal trauma ( right clavicle and right arm ) . there was no history of similar ocular / visual problems in the family . however , there was history of multiple fractures with trivial trauma in elder brother . on examination , his vision in re was 20/120 improving to 20/60 and in le counting finger at 4 feet improving to 20/200 with rapd in le . on fundus examination , optic discs were normal in size , circular , and pale with distinct margin . on confrontation test colour vision ( ishihara chart ) and contrast sensitivity ( pelli robson 's chart ) were reduced in both eyes . ver ( flash ) in re showed 4 v ( amplitude ) , 114 ms ( latency ) , and in le was 2 v , 144 ms . pattern ver in re showed 3 v , 134 ms , while in le there was an extinguished response . based on the above findings , a clinical diagnosis of primary optic atrophy was made keeping a differential diagnosis of compressive optic neuropathy , hereditary optic atrophy , toxic , and nutritional optic neuropathy . 1b ] computed tomography ( ct ) scan brain images showed an abnormally increased density of all the skull bones with narrowing of bilateral optic canal ( white arrows ) . skeletal survey done subsequently also showed diffusely increased bone density of all bones , confirming the diagnosis of osteopetrosis . diameter of bony optic canal was 3.8 mm in the right side and 3.5 mm on the left side . normal average transverse diameter of the optic canal is 3.57 0.61 mm and the longitudinal diameter is 4.82 0.38 mm . 2 ] better demonstrated compression of optic nerve sheath complex on both sides ( white arrows ) . there was no sign of raised intracranial pressure or hydrocephalus . in background of recurrent fractures and optic canal narrowing with abnormal increased density of bones , the diagnosis of compressive optic neuropathy secondary to osteopetrosis - induced optic canal narrowing was made . axial and coronal ct scan brain images show an abnormally increased density of all the skull bones with narrowing of bilateral optic canal ( white arrows ) t2w sagittal mrshowing compression of optic nerve sheath complex on both sides ( white arrows ) . osteopetrosisis was caused by reduced activity of osteoclasts which results in defective remodelling of bone and increased bone density . the defect in bone turnover results in skeletal fragility despite increased bone mass , and it may also cause hematopoietic insufficiency , disturbed tooth eruption , nerve entrapment syndromes , and growth impairment . typically osteopetrosis presents with fractures , short stature , and hypocalcemia , pancytopenia and optic nerve compression and rarely hypogenitalism . there are three clinical groups of osteopetrosis , namely , infantile - malignant autosomal recessive , intermediate autosomal recessive , and autosomal dominant type . the incidence of autosomal recessive osteopetrosis is 1 in 250,000 births and autosomal dominant osteopetrosis is 1 in 20,000 births . investigations recommended are ct scan ( brain , orbit ) , mri ( brain , orbit ) , radionuclide bone scan , genetic analysis , iliac crest bone biopsy , and skeletal survey . patient was treated with high - dose calcitriol to stimulate osteoclast differentiation and anoptic canal decompression was planned . bone marrow transplant has been reported as a successful treatment option to widen the narrow optic canal in infantile osteopetrosis . it has been emphasized that optic nerve decompression should be wide and include not only unroofing of the bony canal but also drilling along both sides of the optic nerve as well as smoothening of the thick , irregular , and highly domed orbital roof by high - speed drilling to facilitate surgical exposure with minimal retraction of the frontal lobe . the aim of this case report is to highlight the possibility of optic nerve compression due to optic canal narrowing in cases of osteopetrosis . optic canal decompression may be considered as a prophylactic measure to preserve vision in partial optic nerve atrophy or in fellow eye .
a 13-year - old child presenting with gross visual impairment was diagnosed as a case of optic atrophy . however , radiological investigations revealed osteopetrosis , which , though rare , can result in optic atrophy . the aim of this case report is to highlight this possibility while evaluating cases of optic atrophy in young patients .
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this little and plain part of the human organism has various functions and its abnormalities might cause serious disabilities . in our case report , we present a unique deformity of the uvula in a 56-year - old woman . the aim of this report was to present a very rare condition , unique case uvula is mucosa covered muscular elongation of the free posterior margin of soft palate ( 1 ) . theories about the reason of its existence in this part of the soft palate are controversial . from ancient times to date , uvula was positioned at the center of diverse health problems , ranging from infectious maladies to allergic reactions ( 2 ) . although this organ seems as an unimportant and plain part of the body , it might express various types of anomalies alone or as a part of a syndrome ( 3 ) . while these deformities are generally related with cleft palate , , we aimed to present a unique case that was referred with a complaint of separate soft tissue in the middle of the soft palate to izmir bozyaka teaching and research hospital in january 2014 , and was diagnosed with unique deformity of uvula ( polyuvula ) . a 56-year - old woman was admitted to our clinic with a history of sore throat for three days . her pain was also referring to her left ear . in her head and neck examination , otoscopy and pharyngeal arches and posterior pharyngeal wall were hyperemic and edematous with an accompanying left upper jugular lymphadenopathy . in addition , we examined a uvula - like elongation from the middle of the soft palate , apart from the original uvula ( figure 1 ) . in her medical history , we learned that the patient was aware of her soft palate anomaly ; however , she had used to live with this without any complaint . we administered an oral empiric antibiotic therapy and anti - inflammatory drug for acute lymphadenitis . after two weeks and with a ten - day antibiotic therapy , patient was free of lymphadenopathy or any complaints . in latin language , uvula means little grape . this little structure means more than just a grape for ear , nose , and throat surgeons , especially in sleep medicine and sleep - related pathologies ( 4 ) . in addition to these modern medical practices , there are many ancient doctrines about uvula , particularly in western africa where uvula is connected to various health problems and uvulectomy is commonly performed to prevent neonates from diseases such as upper respiratory tract infections and chronic coughing . it is also performed to facilitate breastfeeding and speech , improve tolerance to dehydration , and prevent airway obstruction ( 5 , 6 ) . although these marvelous effects seem unrealistic , uvulectomy might be considered logical for heavy snorers ( 4 ) . nonetheless , uvulectomy is not a complication - free procedure and numerous problems might be seen after uvulectomy including velopharyngeal insufficiency and pharyngeal dryness ( 7 ) . various theories have been proposed about the functions of the uvula : it prevents hypernasality by sparing oropharynx from nasopharynx together with soft palate ( 8) ; it acts as a drain for the nasal mucous secretions by directing its flow toward the tongue base ( 9 ) ; olofsson et al . showed that uvula might have an immunological effects ( 10 ) ; and it has a role in the production of uvular sounds present in some languages such as french and arabic languages ( 11 ) . with the abnormalities of uvula more serious forms are generally seen with the major defects of palate . in our case , duplicated uvula seemed harmless and was not related to any kind of complaint . aberrant and elongated uvula is another reported type of uvular abnormality ( 2 ) . in their case report , nachman et al . although duplication of uvula seems harmless , other abnormalities of uvula and soft palate might compromise the palatal dynamics and need to be rehabilitated ( 2 ) .
introduction : deformities of the uvula are considered as the dark area of otorhinolaryngology practice . this little and plain part of the human organism has various functions and its abnormalities might cause serious disabilities.case presentation : in our case report , we present a unique deformity of the uvula in a 56-year - old woman.discussion:the aim of this report was to present a very rare condition , unique case polyuvula , and to review the literature regarding other uvula abnormalities .
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in the previous issue of critical care an interesting observational study suggests a promising avenue of research that has the potential to improve clinical outcomes . the early identification and rapid treatment of haemodynamic shock is widely acknowledged as a vital step towards improving survival . in prehospital care , this process is particularly challenging . limitations of time , equipment , available skill set and environment render the objective diagnosis of haemodynamic shock difficult . the utility of serum lactate as a tool to identify the most seriously ill patients and to monitor their response to treatment has long been recognised [ 3 - 5 ] . this latest investigation describes the prognostic value of peripheral venous or capillary blood lactate concentration , measured in 124 patients before hospital arrival by paramedic ambulance staff using hand - held battery - powered technology . the findings confirm the expected relationship between the prehospital serum lactate concentration and subsequent hospital mortality . these data should encourage further research into the prehospital use of serum lactate to facilitate prompt identification and treatment of haemodynamic shock and/or to indicate those patients who might benefit from advanced activation of medical staff in the destination hospital . the authors suggest that a single value of serum lactate measured in the prehospital environment predicts hospital mortality in this population . whilst lactate levels are clearly much greater in those patients who die , this variable does not appear to have been included in the multivariate analysis . it is the change in serum lactate , between the first measurement in the community and the second on hospital arrival , that is independently associated with death . the importance of this distinction would depend upon how these findings are applied in clinical practice . if lactate measurement is incorporated into routine prehospital care , it would probably be as part of a specific treatment algorithm . indeed , biomarkers can only be used to improve clinical outcome when used as a trigger for a specific intervention , or less commonly when used as a therapeutic target . accurate data on threshold values are essential if lactate measurement is to be used in this way . in this study , receiver operator characteristic curve analysis suggests a lactate concentration of 3.5 mmol / l as the optimal cutoff value for mortality prediction . if lactate is not an independent predictor of outcome , however the sample population is also too small and too heterogeneous to support specific conclusions regarding threshold values for specific subgroups of patients ( for example , septic shock patients ) . for similar reasons , the accuracy of lactate measurement in peripheral venous or capillary blood samples must be carefully considered . this is a simple and attractive approach that allows the measurement of serum lactate in the great majority of patients attended in the prehospital environment . the relationship between the lactate concentration in such samples and those drawn from an arterial or central venous catheter , however , has not been established . anecdotal experience suggests that lactate concentrations are often greater in peripheral blood samples but not by a constant or predictable margin . the authors are to be congratulated for completing this first phase of a promising line of investigation . future research should further clarify the clinical significance of lactate concentrations in patients with haemodynamic shock . interventional trials may then confirm the efficacy of serum lactate measurements to aid the identification of these patients and to guide their subsequent treatment .
early identification of haemodynamic shock is widely acknowledged as a vital step towards improving survival . a report in the previous issue of critical care describes the relationship between lactate concentrations in blood samples analysed in the prehospital environment and subsequent hospital mortality . these preliminary data indicate a promising avenue of research into the treatment of haemodynamic shock . larger observational and interventional trials are needed to confirm the clinical value of serum lactate measurement in the prehospital environment .
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infections by herpes viruses ( herpes simplex type 1 or 2 , varicella zoster virus [ vzv ] ) are frequent in humans , and such viruses tend to persist within cranial nerves , dorsal roots , and autonomic ganglia causing latent infections by virtue of reactivation . reactivation of vzv mainly presents with rash and pain affecting the entire dermatome and less frequently a zoster sine herpete . vzv infection of the central nervous system ( cns ) such as encephalitis , meningitis , myelitis , or vasculitis occurs rarely but is feared because of the numerous unfavorable outcomes of such presentations . cns infection with vzv in young immunocompetent adults is rare and unexpected , and only very few cases have been described so far in the world literature . we bring to limelight , the scenario of a young immunocompetent patient with vzv meningitis . a 33-year - old healthy indian male employed as a software engineer developed intermittent holocranial throbbing headache , which soon progressed to severe continuous headache , associated with fever , photophobia , and vomiting for a duration of 3 days . he also complained of rashes over the skin of his left shoulder blade and left side of his body which was of the same duration . there was no past history of similar illness , contact with people having similar complaints , or chicken pox . the patient was febrile , had neck stiffness and photophobia , and was found to have rashes along the cutaneous distribution of two lower intercostal nerves of the left side . his complete blood count was unremarkable except for raised erythrocyte sedimentation rate ( 30 mm/1 h ) . renal function tests , liver function tests , and serum c - reactive protein were also reported normal . serologies for dengue , typhoid , malaria , infectious mononucleosis , toxoplasma , rubella , cytomegalovirus , and herpes simplex antibodies were negative . human immunodeficiency virus ( hiv ) serology was negative . the cerebrospinal fluid ( csf ) analysis showed cell count of 535 cells/l csf ( differential count : 99% lymphocytes , 01% polymorphs ) , csf protein was 115 mg / dl , csf glucose was 34 mg / dl , and plasma glucose 76 mg / dl . csf grams stain , ziehl nielsen 's acid - fast bacilli stain , india ink stain , cryptococcal antigen , common bacterial antigen assay ( haemophilus influenzae b , streptococcus group b , streptococcus pneumoniae , neisseria meningitidis a cyw 135 , n. meningitidis b , and escherichia coli ) , venereal disease research laboratory , and polymerase chain reaction ( pcr ) for tuberculosis were negative . he was treated with a broad spectrum antibiotic ( ceftriaxone ) and intravenous acyclovir ( dose of 10 mg / kg body weight every 8 h ) for 6 days . gradual improvement in the condition of the patient was observed from 3 day after initiation of parenteral acyclovir . most neurological complications caused by vzv can occur in both primary and reactivated vzv although they seem to appear more frequently in herpes zoster than in vzv . both immunocompetent and immunocompromised patients may suffer from these neurological complications , but they appear to be more frequent and more severe in the latter group . the common neurological complications associated with herpes zoster are postherpetic neuralgia , myelitis , encephalitis , ventriculitis , aseptic meningitis , and white - matter disease . meningitis is a rare complication of vzv infection in a review of 859 patients with varicella - zoster infection ; meningitis was reported in only 0.5% within 60 days of diagnosis incidence increases with age including 2.5 cases/1000 in ages 2150 versus 10.1 cases/1000 in those older than 80 years . the incidence is increased in hiv - seropositive patients including 29.4 cases/1000 person - years as compared to the hiv - seronegative patients with 2.0 cases/1000 person - years . although the incidence of varicella and complications has decreased since the licensure of the vaccine , there may be breakthrough cases of varicella with aseptic meningitis and transient sensorineural hearing loss . patients with vzv meningitis may suffer from high fever , severe headache , cervical rigidity , seizure , ataxia , hemiplegia , and even coma ; these symptoms may appear within days after the appearance of the skin lesion and sometimes without skin lesions also . csf shows increased cell counts and elevated protein levels in the csf using sensitive laboratory analyses ( e.g. , pcr and detection of intrathecal production of specific antibodies ) , recent epidemiological studies found a portion of 529% of vzv in aseptic meningitis and encephalitis and it has been suspected that vzv infections had been underestimated in earlier publications . nevertheless , in immunocompetent patients without neurological deficits ( as in our case ) , vzv meningitis is rare . we , therefore , highlight the importance of considering vzv as a possible cause for meningitis even in previously healthy young patients and the recommended diagnostic lumbar puncture . detailed csf diagnostic procedures including pcr and detection of intrathecal synthesis of antiviral antibodies ( especially for vzv and herpes simplex viruses ) should be considered even though csf cell count and total protein seem to indicate a bacterial infection . since herpes zoster is a viral disease , conservative care or acyclovir administration is the routine treatment , and steroid therapy is employed as an optional addition . the administration of acyclovir within 4872 h of the appearance of zoster effectively relieves acute pain and the vesicles , induces a higher rate of remission , and deters the virus from spreading throughout the patient 's body , and they usually show a full recovery without developing other complications .
meningitis caused by varicella zoster virus ( vzv ) is quite rare among young immunocompetent adults though immunocompromised patients are often seen to be affected by reactivation of vzv presenting with primary clinical features of dermatomal rashes and neurological sequelae . here , we report the clinical scenario of a young , healthy male who had presented with fever , headache , and onset of dermatomal rashes later than the fever and was eventually diagnosed to be a case of vzv meningitis . we would like to highlight the fact that even young immunocompetent patients though rarely , might contract vzv meningitis and clinicians should have a high index of suspicion and keen eyes to catch the more obvious features of vzv infection on complete physical examination and must not harbor any reservations in ordering polymerase chain reaction for vzv dna or initiating aggressive antiviral therapy .
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the index case was a 14-year - old boy transferred from a regional hospital to our unit 24 h after a laparoscopic appendicetomy for perforated appendicitis . abdomen was distended and tender with purulent discharge coming from a peritoneal drain chest x ray demonstrated extensive right pleural effusion ( fig . 1 ) . fluid resuscitation and aggressive broad - spectrum antibiotics were commenced and a pleural drain was placed with improvement in his respiratory status . the intraperitoneal drain was inadvertently removed and some days later he became febrile again . abdominal computed tomography ( ct ) showed multiple locules in an extensive right - sided abdominal collection extending from the subhepatic space to the iliac fossa ( fig . non - operative management with intravenous antibiotics failed to resolve all of these and a laparotomy was performed with drainage of all except a small subhepatic collection . a large sump drain was left in - situ and , following cessation of drainage and a satisfactory clinical state , he was discharged . five days later , he re - presented with severe left pleuritic chest pain and dyspnoea . bilateral pleural effusions and a large high attenuation pericardial effusion were noted on imaging ( helical ct and echocardiography ) ( figs . a previously demonstrated small right subphrenic collection had increased in size . due to the size and possible infective nature of the pericardial effusion , the pericardial effusion was microscopically analysed revealing profuse polymorphs and profuse gram negative rods , gram positive cocci and gram positive rods . the culture of the effusion grew enteric gram negative rods and mixed anaerobes , of which streptococcus anginosus ( s. milleri ) was isolated . his clinical condition improved significantly and the drain was removed on the second post - operative day . pericardial effusions in adults are most commonly idiopathic , followed by infection and malignancy , , . rarer causes include radiation , uraemia and post - acute myocardial infarction . in paediatric cases , the leading causes are infective pericarditis , connective tissue disease , metabolic disorders and malignancies . we searched the ncbi pubmed and medline database for english literature from 1946 to present to determine the frequency and management of pericardial effusion as a complication arising from acute appendicitis . however , after reviewing the papers for their relevance to this case , only two case reports remained . reported a 3-year - old girl with a perinephric abscess extending from a ruptured appendix and subsequent empyema and pericardial effusion both treated by percutaneous drains . the subsequent purulent pericardial effusion was drained by retrosternal pericardiotomy with subsequent pericardiectomy required . up to 55 per cent of children with advanced appendicitis demonstrated the anatomical connections between the retroperitoneum and the mediastinum by depicting ectopic air from radiographs and suggested these connections as providing pathways for bidirectional spread of disease processes into the mediastinum and pleural cavities . the european society of cardiology guidelines recommend surgical drainage over percutaneous drainage in complicated purulent pericarditis . a case series from northern india reported 25 cases of pericardial effusions in children over three years . no cases of intraabdominal infections were found and nine cases were complicated by cardiac tamponade . echo - guided percutaneous drainage and pigtail catheter insertion were found to be safe and effective treatments . contrary to the guideline previously mentioned , this case series calls into question the need for surgical drainage in the treatment of complicated pericardial effusion . this case report and review has demonstrated the extreme rarity of clinically significant exudative pericardial effusion complicating ruptured appendicitis . given the low incidence , it is unlikely that consensus as to the ideal management ( percutaneous v pericardotomy ) will be reached .
introductionwhilst pericardial effusion is a known complication of abdominal pathology , it is rarely reported following ruptured appendicitis and even more rarely requires drainage in that situation . this work has been reported in line with the scare criteria ( agha et al . , 2016).presentation of casewe report a 14-year - old male who developed extensive right hepatorenal and right paracolic abscesses , bilateral pleural effusions and a large pericardial effusion following laparoscopic appendicectomy . due to the size of the effusion , thoracoscopic pericardotomy was required.discussionpericardial effusion is a very rare complication of advanced appendicitis despite a demonstrable connection between the retroperitoneum and the mediastinum . only two cases were reported in our literature search . there is no consensus as to whether percutaneous drainage or pericardiotomy is the treatment of choice.conclusionthe report is presented as a reminder of a rare complication of a common general surgical condition .
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oral contraceptive pills ( ocps ) are commonly used for contraception , polycystic ovarian syndrome , amenorrhea , menorrhagia , dysmenorrhea , endometriosis , etc . , common side effects of ocps include nausea , vomiting , headache , bloating , breast tenderness , swelling of the feet , weight gain , breakthrough bleeding , and venous thromboembolism . neurological adverse effects associated with ocps are migraine , depression , psychosis , and cerebral infarction . chorea caused by ocps is extremely rare and only very few cases are reported in the literature . the onset of chorea usually varies from few weeks to several years after starting therapy . here , we report a case of chorea developing within 1 week of initiating ocps in an adolescent girl with polycystic ovarian disease . a 17-year - old non - obese , unmarried girl presented to our hospital with irregular cycles and hirsutism for 2 years duration . investigations revealed raised lh : fsh ratio ( 3:1 ) and a slightly raised serum testosterone level . she was diagnosed to have polycystic ovarian disease and was prescribed ocps for treatment of irregular cycles . the ocps contained a combination of ethinyl estradiol 0.035 mg and cyproterone acetate 2 mg , which was prescribed once daily for 21 days . the patient presented with complaints of abnormal movements of right extremities within 1 week of starting therapy . there was no previous history suggestive of streptococcal infection , rheumatic fever or drug therapy . on examination , investigations revealed normal blood counts , erythrocyte sedimentation rate , serum electrolytes , renal , liver , and thyroid function tests . she was diagnosed to have ocps induced hemichorea after ruling out other common causes of chorea . neurological side effects of ocps have been linked to alterations in coagulation pathways and secondary vascular complications . chorea is an abnormal involuntary movement disorder characterized by brief , semi - directed , irregular movements that are not repetitive or rhythmic , but appear to flow from one muscle to the next . the first description of chorea associated with ocps was reported by fernando in 1966 and subsequently few similar published reports established ocps as a cause of chorea . no particular oral contraceptive preparation was consistently associated with chorea , and both low and high - dose preparations can cause this movement disorder . it has been well - established that estrogens have a definite but complex modulatory action in dopaminergic systems . hence , the estrogenic component of ocps is most likely the causative factor in the chorea induced by these agents . some cases of ocps induced chorea are caused by reactivation of sydenham 's chorea seen in rheumatic fever . however , ocps induced chorea have no clear relationship with sydenham 's chorea , or streptococcal infection have also been reported , similar to our case . the onset of chorea varies widely from few weeks to several years ( average duration : 9 weeks ) . the shortest time interval between ocp intake and development of chorea reported in the literature is eight weeks in the patient with no significant medical history . in most cases , symptoms subside promptly after withdrawing the offending drug , usually within a period of 23 months . in this index case , causality assessment by naranjo adverse drug reaction probability scale got a score of 6 , suggesting a probable relationship between the reaction and the drug . prior knowledge of this rare adverse effect and high index of suspicion can help in early diagnosis and discontinuation of therapy .
chorea is a neurological adverse effect of oral contraceptive pills ( ocps ) . the onset of chorea following ocps usage varies widely from few weeks to several years . we report a rare case of chorea which developed within a week of starting ocps in an adolescent girl with polycystic ovarian disease .
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single and double , fluorescent and non - fluorescent , in situ hybridization and immunohistochemical stainings were performed using standard protocols . to analyze the ltbp3 loss of function phenotype , we injected anti - sense ltbp3 morpholinos into one - cell stage wt and transgenic embryos . for genetic lineage tracing , a transgenic driver strain expressing cre recombinase in ltbp3 cells and four cre - responsive color switching the driver strain was crossed individually to each of the reporter strains and their double transgenic progenies were analyzed for zsyellow protein fluorescence using confocal microscopy . to follow the migration of zebrafish shf cells , a tracking dye was injected into the zsyellow , amcyan region of tg(nkx2.5::zsyello ) ; tg(cmlc2::csy ) embryos at 24hpf , the embryos were imaged immediately , and then again at 48 and/or 72hpf . a transgenic strain carrying a cdna encoding a constitutively active human tgf type i receptor ( caalk5 ) under control of the zebrafish heat shock promoter was generated and used to rescue the myocardial defect in ltbp3 morphant embryos . full methods and any associated references are available in the online version of the paper at www.natre.com/nature
the four - chambered mammalian heart develops from two fields of cardiac progenitor cells ( cpcs ) distinguished by their spatiotemporal patterns of differentiation and contributions to the definitive heart [ 13 ] . the first heart field differentiates earlier in lateral plate mesoderm , generates the linear heart tube and ultimately gives rise to the left ventricle . the second heart field ( shf ) differentiates later in pharyngeal mesoderm , elongates the heart tube , and gives rise to the outflow tract ( oft ) and much of the right ventricle . because hearts in lower vertebrates contain a rudimentary oft but not a right ventricle [ 4 ] , the existence and function of shf - like cells in these species has remained a topic of speculation [ 410 ] . here we provide direct evidence from cre / lox - mediated lineage tracing and loss of function studies in zebrafish , a lower vertebrate with a single ventricle , that latent - tgf binding protein 3 ( ltbp3 ) transcripts mark a field of cpcs with defining characteristics of the anterior shf in mammals . specifically , ltbp3 + cells differentiate in pharyngeal mesoderm after formation of the heart tube , elongate the heart tube at the outflow pole , and give rise to three cardiovascular lineages in the oft and myocardium in the distal ventricle . in addition to expressing ltbp3 , a protein that regulates the bioavailability of tgf ligands [ 11 ] , zebrafish shf cells co - express nkx2.5 , an evolutionarily conserved marker of cpcs in both fields [ 4 ] . embryos devoid of ltbp3 lack the same cardiac structures derived from ltbp3 + cells due to compromised progenitor proliferation . additionally , small - molecule inhibition of tgf signaling phenocopies the ltbp3-morphant phenotype whereas expression of a constitutively active tgf type i receptor rescues it . taken together , our findings uncover a requirement for ltbp3-tgf signaling during zebrafish shf development , a process that serves to enlarge the single ventricular chamber in this species .
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transurethral resection of bladder tumor ( turbt ) is the treatment of choice for non - muscle - invasive urothelial carcinoma . intraperitoneal perforation is a rare and serious complication of turbt , which can be complicated by increased leakage , systemic absorption of irrigation fluid and by bowel injury . intraperitoneal bladder perforation , although infrequent , is considered so serious that it requires immediate treatment . traditionally , intraperitoneal perforation has been managed with open surgical repair . minimal invasive management such as percutaneous peritoneal drainage , or laparoscopic repair of the bladder defect[24 ] have been described ; however , there are no large series or long - term follow - up studies . laparoscopic bladder repair was safely performed in an old patient as the following case illustrates . she underwent staging turbt after 4 weeks which again showed t1 high grade urothelial carcinoma . deep tumor resection was performed using a flat , equatorial loop to resect the posterior wall tangentially . during the resection which took 20 minutes this was recognized by cystoscopic visualization of the intraperitoneal cavity through a laceration in the middle of the resected area near the dome , while fulgurating the edges at the completion of the procedure . immediately , exploratory laparoscopy was performed by a colleague from the department of general surgery experienced in laparoscopic techniques showing a 20-mm rupture at the posterior wall of the bladder . three trocars were used , a 10 mm umbilical trocar and two 5 mm trocars , one in each iliac fossa . there was no injury to the bowel and the bladder was repaired with two absorbable 2 - 0 vicryl sutures using a one - layer full - thickness suture pattern . a percutaneous intraperitoneal 12f silicon drain was placed to monitor for possible urine extravasation post operation . for bladder drainage , five days after the operation a cystogram revealed no evidence of leak , and the catheter was removed . intraperitoneal bladder perforation is a rare but serious complication that deserves immediate treatment to prevent complications such as peritonitis , uremia , acidosis , hypervolemia due to irrigant fluid absorption , and tumor cells seeding in the peritoneum . bladder perforation is more likely to occur in elderly patients who have a thin bladder wall . the incidence of bladder perforation is difficult to assess because many perforations heal spontaneously and go unnoticed since they do not cause any perioperative and postoperative problems . therefore , bladder perforations following transurethral surgery may occur much more often than observed or reported in the literature . balbay et al . carefully evaluated this by performing a cystogram before and after each turbt , and found that 58% of the cases had some extravasation although the surgeon did not believe there was a perforation . some means to reduce the incidence of bladder perforation are to perform resections in elder patients with special care ( especially for tumors at the dome ) , to avoid overdistension of the bladder , to use the loop in a manner that follows the bladder contour , and to use low power settings on the diathermy unit . whereas in many patients with small extraperitoneal perforations , free bladder catheter drainage and careful observation is sufficient , such treatment is not enough in patients with significant intraperitoneal perforations . the traditional treatment has been a formal laparotomy with drainage of the intraperitoneal fluid , repair of the perforated area , exclusion of small bowel injury , and placement of intraperitoneal drains . as demonstrated in this case and by others,[24 ] advantages over open repair are obvious , including less hospitalization and avoidance of the morbidity associated with open surgery . in our opinion , simple bladder drainage might be insufficient , considering that it does not allow any control of the fluid extravasating into the peritoneal cavity during the postoperative period . continuous or intermittent bladder irrigation can be necessary in these cases to deal with bleeding caused by incomplete tumor resection and insufficient hemostasis . on the other hand , percutaneous placement of a peritoneal drainage tube might put the patient at risk of bowel perforation or injury to abdominal wall vessels . moreover , it avoids open surgery and the limits and risks of the standard conservative approaches . although diagnostic laparoscopy can be performed with either local or regional anesthesia , therapeutic laparoscopic procedures are generally performed using inhalation anesthesia and controlled ventilation . next , consideration must be given to the special equipment and adequate expertise needed for these procedures . as demonstrated in this case , the procedure can be performed in cooperation with any surgeon experienced in laparoscopic techniques . according to a consensus statement from 2004 on bladder injuries , ureters or bladder neck a further major concern of bladder perforation during turbt is the possibility of tumor cell dissemination . unrecognized bladder cancer seeding after resection may alter the natural course of the disease process . existing literature supports that bladder perforation during transurethral resection has a slight but possible chance of extravesical recurrence even for a low stage tumor . noted that of the 11 patients who had bladder perforations during turbt , only 1 had extravesical recurrence . indicated that open procedures to close the bladder were associated with an increased risk of extravesical recurrence and this negatively impacted patient prognosis . their statement was based on an extensive review of more than 3,400 patients and 34 instances of bladder perforation . of course , dissemination alone does not result in metastases , which require target tissue that are receptive and support angiogenesis . laparoscopic repair should be considered in case of inadvertent intraperitoneal bladder perforation during transurethral surgery . it can be safely performed and offers an excellent modality for repair of this rare complication even in old patients .
an intraperitoneal bladder perforation occurred during transurethral tumor resection under general anesthesia in a 82 year old woman . the bladder was repaired with a laparoscopic closure and an indwelling urethral catheter . the histopathology revealed t1 high grade urothelial carcinoma . the patient recovered well and was discharged home on postoperative day 7 . this case highlights the successful use of laparoscopy in the treatment of a rare urological complication .
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granular cell tumors ( gct ) are uncommon soft tissue neoplasms that may be asymptomatic or may present as a slow growing nodule . we present a case of granular cell tumor in the right inguinal region misdiagnosed as a reactive lymph node on cytology . a 63-year - old male presented with swelling in the right inguinal region for the past 10 years with a sudden increase in size since two months . on examination , a single , firm three cm swelling was felt in the right inguinal region . no other lesion such as an ulcer or swelling was noted in the right leg or thigh . there was no history of fever , rigors and loss of weight or appetite in the recent past . an ultrasonogram of the right inguinal mass showed circumscribed hypoechoic mass suggestive of a lymph node shadow . cytosmears examined were moderately cellular with dual cell population , composed predominantly of oval to polygonal cells with abundant cytoplasm and round bland nuclei resembling histiocytes [ figure 1 ] . the background showed few discrete round cells with round nuclei and scanty cytoplasm suggestive of mature lymphocytes [ figure 1 ] . no precursor lymphoid cells were seen on cytosmears . in correlation with clinical and image findings , an impression of reactive lymph node a clinical follow - up and excision was advised if clinically suspicious or lesion further increased in size . ( a ) moderately cellular cytosmear showing clusters of cells with histioid appearance , lymphoid cells and few bare nuclei ( mgg , 100 ) ; ( b ) cytological smear with loose clusters of oval to polygonal cells having basophilic granular cytoplasm ( mgg , 200 ) an excision biopsy was performed which showed skin with a neoplasm located in lower dermis and subcutis . individual cells appeared round to oval with abundant granular eosinophilic cytoplasm and centrally placed bland nuclei [ figure 2 ] . immunohistochemistry showed s100 positivity in nucleus and cytoplasm ( [ figure 2 ] inset ) . section show diffusely arranged tumor cells with granular eosinophilic cytoplasm intervening adnexal structures and presence of lymphoid aggregate ( h and e , 100 ) ; inset show tumor cells showing cytoplasmic and nuclear immunopositivity for s100 protein ( ihc , 100 ) granular cell tumor is a rare benign tumor arising predominantly in skin , subcutaneous tissues and tongue . they may occur at any age , but commonly affects third to fourth decades of life . generally , these are isolated lesions , but can be multiple in about 15% of cases . usually , these tumors behave in a benign fashion , but malignant variant can also occur de novo or from transformation of a benign one . earlier gct was proposed to be derived from immature skeletal muscle cells ( hence , the term granular cell myoblastoma was given ) . however , later ultrastructure and immunohistochemistry studies have proven it to be of schwannian differentiation . granular cell tumors show non - specific features on mri and may only be useful in assessing precise location of tumors which is essential for preoperative evaluation . in our case study , only an ultrasonogram was done . in general , cytological smears of gct show moderate to high cellularity with uniform appearing large cells arranged in syncitium and as isolated cells . cytoplasmic granules are positive for periodic acid schiff stain and resistant to diastase . rarely , intranuclear inclusion has also been reported . the granularity in cytoplasm of tumor cells is due to accumulation of secondary lysosomes resulting in its typical appearance on microscopy and thus deriving its name . in our case , due to the presence of lymphoid cells , the lesion was mistaken for a lymph node and the granular cells were incorrectly identified as histiocytes on cytology . since it was a long - standing inguinal swelling with mild increase in size for the last two months on reviewing the cytosmears , the larger cells appearing as histiocytes were definitely very granular and this is not a feature of sinus histiocytes . misdiagnosis , in our case , was due to several reasons , foremost being the site of occurrence , clinical and radiological opinion of enlarged lymph node and finally misinterpretation of cytosmears . cytological smears in our case showed mature lymphocytes and histopathology of the excised tissue showed features of granular cell tumor with many lymphoid aggregates . this finding suggests that lymphocytes can sometimes feature in cytological smears of gct and can confuse the pathologist . another feature on cytology that can further confound is the occurrence of stripped nuclei from tumor cells of gct resembling nucleus of lymphocyte . granular cell tumor can rarely occur within a lymph node and it is important to know this fact to avoid a mistaken diagnosis of a metastatic tumor . cell block is a useful technique that provides additional information from cytological specimen as it displays microhistology which can be further utilized for immunohistochemical studies . in the present case , our case further emphasizes that cell blocks should be considered in image guided aspiration as the material obtained in such cases is through a specialized invasive technique and a repeat aspiration would be cumbersome task . we present this case to create awareness among the cytologists regarding the difficulties encountered in the cytodiagnosis of granular cell tumor . the possibility of granular cell tumor should be considered when viewing single and cluster of large cells with abundant eosinophilic granular cytoplasm and vesicular nuclei on cytology .
a 63-year - old male presented with sudden increase in size of a right inguinal swelling which was present for the past 10 years . clinical diagnosis of inguinal soft tissue mass / lymph node enlargement was made and patient was investigated further for a conclusive diagnosis . ultrasound examination suggested it to be a lymph node and guided fine needle aspiration cytology was performed . cytology was suggestive of a reactive lymph node . subsequently , an excision biopsy was performed that revealed a granular cell tumor with many lymphoid aggregates . on reviewing the cytosmears , we realised that the granular neoplastic cells were mistaken for histiocytes ; and the lymphoid background and bare nuclei mislead us to consider it a reactive lymph node . since granular cell tumor can occur almost anywhere , a careful examination of cytosmears and knowledge of its distinctive cyto - morphological features would be helpful in providing an accurate diagnosis on cytology .
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most ingested foreign bodies pass through the gastrointestinal tract spontaneously without necessitating any treatment ; however , about 20% require endoscopic or surgical removal1 ) . most swallowed toothbrushes have been found in the esophagus or the stomach of the affected patient , and there has been no previously reported case of a toothbrush in the colon . here , we report a case of a swallowed toothbrush found in the ascending colon with a complicating small hepatic abscess , which was successfully managed with laparotomy . a 31-year - old man presented with a one - week history of intermittent right upper abdominal pain . he saw his local doctor when symptoms began , and was prescribed ranitidine and antacid for dyspepsia with no improvement . he had been treated for 13 years due to a diagnosis of schizophrenia . on examination , , his blood pressure was 110/70 mmhg , and he was not jaundiced or anemic . the coagulation profile was abnormal with a prothrombin time ( international normalized ratio ) of 1.4 and an activated partial thromboplastin time of 40 s. an electrocardiogram was unremarkable . an abdominal radiograph showed parallel rows of short metallic radiodensities in the right mid - abdomen ( figure 1 ) . on ct images , a metallic foreign body was observed in the right colon and a thrombosis of the left portal vein was found to be associated with perfusion changes in the left hepatic lobe , suggesting a chronic intra - abdominal inflammation ( figure 2 ) . colonoscopic examination revealed a toothbrush penetrating the wall of the hepatic flexure with the head pointing toward the ascending colon ( figure 3 ) . we attempted to remove the toothbrush after grasping it with a snare ; this attempt failed due to limited space in the hepatic flexure and risk of trauma to extraluminal organs or vessels . the patient was subsequently taken to the operating room for an exploratory laparotomy . during laparotomy , the toothbrush was found to have caused a fistula between the hepatic flexure of the colon and the liver with a complicating small hepatic abscess . the toothbrush at 19 cm in length was removed , the perforation in the colon was resected in a small wedge , and anastomosis was performed . the peritoneal cavity was irrigated with normal saline , and a 10-fr drain tube was placed in the abscess cavity site . upon further questioning , the patient reported ingesting a toothbrush approximately one year earlier . at that time the toothbrush is a rather unusual foreign body to be found in the gastrointestinal tract . in a recent report , a medline search of the years 1988 to 2000 found 11 articles with approximately 40 cases of toothbrush ingestion2 ) . almost all of the patients were female , ranging from 15 to 23 years of age3 , 4 ) . most of the patients had been diagnosed with psychiatric problems such as bulimia or anorexia nervosa . the characteristic radiographic image of a swallowed toothbrush shows parallel rows of short metallic radiodensities due to the metallic plates that hold the bristles in place4 ) . the toothbrushes were easily identified in the esophagus and stomach of the patients by upper endoscopy . in several cases , there were significant complications related to pressure necrosis , including gastritis , mucosal tears , and perforation3 ) . consequently , prompt removal is recommended to prevent the development of complications . in 1983 , other cases have reported failure of endoscopic removal due to the size or shape of the toothbrush6 , 7 ) . in addition , cases of esophageal perforation during endoscopic toothbrush extraction8 ) and of laparoscopic - assisted toothbrush extraction via gastrostomy after an unsuccessful attempt at endoscopic removal7 ) have been reported . however , surgical removal is often required due to the geometric qualities of the toothbrush . all of the swallowed toothbrushes were found in the esophagus or the stomach of the affected patient , and there has been no previously reported case of a toothbrush in the colon . to our knowledge , this is the first documented case of a swallowed toothbrush found in the colon and persisting in the gastrointestinal tract for approximately one year . for a small foreign body penetrating the wall of the colon with no sign of peritoneal irritation , it may be possible to remove the foreign body and repair the wound endoscopically . in our case , we found the endoscopic approach unsuccessful due to the size of the toothbrush and the narrow space of the hepatic flexure . there was also a complicating abscess and a risk of trauma to extraluminal organs or vessels during endoscopic removal . the case presented demonstrates a very unusual complication of toothbrush ingestion , unlike any that have been reported previously . thus , a typical finding of an unusual foreign body in the colon , especially in patients with psychiatric problems , should make the attending physician consider the possibility of a swallowed toothbrush . because no cases of spontaneous passage have been reported , prompt removal
toothbrush swallowing is a rare event . because no cases of spontaneous passage have been reported , prompt removal is recommended to prevent the development of complications . most swallowed toothbrushes have been found in the esophagus or the stomach of affected patients , and there has been no previously reported case of a toothbrush in the colon . here , we report a case of a swallowed toothbrush found in the ascending colon that caused a fistula between the right colon and the liver , with a complicating small hepatic abscess . this patient was successfully managed using exploratory laparotomy . to our knowledge , this is the first documented case of a swallowed toothbrush found in the colon .
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lichen myxedematosus ( lm ) is a primary mucinosis that is not accompanied by thyroid disease . in the updated classification of lm given by rongioletti et al . [ 1 , 2 ] , the nodular type is one subset of lm that is characterized by multiple nodules on the limbs and trunk with mild or absent papular eruption . herein , we report a case of nodular - type lm for which local injection of corticosteroids was effective . an 18-year - old japanese woman noticed a progressive appearance of nodules on both forearms and on the left cubital fossa and left thigh one year before her initial consultation at our department for further evaluation in 2008 . physical examination revealed elastic hard , slightly elevated , shiny and yellowish to skin - colored nodules of 6 to 10 mm in size on her extremities without confluence into plaque . 1 ) , three on the left forearm , four on the left cubital fossa , and two on the left thigh . the nodules were not itchy . laboratory findings for liver function , free t3 , free t4 , thyroid - stimulating hormone , fasting blood sugar , and hba1c were all within normal limits . xanthoma and dermatofibroma were suspected based on the clinical appearance , and one nodule on the right forearm was taken for skin biopsy . histopathologically , collagen fibers were separated in the reticular to deep dermis without epidermal changes ( fig . basophilic mucin deposits between collagen fibers with proliferation of fibroblasts and infiltration of lymphocytes and plasmacytes around vessels were seen in the dermis . histochemically , the mucin was positive for alcian blue and negative after hyaluronidase digestion ( fig treatment with betamethasone dipropionate ointment was ineffective , but one nodule was completely eliminated by local injection of 0.02 ml triamcinolone acetonide ( 8 mg / ml ) . since triamcinolone acetonide was subsequently recalled and was unavailable for a period , we tried local injections of 0.1 mg prednisolone sodium succinate three times for each nodule . after triamcinolone acetonide reappeared in the japanese market , local injection of 0.02 to 0.05 ml triamcinolone acetonide was performed and another three injected nodules were completely eliminated . lm ( papular mucinosis ) is a disorder characterized by lichenoid papules , nodules and/or plaques due to dermal mucin deposition , and a variable degree of fibrosis without thyroid dysfunction . in an updated classification [ 1 , 2 ] , lm is divided into the following clinicopathologic subsets : ( 1 ) a generalized papular and sclerodermoid form ( scleromyxedema ) with monoclonal gammopathy and systemic manifestations that may be lethal . histologically , the skin shows a microscopic triad of mucin deposition , fibroblast proliferation , and fibrosis ; ( 2 ) localized lm , which is a papular or nodular / plaque eruption without sclerotic features , monoclonal gammopathy , or systemic involvement ; here , the skin shows mucin deposition with variable fibroblast proliferation ; and ( 3 ) atypical forms , which have atypical features or features intermediate between scleromyxedema and localized lm . localized lm is further subdivided into the following four subtypes : ( 1 ) discrete lm , which has firm , smooth , waxy or flesh - colored papules of 2 to 5 mm in size , numbering a few to hundreds on the limbs and trunk in a symmetric pattern . the papules are isolated or form confluent nodules or plaque and rarely resolve spontaneously ; ( 2 ) acral persistent papular mucinosis , which is characterized by a few to multiple , ivory to flesh - colored papules of 2 to 5 mm in size on the back of the hands , wrists , and occasionally the distal aspect of the forearms . papules persist or increase without spontaneous resolution and occur predominantly in females ; ( 3 ) cutaneous mucinosis of infancy , characterized by firm , opalescent papules located on the upper arms , especially the elbows , and the trunk without spontaneous resolution , and ( 4 ) nodular - type lm , characterized by multiple nodules on the limbs and trunk , with a mild or absent papular component . our case was diagnosed as nodular - type lm based on the clinical and histological features . the patient had 8 nodules of 6 to 10 mm in size on the legs and forearms without a symmetric pattern , whereas papules on the limbs and trunk more commonly occur in a symmetric pattern in discrete lm , and occur on the back of the hands and extensor surface of the distal forearms in acral persistent papular mucinosis . histological findings , such as mucin deposition with variable fibroblast proliferation and the absence of monoclonal gammopathy and m protein , provided further reasons for the diagnosis of nodular - type lm . only three previous cases [ 4 , 5 ] characterized by nodular - type lm have been reported , including one from japan . the mean onset age of the four reported patients with nodular - type lm , including our case , was 30.5 years ( range 1848 years ) , which suggests that this subtype appears at a younger age compared to discrete lm and acral persistent papular mucinosis . moreover , three of the four cases were male ( table 1 ) . a standard treatment for localized lm is not described in the literature , but in our case nodules showed complete remission following local injection of triamcinolone acetonide , which suggests that this drug may be of some benefit for this condition .
an 18-year - old japanese woman noticed a progressive appearance of nodules on both forearms and on the left cubital fossa and left thigh one year before her initial consultation at our department . physical examination showed elastic hard , slightly elevated , shiny and yellowish to skin - colored nodules of 6 to 10 mm in size on her extremities . from laboratory and histopathological findings , nodular - type lichen myxedematosus was diagnosed and nodules showed complete remission following local injection of triamcinolone acetonide .
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a 69-year - old man was referred due to newly developed hemoptysis and was diagnosed as operable adenocarcinoma of the right upper lobe of the lung . because the tumor was invading the right main bronchus and azygos vein , however , from the 6th postoperative day , he developed tachypnea , desaturation ( sao2 85% to 90% under room air ) , and atrial fibrillation with rapid ventricle response around 150 beat per minute . despite increased oxygen supply , desaturation gradually progressed , and the patient was intubated for mechanical ventilation . after initiating positive mechanical ventilation , severe subcutaneous emphysema developed . bronchoscopic examination revealed a bronchopleural fistula ( bpf ) on the right main bronchus stump ( fig . exudates in the right pleural cavity were evacuated by closed thoracostomy , and microrganisms such as streptococcus pneumonia and pseudomonas aeruginosa were isolated from the pleural fluid . after 5 days of conservative treatment with antibiotics and pleural cavity drainage , surgery was decided to obliterate bpf because the onset of empyema was relatively acute and stump fistula was too large to expect spontaneous closure . after redo thoracotomy through previous incision , primary closure of bpf was performed by multiple interrupted sutures along with transposition of omental flap to the bronchial stump ( fig . after thorough debridement and irrigation of pleural cavity , the right pleural cavity was packed with sterilized gauze and pericostal sutures were applied to approximate the intercostal space . vacuum - assisted closure ( vac ) sponge ( curavac ; daewoong , seoul , korea ) was applied just above the rib leaving the pleural cavity communicated through intercostal space . a 12 french chest tube was placed into the vac sponge to apply negative pressure . the skin above the vac sponge was widely covered using adhesive drape and negative pressure of 120 mmhg was applied for exudates evacuation . three additional vac dressing changes were performed under general anesthesia on every 3 days after bpf closure . after confirming that no more organisms from the right pleural cavity were isolated by follow - up microbial culture , clagett operation was performed on 9th postoperative days from bpf closure and thoracotomy wound could be closed without additional vac apply . the patient remains asymptomatic without any evidence of infection or wound problem for 7 months follow - up . postpneumonectomy empyema ( ppe ) is a life - threatening complication , and is often related with a bpf . treatment of ppe should be targeted on closure of bpf , effective drainage of pleura cavity , and obliteration of the dead space . one of the most popular approaches to treat ppe with bpf is the closure of bronchial stump followed by irrigation of the pleural space through open window thoracotomy . delayed closure is performed with dead space filled by antibiotics solution ( so - called clagett operation ) . although this approach has been shown to be highly successful , it forces the patient long hospitalization during the sterilization and stressful daily irrigation with dressing change . , novel technique using vac has been widely used for various wound infections , such as deep sternal wound infection and open orthopedic fractures . vac device promotes wound healing by generating negative pressure which leads to granulation tissue formation , removal of exudates , and reduction of edema with increased tissue perfusion by arteriolar dilatation . we borrowed this advantage of vac for the sterilization of pneumonectomy space instead of daily irrigation with dressing change through open thoracotomy . in this patient , peribronchial tissue was fresh enough for early surgical repair because the detection of ppe with bpf was relatively earlier . therefore we thought early sterilization with vac was possible , if we could obliterate the bpf with omental flap transposition . before we decided to conduct this procedure , one of our main concerns was complication induced by negative suction pressure , such as bleeding , disruption of repaired fistula , or mediastinal shifting . in this case , we packed sterilized gauze into pleural cavity to avoid direct contact of negative pressure to mediastinum ( fig . exudates were evacuated by the vac system through communication along the intercostal space . by these methods stated above , we could control the ppe effectively for relatively short period , less cost and trouble for patient . although vac dressing in ppe with bpf is not widely used method , it could be an effective alternative method for the open window sterilization with shorter treatment period in selected patients .
postpneumonectomy empyema is a life - threatening complication , which is often related with a bronchopleural fistula . after surgical repair of fistula , sterilization of infected pleural cavity is important and usually carried out by long - term cyclic irrigation . we report a case in which vacuum - assisted closure device was successfully applied to sterilize the pleural cavity and obliterate bronchopleural fistula .
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pulmonary sling is a vascular abnormality wherein the left pulmonary artery arises from the right pulmonary artery and then traverses between the esophagus and the trachea toward the hilum of the left lung . it was first described by glaevecke and doehle in 1897 . since then , about 100 cases have been reported in the world . the average age at the onset of symptoms , such as respiratory distress and cyanosis , is 2 months and about half have symptoms from birth . so , adult cases are very rare , are most often asymptomatic and usually found incidentally . when combined with complete cartilaginous tracheal rings in the absence of posterior membranous portion , it is called ring - sling complex . in that case , we here report an adult case who was diagnosed as pulmonary sling with complete tracheal ring by ct and bronchoscopy along with a literature review . she had had symptoms of airway obstruction , such as wheezing , hoarseness and mild dyspnea since a young infant . blood pressure was 140/90 mmhg , pulse rate was 80/min , body temperature was 36.5c and respiration rate was 16/min . on physical examination , she was alert , but had a slight dyspneic appearance . there were no cyanosis , chest deformities and cardiac murmurs . fev1 was 1.52l ( 66.2% of predicted value ) and fvc was 1.90l ( 54.5% of predicted value ) on pulmonary function test . complete cartilaginous ring without posterior membranous portion was found on the entire trachea ( fig . 1 ) . the plain chest x - ray showed a right mediastinal mass - like density and a long - segment tracheal narrowing ( fig . 2 ) . the chest ct revealed the anomalous left pulmonary artery originating from the right pulmonary artery and its posterior course between the trachea and the esophagus ( fig . she was discharged without specific treatment because of mild symptoms which are mainly caused by complete tracheal ring and not by tracheal compression due to vascular anomaly . the term vascular ring was introduced to describe the mediastinal vascular anomalies causing tracheobronchial compression by dr . the pulmonary sling is a kind of vascular ring and the term was first used by contro et al . in 1958 to distinguish it from other vascular anomalies . carl et al . reported that when vascular rings were classified into four groups , as follows , 1 ) complete vascular rings with double aortic arch or right aortic arch 2 ) pulmonary sling 3 ) innominate artery compression and 4 ) miscellaneous , pulmonary sling was about 4.5% . embryologically , it has been suggested that it is caused by a maldevelpment of the left ventral pulmonary artery bud due to growth failure or reabsorption . the left lung may capture its arterial supply by connection of the left postbronchial plexus with the right sixth arch through capillaries caudal to the lung bud . then , as the lung bud grows caudally , the course of the left pulmonary artery is behind the developing trachea - bronchial tree . about half have cardiac or tracheo - bronchial anomalies which determine the prognosis . according to christoph dohleman s review , the associated cardiac anomalies were 49% and among those , patent ductus arteriosus , ventricular septal defect and atrial septal defect were most common . tracheal stenosis with complete tracheal rings was the most common anomaly , followed by stenosis of main stem bronchus , tracheal bronchus and tracheo - esophageal fistula . wells et al . proposed a classification system assigning pulmonary sling without complete tracheal rings to type 1 and pulmonary sling with complete tracheal ring to type 2 . ring - sling complex can be classified into a lethal form and a form in which there is enough pars membranacea left to allow sufficient tracheal growth . the two forms can be distinguished only by their clinical course , i.e. , either severe respiratory symptoms with progression or mild symptoms without progression . bronchoscopy and echocardiography should be performed to evaluate the combined cardiac or tracheo - bronchial anomalies . the plain radiographic features include unequal aeration due to compression of the right main bronchus by the anomalous vessel , low carina , horizontal equal - length right and left mainstem bronchi and long - segment tracheal stenosis . diagnostic considerations such as dilated azygous vein , lymphadenopathy , foregut cyst or esophageal neoplasm should be excluded . most cases can be diagnosed by ct or mri , but misinterpretation can occur because of a bifurcation - like structure of the pulmonary artery . the left pulmonary artery can be missed because of its different level of crossing the carina or main bronchus . was the first to propose the now most common corrective surgical procedure : division and reimplantation of the anomalous left pulmonary artery into the pulmonary trunk anterior to the trachea with division of the ligamentum arteriosum . because of high operation mortality and loss of function of the left lung due to obstruction of the reconstructed vessel , a conservative approach with symptomatic management was recommended initially . but recent development of operation technique has resulted in low operation mortality and good patencty of the reconstructed vessel . so , in view of the seriousness of this condition and the fact that reports of surgical management have been encouraging , current policy is to offer an operation as soon as the diagnosis is made .
pulmonary sling is a rare congenital condition in which the left pulmonary artery arises from the right pulmonary artery forming a sling around the trachea causing tracheal compression . many cases are associated with cardiovascular and tracheo - bronchial anomalies . when combined with complete tracheal ring , it is called ring - sling complex . the average onset of symptoms is 2 months and about half have symptoms from birth . the anomaly , if not corrected , can be fatal within the first year of life . in an adult , it is most often asymptomatic and usually found incidentally . we report an adult case who was diagnosed as pulmonary sling with complete tracheal ring by ct and bronchoscopy along with a literature review .
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raised intracranial pressure ( icp ) after traumatic brain injury ( tbi ) is common and associated with increased risk for death and disability . despite decades of animal and human research , successful prevention and treatment of this deadly complication largely eludes the medical community . because of the considerable heterogeneity and severity of the disease , well designed , prospective , randomized studies in neurotrauma are rare . all academic attempts to generate reliable trial data are noteworthy . in this context , we enthusiastically applaud prez - brcena and colleagues for their thoughtful and ambitious research . the study evaluates the use of two barbiturates , pentobarbital and thiopental , in the treatment of refractory intracranial hypertension after tbi . forty - four patients were randomly assigned to receive pentobarbital or thiopental after first - level measures had failed to control icp . in the pentobarbital and thiopental groups , icp was controlled in 18% and 50% of patients , respectively , without any statistically significant difference between groups in the rate of infectious complications or hemodynamic compromise . glasgow outcome scale ( gos ) scores at 6 months revealed a poor neurologic outcome ( death , vegetative state , and severe disability ) in 17 and 12 patients in the pentobarbital and thiopental groups , respectively . no statistical analysis of gos scores was reported , but the study was not powered to detect a difference in outcome . specifically , cranial computed tomography revealed bilateral brain swelling in significantly more patients in the pentobarbital group , and more patients in the thiopental group had evacuated lesions or no swelling at all . in addition , the doses of barbiturate were not equivalent between groups . to compensate for this , the authors attempted to ensure equivalent potency by titrating the dose to electrographic burst suppression ( ebs ) or flat pattern . however , the number of patients in each group that reached ebs was not reported ; therefore , it is unclear whether bioequivalent doses were actually achieved between groups . previous research has shown that the serum concentration at which ebs is reached varies between patients , and serum and cerebrospinal fluid levels correlate poorly with ebs . therefore , ebs is not necessarily an accurate surrogate for barbiturate dose . given that thiopental is more lipophilic than pentobarbital and was infused at a higher initial maintenance rate , it is possible that the thiopental group maintained a higher cerebral concentration of barbiturate . despite these potential confounders , the relevance of this trial can not be overstated . in the current age of multimodality monitoring , individualized treatment paradigms , and combination therapy , these data have important implications and bring to the forefront a number of questions . what is the appropriate goal of barbiturate therapy icp control , ebs , neuroprotection , or a combination of these ? experimental models of ischemia demonstrate that anesthetic doses of barbiturates provide no additional attenuation of brain free fatty acid release than subanesthetic doses . in models of focal infarction , animals that were anesthetized with pentobarbital dosed to preserve an active electroencephalogram had equivalent reductions in infarct volume as those anesthetized to ebs . barbiturates lessen the release of s-100b , excitatory neurotoxins , and amino acid markers of energy failure , but it has not been proven that ebs is a requirement for these neurochemical changes . these data cast doubt on the argument that patients benefit maximally from barbiturate protocols that include ebs as a therapeutic target , especially given the frequent adverse effects of hypotension and immune dysfunction . nevertheless , several studies convincingly demonstrate that barbiturates can treat elevated icp , particularly in patients who are refractory to other management strategies . if barbiturates can lower icp and provide neuroprotection even without ebs , then why has an association with good outcomes not been realized in clinical trials ? perhaps by the time barbiturates are employed for refractory icp often several days after the trauma alternatively , it is plausible that only specific pathophysiologic and cerebral hemodynamic profiles will respond to barbiturate treatment . prez - brcena and coworkers reported that the association of focal lesions with icp control was 3.6 times higher than that for diffuse lesions . improved pressure reactivity indices and cerebral tissue oxygen tension in response to barbiturates , even in the presence of continued elevations in icp , these findings raise the possibility that in select patients barbiturates have the ability to prevent localized ischemia , reverse dysautoregulation , and improve cerebral oxygenation . finally , barbiturates may indeed provide an outcome benefit , but were not superior to other drugs including opiates , mannitol , hypertonic saline , and benzodiazepines that were used in controls . despite insufficient evidence that either pentobarbital or thiopental improves outcomes after tbi , or that one drug is better than the other early combination therapy of barbiturates with hypothermia or progesterone , in concert with multimodality invasive neuromonitoring , holds promise in the treatment of this devastating condition [ 11 - 14 ] . ebs : electrographic burst suppression ; gos : glasgow outcome scale ; icp : intracranial pressure ; tbi : traumatic brain injury .
in their article on the use of barbiturates for the treatment of intracranial hypertension after traumatic brain injury , perez - barcena and colleagues conclude that thiopental was more effective than pentobarbital in decreasing intracranial pressure . here we discuss the limitations of this study and review areas of controversy surrounding barbiturate use in neurocritical care .
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cyclosporine is a lipophilic cyclic peptide of 11 amino acids , while tacrolimus is a macrolide antibiotic . both drugs have been isolated from fungi and possess similar suppressive effects on cell mediated and humoral immune responses . patients treated with the calcineurin inhibitors are at high risk of developing renal injury1 ) . although renal insufficiency induced by calcineurin inhibitors has received the most attention , tubular dysfunctions are also clinically important and will be briefly reviewed in this article . calcineurin inhibitor - associated tubular dysfunction is manifested by metabolic acidosis , hyperkalemia , calcium , phosphate wasting , and magnesium loss . the first case report with post - transplant renal tubular acidosis was described by massry et al . renal tubular acidosis ( rta ) is non - anion gap metabolic acidosis and is generally mild and a symptomatic in kidney recipients3 ) . the reported prevalence of calcineurin inhibitor - associated rta is 13 - 17% intransplanted patients4 - 6 ) . the former form of acidosis is characterized by bicarbonate wasting due to the toxic effects of calcineurin inhibitors . in contrast , distal or type iv rta is characterized by the inability to excrete hydrogen ions6 ) . use of calcineurin inhibitor cyclosporine can frequently cause type 4 rta , a mild hyperchloremic acidosis , sometimes with elevated potassium . this may be reflecting decreased aldosterone activity and suppression of ammonium excretion by hyperkalemia7 ) . there are some reports now that provide some insight as to how that might occur . collecting ducts have 2 types of intercalated cells - the acid or hydrogen ion - secreting alpha - intercalated cells and the bicarbonate - secreting beta - intercalated cells ( fig . 1 ) . the preponderance of which cell dominates rests on the type of diet . an acid diet leads to more alpha - intercalated cells , whereas an alkaline diet leads to more expression of beta - intercalated cells . it has been reported that protein hensin is actually important in mediating transformation between beta - and alpha - intercalated cells . deposition of hensin leads to the conversion of bicarbonate - secreting beta - intercalated cells into the acid - secreting alpha - intercalated cell . fk506 and consequently acid - secreting cells will be less abundant and risk for amild normal anion gap metabolic acidosis will increase8 , 9)(fig . treatment of calcineurin inhibitor - associated acidosis is mainly with oral supplement of bicarbonate4 , 12 ) . synthetic mineralo corticoid is apotential treatment option , but has more frequent side effects10 ) . an elevation in plasma potassium concentration due to reduced efficiency of urinary potassium excretion is common in calcineurin inhibitor - treated patients . it may be severe and potentially life - threatening with concurrent administration of an angiotensin converting enzyme inhibitor or angiotensin receptor blocker . cyclosporine may reduce potassium excretion by altering the function of several transporters , decreasing the activity of the renin - angiotensin - aldosterone system , and impairing tubular responsiveness to aldosterone15 , 16 ) . renal potassium excretion is primarily derived from potassium secretion in the distal cortical nephron via potassium channels in the luminal membrane . this process is stimulated by sodium reabsorption , aldosterone , and the basolateral na , k - atpase pump . in vitro studies suggest that cyclosporine may directly impair the function of the potassium secreting cells in the cortical collecting tubule by affecting each of these steps : reduced activity of the na , k - atpase pump17 - 19 ) , inhibition of the luminal potassium channel20 ) , and increased chloride reabsorption16 ) . cyclosporine increases paracellular or transcellular chloride reabsorption , which prevents generation of lumen - negative potential that drives potassium secretion . calcineurin inhibitors inhibit luminal potassium channels and increase chloride reabsorption via alteration of wnk kinases21)(fig . cyclosporine may also have a secondary effect on potassium homeostasis in patients concurrently treated with a beta - blocker . in this setting , there is often a modest and transient elevation in the plasma potassium concentration due to movement of potassium out of cells into the extracellular fluid22 ) . treatment is similar to that of hyperkalemia in chronic kidney disease : reduction in potassium intake , adjustment in medications , and so forth . one exception occurs in the early posttransplant period , in which renal insufficiency also contributes to the impairment in potassium excretion . administration of a cation exchange resin increases the risk of intestinal necrosis at this time23 ) . cyclosporine inhibits the type iia sodium phosphate cotransporter and probably the type iib sodium phosphate cotransporter of the intestine24 ) . post - transplant hypophophatemia is usually asymptomatic , but can rarely be severe enough to cause severe muscle weakness , including weakness of respiratory muscle . several case reports suggest that hypomagnesemia may contribute to calcineurin inhibitor - induced encephalopathy25 - 27 ) . hypomagnesemia has also been implicated as a contributor to the nephrotoxicity associated with cyclosporine28 ) . animal studies suggest that hypercalciuria induced by cyclosporine administration is associated with an inhibition of calbindin d28k expression , resulting in calcium transport defect in the distal tubule29 ) . in conclusion , attention must also be paid to the calcineurin inhibitor associated renal tubular dysfunction such as renal magnesium wasting , calcium and phosphate wasting , distal tubular acidosis , and impaired renal potassium excretion , in addition to the well known side effects , such as tubulointerstitial fibrosis and glomerular or vascular damage .
nephrotoxicity is the most common and clinically significant adverse effect of calcineurin inhibitors . cyclosporine and tacrolimus nephrotoxicity is manifested by both acute azotemia and chronic progressive renal disease and tubular zdysfunction . an elevation in the plasma potassium concentration due to reduced efficiency of urinary potassium excretion is common in cyclosporine - treated patients ; it may be severe and potentially life - threatening with concurrent administration of an angiotensin converting enzyme inhibitor , which diminishes aldosterone release . tubular injury induced by cyclosporine can also impair acid excretion . this may be presented as a hyperchloremic metabolic acidosis associated with decreased aldosterone activity and suppression of ammonium excretion by hyperkalemia . some patients treated with cyclosporine develop hypophosphatemia due to urinary phosphate wasting . renal magnesium wasting is also common presumably due to drug effects on magnesium reabsorption . hypomagnesemia has also been implicated as a contributor to the nephrotoxicity associated with cyclosporine . both cyclosporine and tacrolimus are associated with hypercalciuria . attention must be paid to drug dose , side effects , and drug interactions to minimize toxicity and maximize efficacy .
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they are caused by distinct viruses that belong to different families ( varicella - zoster virus , a dna virus belonging to the family herpesviridae ; morbilli virus , an rna virus belonging to the family paramyxoviridae ) . owing to sustained high vaccination coverage with two doses of measles vaccine , at the age of one and seven years , measles are virtually eliminated in croatia . in the last decade only sporadic imported cases of measles are reported , with the exception of two small outbreaks , which occured in 2004 and 2008 following importation of measles . since there is no universal chickenpox vaccination in croatia and the number of vaccinated persons upon request is very low , chickenpox is a common childhood disease with an annual incidence of 2530 cases per 1000 children under 15 years of age . the first symptoms of illness ( fever and rash on the trunk and extremities ) appeared during their stay in italy . upon arrival to croatia , on 5 july , two of the children ( 15 and eight years of age ) were admitted to hospital with suspected measles and chickenpox . both children presented with fever up to 39c , dry cough and confluent macular rash wich was most prominent on the face and neck . the 15-year - old daughter had prominent retroauricular confluent rash and koplik spots on the buccal mucosa . five days later , on 10 july an 18-year - old boy from the same family , presented at the infectology clinic with fever up to 39c and dense confluent macular rash , with numerous scabs on the face and entire body . an epidemiological investigation revealed that he had chickenpox with disease onset at the time of their stay in italy about 15 june . the epidemiological investigation focused on ten children from the same family . in six children ( including the two hospitalized girls ) blood samples were taken on 14 july and sent to the refence laboratory at the croatian national institute of public health ( cniph ) for measles and varicella - zoster serology testing . based on clinical manifestations and results of serology testing , four of these six children had simultaneous infection with measles and varicella and two children had chickenpox . four siblings who had no symptoms and had no history of measles immunization were vaccinated with a monovalent measles vaccine following the investigation . serologic tests were performed at who national measles laboratory , department of virology ( cniph ) . specific igm and igg antibodies to measles and varicella - zoster virus ( vzv ) were detected using commercial enzyme - linked immunosorbent assay ( virotech , russelsheim , germany ) . vzv igg positive samples were tested for igg avidity ( euroimmun , lubeck , germany ) . serologic results are presented in table 1 . in two patients ( patiens 1 and 2 ) varicella infection two patients ( patients 3 and 4 ) had positive vzv igm antibodies with low igg avidity . for two patients ( patients 5 and 6 ) , paired samples were obtained three weeks later . in both patients , varicella infection was confirmed by low igg avidity in the second sample ( table 1 ) . table 1serologic results in six patients with suspected measles and varicella infection.patientagesexmeasles virusvaricella - zoster virusigmiggigmiggigg avidity1i11fnegativenegativepositivepositivelow2i12mnegativenegativepositivepositivelow3i15fpositivenegativenegativepositivelow4i8fpositivenegativepositivepositivelow5i18mpositivenegativepositiveequivocal - ii*positivepositivepositivepositivelow6i12fpositivepositivenegativenegative - ii*positivepositivepositiveequivocallow*paired serum samples ( obtained three weeks later ) . paired serum samples ( obtained three weeks later ) . the epidemiological investigation revealed that none of the ten children were vaccinated against measles ( which is mandatory according to the childhood immunization programme in croatia ) or varicella ( which is not mandatory ) . the fact that none of the children were vaccinated represents a public health issue in a relatively small migrant population and indicates that vaccination coverage may be low in this population . simultaneous infection and clinical illnesses of a viral and bacterial etiology is a well known fact , as well as co - infections but not simultaneous illness caused by two distinct viruses . however , clinical disease caused by two viruses at the same time is known to be a feature of only a few viruses , notably hiv , hepatitis b and hepatitis c viruses . our literature search revealed only a few case report of children who had measles and chickenpox at the same time . all these cases occurred at the time when incidences of both measles and chickenpox were high in the populations . due to very high incidence rates of chickenpox and still high incidence of measles in countries with low vaccination coverage , one would expect numerous cases of simultaneous disease in the absence of some kind of immunological interference . the rarity of reports on simultaneous measles and chickenpox illness indicates that recent infection with one virus results in suppression of clinically manifest disease caused by another virus if a person acquires the other infection soon afterward . the simultaneous illness described in our report is quite unexpected because measles is virtually eliminated in croatia and in earlier decades , when measles was a common childhood disease , we have not identified cases of simultaneous clinical illness caused by measles and chickenpox .
we are reporting on a household outbreak of measles , in which cases of simultaneous measles and chickenpox infection occured in children of a family who resided in italy during the incubation period ( june 2011 ) . in three children , fever and generalized confluent macular rash were the dominant symptoms . serology testing revealed simultaneous measles and chickenpox infection in four children .
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focal gingival enlargements are quite frequent lesions in the oral cavity amounting to almost 3.1% of all oral tumors and for 9.6% of gingival lesions . in 1992 , the world health organization classification about this kind of focal overgrowth had tapered toward one universal term peripheral cemento - ossifying fibroma . we , in our case report prefer to call it as peripheral ossifying fibroma ( pof ) . pofs are more frequent in younger ages , peak being in the second decade , although there are cases occurring in older age groups also there is uncertainty for diagnosing focal reactive overgrowths of the gingiva because of their nearly same clinical presentation . the typical appearance of pof is small gingival growth ( epulis ) initially which can attain large sizes so as to cause facial disfigurement . if the lesions grow to larger sizes , there can be a mild erosion of bone . the treatment of pof is straight forward with surgical excision with blade and scalpel commonly followed . a newer approach is the use of diode lasers for its removal , probably having an advantage of lesser bleeding and predicted results . whatever may be the method of removal , it should be sent for histopathological diagnosis . here a 60 year old female presented to us with a chief complaint of swelling on the right maxillary posterior region of jaw since 3 - 4 years . she also gave a history of the slow growth of the mass since last 3-years to reach the present size . the oral examination showed the presence of a solitary pedunculated mass of size 4 cm 2 cm 3 cm which was red in color with lobulated surface on the right maxillary posterior alveolar ridge . on palpation , the mass was firm , nontender , and not fixed to underlying structure . orthopantomogram ( opg ) evaluation showed the presence of a unilocular radiolucent area extending superiorly into the corresponding maxillary sinus , posteriorly to maxillary tuberosity , and anteriorly until the distal end of canine . dento - alveolar scan did not show the extension of mass into maxillary sinus , rather showed the inflamed lining of the sinus . a three dimensional construction image done after dento - alveolar scan confirmed the calcifications [ figure 2c ] within the mass which was seen as radio - opaque areas in opg . after clinical and radiographic investigations pof , peripheral giant cell granuloma ( pgcg ) and pyogenic granuloma ( pg ) ( longstanding ) were considered in differential diagnosis . the clinical presentation of intraoral mass ( a ) orthopantomogram showing the extension of mass . ( b ) dento - alveolar scan showing the extension of lesion and inflamed maxillary sinus lining . ( c ) three - dimensional construction showing calcifications within the mass a comprehensive explanation was given to the patient and sign was taken on the consent form . excisional biopsy was carried out , and the tissue was sent for histopathological examination for confirmatory diagnosis . macroscopically , the gross specimen was measuring 3.5 cm 2 cm 1 cm , creamish brown in color , firm to hard in consistency with lobulated , and pebbly surface . the specimen was cut into two halves , and most representative areas were taken for processing [ figure 3 ] . the complete gross specimen the specimen was fixed in phosphate - buffered neutral formalin for 1 day , subsequently , five - micron paraffin sections were obtained and stained with hematoxylin and eosin ( h and e ) . microscopic analysis of the h and e section showed parakeratinized stratified squamous epithelium covering loosely arranged highly cellular connective tissue stroma [ figure 4a ] . few vascular spaces of varied sizes and inflammatory infiltrate were also seen in underlying connective tissue . based on these histologic features , ( a ) photomicrography showing parakeratinized stratified squamous epithelium covering loosely arranged highly cellular connective tissue stroma . pof is clinically present as a soft nodular mass which may be either pedunculated or sessile present near the interdental papilla . pof histopathologically is described as a lesion which has a fibrous stroma in which there is presence of mineralized tissues such as bone and/or cementum - like . however , this term is discarded as clinical and histopathological presentation is the same in those cases where there is no cementum . some authors have reported the presence of odontogenic epithelium and also that the proliferating cells may be of myofibroblastic origin . some researchers feel that it represents that some kind of chronic irritation is responsible for stimulation of cells in the periodontal ligament . the purpose of reporting this case was to give a brief review to the surgeon that there can be a number of lesions presenting with the same picture but have varying histopathological presentation . it is always better to be aware of the fact and plan the surgery accordingly . pofs are common in the mandibular region and in the second decade of life usually . irrespective of the type of mineralized component , the treatment of choice is surgical excision and there are very few cases of recurrence reported .
focal gingival enlargements are a diverse group of lesions with almost similar clinical presentation but varied etiology and histopathology . the actual cause of peripheral ossifying fibroma ( pof ) has been debated over many years . pofs are usually seen in childhood and younger ages . there is a slight female predilection . it is usually localized , rarely generalized . usually , the treatment option is only surgical excision with minimal chances of recurrence . here , we report a case of pof of the right maxillary posterior region with a review of literature .
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members of high - risk families should be given recommendations which may improve prophylaxis , early diagnosis and treatment [ 1 - 4 ] . during the last 10 years now it is possible to identify several genes involved in the hereditary forms of some types of cancers including breast cancer . hereditary forms of breast cancer are mostly caused by mutations in such genes as brca1 and brca2 and are thought to account for 5 - 10% of all cases of breast cancer . because of high penetration of mutated genes as well as autosomal dominant trait , female family members are frequently affected with breast cancer , therefore taking the family history is a major , effective and cheap way of diagnosing hereditary forms of breast cancer . primary tumour growth is different than sporadic ones , and it seems that early detection of hereditary forms may be more difficult . affected patients with the hereditary form of breast cancer are also at increased risk of the development of the second primary breast cancer . the purpose of this work was to evaluate in lithuanian breast cancer patients such clinical factors as : frequency of diagnosis of primary breast cancer in clinical stage i - iv and frequency of the second primary breast cancer in groups with a hereditary form . in order to identify patients suspected for hereditary breast cancer specific pedigree criteria in 2001 in vilnius university institute of oncology there were 521 patients treated for breast cancer . patients suspected for site specific hereditary breast cancer were identified according to the following pedigree criteria : mother and daughter affected by breast cancer ; one of them developed breast cancer under the age of 50 years . every case of hereditary breast cancer was analysed retrospectively by stage according to tnm classification and compared with the control group which was selected from sporadic cases diagnosed in the same cancer centre and matched for the same age of diagnosis . the relative risk was used as an estimate of the relative effectiveness for the recruitment cases by stage . relative risk was calculated by stage as a ratio at each category to the total number of observation . the frequency of the development of the second primary tumour in the hereditary breast cancer group was calculated . in a group of 521 patients treated with breast cancer in vilnius university institute of oncology in 2001 , there were 26 patients from 25 families with family history indicating diagnosis of site specific hereditary breast cancer . all these patients were affected with primary breast cancer under the age of 50 years , which is in concordance with the early age of onset observed in patients with positive breast cancer family history . we evaluated the frequency of breast cancers diagnosed in clinical stages i - iv in hereditary cancer patients . to avoid possible differences in diagnostic effectiveness between diagnostic centres in lithuania , a control group was selected from sporadic breast cancer cases diagnosed in the same cancer centre and matched for the same age of diagnosis . comparison of breast cancer frequencies diagnosed in clinical stages i - iv is shown in table 1 . we observed much lower frequency of hereditary cases diagnosed in stage i than sporadic ones ( 11% vs. 23% , rr = 0.49 ) . in contrast hereditary breast cancer cases were diagnosed more often than sporadic tumours , in advanced stages ( stage ii in 61% vs. 44% , rr = 1.39 ; and stage iii in 27% vs. 17% , rr = 1.62 , respectively ) . we have not observed any hereditary breast cancer patient diagnosed in clinical stage iv , which can be caused by the low number of studied cases . the low rate of hereditary cancers diagnosed in stage i and high rate of stage ii and iii may suggest that growth of these tumours may be accelerated and standard surveillance is less effective than in a group of patients without family history . similarly to other observations , cancer family history is correlated with an increased risk of contralateral breast cancer . in our group of 26 patients , experience of many foreign hereditary cancer centres proved that hereditary cancer patients as well as their family members must be followed in an institution prepared for biological genetic testing and specialist surveillance . a better understanding of the process of coupling in healthy people with a genetic risk of cancer including genetic consultations is relevant here to make a decision about prevention or screening [ 7 - 11 ] . breast cancer in a study group and in a population by cancer stage * ) matched by age and territory with the study group , 2000 the results of this study show the need to create a lithuanian specialist centre for hereditary cancer diagnosis , treatment , and prophylaxis , which will coordinate and connect work between different specialists . this study was supported by a grant of ec project qlri - ct-1999 - 00063 " development of network of cancer family syndrome registries in eastern europe " .
breast cancer family history has been known to be one of the main cancer risk factors . members of high - risk families should be given recommendations which may improve prophylaxis , early diagnosis and treatment . detection of high - risk families is possible by identification of mutations in cancer susceptibility genes like brca1 and brca2 as well as by family history showing breast and/or ovary cancer aggregation . in a group of 521 breast cancer patients we identified 26 patients with hereditary breast cancer who fulfilled the following criteria : one more relative with breast cancer , vertical transmission , at least one breast cancer patient affected at the age under 50 years . 8 patients of these developed second primary breast cancer . we also compared the frequency of hereditary cancers in stage i - iii with the frequency of respective cancers with negative family history . hereditary breast cancers were diagnosed less frequently in stage i and more frequently in stage ii and iii ( rr = 0.49 , rr = 1.39 , rr = 1.62 , respectively ) . because of importance of family history as well as genetic testing for breast cancer susceptibility genes ( brca1/2 ) , it is necessary to create a nationwide network of hereditary cancer clinics for proper diagnosis , treatment , and prophylaxis of these patients .
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duchenne muscular dystrophy ( dmd ) is an inherited neuromuscular disorder which affects boys . until recently the mean age of death was around 19 but there have been significant improvements in clinical management and they can now expect to live to around 25 years . to investigate , from their own perspectives , how the well - being of young men living with dmd , and that of their families , can be maximised , particularly at the transition to adulthood , and from children 's to adult services . it was an example of inclusive research , involving young men and a national family support group , as well as clinical services . postal survey of family carers in three regions followed by interviews with 40 young men age 15 + with dmd and their families about the issues they faced at transition to adulthood . family carers generally considered that transition planning at school leaving was poor or non - existent and that they lacked information about services . once they had left school or college , the majority of young men lacked meaningful day time activities and friendship networks . much effort has gone into producing policies and processes , with an uncritical assumption that this will lead to better outcomes . the study ( 20072009 ) was funded by the english department of health 's long - term neurological conditions research programme .
introductionduchenne muscular dystrophy ( dmd ) is an inherited neuromuscular disorder which affects boys . until recently the mean age of death was around 19 but there have been significant improvements in clinical management and they can now expect to live to around 25 years.purposeto investigate , from their own perspectives , how the well - being of young men living with dmd , and that of their families , can be maximised , particularly at the transition to adulthood , and from children 's to adult services . theoretically , it drew on the social model of disability and recent critiques . it was an example of inclusive research , involving young men and a national family support group , as well as clinical services.methodspostal survey of family carers in three regions followed by interviews with 40 young men age 15 + with dmd and their families about the issues they faced at transition to adulthood.findingsfamily carers generally considered that transition planning at school leaving was poor or non - existent and that they lacked information about services . once they had left school or college , the majority of young men lacked meaningful day time activities and friendship networks . there was good continuity of health care in one of the three regions.conclusionmuch effort has gone into producing policies and processes , with an uncritical assumption that this will lead to better outcomes.sponsorshipthe study ( 20072009 ) was funded by the english department of health 's long - term neurological conditions research programme .
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penile cancer is rare in developed countries , whereas in india and other asian countries the incidence is decreasing . the incidence of squamous cell carcinoma ( scc ) of penis varies worldwide with age , circumcision , and hygiene practices . phimosis , lack of neonatal circumcision , human papillomavirus ( hpv ) infection , penile lichen sclerosis , exposure to tobacco products , and psoralen ultraviolet a ( puva ) are the known risk factors for development of scc of penis . up to 50% of patients with scc of penis delay seeking medical attention by more than a year due to embarrassment , guilt , fear , ignorance , and personal neglect . the level of denial is substantial , given that the penis is observed and handled on a daily basis . a 60-year - old unmarried male , presented with a history of multiple ulcers over his right groin of 4 months duration . it started as a painless swelling over the right groin , which evolved into multiple ulcers with foul swelling discharge . . he does not give history of arthralgia , loss of weight , or loss of appetite . the patient also gave history of multiple blisters over the trunk and extremities of 1 month duration . there was no history of oral erosions or drug intake prior to the onset of lesions . on general examination , the patient was moderately built and nourished , there was no pallor , icterus , pedal edema , or generalized lymphadenopathy . examination of the right inguinal region showed three tender excavating ulcers of sizes 6 8 , 5 7 , and 3 5 cm , respectively , located adjacent to each other with rolled out , everted edges , and foul smelling purulent , blood - stained discharge . enlarged , grouped , and matted tender inguinal lymphnodes of sizes 5 7 cm were felt below the ulcer . examination of the left inguinal region also revealed enlarged , grouped , tender inguinal nodes of size 8 6 cm present both above and below the inguinal ligament giving the appearance of groove sign of greenblatt [ figure 1 ] . right groin showing multiple excavating ulcers , left groin shows groove sign of greenblatt and multiple tense bullae with erosions over thighs and penile shaft genital examination revealed phimosis . a firm , indurated , non tender mass of size 3 2 cm was felt through the prepucial skin on the glans penis , located on the 11o - clock to 2 o - clock position . multiple tense bullae filled with clear fluid and erosions were present over upper limbs , lower limbs [ figure 1 ] , and trunk . there were no oral erosions . with the above clinical findings , a differential diagnosis of lymphogranuloma venereum ( lgv ) or scc of penis with regional metastasis associated with bullous pemphigoid ( bp ) occurring as a para neoplastic phenomenon were considered . igg antibody for chlamydia trachomatis ( serovars l1 , l2 , l3 ) was negative . vdrl for syphilis and elisa for human immunodeficiency virus ( hiv ) 1 and 2 were nonreactive . wedge biopsy of the ulcer in the right groin and from the penile growth showed ulcerated hyperplastic epidermis with severe dysplasia . the dysplastic epithelial cells invade the basement membrane forming nests with keratin pearls , consistent with keratinizing scc of penis [ figure 2 ] . skin biopsy taken from a tense bulla over the trunk , showed a sub epidermal bulla with mild lymphocytic infiltrate in the upper dermis consistent with bp . the dysplastic epithelial cells are seen to invade the basement membrane hence , a diagnosis of keratinizing scc of penis with regional lymphnode metastasis and bp was confirmed . scc of penis may present either as a flat growth , infiltrating , or papillary growth . the lesion is not visible in most patients since the prepuce is non - retractile . the inguinal nodes increase in size followed by erosion of the skin over the nodes . it starts as a small transient , inconspicuous lesion on the genitalia and spreads to regional lymphnodes resulting in bubo and suppurative lymphadenopathy . in our patient , scc of penis was masquerading as lgv since he presented with history of inguinal bubo , which later ulcerated . moreover , the primary scc was not noticed by the patient and was not visible during examination since the prepuce was non- retractile . a biopsy from the groin ulcer and penile growth that was felt on palpation had confirmed the diagnosis . the occurrence of bp in this patient can be attributed as a paraneoplastic phenomenon to scc of penis . pemphigoid has been reported in numerous patients with malignancies , like breast cancer , b - cell lymphoma , lung cancer , gastric cancer , cancers of colon or rectum , endometrial cancer , and many others . in scc , this could lead to production of antibodies directed against these antigens that results in the development of bp in these patients . to the best of our knowledge this is the first case of scc of penis with bp occurring as a paraneoplastic phenomenon . we present this case to highlight that scc of penis can masquerade as lgv and a high index of suspicion is required for an early diagnosis of scc penis .
we report a case of a 60-year old unmarried male who presented with multiple ulcers and foul smelling discharge from the groin since 4 months and multiple tense bullae over the trunk of 1 month duration . groove sign was present . investigations for lymphogranuloma venereum ( lgv ) and other sexually transmitted diseases were negative . histopathology from the ulcer in the groin and growth in the penis revealed squamous cell carcinoma ( scc ) . skin biopsy of bulla was diagnostic of bullous pemphigoid ( bp ) . we report a rare case of scc masquerading as lgv with bp occurring as a paraneoplastic phenomenon .
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the hypothesis that a systemic or a regional reduction of sympathetic activity for example , induced by thoracic epidural anesthesia might have positive effects on the perfusion and oxygenation ( that is , increase them ) of splanchnic organs like the liver and gut and that reduction of pain improves pulmonary function sounds profound . although interest in this field of research has been increasing over the past years , detailed knowledge about the effects of increased or reduced sympathetic activity on organ perfusion and oxygenation and the mechanisms involved , as well as how these change or sympathetic activity changes immunomodulation during pathophysiological conditions , is still lacking . in recent issues of critical care , freise and colleagues and lauer and colleagues presented studies that provide interesting information concerning this subject . sprague - dawley rats that were fitted with thoracic epidural catheters and treated with cecal ligation and puncture . intravital microscopy was used to investigate sinusoidal diameters , loss of sinusoidal perfusion , sinusoidal blood flow , and permanent leukocyte adhesion to sinusoidal and venolar endothelium . in their experiments , cardiac output measured in an additional group of animals , which were not investigated with intravital microscopy remained constant in animals with induced sepsis with and without epidural anesthesia . from their intravital microscopy results they concluded that sinusoidal blood flow increased in the sepsis group and was normalized in the group with sepsis and thoracic epidural anesthesia however , sinusoidal vasoconstriction was not ameliorated by thoracic epidural anesthesia and nor was liver tissue injury affected . while there is broad agreement that thoracic epidural anesthesia improves postoperative pulmonary function , the underlying mechanisms for example , via reduction of abdominal pain after general abdominal surgery still remain unclear . thoracic epidural anesthesia modulated the nitric oxide ( no ) pathway and exerted positive that is , lower levels of exhaled no effects on pulmonary endothelial integrity in hyperdynamic septic rats , but not in hypodynamic septic rats . in the latter , this study shows the importance of distinguishing between different phases of disease , especially during early ( hyperdynamic ) and late ( hypodynamic ) sepsis . one has to keep in mind that the authors did not describe any differences in volume management within their experimental groups and , thus , intravascular normovolemia could not be proven in either . in general , both studies add interesting results to the necessary discussion about the usefulness of epidural anesthesia during sepsis . why is this so ? increased sympathetic activity plays an important role in the development of different pathophysiological conditions for example , during endotoxemia [ 4 - 7 ] , hemorrhagic shock and even during and after routine abdominal surgical procedures . thus , epidural anesthesia might decrease mortality during sepsis , especially as splanchnic hypoperfusion and hypoxia are said to be key factors in the development of systemic inflammatory response syndrome , sepsis and multiple organ failure . diverse studies , however , have presented contradictory results concerning this , with some reporting decreased mortality in older animal studies and newer meta - analyses and others reporting increased mortality in an animal model . the main problem with all the published studies is that hardly any are comparable with each other . humans and different animals ( for example , pigs , rats , mice , rabbits ) have been used , either systemic or regional reduction of sympathetic activity has been investigated ( effects of clonidine , spinal anesthesia , epidural anesthesia ) , and the method of epidural anesthesia has differed , from lumbar epidural anesthesia in older studies to thoracic epidural anesthesia in recent studies , including or not the nervi accelerantes . however , to prove an effective reduction in sympathetic activity , and especially that the epidural anesthetic is working , is very difficult , especially in clinical situations . hence , the most important considerations for future studies on the effect of epidural anesthesia on sepsis or endotoxemia are normovolaemia at any point of the experiment , a clear definition and timeline of hypodynamic and hyperdynamic circulation in sepsis , the proven spread of the epidural anesthesia , which includes or excludes the nervi accelerantes ( thereby reducing or maintaining cardiac output , respectively ) , and the continuous , proven reduction of sympathetic activity including or excluding the adrenal glands during the different phases of the developing pathophysiological conditions . surrogate parameters like sinusoidal width or the number of perfused sinusoids should be used with care to judge sinusoidal perfusion , as laboratory findings should be treated cautiously if not accompanied by definitive and relevant physiological changes . although studies like those from freise and colleagues and lauer and colleagues have increased our understanding of how reduction of regional sympathetic activity can influence different organ functions during sepsis , we still largely lack understanding of the underlying mechanisms , and this will persist as long as there are no standardized , or at least fairly definitive , studies on reduced sympathetic activity during sepsis . only with these studies
research interest in epidural anesthesia during sepsis has grown over the past years and studies have tried to determine its mechanisms , which should , theoretically , protect organs and reduce morbidity and mortality . however , different experimental approaches in different animal models have provided conflicting results over whether epidural anesthesia has protective or harmful effects and whether these alter depending on the phase of sepsis , the spread of epidural anesthesia or additional supportive therapies . in the future , more standardized research is necessary to integrate the results of all studies , which have been published .
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tuberculosis persists as a common illness in developing countries , and western countries are experiencing a resurgence due to rising numbers of patients with acquired immunodeficiency syndrome1 , 2 ) . intra - abdominal tuberculosis , including peritonitis , enterocolitis , and mesenteric lymphadenitis , is relatively rare among extra - intestinal tuberculosis , although it is of considerable clinical importance3 - 6 ) . in korea , although the exact incidence of intra - abdominal tuberculosis is unknown , tuberculous enterocolitis has been reported to account for 4.8% of all tuberculosis , and therefore , intra - abdominal tuberculosis is clinically relevant in korea . intra - abdominal tuberculosis can present atypical clinicopathological features , which can make a proper diagnosis difficult8 - 10 ) . we present here an unusual case of solitary intra - abdominal tuberculous lymphadenopathy mimicking duodenal gastrointestinal stromal tumor ( gist ) . a 22-year - old woman presented with epigastric discomfort and intermittent dark - colored stools of two months duration . she had no previous illness and denied constitutional symptoms such as fever , weight loss , or night sweating . laboratory studies showed a hemoglobin of 13.9 g / dl , leukocytes of 7,580/mm , and an esr of 17 mm / hr . other biochemical tests results were within normal ranges , and anti - hiv ab was negative . an esophagogastroduodenoscopic ( egd ) examination revealed a round mass of about 3-cm at the duodenal bulb , with normal surrounding mucosa and central ulceration ( figure 1 ) . chest x - ray films showed inactive pulmonary tuberculosis at the left upper lobe , and abdominal computed tomography ( ct ) revealed a well - defined round mass that enhanced as gastric mucosa , at the posterior wall of the duodenal bulb suggestive of duodenal gist ( figure 2 ) . endoscopic ultrasonography ( eus ) showed an ulcerative hypoechogenic mass at the submucosal layer of the duodenal bulb ( figure 3 ) . exploratory laparotomy was performed to exclude the possibility of malignant gist of the duodenum . during laparotomy , a solitary mass was detected around the hepatic artery , which penetrated the duodenal bulb . the mass was excised and a histopathological analysis revealed lymphadenopathy with caseous granuloma ( figure 4 ) . polymerase chain reaction of dna extracted from the node was positive for tuberculosis and acid - fast bacilli were found by ziehl - neelsen tissue staining . the patient was diagnosed with solitary tuberculous lymphadenopathy and discharged on anti - tuberculosis medication . abdominal mesenteric lymphadenopathy is a relatively common manifestation of intra - abdominal tuberculosis , which occurs as a regional component of primary or secondary disease . involved mesenteric nodes may form independent masses or they may adhere to adjacent structures . tuberculosis usually affects multiple lymph node groups simultaneously , though isolated retroperitoneal lymphadenopathy is uncommon11 , 12 ) . most tuberculous lymphadenopathies are visualized by abdominal ct as enlarged nodes with hypodense centers and peripherally hyperdense enhancing rims11 ) as conglomerate mixed density nodal masses , or as enlarged masses with homogeneous density12 ) . in the present case , an exophytic oval mass was noted on an abdominal ct scan on the posterior wall of the duodenal bulb . moreover , it presented as a submucosal mass with central ulceration and suspected bleeding foci on egd examination . most cases of abdominal tuberculosis are complications of pulmonary tuberculosis , and thus in this patient with evidence of inactive pulmonary tuberculosis , a diagnosis of solitary mesenteric tuberculous lymphadenopathy with penetration into the duodenal wall appeared unlikely , and therefore , we suspected that the lesion was a duodenal gist . gists are generally located in the stomach ; only 4% of cases are reported in the duodenum . however , despite their relative infrequency small bowel gists frequently manifest as gastrointestinal bleeding , abdominal pain , or as a palpable mass13 , 14 ) , and can display unpredictable malignant behavior notwithstanding their small size . duodenal gists can thus have a poorer prognosis than gists of the stomach15 , 16 ) . because it is difficult to rule out duodenal gist and because of the likelihood of bleeding or malignancy laparotomy revealed a solitary mesenteric lymphadenopathy penetrating the duodenal bulb , and histopathologic analysis revealed it to be a tuberculous lymphadenopathy . eus - guided fine - needle aspiration ( fna ) has recently emerged as an important modality for diagnosing gist17 ) . moreover , ultrasound - guided fna offers a safe method for obtaining proof of tuberculosis in patients with suspected intra - abdominal tuberculosis18 , 19 ) . it is regrettable that we did not perform fna prior to laparotomy in the present case . the preferred treatment of intra - abdominal tuberculosis is anti - tuberculous medication ; surgical management is normally reserved for complications or diagnostic uncertainty . in conclusion , intra - abdominal tuberculosis can present a variety of conditions and in areas with a high prevalence the possibility of tuberculosis must be considered . tuberculosis is curable when a diagnosis is made sufficiently early and appropriate treatment is started .
tuberculosis remains prevalent in developing countries and has recently re - emerged in the western world . intra - abdominal tuberculosis can mimic a variety of other abdominal disorders , and here we describe a patient with solitary tuberculous mesenteric lymphadenopathy mimicking duodenal gastrointestinal stromal tumor ( gist ) . a 22-year - old woman complained of epigastric discomfort and was presumed to have a duodenal gist after an endoscopic examination and abdominal ct scan . however , exploratory laparotomy revealed an enlarged node penetrating the duodenal bulb , which was diagnosed histopathologically as tuberculous lymphadenitis . this case suggests that in regions with a high prevalence of tuberculosis , intra - abdominal tuberculosis is often mistaken as a malignant neoplasm . a high index of suspicion and the accurate nonsurgical diagnosis of intra - abdominal tuberculosis continues to be a challenge .
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this article is based on previously conducted studies and does not involve any new studies of human or animal subjects performed by any of the authors . data sharing is not applicable to this article as no datasets were generated or analyzed during the current study . this article is distributed under the terms of the creative commons attribution - noncommercial 4.0 international license ( http://creativecommons.org/licenses/by-nc/4.0/ ) , which permits any noncommercial use , distribution , and reproduction in any medium , provided you give appropriate credit to the original author(s ) and the source , provide a link to the creative commons license , and indicate if changes were made .
diabetic retinopathy ( dr ) is a common microvascular complication of diabetes mellitus ( dm ) and is considered as the leading cause of visual impairment in working - aged adults worldwide . dyslipidemia has been associated with dr , but not with progression to the proliferative form of dr , although the exact role in the pathogenesis of dr and diabetic macular edema ( dme ) remains controversial . as a result , a reasonable question arising is whether control of dyslipidemia may alter the course of dr . statins do not appear to have an impact on dr progression . on the other hand , fenofibrate has been found to significantly reduce the rate of progression of dr in patients with pre - existing mild dr , although it has no impact on patient s vision nor on the prevention of dr development in patients with type 2 dm without dr . an interesting point that needs further evaluation is why patients without dr or those with severe dr appear to have no benefit from fenofibrate treatment .
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ot-2 t cells ( 510/well ) were incubated for 30 min at 37c in 96-well u - bottom plates with dcs ( 10/well ) or lps - activated b cells ( 210 per well ) that had been pulsed with antigen . conjugates were enumerated by flow cytometry after the cell mixture was stained at 4c for cd4 , cd11c , and cd19 and repeatedly washed . to visualize t - dc interactions ( fig . 1 ) , ova323-pulsed dcs were injected subcutaneously at 210 per mouse 24 hours prior to intravenous transfer of sap and sap t cells ( 310 each ) . imaging was conducted 12 to 24 hours later . to examine activation phenotypes , 10 dcs and 210 gfp - expressing t cells per mouse 2 ) , 310 ot-2 t cells of each genotype were co - transferred into mice together with 510 wild - type b cells . immunization was done 12 hours prior to cell transfer , and imaging was conducted 24 to 36 hours thereafter . to visualize t - b interactions under non - competitive conditions and to assay t and b cell expansion ( fig . 3 ) , 610 gfp - expressing ot-2 t cells were co - transferred together with 310 cfp - expressing b cells 24 hours prior to immunization . imaging and cytometric analyses were conducted 6072 and 96 hours later , respectively . to visualize gc recruitment and retention of t cells ( fig . 4 ) , 310 cfp - expressing sap and 310 gfp - expressing sap ot-2 t cells were co - transferred with 310 non - fluorescent md4 b cells . dye - labelled nave b cells ( 2410 ) were given 1 day before imaging to provide follicle / gc landmarks . longer than 2 hours , the animal s hydration was maintained by lactated ringer s solution given via a catheter . the typical x - y - z dimension was 0.51.10.51.13 m , and the time resolution was 3045s . for experiments involving co - transfer of two types of dye - labelled t cells , the cells were always reciprocally labelled to control for potential dye - induced behavioural differences . the mann - whitney rank sum test was used to calculate p values for highly skewed distributions . for gaussian - like distributions , ot-2 t cells ( 510/well ) were incubated for 30 min at 37c in 96-well u - bottom plates with dcs ( 10/well ) or lps - activated b cells ( 210 per well ) that had been pulsed with antigen . conjugates were enumerated by flow cytometry after the cell mixture was stained at 4c for cd4 , cd11c , and cd19 and repeatedly washed . 1 ) , ova323-pulsed dcs were injected subcutaneously at 210 per mouse 24 hours prior to intravenous transfer of sap and sap t cells ( 310 each ) . imaging was conducted 12 to 24 hours later . to examine activation phenotypes , 10 dcs and 210 gfp - expressing t cells per mouse 2 ) , 310 ot-2 t cells of each genotype were co - transferred into mice together with 510 wild - type b cells . immunization was done 12 hours prior to cell transfer , and imaging was conducted 24 to 36 hours thereafter . to visualize t - b interactions under non - competitive conditions and to assay t and b cell expansion ( fig . 3 ) , 610 gfp - expressing ot-2 t cells were co - transferred together with 310 cfp - expressing b cells 24 hours prior to immunization . imaging and cytometric analyses were conducted 6072 and 96 hours later , respectively . to visualize gc recruitment and retention of t cells ( fig . 4 ) , 310 cfp - expressing sap and 310 gfp - expressing sap ot-2 t cells were co - transferred with 310 non - fluorescent md4 b cells . dye - labelled nave b cells ( 2410 ) were given 1 day before imaging to provide follicle / gc landmarks . imaging sessions longer than 2 hours , the animal s hydration was maintained by lactated ringer s solution given via a catheter . the typical x - y - z dimension was 0.51.10.51.13 m , and the time resolution was 3045s . for experiments involving co - transfer of two types of dye - labelled t cells , the cells were always reciprocally labelled to control for potential dye - induced behavioural differences . the mann - whitney rank sum test was used to calculate p values for highly skewed distributions . for gaussian - like distributions ,
generation of long - term antibody - mediated immunity depends on the germinal centre ( gc ) reaction , which requires cooperation between antigen - specific t and b lymphocytes . in the human x - linked lymphoproliferative disease and its gene - targeted mouse model , loss - of - function mutations in signalling lymphocyte activation molecule - associated protein ( sap , encoded by sh2d1a ) cause a profound defect in gc formation by an as yet unknown mechanism . using two - photon intravital imaging , here we show that sap deficiency selectively impairs the ability of cd4 + t cells to stably interact with cognate b cells but not antigen - presenting dendritic cells . this selective defect results in a failure of antigen - specific b cells to receive adequate levels of contact - dependent t cell help to expand normally , despite sap/ t cells exhibiting the known characteristics of otherwise competent helper t cells . furthermore , lack of stable interactions with b cells renders sap/ t cells unable to be efficiently recruited to and retained in a nascent gc to sustain the gc reaction . these results offer a compelling explanation for the gc defect due to sap deficiency and provide novel insights into the bi - directional communication between cognate t and b cells in vivo .
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it can involve any site of gastrointestinal tract , but the most common site is the mesentery of the small bowel . its biological behavior is intermediate between benign fibrous tissue proliferation and fibrosarcoma . sometimes , it is often confused with submucosal tumor or malignant neoplasm of gastrointestinal tract , especially gastrointestinal stromal tumors ( gists ) . we described a case of mf which induced obstruction of colon and ureter simultaneously and resulted in a positive positron emission tomography ( pet ) scan . a 49-year - old woman was admitted for constipation and left lower quadrant abdominal pain for seven days . physical examination revealed heperactive bowel sound and palpable mass at left lower quadrant of abdomen . initial complete blood cell counts and blood chemistry results were as follows : white blood cell count , 8.210/l ( normal , 4.510/l-10.510/l ) ; hemoglobin , 12.7 g / dl ; platelet count , 26410/l ; blood urea nitrogen , 24.4 mg / dl ; creatinine , 1.1 mg / dl . 2 ) showed near - complete obstructive mass at sigmoid colon covered with normal mucosa . abdominal ct scan disclosed not only an about 5.65.2 cm sized well circumscribed , locally infiltrative soft tissue mass in left pelvic cavity , but also marked left hydroureteronephrosis and atrophic change of left kidney ( fig . 3 ) , suggestive of longstanding ureteral obstruction . pet scan revealed the corresponding pelvic mass with focal increased activity with a maximum standardized uptake value ( suvmax ) of 4.1 ( fig . light microscopy shows ordely arrangement of uniform fibroblasts associated with moderate amouts of collagen ( fig . 5 ) . immunohistochemistry disclosed that the lesion were negative for cd34 and c - kit , whereas were positive for actin , vimentin . mf is a rare , benign fibrous lesion found in the bowel mesentery or the retroperitoneum . the mesentery of the small bowel is the most common site.1 mf tend to be locally invasive and to recur locally , but do not metastasize.1,2 it can occur spontaneously or after surgical trauma and also is associated with hormonal therapy , familiar polyposis , or gardner 's syndrome.1,3 the diagnosis of mf is based on clinical suspicion , which depends on the location or local effect of tumor . the role of imaging is to define the degree of extension to local structure and tumor relationship to neurovascular structure.4,5 pathologic confirmation was made by microscopic examination and immunohistochemistry . however , gists are potentially misdiagnosed as mf , because of their features such as large size , infiltration of adjacent structures and mitotic activity.6 their diagnostic discrimination is essential because of their very different biological behaviors and their therapeutic strategies.7 the absence of cd34 and s100 expression supports the fibromatous nature of the lesion and may be helpful in discriminating mf from gist.8 in addition , recently a report showed that -catenin separates mf from gist and sclerosing mesenteritis.9 our case showed that the lesion were negative for cd34 , whereas positive for -catenin , which were compatible with fibromatosis . the preferred treatment of mf is wide local surgical excision with a margin of uninvolved tissue.1,3 in the current case , unusually , the mass involved sigmoid colon and ureter at once and result in more aggressive features such as colonic obstruction with local invasion and hydroureteronephrosis . pet scan showed hot uptake in corresponding mass , considered as submucosal tumor of malignant neoplasm such as gist . first , however rare , mf is considered as one of the causes of stenosis of the colon in patients with a history of pelvic surgery . second , pet scan is not helpful for differential diagnosis of other malignant neoplasm from this disease , which is correspond with recent report that mf has a positive interpretation of fluorodeoxyglucose pet scan.10 third , -catenin is useful to differentiate between mf and gist .
mesenteric fibromatosis ( mf ) is a rare benign mesenchymal lesion that can occur throughout the gastrointestinal tract , especially small bowel . its biological behavior is intermediate between benign fibrous tissue proliferation and malignant fibrosarcoma . in previously reported cases of mf , we could find colonic obstruction or ureter obstruction , but simultaneous involvement of colon and ureter was not able to be seen . we described a patient that presented with colonic obstruction and hydroureteronephrosis due to mf at sigmoid colon which mimicked submucosal tumor such as gastrointestinal tumor . this case resulted in a positive positron emission tomography scan suggesting malignant neoplasm , but -catenin positivity on immunohistochemical staining separated mf from gastrointestinal stromal tumor and sclerosing mesenteritis . the clinical course of the patient was improved after surgical resection .
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the patient was a 23-year - old woman , a known case of arnold - chiari malformation with peripheral neuropathy and muscular atrophy , who presented with headache , drowsiness , decreased vision , and severe gait dysfunction lasting for several years . brain magnetic resonance imaging ( mri ) confirmed a hypointense - signal mass in the left hemisphere of the cerebellum causing mass effects on the fourth ventricle , which shifted it , accompanied with dilation of third and lateral ventricles . hypertensive cerebrospinal fluid ( csf ) form of hydrocephaly was seen in the supratentorial region , but the fourth ventricle was normal . the possibility of aqueduct stenosis or obstruction was mentioned ( fig . 1 ) . cervical spine mri revealed a normal spinal column with herniation of the cerebellar tonsil ( fig . 2 ) . brain magnetic resonance imaging . a hypointense signal mass in the left hemisphere of the cerebellum causing mass effects on the fourth ventricle , which shifted it , accompanied with dilation of third and lateral ventricles . tonsillar herniation to the cervical spine is seen . with the impression of tonsillar herniation and cerebellar tumor in the sense of arnold - chiari syndrome , external ventricular drainage with external reservoir a cream - white tight mass was seen , which could not be extracted by suction and thus was totally resected . simultaneously , regarding the arnold - chiari malformation and tonsillar herniation , the patient underwent suboccipital decompression and microscopic laminectomy of c2 vertebrae with excision of foramen magnum and duraplasty . gross examination revealed multiple pieces of irregular cream tissue totally measuring 2.5 2.0 0.7 cm . microscopic assessment confirmed a granulomatous inflammation composed of aggregation of epithelioid histiocytes associated with giant cells and lymphocyte cuffing foci of caseating necrosis compatible with tuberculoma . postoperative spiral chest computed tomography ( ct ) scan showed a normal pulmonary parenchyma without evidence for pulmonary tuberculosis , thus , the patient had a primary extrapulmonary cerebellar tuberculoma ( fig . the patient has been followed to now ; the neurological symptoms were alleviated 6 months subsequent to the surgery . chiari malformations are rare congenital anomalies with an estimated prevalence of 0.1 to 0.5%5 ; however , the true frequency is unknown . in most cases , due to the small posterior fossa the main pathogenesis of this malformation remains the subject of debate and involves patients presenting with a wide spectrum of clinical symptoms.1 arnold - chiari syndrome is usually detected prenatally or at birth , as it is nearly always associated with lumbosacral or thoracic myelomeningocele.1 weakness , stridor , apneic spells , aspiration , and dysphagia are the common manifestations in infancy,6 followed by progressive hydrocephalus in childhood.1 it may be associated with other syndromes like syringomyelia and scoliosis.7 8 our case presented with headache , drowsiness , decreased vision , and severe gait dysfunction lasting for several years . arnold - chiari syndrome should be considered in any fetus or newborn with clinical evidence of a spinal myelomeningocele . neuroimaging plays the main role in confirming the diagnosis , and mri is the best imaging modality for evaluation.9 mri of the brain along with the entire spinal cord ( cervical ) is appropriate to demonstrate downward displacement of the inferior cerebellar vermis and medulla through the foramen magnum into the upper cervical canal . despite these typical findings , brain mri in our case showed an unusual mass in the left cerebellar hemisphere causing mass effect . the patient underwent the most common procedure for arnold - chiari malformations , which is posterior decompression via suboccipital craniotomy with duraplasty.10 11 simultaneously , regarding the cerebellar mass with the primary impression of cerebellar tumor , posterior fossa craniotomy and resection of the mass were performed . postoperative histopathologic evaluation showed that the mass was in fact a granulomatous tuberculoma . because no pulmonary involvement was found in either clinical manifestation or pulmonary ct scan , it was diagnosed as a primary extrapulmonary cerebellar tuberculoma mimicking a cerebellar tumor . although tuberculosis is considered primarily a pulmonary disease , it can affect any organ system . however , central nerves system ( cns ) involvement is so rare , it is associated with potentially devastating complications ; it affects both immunocompetent and immunologically incompetent populations . , only a few numbers of cases with cerebellar tuberculoma have been reported , and most of them are secondary to the pulmonary involvement12 and occurred in immunocompromised patients.13 14 primary cerebellar tuberculoma is rare in an immunologically incompetent patient like our case . to our knowledge , this is the first reported case of primary cerebellar tuberculoma mimicking a posterior fossa tumor in a patient with arnold - chiari malformation . depending upon the involved part of the cns , various spectrums of clinical manifestations intracranial tuberculoma may present as either a solitary or multiple lesions in the brain parenchyma . a ring - enhanced area on ct scan or mri is a characteristic appearance , but when there are no accompanied clinical manifestation or laboratory findings for tuberculosis , it would be so difficult to differentiate it from other cns tumors . despite being rare , cns involvement of tuberculosis always should be kept in mind in any patient with neurological complaints from regions with a high endemic rate of tuberculosis , either in those being immunocompetent or immunologically incompetent .
chiari malformations are a congenital heterogeneous group of disorders characterized by anatomic anomalies of the cerebellum , brain stem , and craniocervical junction associated with downward displacement of the cerebellum , alone or with lower medulla , into the cervical spine canal . the patient was a 23-year - old woman , a known case of arnold - chiari malformation with peripheral neuropathy and muscular atrophy , who presented with headache , drowsiness , decreased vision , and severe gait dysfunction lasting for several years . brain magnetic resonance imaging confirmed a hypointense signal mass in the left hemisphere of the cerebellum causing mass effects on the fourth ventricle , which shifted it , accompanied with dilation of third and lateral ventricles .
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a 55-year - old man presented with difficulties in hand coordination and dressing himself that had first appeared in early 2006 . it took him a long time to put on his clothes because he had difficulty distinguishing between the front and back of clothing , and needed assistance with buttoning and zipping up his clothing . a severe memory disturbance that had also developed a neurological examination showed typical cortical signs including severe apraxia , cortical sensory loss , myoclonus , and alien - limb phenomenon that predominantly affected the right arm . there were no prominent visuospatial problems , including simultanagnosia , visual inattention , oculomotor apraxia , or optic ataxia . a detailed neuropsychological assessment revealed prominent verbal and visual memory deficits with marked frontal executive dysfunctions ( table ) . the patient scored 17/30 on the korena version of mini - mental state examination , with the subscore for time orientation being 3/5 and a delayed three - word recall of 0/3 . he exhibited an abnormal digit span on attention tests and showed severe ideomotor and ideational apraxia on several praxis tasks . his performance on copying in the rey - osterrieth complex figure test was impaired . on the seoul verbal learning test , he was able to recall two items ( < percentile 1 ) with a 20-minute delayed recall . he also scored poorly in delayed recall in the rey - osterrieth complex figure test ( 0/36 , percentile 2 ) . his performances on the tasks of controlled oral - verbal fluency and stroop test were also severely impaired . brain mri performed 18 months after the onset of the symptoms revealed significant cortical atrophy in both parietal areas that were more prominent on the left side with diffuse cortical atrophic changes in t1-weighted images ( fig . brain pet showed prominent asymmetric ( left - dominant ) hypometabolism in both parietal areas , with significant metabolic deficits in the left temporal lobes ( fig . cognitive impairments such as severe amnesia and visuospatial abnormalities were initially thought to be a rare or late presenting trait in cbd , with cognitive functions being relatively spared until the late stages of cbd and higher mental function being relatively preserved in cbd patients.1,7 clinical descriptions of cbd ( mostly from movement disorder clinics ) have emphasized motor manifestations such as parkinsonian features , apraxia , myoclonus , gaze palsies , and alien - limb phenomenon . research focused on the motor symptoms may have led to the notion that cognitive impairment or dementia occurs only in a few patients with cbd.6,7 postmortem pathological studies of cbd show neuronal loss , swollen achromatic neurons , and diffusely stained tau - positive astrocytic plaques . these changes typically involve the cortical and subcortical areas.2,3 asymmetric cortical atrophy involves mainly the superior parietal and frontal lobes , with smaller effects in the temporal and occipital lobes.4 several recent studies have documented that cognitive dysfunctions and language disturbances in the early stage of the disease course are not rare manifestations in cbd patients.8,9 however , the current findings related to episodic memory functioning in cbd are not described well by comprehensive cognitive assessments . our patient showed prominent memory impairment in several cognitive domains upon a detailed neuropsychological evaluation and history taking by his caregiver . the results of the word - list learning test as a verbal memory task indicated severe impairment of encoding , resembling the learning process frequently seen in patients with alzheimer 's disease ( ad ) . very few case studies have found abnormalities with respect to episodic memory test using the story recall test in patients with cbd.10 - 12 in general , cbd patients perform better on story recall and word list tasks than matched ad patients.11,13 the impairment of episodic memory appears to be less severe in cbd patients than in ad patients . in ad , poor strategic processes in frontal lobe dysfunctions or disruption of frontal - subcortical circuits leads to episodic memory impairment . however , the pattern of memory deficits in our patient differed from that typical of ad.12 the prominent memory deficits in our case can be explained by additional cortical hypometabolism in the left temporal area . however , there were no prominent visual complaints with typical presentations of balint 's syndrome in our case . although significant visuospatial and constructive dysfunctions were observed when our patient was asked to draw interlocking pentagons and rey - osterrieth figures , those deficits were augmented by severe hand apraxia . our patient also showed severe frontal subcortical circuit deficits when asked to perform several tasks of executive functioning . the frontal lobe dysfunctions could be explained by the significant hypometabolism in both frontal areas . in summary , our patient presented with severe episodic memory impairment and frontal executive dysfunctions at an early stage of cbd . however , other neurodegenerative diseases such as ad or other focal dementia syndromes associated with parkinsonism can not be completely ruled out without a postmortem pathologic diagnosis .
corticobasal degeneration ( cbd ) is a progressive neurodegenerative disorder characterized by asymmetric parkinsonism associated with apraxia , cortical sensory loss , and alien - limb phenomenon . neuropsychological testing in patients with cbd typically shows deficits in executive functions , praxis , language , and visuospatial functioning , but not in memory . we report a cbd patient with severely impaired memory function but relatively mild motor symptoms . detailed neuropsychological assessment showed significant verbal and visual memory deficits accompanied by frontal executive dysfunctions . our observations indicate that cbd can in rare cases present with severe episodic memory impairment associated with frontal executive dysfunctions in the early stage of illness .
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a 42-year - old indian male had multiple small circumscribed wrinkled sacs like lesions on the shoulders and upper back of 4 months duration ( fig . skin biopsy was taken for histopathological examination and revealed minimal dermal perivascular chronic inflammatory cells infiltrate ( fig . elastic stain ( verhoef - van gieson ) showed loss of elastic fibers in the superficial dermis ( fig . direct immunofluorescence ( dif ) was negative for iga , igg , igm , c1q , c3 and fibrinogen . the patient did not have any symptoms or show any sings of antiphospholipid syndrome ( aps ) , and screening for antiphospholipid antibodies ( anticardiolipin profile , anti--2-glycoprotein , igg and igm , and lupus anticoagulant ) were all negative . antinuclear antibody ( ana ) , ena screen ( smb , smd , rnp-70 , and rnp - a , rnp - c , ssa / ro52 , ssa / ro60 , ssb / la , cenp - b , scl-70 , jo-1 , ribosomal p and histones ) were negative . thyroid panel test showed normal free thyroxine and thyroid stimulating hormone , but the patient had a positive high titer of thyroid peroxidase antibody ( anti - tpo antibody ) > 116 iu/ ml . there are numerous reports and studies that link primary anetoderma ( pa ) to lupus erythematosus , but the relation has not been clearly established [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 ] . moreover , there are isolated repots of pa and autoimmune diseases like primary hypothyroidism , grave 's disease , addison 's disease , sjogren 's syndrome , alopecia areata , vitiligo [ 14 , 18 , 19 ] and multiple sclerosis . now , there is a growing body of evidence to consider pa as a cutaneous sign of positive antiphospholipid antibodies with or without fulfilling the criteria of aps [ 16 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 ] . in our case , there is a high titer of antithyroid peroxidase antibodies , which is consistent with autoimmune thyroiditis . this is in accordance with the work of hodak et al . who described a case of pa with grave 's disease , positive lupus anticoagulant and autoimmune hemolysis . it is important to mention that grave 's disease in the study of hodak et al . had started 5 years after the onset of the pa . in our study , there were no clinical signs or symptoms of thyroid disease at the time of diagnosis ; also free thyroxine and thyroid stimulating hormone were in a normal range , but we have to take the short duration of onset of pa in our case into consideration . also , bergman et al . described a case of primary hypothyroidism that developed 3 years after the onset of anetoderma . from our point of view and the aforementioned literature we have to think of pa as a cutaneous sign of autoimmunity and patients should be examined and carefully tested for autoimmune diseases , especially for antiphospholipid antibodies , lupus erythematosus and also thyroid antibodies . patients should also be followed up because associated autoimmune diseases may develop later in the course of the disease , maybe years after the onset of anetoderma .
anetoderma is a rare elastolytic disorder characterized by circumscribed areas of flaccid skin due to the loss of elastic tissue in the dermis . primary anetoderma is frequently observed in patients with autoimmune diseases or abnormalities especially with antiphospholipid antibodies with or without antiphospholipid syndrome . in this case report we discuss a patient with primary anetoderma with positive antithyroid peroxidase antibodies , which is consistent with autoimmune thyroiditis .
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vertebrobasilar dolichoectasia ( vbd ) is a condition characterized by ectasia , elongation and tortuosity of the basilar artery . it may manifest clinically by compression of the cranial nerves , ischemic symptoms or intracranial bleeding . rarely , the dilated and ecstatic basilar trunk may manifest as obstructive hydrocephalus . we present a 60-year - old male with markedly dilated and elongated basilar trunk compressing the third ventricular outflow and , thus , presenting with hydrocephalus . a 60-year - old male presented to our outpatient department with complaints of headache since 2 years . he also complained of imbalance while walking and episodes of urinary incontinence since the past 5 months . on examination , he had bilateral papilloedema . he underwent further evaluation with magnetic resonance imaging ( mri ) of the brain , computerized tomography ( ct ) of the brain and ct angiography . dilated and tortuous basilar artery was noted extending into the suprasellar space compressing the third ventricular outflow . ct angiography of the brain showed dilated and tortuous left vertebral artery curving in the premesencephalic cistern from left to right and again from right to left . the basilar artery was also tortuous , with a diameter of 7.0 mm and extending into the suprasellar cistern [ figure 1 ] . the patient underwent right - sided ventriculoperitoneal shunt and the headache improved postoperatively . left and middle pictures show dilated ventricles with dolichoectatic basilar artery compressing the midbrain and outflow of the third ventricle . the right picture shows the computerized tomography angiogram with the dilated and elongated basilar trunk extending into the suprasellar space vbd is an anatomic variant that consists of enlargement and dilatation , often associated with a tortuous and elongated vessel . it is known by various names like dolichoectasia , megadolichoectasia fusiform aneurysm of the vertebral and basilar arteries and tortuous vertebrobasilar system . the prevalence of vbd is 4.4% , and it is more commonly observed in women . the major location for vbd is the basilar artery alone ( 40% ) , followed by bilateral vertebral arteries , basilar artery ( 22% ) and both vertebral arteries ( 16% ) . the diagnostic criteria for vbd is a basilar artery or vertebral artery diameter > 4.5 mm or deviation of any portion of them higher than 10 mm from the shortest expected course , or basilar length > 29.5 mm or intracranial vertebral artery length > 23.5 mm . the vertebrobasilar system may be considered elongated if the basilar artery lies lateral to the margin of the clivus or dorsum sellae , or if it bifurcates above the plane of the suprasellar cistern . hypertension , commonly associated with vbd , may cause continued stress on the walls of the artery and degrade the vessel wall by damaging and loosening the collagen and elastin meshwork that comprises the intima . traditionally , vbd has been regarded as atherosclerotic in nature , much like aneurysms of the peripheral vascular system . however , recently , mizutani and aruga suggested that some cases represent a dissecting process . it may also be a congenital vasculopathy of the elastic layer of the arterial wall . it may present with varied clinical syndromes like cerebellar dysfunction , ischemic stroke , transient or permanent motor deficits , central sleep apnea , trigeminal neuralgia , hydrocephalus as well as brain stem compression syndrome . clinical expression of this condition may be due to compression of the cranial nerves or brainstem , ischemia in the vertebrobasilar arterial territory and intracranial bleeding . ikeda et al . studied 7345 adult subjects , and found that 96 of them had asymptomatic vbd . among these 96 subjects , hydrocephalus in vbd can be due to compression of the third ventricle by the ectatic , elongated and tortuous basilar artery . only few cases of hydrocephalus due to direct compression of the aqueduct , foramen of monro or third ventricle have been reported in the literature.[1014 ] most of these cases have been reported in elderly patients . a peculiar mechanism of hydrocephalus by water - hammering effect due to the pulsating blood in the ectatic vessel , which creates cerebrospinal fluid outflow impairment through the third ventricle , has also been described . management of vbd depends on symptomatic manifestations . for asymptomatic patients with vbd , functional testing such as brainstem auditory - evoked potentials ( baeps ) , blink reflex ( br ) and motor - evoked potentials may be useful for long - term monitoring and may help in the decision - making process prior to the surgical approach for relief of subjective symptoms . endoscopic third ventriculostomy may be technically difficult in these cases due to the odd anatomy of the basilar trunk . vbd as a rare cause of obstructive hydrocephalus needs to be kept in mind by neurosurgeons .
dolichoectasia of vertebrobasilar artery is a condition in which the vertebral / basilar artery is elongated , distended and tortuous . it is usually asymptomatic . it may present with compressive or ischemic symptoms . hydrocephalus as a complication of vertebrobasilar dolichoectasia is rare . we present a case of a 60-year - old male with dolichoectasia of the basilar artery causing compression of the third ventricular outflow and , thus , presenting with noncommunicating hydrocephalus .
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a 47-year - old female was referred to department of oral and maxillofacial surgery with a chief complaint of pain and swelling of the lower lip . intra - oral clinical examination revealed a well circumscribed swelling measuring 6 cm 6 cm in dimension i.r.t 41 , 42 , 43 . it was covered with a thin normal looking mucosa , no inflammatory or erythematous areas seen around the swelling . palpation showed a firm mass , bony hard in consistency , not fixed to the underlying tissue . after taking consent from the patient , surgical excision was planned . the patient underwent an uneventful excision under local anesthesia and the excised specimen was sent for a histopathological examination . grossly , the specimen consisted of a gray - white mass measuring 1 cm 1 cm in dimension . serial histological section at 6 each were made , and then , stained with hematoxylin and eosin technique . microscopic observation revealed parakeratinized stratified squamous epithelium cells with an underlying stroma is composed of dense connective tissue with few blood capillaries [ figures 1 and 2 ] . h and e , stained section shows mature lamella lined by stratified squamous epithelium h and e , stained section shows hematopoietic areas the lesion is composed of mature lamellar bone , osteocytes , and adipocytes . the specimen is also showing hematopoietic areas . with these histological findings along with clinical findings suggestive of the osseous choristoma of the labial mucosa a choristoma is defined as a histologically normal tissue proliferation or nodule of a soft tissue type not normally found in the anatomic site of proliferation . these include cartilage , bone , glial tissue , gastric mucosa , and tumor - like masses of sebaceous glands . most frequently observed choristomas of the oral cavity are those that consist of bone . the age range for reported cases of intraoral choristomas is between 8 and 73 years . most intraoral choristomas develop in dorsal surface of the tongue around the foramen caecum , but lesions have also been reported in the tongue , buccal mucosa , and the lingual alveolus of the mandible . clinically , it grows as a pedunculated lesion or firm nodular with size of mass may range between 0.5 and 2.0 cm in size . most patients are unaware of the lesion , but may feel the symptoms of pain , dysphagia , or sometimes may have choking sensation , and nausea also have been reported in some cases . in the literature ( pubmed search revealed ) , 88 cases have been described regarding the osseous choristoma in regard to tongue and buccal mucosa in common , but not in relation to the labial mucosa . the osseous choristoma to arise in the age group of 1262 years of age with the lesion occurring more frequently in the fifth decade of life . it has a slightly predisposition to occur more commonly in females than males with a ratio of 1.5:1 . although there are various theories regarding the etiology of ocs , their exact origins remain mysterious and both developmental and trauma origins have been reported . histologically , these are choristoma consists of a well - circumscribed mass of viable lamellar bone with a well - developed haversian canal system , surrounded by dense , fibrous connective tissue , and covered with stratified squamous epithelium . the lesion reported here is a rarest type of osseous choristoma seen in the labial mucosa and is the first ever reported a case of osseous choristoma originating in the labial mucosa . the number of reported cases or literatures is insufficient , to review this particular type of osseous choristoma . awareness of this entity is required so that an early and accurate diagnosis can be made .
osseous choristoma is a normal bone tissue in an ectopic position . these are slow growing lesions that are usually completely asymptomatic and only present when there is a disruption in the function of the organ due to its large size as it grows . definitive diagnosis is obtained only after the histopathological examination . the etiology remains still questionable . the treatment of choice is surgical excision . here we report a case of choristoma in the lower labial mucosa in a 47-year - old female .
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to report on a case of keratoconjunctivitis with periorbital edema after accidental exposure to undiluted juice of ecballium elaterium fruit during the ripening season of this plant . keratoconjunctivitis with descemet 's membrane folds and superficial upper corneal well - defined edematous areas were noted after an ecballium elaterium fruit burst and its juice splashed into the patient 's left eye . prompt administration of antibiotic and steroid eye drops along with per os antihistamine therapy , resulted in quick regression of symptoms . this report demonstrates the toxic effects of this herb to the eye and also that prompt therapy is effective . a 70-year - old male farmer presented to the emergency department with periorbital pain , periorbital and conjunctival edema , epiphora , hyperemia , irritation , and blurred vision from his left eye after inadvertent exposure to undiluted juice of ecballium elaterium fruit some hours earlier . the symptoms began within minutes after the herb 's fruit bursted and juice splashed into the eye ( fig . slit lamp examination revealed corneal epithelial micro - erosions , corneal superficial well - defined edematous areas mainly in the peripheral upper quadrants ( fig . the patient was treated promptly with eye irrigation , topical steroids for periorbital edema , combination of antibiotic and steroid eye drops along with antihistamine tablets . within four days from the initiation of treatment , exposure to ecballium elaterium fruit juice , mainly in its undiluted form , may cause irritation of the mucous membrane . elaterium is the dried sediment that deposits in the juice of the fruits of ecballium elaterium . it is a powerful cathartic and contains up to 30% of elaterin , which is the active ingredient . the toxic dose is 5 mg and the fatal dose 0.6 g. published adverse reactions related to the toxic effect of this remedy include localized swelling of the uvula , the nasal mucosa and others sites of the upper respiratory tract of allergy - prone patients . also , there is a report of fatal cardiac and renal failure probably related to ecballium elaterium juice intake . regarding the eye , kocak et al . reported a case of late - onset diffuse lamellar keratitis 11 months after lasik , when an ecballium elaterium herb fruit burst and splashed into the patient 's eye . also , raikhlin - eisenkraft and bentur report 6 cases with ocular exposure presented with conjunctivitis , corneal edema , and erosion . this report demonstrates the toxic effect of ecballium elaterium juice to the eye and also , that prompt therapy results in rapid regression of symptoms .
purposeto report on a case of keratoconjunctivitis with periorbital edema after accidental exposure to undiluted juice of ecballium elaterium fruit during the ripening season of this plant.methodscase report.resultskeratoconjunctivitis with descemet 's membrane folds and superficial upper corneal well - defined edematous areas were noted after an ecballium elaterium fruit burst and its juice splashed into the patient 's left eye . prompt administration of antibiotic and steroid eye drops along with per os antihistamine therapy , resulted in quick regression of symptoms.conclusionsthis report demonstrates the toxic effects of this herb to the eye and also that prompt therapy is effective .
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malignant peripheral nerve sheath tumors ( mpnsts ) are a rare variety of soft - tissue sarcoma of ectomesenchymal origin , with an estimated incidence of 0.1/100,000/year . recently , fluorodeoxyglucose ( fdg ) positron emission tomography / computed tomography ( pet / ct ) has been employed in differentiating the malignant from the benign peripheral nerve sheath tumors . the current case highlights an unusual pet / ct presentation of mpnst masquerading clinically as infected dermatoses . a 64-year - old male , working as a gardener , presented with multiple rapidly progressive asymptomatic skin colored raised lesions 2 - 4 cm in diameter , over the medial border of the right foot and lower part of the right leg since 1 month . the lesion was subjected to punch biopsy , with clinical differential diagnosis of sporotrichosis , actinomycetoma , linear cutaneous leishmaniasis and atypical mycobacterial infection . histopathology revealed a malignant mesenchymal tumor , which was confirmed to be a mpnst on immunohistochemistry . soon thereafter , the lesions rapidly increased in size and number and began to ulcerate and become painful [ figure 1a ] . magnetic resonance imaging ( mri ) of the right lower limb revealed multiple enhancing nodular lesions seen involving the skin and subcutaneous tissue . subsequently , a whole body 18f - fdg pet / contrast - enhanced computed tomography ( cect ) scan was performed on a whole body full ring pet / ct camera ( discovery ste 16 , ge ) . 370 mbq of 18f - fdg was administered intravenously subsequent to a 6 h fast . whole body cect scan was performed after intravenous instillation of non - ionic contrast medium . after the ct scan , an emission scan was performed from head to thigh for 2 min per frame . images were reconstructed by 3d vue algorithm ( ge ) and viewed on a xeleris workstation ( ge ) using the volumetric protocol . the study revealed multiple fdg avid nodular lesions ( maximum standardized uptake value 18.6 ) in the right lower limb [ figure 1b ] , which were many more in number compared with those picked up on cutaneous examination . multiple nodular lesions ( arrows ) with evidence of ulceration on the medial aspect of the right leg and foot ( a ) , which show increased fluorodeoxyglucose avidity ( b ) mpnsts arise from a peripheral nerve or its branches or from the nerve sheath . although they may arise spontaneously , up to 50% arise in patients of neurofibromatosis 1 ( nf1 ) . mri is the imaging modality of choice because it can reveal the nerve of origin and its relationship to adjacent structures . although it is well - known that these tumors can extend for considerable distances along nerves , it may not always be possible to delineate the origin from the nerve . a recent study by bilgic et al . has shown that the nerve origin could be identified only in 45 - 56% cases . there have been recent reports of fdg pet / ct in mpnst , most of which have been performed in patients with nf1 . it has been documented as a useful tool in monitoring clinically stable nf1 patients with plexiform neurofibromas as it could predict which were more likely to subsequently grow rapidly . it has also been found that in patients with nf1 harboring mpnsts ; higher fdg uptake is associated with poorer survival rates . in addition , it can assist in guiding targeted needle core biopsies and may provide critical information in tumors that are not amenable to biopsy . it thus plays an important role in the staging , restaging and post - therapy follow - up of mpnst in nf1 . a high fdg uptake has been documented in sporadic mpnsts also , as is well seen in the coronal maximum intensity projection image [ figure 2 ] in the present case . coronal maximum intensity projection image showing high fluorodeoxyglucose uptake in multiple nodular lesions on the right lower limb , predominantly on the medial aspect mpnsts usually present as discrete masses , which may be multifocal , especially in cases of nf1 . the present case was unusual in its presentation , owing to the linear arrangement of multiple nodules over the lower extremity in a gardener , raising the possibility of infectious dermatoses . these multiple hypermetabolic mildly enhancing nodular lesions were restricted to the skin and subcutaneous tissue with no extension into the underlying muscles [ figure 3 ] . the additional lesions detected on pet / ct were also proven to be mpnst based on histopathology with immunocytochemistry [ figure 4 ] . though multimodality therapy , including surgical resection and adjuvant radiotherapy , is available , the prognosis remains dismal . axial contrast - enhanced computed tomography ( a ) and fused positron emission tomography / computed tomography ( b ) images of the leg ( upper row ) and foot ( lower row ) demonstrate multiple hypermetabolic mildly enhancing nodular lesions ( arrows ) involving the skin and subcutaneous tissue with no extension into the underlying muscles ( a ) h and e stain ( 10 [ inset 40 ] ) shows a tumor in the dermis with multiple spindle - shaped cells in whorls with pleomorphic nuclei forming a storiform pattern . ( b ) vimentin stain ( 10 [ inset 40 ] ) for mesenchymal structures is diffusely positive throughout the dermis
malignant peripheral nerve sheath tumors ( mpnsts ) are rare tumors , with an estimated incidence of 0.1/100,000/year . they are regarded as a rare variety of soft - tissue sarcomas that derive from peripheral nerves or from cells associated with the nerve sheath . until 50% of observed mpnsts occur in patients with neurofibromatosis 1 ( nf1 ) . the typical presenting signs and symptoms of a pnst are a palpable mass involving a peripheral nerve , loss of nerve function and/or pain . recently , positron emission tomography ( pet ) has been used to detect 18f - fluorodeoxyglucose uptake in these tumors . most of the pet studies have been reported in patients with nf1 . we report a case of sporadic mpnst masquerading as infectious dermatoses , with an unusual pet / computed tomography presentation .
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we came across 3 cases of sarcomas , other than leiomyosarcomas , reported to arise from the broad ligament . to the best of our knowledge , no case of an embryonal rhabdomyosarcoma ( erms ) of the broad ligament has been reported in literature . we report about a 9-month - old female child with an erms of the broad ligament . the case we present here is a 9-month - old female child , presented to us with the complaints of straining at micturition and defecation since the age of 5 months . the patient was diagnosed with benign partial epilepsy of infancy and was under treatment for the same . no other comorbidities were present . on examination , the patient had multiple caf au lait spots over her body ( more than 5 in number ) . however , there were no other features to suggest neurofibromatosis , like , family history , lisch nodules , axillary or inguinal freckling and osseous lesions . abdominal examination revealed an abdominopelvic mass , in the midline , extending superiorly to just above the umbilicus . a contrast enhanced computed tomography ( cect ) scan of the abdomen and pelvis confirmed the clinical findings of an abdominopelvic mass of about 9.8 cm 6 cm 5 cm extending from the level of acetabulum in the pelvis , to the level of l3 vertebra [ figure 1 ] . the mass was isodense to hypodense on a plain scan with heterogenous enhancement on post - contrast scan . the urinary bladder was pushed to the right side due to mass effect but planes were maintained . the uterus and adnexa were not visualized separately . a percutaneous biopsy done from the mass revealed erms . the metastatic work - up , including a bone scan , cect thorax and bone marrow examination , was normal . contrast enhanced computed tomography scan showing the heterogenously enhancing mass arising from pelvis , with contrast in urinary bladder ( a and b ) . intra - operatively ( c ) , the mass seen arising from the broad ligament , the uterus ( a ) and urinary bladder ( b ) separated the patient received neoadjuvant chemotherapy ( nact ) , as per intergroup rhabdomyosarcoma study group ( irs ) iv protocol ( vincristine , actinomycin and cyclophosphamide ) . an exploratory laparotomy performed revealed a lobulated mass of 10 cm 5 cm 5 cm [ figure 1 ] . the mass had a pedicle and was seen to be arising from the left broad ligament . the uterus and adnexa were separate from the mass , pushed to the right side . the mass was densely adhered to the urinary bladder but could be separated by dissection . as per the tnm and irs staging , our patient was staged as stage i , group iii , with embryonal histology and thus classified as low risk subgroup . rhabdomyosarcoma is the most common soft - tissue sarcoma of children accounting for 3 - 4% of all the childhood cancers . rhabdomyosarcoma is known to be associated with caf au lait spots and neurofibromatosis type - i . our patient though had caf au lait spots , did not have other features suggestive of neurofibromatosis . on reviewing the literature on broad ligament sarcomas ( excluding leiomyosarcomas ) , we came across only 3 such cases . have reported the first of those in 1985 in their series of eight patients of female genital tract sarcomas . the patient was 16 years of age with an alveolar rhabdomyosarcoma of the broad ligament . the second case was a 55-year - old female diagnosed and treated for an undifferentiated pleomorphic sarcoma of the broad ligament reported by bouraoui et al . the third patient , reported by diaz - murillo et al . was a 27-year female with an undifferentiated round cell sarcoma of the broad ligament . our patient is a 9-month - old child with an erms of the broad ligament . the patient reported by diaz - murillo was followed - up for 12 years without any recurrence . the treatment is decided after a tissue diagnosis and metastatic work - up of the patient . our patient after appropriate investigations was diagnosed as a non - metastatic pelvic erms . on the basis of cect scan findings of an abutting urinary bladder and non - visualization of the left ureter , it was decided to start nact ( irs iv protocol ) and reassess for the response . on repeating a cect scan after nact , it was a stable disease ; hence , it was decided to go ahead with surgery for appropriate local control of the tumor . adjuvant radiation therapy was considered in view of the mass adherent to the urinary bladder , but withheld considering the age and low risk group of the patient . broad ligament rhabdomyosarcomas are an extremely rare entity . to the best of our knowledge , this is the first case of an erms arising from the broad ligament in an infant .
broad ligament tumors are uncommon . sarcomas rarely arise from the broad ligament , leiomyosarcomas being the most common . rhabdomyosarcomas of the broad ligament are very rare . to the best of our knowledge , no case of an embryonal rhabdomyosarcoma of the broad ligament has been reported in literature .
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hamartomatous polyps are uncommon and usually found as a part of peutz - jegher syndrome , a rare autosomal dominant disorder associated with mucocutaneous pigmentation and family history . hamartomatous polyps are composed not only of epithelial elements , but also of bundles of smooth muscle cells . such hamartomatous gastric polyps are almost always found in patients with peutz - jeghers syndrome , in association with intestinal polyps . in hamartomatous polyps , there is an increased risk of malignancy in both the gastrointestinal and extra - intestinal sites [ 2 - 4 ] . the risk increases with age ( range , 1 to 33% ) between 30 and 60 years . fifty seven years - old man with a history of epigastric pain was transferred from an outside hospital with the diagnosis of early gastric cancer . physical examination and laboratory no pigmentation was found on the lips and perioral region , hands , buccal mucosa , and feet . endoscopy revealed two masses on the greater curvature side of the prepyloricantrum : a round , 1 cm polypoid lesion and an irregular 3 cm elevated mass with superficial ulcerations ( fig . 1a ) and another 6 cm pedunculated polypoid mass on the greater curvature side of prepyloricantrum ( fig . upper gastrointestinal series showed three polypoid lesions in the gastric antrum which was considered as an advanced , borrmann type i carcinoma preoperatively ( fig . subsequently , the patient underwent a radical subtotal gastrectomy , which revealed a 6 5 cm mass confined to the mucosa of the greater curvature of the lower body and another 5.5 2 cm mass invading into the submucosa of the greater curvature of the antrum ( fig . the 5.5 2 cm sized mass looked like two masses , but was a fused mass intraoperatively . microscopic analysis of the specimen revealed adenocarcinoma within the hamartomatous polyps demonstrated by proliferation of smooth muscle bundles within the lamina propria and between carcinomatous glands ( fig . 4a ) with evidence of epstein - barr virus ( ebv ) in both lesions ( fig . the patient had an uneventful postoperative course and was discharged home on the 9th postoperative day . reported four cases of patients with multiple benign gastric hamartomatous polyps that were not associated with polyposis coli . however , syndrome - associated hamartomatous polyps may have malignant potential as cases of malignant hamartoma have been described [ 8 - 10 ] . from our review of the english - language literature , this case report appears to be the first documented case of adenocarcinoma derived from gastric hamartomatous polyps which was not accompanied by a known syndrome . we present a case report of a patient with malignant gastric hamartomatous polyps in the absence of familial polyposis nor pigmentation . furthermore , this case exhibited positive expression via in ebv - encoded rna ( eber ) in situ hybridization . to date , there is no report about incidence of adenocarcinoma in hamartomatous polyps in eber - positive in situ hybridization .
most hamartomatous polyps in the stomach occur in patients with adenomatous polyposis coli and dysplasia . the authors report a case of a 57-year - old man without prior history of polyposis coli who presented with adenocarcinomas derived from hamartomatous polyps in the stomach . the patient underwent a radical subtotal gastrectomy with billroth i anastomosis . pathology revealed adenocarcinomas with moderate differentiation without evidence of lymph node metastasis in 60 nodes . we report a case of gastric cancers arising from de novo gastric hamartomatous polyps .
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vegf and bfgf contents in various subtypes of grade i meningiomas vegf ( upper panel ) and bfgf ( lower panel ) contents were determined by elisa . n denotes the number of patients . for transitional meningiomas : vegf range [ 0.259.85 ] and bfgf range [ 67.4576.9 ] ; for microcystic meningiomas : vegf range [ 0.715.29 ] and bfgf range [ 164.3520.0 ] ; for meningothelial meningiomas : vegf range [ 0.874.98 ] and bfgf range [ 183.6573.7 ] ; for fibroblastic meningiomas : vegf range [ 0.101.69 ] and bfgf range [ 142.1377.3 ] . * no statistical differences were observed between groups ( mann - whitney u test ) .
the quantitative analysis of vegf using elisa in various subtypes of grade i meningiomas reported higher vegf contents in meningothelial ( 2.38 0.62 pg/g protein , n = 7 ) , transitional ( 1.08 0.21 pg/g protein , n = 13 ) , and microcystic meningiomas ( 1.98 0.87 pg/g protein , n = 5 ) as compared with fibrous ones ( 0.36 0.09 pg/g protein , n = 5 ) . in contrast to vegf , no difference in the concentrations of bfgf was detected . vegf levels did not correlate with meningioma grade ( 1.47 0.23 pg/g versus 2.29 0.58 pg/g for 32 and 16 grade i and ii , resp ) , vascularisation ( 1.53 0.41 pg/g versus 1.96 0.28 pg/g for 24 low and 24 high vascularisated tumours , resp ) , and brain invasion ( 2.32 0.59 pg/g versus 1.46 0.27 pg/g for 7 and 41 patients with and without invasion , resp ) . the elisa procedure is , thus , an interesting tool to ensure vegf and bfgf levels in meningiomas and to test putative correlations with clinical parameters . it is , thus , tempting to speculate that elisa would also be valuable for the quantitative analysis of other angiogenic growth factors and cytokines in intracranial tumours .
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actinomycosis is an infrequent invasive chronic inflammatory disease caused mainly by actinomyces israelii , gram - positive , filamentous , anaerobic bacteria . actinomycosis occurring primarily in urachal remnants is rare and may mislead the clinicians to diagnose urachal carcinoma . we report one such case of urachal actinomycosis in a 50-year - old male with lower abdominal pain and mass palpable mimicking a urachal tumor . a 50-year - old man , hailing from south india , came to the urology outpatient department with complaints of lower abdominal pain , dysuria , and dribbling of urine for 2 months duration . clinically , he was afebrile and per - abdomen examination revealed a vague mass of 7 cm palpable at the suprapubic region . computed tomography scan of the abdomen revealed an irregular mass seen superior to fundus of urinary bladder appearing adherent to mesentery and bowel loops and suggested an urachal remnant associated lesion with infiltration figure 1 . excision of the mass with partial cystectomy and resection of involved ileal segments was done figure 2 . the specimen was sent for histopathological examination . computed tomography of the abdomen showing an irregular mass seen superior to fundus of urinary bladder appearing adherent to mesentery and bowel loops grossly , we received an irregular soft tissue mass measuring 12 cm 15 cm 4 cm with umbilicus on the one side and dome of the bladder on the other side . we also received two loops of intestine with attached serosal mass measuring 4 cm 3 cm 2 cm . after adequate sampling , sections from the urachal remnant revealed actinomycotic colonies surrounded by microabscesses and dense inflammatory fibrotic lesion figure 3 . small intestinal segments showed a similar lesion in the serosa but not in the mucosa , confirming it to be a primary urachal actinomycosis . photomicrograph showing high power view of h and e section of actinomycotic colonies surrounded by microabscesses the patient was started on intravenous penicillin and recovered without complications . the most common risk factor includes use of intrauterine contraceptive devices in female , and others include history of bowel surgery such as perforated acute appendicitis , perforated colonic diverticulitis , penetrating trauma to the abdomen , and persistent urachal remnant . our patient showed two of the risk factors of persistent urachal remnant and an earlier appendicectomy . the infiltrative nature of the bacteria into the surrounding organs can be explained by production of proteolytic enzyme . there are no specific radiological signs to distinguish actinomycotic lesions from malignancy due to its infiltrative nature . definite diagnosis before surgery is difficult unless patient had discharging sinuses in the lower abdomen . demonstration of the colonies of actinomycosis by histopathological examination is the definitive diagnosis , and hence , adequate sampling and extensive scanning must be done . in a study of 33 patients with pelvic actinomycosis by marella et al . , 19 patients had intrauterine contraceptive devices and only four of them had urachal remnants . exploratory laparotomy was done in most of them , and definite diagnosis was made by histopathological examination after excision of the mass . it has a good prognosis and is curable with antibiotics such as penicillin and amoxicillin . it is important to consider it in the differential diagnosis of patients presenting with lower abdominal pain and hard suprapubic mass with or without discharging sinuses . to the best of our knowledge ,
primary actinomycosis occurring in urachal remnants is rarely documented in literature and may mislead the clinicians to diagnose urachal carcinoma . a 50-year - old man came with complaints of lower abdominal pain , dysuria , and dribbling of urine for 2 months . a vague mass of 7 cm was palpable in the suprapubic region . imaging of the abdomen revealed an irregular mass seen superior to fundus of the urinary bladder appearing adherent to mesentery and bowel loops , suggesting an urachal remnant associated lesion with infiltration . provisional diagnosis of urachal carcinoma was considered . excision of the mass with partial cystectomy and resection of involved ileal segments were done . microscopic examination revealed actinomycotic colonies surrounded by microabscesses and dense inflammatory fibrotic lesion . small intestinal segments showed a similar lesion in the serosa .
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traumatic lumbar spondylolisthesis is rare disease and in the literature , different surgical approaches , including anterior , posterior , or combined approaches ( posterior and anterior ) are used to treat the lesion . we treated a case of traumatic lumbosacral spondylolisthesis using posterior approach and the patient showed a satisfactory outcome . at the final follow - up , he was completely asymptomatic , and radiographic images revealed normal lumbar alignment and a solid interbody fusion . traumatic lumbosacral spondylolisthesis can be treated using posterior approach alone to obtain reduction , decompression , and solid fusion . traumatic lumbosacral spondylolisthesis is a rare injury ( 1 - 3 ) ; most of the cases were published as case reports in the literature . the injury results from a complex and high - energy mechanism ( 4 ) or forces , including hyperextension stress ; hyperflexion and compression stress ; or tangential force ( 5 ) . most surgeons believe that the injury should be treated surgically , and in this regard different surgical approaches are used in spine departments . we report a case of traumatic lumbar spondylolisthesis , which was treated using posterior approach to realize the stable 3-column fixation and solid interbody fusion . a 38-year - old man was referred to the affiliated hospital of jinan university , guangzhou , china due to injury in a motorcycle accident on july 25 , 2011 . he was conscious with stable vital signs , but complained of pain in his back and right thigh , numbness and weakness in both lower extremities . upon physical examination , grade 4 power was found in both lower limbs , and the perianal sensation and anal tone were normal too . x - radiographs showed a grade 2 spondylolisthesis of l5 on s1 ( figure 1 ) , fracture of the left transverse process of l4 as well as the fracture of right femur . mri demonstrated traumatic lumbar spondylolisthesis of l5 on s1 , avulsion of the l5 intervertebral disc and compression of the cauda equina ( figure 1 ) . first , open reduction and internal fixation were performed for the fracture of right femur , and then a posterior approach surgery was performed for the traumatic lumbosacral spondylolisthesis using a standard posterior midline incision . during operation , bilateral fracture of the pars interarticularis , disruption of interspinal ligament and flaval ligaments of l5-s1 , and disruption of l5 annulus fibrosus were found . decompression and reduction were done followed by internal fixation using pedicle screws and rods from l4 to s1 . posterolateral fusion was performed at l4 - 5 level ; l5 disk was excised and 2 peek cages were inserted posteriorly with autologous bone grafts . the procedure lasted 145 minutes with intraoperative blood loss of 400 ml , without intraoperative complications . four weeks later , his strength and cutaneous sensation in both lower extremities recovered completely . one and a half year after surgery , at the final follow - up , the patient was completely asymptomatic and radiographs revealed normal lumbar alignment and a solid interbody fusion ( figure 2 ) . in addition , the fracture of right femur obtained bony union and he could stand and walk without any support , and resumed his previous level of physical activities . in the english literature , some cases of traumatic spondylolisthesis were reported , which were treated successfully using conservative methods ( 7 , 8) , but the non - surgical treatment may result in posttraumatic translational instability or chronic low back pain ( 1 , 3 ) . moreover , the rare lesion belonged to a 3-column injury ( 9 ) and need a solid internal fixation . as a result , surgical treatment was a better choice for the injury ( 1 - 3 , 9 ) . in the literature , this kind of injury was treated using different surgical approaches , including anterior ( 10 ) , posterior ( 1 , 2 , 9 ) , or combined approach ( anterior and posterior ) ( 11 , 12 ) , but there is not a decisive criterion to determine which surgical approach to select . in this case , the lesion included a traumatic disruption of the intervertebral disk material , dislocation of l5 vertebral body and bilateral fracture of the pars interarticularis . therefore , excision of intervertebral disc and reduction of l5 vertebral body as well as interbody fusion were needed ( 1 ) . in addition , decompression and internal fixation to avoid further injury to the nerve system , stabilize the spine , and promote the recovery of the nerve system was necessary . compared with anterior or combined approach , the posterior approach is safe , easy , and with minimum complication . moreover , the pedicle - rod system can result in perfect reduction of vertebral body and 3-column fixation . consequently , decompression , fixation , and interbody fusion can be achieved using posterior approach alone . at the same time , the higher risk of blood loss , longer hospital stay and the high cost which is associated with anterior or combined approach can be avoided or decreased . in the present case , this kind of case is rare , and it is difficult to compare different surgical approaches using a large scale , or a clinical controlled trial , but we believe the posterior approach alone may be an optimal selection for this rare injury .
introduction : traumatic lumbar spondylolisthesis is rare disease and in the literature , different surgical approaches , including anterior , posterior , or combined approaches ( posterior and anterior ) are used to treat the lesion.case presentation : we treated a case of traumatic lumbosacral spondylolisthesis using posterior approach and the patient showed a satisfactory outcome . at the final follow - up , he was completely asymptomatic , and radiographic images revealed normal lumbar alignment and a solid interbody fusion.conclusions:traumatic lumbosacral spondylolisthesis can be treated using posterior approach alone to obtain reduction , decompression , and solid fusion .
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chlamydophila pneumoniae is a common etiological factor of the respiratory tract infections , including pharyngitis , bronchitis , and pneumonia . it is estimated that c. pneumoniae is responsible for pneumonia in approximately 10% of cases , and for bronchitis and sinusitis in approximately 5% of cases . the main symptoms of infection include cough , pharyngitis and , hoarseness , often accompanied by sinusitis pharyngitis occurs in the first phase , followed by bronchitis or pneumonia in the second phase of a disease . prolonging cough ( for more than 3 weeks ) is a basic symptom indicating c. pneumoniae infection [ 4 - 6 ] . respiratory tract infections of c. pneumoniae etiology are characterized by a long incubation period , usually lasting 14 - 21 days . untreated infections can lead to many complications , such as exacerbations of bronchial asthma , endocarditis , coronary heart disease , endothelitis , and abortions [ 7 - 9 ] . the aim of this study was to assess the incidence of chlamydial respiratory tract infections in children and adolescents in the lower silesia region of poland in the year 2009 . the study was performed in accordance with the declaration of helsinki for human research and the study protocol was accepted by institutional ethics committee . in 2009 , children treated for various , non - specific respiratory illnesses in different hospital wards , in clinical departments , or as outpatients ( mainly from family practices ) in the lower silesia region were investigated for c. pneumoniae infections . the study materials were 641 throat swabs obtained from 326 girls and 315 boys , aged 11 months to 18 years . past medical history of these patients was indicative of recurrent respiratory tract infections , long - lasting paroxysmal dry cough , rhinitis , and hoarseness . throat swabs from the posterior wall of the pharynx were made before the start of any treatment , with the use of thin , sterile disposable swabs . tests for the presence of c. pneumoniae antigens in throat swabs were performed by an indirect immunofluorescence antibody ( ifa ) technique , using the chlamydia cel pn testing kits ( cellabs pty ltd . , sydney , australia ) . table 1 presents the results of throat swabs examination for c. pneumoniae in 641 children . the positive ifa test results were shown in 276 patients ( 43.1% of the study group ) , including 41.4% of girls and 44.8% of boys . the proportion of positive results was strikingly large , and it should be taken into consideration regarding epidemiologic assessment , differential diagnosis , and therapeutic management . the presence of c. pneumoniae antigens in children depending on the diagnosis and the clinical symptoms is shown in table 2 . in the group of children with features of infection which manifested itself in the form of dry cough , the proportion of positive results for c. pneumoniae was 41.4% , and in the children with cough , rhinitis , and discharge from the throat - 47.2% . an assumption can be made that these are the dominant clinical symptoms of infection , since other symptoms like hoarseness occurred only in 22.9% of the examined subjects . in the group of children with symptoms of infection and having contact with sick people around them , the proportion of positive findings for c. pneumoniae was 48.3% , whereas in the children without infection in their environment this proportion was as low as 29.3% . that shows that there was a high probability of transmitting infection with cough or as droplet infection . in the group of children followed up for 14 days after the end of treatment , the presence of c. pneumoniae antigens was revealed in 54.2% . the latter observation is of a substantial interest to us , since it could have a bearing on further patients ' management . the significance of this observation is , however , hampered by a relatively small number of patients , 48 ( 17.3% ) out of the group of 276 with positive tests for the presence of c. pneumoniae antigens , who were followed up . the following conclusions can be drawn from the findings of this study : in the group of children and adolescents from the lower silesia region a large number of c. pneumoniae airborne and droplet infections were revealed ; the most common clinical symptoms were dry cough lasting for more than 3 weeks and rhinitis ; the diagnosis of the respiratory tract infection in a child caused by chlamydia should be the signal for a thorough examination of people from its closest circle ; patients treated with antibiotics should always be followed up , because in nearly 50% of them the presence of chlamydia pneumoniae can still be detected .
objectiveto assess the incidence of chlamydia pneumoniae respiratory tract infection in children and adolescents in the lower silesia region in poland in 2009.materials and methods641 throat swabs obtained from 326 girls and 315 boys , aged 11 months to 18 years , were assessed diagnostically . the patients enrolled into the study were treated on an outpatient basis due to various , non - specific respiratory ailments . the most common presenting clinical symptom of a respiratory problem was dry cough , which occurred in 295 studied subjects , followed by runny nose and cough with discharge in 176 subjects , and other minor symptoms in 35 subjects . the assessment was conducted by an indirect immunofluorescence antibody ( ifa ) chlamydia testing kit ( cellabs , sydney , australia).resultsoverall , chlamydia infection was detected in the respiratory tract in 43.1% ( 276/641 ) of the children , with no clear gender differences . of the 295 subjects presenting with dry cough , 122 ( 41.4% ) had positive tests for chlamydia . of the 176 subjects with runny nose and cough and the 35 subjects with other symptoms , 83 ( 47.2% ) and 8 ( 22.9% ) had positive tests for chlamydia , respectively . in the asymptomatic children who had direct contact with a chlamydia infected person , there were 29.6% ( 8/27 ) positively tested cases , whereas in the children presenting symptoms , the percentage of positive tests was 48.3% ( 29/60).conclusionsin children living in the lower silesia region of poland , there is a substantial ~50% rate of chlamydia infection , transmitted via airborne droplets . the finding of chlamydia infection should be the signal for testing other subjects from the child 's closest environment .
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self - expandable metal stents ( sems ) are uncommonly used for benign colonic disease because of their lack of removability and their high rate of migration . histology had confirmed the presence of diverticular disease in the sigmoid colon along with inflammatory changes in the tubes , ovaries and part of the uterus , which was resected en - bloc . the patient subsequently underwent reversal of her hartmann s procedure with a de - functioning loop ileostomy . at a clinic review two months post hartmann s reversal , the patient complained of intermittent discharge from the lower end of her laparotomy wound associated with per - rectal intermittent discharge of mucous and blood . contrast studies had revealed the presence of an anastomotic stricture complicated with a colo - cutaneous fistula ( figure 1 ) . contrast study showing stricture and fistula tract after a discussion with the patient , taking in account her obesity , the agreed plan was to adopt a non - operative approach rather than surgery that may result in a permanent end - colostomy . three months post hartmann s reversal a balloon dilatation was carried out successfully via flexible sigmoidoscopy as a joint procedure with a specialist radiologist . four months post hartmann s reversal it was decided to repeat the dilatation then to insert a colonic stent along with percutaneous ethanol injection ( pei ) of the fistula . this was followed by injection of 20mls of absolute ethanol through the abdominal fistulous tract whilst , at the same time , normal saline was injected into the stent via a foley s catheter to dilute the alcohol within the lumen of the colon . a month later , contrast studies showed complete resolution of the anastomotic stricture and no evidence of colo - cutaneous fistula ( figure 2 ) . one year post hartmann s reversal , the patient remained asymptomatic and the fistula had resolved both clinically and radiologically . follow - up contrast study a year post hartmann s reversal , patient was admitted for reversal of her loop ileostomy , which was performed without complications . the use of self - expanding metallic stents for benign colorectal diseases has been regarded previously as controversial ( 1,2 ) . only 3% of stents placed in 567 patients considered in a systematic review in ( 2002 ) were for benign disease , predominantly diverticular disease ( 3 ) . there has been only a few reports of its use for closure of benign stulae in the colon ( 4 ) . in our case the presence of a stricture with stulous tracts was an indication for the use of a covered stent for immediate stula closure . covered sems have a high rate of migration as compared to uncovered sems ( 2,5 ) . in addition , spontaneous migration of sems for benign strictures occurs frequently , usually within the rst month after insertion ( 6 ) . ethanol injection for successful ablation of digestive tract fistula has been mentioned in few case reports in the literature ( 7 ) . to our knowledge , this is the rst case demonstrating sems combined with pei for benign postoperative stulae . although longer follow up and future prospective studies are needed ,
we report successful treatment of colocutanteous stulae at the site of a benign colonic anastomotic stricture using self - expandable metal stents ( sems ) and percutaneous ethanol injection ( pei ) . the stricture and the secondary stulae developed after hartmann s procedure for sigmoid diverticular abscess followed by reversal of the hartmann s procedure with a de - functioning loop ileostomy . although there have been previously reported cases of sems for closure of malignant colonic stula , there are limited reports of sems for closure of benign colonic stulae and none of sems combined with pei for benign colonic fistula . we conclude that this patient s case exemplies the potential applications of sems combined with pei as a feasible option of therapy for benign colorectal disease .
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autonomic dysfunction presents with heterogeneous clinical features and is a multi - system condition affecting nervous , cardiac , gastrointestinal , musculoskeletal and vocal cord function . autonomic dysfunction is often a result of an imbalance in the sympathetic and parasympathetic nervous system . this case study demonstrates the association of vocal cord disorders in autonomic dysfunction and the role of yoga to modulate autonomic balance . a 23-year - old female with a past medical history of severe gastroesophageal reflux disease presented with shortness of breath and fatigue . i do not feel like i am getting enough oxygen to my brain , being suffocated in closed spaces and with certain smells . shortness of breath was associated with inspiratory wheeze , fatigue , chest pain , lightheadedness , headache , numbness / tingling in the arms and legs , and exercise intolerance . alternative lifestyle changes such as using a cold mist humidifier , sleeping flat , and breathing with mouth open were helpful . orthostatic vitals recorded in the supine , sitting , and standing positions at 3 min intervals were positive [ figure 1 ] . work - up included normal electrocardiogram , chest x - ray , chest computed tomography , and echocardiogram . complete blood count , comprehensive metabolic panel , and thyroid - stimulating hormone levels were normal . arterial blood gas showed respiratory alkalosis with compensatory metabolic acidosis both before and after exercise . pulmonary function tests showed hyperventilation and flattening of the inspiratory portion of the volume flow loop . exercise stress test showed an exercise capacity of 59% of normal [ figure 2 ] . she was counseled to increase fluid intake and referred to speech therapy for breathing exercises . orthostatic vital signs recorded at three - minute intervals volume flow loop and exercise stress test results on follow - up , her shortness of breath improved after 6 weeks of a yoga training program including isometric and diaphragmatic breathing exercises . initially , she used to have daily symptoms ; after the yoga program , she had symptoms only a couple of times a week . at times , when she did have shortness of breath , she took deep breaths with her mouth open ( pursed lip inspiration ) , and her shortness of breath improved . this case report demonstrates the association of vocal cord dysfunction with dysautonomia and the central role of an imbalanced autonomic system in the pathophysiology of both , where yoga may provide a therapeutic role in their management . autonomic dysfunction was demonstrated by positive orthostatic vitals and a complex of autonomic symptoms , including headaches , lightheadedness , brain fog , chest pain , gastroesophageal reflux , and numbness / tingling . vocal cord dysfunction was demonstrated by flattening of the inspiratory portion of the flow volume loop and dyskinesia of the vocal cords by laryngoscopy . a multifactorial approach including increased fluid intake , breathing , and isometric exercises resulted in the improvement of symptoms . vocal cord dysfunction is associated with autonomic disorders , including shy - drager syndrome and multiple system atrophy ( msa ) . upper airway obstruction due to vocal cord dysfunction has been observed via laryngoscope in 8 of 10 patients with progressive pan - autonomic failure of shy - drager syndrome . the mechanism of the selective involvement of abductor muscle ( posterior muscle ) of the vocal cord in autonomic dysfunction is unknown . the key to the pathophysiology of both autonomic and vocal cord dysfunction is the role of the vagus nerve in interfacing homeostasis . vocal cord dysfunction , abnormal adduction of vocal cords , and laryngeal hyper - responsiveness are suggested to be the functions of altered autonomic balance . the vagus nerve influences autonomic functions respiratory , cardiac cycles , and gut motility . an inflammatory insult can disrupt the normal functioning of the vagus nerve resulting in an altered autonomic function with paroxysmal movement of the vocal cords . stimuli such as exercise or odors induce parasympathetic reflexes causing airway narrowing at the glottis level . given the central role of the vagus nerve , breathing and isometric exercises may be therapeutic in the management of autonomic dysfunction . inspiratory muscle training ( imt ) is therapeutic for both vocal cord and autonomic dysfunction . in a case report of a patient with exercise - induced paradoxical vocal fold motion ( pvfm ) , imt for 5 weeks resulted in no pvfm symptoms at the end of the study . the results showed increased inspiratory muscle strength , improved maximal exercise capacity , and reduced exercise - induced dyspnea . respiratory maneuvers and isometric exercises are also beneficial in autonomic failure . in a study measuring mean arterial pressure , isometric exercises such as leg muscle tensing reduced orthostatic hypotension . respiratory maneuvers including inspiratory - pursed lips breathing and inspiratory sniffing reduced orthostatic hypotension in autonomic failure via activation of the respiratory pump . the inter - relationship between vocal cord and autonomic dysfunction may have profound implication in the diagnosis and management of autonomic disorders . more correlative data studying the relationship between vocal cord dysfunction and autonomic disorders need to be analyzed . given breathing and isometric exercises are therapeutic to both vocal cord and autonomic dysfunction , there is a need for well - designed clinical trials investigating the role of breathing and isometric exercises in the role of autonomic dysfunction . vocal cord dysfunction is often associated with dysautonomia . given the central role of the vagus nerve in autonomic and vocal cord dysfunction , breathing and isometric exercises aid in the regulation of physiologic responses and
a 23-year - old female with a past medical history of gastroesophageal reflux disease presented with shortness of breath induced by exercise and certain odors . she reported the symptoms of autonomic dysfunction including fatigue , chest pain , lightheadedness , headaches , numbness / tingling in the arms and legs , and exercise intolerance . vital signs were significant for orthostatic intolerance . volume flow loop in the pulmonary function tests showed a flattening of the inspiratory portion characteristic of vocal cord dysfunction . laryngoscopy showed dyskinesia of the left vocal cord , especially after exercise . multifactorial approach was used including increased fluid intake and breathing exercises . after 6 weeks of breathing and isometric exercises , the patient reported improvement in dyspnea after exercise . this case report demonstrates the therapeutic role of breathing and isometric exercises in the management of vocal cord and autonomic dysfunction .
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diabetic retinopathy ( dr ) is a major microvascular complication of diabetes accounting for its leading cause of irreversible blindness worldwide . assessing the risk factors of dr , particularly modified risk factors , is important for early intervention to reduce the onset and progression of dr . these studies consistently established that a longer diabetic duration , hyperglycemia , and hypertension were associated with increased risk of dr . high lipid levels are known to cause endothelial dysfunction due to a reduced bioavailability of nitric oxide and this endothelial dysfunction was suggested to play a role in retinal exudate formation in dr . it was also reported that the peroxidation of lipids in lipoproteins in the vascular wall leads to local production of reactive carbonyl species that mediate recruitment of macrophages , cellular activation and proliferation , and also chemical modification of vascular proteins by advanced lipoxidation end - products which affect both the structure and function of the vascular wall . consequently , it was proposed that , hyperlipidemia might contribute to dr and macular edema ( me ) by endothelial dysfunction and breakdown of the blood retinal barrier leading to exudation of serum lipids and lipoproteins . to determine correlation between severity of dr with serum lipid and other modifiable risk factors in type 2 diabetic patients . to determine correlation between severity of dr with serum lipid and other modifiable risk factors in type 2 diabetic patients . this was a retrospective study done in department of endocrinology and metabolism llrm medical college . a total number of 140 type 2 diabetic patients with dr were recruited from diabetic clinic during may 2011 till june 2012 . information including age , sex , height , body weight ( wt ) , body mass index ( bmi ) , waist - hip ratio ( whr ) , and systolic and diastolic blood pressure was collected from each patient . fasting plasma sugar , low density lipoprotein ( ldl ) , triglyceride level ( tg ) , high density lipoprotein ( hdl ) , glycated hemoglobin ( hba1c ) , creatinine , and 24 h urinary albumin excretion was done for each patient . estimated glomerular filtration rate ( egfr ) was measured by modification of diet in renal disease ( mdrd ) . patients were divided in five groups according to retinopathy status based on early treatment dr study ( etdrs ) disease severity level . statistical analysis was performed with statistical packages for social sciences ( spss ) statistical software ( version 17.0 for windows ) . as shown in table 1 there was statistically significant positive correlation between severity of dr and systolic blood pressure p = 0.005 ( r = 0.974 ) , diastolic blood pressure p = 0.001 ( r = 0.994 ) , ldl p = 0.005 ( r = 0.976 ) , tg p = 0.001 ( r = 0.990 ) , and 24 h urinary albumin p = 0.004 ( r = 0.977 ) . dr was also strongly positively correlated with smoking p = 0.017 ( r = 0.941 ) and duration of diabetes p = 0.003 ( r = 0.981 ) . there was strong negative correlation of dr with hdl p = 0.001 ( r = -0.994 ) and egfr p = 0.002 ( r = -0.987 ) . endothelial dysfunction is a well - known finding in hypercholesterolemic patients and it was proposed that , hyperlipidemia might contribute to dr and me by endothelial dysfunction and breakdown of the blood retinal barrier leading to exudation of serum lipids and lipoproteins . in this study we have evaluated the modifiable risk factor of dr and there correlation with severity of dr . there are conflicting reports in the literature regarding the effect of lipid profile on retinopathy or maculopathy . in etdrs it was shown that patients with high total cholesterol and ldl levels were more likely to have retinal hard exudates compared to patients with normal lipid profile . in our study , we found a significant correlation between serum lipids and dr , but there was no association between hba1c and dr . similarly , chennai urban rural epidemiology study showed that mean cholesterol , triglyceride , and non - hdl levels were higher in patients with dr compared to those without dr . hypercholesterolemia , systolic and diastolic blood pressure , renal function , and urine albumin excretion is significantly associated with progression of dr .
a total number of 140 type 2 diabetic patients with diabetic retinopathy ( dr ) were enrolled in the study from diabetic clinic during may 2011 till june 2012 to determine correlation between severity of dr with serum lipid and other modifiable risk factors in type 2 diabetic patients . information including age , sex , height , body weight , body mass index ( bmi ) , waist - hip ratio ( whr ) , and systolic and diastolic blood pressure was collected from each patient . fasting plasma sugar , low density lipoprotein ( ldl ) , triglyceride level ( tg ) , high density lipoprotein ( hdl ) , glycated hemoglobin ( hba1c ) , creatinine , and 24 h urinary albumin excretion was done for each patient . estimated glomerular filtration rate ( egfr ) was measured by modification of diet in renal disease ( mdrd ) equation . patients were divided in five groups according to retinopathy status based on early treatment dr study ( etdrs ) disease severity level . statistical analysis was performed with statistical packages for social sciences ( spss ) statistical software ( version 17.0 for windows ) . the alpha level was set at p = 0.05 for all tests . statistically significant positive correlation between severity of dr with systolic blood pressure p = 0.005 ( r = 0.974 ) , diastolic blood pressure p = 0.001(r = 0.994 ) , ldl p = 0.005 ( r = 0.976 ) , tg p = 0.001 ( r = 0.990 ) , and 24 h urinary albumin p = 0.004 ( r = 0.977 ) was documented . dr was also strongly positively correlated with smoking p = 0.017 ( r = 0.941 ) and duration of diabetes p = 0.003 ( r = 0.981 ) . there was strong inverse correlation of dr with hdl p = 0.001 ( r = 0.994 ) and egfr p = 0.002 ( r = 0.987 ) . serum lipids were significantly correlated with severity of dr .
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ameloblastic fibro - odontoma ( afo ) is a benign , slow growing , expansile epithelial odontogenic tumor with odontogenic mesenchyme . it may inhibit tooth eruption or displace involved teeth although teeth in the affected area are vital [ 13 ] . radiography shows a well - defined , radiolucent area containing various amounts of radiopaque material of irregular size and form [ 4 , 5 ] . the lesions are usually diagnosed during the first and second decades of life [ 46 ] . it occurs with equal frequency in the maxilla and the mandible and with equal frequency in males and females . microscopically , the lesion is composed of strands , cords , and islands of odontogenic epithelium embedded in a cell - rich primitive ectomesenchyme , resembling the dental papilla . many authors reported that afo is not aggressive and can be treated adequately through a surgical curettage to the lesion without removal of the adjacent teeth [ 1 , 4 , 5 , 7 , 8 ] . an 11-year - old girl presented to our department on referral from another dentist to have a second opinion about a lesion involving the left mandible . she had radiographic examinations , including panoramic , helical , and cone - beam computed tomography . these examinations were accompanied by a presumptive radiographic differential diagnosis of odontoameloblastoma : complex odontoma and afo . the medical , social and family histories were unremarkable , as were the results of a review of systems and a physical examination . the clinical examination did not display any sign of pain or swelling in the left mandible . the initial panoramic radiography revealed a well - defined radiolucent region , which contained an irregular radiopaque mass 3 cm in diameter . this lesion occupied a zone from the lower left second molar area to the left ramus . helical and cone - beam computed tomography showed an expansile well - circumscribed lesion containing at the interior a calcified mass compatible with odontogenic tissue ( figures 2 and 3 ) . considering the clinical and radiographic examinations , our presumptive diagnosis was complex odontoma . the patient underwent enucleation of the lesion and careful curettage of the surgical cavity under general anesthesia . the surgical specimen was fixed in neutral buffered 10% formalin and subjected to pathological analysis . light microscopic examination of sections stained with hematoxylin and eosin revealed strands and islands of odontogenic epithelium showing peripheral palisading and loosely arranged central cells , identical to stellate reticulum , embedded in a myxoid cell - rich stroma resembling the dental papilla ( figure 4 ) . in the present case , the patient presented to our department with previous examinations , including panoramic , helical and cone - beam computed tomography . while these radiographic examintions were given a presumptive diagnosis of odontoameloblastoma by the examinaing radiologists , we believed that the findings were more common in this region . odontoameloblastoma , also known as ameloblastic odontoma , has a more aggressive behavior , similar to an ameloblastoma rather than an odontoma . afo is a benign tumor that exhibits the same benign biologic behavior as that of ameloblastic fibroma , showing inductive changes that lead to the formation of both dentin and enamel . conversely , the term odontoameloblastoma ( or ameloblastic odontoma ) refers to tumors representing a histological combination of ameloblastoma and complex odontoma , which behave in the invasive manner of classic ameloblastoma . according to the revised world health organization ( who ) classification , ameloblastic fibroma and afo cahn and blum postulated that ameloblastic fibroma ( the histologically least differentiated tumor ) develops first into a moderately differentiated form , following afo and eventually into a complex odontoma.however , the concept that these lesions represent a continuum of differentiation is not widely accepted , with other researchers suggesting that they are separate pathologic entities [ 1215 ] . in some studies , the term afo represents a histological combination of ameloblastic fibroma and complex odontoma [ 12 , 16 ] . the majority now agrees that afo exists as a distinct entity , but it can be histologically indistinguishable from immature complex odontoma . the arrangement of the soft tissues and the development stage of the involved tooth are useful criteria for diagnosis . despite numerous efforts , however , there is still considerable confusion concerning the nature of these lesions . afo is relatively rare , with the prevalence among oral biopsies being about 1% and its frequency among odontogenic tumors being reported at 1% to 3% [ 3 , 18 ] . this lesion usually occurs in people less than 20 years old , and age is thus an important characteristic in the differential diagnosis . this lesion is usually found in the molar area [ 6 , 12 ] , and the distribution is roughly equal between the maxilla and mandible [ 6 , 12 ] . many authors reported that afo can be treated adequately through a surgical curettage without removal of the adjacent teeth [ 1 , 4 , 5 , 7 , 8 ] . as noted in the literature , not all lesions previously classified as afo are , in fact , aggressive lesions . if there is a recurrence accompanied by a change of the histological pattern toward a more unorganized fibrous stroma with displacement of the epithelial component , then more extensive treatment procedures appear to be indicated . long - term follow up with short intervals should be maintained in the management of afo .
an 11-year - old girl presented to our department to have a second opinion regarding a lesion involving her left mandible . she had previously undergone several radiographic exams including panoramic , helical , and cone - beam computed tomography . radiographic examinations revealed a well - defined radiolucent region , which contained an irregular radiopaque mass of 3 cm in diameter , localized to the left angle of the mandible . our presumptive diagnosis was complex odontoma . excisional biopsy was performed , and microscopic features showed strands and islands of odontogenic epithelium showing peripheral palisading and loosely arranged central cells , identical to stellate reticulum , embedded in a myxoid cell - rich stroma resembling the dental papilla . dentin and enamel were also presented . the diagnosis was ameloblastic fibro - odontoma , which is a rare mixed odontogenic tumor , derived from epithelial and ectomesenchymal elements that form the dental tissues .
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epidermal nevus with hemifacial hypertrophy can occur in schimmelpenning syndrome , phakomatoses pigmentokeratotica , proteus syndrome and type 2 segmental cowden disease . the epidermal nevus syndromes are a group of congenital syndromes comprising epidermal nevi in conjunction with central nervous system ( cns ) , ocular , musculoskeletal , and other organ anomalies . most epidermal nevi are noticed at birth or develop during the first year of life . reports of associated hemi hypertrophy of the face are multiple and almost always noticed at birth . we herewith report a patient having an epidermal nevus at 25 years of age with onset of gradually progressing hemihypertrophy of face associated with overlying hypertrichosis . genetic analysis done from the skin did not reveal fibrobalst growth factor receptor 3 ( fgfr3 ) mutations . a 26-year - old man reported to skin outpatient department with dark warty growth on left side of cheek for past 6 years . he noticed gradual disproportionate enlargement of the left side of face after the onset of the growth . he had no history of seizures , headaches or any other cns events . on examination , there were linear hyperpigmented verrucous papules with overlying hypertrichosis on left side of cheek extending on to the neck [ figure 1a ] . the left side of the face looked larger than the right [ figure 1b ] . x - ray skull was suggestive of mandible condylar hyperplasia . however , orthopantogram revealed slight widening of ramus ; no other bony abnormality was observed . computed axial tomography of the cranium and brain showed thickening of the frontal bone on the left side [ figure 2 ] . skin biopsy of the representative skin lesion ( face ) showed hyperkeratosis with papillomatosis [ figure 3 ] . genetic analysis was done from the skin in the area affected by the nevus . on genetic analysis of skin samples taken from the nevus , only wild type alleles were revealed for the hotspot loci on fgfr3 and p110 subunit of phosphatidyl inositol-3 - kinase ( pik3ca ) . ( a ) verrucous epidermal nevus with overlying hypertrichosis on left side of face and neck , ( b)- hemihypertrophy of left side of face and scalp ct scan showing thickening of frontal bone on left side section of skin biopsy showing hyperkeratosis , acanthosis , and papillomatosis . keratinocytic nevi and sebaceous nevi can be associated with epidermal nevus syndromes ( enss ) . the epidermal nevus syndrome refers to a sporadic neurocutaneous disorder characterized by epidermal nevi , ophthalmological and neurological manifestations . happle has described multiple phenotypes associated with cutaneous mosaicism and made a strong case for avoiding umbrella terms like epidermal nevus syndrome and organoid nevus syndrome when describing a specific ens . the well - defined syndromes are schimmelpenning syndrome , phakomatoses pigmentokeratotica , nevus comedonicus syndrome , angora hair nevus syndrome , becker 's nevus syndrome , type 2 segmental cowden disease , fgfr3 segmental nevus syndrome , and child ( congenital hemidysplasia with icthyosiform erythroderma and limb defects ) syndrome . of these , schimmelpenning syndrome , phakomatoses pigmentokeratotica , proteus syndrome , and type 2 segmental cowden disease may be associated with hemi hypertrophy of face or limbs . however patients with hemihypertrophy of face usually are reported to have cortical defects like hemimegalencephaly , focal cortical dysplasia , and neuronal migration defects like pachygyria and polymicrogyria heterotopias in the affected hemisphere . our patient had relatively late onset of epidermal nevus associated with left - sided progressive hemihypertrophy of the face and hypertrichosis . hypertrichosis confined to the area of epidermal nevus is described in angora hair nevus syndrome and in a case of epidermal nevus with bony abnormalities described by gobello et al . gobello et al . described a 16-year - old boy with non - epidermolytic epidermal nevus , hypertrichosis and follicular hyperkeratosis in addition to hemihypoplasia of limbs , brachydactyly , clinodactyly , and onychodystrophy . genetic analysis done from the affected skin in this case did not reveal mutations in fgfr3 . in our patient too , only wild type alleles for fgfr3 were seen in affected skin . our patient had hypertrichosis overlying an epidermal nevus with hemihypertrophy of face . while the features do not exactly match those described by gobello et al . genetic analysis done from lesional skin showed only wild - type alleles of both fgfr3 and pik3ca genes . somatic mosaicism for fgfr3 mutations involving skin and other organ systems have been described in patients . if the mutation involves the gonads , such patients are said to be at risk for thanatophoric dysplasia . in many other patients with epidermal nevi , somatic mutations resulting in mosaicism confined to keratinocytes of the affected area have been demonstrated in the fgfr3 gene , specifically at the r248c hotspot , and in the pik3ca gene . child syndrome is caused by nsdhl ( nad ( p ) h steroid dehydrogenase - like ) gene mutations ; type 2 cowden disease by pten ( phosphatase and tensin homolog ) mutations . we report this unique case of late onset epidermal nevus with hypertrichosis and hemihypertrophy as it does not belong to any of the classical epidermal syndromes . a possible association to gobello syndrome can not be confirmed until more molecular diagnostic techniques are developed and employed . hemifacial hypertrophy and hypertrichosis can occur in association with epidermal nevus in the absence of fgfr3 mutations . this does not fit into any of the known ens and could be a new entity .
epidermal nevus syndromes are rare conditions , characterized by different types of keratinocytic or organoid epidermal nevi in association with ocular , neurological , and skeletal manifestations . we present a case of late onset epidermal nevus with hypertrichosis and hemihypertrophy of face . genetic analysis did not reveal presence of fgfr3 or pik3ca mutations . the patient has features that can not be categorized into the present well - known syndromes .
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there are many reports of paraganglioma diagnosed on fine needle aspiration cytology ( fnac ) in the literature . although fnac of a suspected paraganglioma is contraindicated in view of the hypertensive crisis after fine - needle aspiration , this tumor can be an unexpected finding in neck mass fnacs performed in unsuspecting cases . cytologic features of single cell , loose clusters and occasional acinar configuration of large , oval cells with round to oval nuclei , evenly dispersed chromatin , focally discrete nucleoli , moderate anisocytosis and anisonucleosis have been well described for paragangliomas . in the present case in addition to the acinar and discrete arrangement of cells , smears also showed few large loose clusters of cells enveloped by thin vascular channels the so called zellballen pattern , a diagnostic feature on histopathology . we describe a case of a 42-year - old woman presenting with fever on and off since 1 year and neck swelling of 5 months duration . ultrasonography ( usg ) showed a well - defined heterogeneous highly vascular lesion in right cervical region . clinical examination revealed a solitary 3 cm 2 cm non - mobile non - tender mass present at the right angle of mandible . fnac was bloody and pap stained smears revealed cells in clusters , microacinar and rosette like formations as well as singly . the cells were monomorphic , round to plasmacytoid with round to oval nuclei and a moderate amount of pale granular cytoplasm . there were in addition , few large loose clusters of cells enveloped by vascular channels on all sides . these cells showed same cytomorphologic features as surrounding cells [ figure 1a and b ] . ( a ) cells arranged in compact clusters surrounded by vascular channels on all sides ( pap , 100 ) . ( b ) endothelial cells in vascular channel enveloping tumor cells on one surface of the same cluster seen in ( a ) ( pap , 200 ) . ( c ) typical zellballen pattern on histopathology of the same case ( h and e , 100 ) in view of monomorphic cells with the absence of necrosis and mucin , metastatic adenocarcinoma was ruled out . metastasis of medullary carcinoma of the thyroid was considered on cytomorphology , but was ruled out on the basis of a normal thyroid on usg and absence of specific cytologic features . histopathological examination confirmed the diagnosis of a benign carotid body paraganglioma [ figure 1c ] . this case report highlights the presence of loose cell clusters enveloped by thin vascular channels on cytology , a feature which has been occasionally described in anecdotal case reports in intraoperative scrape smears . the presence of cell clusters with vascular coats on cytology should lead to high index of suspicion for pheochromocytoma / paraganglioma ( adrenal or extra adrenal ) in addition to the usual cytological , clinical and radiological features . it is important to diagnose this condition pre - operatively in order to avoid any surgical delay . clusters and discretely scattered cells , with occasional acinar configuration and fine reddish intracytoplasmic granules are the established facts of a paraganglioma . however cell clusters enveloped by thin vascular channels on cytology the so called zellballen pattern diagnostic of histopathology may be considered as a novel insight and should be sought for in cytology smears when paraganglioma is a high suspicion .
paragangliomas are uncommon tumors arising in paraganglion tissue , which is well represented in the head and neck . a correct pre - operative cytological diagnosis helps in planning early and optimal surgery . cytologic features in carotid body tumor and extra adrenal paragangliomas have been well described and documented . in addition to the acinar and discrete arrangement of cells , smears also showed a few large loose clusters of cells enveloped by thin vascular channels - the so - called zellballen pattern , a diagnostic feature on histopathology . zellballen pattern of paraganglioma on cytology is not a common finding and hence is being reported .
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a 42-year - old italian man , who had been living in nigeria because of his job since two years , was urgently repatriated following diagnosis of acute plasmodium falciparum malaria . fourteen days after anti - malarial treatment started , with quick resolution of fever , he suddenly complained of acute left flank pain . laboratory tests disclosed severe anemia ( hemoglobin 7.7 g / dl ) , moderate leukocytosis , markedly elevated c - reactive protein ( 232 mg / l ) , plasma d - dimer ( 2188 ug / l ) , lactic dehydrogenase ( 654 iu / l ) and creatine kinase ( 1255 rapid blood assay tested positive for p. falciparum antigen , with negative blood cultures for bacteria and parasites . prompt investigation with multi - detector ct [ figure 1 ] allowed confident diagnosis of diffuse , moderately hyperdense peritoneal effusion consistent with hemoperitoneum and splenomegaly with hyperdense , stratified subcapsular and perisplenic clotted hematoma . axial unenhanced ( a ) and contrast - enhanced ( b ) images of the upper abdomen reveal splenomegaly with stratified subcapsular and perisplenic hyperdensity consistent with ruptured splenomegaly and clotted hemorrhage . axial unenhanced image of the pelvis ( c ) and post - contrast panoramic coronal - reformatted image ( d ) show moderately hyperdense ( 30 - 40 hu ) effusion ( asterisks ) occupying the douglas pouch as well as other peritoneal compartments urgent laparotomic surgery confirmed abundant hemorrhagic peritoneal effusion without detectable active bleeding . pathology specimen reported 850-g enlarged spleen measuring 17148 cm after fixation , with large capsular interruption and underlying hematoma . histologically , diffuse parenchymal hemorrhage and markedly hypertrophic red pulp were observed , without follicular structure abnormalities nor signs of malignancy . although very uncommon , spontaneous ( non - traumatic ) hemoperitoneum is a life - threatening condition that generally represents a surgical emergency and may prove fatal even if promptly diagnosed and appropriately treated . its differential diagnosis includes bleeding from liver ( mostly hepatocellular adenoma or carcinoma rather than metastases ) or kidney ( particularly angiomyolipomas ) tumors , splenic rupture , visceral aneurysms or pseudo - aneurysms , deep abdominal varices from portal hypertension , excessive anticoagulant treatment , hemodialysis and gynaecological disorders such as ovarian cyst or ectopic pregnancy rupture . because of its widespread availability and extreme acquisition speed , ct currently performed on multi - detector scanners represents the mainstay diagnostic modality to investigate patients presenting to with acute abdomen and signs of hemodynamic instability . at ct , hemoperitoneum is heralded by higher - than - water density ( measuring 30 - 45 hounsfield units , hu ) peritoneal effusion , whereas the even more hyperdense ( 45 - 70 hu ) sentinel blood clot is usually observed nearest to the site of hemorrhage ; sometimes contrast extravasation indicating active bleeding may be detected , a finding that indicates the necessity for emergency surgical or interventional treatment . as in this case , ct confidently identifies the spleen as the injured organ . splenic rupture most usually occurs during various systemic infections , mostly including malaria , cytomegalovirus and epstein - barr virus , so a quick but thorough history and laboratory search for infection is required when spontaneous splenic rupture is diagnosed . uncommonly , rupture may complicate diffuse splenic infiltration such as in amyloidosis or gaucher 's disease , or leukemic , lymphomatous , or malignant disorders . since malaria still represents a major medical problem worldwide ( particularly in tropical and subtropical regions of asia and africa and parts of the americas ) , because of expanding travel and plasmodium resistance clinicians in the western world notably , malaria ranks first among causes of fever among travellers coming back from the tropics . when confronted with acute abdomen with signs of shock in a recent immigrant , tourist or expatriate returning from an endemic nation , malaria complicated by splenic rupture should be strongly suspected . during the course of acute malaria , splenic changes range from asymptomatic , palpable enlargement to serious complications such as infarction , rupture , hematoma , or abscess that occur more frequently in non - immune adults such as western travellers , often despite appropriate prophylaxis or during anti - malarial therapy . whereas splenic infarction has a favourable course and needs to be treated conservatively to avoid postoperative and asplenic morbidity , splenic rupture represents a life - threatening emergency with a non - negligible mortality ( 22% ) and usually requires immediate or delayed surgical splenectomy.[35 ] since clinical , laboratory , and ultrasound findings are usually insufficient to correctly differentiate these two complications , patients coming from overseas with suspected or confirmed malaria presenting to with enlarging tender splenomegaly , left upper abdomen , and/or pleuritic lower chest pain and variable - degree hemodynamic compromission require prompt imaging assessment with contrast - enhanced multi - detector ct that allows confident diagnosis and correct therapeutic choice .
splenic rupture with hemoperitoneum represents a life - threatening surgical emergency . malaria should be highly suspected as the probable underlying disease in returning travellers , expatriates , or recent immigrants from endemic countries . malarial complications involving the spleen occur even with appropriate prophylaxis or during antimalarial therapy . among them , splenic infarction has a favourable course and is treated conservatively , whereas life - threatening rupture requires immediate or delayed splenectomy . computed tomography ( ct ) allows confident differentiation between these two complications by identifying ruptured spleen with clotted hematoma and associated high - density peritoneal effusion ; furthermore , ct allows differential diagnosis from other causes of spontaneous hemoperitoneum .
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gingival hyperplasia is a bizarre condition causing aesthetic , functional , psychological and masticatory disturbance of the oral cavity . investigations are in evolution to establish the genetic linkage and heterogeneity associated with it . this condition may manifest as an autosomal - dominant or , less commonly , an autosomal - recessive mode of inheritance , either as an isolated disorder or as part of a syndrome . autosomaldominant forms of gingival fibromatosis , which are usually non - syndromic , have been genetically linked to the chromosomes 2p21-p22 and 5q13-q22 . in modern times , a mutation in the son - of - sevenless ( sos-1 ) gene has been suggested as a possible cause of isolated ( non - syndromic ) gingival fibromatosis , but no definite linkage has been established . familial gingival fibromatosis is a gradually progressive benign enlargement that affects the marginal gingiva , attached gingival and interdental papilla . the fibromatosis may potentially cover the exposed tooth surfaces , thereby hampering the function of the stomatognathic system . histopathologically , the bulbous increased connective tissue is relatively avascular and has densely arranged collagen - fiber bundles , numerous fibroblasts and mild chronic inflammatory cells . the autosomal - dominant form is often associated with hypertrichosis , corneal dystrophy , nail defects , deafnessand craniofacial deformities whereas in the autosomal - recessive form , facial anomalies with hypertelorism have been observed but most forms are without defects , other than gingival enlargement . clinical abnormalities most commonly associated with gingival fibromatosis are hirsutism , epilepsy , oliogophrenia , mental retardation , nystagmus , strabismus , cataracts , soft tissue tumors and enlarged facial bones . we report a case of non - syndromic familial gingival fibromatosis along with its management . an 8-year - old female patient had reported to the department of pediatric surgery at the government hospital with the complaint of enlargement of gums [ figure 1 , 2 ] . the patient was unable to close her mouth because of severe enlargement of gums in both maxillary and mandibular arches [ figure 3 ] . the patient was referred to the oral surgery outdoor for their opinion . after a detailed history , it was found that the patient 's brother also had the same problem [ figure 5 ] . the patient 's father was treated for the same condition in his childhood by alveolectomy with removal of primary and permanent teeth or tooth buds . she did not have any associated medical conditions , which ruled out the possibility of any syndromic involvement . an orthopentomogram was advised , which showed all primary teeth to be erupted in alveolar bone and developing permanent teeth [ figure 6 ] the treatment plan was to remove the fibrosed gingiva along with removal of mobile deciduous teeth . surgery was planned in two stages after considering the age of the patient and duration of surgery . in the first stage , the mandibular arch was operated [ figure 7 ] and the maxillary arch was operated 2 weeks later . after the second surgery , fine periodontal surgery was performed in both arches and the patient was recalled till 2 years [ figure 8 and 9 ] . on histopathologic examination , thickened acanthotic epithelium with elongated rete ridges was seen with densely arranged collagen fibers , numerous fibroblasts and few chronic inflammatory cells , suggestive of gingival fibromatosis [ figure 10 ] . initially , the patient had difficulty in closure of mouth as new occlusion had to be achieved because the patient did not have any occlusion before the surgery . right lateral extraoral view pre - operative frontal view extraoral intraoral open mouth view intraoral close mouth view orthopentomogram view post - operative intraoral view post - operative frontal extraoral view histopathology shows thickened , acanthotic epithelium , elongated rete ridges with densely arranged collagen - fibers , numerous fibroblasts and few chronic inflammatory cells gingival overgrowth varies from mild enlargement of isolated interdental papillae to segmental or uniform and marked enlargement affecting one or both of the jaws . here , we reported a case of non - syndromic familial generalized gingival fibromatosis with multidisciplinary approach . there are multiple causes of generalized gingival fibromatosis , like mouth - breathing gingivitis , drug - induced gingival overgrowth , scurvy , hereditary gingival fibromatosis , wegener granulomatosis , acanthosis nigricans and idiopathic variety . the precise mechanism of familial gingival fibromatosis is unknown , but it appears to be confined to the fibroblasts that harbor in the gingiva . the hyperplastic response does not involve the periodontal ligament , and occurs peripheral to the alveolar bone within the attached gingiva . the growth is linked with eruption of teeth as seen in the present case , and the presence of teeth may be necessary for the commencement of the process . in severe cases , non - eruption of the primary or permanent teeth may be the chief complaint of the patient . the finest and suggested treatment modality for familial gingival fibromatosis is gingivectomy . literature reports a high - recurrence rate after surgery , and needs a close follow - up .
hereditary gingival fibromatosis is a rare condition that can occur as an isolated disease or as part of a syndrome or chromosomal abnormality . in severe cases , the gingival enlargement may cover the crowns of teeth and cause severe functional and aesthetic concerns . here , we present a case of an 8-year - old girl with severe enlargement of gums in maxilla and mandible . both deciduous and permanent teeth were not erupted in the oral cavity at all . mutation in the son - of - sevenless ( sos-1 ) gene has been associated with the disease . the diagnosis was made based on clinical examination and family history . surgical removal of the hyperplastic tissue was performed under general anesthesia .
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royal free ethics committee for retrospective publication of patient data . written informed consent for publication was obtained from the patients . ma beckles and t wagner not commissioned ; peer - reviewed by vaishali sanchorawala and kaushik sanyal royal free ethics committee for retrospective publication of patient data . written informed consent for publication was obtained from the patients .
lessonamyloidosis is a rare differential diagnosis of a mass detected in the chest . amyloidoma is caused by a local proliferation of clonal b - cells secreting an unstable immunoglobulin light chain which accumulates . fdg - pet scan are useful but not specific . treatment is generally by local resection for treatment of symptoms.we report two cases of amyloidomas , which are rare entities characterised by large local amyloid deposits . these can occur in the upper respiratory tract , soft tissues and central nervous system.1
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pyoderma gangrenosum ( pg ) is a rare inflammatory disease of the skin often associated with systemic inflammatory disease or immunodeficiency . although rare , it is known to occur following trauma or operative procedures . to date nine cases we report a delayed presentation of pg in a patient undergoing mitral valve replacement who was discharged home on postoperative day seven with a normal healing incision and presented two days later with a necrotic - appearing lesion which was later diagnosed as pg . his prior medical history included repair of a cleft palate and a heart murmur . on admission , echocardiogram revealed severe mitral regurgitation with a flail and ruptured posterior mitral leaflet , a patent foramen ovale , pulmonary hypertension , and a markedly dilated left atrium without any vegetations . mitral valve repair and closure of patent foramen ovale were undertaken via a median sternotomy utilizing a short skin incision . his recovery was uneventful , and he was discharged home on postoperative day seven with a normal healing incision . physical examination revealed a stable but alarmingly swollen sternal wound with necrotic margins along the entire length ( fig . the patient was afebrile and hemodynamically stable but was admitted with a presumed wound infection and was started on intravenous vancomycin and cefepime . laboratory work demonstrated a normal white blood cell count of 8.7 , elevated c - reactive protein of 8.8 mg / dl ( normal : 0.01.0 ) , and an elevated erythrocyte sedimentation rate of 44 mm / h ( normal : 013 ) . the following day , his two chest tube sites demonstrated similar lesions . the wounds failed to improve , and after three days of antibiotics , the sternal wound was opened at the bedside and negative pressure wound therapy was initiated . similar lesions blossomed at the sites of previous internal jugular line and subcutaneous heparin injection . on hospital day five , the patient was taken to the operating room for debridement . interestingly , the wound demonstrated minimal purulence , a healing sternum , and persistent superficial necrosis . all sternal wound cultures remained negative ; however , the chest tube wound grew methicillin - resistant staphylococcus aureus . due to the lack of response to debridement and antibiotics , an 8-mm punch biopsy demonstrated granulation tissue with detached acute inflammatory exudate and marked reactive change ( fig . a diagnosis of pyoderma gangrenosum was made , and the patient was started on oral prednisone 80 mg daily . prednisone was slowly tapered starting ten days after initiation , and the patient was discharged home on a tapering dose of oral steroids . the diagnosis of pg in a postoperative setting is difficult as the wound may mimic an infection . in fact , a recent case report shows a death resulting from pg when the disease involved the saphenous vein graft . to date , all ten case reports of pg after cardiac surgery have occurred in the initial week postoperatively ( table 1 ) . what makes our case unique is the delayed presentation ( postoperative day nine ) , just 2 days after he was discharged with a seemingly normal healing wound . the clinical challenge in pg involves ( 1 ) making a prompt diagnosis and ( 2 ) choosing the appropriate treatment . pg in the postoperative period should be considered when the characteristic ulcerative lesions are seen diffusely in all surgical wounds and show minimal improvement with antibiotic treatment or debridement . this was the case in our patient , who presented with necrotic lesions not only all along on his sternotomy wound , but also involving his chest tube sites and his central venous catheter site . these did not show any improvement after a course of antibiotics nor after debridement in the operating room . once suspected , prompt dermatology consultation and skin biopsy aids in confirming the diagnosis . the second challenge is selecting the appropriate treatment as there is no established standard . in previous reports of pg after cardiac surgery , treatments with prednisone , cyclosporine a , or a combination of both have been successful . our patient was treated with a course of oral prednisone alone and showed rapid and marked improvement . although the pathology of pg is not infectious , the lesions compromise the skin barrier significantly , and pg is known to be associated with an underlying immuno - deficient state . in our patient , antibiotics were administered for a presumed wound infection . in fact , one of the wounds from our patient grew methicillin - resistant staphylococcus aureus . while there is no evidence to initiate antibiotics , we do recommend coverage for super - infection in conclusion , pg is a rare complication after cardiac surgery , yet an important entity to recognize as prompt diagnosis is necessary to initiate appropriate treatment . while all other reported cases have occurred within the first week of postoperative period , our case demonstrates that pg can present in a delayed fashion . pg should be suspected when ulcerative lesions appear acutely and diffusely in all surgical wounds and respond minimally to debridement and antibiotic treatment . dh was involved in study design and writing.key learning pointspyoderma gangrenosum can occur following cardiac surgery and can mimic wound infection.making the clinical distinction between pyoderma gangrenosum and wound infection is very important as the treatment is different . pyoderma gangrenosum can occur following cardiac surgery and can mimic wound infection.making the clinical distinction between pyoderma gangrenosum and wound infection is very important as the treatment is different . pyoderma gangrenosum can occur following cardiac surgery and can mimic wound infection . making the clinical distinction between pyoderma gangrenosum and wound
introductionpyoderma gangrenosum ( pg ) is a rare , ulcerative inflammatory skin pathology frequently associated with systemic inflammatory disease . while rare after surgery , recognition of this disease in the post - surgical setting is important as it can mimic wound infection . there have been ten case reports to date of pg occurring immediately after cardiac surgery , with all of them presenting within the first week post-operatively.presentation of casewe herein present a delayed and dramatic presentation of pg nine days after mitral valve replacement and repair of patent foramen ovale , two days after being discharged with a seemingly normal healing wound . diagnosis of this disease in the postoperative period requires high suspicion when the characteristic ulcerative lesions are seen diffusely in all surgical wounds and show minimal improvement with antibiotic treatment or debridement.discussion/conclusionour case highlights the importance of recognizing this disease in the postoperative period , even in a delayed presentation and initially limited to one of the surgical sites . this case calls for an awareness of this disease entity amongst cardiac surgeons as well as intensivists .
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the serum anion gap is a helpful parameter in the clinical diagnosis of various conditions . the commonest application of the anion gap is to classify cases of metabolic acidosis into those that do and those that do not have unmeasured anions in the plasma ( table 1 ) . in this article , we briefly review the significance of the anion gap and the approach to the use of the serum anion gap . as charge balance precluded the existence of any gaps , the more accurate term should really be ' difference between unmeasured anions and unmeasured cations ' which obviously lacks the brevity requisite for practical communication . clinically , anion gap is equal to the difference between the plasma concentrations of the major cation ( na ) and the major measured anions ( cl+hco3 ) . it is important to understand that this entity actually equals [ anionic proteins+inorganic phosphate+sulphate+organic anions]-[potassium+calcium+magnesium+cationic proteins ] ( fig . since there are more unmeasured anions than unmeasured cations , the value of anion gap is usually positive . meq / l , when sodium was determined by flame photometry ( based on the principle of flame atomic emission spectrometry ) and chloride by a colorimetric assay ( mercuric - nitrate - thiocyanate colorimetric assay ) . since the 1980s , ion - selective electrodes for specific ionic species were used for the measurement of serum electrolytes . the difference between the ionic concentration in the electrode ( known ) and the sample creates an electrical potential ( measured ) and the sample ionic concentration the measurement by ion - selective electrodes has caused a shift of the anion gap from 124 meq / l down 63 meq / l2 ) . it is worthy for clinicians to understand the range of normal anion gap and the measuring methods for serum sodium and chloride in the laboratories that support their practice . it is important because an increased anion gap usually is caused by an increase in unmeasured anions , and that most commonly occurs when there is an increase in unmeasured organic acids , that is , an acidosis3 , 4 ) . acids ( eg , lactate and pyruvate ) are protons donors and must be buffered by bicarbonate . the consumption of bicarbonate by the unmeasured anions will increase the anion gap by lowering the serum bicarbonate level . the total numbers of anions and cations are still equal , but the gap is increased because of a lowering of a measured anion , serum bicarbonate . the most common ones can be remembered by the mnemonic mudpiles : methanol , metformin , uremia , diabetic ketoacidosis , ethylene glycol , salicylates and starvation5 , 6 ) . these conditions produce an acid load that consumes bicarbonate , increases the anion gap , and lowers serum ph . if the patient is acidotic and has an elevated anion gap , it is almost certainly caused by one of these conditions , each one with us characteristic signs , symptoms , and laboratory values . whereas the presence for a high anion gap educes the consideration of a differential diagnosis by reflex in daily clinical practice , a low anion gap often does not elicit the same warning to clinicians and hence often remains either undiscovered or neglected . the classical differential diagnosis of a low anion gap has changed since the ion - selective electrode has been introduced . first , it can be an early and sometimes only sign of an underlying disease process such as paraproteinemia . in addition to displacement of sodium - containing water from serum by large amounts of non - sodium - containing paraproteins , some paraproteins ( eg , igg in multiple myeloma ) can have a net positive charge at physiological ph . this leads to an increase in unmeasured cations and a low anion gap7 , 8) . concomitant severe hypercalcemia and hypoalbuminemia are often contributing factors to a low anion gap in multiple myeloma9 ) . since the only cation included in the anion gap calculation is sodium , severe hyperkalemia , hypercalcemia , hypermagnesemia or lithium intoxication theoretically can also lead to a significantly decreased anion gap . second , at normal serum ph of 7.4 , the majority of plasma proteins are anionic . albumin with an average negative charge of 18 per mole at physiological ph has been shown to be responsible for approximately 75% of the unmeasured anions of the normal anion gap . a drop in albumin by 10 g / l therefore will cause the anion gap to fall by approximately 2.5 meq / l at constant ph10 , 11 ) . third , a low anion gap can mask an underlying high anion gap acidosis and potentially delay intervention . while an increase in the anion gap is almost always caused by retained unmeasured anions , a decrease in the anion gap can be generated by multiple mechanisms . in this short review , emphasis is placed on the fact that the serum anion gap is the difference between the unmeasured anions and the unmeasured cations , and any numerical analysis of this entity needs to take this equation into consideration . by exploring all the possible factors that can influence unmeasured cations and unmeasured anions it is advisable for the clinician to know the normal range of the anion gap and the assays used in measuring na and cl in the laboratories supporting their practice .
the anion gap in the serum is useful in the interpretation of acid - base disorders and in the diagnosis of other conditions . in the early 1980s , ion - selective electrodes for specific ionic species were introduced for the measurement of serum electrolytes . this new method has caused a shift of the anion gap from 124 meq / l down 63 meq / l . it is worthy for clinicians to understand the range of normal anion gap and the measuring methods for serum sodium and chloride in the laboratories that support their practice . while an increase in the anion gap is almost always caused by retained unmeasured anions , a decrease in the anion gap can be generated by multiple mechanisms .
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a 44-year - old female without any previous medical history visited our clinic because of the sudden onset of intermittent blurred vision in her left eye . her best - corrected visual acuity ( bcva ) was 20 / 22 and fundus examination revealed multiple retinal , preretinal , and subretinal hemorrhages throughout the retina with tortuous retinal veins in her left eye ( fig . arteriovenous transit time was slightly prolonged to 19 seconds and there was faint leakage in the late phase , but no signs of any vascular non - perfusion ( fig . cardiologic and neurologic work - up and laboratory tests ( homocysteine , protein c and s , fibrinogen , anticardiolipin antibodies , and lupus anticoagulants ) were normal except for weakly positive anticardiolipin igm and mild iron deficiency anemia . we decided to observe the patient for the following two weeks without making a diagnosis . two weeks later , her bcva was slightly decreased to 20 / 25 , and an increased number of retinal hemorrhages with severe disc swelling were noted ( fig . at this time , the patient preferred to undergo medical intervention to improve her subjective symptom . we discussed with her that the drainage site of the retinal vein is located in the lamina cribrosa within the optic nerve and an intravitreal dexamethasone implant may help alleviate the progression of the vein occlusion by reducing optic nerve edema . the patient agreed to the treatment and an intravitreal dexamethasone implant 0.7 mg was injected . five days later , her bcva was 20 / 22 and there were improvements in disc swelling and retinal hemorrhage ( fig . one month later , her bcva improved to 20 / 20 and her subjective visual symptom was completely improved . fundus examination revealed marked improvement of retinal hemorrhages and vascular tortuosity along with almost complete resolution of disc swelling ( fig . a compartment syndrome at the site of the lamina cribrosa is thought to be the key mechanism in the pathophysiology of crvo . the current treatment strategy for crvo is typically directed to complications such as me or neovascularization rather than the venous occlusion itself . although many studies have already shown the benefits of anti - vascular endothelial growth factors or steroids for the treatment of crvo - associated complications , this case suggests that even in early crvo , if associated with optic nerve swelling , steroid treatment could decrease disc swelling , thereby relieving compartment obstruction and improving vascular hemorrhage . steroids may reduce the disc swelling , which subsequently decreases the pressure of the scleral ring and may improve the venous outflow . this may potentially change the natural course of disease progression of crvo and its various subsequent complications .
this case describes the reversal of early central retinal vein occlusion ( crvo ) with disc swelling after intravitreal dexamethasone implant ( ozurdex ) injection . a 44-year - old female presented with sudden - onset intermittent blurred vision in her left eye . fundus examination revealed multiple retinal hemorrhages without macular edema ( me ) . two weeks later , an increased number of retinal hemorrhages with severe disc swelling were noted with still no sign of me . an intravitreal dexamethasone implant was injected . five days later , there were improvements in disc swelling and retinal hemorrhage . one month later , her subjective visual symptoms were completely improved , and fundus examination revealed marked improvement along with almost complete resolution of disc swelling . intravitreal dexamethasone implant injection may potentially change the natural course of crvo progression and its various subsequent complications .
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cutaneous crohn 's was first described by parks et al . , in 1965 , refers to extra intestinal mucocutaneous manifestations of crohn 's disease in which there are noncaeseating , granulomatous mucocutaneous lesions preceding or manifesting after systemic manifestations of intestinal crohn 's disease . other dermatological conditions found to be associated with crohn 's disease include pyoderma gangrenosum , erythema nodosum , erythema multiforme , epidermolysis bullosa acquisita , polyarteritis nodosa and vitiligo . here , we report a case of anogenital crohn 's and its rare association with vitiligo . the case we present here is about a 23-year - old female , presented with vitiligo patch on legs and back for 1 year and vulval swelling with anogenital and oral erosions for 9 months [ figure 1 ] . there were multiple , painful , nonhealing erosions seen on anogenital area and oral cavity [ figures 2 and 3 ] . she also had on- and off - history of loose stools for last 1 month . investigations on admission had shown low hemoglobin 10 gm% , total count of 7100/cumm , differential count of n : 58 , l : 34 , m : 4 , e : 4 , b : 0 , platelet count 4.89l acs / cumm . erythrocyte sedimentation rate was 40 mm in 1 h , blood urea 10 mg / dl , serum creatinine 1 mg / dl , random blood sugar 80 mg / dl . serum glutamic pyruvic transaminase 10 u / l , serum glutamate - oxaloacetate transaminase 54 u / l , serum bilirubin 0.8 mg / dl , prothrombin time 14 s , activated partial thromboplastin time 34 s , total protein 5.3 g , albumin : globulin 2.9:2.4 . koh smear was taken from vaginal discharge , which was negative and report of gram - stain is not available . ultrasonography ( usg ) of patient showed 17 cm bowel segment with 15 mm thickened wall and narrowing of the lumen with the diagnosis of inflammatory bowel disease . multislice computed tomography ( ct ) scan of the abdomen with pelvis showed mild circumferential wall thickening with stratification and increased enhancement . findings were in favor of crohn 's with early sacroillitis . colonoscopy showed dilated bowel segment with edema , erythema , telangiectasia , and few ulcers [ figure 4 ] . biopsy from anal wall showed acute as well as chronic inflammation with noncaseating granulomas suggestive of crohn 's disease [ figure 5 ] . patient was treated with tablet mesalamine ( 800 mg ) eight hourly and capsule doxycycline ( 100 mg ) and neomycin cream on ulcers . clinical photograph of vitiligo patches on legs clinical photograph of patient showing vulval edema clinical photograph showing anal erosions colonoscopy showing erosions in the terminal ileum histopathological picture crohn 's disease , first described by sir crohn et al . in 1932 , is a type of inflammatory bowel disease that may affect any part of the gastrointestinal tract from mouth to anus . it affects 1 in 400,000 - 600,000 people globally with male : female ratio of 1:1.2 . the age of onset of cutaneous crohn 's is 10 - 69 years . in our case , the basic underlying etiology of crohn 's is currently unknown , though various theories suggest an interaction between environmental , immunological and bacteriological factors in genetically susceptible individuals ( nod2 gene ) . the lesions in cutaneous crohn 's can be classified as : ( 1 ) specific lesions of cutaneous crohn 's disease , ( 2 ) reactive dermatoses , ( 3 ) cutaneous manifestations secondary to malabsorption , and ( 4 ) cutaneous manifestations secondary to treatment . cutaneous crohn 's disease can also be divided clinically into two forms : genital ( 56% ) and extra genital ( 44% ) . approximately , two - third of children and half of adults with crohn 's disease present with genital involvement . anogenital signs and symptoms ( in approximately 25% in illeal and 80% in colonic involvement ) may appear before the other systemic manifestations and may be the first sign in the diagnosis of crohn 's disease . cutaneous manifestations associated with crohn 's disease are erythema nodosum , pyoderma gangrenosum , epidermolysis bullosa acquisita , polyarteritis nodosa , and vitiligo . in our case , association of crohn 's disease and vitiligo has been seen in other studies also . in the study by tanusin et al . mcpoland and moss have reported a case of crohn 's disease and vitiligo . in both studies , the differentials of chronic vulval edema with erosions are granulomatous vulvitis , sarcoidosis , and chronic lymphedema due to obstruction , tuberculosis , subcutaneous mycoses , hidradenitis suppurativa and langerhans cell histiocytosis . clinically , presence of chronic erosions , swelling , and erythematous discoloration of genitals with gastrointestinal symptoms and on investigation typical findings in usg , ct scan and colonoscopy favors diagnosis of crohn 's disease . absence of lymphadenopathy , vegetating growth , scarring , and negative x - ray findings excludes other differential conditions . although histological picture of cutaneous crohn 's may be indistinguishable from other granulomatous diseases , but histopathology of skin along with colonoscopy findings together leads to diagnosis of crohn 's disease . many treatment modalities are available as systemic steroids , sulfasalazine , mesalamine ( active moiety of sulfasalazine ) , oral metronidazole , hyperbaric oxygen , and antitumor necrosis factor- antibody ( infliximab ) . our patient was treated symptomatically with tablet mesalamine ( 800 mg ) eight hourly , capsule doxycycline ( 100 mg ) and topically neomycin cream and was referred to higher center for operative procedures .
cutaneous crohn 's or anogenital granulomatosis is a rare disorder , which presents commonly as swelling and erosions on anogenital area and associated with features of intestinal crohn 's disease . we report a case of 23-year - old female who presented with vitiligo patches on back and legs for 1-year , painful edema and ulcers on anogenital area and oral cavity for 9 months and diarrhea with bleeding per annum for 1 month . features on ultrasonography , multislice computed tomography scan , colonoscopy , and biopsy from nodule on anal wall were suggestive of granulomatous crohn 's disease . thus , we present the case due to its rarity .
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the so - called schloffer tumor ( st ) is a rare inflammatory pseudotumor with aggressive connective tissue proliferation and frequent infiltration of neighboring abdominal organs . it usually appears several years after abdominal surgery or trauma , and causes substantial problems with the interpretation of clinical and morphological findings . there are few case - reports published and all of them show tumors in the surgical site of the previous surgery . a 32-year - old man was referred to our hospital complaining of a painful mass in the left hypochondrium , postprandial distension and a weight loss of about 14 kg in the previous three months . ultrasonography ( usg ) of the abdomen showed a 2 cm 2 cm hypoechoic lesion in contact with the abdominal wall ( fig . 1 ) . computerized tomography ( ct ) of the abdomen showed a heterogeneous mass in the great omentum below the stomach and above the transverse colon ( fig . laparoscopic exploration revealed an omental mass firmly attached to the abdominal wall . a great deal of purulent fluid spread during the procedure . due to the difficult exploration of the abdominal cavity , the procedure was converted to hand assisted laparoscopy by means of a wound retractor ( alexis , applied medical , rancho santa margarita , ca , usa ) , enlarging 4 cm the incision of the 5 mm trocar on the left hypochondrium . we find an omental tumor involving the stomach and the transverse colon . inside the mass there were purulent material and non - absorbable polypropylene sutures ( figs . 3 and 4 ) . debridement of the wall abscess was performed and a piece of the wall was sent for frozen histology examination which proved intra - operative , negative for neoplasic cells . an intra - operative fistulography revealed that the abscess cavity was not communicated with the bowel lumen . this tumor was first described by schloffer in 1809 . he evaluated four patients with chronic inflammatory tumor of the abdominal wall following inguinal hernioplasties . bacteriologic study reveals low virulent germ , generally staphylococcus , and a thick wall of fibrous tissue . the volume varies from a small tumor to giant masses of 2 or 3 kg with a central abscess . histological examination reveals abundant fibrous granulation tissue and lymphocyte infiltration caused by foreign body reaction . these tumors occur postoperatively after an undetermined free period , which can range from months to years . in abdominal surgery it has been described after appendectomy , hernioplasty , hysterectomy , oophorectomy , gastrectomy , cholecystectomy , biliary surgery or colonic resections . abdominal wall , stomach , colon , omentum , testis , and bladder can be invaded . the tumor - like symptoms predominate over inflammatory symptoms , and general status is not affected . the sonographic appearance of suture granulomas is characterized by hyper echoic double lines ( rail - like lines ) or single lines within a hypoechoic lesion . it was suggested that st should also be taken into consideration as differential diagnosis in patients with a history of past operations and cumulative operative wounds of the abdomen revealed by fdg - pet . accumulation images in fdg ( ring - shaped appearance ) should alert surgeons of the possibility of diagnosing foreign body granuloma , in addition to patient 's history , us and ct findings . some authors suggest that complete tumor resection should always be performed , and others just simply drain the tumor . in our case , we only performed abscess drainage and removal of non - absorbable suture material after histological confirmation of benignity . the interesting thing about this case is the appearance of the tumor after a one - year interval , in a place far away from surgical site . the patient relates he had had a large left hernia , surely with omentum inside . when a tumor appears after surgery , st should always be suspected . in many laparotomies performed on daily basis using non - absorbable suture material , we suggest that a mini - invasive approach to these tumors should always be performed . complete resection is not required , if we have a negative frozen histologic examination . a simple drainage and removal of suture material , without major resections , solves the problem of these patients . the authors have no financial and personal relationships with other people or organizations that could inappropriately inuence ( bias ) this submission . written informed consent was obtained from the patient for publication of this case report and its accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request . carlos m. canullan , nicolas f. baglietto and roberto f. klappenbach : performed the operation . juan alvarez rodriguez , luis t. chiappetta porras : study design and interpretation.key learning pointsschloffer tumor is an inflammatory pseudotumor.it usually appears several years after abdominal surgery.we suggest a mini - invasive approach , a simple drainage and removal of suture material , without major resections . schloffer tumor is an inflammatory pseudotumor.it usually appears several years after abdominal surgery.we suggest a mini - invasive approach , a simple drainage and removal of suture material , without major resections . we suggest a mini - invasive approach , a simple drainage and removal of suture material , without major resections .
introductionthe so - called schloffer tumor ( st ) is a rare inflammatory pseudotumor . it usually appears several years after abdominal surgery or trauma.presentation of casea 32-year - old man was referred to our hospital complaining of a painful mass in the left hypochondrium , postprandial distension and a weight loss of about 14 kg . he had had a left inguinal hernioplasty without mesh the previous year . ultrasonography of the abdomen showed a 2 cm 2 cm hypoechoic lesion in contact with the abdominal wall . computerized tomography of the abdomen showed a heterogeneous mass in the great omentum.laparoscopic exploration revealed an omental mass firmly attached to the abdominal wall . a great deal of purulent fluid spread during the procedure . due to the difficult exploration , the procedure converted to hand assisted laparoscopy . we find an omental tumor involving the stomach and the transverse colon . inside the mass there were purulent material and non - absorbable sutures . a drain was left inside the cavity of the abscess . histological examination showed chronic inflammation.discussionst characteristically presents a central chronic abscess containing non - absorbable sutures . it has been described after appendectomy , hernioplasty , hysterectomy , gastrectomy or colonic resections . although benign , its progressive growth and infiltrating behavior resemble malignant tumors.conclusionwe suggest that a mini - invasive approach should always be performed . the interesting thing about this case is the appearance of the tumor in a place far away from the previous surgical site . a simple drainage and removal of suture material solves the problem of these patients .
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thyroid gland is rarely affected by tuberculosis , even in countries where tuberculosis is common . children constitute very small proportion of these cases and according to terzidis et al . and razmpa et al . we present a case of 11-year - old female child of thyroid tuberculosis who presented with a solitary thyroid nodule for 2 months and was diagnosed on cytological examination . the present case is about an 11 years female child presented with a history of swelling in the thyroid region for preceding 2 months . on physical examination , thyroid gland was enlarged measuring 4.0 2.5 cm , firm in consistency and moving with deglutition . computed tomography ( ct ) scan neck showed large bilateral lobes of thyroid right measuring 21 13 mm and left 16 18 mm showing heterogeneous tissue density with evidence of necrosis , bilateral cervical lymphadenopathy . x - ray chest revealed non - homogenous opacity in the lower zone of lung on the right side . enlargement of hilar and mediastinal lymph nodes , right side minimal pleural effusion and pleural thickening were found on ct scan of the thoracic cavity . ultrasound of the abdomen revealed enlarged lymph nodes in periportal , portal , peripancreatic para - aortic regions . hemoglobin was 10% g and erythrocyte sedimentation rate was 40 mm in 1 h. peripheral smear of blood revealed microcytic hypochromic anemia . t3 , t4 and thyroid stimulating hormone were within the normal limits and anti - thyroid peroxidase antibody was negative . on fine - needle aspiration from the thyroid swelling , thick cheesy fluid was aspirated . the fluid was centrifuged and smears of the sediment showed caseating necrosis , few epithelioid cell granulomas and occasional benign follicular epithelial cells [ figures 1 and 2 ] . based on caseating necrosis , epithelioid cell granulomas and demonstration of afb , diagnosis of thyroid tuberculosis was made . with treatment with antitubercular drugs , benign follicular cells and background of caseating necrosis ( giemsa stain , 400 ) epithelioid cell granuloma with caseating necrotic background ( giemsa stain , 400 ) the estimation of prevalence of this condition is difficult . however , reported prevalence rate varied from 0.1% to 1% . rarity of tuberculosis in thyroid has been attributed to bactericidal property of colloid , high vascularity and excess of iodine , enhanced activity of phagocytes in hyperthyroidism and possible antitubercular roles of thyroid hormone . involvement of thyroid by tuberculosis can occur by hemetogenous and lymphatic routes or by direct extension from cervical lymphadenitis . earlier the criteria described for diagnosis of thyroid tuberculosis were finding of afb within thyroid , a necrotic or abscessed gland and presence of tuberculous focus outside the thyroid . since it is not always possible to demonstrate afb , recently it is stated that multiple coalesced and caseating epithelioid cell granulomas along with giant cells are considered to be diagnostic of thyroid tuberculosis . in our case , the diagnosis of tuberculosis of thyroid was confirmed by demonstration of tubercular bacili in the specimen . other extra thyroid lesions were also found in the present case which is similar to findings noted in many previous reports . although pathological assessment of these extra thyroid tissues was not done in the present case to avoid invasive investigations , however , regression of these lesions with anti - tubercular drugs lends evidence to the possible tuberculous etiology in these lesions as well . one of the peculiarities of our case is that involvement of large number abdominal lymph nodes ( periportal , portal , peripancreatic para - aortic regions ) besides cervical , mediastinal lymph nodes and pulmonary lesion . to the best of our knowledge , no case of thyroid tuberculosis was reported in the past wherein such widespread extra thyroid involvement was observed . usually the tubercular involvement of lymph nodes in such patients was limited to one or two lymph node regions with or without pulmonary tuberculosis . khan et al . in their study have reported four cases of thyroid tuberculosis , among them one case had cervical and axillary lymphadenopathy , another case had tuberculosis of cervical lymph nodes and third one had pulmonary tuberculosis and remaining one case did not reveal any involvement of extra thyroid tissue . likewise in the case reported by oklah and al - kaisi concomitant pulmonary tuberculosis and tubercular mediastinal lymphadenitis was found and mondal and patra in a study of 18 cases of thyroid tuberculosis found three cases had associated tubercular cervical lymphadenitis and four cases had pulmonary tuberculosis . other atypicality is the younger age of our case , only very few cases were reported in this age group in the past , the most of previously reported patients were adults with the median age around 40s and . there are case reports that suggest the possibility of primary thyroid tuberculosis in which there is no known focus of tuberculosis outside thyroid and such condition is even more uncommon . like in current case , thyroid function test is generally found to be normal in these patients , but cases of thyrotoxicosis and hypothyroidism have also been reported . being a rare entity , tuberculosis as the cause of thyroid swelling is unlikely to be suspected clinically . however , like in the present case , fine - needle aspiration has also been found to be diagnostic in cases of thyroid tuberculosis thus preventing the unnecessary surgery . differential diagnosis of granulomatous inflammation in thyroid includes subacute thyroiditis , goitrous autoimmune thyroiditis , tuberculosis and sarcoidosis . it may be concluded that in cases of thyroid swellings tubercular thyroiditis should be considered as one of the differential diagnosis .
thyroid tuberculosis is a rare disease even in countries where tuberculosis is endemic . clinically tuberculosis is not often suspected in cases of thyroid nodule or swelling . we report a case of 11 years female child who presented with a thyroid swelling . fine - needle aspiration cytology revealed caseating epithelioid granulomas and acid fast bacilli . patient improved with antitubercular drugs . tuberculosis may be considered as differential diagnosis of thyroid swelling .
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in alzahra university hospital of isfahan , iran , the a 42-year - old homemaker woman , who used to suffer from rheumatoid arthritis since 2004 was admitted . during this time , rheumatoid factor and anti - cyclic citrollinated peptide ( anti - ccp ) always had been negative . in this period , she had wrists , shoulders , knees , and ankles involvement , and never been improved completely . the patient was under the treatment of oral methotrexate ( 10 mg per week ) , sulfasalazine ( 1500 mg / day ) , diclofenac ( 50 mg / day ) , and low dose prednisolone ( 7.5 mg / day ) . dose escalation did not improve disease activity score of 28 joints ( das28 > 5.1 ) and laboratory parameters . she was admitted to the hospital due to refractory joints pain , tenderness , leukocytosis ( 12 10/ul ) , and elevated esr ( 65 mm / h ) and crp ( 42 mg / l ) . in addition , she received steroid pulses ( iv methylprednisolone sodium succinate 500 milligram ) during each admission . she was the candidate for biologic therapy , but she could not afford it . due to this problem after four weeks , she felt better , and ten - derness decreased , but swelling continued . esr ( 35 mm / h ) and crp ( 22 mg / l ) improved slightly ; but during this period , blood pressure and serum creatinine increased . unfortunately , by tapering the drug dosage , the clinical and laboratory signs recurred . in respect of anti - inflammatory effect of pamidronate , after signing the inform consent by the patient , we decided to start single infusion of 60 mg drug for her . ( in a 3-month period , patient received three infusions of 60 mg pamidronate ) . after the second infusion , all the clinical and laboratory indices of the patient resolved completely . disease activity index decreased to less than 2.6 ( das28 < 2.6 ) ( table 1 ) . das28 and changes of its components during the pamidronate infusion courses ( first case ) by ameliorating of the joint tenderness and swelling , we discontinued cyclosporine and tapered other drugs gradually . after three months of the last drug injection , the disease was under control by 7.5 mg of oral methotrexate per week and 1 gram of oral sulfasalazine per day . in alzahra university hospital of isfahan , a 23-year - old homemaker suffered from rheumatoid arthritis since 2007 , was addmitted . in the disease course , she had right wrist , elbows , shoulders , jaw , knees , and ankles involvement . in the laboratory examinations , she had a negative rheumatoid factor and anti - ccp , elevated esr ( 87 mm / h ) and crp ( 45 mg / l ) , and leukocytosis ( 13.4 10/ul ) . in the disease course , she was under the treatment of oral methotrexate ( 10 mg / week ) , sulfasalazine ( 2000 mg / day ) , naproxen ( 500 mg / day ) , and prednisolone ( 7.5 mg / day ) . this patient , as the previous case , could not afford biologic drugs . in this regrad , we started oral cyclosporine a ( 3mg / kg ) due to refractoriness to conventional dmards . after three weeks , patient felt better and disease activity index , esr , crp and leukocytosis improved . however , after the dose reduction in each time , clinical and laboratory signs of disease relapsed . in this respect , after signing the inform consent by the patient , we decided to start intravenous pamidronate 60 mg per month until three months . after four weeks , laboratory and clinical signs of the patient decreased and after eight weeks , the entire patient 's joint tenderness improved . das28 and changes of its components during the pamidronate infusion courses ( second case ) even so , we gradually started to tapered drugs . after 3 months of the last infusion , the patient only received one - gram oral sulfasalazine per day and other drugs were discontinued . rheumatoid arthritis is a disabling disease that in some cases is refractory to conventional dmards . rheumatoid factor is positive in most of the cases , but some cases are seronegative and resistant.3 biologic drugs most commonly are used for refractory cases to conventional dmards,6 but they are expensive . pamidronate is a cheap interavenous bisphosphonate that has some anti - inflammatory properties by increasing apoptosis of monocytes.2 we reported two cases of severe seronegative ra that responded to this drug . by this report , we can schedule a more extensive research in this subject to identify the useful property of pamidronate . ms and pm carried out the design and coordinated the study , participated in data collection and prepared the manuscript .
pamidronate is a bisphosphonate derivative that can inhibit bone resorption by actions on osteoclasts and increase bone density in spite of treatment with steroids . this drug has the anti - inflammatory effect by increase apoptosis of monocytes . 5 - 10 percent of rheumatoid arthritis patients is seronegative and may be resistant to conventional disease modifying anti rheumatic drugs ( dmards ) . intravenous ( iv ) pamidronate can be effective in disease control in seronegative rheumatoid arthritis . we report two cases of seronegative and drug resistant rheumatoid arthritis that favorably responds to pamidronate .
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we present a case of an 82-year - old female , an immigrant from brazil , with past medical history of atrial fibrillation , colon cancer , and uterine cancer status post abdominal surgeries on remission who presented to our emergency room with severe abdominal discomfort , nausea , and 78 episodes of bilious vomiting without hematemesis . she did not have any constipation or diarrhea , and her last bowel movement was a day prior to presentation . she was saturating 93% in room air and her temperature was 97.8f . physical examination revealed a diffusely tender abdomen with rebound but no guarding or rigidity was present . she had a hemoglobin level of 13 g / dl , a white blood cell count of 10,000 per microliter , and a platelet count of 80,000 per microliter . she had a lactate level of 1.8 mmol / l , and her lipase level was 29 abdominal x - ray showed centrally dilated small bowel with large amount of stool within the rectum . computed tomography ( ct ) scan of the abdomen showed circumferential intramural air within the proximal mid stomach ( figs . 1 and 2 ) and intrahepatic portal venous air within the left lobe of the liver ( figs . 3 and 4 ) consistent with emphysematous gastritis with no evidence of small bowel obstruction . ct abdomen showing intramural air ( yellow arrow ) within the proximal mid stomach consistent with emphysematous gastritis . ct abdomen / coronal view showing intramural air ( yellow arrow ) within the proximal mid stomach consistent with emphysematous gastritis . ct abdomen / coronal view showing portal venous air ( yellow arrow ) within the left lobe of the liver . ct abdomen showing portal venous air ( yellow arrow ) within the left lobe of the liver . she was treated with intravenous hydration , intravenous vancomycin , cefepime , and metronidazole and kept npo ( nil per os ) . two conditions are included in the differential diagnosis of intramural gas in stomach : emphysematous gastritis and gastric emphysema ( 1 ) . emphysematous gastritis , first described in 1889 , is a rare form of phlegmonous gastritis caused by gas - forming organisms invading the stomach wall through a mucosal defect . recent systematic review suggested a mortality rate of 55% for emphysematous gastritis and 29% for gastric emphysema ( 4 ) . the most common predisposing factors for emphysematous gastritis include ingestion of caustic substances , alcohol abuse , abdominal surgery , diabetes , immunosuppression , and phytobezoars ( 2 , 3 ) . abundant blood supply , effective mucosal barrier , and an acidic ph make the stomach a very uncommon site for this finding ( 5 ) . , clostridium spp . , pseudomonas aeruginosa , staphylococcus aureus ( 2 , 3 ) , sarcina spp . previous reports have suggested a high mortality rate in cases with the finding of intramural stomach gas in association with hepatic portal venous gas ( 8 , 9 ) . gastric emphysema is a more benign condition in which air usually reaches within the wall from outside ( 1 ) . the predisposing factors for gastric emphysema are mucosal disruption due to procedures like endoscopy ( 10 ) or nasogastric tube placement ( 11 ) , increased intraluminal pressure secondary to pyloric or enteral stenosis ( 12 , 13 ) , gastric paresis and forceful vomiting , and chest wall injuries . it is important to differentiate emphysematous gastritis from gastric emphysema mostly because of the difference in management and prognosis . emphysematous gastritis usually presents in a sick , hemodynamically unstable patient and has increased mortality ( 15 ) . it might also have an increased association of radiological finding of gas in the portal venous system in addition to the intramural stomach gas ( 5 ) . in contrast , patients with gastric emphysema generally present with milder symptoms of dyspepsia and epigastric discomfort or may be asymptomatic . they are usually managed with conservative management and observation ( 15 ) . a cystic mottled pattern is usually associated with emphysematous gastritis , while linear lucency along the greater curvature of the stomach is present in gastric emphysema . radiologists use terminologies like linear , curvilinear , or cystic to describe non - gangrenous changes , whereas bubbly signifies gangrenous gastritis ( 14 , 15 ) . our patient had prior history of multiple cancers and advanced alzheimer 's disease and was bed bound . we concluded that she developed emphysematous gastritis secondary to her immunosuppressed status and possible mucosal tears in the stomach from multiple bouts of vomiting . emphysematous gastritis was diagnosed based on the characteristic radiographic signs and a good response to antibiotics . the radiological finding of intramural stomach gas and portal venous air predicted a poor prognosis . however , she had a stable hospital course and resolution with medical management , likely due to early diagnosis and initiation of appropriate treatment . the authors have not received any funding or benefits from industry or elsewhere to conduct this study .
emphysematous gastritis is a severe and rare form of gastritis with characteristic findings of intramural gas in the stomach . it is an acute life - threatening condition resulting from gas - producing microorganisms invading the stomach wall . early diagnosis and initiation of treatment with bowel rest , hydration , and intravenous broad - spectrum antibiotics is imperative for an effective outcome . surgical intervention is reserved for perforations , peritonitis , strictures , and uncontrolled disseminated sepsis . we present a case of an 82-year - old female with prior history of colon and uterine cancer on remission treated with surgeries who presented with bilious vomiting , abdominal discomfort , and nausea . she was tachycardic and had a diffusely tender abdomen with rebound on examination . her laboratory results including blood count , serum chemistry , and coagulation studies were normal . she was diagnosed with emphysematous gastritis based on the characteristic radiographic findings of intramural stomach gas and also the presence of gas in the portal venous system . it is important to differentiate emphysematous gastritis from gastric emphysema because of the difference in management and prognosis , as emphysematous gastritis has a worse outcome and requires aggressive management . despite an anticipated poor prognosis due to the known grave outcomes of emphysematous gastritis , our patient was successfully managed with conservative treatment . we concluded that she developed emphysematous gastritis probably secondary to immunosuppression and possible mucosal tears from multiple bouts of vomiting . she had a stable hospital course and resolution with medical management most likely due to early diagnosis and initiation of appropriate treatment .
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cardiac calcified amorphous tumors ( cats ) are extremely rare cardiac masses which can arise in all four cardiac chambers.1)2 ) while several causes of cardiac cats have been suggested , the true etiology is not still clear . cardiac cats are usually benign , but sometimes cause diverse symptoms due to obstruction or embolization.1)3 ) we recently encountered a patient with a cardiac cat causing multiple , calcific , pulmonary emboli and right - sided heart failure . a cardiac cat has not been reported previously in korea . a 33-year - old man sought evaluation in our outpatient clinic for progressive pretibial pitting edema and shortness of breath on exertion . there was no personal or family history of thromboembolic , hematologic , inflammatory , or malignant diseases . the physical examination revealed mild icteric sclera , a palpable liver , a distended abdomen , and jugular vein distention . a chest x - ray showed a dense calcification within the cardiac silhouette from the left lateral view . an electrocardiogram revealed incomplete right bundle branch block , right atrial enlargement , and right ventricular hypertrophy . echocardiography demonstrated a diffuse calcified mass affecting the tricuspid chordal apparatus and the free wall of the right ventricle ( rv ) , resulting in severe tricuspid regurgitation and markedly increased rv systolic pressure ( 70 mmhg ) ( fig . 1 ) . on laboratory analysis , the serum parathyroid hormone of 50.07 pg / ml ( normal , 15 - 65 pg / ml ) , calcium of 8.1 mg / dl ( normal , 8.0 - 10.5 mg / dl ) , creatinine of 1.0 mg / dl ( normal , 0.5 - 1.4 mg / dl ) , and glucose of 100 mg / dl ( normal , 70 - 110 mg / dl ) levels were within normal limits . a chest computed tomography ( ct ) showed multiple pulmonary thromboemboli , possibly calcific , which were noted on non - contrast ct imaging ( fig . 2 ) . fluoroscopic imaging showed an irregular - shaped calcified mass in the rv which changed in shape and size during the cardiac cycle ( fig . 3 ) . cardiac magnetic resonance imaging ( mri ) demonstrated a tubular calcified mass , which was separated from the right ventricular myocardium , extending from just below the tricuspid valve annulus to the right ventricular outflow tract , suggesting a cat or calcific fibroma ( fig . an endomyocardial biopsy was not performed due to the risk of right ventricular rupture or prolapse . heart - lung transplantation was deferred until the pulmonary arterial pressure improved and empirical anticoagulation was administered . a cardiac cat was first reported in 1997.4 ) cardiac cats can arise in all four chambers of the heart,1)2 ) although the proportion in each chamber is not known . although mural thrombi,4)5 ) chest trauma,1 ) and increased parathyroid hormone and calcium phosphate product levels in hemodialysis patients6 ) have been described as the causes for cardiac cats , the pathogenesis of these lesions is still unknown . in the present case histologically , a cardiac cat is characterized by nodular calcium deposits over a matrix of fibrin and/or amorphous fibrin - like material , hyalinization , inflammatory cells , and degenerated hematologic elements.7 ) clinical tests usually show cardiac cats to be benign , although they may cause obstruction or embolism,4 ) and cases can evolve fatally.5 ) an endomyocardial biopsy was not performed because of the risk of right ventricular rupture and further calcific embolization.8 ) surgical removal of the tumor may be indicated if embolism has occurred or seems likely . complete surgical resection should be pursued if possible , considering its recurrence.5 ) heart transplantation may be considered if not possible . we chose heart - lung transplantation in the present patient who had multiple calcified emboli and severe right ventricular dysfunction . during the differential diagnosis , cardiac neoplasias , especially myxomas and fibromas , are considerations , particularly if they are calcified , as are conditions involving infection or thrombosis.9 ) due to the lack of histology , calcified atrial myxoma , calcified thrombi or other cardiac neoplasms should be also considered as a differential diagnosis of calcific mass of rv . echocardiography , and ct and mri provide important information on the size and shape , attachment site , and pattern of movement of the calcified tumor . myxomas usually have a short , broad - based attachment and are pedunculated , although calcification may develop in approximately 10% of myxomas . ct scans can detect intracardiac masses and define the extracardiac extension.10 ) ct scans can also detect even minute amounts of calcium , which facilitated detection of the calcific pulmonary embolism in the present case . we recommend utilizing a multimodality imaging approach to accurately characterize intracardiac masses and their complications .
cardiac calcified amorphous tumors ( cats ) can arise in all four chambers of the heart . cardiac cats can cause diverse symptoms according to their locations , and mass or embolic effects . pulmonary emboli arising from cardiac cats have been reported , but the true incidence is unknown due to their rarity . herein we report a rare case with diffuse cats in the right ventricle which caused a calcific pulmonary embolism and right - sided heart failure . echocardiography , chest non - contrast computed tomography , and cardiac magnetic resonance imaging helped us diagnose the cats . we recommend the usefulness of a multimodality imaging approach to characterize intracardiac masses and their complications accurately .
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in their article in the present issue of critical care , wierdsma and colleagues validated a novel and feasible method to measure the degree to which enteral nutrition ( en ) is absorbed by the gastrointestinal tract . the accuracy of simply weighing daily faecal production to identify gastrointestinal dysfunction was validated against three reference methods . the contribution of protein and fat to the faecal nutritional losses were estimated by labour - intensive chemical analyses . the authors identified a daily faecal weight above > 350 g as a reliable indicator for gastrointestinal malabsorption . of course , these results need to be confirmed in a larger study population , including the most critically ill and those with known gastrointestinal problems . the present study is of methodological and conceptual importance to nutritional research and clinical nutrition management . first , the validation of this new assessment technique has been done in a very accurate and complete way and thereby provides a new reliable tool . secondly , these results focus on a rarely addressed problem in the critically ill : is the en administered to a patient truly absorbed ? in 9 out of the 48 stable patients in this trial , the nutrition was only partially absorbed . this 19% represents a high incidence of gastrointestinal dysfunction since patients with known gastrointestinal problems were not included . previous studies assessed and treated diarrhoea in critically ill patients , measuring the frequency , liquidity and volume of stools but not the proportion of en energy , proteins and fat lost by the patient . whether studying the effect of nutritional interventions or managing nutrition in clinical practice , we will have to take into account these data on gastrointestinal energy losses . the discrepancy between prescribed and effectively infused en doses in critically ill patients is relevant . interruptions of en for procedures , emesis , high gastric residual volumes ( grvs ) , diarrhoea and feeding tube replacement are important culprits . en lost in grvs being discarded after grv assessment can be measured using a refractometer . an alternative is to reinject grvs after measuring or to refrain from measuring grvs , since evidence supporting this practice is limited . briefly , we should be aware that the amount of en effectively taken up by the patient is the prescribed volume minus the volume not administered to the patients , minus the en discarded with grv minus the en lost in faeces . indirect calorimetry , however , does not measure energy needs but energy consumption , and indirect calorimetry is less reliable in patients on renal replacement therapy , aggressive ventilation and other conditions often present in the critically ill . a recent randomised trial showed more morbidity in the icu , but an unexplained reduction in hospital mortality in the per - protocol analysis , when feeding was guided by indirect calorimetry . recent trials found no benefit in early en administration up to the calculated target as compared with low en intake . finally , what should be done if en uptake is insufficient ? probiotics or fibre - rich en reduced the volume or frequency of diarrhoea in critically ill patients in some of the few randomised controlled trials on this topic . absorption of en by dysfunctional gut could be facilitated by the absence of proteins or even peptides in , respectively , semi - elemental en or elemental en ; however , trials in critically ill patients failed to provide convincing evidence . the best controlled method to effectively complete insufficient en would be the intravenous administration of nutrition : parenteral nutrition . supplementing insufficient en with parenteral nutrition during the first week of critical illness , however , resulted in more infections and delayed recovery in a large randomised controlled trial . in conclusion , the optimal en dose during different stages of critical illness is not yet known . stool weight measurements could improve our knowledge of true en absorption , however , and could thus lead to more accurate research and clinical practice . ku leuven received an institutional partial ( < 30% ) , unconditional and nonrestrictive research grant for the epanic trial from baxter healthcare ( maurepas , france ) . mpc receives funding from the research foundation flanders , belgium ( fwo doctoral fellowship ) , and dm from the clinical research fund of the university hospitals leuven , belgium ( postdoctoral fellowship ) .
the nutrition dose truly absorbed by a patient is crucial information in the management or the investigation of nutrition during critical illness . in the present issue of critical care , assessment of nutritional losses in stools was studied . these losses together with enteral nutrition lost in gastric fluids and enteral nutrition prescribed but never infused make up the difference between the dose supposedly given to a patient and the amount effectively taken up . additionally , the optimal dosing and timing of nutrition during critical illness are still debated . when enteral nutrition is insufficient , the options are limited .
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a 45-year - old male patient reported with a gingival growth on the right anterior region of the mandible for one year and also complained of pain in the same region for 6 to 7 months . the exophytic lesion was small initially and increased gradually to the current size displacing the adjacent teeth . extraorally , a diffuse swelling measuring around 1.51.5 cm in size was seen on the right lower portion of the face . 1a ) at the interdental gingiva between canine and the first premolar , which was roughly circular , measuring about 3.53.5 cm in size , extended labially from the attached gingiva to the lingual sulcus of the right mandibular incisors , canine , and premolar . 1b ) was pebbled and rough with indentations of the opposing teeth with mesially displaced canine . color of the lesion was normal as that of the surrounding mucosa and firm in consistency . 2 ) showed the presence of characteristic multilocular soap bubble appearance in the region of canine and first premolar , and knife edge shaped root resorption pattern in relation to the first premolar . 3 ) showed radiolucency measuring 44 cm in diameter extending from the distal aspect of lateral incisor to the mesial aspect of second premolar . the follicles were lined by a single layer of tall columnar ameloblast - like cells and little cystic degeneration . ameloblastoma usually manifests as an intraosseous or central lesion or rarely as a peripheral epulis - like lesion.1 the lesion may arise most commonly from cell rests of enamel organ , epithelium of odontogenic cysts , disturbances of the developing enamel organ , and basal cells of oral epithelium . clinico - radiographically , ameloblastoma can be divided into 3 distinct patterns : ( 1 ) the conventional solid / multicystic ( 86% of all cases ) , ( 2 ) the unicystic ( 13% of all cases ) , and ( 3 ) the peripheral ( 1% of all cases ) ( extraosseous).2 various histologic forms have been described which include follicular , plexiform , acanthomatous , granular cell , desmoplastic , and basal cell patterns.2 follicular ameloblastoma presents as a painless swelling or slow expansion of the jaws , and it is described as multilocular expansile radiolucency that occurs most frequently in mandibular molar / ramus area.9 desmoplastic variant occurs more frequently in the anterior mandibular region10 and presents a mixed radiopaque and radiolucent appearance . unicystic ameloblastoma is usually seen in younger age and most commonly associated with pericoronal radiolucency with unerupted 3rd molar ( 38%).11 in this particular case , the clinical presentation of the follicular ameloblastoma was different from the usual presentation , which included exophytic growth , uncommon location , and absence of expansion of the cortex . clinically differential diagnosis of this exophytic growth included peripheral ameloblastoma , peripheral odontogenic fibroma , peripheral giant cell granuloma , and other peripheral hyperplastic swellings superficial to the alveolar ridge.9 the final diagnosis was made by the clinical , radiological , and histopathological features after the complete excision of the lesion . kuru first reported an intraosseous ameloblastoma having penetrated through the alveolar bone , fused with the oral epithelium , and eventually presenting itself clinically as a ' peripheral lesion'.6 our case had the similar clinical and radiological features of the previously reported case by kuru but with no expansion of the cortex . two other similar cases have been reported by tongdee and ganggakavin7 in 1978 and stevenson and austin8 in 1990 , respectively . the exophytic growth is also a characteristic finding of the peripheral ( extraosseous ) ameloblastoma which is very rare . radiographically , or at surgery , a superficial erosion of the bone or a superficial bony depression -known as cupping or saucerization- may be noticed , a finding that is thought to be due to pressure resorption , in contrast to resorption caused by neoplastic invasion.5 the diagnostic criteria of peripheral ameloblastoma include the origin from the overlying epithelium , presence of odontogenic epithelium islands in the lesion , and lack of potential to bone infiltration.12 also , peripheral odontogenic fibroma and peripheral giant - cell granuloma share the same clinical characteristic of exophytic growth most commonly at mandibular premolar region , despite the histopathological difference . however , radiographically , both of them create depression / resorption in the underlying bone very rarely.13 most oral surgeons and pathologists unquestionably consider peripheral ameloblastoma to be a nonaggressive lesion without actual infiltration into the underlying bone.14 one of the main problems regarding peripheral ameloblastoma is its possible origin . the two main theories are the following : ( 1 ) origin from the extra osseous epithelial remnants of dental lamina and its organ derivatives within the underlying connective tissue ; ( 2 ) origin from the basal cell layer of the oral mucosa , which is believed to have odontogenic potential.15 those lesions that are entirely separated from the overlying surface epithelium probably arise from the odontogenic remnants , however this hypothesis can be questioned if there is continuity between the tumor and the surface epithelium . in conclusion , even though the incidence of intraosseous ameloblastoma manifesting as exophytic lesion is very rare , it may have to be considered in the differential diagnosis of exophytic lesions at the region of mandibular canine premolar region along with peripheral ameloblastoma , peripheral odontogenic fibroma , peripheral giant cell granuloma , and rarely peripheral hyperplastic swellings superficial to the alveolar ridge .
intraosseous ameloblastoma is the most common and simple type of ameloblastoma prevalent among odontogenic tumors . clinico - radiographically intraosseous ameloblastoma presents as slow , painless swelling or expansion of the jaws and described as multilocular expansile radiolucency that occurs most frequently in mandibular molar / ramus area . this article describes a case of follicular ameloblastoma involving 45 year old male which is different from the usual presentation , which includes - exophytic growth , different location and without expansion of the cortex .
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the study was conducted from march 2008 till september 2008 among 139 hcv seropositive cases ( identified during the period ) of 1860 years of age with both sexes included . the cases were identified at nepal red cross society ( nrcs ) , central blood transfusion service ( cbts ) , kathmandu , nepal . information of the subjects such as age and gender was accessed from the standard blood donor questionnaire form of cbts recorded by health professionals from the blood donors . samples were tested with confidentiality and identified by the sample code number given during the sample collection . ethical approval to conduct the study was taken from cbts . who strategy 2 for surveillance diagnosis of hiv was used for the test of the samples ( 9 ) . blood samples were tested for hcv using elisa test kits eiagen hcv ab kit ( adaltis , italy ) confirmed with sd bioline hcv ( standard diagnostics , inc . hcv seropositive cases were tested for hiv , hbv and syphilis with elisa test kits enzygnost anti - hiv plus ( dade behring , germany ) , enzygnost hbsag 5.0 ( dade behring , germany ) , sd syphilis elisa 3.0 ( standard diagnostics , inc . , korea ) respectively . the positive test results for hiv , hbv and syphilis were confirmed with rapid immunochromatographic test kits sd bioline hiv- 3.0 ( standard diagnostics , inc . , korea ) and virucheckhbsag ( orchid biomedical systems , india ) respectively while syphilis was confirmed with the same elisa test kit . test results with seropositivity for hiv , hbv and syphilis among hcv positive samples were considered to determine the co - infection rate . among the 139 hcv seropositive cases , eight of them were determined to have co - infections with total co - infection rate of 5.75% ( 95% ci=2.52 - 11.03 ) . co - infection rate of hiv among hcv was 3.59% , hbv among hcv was 0.71% and syphilis among hcv was 1.43% ( table 1 ) . all the co - infection cases were male though 11 hcv seropositive females were included in the study . hiv / hcv co - infection was seen in the age group between 2150 years . hbv / hcv co - infection in the age group 3140 years and syphilis / hcv in the age group 2130 years . prevalence of hiv , hbv and syphilis co - infection among hcv cases indicates that the infections could be transmitted simultaneously due to their common modes of transmission . hcv is more commonly transmitted among idus and the co - infection of hiv , hbv and syphilis with hcv can be due to transmission modes such as sharing of needles or unsafe sexual transmission among such risk groups . hiv / hcv was the most common co - infection followed by syphilis / hcv and hbv / hcv . prevalence of higher hiv / hcv co - infection in this study can be due to the practice of injecting drug use as many idus who become infected with hiv are already infected with hcv . hiv / hcv co - infection determined is lower than the reported co - infection rate of 10.8% with hcv and hiv ( 4 ) . co - infection rate of hiv in hcv estimated is similar to the reported rate of hcv co - infection in hiv patients in north india ( 2.43% ) and 2.2% reported in south india ( 10 , 11 ) . hbv / hcv co - infection is lower than the hcv / hbv co - infection rate of 1.67% reported in nepalese blood donors ( 5 ) . lower co - infections rate in this study is due to the low - risk group of population ( healthy looking blood donors ) considered ; decreasing prevalence of transfusion transmissible infections ( 3 ) . higher hiv / hcv co - infection indicates hiv risks associated with the injecting drugs users who are usually infected with hcv . prevalence of co - infections observed in the study in only males indicates indulgence of such gender groups in unsafe practices that could have transmitted the multiple infections . monitoring of co - infections is necessary to commence immediate treatment of the cases besides prevention of transfusion associated risks with such blood donors . co - infection of hiv , hbv and syphilis with hcv is prevalent in the blood donors of kathmandu , nepal . further investigations can be carried out with larger sample size and in high risk groups with other infections sharing common modes of transmission to estimate the co - infections in the general population of the country . the authors have not received any funding or benefits from industry or elsewhere to conduct this study .
backgroundhiv , hbv , syphilis and hcv share common modes of transmission.objectivethe study was aimed to determine the co - infection rate of hiv , hbv and syphilis among hcv seropositive identified blood donors.methodsthe study was conducted on blood samples screened as hcv seropositive at nepal red cross society , central blood transfusion service , kathmandu , nepal . hcv seropositive samples were further tested for hiv , hbv and syphilis.resultseight co - infections were observed in 139 hcv seropositives with total co - infection rate of 5.75% ( 95% ci=2.52 - 11.03).conclusionco - infection of hiv , hbv and syphilis with hcv is prevalent in the healthy looking blood donors of kathmandu , nepal .
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glomus tumor was first described by wood as early as 1821 , but the characteristic histological description was given by masson . they are mostly located in the subungual region but occur less frequently in other nail unit region and extradigital sites . characteristic triad of symptoms of temperature sensitivity , severe pain and localized tenderness can be noted in 63 - 100% of the patients . a 36-year - old female was referred from surgery department , with history of pain in the left thumb since 10 years . she noticed splitting of nail 3 years back , which gradually progressed to involve the whole length of nail . , there was a small swelling with indistinct margins just behind the proximal nail fold . there was also longitudinal split in the nail which was extending from the free end of the nail plate to the proximal nail fold [ figures 1 and 2 ] . no color changes either in the nail plate or in the proximal nail fold were noted . intraoperatively , a semitranslucent mass of 2 mm 3 mm size was found . it was excised and sent for histopathological examination . these tumor cells had sharply punched out round to oval nucleus and well - defined borders . there were some dilated vessels with clusters of tumor cells in their walls [ figure 3 ] . fullness in posterior nail fold region glomus tumor during exploration tumor cells invading vessel wall ( magnification 10 ) subungual location is the preferred site for digital glomus tumor , but can occur in other areas also . in our patient , the site involved was in posterior nail fold region , which is a rare occurrence . reported the same in only one of his 28 glomus tumor patients evaluated by magnetic resonance ( mr ) imaging . the most common site in his study was the reticular dermis of nail bed in rest of the patients . our patient had the classical triad of symptoms - temperature sensitivity , severe pain and localized tenderness . bhaskaranand and navadgi reviewed different clinical tests to diagnose glomus tumors and opined that cold sensitivity test is the most accurate test to diagnose glomus tumors . positive cold sensitivity is also reported by vasisht et al . in 84% of their patients . erythronychia and distal onycholysis were seen in all the glomus tumor patients studied by dominguez - charit et al . noted the same in 76% of patients and only 24% of his patients had blue blush of nail in their study . radiographic examination was inconclusive in our patient and the same was reported by bhaskaranand and navadgi in their study . use of high resolution magnetic resonance imaging ( hr - mri ) and high variable frequency ultrasound ( hvfus ) in preoperative assessment will help in the outcome of surgical treatment . bhaskaranand and navadgi employed the use of double tourniquet , one at the mid arm and the other at the base of digit , for better visualization of the tumor during exploration . patient was followed up and it was noticed that she had recurrence of pain after 6 months small size and difficulty in locating the tumor by traditional methods were the contributory factors . but due to their high cost , glomus tumors continue to cause diagnostic difficulty to the treating physician because of their small size and lack of simple cost - effective investigation .
glomus tumor is a benign tumor arising from the neuromyoarterial plexus concentrated beneath the nail . this plexus is an arteriovenous anastomosis functioning without the intermediary capillary bed . etiology is not exactly known . it is debilitating to the patient because of the chronicity of symptoms and lack of proper investigation which will help in identifying the tumor at an early stage . we report a case of glomus tumor situated in the proximal nail fold region and causing longitudinal splitting of nail .
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it is a relatively rare condition , typically benign and resolves with conservative therapies ( 1 ) . this has been occasionally described in the surgical published work , usually in the context of thoracic surgery or as a complication of spinal instrumentation ( 2 ) . it initially described by gordon and hardman in 1977 as intraspinal air ( 3 ) . since then , especially with the advent of computed tomography ( ct ) , there has been an increasing number of case reports describing the presence of air within the spinal canal ( 4 ) . in 1987 , pneumorrhachis may be an indication of substantial vertebral column injury , especially when paraspinal hematoma , rib fractures , transverse process fractures , clavicle fractures , subcutaneous air , intramuscular air , pneumothorax , or hemothorax are also present ( 2 , 6 - 8 ) . pneumorrhachis has also been reported with isolated head trauma and in the absence of an identifiable fracture within the skull or spine ( 9 , 10 ) . nontraumatic causes of pneumorrhachis include infection ( 11 ) . despite known conditions that can result in pneumorrhachis the clinical significance and neurologic outcome for patients with pneumorrhachis , we report a case of pneumorrhachis with fracture in lumbar spine and history of ankylosing spondylitis . the axial thoracic spinal ct scan of patient a 39-year - old man was admitted because of low back pain and dyspnea after locating between motor vehicle and wall 3 days before admission . he suffered from ankylosing spondylitis and was under corticosteroid therapy . on arrival , his glasgow coma scale was 15/15 with normal neurological examination . he was able to move his lower extremity , but it was limited by pain . vital signs were stable and in radiographic examination , there was rib fracture in three ribs , hemothorax , subcutaneous emphysema , and lumbar vertebra fracture in l2-l4 . ct scan showed bilateral pleural effusion , fracture of ribs number 8 , 9 and 10 in lower left side of thorax , fracture of vertebra in l2-l4 , and air bubbles in upper thoracic spinal canal ( figure 1 ) . the patient underwent lumbar surgery ( laminectomy and cord decompression ) and fusion of l2-l4 by screw . in according to neurosurgery consult no action performed on air bubbles . pneumorrhachis , as the presence of free intra - spinal air , is also called aerorachia or epidural emphysema . it usually occurs in the epidural space but may be spread within the subarachnoid space with the distraction of dura meter ( 1 ) . a significant increase in intra alveolar pressure and intra alveolar pressure was increased in several conditions such as acute asthma , recurrent vomiting and closed thoracic trauma . this led to alveolar rupture and air movement along the bronchovascular axis up to the mediastinum . the collected air then disassociates the pleura from the aorta and the parietal pleura from the spine , subsequently inflowing the extradural space via the intervertebral foramina ( 12 ) . in addition , subarachnoid air may easily move cranially and caudally and may cause back and/or local pain and headache and/or nervous tissue compression ( brain and spinal cord ) by a valve mechanism . however , in the present case , there are not strong evidence of air diffusion between the pleura and the epidural space , it is the only pathophysiological mechanism stated in the literature ( 1 ) . magnetic resonance imaging is the most accurate investigation for the assessment of the extent of the condition . the best explanation for the radiologic findings in our patient is that air , under pressure in the pleural space , entered the spinal canal and then the subarachnoid space directly through tears of the parietal pleura and the spinal meninges . tension pneumothorax and thoracic spinal fracture should be considered in the differential diagnosis of both pneumorrhachis and pneumocephalus . intraspinal air is usually asymptomatic , self - limiting and resolves with conservative therapies , but in a rare number of cases , pneumorrhachis can cause cord compression and may even require decompressive surgery . appropriate antibiotic prophylaxis must be considered , however , due to increased risk of infection of the underlying breach in the dura in traumatic etiologies ( 2 ) . in comparison with others , sinha and mantle has been described a case of pneumorrhachis with rapid deterioration to death ( 14 ) . , they presented a case of tension pneumocephalus and pneumorrhachis secondary to a subarachnoid pleural fistula after thoracic spinal surgery ( 16 ) . valente et al reported a 21-year - old male with severe pneumocephalus and pneumorrhachis who was made a full recovery without any neurological complication ( 17 ) . it is usually asymptomatic and self - limiting , but its presence should alert the attending trauma physician to carry out diagnostic workup for associated injury and treat the underlying cause ( 18 ) . when seen in a trauma patient all authors passed four criteria for authorship contribution based on recommendations of the international committee of medical journal editors .
pneumorrhachis as a relatively rare condition may be an indication of substantial intra - spinal column injury . here we report a 39-year - old man was admitted because of low back pain and dyspenea after locating between motor vehicle and wall three days before admission . on arrival , physical exams and vital signs were normal . computed tomography ( ct ) scan showed bilateral pleural effusion , fracture of ribs number 8 , 9 and 10 in lower left side of thorax , fracture of vertebra in l2-l4 , and air bubbles in upper thoracic spinal canal .
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reticulohistiocytosis is a spectrum of disorders ranging from solitary and diffuse cutaneous forms without systemic involvement to multicentric reticulohistiocytosis ( mr ) . the primary skin lesion , reticulohistiocytoma , usually presents as a firm , skin colored , yellowish or reddish papule or nodule . in multiple cutaneous reticulohistiocytoma ( mcr ) , there is onset of multiple lesions without underlying systemic illness , whereas in mr there are extensive skin lesions in association with a severe , often destructive arthropathy , and systemic features . mcr is more common in young adult males and mr in middle aged women associated with systemic symptoms . both the variants are characterized in histo - pathology by the presence of large mononucleated or multinucleated histiocytes with an abundance of eosinophilic , homogenous to finely granular cytoplasm having a ground glass appearance . we report here a case of mcr in a middle aged female for its rarity and the importance of ruling out mr in such a patient . a 35-year - old female presented with gradual onset of multiple skin colored , asymptomatic pea sized swellings , which started with face and gradually involved back , arms , and legs . there was no history of preceding local trauma , discharge , pain in these lesions . there were no constitutional symptoms like fever , joint pain , and complaints referable to other systems . the patient 's main concern was regarding the cosmetic appearance as lesions were present over the face . her family history was negative and she had no history of any other chronic illness . mucocutaneous examination revealed multiple ( 20 in number ) , discrete , skin colored nodules , 12 cm in size scattered over her face , back , abdomen , thighs , and lower legs [ figure 1 ] . her complete blood count along with general blood picture , liver , and renal function tests , fasting lipid profile and urinalysis were normal . rheumatoid factor and antinuclear antibodies were found to be within normal limits . on the basis of history and clinical examination , differential diagnosis of xanthoma , reticulohistiocytoma , sarcoidosis , and histoid leprosy ( a ) multiple discrete skin colored nodular lesions over back ( arrow ) ( b ) discrete skin colored nodular lesions over face , ( c ) discrete skin colored nodular lesions over back , ( d ) discrete skin colored nodular lesions over posterior aspect of left thigh and leg excisional biopsy of a nodule over the trunk was done and histopathological examination revealed circumscribed large focus of diffuse dense infiltrate of large histiocytes and histiocytic giant cells with scattering of lymphocytes and a few neutrophils [ figure 2 ] . several histiocytic giant cells resembling the touton giant cells were also seen [ figure 3 ] . overlying epidermis showed flattening and thinning and was covered by parakeratosis containing collection of neutrophils . based on clinical and histopathological findings a diagnosis of multiple cutaneous reticulohistocytoma the nature of the disease was explained to the patient and was referred to the plastic surgeon for the cosmetic correction of the facial lesion . skin biopsy showing lymphocytic infiltrate with histiocytes and giant cells [ h and e , 100 ] skin biopsy showing multinucleated giant cells ( arrow ) with an amorphous eosinophilic cytoplasm [ h and e , 400 ] non - langerhans cells histiocytosis represents a broad group of different disorders characterized commonly by proliferation of histiocytes other than langerhans cells . mcr represents a unique pattern in the spectrum of the reticulohistiocytoses , characterized by histiocytic proliferations of the skin and soft tissues . it may represent an abnormality histiocytic reaction to different stimuli . local trauma may play a role in the pathogenesis of solitary cutaneous histiocytoma , whereas in diffuse forms , the association with internal malignancies and autoimmune diseases suggest an immunologic basis . reticulohistiocytomas are usually solitary lesions of less than 1 cm in diameter . however , multiple lesions and large reticulocytomas have been rarely reported . histopathology of reticulohistiocytoma often shows mid - dermal infiltration of mononuclear histiocytes and multinucleated histiocytes with a ground - glass appearance and a variable number of vacuolated , spindle shaped , and xanthomatized mononuclear histiocytes . vimentin is universally positive but all other markers like s-100 , desmin , and smooth muscle - specific actin remain negative . pulsed dye laser , oral corticosteroids , and methotrexate has been tried in the treatment of extensive lesions . the only concern in our patient was regarding the physical appearance of the lesions as they were also present over the face . however , patient of mcr needs to be followed up regularly for long time to look for the development of mr which has various life threatening systemic complications . this case thus lays emphasis on considering mcr in the differential diagnosis of asymptomatic skin colored papulo - nodular lesions and adequate systemic evaluation and follow up of such patients .
multiple cutaneous reticulohistiocytoma ( mcr ) and multicentric reticulohistiocytosis ( mr ) are rare , idiopathic histiocytic granulomatous disorders presenting in a spectrum . a 35-year - old female presented with multiple , firm , discrete , asymptomatic nodules , 1 - 2 cm in size over face , back , abdomen , thighs , and legs . there were no systemic symptoms . histopathology of a nodule over trunk showed diffuse , dense infiltrate of large histiocytes , and histiocytic giant cells . the histiocytes had rounded vesicular nuclei and abundant pink homogenously stained ground glass cytoplasm . the diagnosis of mcr was made . systemic evaluation did not reveal any abnormality . considering the cosmetic appearance of facial lesions , patient was referred to the plastic surgeon . no treatment was advised for rest of lesions and patient was asked to review every 6 months or if any new complaints develop .
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about 30 years ago , methyldopa and penicillin were the two medications most commonly associated with drug - induced autoimmune hemolytic anemia ( diiha ) . methyldopa and intravenous penicillin accounted for 67% and 25% , of all drug - induced immune hemolytic anemia , respectively . currently , most cases of diiha are attributed to second- and third - generation cephalosporins , most commonly ceftriaxone , and this drug has become the most common cause of antibiotic - induced hemolysis . we present a case of diiha following ceftriaxone use , managed successfully with withdrawal of the drug and supportive measures . since this antibiotic is widely used by clinicians across all specialties , it is important to be aware of this possibility to enable us to make an early diagnosis . a 60-years - old lady , with no past history of any drug allergies , presented with fever and productive cough of five days duration . clinically , she had fever , tachycardia , tachypnea with crackles in the right mammary area . x - ray chest confirmed pneumonia in the right middle zone [ figure 1 ] . on admission , her investigation were as in [ table 1 ] x - ray chest of the patient showing consolidation in right middle zone laboratory parameters of the patient suffering from ceftrixone - induced hemolysis a diagnosis of community - acquired pneumonia was made , and patient was started on intravenous ceftriaxone one gram 12 hourly . her hemoglobin dropped down to 7.5 g / dl within 24 hrs and further to 5.5 g / dl after 72 hrs , with peripheral blood smear showing marked polychromasia with three nucleated red cells per 100 white blood cells , schistocytes , a corrected reticulocyte count of 4.0% and ldh of 1221 units / l . serum bilirubin rose to 2 mg / dl with indirect of 1.5 mg / dl . a diagnosis of ceftriaxone - induced aiha was made , and the drug was stopped immediately . two units of packed red blood cells were transfused as she had symptomatic anemia , her hemoglobin increased to 8.6 g / dl and thereafter to 9.8 g / dl at discharge , after two weeks with no further deterioration . ceftriaxone - induced urticaria , rash , exanthem , and pruritus are the most common adverse effects and occur in about three percent of patients . the first case of hemolysis induced by ceftriaxone was reported in 1991 by garratty et al . in a 52-year - old woman who was treated with ceftriaxone . this was the first case of immune hemolytic anemia associated with ceftriaxone , and also the first case of fatal cephalosporin - induced hemolytic anemia . thereafter , in 1995 , a case report of a 24-month - old boy with sickle cell disease who had cardiac arrest and died 36 hours later from multiple organ failure after starting ceftriaxone . another case of a 16-year - old girl was reported in 1999 who had developed ceftriaxone - induced intravascular hemolysis leading to acute renal failure and death . drug - dependent antibodies ( antibodies react in vitro with rbc 's , only in presence of drug ) are produced by antibiotics like ceftriaxone and pipercillin . these drugs attach to rbc , but do not bind covalently to rbc membrane proteins . combination of drug and antibody creates an immunogen , which activates complement and results in acute intravascular hemolysis . drug - independent antibodies ( drug not required to be present to detect antibodies in vitro ) are produced by drugs as fludarabine , methyldopa , and penicillins . these drugs combine covalently with rbc membrane proteins , and the antibody - coated rbc are taken up by macrophages . ceftriaxone - induced aiha , though rare , can be fatal if not thought of . it needs high index of clinical suspicion in patients treated with ceftriaxone who develop sudden drop in hemoglobin , hemoglobinuria , and evidence of hemolysis in peripheral blood smear . beta lactam antibiotics should be used with caution in patients who have history of adverse effects to ceftriaxone because of cross reactivity . according to the world health organization ( who ) system of causality definitions , the adr ( adverse drug reaction ) in this reported case is categorized as probable with naranjp algorithm score of six , as the patient developed hemolysis after intravenous administration of ceftriaxone . there was no other possible cause of hemolysis as no other drug was used with ceftriaxone . rechallenge was not done due to inherent risk involved , but the patient showed improvement after stopping the drug . though drug - induced autoimmune hemolytic anemia is a rare adverse reaction , they may be fatal in some cases .
immune hemolytic anemia is a rare adverse effect of ceftriaxone , a third - generation cephalosporin , which is a commonly used antibiotic . we describe a 60-years - old lady , a case of community - acquired pneumonia , who developed severe hemolysis after the first dose of ceftriaxone . her hemoglobin dropped from 9.6 g /dl to 5.5 g /dl . however , she improved after discontinuation of the drug and blood transfusion . this report serves as a reminder to medical fraternity that life - threatening hemolysis can rarely follow administration of ceftriaxone .
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to report a case of a young patient with retinitis pigmentosa ( rp ) , essential iris atrophy , and glaucoma . this report presents a case of a 22-year - old female patient with unilateral glaucoma , increased intraocular pressure , increased cup disc ratio , iris atrophy , peripheral anterior synechiae , and bilateral rp . essential iris atrophy is one of the subtypes of iridocorneal endothelial ( ice ) syndrome , which is a spectrum of disorders characterized by corneal proliferative endotheliopathy , associated with corneal edema , anterior chamber and iris stroma abnormalities , and glaucoma.1 other variants of ice syndrome are chandler s syndrome and cogan - reese syndrome . it is unilateral , and it is associated with iris holes , peripheral anterior synechiae , and corneal alterations.1 approximately 50% of the cases present glaucoma , and it is probably caused by peripheral anterior synechiae and by a cellular membrane that covers the trabecular surface . the treatment for glaucoma is clinical ; however , there is frequent surgical indication of trabeculectomy . retinitis pigmentosa ( rp ) is characterized by progressive decrease in night vision and progressive visual field loss due to cellular retinal dystrophy . the fundoscopy shows characteristic bone - spicules pigment.2 rp does not present a defined inheritance pattern , and it might occur in an autosomal dominant , recessive , or x - linked fashion.3 it can also be related to other systemic and ocular manifestations . a 22-year - old white female patient reported the diagnosis of rp that she had 7 years before . having gone through many examinations along this period , 1 year ago , it was diagnosed increased intraocular pressure ( iop ) in the right eye ( od ) . the patient had misused hypotensive eye drops : prostaglandin analogs , carbonic anhydrase inhibitors , and b - adrenergic antagonists . patient consent was obtained before undergoing treatment . in the ophthalmic examination , the best - corrected visual acuity was od : 20/50 ( 1,00 cyl 115 ) and left eye ( os ) : 20/25 ( + 2,00 sph 1,00 cyl 10 ) . the slit lamp examination showed multiple iris holes and corectopia in od ( figures 1 and 2 ) , clear cornea in both eyes ( ou ) , and no alterations in os . iop by goldmann applanation tonometry was od : 34 mmhg and os : 16 mmhg at 3 pm . the gonioscopy revealed 360 isolated peripheral anterior synechiae in od ( figure 3 ) and a visible open - angle up to ciliary body in os . disc ratio 0.9 vertical ( v ) 0.9 horizontal ( h ) , visible lamina cribrosa pores , preserved macula , and peripheral pigment mobilization in od . in os , disc ratio 0.3 v 0.3 h , preserved macula , and peripheral pigment mobilization . the automated perimetry ( figures 6 and 7 ) and manual perimetry ( figures 8 and 9 ) showed central island vision in od and ring scotoma in os . the ultrasound pachymetry was 524 m and 530 m in od and os , respectively . the specular microscopy revealed pleomorphism and polymegathism in ou ( figures 10 and 11 ) . after failure of the clinical treatment to diminish iop in od , trabeculectomy was performed in this eye and iop was controlled . we have reported the case of a young patient presenting with rp and ice syndrome with glaucoma . although there are some reports of association of rp and glaucoma in the literature , we have not found any report of association between rp and ice syndrome at pubmed . the most frequent glaucoma associated with rp is angle - closure glaucoma.2,46 the prevalence of glaucoma in patients with rp can be up to 2.3%.4 however , a study of 40 patients with rp found a prevalence of 12.5% of primary glaucoma.7 in the literature , there are reports of glaucoma associated with rp in inherited isolated or secondary cases , such as sturge weber syndrome,8,9 retinal neovascularization,10 familial nephropathy,11 bilateral ectopia lentis,12 and fuchs heterochromic cyclitis.13 there are also case reports of patients with rp who mimic glaucomatous visual field defect14 and abnormal nerve fiber layer of the retina , similar to those found in patients with glaucoma.15 in this case , we believe that glaucoma is associated with ice syndrome because the patient has unilateral glaucoma and iris essential atrophy in the same eye . the patient also presents alterations in specular microscopy and in the anterior chamber in od , which might be related to essential iris atrophy . the perimetry examinations demonstrate a glaucoma typical defect in od and a rp typical defect in os .
purposeto report a case of a young patient with retinitis pigmentosa ( rp ) , essential iris atrophy , and glaucoma.case reportthis report presents a case of a 22-year - old female patient with unilateral glaucoma , increased intraocular pressure , increased cup disc ratio , iris atrophy , peripheral anterior synechiae , and bilateral rp.discussionthe patient presented glaucoma due to the iridocorneal endothelial syndrome , despite low age . rp is a bilateral disorder that may be associated with angle - closure glaucoma .
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chronic graft versus host disease ( cgvhd ) belongs to the most serious and frequent ( 3070% ) complications in patients undergoing hemopoietic stem cells transplantation ( hsct ) for hematological malignancies . musculoskeletal and skin cgvhd , by inducing fibrotic changes in tissues , may result in reduction of joints range of motion ( rom ) , loss of muscular strength and , finally , in functional impairment , compromising the activities of daily living ( adl ) . supportive cares , such as physical rehabilitation and occupational therapy , although recommended as ancillary therapy , have been rarely experienced in such a condition . , recently presented , dealt with a single case of motor rehabilitation in a patient with cgvhd - related contractures . at the best of our knowledge , it represents the first reported case of extensive cgvhd with skin and musculoskeletal involvement treated with physical rehabilitation in an adult patient . thereby , we described an additional case of a patient with similar cgvhd involvement recently treated with physical rehabilitation within our homecare program . a 39-year - old man affected by chronic myeloid leukemia ( cml ) in third relapse after allogeneic hsct and not eligible for further active therapy was referred to our homecare service as advanced / terminal patient in june 2008 . at diagnosis , in 1992 , he had been submitted to hla identical sibling allogeneic hsct , obtaining complete remission ( cr ) ; in 1998 , a disease relapse , as accelerate phase of cml , occurred . therefore , a second hla identical sibling allogeneic hsct , from a different donor , was performed and both hematological and cytogenetic remission were achieved . in 2004 , the patient developed a second hematological relapse ( myeloid blast crisis ) and he was given donor lymphocyte infusions ( dli ) and imatinib ; after three dlis at escalating doses , a molecular cr was obtained . however , 8 months later , he developed extensive cgvhd ( skin , mouth , eye ) and , therefore , immunosuppressive therapy ( steroids and extracorporeal photoferesis ) was started , without improvement , such that cgvhd progressed with the addition of lung involvement . therefore , the patient received multiple lines of immunosuppressive drugs ( rituximab , cyclosporine , plaquenil and mycophenolate ) with only a poor response . meanwhile , in june 2007 , he developed a third relapse ( extramedullary lung and bone involvement ) and was treated with imatinib and dasatinib without response and therefore he was considered not eligible for further causal therapy . at admission in the homecare service , the patient presented with an extensive skin and musculoskeletal cgvhd ; barthel index ( bi ) , as adl measure , was 40 ( moderate severe reduction ; normal = 100 ) , as a result of diffuse contractures with severe reduction of joints rom [ table 1 ] ; secondary , legs muscles hypotrophy was increased due to spinal cord disease - related compression . the patient was assisted with a fully homecare program , with medical and nursing periodic examination , transfusions support and motor rehabilitation . both physical and occupational therapy were promptly started , with particular attention to stretching exercises for joints ; planned intensity was 3 sessions per week . after 4 weeks of treatment , planned intensity was respected and no rehabilitation - related complications were noted . although bi did not improve , the rom of treated joints increased and the mean rom improvement ( expressed as percentage of baseline value ) was 52.5% [ table 1 ] ; both motor skills , psychological aspects and patient quality of life had significant amelioration . after 5 weeks , rehabilitation was discontinued because of infectious pneumonia and , 1 month later , an attempt to restart failed due to rapid deterioration . in november 2008 , range of motion in major joints : normal value , baseline value ( t0 ) , after 1 month of treatment ( t1 ) and improvement expressed as percentage of baseline value ( ) musculoskeletal involvement , in the course of cgvhd , is a rare phenomenon ; larger reported series describe cgvhd - related fasciitis or myositis in less than 1% of the patients undergoing allogeneic sct . musculoskeletal cgvhd , expression of an immunological response toward recipient antigens , is considered an organ involvement in the course of a widespread disease , and systemic immunosuppressive therapy is the standard approach of treatment . however , local treatment should be used in order to improve response and to reduce toxicity , allowing a prompt and rapid escalation of systemic treatment . although data derived from experiences in different immunologically mediated musculoskeletal contractures suggest an important role of physical rehabilitation , such a therapy remains a poorly explored issue in patients with cgvhd , with few data reported in adult patients . although limited , confirm the possibility of almost temporary results , also in long - lasting cgvhd - related contractures . monitoring for musculoskeletal involvement in patients at risk for or with initial features of cgvhd is required , both to prospectively evaluate cgvhd - related muscoloskeletal involvement and to enroll patients at an initial disease stage . clinical trials have to be developed to adequately assess feasibility , safety and efficacy of motor rehabilitation in response to the need to prevent disease progression toward rom limitation and consequent disability .
chronic graft versus host disease ( cgvhd ) is a frequent complication of allogeneic stem cell transplantation . extensive musculoskeletal and skin involvement may induce severe functional impairment , disability and quality of life deterioration . physical rehabilitation is recommended as ancillary therapy in these forms , but experiences are sparse . a 39-year - old man affected by musculoskeletal and skin chronic graft versus host disease ( cgvhd ) was treated with a homecare - based motor rehabilitation program during palliation for disease progression . significant functional improvement was obtained . motor rehabilitation should be strongly considered for patients with musculoskeletal cgvhd , both in the palliative and in the curative phase of disease .
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the unroofed coronary sinus ( cs ) syndrome is a rare entity ( 1 - 5 ) . approximately , 75% of cases with the unroofed cs syndrome are associated with a persistent left superior vena cava ( svc ) ( 1 ) . there is no reported case of an unroofed cs syndrome accompanied by the cs stenosis in the english literature . the clinical presentation of the unroofed cs is generally determined by the size of the defect between the cs and the left atrium ( i.e. the degree of a left - to - right shunt ) and associated abnormalities ( 2 ) . we present a case of the complete unroofed cs syndrome with rare associated abnormalities ( i.e. the cs stenosis and a collateral pathway through the small cardiac vein and thebesian vein , but the absence of a persistent left svc ) incidentally identified on coronary computed tomography ( ct ) angiography . we also propose an explanation of the asymptomatic presentation in this case in spite of the large defect between the cs and the left atrium . a 43-year - old male presented with intermittent atypical chest pain of one month 's duration . coronary ct angiography from the tracheal bifurcation to the base of the heart ( lightspeed vct , ge healthcare , milwaukee , wi , usa ) to evaluate the underlying cause of the chest pain demonstrated normal coronary arteries . however , a large defect ( approximately 2.8 cm in diameter ) between the cs and the left atrium and stenosis of the cs orifice were identified ( figure 1 a and 1b ) . the collateral flow from the cs to the right atrium through the small cardiac vein and thebesian vein there was no evidence of the enlargement of any cardiac chamber , and nor was there a persistent left svc identified ( figure 1 a and 1b ) . no abnormality was identified at transthoracic echocardiography , although the ct reading was available to the cardiologist performing echocardiography . transesophageal echocardiography was not performed because the patient was asymptomatic except for the chest pain . a diagnosis of the unroofed cs syndrome with the cs stenosis in the absence of a persistent left svc was made . the unroofed cs syndrome is a rare congenital anomaly that is often associated with a persistent left svc ( 1 - 5 ) . the classification of the unroofed cs syndrome varies in the literature ( 1 - 5 ) . it can be classified simply complete or partial depending on the size of the defect between the cs and the left atrium . no previous report exists of a complete unroofed cs syndrome and absence of a persistent left svc in combination with the cs stenosis leading to a collateral pathway through the small cardiac vein and thebesian vein identified on coronary ct angiography . this unusual combination of three congenital anomalies remained asymptomatic into adulthood , when the patient presented for unrelated reasons . the clinical presentation of the unroofed cs syndrome varies from the absence of symptoms to severe right heart failure , and is mainly determined by the size of the cs defect between the cs and the left atrium ( i.e. the degree of left - to - right shunting ) and associated anomalies such as a persistent left svc ( i.e. brain abscess or infarction caused by a right - to - left shunt ) ( 1 - 5 ) . the present case had no related symptoms in spite of a large defect between the cs and the left atrium . this was presumably due to the associated cs stenosis and the restriction of the flow by the formation of a collateral flow through the small cardiac and thebesian veins between the cs and the right atrium . brain abscess or infarction may be a complication in patients with the unroofed cs syndrome due to paradoxical embolism caused by a right - to - left shunt ( 1 ) . as there was no associated persistent left svc , the possibility of a right - to - left shunt was eliminated . although the underlying cause of chest pain in the present case was not delineated clearly , the chest pain did not seem to be directly related to the unroofed cs syndrome as it had developed recently without accompanying symptoms . in this instance , multiple detector computed tomography ( mdct ) with its high spatial resolution provided not only precise anatomic detail of the unroofed cs syndrome and the associated anomalies , but also a possible explanation for the patient s clinical presentation . in conclusion , the presence of the cs stenosis and the absence of a persistent left svc in a patient with an unroofed cs syndrome may result in an asymptomatic presentation , even in the setting of a large cs defect .
we describe a patient with an asymptomatic complete unroofed coronary sinus ( cs ) syndrome associated with the cs stenosis in the absence of a persistent left superior vena cava ( svc ) as identified on coronary computed tomography angiography . there was a large defect between the cs and the left atrium ( i.e. a large left - to - right shunt ) , but an unusual combination of the absence of a persistent left svc ( i.e. no risk for brain abscess due to the absence of a right - to - left shunt ) and the cs stenosis ( i.e. a markedly reduced degree of a left - to - right shunt ) , resulting in an asymptomatic presentation .
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one of the key tools for the operating surgeon is the sense of touch . through that , the surgeon is often able to determine inflammation and induration , create dissection planes , and identify vascular structures . the circumstances are wide ranging from the assessment of bowel viability in preparation for a bowel resection and reanastomosis to assessing patency after vascular reconstruction , from identifying arterial blood vessels to detecting stenoses or other abnormalities . extension of this technology to laparoscopic surgery is a logical step , particularly in view of the lack of tactile sensation available during laparoscopy . laparoscopic ultrasound has been used in the detection and staging of abdominal malignancies , identification of common bile duct stones , and in the prevention of injuries during laparoscopic cholecystectomy . most reports , however , comment on the significant learning curve associated with the technique and use of the equipment . what is needed is a simple method to apply laparoscopically , what the general surgeon is comfortable performing during open surgery . co. doppler probe ( 8.1 mhz ) and a 15-inch section of thick - walled , 9.5-mm od stainless steel tubing , a laparoscopic doppler probe ( ldp ) was constructed . the parts were separately gas - sterilized , and a small segment of penrose drain was used to create an airtight seal ( figure 1 ) . the probe was constructed in the first few minutes of the operative cases by the first assistant , while the primary surgeons were scrubbing ( figure 2 ) . the ldp thus constructed was passed through a 10-mm port , allowing positive identification and assessment of vascular structures ( figure 3 ) . all the materials necessary for construction of the laparoscopic doppler probe . the constructed laparoscopic doppler probe . the probe was used to identify liver hemangiomas , iliac vessels during pelvic dissection , and mesenteric vessels during hemi - colectomies . surgeons using the probe were surveyed , and all agreed that the probe was simple to use and added valuable information to their dissections . its use in preventing complications during laparoscopic cholecystectomy has been extensively studied . until just recently , however , its widespread application has been somewhat limited because of the size of the probe , the size of the ultrasound machinery , and the lack of , until recently , an end - on - probe . similarly , the unfamiliarity of ultrasound to general surgeons has created a significant learning curve when ultrasound is being applied , not to mention being applied in a laparoscopic fashion . what is needed is a convenient , simple , cost - effective way to get some of the advantages that ultrasound offers at the disposal of the laparoscopic surgeon . the ldp described herein can be easily made during a laparoscopic procedure on a back table with low - cost materials just before the procedure begins , or any time during the procedure that a doppler may be of use . it provides useful information on the location of vascular structures as in during a laparoscopic colectomy where identification of the inferior mesenteric vessels is readily accomplished and eliminates any misunderstanding of the vascular anatomy . it has also been used by our surgeons in preperitoneal hernia dissection where both the femoral artery and vein are identified relatively simply by compressing the overlying soft tissues onto the vasculature . the ldp may also provide information on the patency and flow characteristics of vascular anastomoses as laparoscopic techniques evolve to include more vascular procedures . the ldp was originally constructed to identify a hemangioma on the surface of the liver during a routine laparoscopic cholecystectomy by using a penrose drain to create an airtight seal and the delivery portion of an endoscopic retrieval bag as the delivery tube . since its inception , the ldp has been applied to a variety of general surgical procedures by a number of surgeons at our institution . during the 1-month trial period , it was used to positively identify the cystic artery during gallbladder dissection , the mesenteric blood vessels during laparoscopic colectomy , and the femoral vessels during laparoscopic hernia repair . it was found to be quick to construct , easy to use , and provided useful information to the operating surgeon . it is now available for routine use by any surgeon at our institution . to our knowledge , the first device , the endoscopic pulse detector , was designed to aid the surgeon in identifying the cystic artery during dissection in the triangle of calot . no erroneous assessments were made , and glavic et al found the device safe , reliable , and easy to use . we agree with their conclusions that an instrument to identify vascular structures during laparoscopic surgery should be part of the standard instrumentation of an advanced laparoscopic tray ; however , we do not see the need to reinvent the wheel with an endoscopic pulse detector . the second report , concerns the use of a smart needle a doppler flow transducer used to identify the testicular artery during laparoscopic varicocelectomy . these authors sought to preserve the testicular artery and ligate only the testicular vein ; they therefore used ultrasound assessment to achieve that goal . while we applaud their procedure , again , we do not feel that new equipment is necessary to be developed or purchased . the ldp is simply the extrapolation of a device used in open surgery , applied to laparoscopy , through an inexpensive delivery system . it can be placed through a 10-mm port and will likely become one of the cheapest instruments used during laparoscopic surgery . further , the ldp can be utilized to identify vascular structures and provide valuable information to the laparoscopic surgeon . with experience ,
background : intraoperative ultrasound has been used extensively during open surgery to assess bowel viability , to identify vascular structures , and to assess for congenital abnormalities . the extension of this technology in laparoscopic procedures has been hampered by the size of the equipment and the significant learning curve that accompanies its use.methods:using a readily available parks inst . co. doppler probe ( 8.1 mhz ) and a 15-inch section of thick - walled , 9.5-mm od stainless steel tubing , a laparoscopic doppler probe was constructed . the parts were separately gas - sterilized , and a small segment of penrose drain was used to create an airtight seal . the probe was passed through a 10-mm port , allowing assessment of vascular structures.results:two laparoscopic doppler probes were available for evaluation during a 1-month period at our hospital . surgeons were then surveyed at the end of the 1-month period as to the utility of the devices.conclusions:the laparoscopic doppler probe was used to identify the cystic artery during gallbladder dissection , to assess mesenteric blood vessels during laparoscopic colectomy , and to identify femoral vessels during laparoscopic preperitoneal hernia repair . it was found to be quick to construct , easy to use , and provided useful information to the operating surgeon .
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