Sweet's Syndrome in a Case of Ulcerative Colitis-Case Report and Review of Literature.

ABSTRACT: Sweet syndrome,also known as acute febrile neutrophilic dermatosis , is one of the rare cutaneous association of ulcerative colitis.Only few cases of Sweet syndrome associated with ulcerative colitis have been reported in literature. We herein describe a case of young female with acute exacerbation of ulcerative colitis associated with erythematous , papular skin lesions which on biopsy were consistent with Sweet syndrome. Treatment with intravenous steroids resulted in improvement of ulcerative colitis and disappearance of cutaneous lesions. Cutaneous lesions of Sweet syndrome in ulcerative colitis parallel the bowel disease activity in majority of the cases but sometimes may precede the intestinal symptoms and rarely may appear after procto-colectomy for acute severe ulcerative colitis.Introduction.

Prediction of nonalcoholic fatty liver in prospective liver donors.

BACKGROUND: Metabolic risk factors should be important in addition to imaging for prediction of steatosis in prospective liver donors. MATERIALS AND METHODS: The study group included all prospective liver donors who had a liver biopsy during workup. Risk factors of metabolic syndrome were analyzed, and body mass index (BMI) ≥25 kg/m2 was used in place of waist circumference. Three BMI cutoffs (25, 28, and 30 kg/m2 ) and two CT-measured liver attenuation index (LAI) cutoffs (<5 and ≤10) were used for steatosis assessment of ≥5%, ≥10%, and ≥20%. RESULTS: Of the 573 prospective donors (307 females), 282 (49.2%) donors had nonalcoholic fatty liver (NAFL). When donors with NAFL were compared with donors having normal histology, multivariate analysis showed BMI, ALT, triglycerides, and LAI as significant predictors of NAFL. BMI ≥25 kg/m2 and LAI <10 were better cutoffs. The presence of ≥2 metabolic risk factors had better sensitivity than CT-LAI for the presence of NAFL and ≥20% steatosis (58% and 54% vs 47% and 22%, respectively, for CT-LAI ≤10). The presence of LAI >10 and <2 metabolic risk factors predicted <10% steatosis with 96% specificity and 92% positive predictive value. CONCLUSION: The presence of ≥2 metabolic risk factors improves sensitivity of CT-LAI for prediction of donor steatosis.

Gastrointestinal Histoplasmosis and CMV Co-Infection in an Immunocompetent Host.

A middle aged male with no known comorbidities presented with history of colicky abdominal pain, low grade fever and weight loss. Laboratory parameters were normal except low albumin. Imaging showed multiple areas of mural thickening with enhancement in jejunum & ileum. On Colonoscopy there was a thickened and deformed ileum with multiple ulcers. The biopsy showed co-infection of CMV and histoplasma, urine antigen for histoplasma was positive and CMV DNA detected in blood. He was successfully treated with combination of Valgancyclovir and Amphotericin-B followed by itraconazole.

Gastric Angioinvasive Mucormycosis in Immunocompetent Adult, A Rare Occurrence.

Mucormycosis is a rare, opportunistic fungal infection that occurs almost exclusively in immunocompromised hosts such as patients with diabetes mellitus, leukemia, lymphoma, renal disease, septicemia, burns, malnutrition, and following long-term treatment with steroids and antibiotics. Based on the clinical presentation and involvement, mucormycosis is classified as six major forms, namely, rhinocerebral, pulmonary, cutaneous, gastrointestinal (GI), disseminated and miscellaneous, with rhinocerebral and pulmonary being the common forms. GI mucormycosis is rare, accounting for only 7% of all cases; however, the mortality rate is as high as 85%. Here we report a case of a young immunocompetent male who developed gastric invasive mucormycosis during an acute illness and succumbed to it despite all supportive care.

Estimation of normal values of serum transaminases based on liver histology in healthy Asian Indians.

BACKGROUND AND AIM: Liver biopsy-based studies have shown that serum levels of aminotransferases are lower than conventional cut-off of 40 IU/mL in persons with normal histology. There is no such study in Indian population based on liver histology. This study aims to estimate normal values of serum aminotransferases in healthy Indian population with normal liver histology. METHODS: This retrospective study includes all liver donors who underwent liver donation at our centre and had a preoperative liver biopsy done for various reasons. All the donors had negative viral markers. Nonalcoholic fatty liver (NAFL) was defined as > 5% hepatocytes having steatosis and no changes of steatohepatitis. RESULTS: The study included 331 donors (147 males) with the ages of 35.7 ± 10.2 years. NAFL was present in 167 donors (50.4%). In comparison with male donors with normal histology (n = 67), donors with NAFL (n = 80) had significantly higher age, body mass index, aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase, gamma-glutamyl transpeptidase, total cholesterol, low-density lipoprotein, and fasting blood sugar. In comparison with female donors with normal histology, donors with NAFL had significantly higher body mass index, ALT, and triglycerides; however, there was no significant difference regarding other parameters. Of the AST and ALT in normal histology donors, 95th percentile were 33.8 IU/L and 38.6 IU/L for males and 31 IU/L and 35.2 IU/L for females. Twenty-five donors had lean NAFL (body mass index < 23 kg/m2). CONCLUSION: Serum aminotransferase values in healthy Asian Indian population with normal histology are provided. Histological NAFL is present in half of apparently normal donors, and it has different clinical and biochemical associations in males and females.

The etiological profile of chronic organic non-bloody diarrhea in India: Emergence of inflammatory bowel disease as a dominant cause.

BACKGROUND: Chronic non-bloody diarrhea may be attributed either to functional or organic diseases. The latter category may present with malabsorption syndrome if there is extensive involvement of the small bowel, whereas diseases of the large bowel may only present with diarrhea sans malabsorption. Indian data has predominantly focussed on the etiological spectrum of malabsorption syndrome in adults. The primary aim of the current study was to evaluate etiological spectrum of chronic organic non-bloody diarrhea in India. METHODS: This prospective observational study was done at a tertiary care hospital in North India. Patients ≥ 18 years presenting with chronic non-bloody diarrhea of > 4 weeks duration were enrolled in the study after exclusion of patients with IBS and anal incontinence. RESULTS: During the study period of 12 months, 100 patients with chronic organic non-bloody diarrhea were evaluated. A definite etiological diagnosis was made in 97 patients (97%). The mean age of the patients was 48 ± 16.7 years (58% males). The median duration of diarrhea was 5.5 months (interquartile range [IQR] 3.5, 11). Inflammatory bowel disease (IBD) accounted for 45% of the cases making it the predominant cause for organic diarrhea. GI infections and adult-onset celiac disease accounted for 18% and 9% of the cases, respectively. Pancreatic disease, benign or neoplastic, accounted for 6% of the total cases. Notably, gastrointestinal (GI) malignancies manifesting as chronic non-bloody diarrhea were diagnosed in 5% of the patients. CONCLUSION: Our data suggests a paradigm shift in the etiological spectrum of chronic organic non-bloody diarrhea in India with the emergence of IBD as the predominant cause displacing GI infections.

Cytomegalovirus Duodenitis Causing Persistent Hypoalbuminemia and Ascites After Liver Transplantation.

A 44-year-old male had persistent hypoalbuminemia and ascites after liver transplantation. Imaging of the liver and gastrointestinal system was normal. Urine examination was negative for proteinuria. A diagnosis of protein-losing enteropathy was suspected, and a duodenal biopsy was done. Duodenal biopsy was positive for cytomegalovirus (CMV). The patient improved with CMV treatment.

Fine-needle aspiration cytology of parotid mucinous myoepithelioma with follow-up histopathology: A case report.

Myoepitheliomas are rare tumours of salivary glands. Mucinous myoepithelioma (MM) is a newly described variant with rare cases reported in the literature. This case report highlights cytopathologic features with histologic follow up of MM.

Nonalcoholic Fatty Liver Disease in Living Donor Liver Transplant Recipients: A Histology-Based Study.

Background: Recurrent or de novo nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH) are common after liver transplantation (LT) and may be associated with rapid progression to fibrosis; however, there is limited data in this regard after living donor liver transplantation (LDLT). Material and methods: This is a retrospective study at a high volume LDLT center of all liver biopsies performed in patients with post-transplant NAFLD diagnosed on ultrasound of the abdomen. Liver biopsy was indicated for raised transaminases and/or high liver stiffness on TE. The association between these prebiopsy parameters and inflammation and fibrosis on histology was analyzed. Data are shown as mean ± standard deviation or median (25-75 interquartile range). Results: The study cohort consisted of 31 males and 3 females, aged 43 ± 10 years. The LT to liver biopsy interval was 44 (28-68) months. The prebiopsy AST and ALT were 71 (38-119) and 66 (50-156), respectively. The histology suggested no nonalcoholic steatohepatitis (NASH) in 7 (20%), borderline NASH in 15 (44%), and NASH in 12 (35%) patients. A total of 15 patients (44%) had stage 1 or stage 2 fibrosis. The proportion of patients having fibrosis was significantly higher in patients with NASH (83%) compared to patients with borderline NASH (33%) or no NASH (none had fibrosis, P = 0.001). Among 18 patients who underwent TE (on FibroScan), liver stiffness was significantly higher in patients with fibrosis [18.1 (9.7-22.5)] than in those without fibrosis [9.7 (4.0-12.7); P = 0.043]. Conclusion: Over a third of the LDLT recipients with post-transplant NAFLD developed NASH, and nearly half, borderline NASH 3-5 years after transplant. Most with established NASH also had fibrosis on histology. Prevention of risk factors and early diagnosis is warranted in these patients.

Common variable immunodeficiency syndrome with chronic diarrhoea.

Common variable immunodeficiency syndrome (CVID) is a heterogeneous disorder characterised by diminished levels of IgG, IgA and/or IgM, and recurrent bacterial infections. Sinopulmonary infections are most commonly reported followed by gastrointestinal (GI) infections. GI tract represents the largest immune organ with abundance of lymphoid cells, its involvement can manifest variably ranging from asymptomatic involvement to florid symptoms and signs. Diffuse nodular lymphoid hyperplasia (DNLH) of the GI tract is characterised by numerous small polypoid nodules of variable size in the small intestine, large intestine or both. It is commonly seen in association to immunodeficiency states such as CVID, IgA deficiency and chronic infections due to Giardia lamblia and Helicobacter pylori and cryptosporidiosis. Repetitive antigenic stimulation leads to lymphoid hyperplasia. We herein describe a case of DNLH of the intestine and another case of duodenal cytomegalovirus (CMV) infection associated with CVID.

Skin involvement in Burkitt's lymphoma.

Skin involvement in Burkitt's lymphoma (BL) is rare, more so in the pediatric age group. There are very few cases of BL involving skin either at presentation or relapse reported in literature. We report a case of a 9-years old boy with stage 4 Burkitt's lymphoma with skin involvement who tested negative for human immune-deficiency virus.

Gastrointestinal Histoplasmosis: A Case Series.

Histoplasmosis is an invasive mycosis caused by inhalation of the spores of dimorphic fungi Histoplasma capsulatum. The disease manifests in the lung as acute or chronic pulmonary histoplasmosis and in severe cases gets disseminated in multiple organs like skin, adrenal gland, central nervous system, lymph node, liver, spleen, bone marrow, and gastrointestinal tract. It occurs most commonly in immunodeficient patients like HIV-positive patients and transplant recipients, while immunocompetent hosts are affected rarely. In cases of gastrointestinal histoplasmosis, the samples are collected for culture and biopsy should be sent for histopathological examination for definitive diagnosis. We conducted a retrospective study of colonic biopsies performed in the department of gastroenterology in a tertiary care hospital of north India from January 2014 to December 2015. Five cases of colonic histoplasmosis were diagnosed on histopathology out of which 4 patients were from north India while 1 patient was from Myanmar. The patients presented with various complaints, including loose stools, diarrhea, altered bowel habits, and gastrointestinal bleeding. The prognosis is very good after early and aggressive treatment while the disease is fatal if it remains untreated. In our study, 2 patients died within few days of diagnosis due to delay in the diagnosis, dissemination, and associated complications. Other patients were started on amphotericin B deoxycholate and are under follow-up. An early diagnosis of gastrointestinal histoplasmosis is important as appropriate treatment leads to long-term survival while untreated cases are almost fatal.

Adrenal Histoplasmosis in Immunocompetent Patients Presenting as Adrenal Insufficiency.

OBJECTIVE: Histoplasmosis is an infectious disease caused by the dimorphic fungus Histoplasma capsulatum, endemic in central and eastern states of United States, South America and Africa. India is considered to be non-endemic area for histoplasmosis. Disseminated histoplasmosis may affect almost all systems. Disseminated histoplasmosis with asymptomatic adrenal involvement has been described in immunocompromised patients; whereas isolated adrenal involvement with adrenal insufficiency as the presenting manifestation of the disease is rare. MATERIAL AND METHOD: Twelve patients from a non-endemic area with adrenal histoplasmosis, who were immunocompetent and diagnosed as adrenal histoplasmosis by cytology/histopathology between January 2012 to December 2014 were studied. 18F-FDG PET/CT (fluorodeoxyglucose positron emission tomography/computed tomography) was used to assess the extent of involvement. RESULTS: There were a total of 12 immunocompetent males (mean age: 56.9 years). Ten patients had bilateral adrenal involvement and two had a unilateral left adrenal mass. All the patients had histopathologically/cytologically proven adrenal histoplasmosis. Two patients had simultaneous histoplasmosis of other sites, one in the epiglottis and the other in the alveolus. 18F-FDG PET/CT was performed in 10 patients showing high FDG uptake in the adrenals. All these patients received Amphotericin B and/or Itraconazole treatment that led to symptomatic improvement. CONCLUSION: A diagnosis of invasive fungal infection requires a high index of suspicion, especially in immunocompetent patients who present with nonspecific symptoms, clinical signs, laboratory and radiological features that can resemble adrenal neoplasms. Clinical specimens must be sent for cytopathology/histopathology together with fungal culture for a definite diagnosis and appropriate management.

Nonalcoholic fatty liver is not associated with incident chronic kidney disease: a large histology-based comparison with healthy individuals.

BACKGROUND: Nonalcoholic steatohepatitis or fibrosis is associated with the increased prevalence of impaired kidney function. It is not known whether nonalcoholic fatty liver (NAFL), which is steatosis without inflammation or fibrosis, is associated with renal impairment as these individuals are not candidates for liver biopsy. MATERIALS AND METHODS: The study group included all liver donors who underwent a preoperative liver biopsy for various reasons. NAFL was defined as more than 5% hepatocytes having steatosis and no changes of steatohepatitis and/or fibrosis. Individuals with NAFL were compared with individuals with normal liver histology. Estimated glomerular filtration rate was calculated with the Modification of Diet in Renal Disease and Chronic Kidney Disease Epidemiology Collaboration methods. RESULTS: The mean age of the study group was 35±10.3 years and the mean BMI was 26±3.2 kg/m. A total of 187 adults having NAFL (80 men) were compared with 186 (88 men) individuals with normal liver histology (controls). Individuals with steatosis had significantly higher BMI (26.8±3.5 vs. 25.5±3.8 kg/m, P<0.001) and serum alanine transaminase levels (33.8±13.3 vs. 27.2±9.8 IU/l, P<0.000). There was no significant difference in the estimated glomerular filtration rate between the normal histology group and the NAFL group with Modification of Diet in Renal Disease (115±21.8 vs. 114.4±23.2 ml/min, P=0.803) and Chronic Kidney Disease Epidemiology Collaboration methods (113.6±14.8 vs. 113±14.7 ml/min, P=0.668). There was no significant difference as regards serum creatinine (0.73±0.16 vs. 0.74±0.16 mg%, P=0.536), proteinuria (P=0.121), diabetes (1 vs. 4, P=0.372), and hypertension (2 vs. 6, P=0.174) in individuals of both groups. CONCLUSION: Presence of NAFL does not adversely affect renal function.

ALK Positive Anaplastic Large Cell Lymphoma Presenting as Extensive Bone Involvement.

Anaplastic lymphoma kinase (ALK) positive Anaplastic large cell lymphoma (ALCL) represents approximately 2% of all Non-Hodgkin's lymphomas that commonly involves nodal as well as a wide variety of extra nodal sites, as skin, soft tissue, bones and lungs, although primary or secondary involvement of bone is rare. Herein, we report a case of 14-year-old female child presented as extensive bony involvement with a clinical diagnosis of bone tumour/ small round cell tumour, which was proved to be ALK positive ALCL on histopathological examination.

Genotype 3 and higher low-density lipoprotein levels are predictors of good response to treatment of recurrent hepatitis C following living donor liver transplantation.

BACKGROUND: Treatment of recurrent hepatitis C after liver transplantation is associated with poor sustained virological response (SVR) in genotype 1, and data on genotype 3 is limited to small numbers. We report one of the largest series of genotype 3 patients treated for recurrent hepatitis C following living donor liver transplantation (LDLT). METHODS: From January 2002 to November 2013, of 1349 transplants, 359 patients had hepatitis C. Patients with histological recurrence were treated with pegylated interferon in escalating dose regimen for 48 weeks and weight-based ribavirin. Virological response was defined as rapid virological response (RVR-4 weeks), early virological response (EVR-12 weeks), end of treatment response (ETR-48 weeks), and SVR (24 weeks after end of treatment). SVR and no-SVR groups were compared for age, sex, body mass index (BMI), diabetes, total cholesterol, low-density lipoprotein (LDL), high-density lipoprotein (HDL), triglycerides, ferritin, genotype, and hepatitis C virus (HCV) RNA levels before initiation of treatment. RESULTS: The study included 67 patients who had at least 18 months of follow up after treatment initiation (45 males), aged 51 ± 6.3 years. Treatment was started after 16 months (median); baseline median RNA was 2.7 × 10(6) IU/mL. There was no significant difference between the SVR and no-SVR groups regarding age, sex ratio, follow up period, total cholesterol, triglycerides, HDL, BMI, prevalence of diabetes, HCV RNA, and ferritin levels. Genotype 3, RVR, EVR, ETR, and higher LDL levels were significantly associated with SVR. Genotype 3 had a significantly higher SVR rate of 71 % as compared to an SVR rate of only 14 % in genotype 1, p = 0.0003. Absence of RVR and EVR predicted treatment failure with a specificity of 88 % and 92 %, respectively. CONCLUSION: Genotype 3 and higher LDL levels predict SVR in patients treated for hepatitis C recurrence following LDLT, whereas absence of RVR and EVR strongly predict treatment failure.

Rapid Reversal of Liver Steatosis With Life Style Modification in Highly Motivated Liver Donors.

BACKGROUND: Liver steatosis is the leading cause of donor rejection in living donor liver transplantation. Rapid weight loss is difficult to achieve in a short period of time, moreover it has been thought to worsen liver histology. METHODS: Donors who had significant steatosis based on liver biopsy were recommended 1200 Kcal/day and a minimum of 60 min/day moderate cardio training. Two patients were advised statins for dyslipidemia. None of the donors had metabolic syndrome. A second ultrasound guided liver biopsy was done at 28 ± 10 days. Donors with nonalcoholic steatohepatitis/fibrosis or >30% steatosis were not included. RESULTS: From July 2010 to January 2015, 16 donors were advised aggressive life style modification after initial biopsy; 15 (10 males, age 27.5 ± 6.5 years, baseline body mass index 28.4 ± 2.1 Kg/M(2)) successfully reduced weight and 14 underwent donation after favorable second biopsy. Mean weight loss was 7 ± 4.3 kg (8.4 ± 4.6%). Second liver biopsy was done at 28 ± 10 days, there was decrease in steatosis in all but one including normalization of liver biopsy in 7 donors. Three donors had mild inflammation on first biopsy and they had improvement in second biopsy. All the donors and their recipients had an uneventful post-operative course. CONCLUSION: Steatosis can be reversed in a short duration by aggressive life style modifications in highly motivated liver donors.