Natural History and Treatment Outcomes of Severe Autoimmune Hepatitis.

GOALS: The aim of this study was to analyze the natural history and treatment outcomes of autoimmune hepatitis (AIH) variants presenting with severe-AIH. BACKGROUND: Severe acute presentation is an uncommon manifestation of AIH, and it remains poorly characterized. MATERIALS AND METHODS: We included 101 patients with AIH from January 2011 to December 2015. Patients were classified as seropositive-AIH and seronegative-AIH. Patients with acute liver failure, acute-on-chronic liver failure, and severe acute hepatitis were defined as severe-AIH patients. Patient characteristics and treatment outcomes with follow-up until 12 months were analyzed between the different groups. RESULTS: Out of 101 cases, 24 (23.76%) had severe AIH. Of them 9 (37.5%) had severe acute hepatitis, 3 (12.5%) had acute liver failure, and 12 (50%) had acute-on-chronic liver failure. Seronegative-AIH patients presented with severe-AIH significantly more frequently compared with seropositive-AIH patients (50% vs. 20.27%, P=0.022). Severe-AIH had 50% complete responders, 25% partial responders, and 25% treatment failures. Jaundice (88.88% vs. 68.7%, P=0.048), encephalopathy (55.55% vs. 6.66%, P=0.014), and higher international normalized ratio values (2.17±0.60 vs. 1.82±0.14, P=0.038) were factors associated with nonresponse rather than the presence or absence of autoantibodies in severe-AIH. The hazard ratio for predicting remission in the non-severe AIH group as compared with the severe-AIH group was 1.502, which was statistically not significant (95% CI, 0.799-2.827; P=0.205). CONCLUSION: Approximately 24% of patients with AIH have severe-AIH. Conventional autoantibodies are often absent in severe-AIH; however, it does not alter the outcome. Immunosuppressants should be given expediently in patients with severe-AIH.

Changing clinical profile and factors associated with liver enzyme abnormalities among HIV-infected persons.

The spectrum of liver disease among HIV-infected patients is changing. In the era of antiretroviral therapy, opportunistic infections are diminishing and deranged liver function appears to be due usually to drug-induced liver injury, alcohol, non-alcoholic steatohepatitis (NASH) or chronic hepatitis B. To test this hypothesis, 98 HIV-positive patients with deranged liver function were compared with matched HIV-positive patients with normal liver function and likewise matched HIV-negative patients with normal liver function tests.

Most overweight and obese Indian children have nonalcoholic fatty liver disease.

 Background and rationale. Nonalcoholic fatty liver disease (NAFLD) is the most common cause of pediatric liver disease in western countries. Its prevalence in Indian subcontinent is not well studied. MATERIAL AND METHODS: In a school based cross sectional study we have screened overweight and obese children in the age group of 11 to 15 years for NAFLD. Ultrasonography, elevated serum transaminases, fibroscan were used for defining NAFLD. Dietary habits, blood pressure, serum lipid profile, blood counts and insulin resistance were recorded. The relation of fibrosis 4 score, pediatric NAFLD fibrosis index, aspartate transaminases to platelet ratio index (APRI) with fibroscan was evaluated. RESULTS: Out of 616 students screened 198 were overweight and obese. Hundred students and their parents gave informed consent for the further evaluation. The prevalence of NAFLD was 62% in overweight and obese children. Fatty liver was found in 50 % students on ultrasonography, liver stiffness (≥ 6.1 Kilopascals) in 23% and raised alanine transaminase in 30%. Hypertension, dyslipidemia, diabetes mellitus and insulin resistance were seen in 6%, 18%, 2% and 66% students respectively. Systolic hypertension, serum triglyceride, aspartate transaminase, APRI was significantly higher in the NAFLD group. On binary logistic regression only systolic hypertension was an independent risk factor for NAFLD. CONCLUSION: In conclusion NAFLD is common in asymptomatic overweight and obese Indian children. Systolic hypertension is the only independent factor associated with NAFLD. Fibroscan has limited role for screening. We recommend screening for NAFLD in this high risk group with alanine transaminases and ultrasonography.

Congenital Esophageal Duplication Cyst: A Rare Cause of Dysphagia in an Adult.

Esophageal duplication cyst is a rare congenital embryonal gastrointestinal (GI) malformation which is diagnosed most commonly in childhood. In adults, they can present with a variety of symptoms ranging from dysphagia, chest pain, epigastric discomfort, and vomiting to more serious complications including infections, hemorrhage, and ulcerations. A 30-year-old male presented with gradually progressive dysphagia to solids for 4 months without significant weight loss. Clinical examination and routine laboratory examination were unrevealing. Upper GI endoscopy revealed a well-defined submucosal lesion bulging into the esophageal lumen involving the right antero-lateral wall of the distal esophagus. The overlying mucosa was normal with mild luminal narrowing but gastroscope could be negotiated across this narrowing. Differential diagnosis included lipoma, leiomyoma or GI stromal tumors. Contrast-enhanced computed tomography of thorax revealed a 3.5 × 2.3 × 3 cm well-defined homogenous hypodense lesion involving the right antero-lateral wall of the distal thoracic esophagus with likely possibility of submucosal or intramural lesion. Subsequently, endoscopic ultrasonography (EUS) revealed a 3.3 × 2.8 cm homogenous hypoechoic lesion without any vascularity involving the distal esophagus wall suggestive of duplication cyst. The lesion was intramural in location as muscularis propria was seen to go around the lesion. Bronchogenic cyst was excluded due to absence of cartilage and close proximity of the cyst to lumen. Fine-needle aspiration was not attempted in view of high risk of introducing infection. Being symptomatic, the patient underwent complete surgical excision of the cyst with exteriorization of the base which on histopathology confirmed duplication cyst. Esophageal duplication cysts are exceedingly rare congenital embryonal malformations with estimated prevalence of 0.0122% arising from aberration of posterior division of embryonic foregut at 3 - 4 weeks of gestation. This case shows that duplication cysts can rarely masquerade as submucosal tumor in adults and EUS is highly accurate in pre-operative diagnosis and therapeutic decision making. Literature search revealed only a handful of cases of EUS-guided diagnosis of esophageal duplication cyst reported in the literature.

Alström Syndrome with Portal Hypertension.

Alstrom syndrome is an autosomal recessive multisystem disorder caused by mutation in ALMS1 (2p13). Very few cases of same are reported so far of same. We report a case of Alstrom syndrome (AS) who presented with type II diabetes mellitus and portal hypertension. Unilateral anorchia with hypergonadotropic hypogonadism is another unique feature of our case.

Metronidazole-Induced Encephalopathy in Alcoholic Liver Disease: A Diagnostic and Therapeutic Challenge.

BACKGROUND: Acute encephalopathy in a patient with alcoholic liver disease (ALD) is a commonly encountered emergency situation occurring most frequently due to liver failure precipitated by varying etiologies. Acute reversible cerebellar ataxia with confusion secondary to prolonged metronidazole use has been reported rarely as a cause of encephalopathy in patients with ALD. CASE REPORT: We describe a decompensated ALD patient with recurrent pyogenic cholangitis associated with hepatolithiasis who presented to the emergency department with sudden-onset cerebellar ataxia with dysarthria and mental confusion after prolonged use of metronidazole. Magnetic resonance imaging (MRI) of the brain was suggestive of bilateral dentate nuclei hyper intensities on T2 and fluid-attenuated inversion recovery sections seen classically in metronidazole-induced encephalopathy (MIE). Decompensated liver cirrhosis resulted in decreased hepatic clearance and increased cerebrospinal fluid concentration of metronidazole leading to toxicity at a relatively low total cumulative dose of 22 g. Both the clinical symptoms and MRI brain changes were reversed at 7 days and 6 weeks, respectively, after discontinuation of metronidazole. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: A patient with ALD presenting with encephalopathy creates a diagnostic dilemma for the emergency physician regarding whether to continue metronidazole and treat for hepatic encephalopathy or to suspect for MIE and withhold the drug. Failure to timely discontinue metronidazole may worsen the associated hepatic encephalopathy in these patients. Liver cirrhosis patients have higher mean concentration of metronidazole and its metabolite in the blood, making it necessary to keep the cumulative dose of metronidazole to < 20 g in them.

Unusual complication of amebic liver abscess: Hepatogastric fistula.

Amebic liver abscess is a parasitic disease which is often encountered in tropical countries. A hepatogastric fistula secondary to an amebic liver abscess is a rare complication of this disease and there are only a handful of reported cases in literature. Here we present a case of an amebic liver abscess which was complicated with the development of a hepatogastric fistula. The patient presented with the Jaundice, pain and distension of abdomen. The Jaundice and pain improved partially after he had an episode of brownish black colored increase in frequency of stools for 5 to 6 d. Patient also had ascites and anemia. He was a chronic alcohol drinker. Esophagogastroduodenoscopy performed in view of the above findings. It showed a fistulous opening with bilious secretions along the lesser curvature of the stomach. On imaging multiple liver abscesses seen including one in sub capsular location. The patient was managed conservatively with antiamebic medications along with proton pump inhibitors. The pigtail drainage of the sub capsular abscess was done. The patient improved significantly. The repeat endoscopy performed after about two months showed reduction in fistula size. A review of the literature shows that hepatogastric fistulas can be managed conservatively with medications and drainage, endoscopically with biliary stenting or with surgical excision.