Symptomatic volvulus due to mid gut malrotation in an adult.

Anomalies of rotation and fixation of gut occur most commonly in the neonatal period and usually become symptomatic in infancy. We report an adult patient with symptomatic volvulus due to mid gut malrotation.

Primary biliary cirrhosis: an Indian experience.

Primary biliary cirrhosis (PBC) is believed to be rare in India. We analyzed our data pertaining to patients with PBC seen in a tertiary referral center over a 5-year period. The diagnosis of PBC was based on liver biochemistry, histology and antimitochondrial antibodies, in the absence of biliary obstruction. Five patients, all women, were diagnosed to have PBC. Pruritus, jaundice and fatigue were the most common initial symptoms. Hepatomegaly was seen in 4 of 5 patients. Associated autoimmune diseases were present in 2 patients. All patients presented with mild hyperbilirubinemia (< or = 6 mg/dL) with disproportionately raised serum alkaline phosphatase level. AMA was positive in 4 patients. Liver biopsy showed stage III-IV disease in 3 of 4 patients. The clinical presentation and course of PBC in India are similar to the experience in the West.

Paraganglionoma of extrahepatic biliary tract causing obstructive jaundice.

We report a young woman with paraganglionoma arising from the extrahepatic bile duct presenting with acute obstructive jaundice. The patient underwent excision of the gall bladder and extrahepatic bile duct with the tumor, and Roux-en-Y hepaticojejunostomy. She is asymptomatic 9 months later, with normal biochemical investigations and imaging.

Clinical and endoscopic features of hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) in India.

Hereditary hemorrhagic telangiectasis (HHT) is an autosomal dominant disease characterized by recurrent epistaxis and telangiectasia of the skin and mucous membranes. Most reports of HHT are from Europe and N. America. In this report of 7 patients from India we postulate that increased skin pigmentation in Asians and Negroids masks the cutaneous manifestations of the disease but without any discernible effect on mucosal lesions. The median hemoglobin value in these patients was 4 g/dl. Endoscopic lesions in the stomach or duodenum were detected in six patients. Most patients in our report (5/7) presented with a chronic iron deficiency anaemia. The treatment of HHT is mainly supportive although our preliminary data shows that low dose ethinyl estradiol therapy decreases transfusion requirement in these patients.