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.

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.

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.

Bilateral persistent sciatic artery with bilateral aneurysms: case report and review of the literature. ?

Persistent sciatic artery is a rare anomaly, only 33 cases having been reported to date. A 34th case is described in this report., The patient, a 45-year-old man, had bilaterally persistent sciatic arteries with bilateral aneurysms and acute ischemia due to distal embolization. The literature regarding the embryology, incidence and the problems of diagnosis and management of this rare condition is reviewed. The authors emphasize that in cases of aneurysm formation if the sciatic nerve adheres to the aneurysm no attempt should be made to excise the aneurysmal sac.

Isolated gastric varices: prevalence, clinical relevance and natural history.

BACKGROUND: Isolated ectopic gastric varices (IGV2) are present either in the body or antrum of the stomach or upper duodenum. The prevalence, natural history and clinical significance of these varices has not been adequately described. MATERIALS AND METHODS: Consecutive patients with portal hypertension, prospectively studied and diagnosed to have IGV2, were assessed for their time of appearance - primary (at first presentation) or secondary (after obliteration of oesophageal varices), association with other varices, portal hypertensive gastropathy and any overt bleeding. RESULTS: Fifty-three of the 1128 (4.7%) patients had IGV2. The IGV2 were commonly seen in the antrum (53%), duodenum (32%), or at both sites (11%) and rarely in body and fundus (4%). IGV2 were predominantly (84.9%) secondary in origin, developing after oesophageal variceal obliteration. The median time for emergence of secondary IGV2 was 8.2 months for patients with cirrhosis, 12.8 months for non-cirrhotic portal fibrosis and 10.8 months for extra-hepatic portal vein obstruction. Eight (15%) patients had primary IGV2, 6 of them had underlying portal vein obstruction. Portal gastropathy (p < 0.05) and UGI bleeding were more common in the secondary than in primary IGV2. Bleeding due to IGV2 was seen only in 3 (5.7%) patients during a mean follow-up of 36.3 +/- 12.1 months, and could be successfully managed with endoscopic ligation or obliteration. CONCLUSIONS: Isolated ectopic gastric varices are not uncommon and generally develop following obliteration of main variceal columns. They rarely bleed and often can be managed with endoscopic interventions.

Comparison of endoscopic ligation and propranolol for the primary prevention of variceal bleeding.

BACKGROUND AND METHODS: We compared propranolol therapy and endoscopic ligation for the primary prevention of bleeding from esophageal varices. This prospective, controlled trial included consecutive eligible patients who had large varices (>5 mm in diameter) that were at high risk for bleeding. The patients were assigned to either propranolol therapy, at a dose sufficient to decrease the base-line heart rate by 25 percent, or variceal ligation, to be performed weekly until the varices were obliterated or so reduced in size that it was not possible to continue treatment. RESULTS: Of the 89 patients, 82 of whom had cirrhosis of the liver, 44 received propranolol and 45 underwent variceal ligation. The mean (+/-SD) duration of follow-up in each group was 14+/-9 and 13+/-10 months, respectively. The mean time required to achieve an adequate reduction in the heart rate was 2.5+/-1.7 days; the mean number of sessions needed to complete variceal ligation was 3.2+/-1.1. After 18 months, the actuarial probability of bleeding was 43 percent in the propranolol group and 15 percent in the ligation group (P=0.04). Twelve patients in the propranolol group and four in the ligation group had bleeding. Three of the four in the ligation group had bleeding before their varices had been obliterated. Nine patients in the ligation group had recurrent varices, a mean of 3.7 months after the initial treatment. Five patients in each group died; bleeding from the varices was the cause of death of four patients in the propranolol group and of three in the ligation group. There were no serious complications of variceal ligation; in the propranolol group, treatment was stopped in two patients because of side effects. CONCLUSIONS: In patients with high-risk esophageal varices, endoscopic ligation of the varices is safe and more effective than propranolol for the primary prevention of variceal bleeding.