Progressive familial intrahepatic cholestasis: A case with improvement in liver tests and growth following partial external biliary diversion.

A 2½-year-old boy presented with pruritus and jaundice of 2 weeks duration. On investigation, serum total bilirubin was 23.4 mg/dL and gamma glutamyl transpeptidase was normal. Liver biopsy was consistent with progressive familial intrahepatic cholestasis (PFIC). A partial external biliary diversion (PEBD) was done. Pruritus disappeared, growth improved and serum total bilirubin became normal, 2 months after surgery. This is the first report from India, of PFIC treated with PEBD and suggests that PEBD should be considered in patients with PFIC even if bridging fibrosis is present.

Sub-site specific time trends of carcinoma stomach in North Kerala--a retrospective analysis.

The time trends in the incidence of carcinoma stomach in the various sub-sites in stomach have been documented in the western population. The incidence of carcinoma of gastric cardia is found to be increasing, while that of the antrum is seen to be decreasing. Data from tropics is lacking on this aspect. This retrospective study was performed to look into the site-specific time trends of carcinoma stomach. Endoscopy registers maintained in the Department of Gastroenterology, Medical College, Kozhikode, Kerala in South India were screened over the period of 20 years from 1982 to 2001. Histopathologically confirmed cases of adenocarcinoma were included in the study. The cases were categorized according to different areas of stomach--cardia, fundus, body and antrum as per International classification of diseases 0-9 (zero-nine) codes. Time trend for each sub site was calculated using one-way ANOVA and Chi-square for trend. Of the 26,886 upper GI endoscopies done over 20 years, 1771 were found to be gastric adenocarcinomas. Gastric antrum was the most common site of cancer over the years (47.8%). There was a significant rising trend for gastric cancer of the cardia without a similar trend for the cancer of the body and antrum during the study period. The sub site-specific time trends of carcinoma stomach are not similar to the pattern seen in western countries. There is a rising trend for the cancer of the cardia with a stable trend for distal cancers over 20 years.

Pancreatic pseudocyst presenting as odynophagia.

A 40-year-old man presented with abdominal pain and odynophagia. CT scan revealed a pseudocyst in the posterior mediastinum and pleural effusion complicating pancreatitis. He was managed with transpapillary pancreatic duct stenting along with other supportive measures including octreotide, and he improved symptomatically. The stent was removed after four months; there was no pseudocyst on follow up CT scan.

Liver adenomatosis.

A 27-year-old man presented with 2 months' history of right hypochondrial pain, fever and mild transient pruritus. On examination, he had firm, nontender and nodular hepatomegaly. Imaging modalities showed multiple heterogeneous lesions of varying size in the liver. Liver biopsy was consistent with hepatocellular adenoma.

Autonomic dysfunction in extra-hepatic portal vein obstruction: correlation with cardiovascular haemodynamics.

Various autonomic dysfunctions (AD) are known to occur in cirrhosis of the liver. The haemodynamic abnormalities of cirrhosis have been correlated with AD and have prognostic implications. The relevance of AD in extrahepatic portal vein obstruction (EHPVO) is not well established. We evaluated AD and cardiac indices in 30 patients, 19 male and 11 female and compared the results with those of 10 controls. The mean age of the patients and controls was 23.77 +/- 1.33 and 20.5 +/- 2.51 years, respectively. Five standard autonomic function tests were done in all the patients. Cardiac output (CO) was measured by echocardiography. Anthropometric measurements were done to determine the cardiac index (CI = Cardiac output/Body surface area) and indicized peripheral vascular resistance (iPVR) was calculated using the formula: mean arterial pressure (MAP) x 80/CI. Each autonomic function test was given a score and the results were interpreted as normal, early or definite, according to the score. AD was recorded as normal in 5, early in 11 and definite in 14 patients. None of the controls had any abnormality in autonomic function. There was a significant difference in the baseline heart rate of controls and patients (76 +/- 2.55 v. 98.9 +/- 2.96 beats/min). There was no difference in the MAP (92.65 +/- 1.71 v. 81.7 +/- 1.99 mmHg), CI (2.99 +/- 0.15 v. 3.23 +/- 0.08), iPVR (2533.59 +/- 124 v. 2176 + 104). CI, iPVR and MAP were also calculated separately in patients in the normal (N), early (E) and definite (D) AD groups. Their respective values were as follows CI: N 3.44 +/- 0.19, E 3.44 +/- 0.19, D 3.23 +/- 0.6; iPVR: N 2150 +/- 75.4, E 2140 +/- 180, D 2372 +/- 142; MAP: N 86 +/- 3.01, E 85.8 +/- 3.59, D 90.79 +/- 3.09. Results are expressed as mean +/- SE. Unlike in cirrhotics, cardiovascular haemodynamics are not altered in patients with EHPVO, even in the presence of AD.

Carbamazepine-induced eosinophilic enteritis.

We describe a 15-year-old boy who was on carbamazepine for the past 8 years for seizure disorder, who developed recurrent episodes of small bowel obstruction. Full-thickness small bowel biopsy obtained at laparotomy was consistent with eosinophilic enteritis. He improved clinically after tapering the drug.