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Wolman disease (WD) is a rare, inherited, rapidly fatal condition presenting in early infancy. The disease manifests in the first month of life with failure to thrive, vomiting, diarrhea, abdominal distension, hepatosplenomegaly and bilateral adrenal calcification and is nearly always fatal before the age of 1 year. Barring a case report of isolated fetal ascites, there is no report of intractable ascites as the presentation of WD till date. We report two siblings with WD who both had intractable ascites and required therapeutic paracentesis, albumin infusion, and diuretics to control tense ascites. Although rare, WD should be considered in the differential diagnosis of infantile ascites.

Background: Primary mesenchymal tumors of the gastrointestinal tract are a heterogeneous group of tumors with a wide clinical spectrum, of which gastrointestinal stromal tumors (GIST) typically occur in middle-aged to older individuals. This study evaluated the clinicopathological and morphological features of GIST of the intestinal tract., Methods: The study included 108 gastrointestinal and 13 extra-gastrointestinal stromal tumors involving the mesentry and retroperitoneum between January 1989 and July 2007. Immunohistochemical expression of CD117, CD34, SMA, Desmin, S100, and Ki-67 were studied., Results: GIST comprised 108 of 120 (90%) of the mesenchymal tumors. The tumor was located in the stomach in the majority (55%) of patients followed by small intestine (30%), retroperitoneum (7%) and the colorectum (4%). There was a significant correlation between tumor size and mitotic index, with larger tumors having higher mitotic index (p < 0.001). Mitotic index per 5 mm(2) correlated with high cellularity (p < 0.001), presence of necrosis (p < 0.001) and presence of mucosal invasion (p = 0.01). Expression of CD117 was seen in 94%, CD34 in 59%, SMA in 41%, S-100 in 33%, and desmin in 4% of tumors., Conclusion: We found GIST to be the most common mesenchymal tumor of the gastrointestinal tract.

Primary gastric lymphoma, an uncommon gastric tumor caused by infection with Helicobacter pylori, is rarely associated with gastric amyloidosis. Chronic bacterial infection is known to cause amyloidosis. We report a 53-year-old man who had an antral and duodenal mass with narrowing and ulceration on endoscopy and CT scan; endoscopic biopsy revealed gastric amyloidosis. Rapid urease test and serology for H. pylori were positive. Histology of resected specimen of distal stomach revealed primary gastric lymphoma, amyloid deposits and spiral organisms suggestive of H. pylori. Rectal biopsy was negative for amyloid. He remained well on follow-up after surgery and eradication of H. pylori.

Background: Despite a possible role of Helicobacter pylori in gastric carcinoma (GC), its pathogenesis is not clear. There is scanty data on apoptosis in GC in relation to H. pylori and CagA antibody. Therefore, we studied gastric epithelial apoptosis in GC and non-ulcer dyspepsia (NUD) with or without H. pylori infection, and the degree of apoptosis in relation to CagA antibody status., Methods: 20 patients each with GC and NUD were investigated for H. pylori using rapid urease test (RUT), IgG anti-H. pylori and anti-CagA antibodies, histology of endoscopically normal-looking mucosa for H. pylori, intestinal metaplasia (IM), and apoptosis using TUNEL assay. Positivity to one tissue-based (RUT or histology) and one serology based (anti-H. pylori or CagA IgG) test was taken as diagnostic of active H. pylori infection, and negative result in both tissue-based tests suggested its absence., Results: Patients with GC more often had anti-H. pylori IgG (16 of 20 vs. 8 of 20; p=0.02) and a trend towards higher apoptotic index (AI) (48.6 [19.2 to 71.7] vs. 41.4 [11.7 to 63.6]; p=0.06) than NUD. AI was higher in GC (66.7 [57.5 to 71.7] vs. 32.6 [19.2 to 39.8]; p<0.0001) and NUD (58.6 [50.7 to 63.6] vs. 24.4 [11.7 to 32.2]; p<0.0001) infected with H. pylori than in those without infection. AI was also higher in GC than in NUD with H. pylori infection (66.7 [57.5 to 71.7] vs. 58.6 [50.7 to 63.6]; p=0.01). Four of the 20 patients with GC and none with NUD had IM (p=ns). There was no difference in AI in relation to CagA antibody. AI positively correlated with patients' age in presence of H. pylori infection (correlation coefficient=0.5, p=0.03) but not in its absence., Conclusion: Exaggerated apoptosis may play a role in H. pylori-mediated gastric diseases including carcinogenesis. AI increases with aging in patients infected with H. pylori.

Background: The etiology of malabsorption syndrome (MAS) may differ in different geographical regions. Limited data are available on the etiological spectrum of MAS among Indian adults., Methods: Ninety-nine consecutive adult patients with MAS (urine d-xylose <1 g/5 g/5 h with or without increased fecal fat (> or =7 g/24 h) were evaluated for cause of MAS using standard criteria. Past medical records were examined to know the nature of treatment received., Results: The etiology of MAS was: tropical sprue 39, celiac disease 9, Crohn's disease 9, giardiasis 8, small intestinal bacterial overgrowth in absence of another cause of MAS 8, panhypogammaglobulinemia 2 (one with strongyloidiasis), intestinal lymphangiectasia 1, intestinal tuberculosis 4, idiopathic 15, acquired immunodeficiency syndrome 2, and amyloidosis 2. Twenty-eight patients had received anti-tubercular treatment earlier., Conclusions: Tropical sprue, celiac disease and Crohn's disease are common causes of MAS in Indian adults. Inappropriate anti-tubercular treatment is common in them and needs to be discouraged.

Objectives: To analyze the morphological changes in bile ducts following endobiliary stent insertion, and consequent technical problems encountered at surgery., Methods: Data on bile duct morphology--gross (luminal diameter and wall thickness) and microscopic (histological changes in bile duct wall graded semiquantitatively)--and operative parameters related to bile duct dissection (grade of difficulty in dissection) were collected prospectively in 31 consecutive patients undergoing pancreatico-duodenectomy. These data were compared between patients who had undergone preoperative endoscopic biliary stent placement (n=17) and those who had not (n=14)., Results: Mean duration of stenting before surgery was 34 (range 10-120) days. Stented ducts were significantly narrower (luminal diameter 9 [7-12] mm vs. 17.5 [8-23] mm; p=0.0001) and had thicker walls (2.3 [1.3-3.5] mm vs. 1.85 [0.8-2.2] mm; p=0.004) compared to non-stented ones. On microscopy, stented ducts had advanced grades of submucosal gland hypertrophy, fibrosis and inflammatory cell infiltrate. Difficulty in bile duct dissection was encountered more often in patients who had been stented than in those without stents, though the difference was not statistically significant., Conclusion: Endobiliary stent placement results in significant morphological and fibroproliferative inflammatory changes in bile ducts, making dissection difficult.