Primary systemic amyloidosis with giant hepatomegaly and a swiftly progressive course.

Although the involvement of the liver is common in systemic amyloidosis (AL), clinical features of hepatic dysfunction and liver chemistry abnormalities are often mild or absent. A mild increase in the serum alkaline phosphatase value is the most common finding. Hypertransaminasemia, hyperbilirubinemia, and portal hypertension with ascites and gastroesophageal varices occur late in the course of the disease and predict a short survival. We describe the case of a 58-year-old woman with AL, whose dramatic and unusual clinical picture, consisting of giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, esophageal varices, and ascites, was rapidly complicated by severe obstructive cholestasis

Orocecal transit time and bacterial overgrowth in patients with Crohn's disease

bacterial overgrowth is frequent in patients with crohn's disease (CD) and can contribute to symptoms. motility abnormalities can predispose to bacterial overgrowth. the hydrogen (H-2) and methane (CH4) breath test is a sensitive and simple tool for the diagnosis of bacterial overgrowth and for the evaluation of orocecal transit time (OCTT), In this study, we investigated the prevalence of OCTT modifications and bacterial overgrowth in a series of consecutive adult patients with CD. In 43 healthy subjects and 67 patients with CD, we performed the lactulose breath test using a gas analyzer that offers the opportunity of measuring both H-2 and CH4. Of the patients, 24 had undergone an ileocolic resection before the test with ablation of the ileocecal valve, at the time of the test 15 patients had active disease, whereas in 52 subjects the disease was quiescent. fifty-seven patients and forty controls were evaluable for OCTT and bacterial overgrowth. In 10 patients and in 3 controls, no H-2 or CH4 peak was recorded during the 8-hour test. Out of 57 patients, 13 (23%) were affected by bacterial overgrowth. The prevalence of bacterial overgrowth was higher in patients with previous surgery (30%) than in nonoperated patients (18%). in all patients with bacterial overgrowth, an antibiotic treatment induced a normalization of the test and an improvement of the symptoms. We observed a longer OCTT in the patients compared to controls, although this difference was not statistically significant (154 +/- 45 vs. 136 +/- 45 minutes). OCTT was significantly longer compared to controls in the 14 CD patients with previous ileocolic resection (180 +/- 53 vs. 136 + 45 minutes; p < 0.004). In conclusion, we found that a significant proportion of unselected patients with CD has bacterial overgrowth and prolongation of OCTT. ee suggest that the modifications in OCTT in patients with CD can predispose to bacterial overgrowth. The lactulose breath test is a simple method that can be more widely used in patients with CD