Chronic Cholestasis Associated with Turner's Syndrome

Liver abnormalities seem a very rare association in Turner's syndrome, only reported in a few anecdotal cases. We report the clinical and morphological findings in 3 patients who presented with cholestasis. One of them had a definite diagnosis of primary sclerosing cholangitis, in whom the natural history was complicated by the development of two distinct tumors. A review of the few cases described in the literature and our own case reports support the hypothesis that liver disease in Turner's syndrome may have a variety of expressions including cholestasis

Double-blind, controlled study of tauroursodeoxycholic acid in elderly patients with hepatitis C virus-related cirrhosis.

Abstract: To date no effective drug has been available for the treatment of hepatitis C virus (HCV)-related cirrhosis in elderly patients. A randomized, double-blind, placebo-controlled study of tauroursodeoxycholic acid (TUDCA) was conducted in elderly patients with HCV-related cirrhosis. Forty patients (14 men and 26 women; mean age, 70 years; age range, 65 to 79 years) were randomly allocated to receive either TUDCA 500 mg/d or placebo for 6 months. At baseline, the 2 groups were comparable in terms of the frequency of the major clinical symptoms of fatigue and dyspepsia; however, pruritus was significantly more severe in the TUDCA treatment group (P < 0.05). The following assessments were made at baseline and after 3 and 6 months of treatment: a complete clinical examination and determination of aspartate aminotransferase, alanine aminotransferase, gamma-glutamyltransferase (GGT), alkaline phosphatase, total cholesterol, triglycerides, fractioned bilirubin, prothrombin time, and serum albumin. Liver ultrasonography was performed at baseline and at the end of treatment. Each clinical symptom was assessed on a 4-point scale, in which 0 = absent, 1 = mild, 2 = moderate, and 3 = severe. During the study period, 7 patients (3 in the TUDCA-treated group and 4 in the placebo group) were withdrawn; 33 patients completed the study. Pruritus decreased significantly in the TUDCA group (0.353) compared with an increase in the placebo group (0.067) (P < 0.05). Dyspepsia and fatigue did not show any statistically significant changes in either group. In the placebo group, transaminases had decreased significantly (P < 0.05) after 3 months; this decrease was maintained at 6 months. In the TUDCA group, a significant reduction in transaminase values was reached at 6 months (P < 0.01). The other biochemical variables did not show any significant change in either group. Results of this study suggest that TUDCA is ineffective in inducing a biochemical remission in HCV-related cirrhosis in the elderly

Cysteine and glutathione in the liver of patients with primary biliary cirrhosis.

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