Asymptomatic primary biliary cirrhosis. A progress report on long-term follow-up and natural history.

Thirty-six patients presenting with asymptomatic primary biliary cirrhosis have been followed for a median period of 11.4 yr, extending by 5 yr a previously reported median follow-up study of 6 yr. Life table survival analysis indicates that the overall survival of this subgroup of patients with primary biliary cirrhosis continues to remain similar to that of the general population (p = 0.

Liver ultrastructure in mitochondrial urea cycle enzyme deficiencies and comparison with Reye's syndrome.

The liver ultrastructural findings in two girls with partial carbamyl phosphate synthetase I (CPS) deficiency and their heterozygote parents and two siblings with ornithine transcarbamylase (OTC) deficiency are described. Liver ultrastructure in the four patients with inherited deficiencies of urea cycle enzymes showed minimal alterations with essentially normal mitochondria when biopsy was performed during periods of good control of their hyperammonemia. Mitochondrial ultrastructure was also essentially normal in the heterozygotes for carbamyl phosphate synthetase I deficiency.

The prognostic importance of clinical and histologic features in asymptomatic and symptomatic primary biliary cirrhosis.

To determine the life expectancy of patients with primary biliary cirrhosis, we analyzed survival data from 280 patients with either symptomatic (243) or asymptomatic (37) disease. Patients were followed for up to 19 years (mean, 6.9 years).

A prospective morphologic evaluation of hepatic toxicity of chenodeoxycholic acid in patients with cholelithiasis: the National Cooperative Gallstone Study.

A sample of 126 patients with cholelithiasis was treated with chenodeoxycholic acid (CDCA) (375 or 750 mg g.d.) for 2 years.

Arteriohepatic dysplasia: a benign syndrome of intrahepatic cholestasis with multiple organ involvement.

Arteriohepatic dysplasia (Alagille's syndrome) is presumed to be one of the familial intrahepatic cholestatic syndromes, all of which present with neonatal jaundice or failure to thrive, or both. We report the findings in five patients with this syndrome, four of whom have been followed into adulthood. In addition to hepatic dysfunction, patients had abnormalities of the cardiovascular system, eyes, bones, central nervous system, kidney, endocrine system, and habitus.