Intrahepatic Cholestasis for 15 Years without Cirrhosis

Whether prolonged cholestasis is followed by hepatic rrhosis is still controversial. We have studied two unrelated Children who have had cholestasis for 15 years, but neither whom have developed cirrhosis. Both have severe growth tardation, peculiar facies, pulmonic stenosis, transitory lal tubular acidosis, and vitamin D-resistant rickets. The tients presented in infancy with hepatomegaly and direct perbilirubinemia; liver biopsy at that time revealed cho- asis and paucity of bile ducts. Subsequent serial liver isies have continued to demonstrate cholestasis, but there been no evidence of cirrhosis. Electron microscopy has lied swollen and blunted microvilli of the canalicular rane of the hepatocyte. The patients have had elevated cids in the serum as well as a reversed ratio of tri- to oxy bile acids. Treatment with cholestyramine and pattobarbital has brought about symptomatic relief from esot pruritus and excoriation and has lowered the level of bile acids, although they are still above the normal e. These findings suggest that cholestasis accompanied elevated and reversed bile acid ratio does not univer-ting cause hepatic cirrhosis.