Progressive Intrahepatic Cholestasis of Infancy and Childhood

摘要: A patient who developed chronic cholestatic jaundice during the 1st year of life and eventually died liver cell failure at age 18 years is described. During terminal illness Kayser-Fleischer-like rings were observed serum concentrations total copper ceruloplasmin elevated. At autopsy, a mixed macronodular micronodular cirrhosis was found cholangiography dissection bile ducts revealed no obstructive lesion biliary tract. There family history hepatobiliary disease. Liver biopsies obtained ages 5 7 showed accumulation droplets in hepatocytes, normal-appearing ducts, significant fibrosis, intact lobular architecture. Striking features presence Mallory bodies marked excess (2,175 μg per g dry weight). The latter two findings, as well elevated ceruloplasmin, may be attributable to cholestasis se. This study emphasizes clinical therapeutic problems posed by unknown etiology childhood