[Liver cirrhosis in metabolic disorders].
The most early cirrhosis is observed in newborns with neonatal hemachromatosis. Early cirrhosis occurs in hereditary tyrosinemia type I, peroxisomal diseases and glycogen storage disease (type IV). In Wilson's disease, a case complicated with cirrhosis was reported in a 4-year-old patient. Slowly progressive cirrhosis is seen in patients with familial progressive intrahepatic cholestasis. Focal biliary cirrhosis is found in cystic fibrosis of the pancreas. Moreover, many other metabolic disorders, except for urea cycle disorders, are occasionally or rarely complicated with cirrhosis. Early diagnosis and proper management could prevent the development of cirrhosis in patients with galactosemia, hereditary fructose intolerance, etc. The occurrence of hepatoma must be monitored in these patients. Liver transplantation is indicated in a part of the patients with cirrhosis.
Tazawa
Y
Y
Department of Pediatrics, Akita University School of Medicine.
D006528:Carcinoma, Hepatocellular / Q000209:etiology; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000209:etiology* / Q000628:therapy; D008113:Liver Neoplasms / Q000209:etiology; D016031:Liver Transplantation; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications*