title: Other genetic liver diseases in children.
abstract: Wilson disease is rare but proteiform, and should be suspected in any child with liver disease and older than 3 years of age. The treatment is very efficient, and must be taken life-long. Fifteen percent of patients with alpha-1-antitrypsin deficiency develop a neonatal jaundice, and 3% a cirrhosis in childhood. There is no specific treatment except liver transplantation. Five percent of cystic fibrosis patients develop a cirrhosis, with a very slow progression. Milder abnormalities are frequent, as well as biliary stones. Liver disease in ciliopathies may be a congenital hepatic fibrosis, with risks of portal hypertension and cholangitis, or a more variable biliary disease. Gilbert disease is frequent and benign. Crigler-Najjar syndrome is rare, severe, and may be an indication for liver or liver-cell transplantation.
authors.affiliation: Hôpital Necker-Enfants-malades, Hepatogastroenterology-nutrition unit, 149, rue de Sèvres, 75015 Paris, France.
authors.forename: Florence
authors.initials: F
authors.lastname: Lacaille
mesh_terms: D002614:Chelating Agents / Q000627:therapeutic use; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery; D008103:Liver Cirrhosis / Q000209:etiology; D017093:Liver Failure / Q000209:etiology / Q000601:surgery; D016031:Liver Transplantation; D000515:alpha 1-Antitrypsin / Q000235:genetics; D019896:alpha 1-Antitrypsin Deficiency / Q000175:diagnosis* / Q000235:genetics
chemical_list: D002614:Chelating Agents; D000515:alpha 1-Antitrypsin