title: [Morbus Wilson--pathogenesis, diagnosis, therapy, and course (author's transl)].
abstract: During childhood, Wilson's disease becomes manifest mostly in the hepatic form. In children every case of cirrhosis of the liver, hemolysis with high levels of conjugated bilirubin in the serum, and otherwise in explicable tremor make it imperative to exclude or confirm the existence of Wilson's disease. A false diagnosis often delays the start of therapy with d-penicillamine and low-copper diet. The prognosis, which was still fatal a few years ago, has improved considerably thanks to new therapeutic possibilities.
mesh_terms: D000367:Age Factors; D002570:Ceruloplasmin / Q000032:analysis; D002648:Child; D003300:Copper / Q000097:blood / Q000652:urine; D006461:Hemolysis; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D006801:Humans; D008103:Liver Cirrhosis / Q000209:etiology; D010396:Penicillamine / Q000627:therapeutic use; D011379:Prognosis
chemical_list: D003300:Copper; D002570:Ceruloplasmin; D010396:Penicillamine
vernacular_title: Morbus Wilson--Pathogenese, Diagnose, Therapie und Verlauf.