[Wilson's liver disease in children and adolescents (author's transl)].
Hepatic symptoms are usually the first in Wilson's disease of children and adolescents, while neurologic symptoms and the corneal ring are still missing. Liver lesions due to copper accumulation may develop throughout years without clinical symptoms or biochemical abnormalities. Hemolytic jaundice or gastrointestinal bleeding are the presenting symptoms in some cases. In spite of being a rare syndrom Wilson's disease ought to be considered after hepatitis B or autoimmune liver disease have been excluded as causes of juvenile cirrhosis of the liver. If life-long treatment with D-penicillamin is started in an early stage of Wilson's disease, prognosis is rather good.
D000293:Adolescent; D001707:Biopsy, Needle; D002570:Ceruloplasmin / Q000032:analysis; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006509:Hepatitis B / Q000175:diagnosis; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D011379:Prognosis
D002570:Ceruloplasmin
Die hepatische Manifestationsform des Morbus Wilson im Kindes- und Jugendalter.