title: [Treatment of Wilson's disease in children. Five case reports].
abstract: Reporting 5 cases of Wilson' disease occuring in children and expressed by a major liver involvement, the practical aspects of therapy are discussed. In 4 cases, follow-up exceeded 3 years. 1. D-Penicillillamine, a chelating agent, is administered for the purpose of inverting the cupric balance. Cupruria has, therefore, no absolute value and must be interpreted according to multiple factors. 2. The effects of D-penicillamine on the liver, as assessed by clinical, biological and histological data, appears encouraging. Laparoscopic examinations, so as to appreciate the evolution of the cirrhosis are still lacking. 3. The decrease in spleen size and signs of hypersplenism probably illustrate the reversibility of the portal hypertension. In Wilson's disease, portal hypertension requires special measures which exclude surgical portal diversion.
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D003300:Copper / Q000378:metabolism; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology / Q000601:surgery; D008099:Liver / Q000378:metabolism; D008297:Male; D010396:Penicillamine / Q000627:therapeutic use*; D011167:Portacaval Shunt, Surgical
chemical_list: D003300:Copper; D010396:Penicillamine
vernacular_title: Le traitement de la maladie de Wilson chez l'enfant. A propose de cinq observations