Fulminant hepatic and renal failure complicating Wilson's disease.
We report a case of fatal fulminant liver failure complicating Wilson's disease that is unique in several respects. The illness supervened after several years of medical noncompliance in a patient who had been previously diagnosed and successfully treated with penicillamine. Re-institution of penicillamine therapy 2 weeks prior to the fulminant decompensation failed to prevent it. Renal failure in this patient was apparently secondary to rhabdomyolysis. Addition of penicillamine to a peritoneal dialysis solution allowed chelation and removal of over 14 mg per day of copper but without apparent benefit. Exchange transfusion and high dose dexamethasone therapy (24 mg/day) were equally ineffective in reversing the liver failure. Other reported cases have also been fatal. The best treatment for fulminant Wilson's disease is prevention by diagnosis in a pre-symptomatic stage and institution of carefully supervised life-long therapy with penicillamine.
D058186:Acute Kidney Injury / Q000209:etiology*; D000328:Adult; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008107:Liver Diseases / Q000209:etiology*; D008297:Male; D010349:Patient Compliance; D010396:Penicillamine / Q000627:therapeutic use; D012206:Rhabdomyolysis / Q000150:complications
D010396:Penicillamine