Fulminant hepatic and renal failure complicating Wilson's disease.

Abstract

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.

• Authors: W G Rector Jr, T Uchida, G C Kanel, A G Redeker, T B Reynolds
• Journal: Liver (Liver) Vol. 4 Issue 6 Pg. 341-7 (Dec 1984) ISSN: 0106-9543 [Print] Denmark
• PMID: 6521614 (Publication Type: Case Reports, Journal Article)

Chemical References

• Penicillamine

Topics

• Acute Kidney Injury (etiology)
• Adult
• Hepatolenticular Degeneration (complications, drug therapy, pathology)
• Humans
• Liver (pathology)
• Liver Diseases (etiology)
• Male
• Patient Compliance
• Penicillamine (therapeutic use)
• Rhabdomyolysis (complications)