A Case of Primary Renal Amyloidosis with Fulminant Hepatic Failure

Korean Journal of Nephrology 2009;28(5):490-495.

Geon Tae Park, M.D., Seong Cho, M.D., Sung Rok Kim, M.D., Oh Wen Kwon, M.D., Jae Gon Woo, M.D., Ji Eun Yi, M.D. and Gyung Won Jung, M.D.

Departments of Internal Medicine, Sungkyunkwan University Collage, of Medicine, Samsung Hospital, Masan, Korea

증례 : 전격성 간부전의 경과를 보인 원발성 신 아밀로이드 증 1예

박건태, 조 성, 김성록, 권오언, 우재곤, 이지은, 정경원

성균관대학교 의과대학 마산삼성병원 내과

Abstract

We report the case of a patient with primary renal amyloidosis, who was progressed to fulminant hepatic failure. A 68-year-old man visited our hospital for evaluation of anorexia, weight loss and hepatomegaly. Laboratory examination revealed proteinuria and azotemia. He was confirmed to have primary amyloidosis by renal biopsy. His renal function progressed to deteriorate rapidly with marked deterioration of liver function with cholestasis. Peritoneal dialysis and steroid therapy were started for reduction of urine output and rapid progression of azotemia, and aggravation of liver function. Azotemia was improved after peritoneal dialysis but the liver function worsened leading to hepatic failure, causing death. If jaundice has progressed in any patients with primary amyloidosis, liver transplantation and stem cell transplantation should be considered for preparation of hepatic failure.

Key Words: Amyloidosis, Liver failure