κ I light chain AL amyloidosis presenting with rapidly progressive renal and hepatic failure with unusual renal amyloid distribution

Yuji Oe, Izaya Nakaya, Mayumi Yahata, Okinora Murata, Hiroshi Yaegashi, Tsutomu Sakuma, Hiroshi Sato, Juris J. Liepnieks, Merrill D. Benson, Jun Soma

研究成果: Article査読

2 被引用数 (Scopus)

抄録

A 65-year-old man suffering from generalized edema and jaundice was admitted to our hospital. Laboratory findings revealed marked renal dysfunction with heavy proteinuria as well as liver dysfunction with severe obstructive jaundice. On renal biopsy, the diagnosis of AL amyloidosis associated with κ I light chain was made. Interestingly, amyloid deposits were restricted to the glomeruli. Although hemodialysis was initiated, the patient died due to further deterioration of hepatic function. On autopsy, severe intrahepatic cholestasis was observed, and there was marked deposition of AL amyloid in the liver. Literature reviews showed that rapidly progressive renal failure is common in AL amyloidosis patients who presented with acute hepatic failure due to severe intrahepatic cholestasis. However, the detailed renal pathology in this condition has not been documented. The present case is very interesting because rapidly progressive renal and hepatic failure was simultaneously observed, and renal amyloid deposition was restricted to the glomeruli.

本文言語English
ページ(範囲)66-70
ページ数5
ジャーナルClinical nephrology
77
1
DOI
出版ステータスPublished - 2012 1 1

ASJC Scopus subject areas

  • 腎臓病学

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