論文

基本情報

氏名 加藤 正樹
氏名(カナ) カトウ マサキ
氏名(英語) KATO MASAKI
所属 中村学園大学 栄養科学部 栄養科学科
職名 教授

題名

A case of fatal intrahepatic cholestasis with primary AL amyloidosis: Is early diagnosis possible?

単著・共著の別

著者

Shinichiro Takao
Kosuke Tanaka
Masayuki Miyazaki
Masatake Tanaka
Tomoko Ohashi
Masaki Kato
Kazuhiro Kotoh
Shinichi Aishima
Ryoichi Takayanagi

担当区分

概要

Immunoglobulin light chain-associated (AL) amyloidosis is a multisystemic disorder characterized by extracellular deposition of immunoglobulin light chain produced by a proliferative plasma cell clone. Although the liver is the major organ involved in AL amyloidosis, hepatic involvement is often clinically asymptomatic and severe intrahepatic cholestasis as the primary manifestation of the disease is rare. A 60-year-old man with severe jaundice, massive ascites and highly elevated alkaline phosphatase was diagnosed with AL amyloidosis by a transjugular liver biopsy. He had undergone a yearly medical check that showed no abnormalities except for mild elevation of serum γ-glutamyltransferase at 1 year before admission. Owing to his poor condition and rapidly progressive liver and renal dysfunction, neither stem cell transplantation nor a combination of chemotherapeutic agents could be applied, and he died 1.5 months after admission. An autopsy revealed amyloid deposition in the systemic organs, and there was no evidence of multiple myeloma. Continuous elevation of γ-glutamyltransferase may be a useful marker for early diagnosis of fatal hepatic amyloidosis. © 2013 Springer Japan.

発表雑誌等の名称

Clinical Journal of Gastroenterology

出版者

6

5

開始ページ

386

終了ページ

389

発行又は発表の年月

2013/10/01

査読の有無

有り

招待の有無

無し

記述言語

英語

掲載種別

研究論文(学術雑誌)

国際・国内誌

国際共著

ISSN

1865-7257

eISSN

DOI

10.1007/s12328-013-0406-x

Cinii Articles ID

Cinii Books ID

Pubmed ID

PubMed Central 記事ID

形式

無償ダウンロード

JGlobalID

arXiv ID

ORCIDのPut Code

DBLP ID