Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jan 14, 2018; 24(2): 290-296
Published online Jan 14, 2018. doi: 10.3748/wjg.v24.i2.290
Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report
Takahiro Amano, Tokuhiro Matsubara, Tsutomu Nishida, Hiromi Shimakoshi, Akiyoshi Shimoda, Aya Sugimoto, Kei Takahashi, Kaori Mukai, Masashi Yamamoto, Shiro Hayashi, Sachiko Nakajima, Koji Fukui, Masami Inada
Takahiro Amano, Tokuhiro Matsubara, Tsutomu Nishida, Hiromi Shimakoshi, Akiyoshi Shimoda, Aya Sugimoto, Kei Takahashi, Kaori Mukai, Masashi Yamamoto, Shiro Hayashi, Sachiko Nakajima, Koji Fukui, Masami Inada, Department of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, Toyonaka, Osaka 560-8565, Japan
Author contributions: Matsubara T, Amano T and Nishida T conceived and designed the study, acquired and analyzed the data, drafted the article; Shimakoshi H, Shimoda A, Sugimoto A and Takahashi K interpreted the data; All authors gave final approval of the manuscript.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Tokuhiro Matsubara, MD, PhD, Department of Gastroenterology and Hepatology, Toyonaka Municipal Hospital, 4-14-1 Shibahara, Toyonaka, Osaka 560-8565, Japan. tmatsubara@chp.toyonaka.osaka.jp
Telephone: +81-6-68430101 Fax: +81-6-68583531
Received: October 16, 2017
Peer-review started: October 16, 2017
First decision: November 8, 2017
Revised: November 23, 2017
Accepted: November 28, 2017
Article in press: November 28, 2017
Published online: January 14, 2018
Abstract

A 64-year-old woman was referred to our hospital with jaundice of the bulbar conjunctiva and general fatigue. After admission, she developed hepatic encephalopathy and was diagnosed with fulminant hepatitis based on the American Association for the Study of Liver Disease (AASLD) position paper. Afterwards, additional laboratory findings revealed that serum ceruloplasmin levels were reduced, urinary copper levels were greatly elevated and Wilson’s disease (WD)-specific routine tests were positive, but the Kayser-Fleischer ring was not clear. Based on the AASLD practice guidelines for the diagnosis and treatment of WD, the patient was ultimately diagnosed with fulminant WD. Then, administration of penicillamine and zinc acetate was initiated; however, the patient unfortunately died from acute pneumonia on the 28th day of hospitalization. At autopsy, the liver did not show a bridging pattern of fibrosis suggestive of chronic liver injury. Here, we present the case of a patient with clinically diagnosed late-onset fulminant WD without cirrhosis, who had positive disease-specific routine tests.

Keywords: Wilson’s disease, Fulminant hepatitis, Late-onset, Liver cirrhosis, Copper

Core tip: A 64-year-old woman was referred to our hospital with hepatopathy. After admission, she developed hepatic encephalopathy. Laboratory findings revealed that serum ceruloplasmin levels were reduced, serum and urinary copper levels were greatly elevated and Wilson’s disease (WD)-specific routine tests were positive. She was diagnosed with fulminant WD based on the American Association for the Study of Liver Disease practice guidelines. At autopsy, the liver did not show a bridging pattern of fibrosis suggestive of chronic liver injury. Here, we present the first case of a patient with clinically diagnosed late-onset fulminant WD without cirrhosis.