Clinically diagnosed late-onset fulminant Wilson's disease without cirrhosis: A case report

作     者：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 

作者机构：Department of Gastroenterology and Hepatology Toyonaka Municipal Hospital 

出 版 物：《World Journal of Gastroenterology》 (世界胃肠病学杂志（英文版）)

年 卷 期：2018年第24卷第2期

页      面：290-296页

核心收录：

学科分类：1002[医学-临床医学] 100204[医学-神经病学] 10[医学] 

主　　题：Wilson’s disease Fulminant hepatitis Lateonset Liver cirrhosis Copper 

摘      要：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 28 th 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.