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

被引:10
作者
Amano, Takahiro [1 ]
Matsubara, Tokuhiro [1 ]
Nishida, Tsutomu [1 ]
Shimakoshi, Hiromi [1 ]
Shimoda, Akiyoshi [1 ]
Sugimoto, Aya [1 ]
Takahashi, Kei [1 ]
Mukai, Kaori [1 ]
Yamamoto, Masashi [1 ]
Hayashi, Shiro [1 ]
Nakajima, Sachiko [1 ]
Fukui, Koji [1 ]
Inada, Masami [1 ]
机构
[1] Toyonaka City Hosp, Dept Gastroenterol & Hepatol, 4-14-1 Shibahara, Toyonaka, Osaka 5608565, Japan
关键词
Wilson's disease; Fulminant hepatitis; Late-onset; Liver cirrhosis; Copper; ACUTE LIVER-FAILURE; PRACTICE GUIDELINE;
D O I
10.3748/wjg.v24.i2.290
中图分类号
R57 [消化系及腹部疾病];
学科分类号
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 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.
引用
收藏
页码:290 / 296
页数:7
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