3 years ago

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

Aya Sugimoto, Takahiro Amano, Koji Fukui, Hiromi Shimakoshi, Masami Inada, Masashi Yamamoto, Tsutomu Nishida, Kaori Mukai, Kei Takahashi, Shiro Hayashi, Tokuhiro Matsubara, Akiyoshi Shimoda, Sachiko Nakajima
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.

Publisher URL: http://doi.org/10.3748/wjg.v24.i2.290

DOI: 10.3748/wjg.v24.i2.290

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