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

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. Afte...

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Autores principales: Amano, Takahiro, Matsubara, Tokuhiro, Nishida, Tsutomu, Shimakoshi, Hiromi, Shimoda, Akiyoshi, Sugimoto, Aya, Takahashi, Kei, Mukai, Kaori, Yamamoto, Masashi, Hayashi, Shiro, Nakajima, Sachiko, Fukui, Koji, Inada, Masami
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5768947/
https://www.ncbi.nlm.nih.gov/pubmed/29375214
http://dx.doi.org/10.3748/wjg.v24.i2.290
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author Amano, Takahiro
Matsubara, Tokuhiro
Nishida, Tsutomu
Shimakoshi, Hiromi
Shimoda, Akiyoshi
Sugimoto, Aya
Takahashi, Kei
Mukai, Kaori
Yamamoto, Masashi
Hayashi, Shiro
Nakajima, Sachiko
Fukui, Koji
Inada, Masami
author_facet Amano, Takahiro
Matsubara, Tokuhiro
Nishida, Tsutomu
Shimakoshi, Hiromi
Shimoda, Akiyoshi
Sugimoto, Aya
Takahashi, Kei
Mukai, Kaori
Yamamoto, Masashi
Hayashi, Shiro
Nakajima, Sachiko
Fukui, Koji
Inada, Masami
author_sort Amano, Takahiro
collection PubMed
description 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.
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spelling pubmed-57689472018-01-27 Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report Amano, Takahiro Matsubara, Tokuhiro Nishida, Tsutomu Shimakoshi, Hiromi Shimoda, Akiyoshi Sugimoto, Aya Takahashi, Kei Mukai, Kaori Yamamoto, Masashi Hayashi, Shiro Nakajima, Sachiko Fukui, Koji Inada, Masami World J Gastroenterol Case Report 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. Baishideng Publishing Group Inc 2018-01-14 2018-01-14 /pmc/articles/PMC5768947/ /pubmed/29375214 http://dx.doi.org/10.3748/wjg.v24.i2.290 Text en ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ 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.
spellingShingle Case Report
Amano, Takahiro
Matsubara, Tokuhiro
Nishida, Tsutomu
Shimakoshi, Hiromi
Shimoda, Akiyoshi
Sugimoto, Aya
Takahashi, Kei
Mukai, Kaori
Yamamoto, Masashi
Hayashi, Shiro
Nakajima, Sachiko
Fukui, Koji
Inada, Masami
Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report
title Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report
title_full Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report
title_fullStr Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report
title_full_unstemmed Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report
title_short Clinically diagnosed late-onset fulminant Wilson’s disease without cirrhosis: A case report
title_sort clinically diagnosed late-onset fulminant wilson’s disease without cirrhosis: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5768947/
https://www.ncbi.nlm.nih.gov/pubmed/29375214
http://dx.doi.org/10.3748/wjg.v24.i2.290
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