Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder

Zellweger spectrum disorders (ZSDs) are known to present with variable hepatic manifestations ranging from benign hepatosplenomegaly and elevated liver enzymes to advanced liver cirrhosis with hepatocellular carcinoma. However, the progression of liver disease in ZSD patients over time is poorly cha...

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Detalles Bibliográficos
Autores principales: Hsu, Mark, Subhash, Amith
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2023
Materias:
Single Case
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9996246/
https://www.ncbi.nlm.nih.gov/pubmed/36910879
http://dx.doi.org/10.1159/000529353
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author Hsu, Mark
Subhash, Amith
author_facet Hsu, Mark
Subhash, Amith
author_sort Hsu, Mark
collection PubMed
description Zellweger spectrum disorders (ZSDs) are known to present with variable hepatic manifestations ranging from benign hepatosplenomegaly and elevated liver enzymes to advanced liver cirrhosis with hepatocellular carcinoma. However, the progression of liver disease in ZSD patients over time is poorly characterized due to scarcity of the disease. Herein, we report a case of newly diagnosed liver cirrhosis in a ZSD patient with rapid progression and fatal outcome to demonstrate key clinical learning points.
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spelling pubmed-99962462023-03-10 Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder Hsu, Mark Subhash, Amith Case Rep Gastroenterol Single Case Zellweger spectrum disorders (ZSDs) are known to present with variable hepatic manifestations ranging from benign hepatosplenomegaly and elevated liver enzymes to advanced liver cirrhosis with hepatocellular carcinoma. However, the progression of liver disease in ZSD patients over time is poorly characterized due to scarcity of the disease. Herein, we report a case of newly diagnosed liver cirrhosis in a ZSD patient with rapid progression and fatal outcome to demonstrate key clinical learning points. S. Karger AG 2023-03-08 /pmc/articles/PMC9996246/ /pubmed/36910879 http://dx.doi.org/10.1159/000529353 Text en © 2023 The Author(s). Published by S. Karger AG, Basel https://creativecommons.org/licenses/by-nc/4.0/This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Single Case
Hsu, Mark
Subhash, Amith
Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder
title Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder
title_full Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder
title_fullStr Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder
title_full_unstemmed Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder
title_short Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder
title_sort acute late-onset cirrhosis in zellweger spectrum disorder
topic Single Case
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9996246/
https://www.ncbi.nlm.nih.gov/pubmed/36910879
http://dx.doi.org/10.1159/000529353
work_keys_str_mv AT hsumark acutelateonsetcirrhosisinzellwegerspectrumdisorder
AT subhashamith acutelateonsetcirrhosisinzellwegerspectrumdisorder

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