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...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
Karger Publishers
2023-03-01
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| Series: | Case Reports in Gastroenterology |
| Subjects: | |
| Online Access: | https://www.karger.com/Article/FullText/529353 |
| _version_ | 1827971983428550656 |
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| author | Mark Hsu Amith Subhash |
| author_facet | Mark Hsu Amith Subhash |
| author_sort | Mark Hsu |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-04-09T19:15:59Z |
| format | Article |
| id | doaj.art-b003202650324d7db3f15f518f1b8e9a |
| institution | Directory Open Access Journal |
| issn | 1662-0631 |
| language | English |
| last_indexed | 2024-04-09T19:15:59Z |
| publishDate | 2023-03-01 |
| publisher | Karger Publishers |
| record_format | Article |
| series | Case Reports in Gastroenterology |
| spelling | doaj.art-b003202650324d7db3f15f518f1b8e9a2023-04-06T05:42:14ZengKarger PublishersCase Reports in Gastroenterology1662-06312023-03-0117116817110.1159/000529353529353Acute Late-Onset Cirrhosis in Zellweger Spectrum DisorderMark Hsu0Amith Subhash1Department of Internal Medicine, Kirk Kerkorian School of Medicine at UNLV, Las Vegas, NV, USADepartment of Gastroenterology, Kirk Kerkorian School of Medicine at UNLV, Las Vegas, NV, USAZellweger 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.https://www.karger.com/Article/FullText/529353liver cirrhosiszellweger spectrum disorderinfantile refsum diseaseperoxisomal biogenesis disordersliver disease |
| spellingShingle | Mark Hsu Amith Subhash Acute Late-Onset Cirrhosis in Zellweger Spectrum Disorder Case Reports in Gastroenterology liver cirrhosis zellweger spectrum disorder infantile refsum disease peroxisomal biogenesis disorders liver disease |
| 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 | liver cirrhosis zellweger spectrum disorder infantile refsum disease peroxisomal biogenesis disorders liver disease |
| url | https://www.karger.com/Article/FullText/529353 |
| work_keys_str_mv | AT markhsu acutelateonsetcirrhosisinzellwegerspectrumdisorder AT amithsubhash acutelateonsetcirrhosisinzellwegerspectrumdisorder |