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Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease
Wilson disease (WD) is estimated present in 6%–12% of patients younger than 40 years hospitalized with acute liver failure (ALF). Fulminant WD carries a poor prognosis without treatment. A 36-year-old man with HIV, chronic hepatitis B virus, and alcohol use had ceruloplasmin 6.4 mg/dL and 24-hour ur...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Wolters Kluwer
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10328575/ https://www.ncbi.nlm.nih.gov/pubmed/37426568 http://dx.doi.org/10.14309/crj.0000000000001084 |
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author | Diamond, Ethan Newman, Jacob Schalet, Reid Lap, Coen J. Abutaleb, Ameer O. |
author_facet | Diamond, Ethan Newman, Jacob Schalet, Reid Lap, Coen J. Abutaleb, Ameer O. |
author_sort | Diamond, Ethan |
collection | PubMed |
description | Wilson disease (WD) is estimated present in 6%–12% of patients younger than 40 years hospitalized with acute liver failure (ALF). Fulminant WD carries a poor prognosis without treatment. A 36-year-old man with HIV, chronic hepatitis B virus, and alcohol use had ceruloplasmin 6.4 mg/dL and 24-hour urine copper 180 μg/L. WD workup was otherwise negative, including ophthalmic examination, hepatic copper quantification, ATP7B sequencing, and brain MRI. ALF commonly features copper dysregulation. Few studies on WD biomarkers have included fulminant WD. Our patient with WD biomarkers and other causes of liver failure highlights the need to study copper dysregulation in ALF. |
format | Online Article Text |
id | pubmed-10328575 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Wolters Kluwer |
record_format | MEDLINE/PubMed |
spelling | pubmed-103285752023-07-08 Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease Diamond, Ethan Newman, Jacob Schalet, Reid Lap, Coen J. Abutaleb, Ameer O. ACG Case Rep J Case Report Wilson disease (WD) is estimated present in 6%–12% of patients younger than 40 years hospitalized with acute liver failure (ALF). Fulminant WD carries a poor prognosis without treatment. A 36-year-old man with HIV, chronic hepatitis B virus, and alcohol use had ceruloplasmin 6.4 mg/dL and 24-hour urine copper 180 μg/L. WD workup was otherwise negative, including ophthalmic examination, hepatic copper quantification, ATP7B sequencing, and brain MRI. ALF commonly features copper dysregulation. Few studies on WD biomarkers have included fulminant WD. Our patient with WD biomarkers and other causes of liver failure highlights the need to study copper dysregulation in ALF. Wolters Kluwer 2023-07-07 /pmc/articles/PMC10328575/ /pubmed/37426568 http://dx.doi.org/10.14309/crj.0000000000001084 Text en © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. |
spellingShingle | Case Report Diamond, Ethan Newman, Jacob Schalet, Reid Lap, Coen J. Abutaleb, Ameer O. Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease |
title | Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease |
title_full | Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease |
title_fullStr | Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease |
title_full_unstemmed | Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease |
title_short | Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease |
title_sort | dysregulation of copper metabolism in a patient with acute-on-chronic liver failure worked up for fulminant wilson disease |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10328575/ https://www.ncbi.nlm.nih.gov/pubmed/37426568 http://dx.doi.org/10.14309/crj.0000000000001084 |
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