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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...

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Autores principales: Diamond, Ethan, Newman, Jacob, Schalet, Reid, Lap, Coen J., Abutaleb, Ameer O.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer 2023
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.
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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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