Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease

Document Type

Journal Article

Publication Date

7-1-2023

Journal

ACG case reports journal

Volume

10

Issue

7

DOI

10.14309/crj.0000000000001084

Keywords

Wilson disease; acute-on-chronic liver failure; copper

Abstract

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.

Department

Surgery

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