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Case Reports
. 2023 Jul 7;10(7):e01084.
doi: 10.14309/crj.0000000000001084. eCollection 2023 Jul.

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

Ethan Diamond  1 , Jacob Newman  1 , Reid Schalet  1 , Coen J Lap  1 , Ameer O Abutaleb  1   2
Affiliations
Case Reports

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

Ethan Diamond et al. ACG Case Rep J. .

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.

Keywords: Wilson disease; acute-on-chronic liver failure; copper.

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