Successful treatment of acute liver failure due to Wilson’s disease: Serendipity or fortuity?

doi:10.4254/wjh.v16.i8.1111

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Aug 27, 2024 (publication date) through Mar 2, 2026

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World Journal of Hepatology

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1948-5182

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Retrospective Study

World J Hepatol. Aug 27, 2024; 16(8): 1111-1119
Published online Aug 27, 2024. doi: 10.4254/wjh.v16.i8.1111

Successful treatment of acute liver failure due to Wilson’s disease: Serendipity or fortuity?

Valeria Delle Cave, Fabiola Di Dato, Pier Luigi Calvo, Maria Immacolata Spagnuolo, Raffaele Iorio

Valeria Delle Cave, Fabiola Di Dato, Maria Immacolata Spagnuolo, Raffaele Iorio, Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples 80131, Italy

Pier Luigi Calvo, Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, Turin 10126, Italy

Co-first authors: Valeria Delle Cave and Fabiola Di Dato.

Author contributions: Iorio R designed the research study; Iorio R, Delle Cave V, and Di Dato F performed the research and wrote the manuscript; Iorio R, Delle Cave V, Di Dato F, Spagnuolo MI, and Calvo PL reviewed and edited the manuscript for important intellectual content. All authors have read and approved the final manuscript. Delle Cave V and Di Dato F contributed equally to this work as co-first authors.

Institutional review board statement: Consent of the bioethics committee was waived because of the retrospective nature of the study.

Informed consent statement: Patients were not required to give informed consent to the study because the analysis used anonymous clinical data.

Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.

Data sharing statement: All relevant data are within the paper.

Corresponding author: Raffaele Iorio, MD, Associate Professor, Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Via Pansini 5, Naples 80131, Italy. riorio@unina.it

Received: April 27, 2024
Revised: May 28, 2024
Accepted: June 18, 2024
Published online: August 27, 2024
Processing time: 116 Days and 7.3 Hours

Abstract

BACKGROUND

Acute liver failure (ALF) may be the first and most dramatic presentation of Wilson’s disease (WD). ALF due to WD (WD-ALF) is difficult to distinguish from other causes of liver disease and is a clear indication for liver transplantation. There is no firm recommendation on specific and supportive medical treatment for this condition.

AIM

To critically evaluate the diagnostic and therapeutic management of WD-ALF patients in order to improve their survival with native liver.

METHODS

A retrospective analysis of patients with WD-ALF was conducted in two pediatric liver units from 2018 to 2023.

RESULTS

During the study period, 16 children (9 males) received a diagnosis of WD and 2 of them presented with ALF. The first was successfully treated with an unconventional combination of low doses of D-penicillamine and zinc plus steroids, and survived without liver transplant. The second, exclusively treated with supportive therapy, needed a hepatotransplant to overcome ALF.

CONCLUSION

Successful treatment of 1 WD-ALF patient with low-dose D-penicillamine and zinc plus steroids may provide new perspectives for management of this condition, which is currently only treated with liver transplantation.

Keywords: Fulminant liver failure; Copper; Chelator; Zinc; Steroids; Liver transplantation; Autoimmune hepatitis; Prognostic score; 24 hour-urinary copper excretion; Native liver

Core Tip: There is no firm recommendation on the management of acute liver failure due to Wilson's disease, for which liver transplantation is the only reliable treatment option. The retrospective study highlights the successful treatment of a patient with an unconventional low-dose therapy of D-penicillamine and zinc plus steroids with the aim of opening new perspectives in the management of the condition, stimulating a critical evaluation on the topic and thus promoting the use of all possible therapeutic opportunities to increase survival with the native liver.