Fulminant Wilson Disease in Children: Recovery After Plasma Exchange Without Transplantation.

Abstract

OBJECTIVES:

Since 2005, a New Wilson Index (NWI) ≥11 is used as a predictor of death without transplantation in fulminant Wilson disease (WD). Plasma exchange is advocated as a new treatment modality.

METHODS:

We present a patient with fulminant WD treated with plasma exchange. All published cases applying plasma exchange for fulminant WD were reviewed systematically.

RESULTS:

A 14-year-old girl presented with hemolysis and fulminant liver failure. She had no encephalopathy; NWI was 14. As a bridge to transplantation plasma exchange was started immediately. Complete remission was achieved with plasma exchange and later chelation therapy with D-penicillamine. She is now at 3-year transplant-free survival. Literature review identified 37 patients presenting with fulminant WD and NWI ≥11 who were treated with plasma exchange. Seventeen of these patients (ie, 46%) recovered without transplantation.

CONCLUSIONS:

Multiple case reports and case series demonstrate transplant free survival after plasma exchange and subsequent chelation therapy, despite a NWI ≥11. Plasma exchange affects the clinical course and is a therapeutic option in children and young adults presenting with fulminant WD.

• Authors: Renee Proost, David Cassiman, Elena Levtchenko, Eva Morava-Kozicz, Jef Neirynck, Peter Witters
• Journal: Journal of pediatric gastroenterology and nutrition (J Pediatr Gastroenterol Nutr) Vol. 71 Issue 6 Pg. 720-725 (12 2020) ISSN: 1536-4801 [Electronic] United States
• PMID: 32804913 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Penicillamine

Topics

• Adolescent
• Child
• Female
• Hepatolenticular Degeneration (therapy)
• Humans
• Liver Failure, Acute (therapy)
• Liver Transplantation
• Penicillamine
• Plasma Exchange
• Young Adult