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.
Original language | English (US) |
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Pages (from-to) | 720-725 |
Number of pages | 6 |
Journal | Journal of pediatric gastroenterology and nutrition |
Volume | 71 |
Issue number | 6 |
DOIs | |
State | Published - Dec 1 2020 |
Keywords
- Wilson disease
- acute liver failure
- children
- liver transplantation
- plasma exchange
ASJC Scopus subject areas
- Gastroenterology
- Pediatrics, Perinatology, and Child Health