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

Journal Contribution - Journal Article

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.
Journal: Journal of Pediatric Gastroenterology and Nutrition
ISSN: 0277-2116
Issue: 6
Volume: 71
Pages: 720 - 725
Publication year:2020
BOF-publication weight:1
CSS-citation score:1
Authors from:Higher Education