title: Plasma exchange for hemolytic crisis and acute liver failure in Wilson disease.
abstract: Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8 d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.
authors.affiliation: Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India.
authors.forename: Nishant
authors.initials: N
authors.lastname: Verma
authors.forename: Gautham
authors.initials: G
authors.lastname: Pai
authors.forename: Pankaj
authors.initials: P
authors.lastname: Hari
authors.forename: Rakesh
authors.initials: R
authors.lastname: Lodha
mesh_terms: D000743:Anemia, Hemolytic / Q000209:etiology / Q000628:therapy*; D002675:Child, Preschool; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications*; D006801:Humans; D017114:Liver Failure, Acute / Q000209:etiology / Q000628:therapy*; D010951:Plasma Exchange