title: Copper removal strategies for Wilson's disease crisis in the ICU.
abstract: Wilson's disease is a rare, inherited, autosomal recessive disorder of copper metabolism which leads to an accumulation of copper in body tissues. If a patient develops a Wilson's crisis, mortality can approach 100%. The treatment of such patients is mostly organ support but a possible treatment goal is to try to rapidly remove copper from their system. We performed a literature search on methods for de-coppering strategies for patients in intensive care with known Wilson's disease. We found 11 case reports where therapeutic plasma exchange was used and six case reports where various forms of albumin dialysis were used as techniques for rapidly reducing serum copper levels. To date, the case reports are encouraging that therapeutic plasma exchange and albumin dialysis can either delay or prevent the need for liver transplantation in patients with fulminant hepatic failure due to Wilson's disease. However, these case reports are mainly in the paediatric or young adult population, thus further studies in adults are warranted.
authors.affiliation: Department of Intensive Care Medicine, Burns Trauma and Critical Care Research Centre, Royal Brisbane and Women's Hospital, Herston, Queensland.
authors.forename: H V
authors.initials: HV
authors.lastname: Reynolds
authors.forename: C R
authors.initials: CR
authors.lastname: Talekar
authors.forename: J
authors.initials: J
authors.lastname: Bellapart
authors.forename: B A
authors.initials: BA
authors.lastname: Leggett
authors.forename: R J
authors.initials: RJ
authors.lastname: Boots
mesh_terms: D003300:Copper / Q000097:blood / Q000302:isolation & purification*; D006527:Hepatolenticular Degeneration / Q000628:therapy*; D006801:Humans; D007362:Intensive Care Units; D016031:Liver Transplantation; D010951:Plasma Exchange
chemical_list: D003300:Copper
keywords: Wilson’s disease; albumin dialysis; copper; haemolysis; molecular absorbent recirculating system; plasma exchange