title: Liver transplantation in Wilson's disease: Single center experience from Saudi Arabia.
abstract: OBJECTIVE
  To determine liver transplantation outcomes in Wilson's disease (WD) patients, focusing on neurological manifestations.
  
  
  METHODS
  This retrospective study assessed data from 16 WD patients (nine males, 56%) who had liver transplants between 1991 and 2007. Survival, graft function, and neurological complications were assessed during a follow-up period of up to 15 years. In addition, each patient's medical record was reviewed in detail to find the type of Wilson's disease (hepatic or hepatic plus neurological WD), indication for liver transplantation, use of chelating agents prior to transplantation, immediate and long term complications following transplantation, the donor details, and the pathology of explanted liver.
  
  
  RESULTS
  End-stage liver disease was the indication for transplantation in all 16 WD patients. Four patients displayed WD-related neurological symptoms in addition to liver disease. Living-related liver transplantation was done in three cases. One patient died on postoperative day 6 due to primary graft non-function. One-year post liver transplant survival was 94%. Neurological manifestations of all four patients disappeared during their follow-up. Four patients developed acute cellular rejection, but all responded to treatment. One patient developed chronic ductopenic rejection after 15 years post-transplantation and their graft failed; this patient is currently waiting for re-transplantation. Fourteen patients (88%) are still living. The long-term average survival is currently 10.5 years, with a current median survival of 8 years. Long-term graft survival is currently 81%.
  
  
  CONCLUSIONS
  Short- and long-term survival in WD patient liver transplantation was excellent, and neurological and psychological WD manifestations disappeared during long-term follow-up.
authors.affiliation: Musthafa Chalikandy Peedikayil, Hamad Ibrahim Al Ashgar, Abdullah Al Mousa, Khalid Al Kahtani, Department of Medicine, King Faisal Specialist, Hospital and Research Center, Pin 11211, Riyadh, Saudi Arabia.
authors.forename: Musthafa Chalikandy
authors.initials: MC
authors.lastname: Peedikayil
authors.forename: Hamad Ibrahim
authors.initials: HI
authors.lastname: Al Ashgar
authors.forename: Abdullah
authors.initials: A
authors.lastname: Al Mousa
authors.forename: Mohammed
authors.initials: M
authors.lastname: Al Sebayel
authors.forename: Khalid
authors.initials: K
authors.lastname: Al Kahtani
authors.forename: Faisal Aba
authors.initials: FA
authors.lastname: Alkhail
keywords: Liver transplantation; Neurological; Penicillamine; Psychiatric; Saudi Arabia; Transplantation; Wilson’s disease
references.citation: Hepatology. 2008 Jun;47(6):2089-111
references.pmid: 18506894
references.citation: Eur J Neurol. 2004 Feb;11(2):121-4
references.pmid: 14748773
references.citation: Liver Transpl. 2000 Sep;6(5):662-4
references.pmid: 11041671
references.citation: Lancet. 1971 Mar 13;1(7698):505-8
references.pmid: 4100432
references.citation: Hepatology. 1994 Mar;19(3):583-7
references.pmid: 8119682
references.citation: Saudi J Gastroenterol. 1996 Jan;2(1):50-2
references.pmid: 19864842
references.citation: Clin Transplant. 2008 Mar-Apr;22(2):216-21
references.pmid: 18339142
references.citation: Nat Genet. 1995 Feb;9(2):210-7
references.pmid: 7626145
references.citation: Trop Gastroenterol. 1993 Jul-Sep;14(3):91-8
references.pmid: 8109051
references.citation: J Clin Gastroenterol. 2006 Nov-Dec;40(10):936-41
references.pmid: 17063115
references.citation: Trop Geogr Med. 1988 Apr;40(2):131-8
references.pmid: 3407002
references.citation: J Hepatol. 2012 Mar;56(3):671-85
references.pmid: 22340672
references.citation: Pediatr Transplant. 2006 Aug;10(5):639-43
references.pmid: 16857005
references.citation: Gastroenterology. 1991 Mar;100(3):762-7
references.pmid: 1993498
references.citation: Liver Transpl Surg. 1999 Nov;5(6):467-74
references.pmid: 10545532
references.citation: Hepatology. 1984 Jan-Feb;4(1 Suppl):15S-17S
references.pmid: 6363255
references.citation: Transplant Proc. 2003 Jun;35(4):1445-6
references.pmid: 12826186