TY - JOUR
T1 - Retrospective analysis of long-term outcome 10 years after liver transplantation for Wilson disease
T2 - experience over three decades
AU - Ismail, Mohamed Saleh
AU - Hassan, Manal
AU - Martinez-Camacho, Alvaro
AU - May, Sarah B.
AU - Goss, John A.
AU - Kanwal, Fasiha
AU - Jalal, Prasun K.
N1 - Publisher Copyright:
© 2020 Steunstichting ESOT. Published by John Wiley & Sons Ltd
PY - 2020/8/1
Y1 - 2020/8/1
N2 - We evaluated long-term outcomes for patients with Wilson disease (WD) after liver transplantation (LT) and searched for risk factors for poor survival. Retrospective analysis of UNOS/OPTN data identified 156 pediatric and 515 adult cases of LT for WD between 1987 and 2016. Comparison cases were 10 442 pediatric and 104 874 adult non-WD transplant recipients. Survival was calculated using Kaplan–Meier analysis. Recipient, donor, and surgical variables were compared by Cox regression. Survival rates 3, 5, and 10 years after LT for adult WD patients (87.5%, 85.4%, and 80.5%, respectively) were significantly higher than those for non-WD patients (P < 0.001); survival rates for pediatric WD patients (90.5%, 89.7%, and 86.5%, respectively) did not differ significantly from non-WD patients. Graft survival in adult and pediatric patients followed similar trends. Regression analysis identified older age, female gender, and use of life support at the time of transplant as risk factors for decreased survival for adults with WD, and younger age, male gender, obesity, and high serum creatinine at the time of transplant as risk factors for poor survival in pediatric recipients with WD. Presentation with fulminant liver failure was not associated with survival in WD patients. No donor characteristic predicted poor survival. Long-term patient and graft survival after LT is excellent for both adult and pediatric WD patients.
AB - We evaluated long-term outcomes for patients with Wilson disease (WD) after liver transplantation (LT) and searched for risk factors for poor survival. Retrospective analysis of UNOS/OPTN data identified 156 pediatric and 515 adult cases of LT for WD between 1987 and 2016. Comparison cases were 10 442 pediatric and 104 874 adult non-WD transplant recipients. Survival was calculated using Kaplan–Meier analysis. Recipient, donor, and surgical variables were compared by Cox regression. Survival rates 3, 5, and 10 years after LT for adult WD patients (87.5%, 85.4%, and 80.5%, respectively) were significantly higher than those for non-WD patients (P < 0.001); survival rates for pediatric WD patients (90.5%, 89.7%, and 86.5%, respectively) did not differ significantly from non-WD patients. Graft survival in adult and pediatric patients followed similar trends. Regression analysis identified older age, female gender, and use of life support at the time of transplant as risk factors for decreased survival for adults with WD, and younger age, male gender, obesity, and high serum creatinine at the time of transplant as risk factors for poor survival in pediatric recipients with WD. Presentation with fulminant liver failure was not associated with survival in WD patients. No donor characteristic predicted poor survival. Long-term patient and graft survival after LT is excellent for both adult and pediatric WD patients.
KW - United Network for Organ Sharing
KW - Wilson disease
KW - graft survival
KW - liver transplantation
KW - patient survival
KW - standard transplant analysis and research
UR - http://www.scopus.com/inward/record.url?scp=85084436305&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85084436305&partnerID=8YFLogxK
U2 - 10.1111/tri.13622
DO - 10.1111/tri.13622
M3 - Article
C2 - 32314442
AN - SCOPUS:85084436305
SN - 0934-0874
VL - 33
SP - 925
EP - 935
JO - Transplant International
JF - Transplant International
IS - 8
ER -