TY - JOUR
T1 - Outcomes of Liver Transplant for Adults With Wilson’s Disease
AU - Ferrarese, Alberto
AU - Morelli, Maria Cristina
AU - Carrai, Paola
AU - Milana, Martina
AU - Angelico, Mario
AU - Perricone, Giovanni
AU - Belli, Luca Saverio
AU - Marrone, Giuseppe
AU - Grieco, Antonio
AU - Martini, Silvia
AU - Manini, Matteo Angelo
AU - Fagiuoli, Stefano
AU - Toniutto, Pierluigi
AU - Galeota Lanza, Alfonso
AU - Bhoori, Sherrie
AU - Petta, Salvatore
AU - Giannini, Edoardo G.
AU - Burra, Patrizia
PY - 2020/4/1
Y1 - 2020/4/1
N2 - Wilson’s disease (WD) is a rare genetic disorder with protean manifestations. Even if liver transplantation (LT) could represent an effective therapeutic option for patients with end-stage liver disease, it has remained controversial in the presence of neuropsychiatric involvement. This study aimed to examine the frequency of adult LT for WD in Italy, focusing on the disease phenotype at the time of LT. A retrospective, observational, multicenter study was conducted across Italy exploring the frequency and characteristics of adults transplanted for WD between 2006 and 2016. A total of 29 adult WD patients underwent LT during the study period at 11 Italian LT centers (accounting for 0.4% of all LTs performed), and 27 of them were considered in this analysis (male/female, n = 9/18; age at LT, 29 years [19-60 years]; median Model for End-Stage Liver Disease score at LT, 27 [6-49]). Isolated hepatic phenotype was the indication for LT in 17 (63%) patients, whereas 2 (7%) patients underwent LT for neurological impairment on compensated liver disease. Overall 1- and 5-year patient survival was excellent (88% and 83%, respectively). Neuropsychiatric symptoms early after LT completely recovered in only a few patients. In conclusion, WD remains an uncommon, unusual indication for LT in Italy, displaying good post-LT graft and patient survival. Because isolated neuropsychiatric involvement represents a rare indication to LT, more data are needed to properly assess the value of LT for WD in this subset of patients.
AB - Wilson’s disease (WD) is a rare genetic disorder with protean manifestations. Even if liver transplantation (LT) could represent an effective therapeutic option for patients with end-stage liver disease, it has remained controversial in the presence of neuropsychiatric involvement. This study aimed to examine the frequency of adult LT for WD in Italy, focusing on the disease phenotype at the time of LT. A retrospective, observational, multicenter study was conducted across Italy exploring the frequency and characteristics of adults transplanted for WD between 2006 and 2016. A total of 29 adult WD patients underwent LT during the study period at 11 Italian LT centers (accounting for 0.4% of all LTs performed), and 27 of them were considered in this analysis (male/female, n = 9/18; age at LT, 29 years [19-60 years]; median Model for End-Stage Liver Disease score at LT, 27 [6-49]). Isolated hepatic phenotype was the indication for LT in 17 (63%) patients, whereas 2 (7%) patients underwent LT for neurological impairment on compensated liver disease. Overall 1- and 5-year patient survival was excellent (88% and 83%, respectively). Neuropsychiatric symptoms early after LT completely recovered in only a few patients. In conclusion, WD remains an uncommon, unusual indication for LT in Italy, displaying good post-LT graft and patient survival. Because isolated neuropsychiatric involvement represents a rare indication to LT, more data are needed to properly assess the value of LT for WD in this subset of patients.
UR - http://www.scopus.com/inward/record.url?scp=85082094002&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85082094002&partnerID=8YFLogxK
U2 - 10.1002/lt.25714
DO - 10.1002/lt.25714
M3 - Article
C2 - 31901209
AN - SCOPUS:85082094002
VL - 26
SP - 507
EP - 516
JO - Liver Transplantation
JF - Liver Transplantation
SN - 1527-6465
IS - 4
ER -