title: Alpha-1-antitrypsin deficiency: outcomes after liver transplantation.
abstract: Alpha-1-antitrypsin deficiency (AAT) is the most common inherited metabolic disease leading to liver transplantation (LT) in children and adults. The aim of the study was to determine transplantation trends and survival of LT recipients with AAT. Using the UNOS (United Network for Organ Sharing) database, we identified 567 patients who underwent LT and 3 who received lung and LT from 1995 to 2004. AAT accounted for 1.06% of all adult LTs and 3.51% for pediatric LT. The 1-, 3-, and 5-year patient survival was 89%, 85%, and 83%, respectively, for adults versus 92%, 90%, and 90% for pediatric patients (P = .04), and graft survival was 83%, 79%, and 77% for adults versus 84%, 81%, and 78% for pediatric patients (P = .51). By regression analysis, age was the only predictor for patient survival (P = .04). In conclusion, adult and pediatric LT recipients with AAT are predominantly of Caucasian ethnicity and have an excellent post-LT survival.
authors.affiliation: Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio 45267, USA. nyingi.kemmer@uc.edu
authors.forename: N
authors.initials: N
authors.lastname: Kemmer
authors.forename: T
authors.initials: T
authors.lastname: Kaiser
authors.forename: V
authors.initials: V
authors.lastname: Zacharias
authors.forename: G W
authors.initials: GW
authors.lastname: Neff
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D002648:Child; D002675:Child, Preschool; D016208:Databases, Factual; D005260:Female; D006085:Graft Survival / Q000502:physiology*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000401:mortality / Q000502:physiology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D019896:alpha 1-Antitrypsin Deficiency / Q000601:surgery*