title: Excellent survival after liver transplantation for isolated polycystic liver disease: an European Liver Transplant Registry study.
abstract: Patients with end-stage isolated polycystic liver disease (PCLD) suffer from incapacitating symptoms because of very large liver volumes. Liver transplantation (LT) is the only curative option. This study assesses the feasibility of LT in PCLD. We used the European Liver Transplant Registry (ELTR) database to extract demographics and outcomes of 58 PCLD patients. We used Kaplan-Meier survival analysis for survival rates. Severe abdominal pain (75%) was the most prominent symptom, while portal hypertension (35%) was the most common complication in PCLD. The explantation of the polycystic liver was extremely difficult in 38% of patients, because of presence of adhesions from prior therapy (17%). Karnofsky score following LT was 90%. The 1- and 5-year graft survival rate was 94.3% and 87.5%, while patient survival rate was 94.8% and 92.3%, respectively. Survival rates after LT for PCLD are good.
authors.affiliation: Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands.
authors.forename: Loes
authors.initials: L
authors.lastname: van Keimpema
authors.forename: Frederik
authors.initials: F
authors.lastname: Nevens
authors.forename: René
authors.initials: R
authors.lastname: Adam
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Porte
authors.forename: Panagiotis
authors.initials: P
authors.lastname: Fikatas
authors.forename: Thomas
authors.initials: T
authors.lastname: Becker
authors.forename: Preben
authors.initials: P
authors.lastname: Kirkegaard
authors.forename: Herold J
authors.initials: HJ
authors.lastname: Metselaar
authors.forename: Joost P H
authors.initials: JP
authors.lastname: Drenth
mesh_terms: D000328:Adult; D003560:Cysts / Q000175:diagnosis / Q000601:surgery; D005060:Europe / Q000453:epidemiology; D005260:Female; D006085:Graft Survival; D006801:Humans; D053208:Kaplan-Meier Estimate; D008107:Liver Diseases / Q000175:diagnosis / Q000601:surgery; D016031:Liver Transplantation / Q000401:mortality* / Q000502:physiology; D008297:Male; D008875:Middle Aged; D012042:Registries; D016896:Treatment Outcome