title: Center is an important indicator for choice of invasive therapy in polycystic liver disease.
abstract: Polycystic liver disease (PLD) is a rare genetic disorder with progressive cyst growth as the primary phenotype. Therapy consists of volume reduction through invasive surgical or radiological procedures. To understand the process of treatment decision, our aim was to identify factors that increased the likelihood of treatment. We performed a cross-sectional study using an international population of patients with PLD. We collected data on the following therapies: liver transplantation, resection, fenestration, and aspiration sclerotherapy. Data on the potential determinants, sex, center, autosomal dominant polycystic kidney disease (ADPKD), autosomal dominant polycystic liver disease (ADPLD), age at diagnosis, symptoms, and phenotype, were included. We corrected for follow-up time. We included 578 patients in our study, and 35% underwent invasive therapy. Multivariate regression analysis showed that number of symptoms and age at diagnosis of PLD increased the likelihood of treatment (respectively, RR: 1.4, P < 0.001 and RR = 1.4, P = 0.03). The choice for liver transplantation or aspiration sclerotherapy was center dependent (RR: 0.7, P < 0.001 and RR: 1.1, P = 0.03, respectively). The results of our international cross-sectional study suggest that a higher number of symptoms and every 10 years of PLD diagnosis increase the risk to undergo treatment by 40%. The choice to elect a particular modality is center dependent.
authors.affiliation: Department of Gastroenterology and Hepatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
authors.forename: Hedwig M A
authors.initials: HM
authors.lastname: D'Agnolo
authors.affiliation: Radboud Institute for Health Sciences, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
authors.forename: Wietske
authors.initials: W
authors.lastname: Kievit
authors.affiliation: Department of Gastroenterology and Hepatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
authors.forename: Kim N
authors.initials: KN
authors.lastname: van Munster
authors.affiliation: Department of Gastroenterology and Hepatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
authors.forename: Jouke J H
authors.initials: JJ
authors.lastname: van der Laan
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Leuven, Leuven, Belgium.
authors.forename: Frederik
authors.initials: F
authors.lastname: Nevens
authors.affiliation: Department of Gastroenterology and Hepatology, Radboud University Medical Center Nijmegen, Nijmegen, The Netherlands.
authors.forename: Joost P H
authors.initials: JP
authors.lastname: Drenth
mesh_terms: D000328:Adult; D000367:Age Factors; D003430:Cross-Sectional Studies; D003560:Cysts / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005799:Genes, Dominant; D006761:Hospitals / Q000145:classification*; D006801:Humans; D008107:Liver Diseases / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D010641:Phenotype; D016891:Polycystic Kidney, Autosomal Dominant / Q000601:surgery; D012042:Registries; D012189:Retrospective Studies; D015911:Sclerotherapy / Q000379:methods*; D016896:Treatment Outcome
keywords: autosomal dominant polycystic kidney disease; autosomal dominant polycystic liver disease; polycystic liver disease; surgery; treatment