{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,14]],"date-time":"2026-04-14T00:36:17Z","timestamp":1776126977115,"version":"3.50.1"},"reference-count":27,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2018,10,8]],"date-time":"2018-10-08T00:00:00Z","timestamp":1538956800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001254","name":"Ipsen Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001254","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2019,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background &amp; Aims<\/jats:title><jats:p>Polycystic liver disease (PLD) occurs in two genetic disorders, autosomal\u2010dominant polycystic kidney disease (ADPKD) and autosomal\u2010dominant polycystic liver disease (ADPLD). The aim of this study is to compare disease severity between ADPKD and ADPLD by determining the association between diagnosis and height\u2010adjusted total liver volume (hTLV).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We performed a cross\u2010sectional analysis with hTLV as endpoint. Patients were identified from the International PLD Registry (&gt;10 liver cysts) and included in our analysis when PLD diagnosis was made prior to September 2017, hTLV was available before volume\u2010reducing therapy (measured on computed tomography or magnetic resonance imaging) and when patients were tertiary referred. Data from the registry were retrieved for age, diagnosis (ADPKD or ADPLD), gender, height and hTLV.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 360 patients (ADPKD n\u00a0=\u00a0241; ADPLD n\u00a0=\u00a0119) met our inclusion criteria. Female ADPKD patients had larger hTLV compared with ADPLD (<jats:italic>P<\/jats:italic>\u00a0=\u00a00.008). In a multivariate regression analysis, ADPKD and lower age at index CT were independently associated with larger hTLV in females, whereas in males a higher age was associated with larger hTLV. Young females (\u226451\u00a0years) had larger liver volumes compared with older females (&gt;51\u00a0years) in ADPKD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Aetiology is presented as a new risk factor associated with PLD severity. Young females with ADPKD represent a subgroup of PLD patients with the most severe phenotype expressed in hTLV.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.13965","type":"journal-article","created":{"date-parts":[[2018,9,18]],"date-time":"2018-09-18T03:25:00Z","timestamp":1537241100000},"page":"575-582","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Severity in polycystic liver disease is associated with aetiology and female gender: Results of the International PLD Registry"],"prefix":"10.1111","volume":"39","author":[{"given":"Ren\u00e9 M. M.","family":"van Aerts","sequence":"first","affiliation":[{"name":"Department of Gastroenterology and Hepatology Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wietske","family":"Kievit","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michiel E.","family":"de Jong","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Curie","family":"Ahn","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Seoul National University Hospital Seoul South Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jes\u00fas M.","family":"Ba\u00f1ales","sequence":"additional","affiliation":[{"name":"Department of Liver and Gastrointestinal Diseases, Biodonostia Research Institute\u2014Donostia University Hospital, IKERBASQUE, CIBERehd University of the Basque Country (UPV\/EHU) San Sebasti\u00e1n Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jana","family":"Reiterov\u00e1","sequence":"additional","affiliation":[{"name":"Department of Nephrology, 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frederik","family":"Nevens","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology University Hospital KU Leuven Leuven Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8027-3073","authenticated-orcid":false,"given":"Joost P. 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