{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T21:53:07Z","timestamp":1791237187481,"version":"4.1.0"},"reference-count":35,"publisher":"Springer Science and Business Media LLC","license":[{"start":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T00:00:00Z","timestamp":1791158400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T00:00:00Z","timestamp":1791158400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Ministry of Health and Welfare, Japan","award":["JPMH20FC1023"],"award-info":[{"award-number":["JPMH20FC1023"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Gastroenterol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Polycystic liver disease (PLD) is often asymptomatic, but some patients develop symptoms or complications requiring care or intervention. The nationwide burden of symptomatic PLD remains poorly defined. We conducted a nationwide survey to estimate the number and prevalence of patients with symptomatic PLD in Japan and characterize clinical features, treatment patterns, healthcare utilization, and outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A nationwide two-stage questionnaire survey was conducted. Symptomatic PLD was defined as 10 or more hepatic cysts with cyst-related symptoms or complications. In the first-stage survey, 3127 departments were selected by stratified random sampling to estimate the number of patients treated or followed up during 2021\u20132023. In the second-stage survey, clinical data were collected for patients treated or followed up in 2023.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The estimated number of patients was 6500 during 2021\u20132023 and 3570 in 2023, corresponding to an annual prevalence of 28.6 per million. Among 557 patients with clinical data, 62% were female and 67.2% met the diagnostic criteria for polycystic kidney disease. Abdominal distension, abdominal pain, and cyst infection were the most frequent manifestations. More than half underwent hepatic cyst treatment, and 60.4% required consultation with other departments or institutions. Cyst infection and liver failure accounted for a subset of deaths. Mortality differed significantly among Gigot classification groups and was highest in type III.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This nationwide survey provides the first comprehensive epidemiological and clinical characterization of symptomatic PLD in Japan and establishes a benchmark for future disease severity classification, clinical management, and prospective studies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00535-026-02522-1","type":"journal-article","created":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T04:34:54Z","timestamp":1791174894000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Healthcare burden and clinical spectrum of symptomatic polycystic liver disease in Japan: a nationwide epidemiological survey"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9884-8039","authenticated-orcid":false,"given":"Kota","family":"Tsuruya","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoko","family":"Ohfuji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatsuya","family":"Suwabe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusuke","family":"Mishima","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshitaka","family":"Arase","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunji","family":"Hirose","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satsuki","family":"Ieda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Junichi","family":"Hoshino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shintaro","family":"Yagi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiyoshi","family":"Hasegawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatehiro","family":"Kagawa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,10,5]]},"reference":[{"key":"2522_CR1","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1038\/nrgastro.2012.254","volume":"10","author":"TJ Gevers","year":"2013","unstructured":"Gevers TJ, Drenth JP. 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