{"@context":{"@vocab":"https://cir.nii.ac.jp/schema/1.0/","rdfs":"http://www.w3.org/2000/01/rdf-schema#","dc":"http://purl.org/dc/elements/1.1/","dcterms":"http://purl.org/dc/terms/","foaf":"http://xmlns.com/foaf/0.1/","prism":"http://prismstandard.org/namespaces/basic/2.0/","cinii":"http://ci.nii.ac.jp/ns/1.0/","datacite":"https://schema.datacite.org/meta/kernel-4/","ndl":"http://ndl.go.jp/dcndl/terms/","jpcoar":"https://github.com/JPCOAR/schema/blob/master/2.0/"},"@id":"https://cir.nii.ac.jp/crid/1360302865543092736.json","@type":"Article","productIdentifier":[{"identifier":{"@type":"DOI","@value":"10.1007/s10157-023-02372-8"}},{"identifier":{"@type":"URI","@value":"https://link.springer.com/content/pdf/10.1007/s10157-023-02372-8.pdf"}},{"identifier":{"@type":"URI","@value":"https://link.springer.com/article/10.1007/s10157-023-02372-8/fulltext.html"}},{"identifier":{"@type":"PMID","@value":"37924433"}}],"resourceType":"学術雑誌論文(journal article)","dc:title":[{"@value":"Public support for patients with intractable diseases in Japan: impact on clinical indicators from nationwide registries in patients with autosomal dominant polycystic kidney disease"}],"description":[{"notation":[{"@value":"Clinical practice guidelines recommend antihypertensive and tolvaptan therapies for patients with autosomal dominant polycystic kidney disease (ADPKD) in Japan. However, tolvaptan therapy may pose an economic burden. The Japanese Ministry of Health, Labour and Welfare supports patients with intractable diseases. This study aimed to confirm the impact of the intractable disease system in Japan on the clinical treatment of ADPKD.We analyzed the data of 3768 patients with ADPKD having a medical subsidy certificate from the Japanese Ministry of Health, Labour and Welfare in 2015-2016. The following quality indicators were use: the adherence rate to the 2014 clinical practice guideline for polycystic kidney disease (prescription rates of antihypertensive agents and tolvaptan in this cohort) and the number of Japanese patients with ADPKD nationwide started on renal replacement therapy in 2014 and 2020.Compared with new applications from 2015 to 2016, the prescription rates of antihypertensives and tolvaptan for the indicated patients at the 2017 renewal application increased by 2.0% (odds ratio = 1.41, p = 0.008) and 47.4% (odds ratio = 10.1, p  0.001), respectively. These quality indicators improved with antihypertensive treatment, especially in patients with chronic kidney disease stages 1-2 (odds ratio = 1.79, p = 0.013) and in those aged  50 years (odds ratio = 1.70, p = 0.003). The number of patients with ADPKD who were started on renal replacement therapy in Japan decreased from 999 in 2014 to 884 in 2020 in the nationwide database (odds ratio = 0.83, p  0.001).The Japanese public intractable disease support system contributes to improvement of ADPKD treatment."}]}],"creator":[{"@id":"https://cir.nii.ac.jp/crid/1380302865543092748","@type":"Researcher","foaf:name":[{"@value":"Hiroshi Kataoka"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092619","@type":"Researcher","foaf:name":[{"@value":"Yosuke Shimada"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092864","@type":"Researcher","foaf:name":[{"@value":"Tomonori Kimura"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092782","@type":"Researcher","foaf:name":[{"@value":"Saori Nishio"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092758","@type":"Researcher","foaf:name":[{"@value":"Shinya Nakatani"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092781","@type":"Researcher","foaf:name":[{"@value":"Toshio Mochizuki"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092773","@type":"Researcher","foaf:name":[{"@value":"Ken Tsuchiya"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092750","@type":"Researcher","foaf:name":[{"@value":"Junichi Hoshino"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092618","@type":"Researcher","foaf:name":[{"@value":"Fumihiko Hattanda"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092742","@type":"Researcher","foaf:name":[{"@value":"Haruna Kawano"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092777","@type":"Researcher","foaf:name":[{"@value":"Kazushige Hanaoka"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092617","@type":"Researcher","foaf:name":[{"@value":"Sumi Hidaka"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092775","@type":"Researcher","foaf:name":[{"@value":"Daisuke Ichikawa"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092612","@type":"Researcher","foaf:name":[{"@value":"Eiji Ishikawa"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092792","@type":"Researcher","foaf:name":[{"@value":"Kiyotaka Uchiyama"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092762","@type":"Researcher","foaf:name":[{"@value":"Hiroki Hayashi"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092754","@type":"Researcher","foaf:name":[{"@value":"Shiho Makabe"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092749","@type":"Researcher","foaf:name":[{"@value":"Shun Manabe"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092763","@type":"Researcher","foaf:name":[{"@value":"Michihiro Mitobe"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092787","@type":"Researcher","foaf:name":[{"@value":"Akinari Sekine"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092609","@type":"Researcher","foaf:name":[{"@value":"Tatsuya Suwabe"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092779","@type":"Researcher","foaf:name":[{"@value":"Hirayasu Kai"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092752","@type":"Researcher","foaf:name":[{"@value":"Mahiro Kurashige"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092756","@type":"Researcher","foaf:name":[{"@value":"Koichi Seta"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092786","@type":"Researcher","foaf:name":[{"@value":"Keiji Shimazu"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092865","@type":"Researcher","foaf:name":[{"@value":"Tomofumi Moriyama"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092759","@type":"Researcher","foaf:name":[{"@value":"Mai Sato"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092757","@type":"Researcher","foaf:name":[{"@value":"Tadashi Otsuka"}]},{"@id":"https://cir.nii.ac.jp/crid/1420001326226540160","@type":"Researcher","personIdentifier":[{"@type":"KAKEN_RESEARCHERS","@value":"90742247"},{"@type":"NRID","@value":"1000090742247"},{"@type":"NRID","@value":"9000001092736"},{"@type":"NRID","@value":"9000415046098"},{"@type":"NRID","@value":"9000016665448"},{"@type":"NRID","@value":"9000411647703"},{"@type":"NRID","@value":"9000414521440"},{"@type":"RESEARCHMAP","@value":"https://researchmap.jp/katayamk"}],"foaf:name":[{"@value":"Kan Katayama"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092790","@type":"Researcher","foaf:name":[{"@value":"Wataru Shimabukuro"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092776","@type":"Researcher","foaf:name":[{"@value":"Takuya Fujimaru"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092766","@type":"Researcher","foaf:name":[{"@value":"Kenichiro Miura"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092767","@type":"Researcher","foaf:name":[{"@value":"Koichi Nakanishi"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092736","@type":"Researcher","foaf:name":[{"@value":"Shigeo Horie"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092788","@type":"Researcher","foaf:name":[{"@value":"Kengo Furuichi"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092778","@type":"Researcher","foaf:name":[{"@value":"Hirokazu Okada"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092740","@type":"Researcher","foaf:name":[{"@value":"Ichiei Narita"}]},{"@id":"https://cir.nii.ac.jp/crid/1380302865543092791","@type":"Researcher","foaf:name":[{"@value":"Satoru Muto"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"13421751"},{"@type":"EISSN","@value":"14377799"}],"prism:publicationName":[{"@value":"Clinical and Experimental Nephrology"}],"dc:publisher":[{"@value":"Springer Science and Business Media LLC"}],"prism:publicationDate":"2023-06-27","prism:volume":"27","prism:number":"10","prism:startingPage":"809","prism:endingPage":"818"},"reviewed":"false","dc:rights":["https://www.springernature.com/gp/researchers/text-and-data-mining","https://www.springernature.com/gp/researchers/text-and-data-mining"],"url":[{"@id":"https://link.springer.com/content/pdf/10.1007/s10157-023-02372-8.pdf"},{"@id":"https://link.springer.com/article/10.1007/s10157-023-02372-8/fulltext.html"}],"createdAt":"2023-06-27","modifiedAt":"2023-11-04","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=Japan","dc:title":"Japan"},{"@id":"https://cir.nii.ac.jp/all?q=Tolvaptan","dc:title":"Tolvaptan"},{"@id":"https://cir.nii.ac.jp/all?q=Humans","dc:title":"Humans"},{"@id":"https://cir.nii.ac.jp/all?q=Registries","dc:title":"Registries"},{"@id":"https://cir.nii.ac.jp/all?q=Polycystic%20Kidney,%20Autosomal%20Dominant","dc:title":"Polycystic Kidney, Autosomal Dominant"},{"@id":"https://cir.nii.ac.jp/all?q=Antidiuretic%20Hormone%20Receptor%20Antagonists","dc:title":"Antidiuretic Hormone Receptor Antagonists"},{"@id":"https://cir.nii.ac.jp/all?q=Antihypertensive%20Agents","dc:title":"Antihypertensive Agents"}],"project":[{"@id":"https://cir.nii.ac.jp/crid/1040569382227330432","@type":"Project","projectIdentifier":[{"@type":"KAKEN","@value":"21K08255"},{"@type":"JGN","@value":"JP21K08255"},{"@type":"URI","@value":"https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-21K08255/"}],"notation":[{"@language":"ja","@value":"巣状糸球体硬化症発症におけるポドサイト特異的遺伝子CRB2の機能解明"},{"@language":"en","@value":"Elucidation of the function of podocyte-specific gene CRB2 in the development of focal segmental glomerulosclerosis"}]}],"relatedProduct":[{"@id":"https://cir.nii.ac.jp/crid/1360009142924819072","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Prediction of Renal Prognosis in Patients with Autosomal 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A review of ADPKD focusing on the burden of disease and tolvaptan as a new treatment"}]},{"@id":"https://cir.nii.ac.jp/crid/1360283695000186624","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Long-term safety profile of tolvaptan in autosomal dominant polycystic kidney disease patients: TEMPO Extension Japan Trial"}]},{"@id":"https://cir.nii.ac.jp/crid/1360292619703127168","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1360294643837521664","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Urinary Aquaporin 2 as a Potential Indicator Predicting Tolvaptan Response in Patients With ADPKD"}]},{"@id":"https://cir.nii.ac.jp/crid/1360294643838533376","@type":"Article","resourceType":"学術雑誌論文(journal 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with early-onset intracranial aneurysms in patients with autosomal dominant polycystic kidney disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1361137044238192768","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Annual Dialysis Data Report 2014, JSDT Renal Data Registry (JRDR)"}]},{"@id":"https://cir.nii.ac.jp/crid/1361981470968124160","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Autosomal Dominant Polycystic Kidney Disease"}]},{"@id":"https://cir.nii.ac.jp/crid/1362544421299158656","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Characteristics of a medical care program for specific diseases in Japan in an era of changing cost-sharing"}]},{"@id":"https://cir.nii.ac.jp/crid/1362825893824199040","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Adherence to Stage-Specific Treatment Guidelines for Patients With Colon 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disease"}]},{"@id":"https://cir.nii.ac.jp/crid/2050307417127914496","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Effect of tolvaptan in Japanese patients with autosomal dominant polycystic kidney disease : a post hoc analysis of TEMPO 3:4 and TEMPO Extension Japan"}]},{"@id":"https://cir.nii.ac.jp/crid/2050870367074121472","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Autosomal dominant polycystic kidney disease : recent advances in pathogenesis and potential therapies"}]},{"@id":"https://cir.nii.ac.jp/crid/2050870367076253568","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Tolvaptan promotes urinary excretion of sodium and urea : a retrospective cohort study"}]},{"@id":"https://cir.nii.ac.jp/crid/2051151842062032256","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Initial decline in eGFR to predict tolvaptan response in autosomal-dominant polycystic kidney disease"}]},{"@id":"https://cir.nii.ac.jp/crid/2051433317027842944","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Evidence-based clinical practice guidelines for polycystic kidney disease 2014"}]},{"@id":"https://cir.nii.ac.jp/crid/2051433317036819328","@type":"Article","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"Safety and efficacy of Tolvaptan in real-world patients with autosomal dominant polycystic kidney disease- interim results of SLOW-PKD surveillance"}]},{"@id":"https://cir.nii.ac.jp/crid/2051433317036821760","@type":"Article","resourceType":"学術雑誌論文(journal article)","relationType":["references"],"jpcoar:relatedTitle":[{"@value":"A digest from evidence-based Clinical Practice Guideline for Polycystic Kidney Disease 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