{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,26]],"date-time":"2026-10-26T20:09:46Z","timestamp":1793045386821,"version":"4.5.0"},"reference-count":16,"publisher":"BMJ","issue":"6","license":[{"start":{"date-parts":[[2022,6,15]],"date-time":"2022-06-15T00:00:00Z","timestamp":1655251200000},"content-version":"unspecified","delay-in-days":14,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100013793","name":"Polycystic Kidney Disease Charity","doi-asserted-by":"publisher","award":["N\/A"],"award-info":[{"award-number":["N\/A"]}],"id":[{"id":"10.13039\/100013793","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Open"],"accepted":{"date-parts":[[2022,5,31]]},"published-print":{"date-parts":[[2022,6]]},"abstract":"<jats:sec>\n                  <jats:title>Objectives<\/jats:title>\n                  <jats:p>Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney condition, accounting for 7%\u201310% of patients with kidney failure. Fundamental basic science and clinical research on ADPKD is underway worldwide but no one has yet considered which areas should be prioritised to maximise returns from limited future funding. The Polycystic Kidney Disease Charity began a priority setting partnership with the James Lind Alliance (JLA) in the UK in 2019\u20132020 to identify areas of uncertainty in the ADPKD care pathway and allow patients, carers and healthcare professionals to rank the 10 most important questions for research.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Design<\/jats:title>\n                  <jats:p>The scope covered ADPKD diagnosis and management, identifying new treatments to prevent\/slow disease progression and practical, integrated patient support (<jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" ext-link-type=\"uri\" xlink:href=\"https:\/\/pkdcharity.org.uk\/research\/for-researchers\/adpkd-research-priorities\">https:\/\/pkdcharity.org.uk\/research\/for-researchers\/adpkd-research-priorities<\/jats:ext-link>). We used adapted JLA methodology. Initially, an independent information specialist collated uncertainties in ADPKD care from recent consensus conference proceedings and additional literature. These were refined into indicative questions with Steering Group oversight. Finally, the 10 most important questions were established via a survey and online consensus workshop.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Setting<\/jats:title>\n                  <jats:p>UK.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Participants<\/jats:title>\n                  <jats:p>747 survey respondents (76% patients, 13% carers, 11% healthcare professionals); 23 workshop attendees.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>117 uncertainties in ADPKD care were identified and refined into 35 indicative questions. A shortlist of 17 questions was established through the survey. Workshop participants reached agreement on the top 10 ranking. The top three questions prioritised by patients, carers and healthcare professionals centred around slowing disease progression, identifying persons for early treatment and organising care to improve outcomes.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Our shortlist reflects the varied physical, psychological and practical challenges of living with and treating ADPKD, and perceived gaps in knowledge that impair optimal care. We propose that future ADPKD research funding takes these priorities into account to focus on the most important areas and to maximise improvements in ADPKD outcomes.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/bmjopen-2021-055780","type":"journal-article","created":{"date-parts":[[2022,6,15]],"date-time":"2022-06-15T13:05:21Z","timestamp":1655298321000},"page":"e055780","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Research priorities for autosomal dominant polycystic kidney disease: a UK priority setting partnership"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3659-7438","authenticated-orcid":false,"given":"Tess","family":"Harris","sequence":"first","affiliation":[{"name":"Polycystic Kidney Disease Charity, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah R","family":"Bridges","sequence":"additional","affiliation":[{"name":"HB Health Comms Ltd, Orpington, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy D","family":"Brown","sequence":"additional","affiliation":[{"name":"London Kidney Network, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Natasha L","family":"O\u2019Brien","sequence":"additional","affiliation":[{"name":"ADPKD patient representative, Reading, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ann C","family":"Daly","sequence":"additional","affiliation":[{"name":"Birmingham Women\u2019s and Children\u2019s Hospitals NHS Foundation Trust, Birmingham, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bharat K","family":"Jindal","sequence":"additional","affiliation":[{"name":"Royal College of General Practitioners Yorkshire Faculty, Huddersfield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gillian S","family":"Mundy","sequence":"additional","affiliation":[{"name":"ADPKD Patient Representative, Newcastle, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7211-5400","authenticated-orcid":false,"given":"Albert","family":"Ong","sequence":"additional","affiliation":[{"name":"Academic Nephrology Unit, The Henry Wellcome Laboratories for Medical Research, University of Sheffield Medical School, Sheffield, UK"},{"name":"University of Sheffield, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Albert J","family":"Power","sequence":"additional","affiliation":[{"name":"North Bristol NHS Trust, Bristol, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard N","family":"Sandford","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, University of Cambridge, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Sayer","sequence":"additional","affiliation":[{"name":"University of Newcastle upon Tyne, Newcastle upon Tyne, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roslyn J","family":"Simms","sequence":"additional","affiliation":[{"name":"Northern General, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia D","family":"Wilson","sequence":"additional","affiliation":[{"name":"Department of Renal Medicine, Royal Free NHS Foundation Trust, University College London, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul J D","family":"Winyard","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryrose","family":"Tarpey","sequence":"additional","affiliation":[{"name":"James Lind Alliance, Southampton, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2022,6,15]]},"reference":[{"key":"2025101813305565000_12.6.e055780.1","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2018050493"},{"key":"2025101813305565000_12.6.e055780.2","first-page":"1356","article-title":"Prevalence of autosomal dominant polycystic kidney disease in the European Union","volume":"32","author":"Willey","year":"2017","journal-title":"Nephrol Dial Transplant"},{"key":"2025101813305565000_12.6.e055780.3","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(15)60907-2"},{"key":"2025101813305565000_12.6.e055780.4","doi-asserted-by":"publisher","DOI":"10.1038\/ki.2014.120"},{"key":"2025101813305565000_12.6.e055780.5","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(18)32782-X"},{"key":"2025101813305565000_12.6.e055780.6","doi-asserted-by":"crossref","DOI":"10.1186\/1471-2369-14-179","article-title":"Quality of life of patients with ADPKD\u2014Toranomon PKD QOL study: cross-sectional study","volume":"14","author":"Suwabe","year":"2013","journal-title":"BMC Nephrol"},{"key":"2025101813305565000_12.6.e055780.7","doi-asserted-by":"publisher","DOI":"10.1053\/j.ajkd.2013.08.017"},{"key":"2025101813305565000_12.6.e055780.8","doi-asserted-by":"publisher","DOI":"10.1038\/ki.2015.59"},{"key":"2025101813305565000_12.6.e055780.9","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1097\/NCC.0000000000000702","article-title":"How far do research priority setting exercises influence what research is undertaken: a little, a lot, or not at all?","volume":"42","author":"Gibson","year":"2019","journal-title":"Cancer Nurs"},{"key":"2025101813305565000_12.6.e055780.10","unstructured":"James Lind Alliance . The James Lind alliance guidebook version 10, 2021. Available: https:\/\/www.jla.nihr.ac.uk\/jla-guidebook\/"},{"key":"2025101813305565000_12.6.e055780.11","unstructured":"James Lind Alliance . Autosomal dominant polycystic kidney disease (ADPKD) priority setting partnership: scope, 2019. Available: https:\/\/pkdcharity.org.uk\/research\/for-researchers\/adpkd-research-priorities [Accessed 21 Oct 2019]."},{"key":"2025101813305565000_12.6.e055780.12","article-title":"Interventions for preventing the progression of autosomal dominant polycystic kidney disease","volume":"2015","author":"Bolignano","year":"2015","journal-title":"Cochrane Database Syst Rev"},{"key":"2025101813305565000_12.6.e055780.13","first-page":"847","article-title":"Determining the research priorities for patients with chronic kidney disease not on dialysis","volume":"32","author":"Hemmelgarn","year":"2017","journal-title":"Nephrol Dial Transplant"},{"key":"2025101813305565000_12.6.e055780.14","doi-asserted-by":"crossref","first-page":"255","DOI":"10.1053\/j.ajkd.2020.05.024","article-title":"Establishing a core outcome set for autosomal dominant polycystic kidney disease: report of the standardized outcomes in Nephrology-Polycystic kidney disease (SONG-PKD) consensus workshop","volume":"77","author":"Cho","year":"2021","journal-title":"Am J Kidney Dis"},{"key":"2025101813305565000_12.6.e055780.15","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1053\/j.ajkd.2020.01.005","article-title":"Core outcome domains for trials in autosomal dominant polycystic kidney disease: an international Delphi survey","volume":"76","author":"Cho","year":"2020","journal-title":"Am J Kidney Dis"},{"key":"2025101813305565000_12.6.e055780.16","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1111\/nep.13566","article-title":"Identifying patient-important outcomes in polycystic kidney disease: an international nominal group technique study","volume":"24","author":"Cho","year":"2019","journal-title":"Nephrology"}],"container-title":["BMJ Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bmjopen-2021-055780","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T20:31:07Z","timestamp":1760819467000},"score":1,"resource":{"primary":{"URL":"https:\/\/bmjopen.bmj.com\/lookup\/doi\/10.1136\/bmjopen-2021-055780"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6]]},"references-count":16,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2022,6,15]]},"published-print":{"date-parts":[[2022,6]]}},"alternative-id":["10.1136\/bmjopen-2021-055780"],"URL":"https:\/\/doi.org\/10.1136\/bmjopen-2021-055780","relation":{},"ISSN":["2044-6055","2044-6055"],"issn-type":[{"value":"2044-6055","type":"print"},{"value":"2044-6055","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6]]}}}