{"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":1793045386794,"version":"4.5.0"},"reference-count":10,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"8","funder":[{"DOI":"10.13039\/100001296","name":"PKD Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001296","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Kidney360"],"published-print":{"date-parts":[[2022,8,25]]},"abstract":"\n Key Points<\/jats:title>\n \n \n \n As of October 2021, more than 1500 autosomal dominant polycystic kidney disease (ADPKD) patients have signed up to participate in the ADPKD Registry, which collects patient-reported data.<\/jats:p>\n <\/jats:list-item>\n \n The Registry is a valuable tool for dissemination of information about studies for which individuals may qualify\u2014seven studies so far.<\/jats:p>\n <\/jats:list-item>\n \n By incorporating two validated outcome measures, the ADPKD-IS and ADPKD-PDS, the Registry holds unique data on a national scale.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:p>\n <\/jats:sec>\n \n Background<\/jats:title>\n Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of inherited kidney disease worldwide. Over the past five years, the therapeutic pipeline for ADPKD has expanded, leading to a growing need for patient enrollment in clinical trials and improved understanding of patient-centered outcomes that can be used in trial design. To advance these goals, the Polycystic Kidney Disease Foundation (PKDF) established a national web-based ADPKD Registry.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n The ADPKD Registry is hosted on a secure, HIPAA-compliant, online platform (IQVIA, oc-meridian.com\/pkdcure). Participants are consented through the online system and complete a series of modules. The Core Questionnaire includes patient-reported diagnosis, latest creatinine values, and comorbidities. Additional modules include surveys of family history, diet, quality of life, extrarenal manifestations, and attitudes surrounding research participation.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n As of October 2021, 1563 ADPKD patients across the United States have registered and completed the Core Questionnaire. Participants have a median age of 44 years and are 72% women, 93% White, with 4% self-identifying as Hispanic\/Latino and 2% as Black. All CKD stages are present, including post kidney transplant. To date, seven clinical studies have used the Registry as a recruitment tool. Additionally, quality-of-life burden scores revealed a correlation with disease stage as determined by kidney function.<\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n The Registry described here is the only one of its kind and is a valuable longitudinal research tool encompassing all stages of ADPKD. The registry will allow investigators to pursue a range of research questions related to the management of ADPKD, including definition of health-related quality of life (HRQoL) outcomes and recruitment for a variety of observational and therapeutic clinical protocols.<\/jats:p>\n <\/jats:sec>","DOI":"10.34067\/kid.0002372022","type":"journal-article","created":{"date-parts":[[2022,5,23]],"date-time":"2022-05-23T12:23:25Z","timestamp":1653308605000},"page":"1350-1358","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":8,"title":["Design and Basic Characteristics of a National Patient-Powered Registry in ADPKD"],"prefix":"10.34067","volume":"3","clinical-trial-number":[{"clinical-trial-number":"nct03918447","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct04534985","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct04064346","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct04152837","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03273413","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct04536688","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct04534985","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03995901","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct04338048","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03273413","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct03918447","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0119-3724","authenticated-orcid":false,"given":"Elise","family":"Hoover","sequence":"first","affiliation":[{"name":"PKD Foundation, Kansas City, Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald D.","family":"Perrone","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Nephrology, Tufts Medical Center, Boston, Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chris","family":"Rusconi","sequence":"additional","affiliation":[{"name":"PKD Foundation, Kansas City, Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beverly","family":"Benson","sequence":"additional","affiliation":[{"name":"PKD Foundation, Kansas City, Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5809-9961","authenticated-orcid":false,"given":"Neera K.","family":"Dahl","sequence":"additional","affiliation":[{"name":"Section of Nephrology, Yale University School of Medicine, New Haven, Connecticut"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berenice","family":"Gitomer","sequence":"additional","affiliation":[{"name":"Division of Renal Diseases and Hypertension, University of Colorado Anschutz Medical Campus, Aurora, Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy","family":"Manelli","sequence":"additional","affiliation":[{"name":"PKD Foundation, Kansas City, Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8981-1843","authenticated-orcid":false,"given":"Michal","family":"Mrug","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Veterans Affairs Medical Center and University of Alabama at Birmingham, Birmingham, Alabama"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meyeon","family":"Park","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Department of Medicine, University of California San Francisco, San Francisco, California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1693-0734","authenticated-orcid":false,"given":"Stephen L.","family":"Seliger","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Nephrology, University of Maryland School of Medicine, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6472-9325","authenticated-orcid":false,"given":"Milind A.","family":"Phadnis","sequence":"additional","affiliation":[{"name":"Department of Biostatistics. University of Kansas Medical Center, Kansas City, Kansas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadeesha","family":"Thewarapperuma","sequence":"additional","affiliation":[{"name":"Department of Biostatistics. University of Kansas Medical Center, Kansas City, Kansas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Terry J.","family":"Watnick","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Nephrology, University of Maryland School of Medicine, Baltimore, Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2022,8,25]]},"reference":[{"key":"B2-20250825","doi-asserted-by":"crossref","first-page":"1765","DOI":"10.2215\/CJN.03960318","article-title":"Recent advances in the management of autosomal dominant polycystic kidney disease","volume":"13","author":"Chebib","year":"2018","journal-title":"Clin J Am Soc Nephrol"},{"key":"B9-20250825","article-title":"National Kidney Foundation practice guidelines for chronic kidney disease: Evaluation, classification, and stratification"},{"key":"B11-20250825","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1053\/j.ajkd.2017.08.020","article-title":"Development of the Autosomal Dominant Polycystic Kidney Disease Impact Scale: A new health-related quality-of-life instrument","volume":"71","author":"Oberdhan","year":"2018","journal-title":"Am J Kidney Dis"},{"key":"B12-20250825","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1038\/ki.1992.195","article-title":"Factors affecting the progression of renal disease in autosomal-dominant polycystic kidney disease","volume":"41","author":"Gabow","year":"1992","journal-title":"Kidney Int"},{"key":"B13-20250825","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1093\/qjmed\/hct082","article-title":"The natural history of autosomal dominant polycystic kidney disease: 30-year experience from a single centre","volume":"106","author":"Thong","year":"2013","journal-title":"QJM"},{"key":"B14-20250825","doi-asserted-by":"crossref","first-page":"e92232","DOI":"10.1371\/journal.pone.0092232","article-title":"Clinical characteristics and disease predictors of a large Chinese cohort of patients with autosomal dominant polycystic kidney disease","volume":"9","author":"Chen","year":"2014","journal-title":"PLoS One"},{"key":"B15-20250825","first-page":"2106","article-title":"Health-related quality of life across all stages of autosomal dominant polycystic kidney disease","volume":"32","author":"Eriksson","year":"2017","journal-title":"Nephrol Dial Transplant"},{"key":"B16-20250825","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1053\/j.ajkd.2013.08.017","article-title":"Health-related quality of life in patients with autosomal dominant polycystic kidney disease and CKD stages 1\u20134: A cross-sectional study","volume":"63","author":"Miskulin","year":"2014","journal-title":"Am J Kidney Dis"},{"key":"B17-20250825","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.34067\/KID.0004002020","article-title":"Baseline characteristics and patient-reported outcomes of ADPKD patients in the multicenter TAME-PKD clinical trial","volume":"1","author":"Seliger","year":"2020","journal-title":"Kidney360"},{"key":"B18-20250825","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1186\/s13023-018-0836-0","article-title":"Rare disease registries: Potential applications towards impact on development of new drug treatments","volume":"13","author":"Jansen-van der Weide","year":"2018","journal-title":"Orphanet J Rare Dis"}],"container-title":["Kidney360"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.34067\/KID.0002372022","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T20:01:27Z","timestamp":1786478487000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.34067\/KID.0002372022"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,25]]},"references-count":10,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2022]]}},"URL":"https:\/\/doi.org\/10.34067\/kid.0002372022","relation":{},"ISSN":["2641-7650"],"issn-type":[{"value":"2641-7650","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,25]]},"assertion":[{"value":"2022-03-29","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-05-20","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}