{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T00:58:40Z","timestamp":1791853120814,"version":"4.3.4"},"reference-count":27,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","funder":[{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"crossref","award":["R01 DK113111"],"award-info":[{"award-number":["R01 DK113111"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100000062","name":"National Institute of Diabetes and Digestive and Kidney Diseases","doi-asserted-by":"crossref","award":["U54 DK126126"],"award-info":[{"award-number":["U54 DK126126"]}],"id":[{"id":"10.13039\/100000062","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["JASN"],"published-print":{"date-parts":[[2026,5]]},"abstract":"\n Key Points<\/jats:title>\n \n \n \n Data were collected from a cohort of 759 patients with autosomal dominant polycystic kidney disease that were followed for a median of 10.2 years.<\/jats:p>\n <\/jats:list-item>\n \n Prognostic models were developed to predict kidney failure using routinely obtained clinical information and externally validated.<\/jats:p>\n <\/jats:list-item>\n \n Risk predictions had good discrimination and calibration over a time horizon of 15 years.<\/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 a common cause of kidney failure. Progression is highly variable, and accurate prognostic information is needed to guide early treatment decisions. The objective of this study was to develop a multivariable predictive model for progression to kidney failure.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n We developed prognostic models using Cox regression for the outcome of kidney failure, defined as eGFR <15 ml\/min per 1.73 m\n 2<\/jats:sup>\n , dialysis, or kidney transplantation. The development dataset consisted of participants in the Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) and Halt Progression of Polycystic Kidney Disease study A (HALT-A) studies. The validation dataset consisted of patients with ADPKD in the Mayo clinical registry aged 15\u201349 years, with eGFR \u226560 ml\/min per 1.73 m\n 2<\/jats:sup>\n . Predictor variables in the base model were age, sex, creatinine or eGFR, ADPKD genotype, and Mayo Imaging Class. Clinical and laboratory data were evaluated in the full model.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n The development cohort included 759 patients with baseline eGFR of 91\u00b129 ml\/min per 1.73 m\n 2<\/jats:sup>\n (mean\u00b1SD), of whom 16% reached end point after median (interquartile range) follow-up of 10.2 (5.5\u201316.7) years. The validation cohort included 535 patients with baseline eGFR of 90\u00b131 ml\/min per 1.73 m\n 2<\/jats:sup>\n (mean\u00b1SD), of whom 11% reached end point after median (interquartile range) follow-up of 5.5 (1.5\u201313.7) years. The full model, including age, sex, serum creatinine, Mayo Imaging Class, total carbon dioxide, hemoglobin, diastolic BP, and body mass index, had a C-index of 0.81 (95% confidence interval, 0.72 to 0.90) in the validation cohort and 0.75 (95% confidence interval, 0.62 to 0.88) when ADPKD genotype was also included. Risk predictions from base and full models were well-calibrated out to 15 years.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n In persons with early ADPKD and preserved eGFR, a model using routinely obtained clinical information quantified risk of kidney failure over 15 years.<\/jats:p>\n <\/jats:sec>","DOI":"10.1681\/asn.0000000950","type":"journal-article","created":{"date-parts":[[2026,1,19]],"date-time":"2026-01-19T12:00:19Z","timestamp":1768824019000},"page":"1010-1021","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":3,"title":["Development and Validation of a Multivariable Prediction Model for Kidney Failure in Early Autosomal Dominant Polycystic Kidney Disease"],"prefix":"10.1681","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1776-2533","authenticated-orcid":false,"given":"Alan S.L.","family":"Yu","sequence":"first","affiliation":[{"name":"Division of Nephrology and Hypertension and the Jared Grantham Kidney Institute, University of Kansas Medical Center, Kansas City, Kansas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aaron","family":"Cohen","sequence":"additional","affiliation":[{"name":"Department of Epidemiology and Biostatistics, Indiana University School of Public Health, Bloomington, Indiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vicente E.","family":"Torres","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3949-5720","authenticated-orcid":false,"given":"Fouad T.","family":"Chebib","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Mayo Clinic, Jacksonville, Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas P.","family":"Landsittel","sequence":"additional","affiliation":[{"name":"Department of Epidemiology and Biostatistics, Indiana University School of Public Health, Bloomington, Indiana"},{"name":"Department of Biostatistics, University at Buffalo, The State University of New York, New York, New York"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arlene B.","family":"Chapman","sequence":"additional","affiliation":[{"name":"Division of Nephrology, University of Chicago School of Medicine, Chicago, Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michal","family":"Mrug","sequence":"additional","affiliation":[{"name":"Division of Nephrology, University of Alabama and the Department of Veterans Affairs Medical Center, Birmingham, Alabama"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5304-6593","authenticated-orcid":false,"given":"Peter C.","family":"Harris","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederic F.","family":"Rahbari-Oskoui","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Emory University School of Medicine, Atlanta, Georgia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8617-6768","authenticated-orcid":false,"given":"Erin","family":"Ables","sequence":"additional","affiliation":[{"name":"Department of Epidemiology and Biostatistics, Indiana University School of Public Health, Bloomington, Indiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5262-9693","authenticated-orcid":false,"given":"Chelsie","family":"Parker","sequence":"additional","affiliation":[{"name":"Department of Epidemiology and Biostatistics, Indiana University School of Public Health, Bloomington, Indiana"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0001-0581-1211","authenticated-orcid":false,"given":"Fadi George","family":"Munairdjy Debeh","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Mayo Clinic, Jacksonville, Florida"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maroun","family":"Chedid","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-6487-8312","authenticated-orcid":false,"given":"Doaa","family":"Elbarougy","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6304-4890","authenticated-orcid":false,"given":"Kyongtae Ty","family":"Bae","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Radiology, University of Hong Kong, Pok Fu Lam, Hong Kong"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William M.","family":"Bennett","sequence":"additional","affiliation":[{"name":"Legacy Good Samaritan Hospital, Portland, Oregon"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,12,3]]},"reference":[{"issue":"2","key":"B2-20260526","first-page":"73","article-title":"The neonate with adult-type autosomal dominant polycystic kidney disease","volume":"2","author":"Chevalier","year":"1981","journal-title":"Int J Pediatr Nephrol."},{"issue":"3","key":"B3-20260526","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1002\/ajmg.1320510314","article-title":"Autosomal dominant polycystic kidney disease in the fetus","volume":"51","author":"Michaud","year":"1994","journal-title":"Am J Med Genet."},{"issue":"5","key":"B4-20260526","doi-asserted-by":"crossref","first-page":"889","DOI":"10.2215\/CJN.00550110","article-title":"Evidence of extraordinary growth in the progressive enlargement of renal cysts","volume":"5","author":"Grantham","year":"2010","journal-title":"Clin J Am Soc Nephrol."},{"issue":"20","key":"B5-20260526","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1056\/NEJMoa054341","article-title":"Volume progression in polycystic kidney disease","volume":"354","author":"Grantham","year":"2006","journal-title":"N Engl J Med."},{"issue":"5","key":"B6-20260526","doi-asserted-by":"crossref","first-page":"1253","DOI":"10.1016\/j.kint.2018.12.023","article-title":"Long-term trajectory of kidney function in autosomal-dominant polycystic kidney disease","volume":"95","author":"Yu","year":"2019","journal-title":"Kidney Int."},{"issue":"4","key":"B7-20260526","doi-asserted-by":"crossref","first-page":"710","DOI":"10.2215\/CJN.01581105","article-title":"Incident renal events and risk factors in autosomal dominant polycystic kidney disease: a population and family-based cohort followed for 22 years","volume":"1","author":"Dicks","year":"2006","journal-title":"Clin J Am Soc Nephrol."},{"issue":"1","key":"B8-20260526","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1681\/ASN.2013101138","article-title":"Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials","volume":"26","author":"Irazabal","year":"2015","journal-title":"J Am Soc Nephrol."},{"issue":"10","key":"B9-20260526","doi-asserted-by":"crossref","first-page":"2458","DOI":"10.1681\/ASN.2018060590","article-title":"A practical guide for treatment of rapidly progressive ADPKD with tolvaptan","volume":"29","author":"Chebib","year":"2018","journal-title":"J Am Soc Nephrol."},{"issue":"5","key":"B10-20260526","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1093\/ndt\/gfab312","article-title":"An update on the use of tolvaptan for autosomal dominant polycystic kidney disease: consensus statement on behalf of the ERA Working Group on Inherited Kidney Disorders, the European Rare Kidney Disease Reference Network and Polycystic Kidney Disease International","volume":"37","author":"M\u00fcller","year":"2022","journal-title":"Nephrol Dial Transplant."},{"issue":"5","key":"B11-20260526","doi-asserted-by":"crossref","first-page":"591","DOI":"10.2215\/CJN.0000000000000427","article-title":"Validation of the Mayo imaging classification system for predicting kidney outcomes in ADPKD","volume":"19","author":"Bais","year":"2024","journal-title":"Clin J Am Soc Nephrol."},{"issue":"3","key":"B12-20260526","doi-asserted-by":"crossref","first-page":"640","DOI":"10.2215\/CJN.03250410","article-title":"Potentially modifiable factors affecting the progression of autosomal dominant polycystic kidney disease","volume":"6","author":"Torres","year":"2011","journal-title":"Clin J Am Soc Nephrol."},{"issue":"3","key":"B13-20260526","doi-asserted-by":"crossref","first-page":"479","DOI":"10.2215\/CJN.09500911","article-title":"Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease","volume":"7","author":"Chapman","year":"2012","journal-title":"Clin J Am Soc Nephrol."},{"issue":"3","key":"B14-20260526","doi-asserted-by":"crossref","first-page":"942","DOI":"10.1681\/ASN.2015010016","article-title":"The PROPKD score: a new algorithm to predict renal survival in autosomal dominant polycystic kidney disease","volume":"27","author":"Cornec-Le Gall","year":"2016","journal-title":"J Am Soc Nephrol."},{"issue":"1","key":"B15-20260526","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1186\/s12882-017-0804-2","article-title":"A model to predict disease progression in patients with autosomal dominant polycystic kidney disease (ADPKD): the ADPKD outcomes model","volume":"19","author":"McEwan","year":"2018","journal-title":"BMC Nephrol."},{"issue":"2","key":"B16-20260526","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/j.ekir.2017.10.004","article-title":"Urinary biomarkers to identify autosomal dominant polycystic kidney disease patients with a high likelihood of disease progression","volume":"3","author":"Messchendorp","year":"2018","journal-title":"Kidney Int Rep."},{"issue":"24","key":"B17-20260526","doi-asserted-by":"crossref","first-page":"2255","DOI":"10.1056\/NEJMoa1402685","article-title":"Blood pressure in early autosomal dominant polycystic kidney disease","volume":"371","author":"Schrier","year":"2014","journal-title":"New Engl J Med."},{"issue":"19","key":"B18-20260526","doi-asserted-by":"crossref","first-page":"1737","DOI":"10.1056\/NEJMoa2102953","article-title":"New Creatinine- and cystatin C-Based equations to estimate GFR without race","volume":"385","author":"Inker","year":"2021","journal-title":"N Engl J Med."},{"issue":"9","key":"B19-20260526","doi-asserted-by":"crossref","first-page":"2872","DOI":"10.1681\/ASN.2015050583","article-title":"Predicted mutation strength of nontruncating PKD1 mutations aids genotype-phenotype correlations in autosomal dominant polycystic kidney disease","volume":"27","author":"Heyer","year":"2016","journal-title":"J Am Soc Nephrol."},{"issue":"10","key":"B20-20260526","doi-asserted-by":"crossref","first-page":"1105","DOI":"10.1002\/sim.4154","article-title":"On the C-statistics for evaluating overall adequacy of risk prediction procedures with censored survival data","volume":"30","author":"Uno","year":"2011","journal-title":"Stat Med."},{"issue":"3","key":"B21-20260526","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1016\/j.kint.2017.09.027","article-title":"Baseline total kidney volume and the rate of kidney growth are associated with chronic kidney disease progression in autosomal dominant polycystic kidney disease","volume":"93","author":"Yu","year":"2018","journal-title":"Kidney Int."},{"issue":"15","key":"B22-20260526","doi-asserted-by":"crossref","first-page":"e138724","DOI":"10.1172\/jci.insight.138724","article-title":"The value of genotypic and imaging information to predict functional and structural outcomes in ADPKD","volume":"5","author":"Lavu","year":"2020","journal-title":"JCI Insight."},{"issue":"15","key":"B23-20260526","doi-asserted-by":"crossref","first-page":"1553","DOI":"10.1001\/jama.2011.451","article-title":"A predictive model for progression of chronic kidney disease to kidney failure","volume":"305","author":"Tangri","year":"2011","journal-title":"JAMA."},{"issue":"6","key":"B24-20260526","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1016\/j.kint.2018.01.009","article-title":"Predicting timing of clinical outcomes in patients with chronic kidney disease and severely decreased glomerular filtration rate","volume":"93","author":"Grams","year":"2018","journal-title":"Kidney Int."},{"issue":"4","key":"B25-20260526","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1053\/j.ajkd.2013.01.017","article-title":"Association of serum bicarbonate with risk of renal and cardiovascular outcomes in CKD: a report from the chronic renal insufficiency cohort (CRIC) study","volume":"62","author":"Dobre","year":"2013","journal-title":"Am J Kidney Dis."},{"issue":"8-9","key":"B26-20260526","doi-asserted-by":"crossref","first-page":"603","DOI":"10.1159\/000527036","article-title":"Relationship between metabolic acidosis and chronic kidney disease progression across racial and ethnic groups: an observational, retrospective cohort study","volume":"53","author":"Tangri","year":"2022","journal-title":"Am J Nephrol."},{"issue":"12","key":"B27-20260526","doi-asserted-by":"crossref","first-page":"2248","DOI":"10.1093\/ndt\/gfaa283","article-title":"Serum bicarbonate is associated with kidney outcomes in autosomal dominant polycystic kidney disease","volume":"36","author":"Blijdorp","year":"2021","journal-title":"Nephrol Dial Transplant."},{"issue":"2S","key":"B29-20260526","first-page":"S1","article-title":"KDIGO 2025 clinical practice guideline for the evaluation, management, and treatment of autosomal dominant polycystic kidney disease (ADPKD)","volume":"107","year":"2025","journal-title":"Kidney Int."}],"container-title":["Journal of the American Society of Nephrology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.1681\/ASN.0000000950","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T19:26:57Z","timestamp":1786130817000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.1681\/ASN.0000000950"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,12,3]]},"references-count":27,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.1681\/asn.0000000950","relation":{},"ISSN":["1046-6673","1533-3450"],"issn-type":[{"value":"1046-6673","type":"print"},{"value":"1533-3450","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,12,3]]},"assertion":[{"value":"2025-04-02","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-25","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}