{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T19:24:57Z","timestamp":1792092297586,"version":"4.4.0"},"reference-count":30,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2020,6,1]],"date-time":"2020-06-01T00:00:00Z","timestamp":1590969600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,6,1]],"date-time":"2020-06-01T00:00:00Z","timestamp":1590969600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,1,13]],"date-time":"2020-01-13T00:00:00Z","timestamp":1578873600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01KG1104"],"award-info":[{"award-number":["01KG1104"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["2010-024300-10"],"award-info":[{"award-number":["2010-024300-10"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["NCT01485978"],"award-info":[{"award-number":["NCT01485978"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009890","name":"American Society of Pediatric Nephrology","doi-asserted-by":"publisher","award":["NCT00622544"],"award-info":[{"award-number":["NCT00622544"]}],"id":[{"id":"10.13039\/100009890","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["kidney-international.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Kidney International"],"published-print":{"date-parts":[[2020,6]]},"DOI":"10.1016\/j.kint.2019.12.015","type":"journal-article","created":{"date-parts":[[2020,1,17]],"date-time":"2020-01-17T11:20:28Z","timestamp":1579260028000},"page":"1275-1286","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":152,"title":["A multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport\u2019s syndrome"],"prefix":"10.1016","volume":"97","author":[{"given":"Oliver","family":"Gross","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Burkhard","family":"T\u00f6nshoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lutz T.","family":"Weber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Pape","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kay","family":"Latta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henry","family":"Fehrenbach","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Baerbel","family":"Lange-Sperandio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hildegard","family":"Zappel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Hoyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hagen","family":"Staude","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"K\u00f6nig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"John","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jutta","family":"Gellermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernd","family":"Hoppe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Galiano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Britta","family":"Hoecker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rasmus","family":"Ehren","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Lerch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clifford E.","family":"Kashtan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Harden","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Boeckhaus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tim","family":"Friede","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Koziolek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten Paul","family":"Bramlage","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frauke","family":"Weber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanja","family":"Albrecht-Nock","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Sonntag","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny","family":"Frese","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Kettwig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reinhard","family":"Hilgers","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Hansen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirja","family":"Wedekin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Meyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Klaiber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Gessner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Max","family":"Liebau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne-Kristin","family":"Vogt-Weigeldt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Therese","family":"Jungraithmayr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Ponsel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrike","family":"Jacoby","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Konrad","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitta","family":"Kranz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens","family":"Koenig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Loechtermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Pohl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ralf","family":"Husain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katrin","family":"Mueller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Thumfart","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gesa","family":"Schalk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Feldkoetter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katja","family":"Sauerstein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelin","family":"Muschiol","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heiko","family":"Billing","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frauke","family":"Wilkening","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.kint.2019.12.015_bib1","doi-asserted-by":"crossref","first-page":"1888","DOI":"10.1016\/S0140-6736(17)30788-2","article-title":"Global kidney health 2017 and beyond: a roadmap for closing gaps in care, research, and policy","volume":"390","author":"Levin","year":"2017","journal-title":"Lancet"},{"key":"10.1016\/j.kint.2019.12.015_bib2","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1016\/S0140-6736(18)33000-9","article-title":"Glomerular podocytes in kidney health and disease","volume":"393","author":"Assady","year":"2019","journal-title":"Lancet"},{"key":"10.1016\/j.kint.2019.12.015_bib3","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1056\/NEJMoa1806891","article-title":"Diagnostic utility of exome sequencing for kidney disease","volume":"380","author":"Groopman","year":"2019","journal-title":"N\u00a0Engl J Med"},{"issue":"9","key":"10.1016\/j.kint.2019.12.015_bib4","doi-asserted-by":"crossref","DOI":"10.1038\/ejhg.2014.254","article-title":"Clinical utility gene card for: Alport syndrome\u2014update 2014","volume":"23","author":"Hertz","year":"2015","journal-title":"Eur J Hum Genet"},{"key":"10.1016\/j.kint.2019.12.015_bib5","doi-asserted-by":"crossref","first-page":"2543","DOI":"10.1056\/NEJMra022296","article-title":"Alport\u2019s syndrome, Goodpasture\u2019s syndrome, and type IV collagen","volume":"348","author":"Hudson","year":"2003","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.kint.2019.12.015_bib6","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1038\/nrneph.2012.259","article-title":"Alport syndrome\u2014insights from basic and clinical research","volume":"9","author":"Kruegel","year":"2013","journal-title":"Nat Rev Nephrol"},{"key":"10.1016\/j.kint.2019.12.015_bib7","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1016\/j.kint.2017.12.018","article-title":"Alport syndrome: a unified classification of genetic disorders of collagen IV \u03b1345: a position paper of the Alport Syndrome Classification Working Group","volume":"93","author":"Kashtan","year":"2018","journal-title":"Kidney Int"},{"key":"10.1016\/j.kint.2019.12.015_bib8","doi-asserted-by":"crossref","first-page":"494","DOI":"10.1038\/ki.2011.407","article-title":"Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy","volume":"81","author":"Gross","year":"2012","journal-title":"Kidney Int"},{"key":"10.1016\/j.kint.2019.12.015_bib9","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1046\/j.1523-1755.2003.00779.x","article-title":"Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome","volume":"63","author":"Gross","year":"2003","journal-title":"Kidney Int"},{"key":"10.1016\/j.kint.2019.12.015_bib10","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1038\/sj.ki.5002115","article-title":"Choosing a mouse model to study the molecular pathobiology of Alport glomerulonephritis","volume":"71","author":"Cosgrove","year":"2007","journal-title":"Kidney Int"},{"key":"10.1016\/j.kint.2019.12.015_bib11","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1016\/j.matbio.2018.04.008","article-title":"Alport syndrome and Pierson syndrome: diseases of the glomerular basement membrane","volume":"71\u201372","author":"Funk","year":"2018","journal-title":"Matrix Biol"},{"key":"10.1016\/j.kint.2019.12.015_bib12","doi-asserted-by":"crossref","first-page":"13","DOI":"10.1159\/000381480","article-title":"A\u00a0multidrug, antiproteinuric approach to Alport syndrome: a ten-year cohort study","volume":"130","author":"Daina","year":"2015","journal-title":"Nephron"},{"key":"10.1016\/j.kint.2019.12.015_bib13","first-page":"436046","article-title":"Safety and efficacy of the ACE-inhibitor ramipril in Alport syndrome: the double-blind, randomized, placebo-controlled, multicenter phase III EARLY PRO-TECT Alport trial in pediatric patients","volume":"2012","author":"Gross","year":"2012","journal-title":"ISRNPediatr"},{"key":"10.1016\/j.kint.2019.12.015_bib14","doi-asserted-by":"crossref","first-page":"1639","DOI":"10.1056\/NEJMoa0902066","article-title":"Strict blood-pressure control and progression of renal failure in children","volume":"361","author":"W\u00fchl","year":"2009","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.kint.2019.12.015_bib15","doi-asserted-by":"crossref","first-page":"2225","DOI":"10.1681\/ASN.2018010036","article-title":"Early proteinuria lowering by angiotensin-converting enzyme inhibition predicts renal survival in children with CKD","volume":"29","author":"van den Belt","year":"2018","journal-title":"J\u00a0Am Soc Nephrol"},{"key":"10.1016\/j.kint.2019.12.015_bib16","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1007\/s00467-012-2138-4","article-title":"Clinical practice guidelines for the treatment of Alport syndrome. a statement of the Alport Syndrome Research Collaborative","volume":"28","author":"Kashtan","year":"2012","journal-title":"Ped Nephrol"},{"key":"10.1016\/j.kint.2019.12.015_bib17","doi-asserted-by":"crossref","first-page":"1844","DOI":"10.1016\/S0140-6736(14)60659-0","article-title":"Rare inherited kidney diseases: challenges, opportunities, and perspectives","volume":"383","author":"Devuyst","year":"2014","journal-title":"Lancet"},{"key":"10.1016\/j.kint.2019.12.015_bib18","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1681\/ASN.2008030287","article-title":"New equations to estimate GFR in children with CKD","volume":"20","author":"Schwartz","year":"2009","journal-title":"J\u00a0Am Soc Nephrol"},{"key":"10.1016\/j.kint.2019.12.015_bib19","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1186\/s13023-018-0919-y","article-title":"Recent advances in methodology for clinical trials in small populations: the InSPiRe project","volume":"13","author":"Friede","year":"2018","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.kint.2019.12.015_bib20","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1002\/sim.7991","article-title":"Model averaging for robust extrapolation in evidence synthesis","volume":"38","author":"R\u00f6ver","year":"2019","journal-title":"Stat Med"},{"key":"10.1016\/j.kint.2019.12.015_bib21","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1002\/jrsm.1217","article-title":"Meta-analysis of few small studies in orphan diseases","volume":"8","author":"Friede","year":"2017","journal-title":"Res Syn Meth"},{"key":"10.1016\/j.kint.2019.12.015_bib22","doi-asserted-by":"crossref","unstructured":"Torra R, Furlano M. New therapeutic options for Alport syndrome [epub ahead of print]. Nephrol Dial Transplant. https:\/\/doi.org\/10.1093\/ndt\/gfz131. Accessed February 18, 2020.","DOI":"10.1093\/ndt\/gfz131"},{"key":"10.1016\/j.kint.2019.12.015_bib23","doi-asserted-by":"crossref","first-page":"1492","DOI":"10.1136\/bmj.319.7223.1492","article-title":"Calculating the number needed to treat for trials where the outcome is time to an event","volume":"319","author":"Altman","year":"1999","journal-title":"BMJ"},{"key":"10.1016\/j.kint.2019.12.015_bib24","doi-asserted-by":"crossref","first-page":"2225","DOI":"10.1056\/NEJMoa1415463","article-title":"Intensive supportive care plus immunosuppression in IgA nephropathy","volume":"373","author":"Rauen","year":"2015","journal-title":"N\u00a0Engl J Med"},{"key":"10.1016\/j.kint.2019.12.015_bib25","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1038\/nrneph.2013.109","article-title":"The glomerular basement membrane as a barrier to albumin","volume":"9","author":"Suh","year":"2013","journal-title":"Nat Rev Nephrol"},{"key":"10.1016\/j.kint.2019.12.015_bib26","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/S2213-8587(18)30314-0","article-title":"Chronic Kidney Disease Epidemiology Collaboration. Change in albuminuria as a surrogate endpoint for progression of kidney disease: a meta-analysis of treatment effects in randomised clinical trials","volume":"7","author":"Heerspink","year":"2019","journal-title":"Lancet Diabetes Endocrinol"},{"key":"10.1016\/j.kint.2019.12.015_bib27","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1007\/s00467-016-3452-z","article-title":"Prospective study on the potential of RAAS blockade to halt renal disease in Alport syndrome patients with heterozygous mutations","volume":"32","author":"Stock","year":"2017","journal-title":"Pediatr Nephrol"},{"key":"10.1016\/j.kint.2019.12.015_bib28","article-title":"Angiotensin converting enzyme inhibitors and risk of lung cancer: population based cohort study","volume":"363","author":"Hicks","year":"2018","journal-title":"BMJ"},{"key":"10.1016\/j.kint.2019.12.015_bib29","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1038\/nrneph.2017.167","article-title":"Genomic medicine for kidney disease","volume":"14","author":"Groopman","year":"2018","journal-title":"Nat Rev Nephrol"},{"key":"10.1016\/j.kint.2019.12.015_bib30","doi-asserted-by":"crossref","first-page":"2371","DOI":"10.1056\/NEJMe1904740","article-title":"Clinical credence\u2014SGLT2 inhibitors, diabetes, and chronic kidney disease","volume":"380","author":"Ingelfinger","year":"2019","journal-title":"N\u00a0Engl J Med"}],"container-title":["Kidney International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0085253820300260?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0085253820300260?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T23:20:44Z","timestamp":1778714444000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0085253820300260"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6]]},"references-count":30,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2020,6]]}},"alternative-id":["S0085253820300260"],"URL":"https:\/\/doi.org\/10.1016\/j.kint.2019.12.015","relation":{},"ISSN":["0085-2538"],"issn-type":[{"value":"0085-2538","type":"print"}],"subject":[],"published":{"date-parts":[[2020,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"A multicenter, randomized, placebo-controlled, double-blind phase 3 trial with open-arm comparison indicates safety and efficacy of nephroprotective therapy with ramipril in children with Alport\u2019s syndrome","name":"articletitle","label":"Article Title"},{"value":"Kidney International","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.kint.2019.12.015","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 International Society of Nephrology. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}