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At least 20% of children with ADPKD have relevant, yet mainly asymptomatic disease manifestations such as hypertension or proteinuria (in line with findings in adults with ADPKD, where hypertension and cardiovascular damage precede decline in kidney function). We propose an algorithm for work-up and management based on current recommendations that integrates the need to screen regularly for hypertension and proteinuria in offspring of affected parents with different options regarding diagnostic testing, which need to be discussed with the family with regard to ethical and practical aspects. Indications and scope of genetic testing are discussed. Pharmacological management includes renin-angiotensin system blockade as first-line therapy for hypertension and proteinuria. The vasopressin receptor antagonist tolvaptan is licensed for delaying disease progression in adults with ADPKD who are likely to experience kidney failure. A clinical trial in children is currently ongoing; however, valid prediction models to identify children likely to suffer kidney failure are lacking. Non-pharmacological interventions in this population also deserve further study.<\/jats:p>","DOI":"10.1007\/s00467-021-04974-4","type":"journal-article","created":{"date-parts":[[2021,3,7]],"date-time":"2021-03-07T02:02:32Z","timestamp":1615082552000},"page":"473-487","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["The wind of change in the management of autosomal dominant polycystic kidney disease in childhood"],"prefix":"10.1007","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1296-9081","authenticated-orcid":false,"given":"Charlotte","family":"Gimpel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten","family":"Bergmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Djalila","family":"Mekahli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,3,7]]},"reference":[{"key":"4974_CR1","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1053\/j.ajkd.2007.10.037","volume":"51","author":"BY Reed","year":"2008","unstructured":"Reed BY, McFann K, Bekheirnia MR, Nobkhthaghighi N, Masoumi A, Johnson AM, Shamshiraz AA, Kelleher CL, Schrier RW (2008) Variation in Age at ESRD in Autosomal Dominant Polycystic Kidney Disease. 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C.G. is supported by the German Federal Ministry of Research and Education (BMBF grant 01GM1903I). C.B. holds a part-time faculty appointment at the University of Freiburg in addition to his employment with the Limbach Group for which he heads and manages Limbach Genetics. His research lab receives support from the Deutsche Forschungsgemeinschaft (DFG) (BE 3910\/8-1 and BE 3910\/9-1,\u00a0and Collaborative Research Center SFB 1453) and the Federal Ministry of Education and Research (BMBF, 01GM1903I and 01GM1903G). D.M. is supported by the Research Foundation Flanders (FWO) (G0C8920N) and UZ Leuven (Belgium). D.M. (representing her institution KU Leuven) reports research grants and\/or participation in advisory boards for Otsuka, Galapagos and Genzyme, all outside the submitted work.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}