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International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people

Research output: Contribution to journalReview article

Charlotte Gimpel, Carsten Bergmann, Detlef Bockenhauer, Luc Breysem, Melissa A. Cadnapaphornchai, Metin Cetiner, Jan Dudley, Francesco Emma, Martin Konrad, Tess Harris, Peter C. Harris, Jens König, Max C. Liebau, Matko Marlais, Djalila Mekahli, Alison M. Metcalfe, Jun Oh, Ronald D. Perrone, Manish D. Sinha, Andrea Titieni & 4 more Roser Torra, Stefanie Weber, Paul J.D. Winyard, Franz Schaefer

Original languageEnglish
Pages (from-to)713-726
Number of pages14
JournalNature Reviews Nephrology
Issue number11
Publication statusPublished - 1 Nov 2019

King's Authors


These recommendations were systematically developed on behalf of the Network for Early Onset Cystic Kidney Disease (NEOCYST) by an international group of experts in autosomal dominant polycystic kidney disease (ADPKD) from paediatric and adult nephrology, human genetics, paediatric radiology and ethics specialties together with patient representatives. They have been endorsed by the International Pediatric Nephrology Association (IPNA) and the European Society of Paediatric Nephrology (ESPN). For asymptomatic minors at risk of ADPKD, ongoing surveillance (repeated screening for treatable disease manifestations without diagnostic testing) or immediate diagnostic screening are equally valid clinical approaches. Ultrasonography is the current radiological method of choice for screening. Sonographic detection of one or more cysts in an at-risk child is highly suggestive of ADPKD, but a negative scan cannot rule out ADPKD in childhood. Genetic testing is recommended for infants with very-early-onset symptomatic disease and for children with a negative family history and progressive disease. Children with a positive family history and either confirmed or unknown disease status should be monitored for hypertension (preferably by ambulatory blood pressure monitoring) and albuminuria. Currently, vasopressin antagonists should not be offered routinely but off-label use can be considered in selected children. No consensus was reached on the use of statins, but mTOR inhibitors and somatostatin analogues are not recommended. Children with ADPKD should be strongly encouraged to achieve the low dietary salt intake that is recommended for all children.

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