Universität zu Köln

Gimpel, Charlotte ORCID: 0000-0003-1296-9081, Bergmann, Carsten, Bockenhauer, Detlef ORCID: 0000-0001-5878-941X, Breysem, Luc, Cadnapaphornchai, Melissa A., Cetiner, Metin, Dudley, Jan, Emma, Francesco, Konrad, Martin, Harris, Tess, Harris, Peter C., Koenig, Jens, Liebau, Max C., Marlais, Matko ORCID: 0000-0001-7503-7893, Mekahli, Djalila, Metcalfe, Alison M., Oh, Jun, Perrone, Ronald D., Sinha, Manish D., Titieni, Andrea, Torra, Roser ORCID: 0000-0001-8714-2332, Weber, Stefanie, Winyard, Paul J. D. and Schaefer, Franz (2019). International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. Nat. Rev. Nephrol., 15 (11). S. 713 - 727. NEW YORK: NATURE PUBLISHING GROUP. ISSN 1759-507X

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Abstract

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.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Gimpel, Charlotte UNSPECIFIED orcid.org/0000-0003-1296-9081 UNSPECIFIED Bergmann, Carsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Bockenhauer, Detlef UNSPECIFIED orcid.org/0000-0001-5878-941X UNSPECIFIED Breysem, Luc UNSPECIFIED UNSPECIFIED UNSPECIFIED Cadnapaphornchai, Melissa A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Cetiner, Metin UNSPECIFIED UNSPECIFIED UNSPECIFIED Dudley, Jan UNSPECIFIED UNSPECIFIED UNSPECIFIED Emma, Francesco UNSPECIFIED UNSPECIFIED UNSPECIFIED Konrad, Martin UNSPECIFIED UNSPECIFIED UNSPECIFIED Harris, Tess UNSPECIFIED UNSPECIFIED UNSPECIFIED Harris, Peter C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Koenig, Jens UNSPECIFIED UNSPECIFIED UNSPECIFIED Liebau, Max C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Marlais, Matko UNSPECIFIED orcid.org/0000-0001-7503-7893 UNSPECIFIED Mekahli, Djalila UNSPECIFIED UNSPECIFIED UNSPECIFIED Metcalfe, Alison M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Oh, Jun UNSPECIFIED UNSPECIFIED UNSPECIFIED Perrone, Ronald D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sinha, Manish D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Titieni, Andrea UNSPECIFIED UNSPECIFIED UNSPECIFIED Torra, Roser UNSPECIFIED orcid.org/0000-0001-8714-2332 UNSPECIFIED Weber, Stefanie UNSPECIFIED UNSPECIFIED UNSPECIFIED Winyard, Paul J. D. UNSPECIFIED UNSPECIFIED UNSPECIFIED Schaefer, Franz UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-129844
DOI:	10.1038/s41581-019-0155-2
Journal or Publication Title:	Nat. Rev. Nephrol.
Volume:	15
Number:	11
Page Range:	S. 713 - 727
Date:	2019
Publisher:	NATURE PUBLISHING GROUP
Place of Publication:	NEW YORK
ISSN:	1759-507X
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language AMBULATORY BLOOD-PRESSURE; NONSTEROIDAL ANTIINFLAMMATORY DRUGS; INHERITED GENETIC CONDITIONS; LONG-ACTING SOMATOSTATIN; MITRAL-VALVE PROLAPSE; LEFT-VENTRICULAR MASS; CHRONIC-RENAL-FAILURE; FOLLOW-UP; GUIDELINE MANAGEMENT; FAMILY COMMUNICATION Multiple languages Urology & Nephrology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/12984