TY  - INPR
PB  - Nature Publishing Group
N2  - 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.
UR  - https://doi.org/10.1038/s41581-019-0155-2
Y1  - 2019/05/22/
JF  - Nature Reviews Nephrology
N1  - This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article?s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article?s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.
TI  - International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people
AV  - public
ID  - discovery10075035
SN  - 1759-5061
A1  - Gimpel, C
A1  - Bergmann, C
A1  - Bockenhauer, D
A1  - Breysem, L
A1  - Cadnapaphornchai, MA
A1  - Cetiner, M
A1  - Dudley, J
A1  - Emma, F
A1  - Konrad, M
A1  - Harris, T
A1  - Harris, PC
A1  - König, J
A1  - Liebau, MC
A1  - Marlais, M
A1  - Mekahli, D
A1  - Metcalfe, AM
A1  - Oh, J
A1  - Perrone, RD
A1  - Sinha, MD
A1  - Titieni, A
A1  - Torra, R
A1  - Weber, S
A1  - Winyard, PJD
A1  - Schaefer, F
ER  -