International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people
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International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people. / Gimpel, Charlotte; Bergmann, Carsten; Bockenhauer, Detlef; Breysem, Luc; Cadnapaphornchai, Melissa A; Cetiner, Metin; Dudley, Jan; Emma, Francesco; Konrad, Martin; Harris, Tess; Harris, Peter C; König, Jens; Liebau, Max C; Marlais, Matko; Mekahli, Djalila; Metcalfe, Alison M; Oh, Jun; Perrone, Ronald D; Sinha, Manish D; Titieni, Andrea; Torra, Roser; Weber, Stefanie; Winyard, Paul J D; Schaefer, Franz.
in: NAT REV NEPHROL, Jahrgang 15, Nr. 11, 11.2019, S. 713-726.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Review › Forschung
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TY - JOUR
T1 - International consensus statement on the diagnosis and management of autosomal dominant polycystic kidney disease in children and young people
AU - Gimpel, Charlotte
AU - Bergmann, Carsten
AU - Bockenhauer, Detlef
AU - Breysem, Luc
AU - Cadnapaphornchai, Melissa A
AU - Cetiner, Metin
AU - Dudley, Jan
AU - Emma, Francesco
AU - Konrad, Martin
AU - Harris, Tess
AU - Harris, Peter C
AU - König, Jens
AU - Liebau, Max C
AU - Marlais, Matko
AU - Mekahli, Djalila
AU - Metcalfe, Alison M
AU - Oh, Jun
AU - Perrone, Ronald D
AU - Sinha, Manish D
AU - Titieni, Andrea
AU - Torra, Roser
AU - Weber, Stefanie
AU - Winyard, Paul J D
AU - Schaefer, Franz
PY - 2019/11
Y1 - 2019/11
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.
AB - 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.
KW - Adolescent
KW - Child
KW - Combined Modality Therapy
KW - Directive Counseling
KW - Humans
KW - Mass Screening
KW - Polycystic Kidney, Autosomal Dominant/complications
KW - Referral and Consultation
KW - Risk Assessment
U2 - 10.1038/s41581-019-0155-2
DO - 10.1038/s41581-019-0155-2
M3 - SCORING: Review article
C2 - 31118499
VL - 15
SP - 713
EP - 726
JO - NAT REV NEPHROL
JF - NAT REV NEPHROL
SN - 1759-5061
IS - 11
ER -