TY - JOUR
T1 - Definition, diagnosis and clinical management of non-obstructive kidney dysplasia
T2 - a consensus statement by the ERKNet Working Group on Kidney Malformations
AU - Kohl, Stefan
AU - Avni, Fred E
AU - Boor, Peter
AU - Capone, Valentina
AU - Clapp, William L
AU - De Palma, Diego
AU - Harris, Tess
AU - Heidet, Laurence
AU - Hilger, Alina C
AU - Liapis, Helen
AU - Lilien, Marc
AU - Manzoni, Gianantonio
AU - Montini, Giovanni
AU - Negrisolo, Susanna
AU - Pierrat, Marie-Jeanne
AU - Raes, Ann
AU - Reutter, Heiko
AU - Schreuder, Michiel F
AU - Weber, Stefanie
AU - Winyard, Paul J D
AU - Woolf, Adrian S
AU - Schaefer, Franz
AU - Liebau, Max C
N1 - Publisher Copyright:
© 2022 The Author(s). Published by Oxford University Press on behalf of the ERA.
PY - 2022/12/1
Y1 - 2022/12/1
N2 - Kidney dysplasia is one of the most frequent causes of chronic kidney failure in children. While dysplasia is a histological diagnosis, the term 'kidney dysplasia' is frequently used in daily clinical life without histopathological confirmation. Clinical parameters of kidney dysplasia have not been clearly defined, leading to imprecise communication amongst healthcare professionals and patients. This lack of consensus hampers precise disease understanding and the development of specific therapies. Based on a structured literature search, we here suggest a common basis for clinical, imaging, genetic, pathological and basic science aspects of non-obstructive kidney dysplasia associated with functional kidney impairment. We propose to accept hallmark sonographic findings as surrogate parameters defining a clinical diagnosis of dysplastic kidneys. We suggest differentiated clinical follow-up plans for children with kidney dysplasia and summarize established monogenic causes for non-obstructive kidney dysplasia. Finally, we point out and discuss research gaps in the field.
AB - Kidney dysplasia is one of the most frequent causes of chronic kidney failure in children. While dysplasia is a histological diagnosis, the term 'kidney dysplasia' is frequently used in daily clinical life without histopathological confirmation. Clinical parameters of kidney dysplasia have not been clearly defined, leading to imprecise communication amongst healthcare professionals and patients. This lack of consensus hampers precise disease understanding and the development of specific therapies. Based on a structured literature search, we here suggest a common basis for clinical, imaging, genetic, pathological and basic science aspects of non-obstructive kidney dysplasia associated with functional kidney impairment. We propose to accept hallmark sonographic findings as surrogate parameters defining a clinical diagnosis of dysplastic kidneys. We suggest differentiated clinical follow-up plans for children with kidney dysplasia and summarize established monogenic causes for non-obstructive kidney dysplasia. Finally, we point out and discuss research gaps in the field.
KW - chronic renal failure
KW - chronic renal insufficiency
KW - guidelines
KW - pediatrics
KW - ultrasonography
UR - http://www.scopus.com/inward/record.url?scp=85152683222&partnerID=8YFLogxK
U2 - 10.1093/ndt/gfac207
DO - 10.1093/ndt/gfac207
M3 - Article
C2 - 35772019
SN - 0931-0509
VL - 37
SP - 2351
EP - 2362
JO - Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
JF - Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
IS - 12
ER -