Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations
Kohl, Stefan; Avni, Fred E; Boor, Peter; Capone, Valentina; Clapp, William L; De Palma, Diego; Harris, Tess; Heidet, Laurence; Hilger, Alina C; Liapis, Helen; Lilien, Marc; Manzoni, Gianantonio; Montini, Giovanni; Negrisolo, Susanna; Pierrat, Marie-Jeanne; Raes, Ann; Reutter, Heiko; Schreuder, Michiel F; Weber, Stefanie; Winyard, Paul J D; Woolf, Adrian S; Schaefer, Franz; Liebau, Max C
(2022) Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association, volume 37, issue 12, pp. 2351 - 2362
(Article)
Abstract
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
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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.
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Keywords: chronic renal failure, chronic renal insufficiency, guidelines, pediatrics, ultrasonography, Nephrology, Transplantation
ISSN: 0931-0509
Publisher: Oxford University Press
Note: Publisher Copyright: © 2022 The Author(s). Published by Oxford University Press on behalf of the ERA.
(Peer reviewed)