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Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations.


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 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.

SUBMITTER: Kohl S 

PROVIDER: S-EPMC9681917 | biostudies-literature | 2022 Nov

REPOSITORIES: biostudies-literature

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Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations.

Kohl Stefan S   Avni Fred E FE   Boor Peter P   Capone Valentina V   Clapp William L WL   De Palma Diego D   Harris Tess T   Heidet Laurence L   Hilger Alina C AC   Liapis Helen H   Lilien Marc M   Manzoni Gianantonio G   Montini Giovanni G   Negrisolo Susanna S   Pierrat Marie-Jeanne MJ   Raes Ann A   Reutter Heiko H   Schreuder Michiel F MF   Weber Stefanie S   Winyard Paul J D PJD   Woolf Adrian S AS   Schaefer Franz F   Liebau Max C MC  

Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 20221101 12


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 therapie  ...[more]

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