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

Kohl S, Avni FE, Boor P, Capone V, Clapp WL, De Palma D, Harris T, Heidet L, Hilger AC, Liapis H, Lilien M, Manzoni G, Montini G, Negrisolo S, Pierrat MJ, Raes A, Reutter HM, Schreuder MF, Weber S, Winyard PJD, Woolf AS, Schaefer F, Liebau MC (2022)

Publication Type: Journal article

Publication year: 2022

Journal

DOI: 10.1093/ndt/gfac207

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.

Authors with CRIS profile

Involved external institutions

Universität zu Köln Germany (DE) Universitätsklinikum Aachen (UKA) Germany (DE) Centre Hospitalier Régional Universitaire de Lille (CHRU de Lille) France (FR) Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico Italy (IT) University of Padua / Universita degli Studi di Padova Italy (IT) Universitätsklinikum Heidelberg Germany (DE) Radboud University Nijmegen Netherlands (NL) Philipps-Universität Marburg Germany (DE) University Hospital Ghent Belgium (BE) University of Manchester United Kingdom (GB) University College London (UCL) United Kingdom (GB) Polycystic Kidney Disease (PKD) Charity United Kingdom (GB) University Medical Centre Utrecht (UMC Utrecht) Netherlands (NL) Ludwig-Maximilians-Universität (LMU) Germany (DE) ASST dei Sette Laghi Italy (IT) Université de Paris France (FR) University of Florida United States (USA) (US)

How to cite

APA:

Kohl, S., Avni, F.E., Boor, P., Capone, V., Clapp, W.L., De Palma, D.,... Liebau, M.C. (2022). Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations. Nephrology Dialysis Transplantation. https://doi.org/10.1093/ndt/gfac207

MLA:

Kohl, Stefan, et al. "Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations." Nephrology Dialysis Transplantation (2022).

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