Inherited kidney disease and CAKUT are common causes of kidney failure requiring kidney replacement therapy : an ERA Registry study
Ortiz, Alberto (Universidad Autónoma de Madrid)
Kramer, A. (Amsterdam Public Health Research Institute)
Ariceta Iraola, Gema (Hospital Universitari Vall d'Hebron)
Rodríguez Arévalo, Olga Lucia (Universitat Politècnica de València)
Gjerstad, A.C. (Oslo University Hospital (Oslo, Noruega))
Santiuste, C. (Centro de Investigación Biomédica en Red de Epidemiología y Salud Pública)
Trujillo-Alemán, S. (Servicio Canario de la Salud)
Ferraro, Pietro Manuel (Università degli Studi di Verona)
Methven, S. (Aberdeen Royal Infirmary (Aberdeen, Escòcia))
Santamaría, Rafael (Hospital Universitario Reina Sofía (Còrdova, Espanya))
Naumovic, R. (University of Belgrade)
Resic, H. (Society of Nephrology and Dialysis of Bosnia and Herzegovina)
Hommel, K. (Holbaek Hospital)
Segelmark, M. (Skåne University Hospital (Suècia))
Ambühl, P.M. (Waid and Triemli City Hospital)
Sorensen, S. S. (University Hospital of Copenhagen)
Parmentier, C. (Armand-Trousseau Children's Hospital)
Vidal, E. (University Hospital of Padova (Pàdua, Itàlia))
Bakkaloglu, S.A. (Gazi University)
Plumb, L. (University of Bristol Medical School)
Palsson, R. (University of Iceland)
Kerschbaum, J. (Medical University Innsbruck)
ten Dam, M.A.G.J. (Canisius-Wilhelmina Hospital)
Stel, V.S. (Amsterdam Public Health Research Institute)
Jager, Kitty (Amsterdam Public Health Research Institute)
Torra Balcells, Roser (Institut de Recerca Sant Pau)
Universitat Autònoma de Barcelona. Departament de Medicina

Date:	2025
Abstract:	Background. Inherited kidney diseases (IKDs) and congenital anomalies of the kidney and urinary tract (CAKUT) are causes of kidney failure requiring kidney replacement therapy (KRT) that major renal registries usually amalgamate into the primary renal disease(PRD) category 'miscellaneous' or in the glomerulonephritis or pyelonephritis categories. This makes IKDs invisible (except for polycystic kidney disease) and may negatively influence the use of genetic testing, which may identify a cause for IKDs and some CAKUT. Methods. We re-examined the aetiology of KRT by composing a separate IKD and CAKUT PRD group using data from the European Renal Association (ERA) Registry. Results. In 2019, IKD-CAKUT was the fourth most common cause of kidney failure among incident KRT patients, accounting for 8. 9% of cases [IKD 7. 4% (including 5. 0% autosomal dominant polycystic kidney disease), CAKUT 1. 5%], behind diabetes (23. 0%), hypertension (14. 4%) and glomerulonephritis (10. 6%). IKD-CAKUT was the most common cause of kidney failure among patients <20 years of age (41. 0% of cases), but their incidence rate was highest among those ages 45-74 years (22. 5 per million age-related population). Among prevalent KRT patients, IKD-CAKUT (18. 5%) and glomerulonephritis (18. 7%) were the two most common causes of kidney failure overall, while IKD-CAKUT was the most common cause in women (21. 6%) and in patients <45 years of age (29. 1%). Conclusion. IKD and CAKUT are common causes of kidney failure among KRT patients. Distinct categorization of IKD and CAKUT better characterizes the epidemiology of the causes of chronic kidney disease (CKD) and highlights the importance of genetic testing in the diagnostic workup of CKD.
Grants:	Instituto de Salud Carlos III PMP21/00109 Instituto de Salud Carlos III PI22/00361 Ministerio de Economía y Competitividad RD16/0009
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	CAKUT ; Aetiology ; Epidemiology ; Genetic kidney disease ; Inherited kidney disease ; Kidney failure ; Kidney replacement therapy
Published in:	Nephrology Dialysis Transplantation, Vol. 40 Núm. 5 (january 2025) , p. 1020-1031, ISSN 1460-2385

DOI: 10.1093/ndt/gfae240
PMID: 39508350

12 p, 2.0 MB

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