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Inherited kidney disease and CAKUT are common causes of kidney failure requiring kidney replacement therapy : an ERA Registry study

Ortiz, Alberto ; Kramer, Anneke ; Ariceta, Gema ; Rodríguez Arévalo, Olga L. ; Gjerstad, Ann C. ; Santiuste, Carmen ; Trujillo-Alemán, Sara ; Ferraro, Pietro Manuel ; Methven, Shona and Santamaría, Rafael , et al. (2025) In Nephrology Dialysis Transplantation 40(5). p.1020-1031
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... (More)

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.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
aetiology, CAKUT, epidemiology, genetic kidney disease, inherited kidney disease, kidney failure, kidney replacement therapy
in
Nephrology Dialysis Transplantation
volume
40
issue
5
pages
12 pages
publisher
Oxford University Press
external identifiers
  • pmid:39508350
  • scopus:105003878471
ISSN
0931-0509
DOI
10.1093/ndt/gfae240
language
English
LU publication?
yes
additional info
Publisher Copyright: © The Author(s) 2024. Published by Oxford University Press on behalf of the ERA.
id
74be0b6e-b94d-420f-b99e-ac25118ba48b
date added to LUP
2025-08-05 15:22:27
date last changed
2025-08-06 03:00:03
@article{74be0b6e-b94d-420f-b99e-ac25118ba48b,
  abstract     = {{<p>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 &lt;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 &lt;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.</p>}},
  author       = {{Ortiz, Alberto and Kramer, Anneke and Ariceta, Gema and Rodríguez Arévalo, Olga L. and Gjerstad, Ann C. and Santiuste, Carmen and Trujillo-Alemán, Sara and Ferraro, Pietro Manuel and Methven, Shona and Santamaría, Rafael and Naumovic, Radomir and Resic, Halima and Hommel, Kristine and Segelmark, Mårten and Ambühl, Patrice M. and Sorensen, Søren S. and Parmentier, Cyrielle and Vidal, Enrico and Bakkaloglu, Sevcan A. and Plumb, Lucy and Palsson, Runolfur and Kerschbaum, Julia and ten Dam, Marc A.G.J. and Stel, Vianda S. and Jager, Kitty J. and Torra, Roser}},
  issn         = {{0931-0509}},
  keywords     = {{aetiology; CAKUT; epidemiology; genetic kidney disease; inherited kidney disease; kidney failure; kidney replacement therapy}},
  language     = {{eng}},
  month        = {{05}},
  number       = {{5}},
  pages        = {{1020--1031}},
  publisher    = {{Oxford University Press}},
  series       = {{Nephrology Dialysis Transplantation}},
  title        = {{Inherited kidney disease and CAKUT are common causes of kidney failure requiring kidney replacement therapy : an ERA Registry study}},
  url          = {{http://dx.doi.org/10.1093/ndt/gfae240}},
  doi          = {{10.1093/ndt/gfae240}},
  volume       = {{40}},
  year         = {{2025}},
}