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

Subject:         DOI: 10.1093/ndt/gfae240 Nephrology Dialysis Transplantation, 40, 5, 6-11-2024. Abstract: 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.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.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%).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.


Keywords:        CAKUT, epidemiology, aetiology, genetic kidney disease, inherited kidney disease, kidney failure, kidney replacement therapy
Author:          Ortiz Alberto, Kramer Anneke, Ariceta Gema, Rodríguez Arévalo Olga L., Gjerstad Ann C., Santiuste Carmen, Trujillo-Alemán Sara, Ferraro Pietro Manuel, Methven Shona, Santamaría Rafael, Naumovic Radomir, Resic Halima, Hommel Kristine, Segelmark Mårten, Ambühl Patrice M., Sorensen Søren S., Parmentier Cyrielle, Vidal Enrico, Bakkaloglu Sevcan A., Plumb Lucy, Palsson Runolfur, Kerschbaum Julia, ten Dam Marc A. G. J., Stel Vianda S., Jager Kitty J., Torra Roser
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     Item: 
      Title: INTRODUCTION
      Destination: section*.2
     Item: 
      Title: MATERIALS AND METHODS
      Destination: section*.4
      Children: 
       Item: 
        Title: Patient population
        Destination: section*.6
       Item: 
        Title: Primary renal disease groups
        Destination: section*.8
       Item: 
        Title: Incidence and prevalence analyses
        Destination: section*.10
       Item: 
        Title: Time trend analyses
        Destination: section*.12
     Item: 
      Title: RESULTS
      Destination: section*.14
      Children: 
       Item: 
        Title: IKD and CAKUT combined was the fourth most common cause of incident KRT
        Destination: section*.16
       Item: 
        Title: IKD and CAKUT combined were the most common cause of incident KRT in patients <20 years of age, but the incidence rate peaked at 45-74 years
        Destination: section*.18
       Item: 
        Title: IKD and CAKUT combined contributed more to the incidence of KRT in women than in men
        Destination: section*.20
       Item: 
        Title: Specific IKD and CAKUT causes of incident KRT
        Destination: section*.22
       Item: 
        Title: IKD and CAKUT combined was the most common cause of prevalent KRT in women
        Destination: section*.24
       Item: 
        Title: Specific IKD and CAKUT causes in prevalent KRT
        Destination: section*.26
       Item: 
        Title: Increasing incidence and prevalence of KRT for IKD but not CAKUT
        Destination: section*.28
     Item: 
      Title: DISCUSSION
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     Item: 
      Title: SUPPLEMENTARY DATA
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     Item: 
      Title: ACKNOWLEDGEMENTS
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      Title: FUNDING
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   Info: 
    Title: Inherited kidney disease and CAKUT are common causes of kidney failure requiring kidney replacement therapy: an ERA Registry study

    Author: Ortiz Alberto, Kramer Anneke, Ariceta Gema, Rodríguez Arévalo Olga L., Gjerstad Ann C., Santiuste Carmen, Trujillo-Alemán Sara, Ferraro Pietro Manuel, Methven Shona, Santamaría Rafael, Naumovic Radomir, Resic Halima, Hommel Kristine, Segelmark Mårten, Ambühl Patrice M., Sorensen Søren S., Parmentier Cyrielle, Vidal Enrico, Bakkaloglu Sevcan A., Plumb Lucy, Palsson Runolfur, Kerschbaum Julia, ten Dam Marc A. G. J., Stel Vianda S., Jager Kitty J., Torra Roser
    Subject: DOI: 10.1093/ndt/gfae240 Nephrology Dialysis Transplantation, 40, 5, 6-11-2024. Abstract: 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 &#x2018;miscellaneous&#x2019; 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.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.In 2019, IKD-CAKUT was the fourth most common cause of kidney failure among incident KRT patients, accounting for 8.9&#x0025; of cases &#x005B;IKD 7.4&#x0025; (including 5.0&#x0025; autosomal dominant polycystic kidney disease), CAKUT 1.5&#x0025;&#x005D;, behind diabetes (23.0&#x0025;), hypertension (14.4&#x0025;) and glomerulonephritis (10.6&#x0025;). IKD-CAKUT was the most common cause of kidney failure among patients &#x003C;20&#x00A0;years of age (41.0&#x0025; of cases), but their incidence rate was highest among those ages 45&#x2013;74&#x00A0;years (22.5 per million age-related population). Among prevalent KRT patients, IKD-CAKUT (18.5&#x0025;) and glomerulonephritis (18.7&#x0025;) were the two most common causes of kidney failure overall, while IKD-CAKUT was the most common cause in women (21.6&#x0025;) and in patients &#x003C;45&#x00A0;years of age (29.1&#x0025;).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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               <rdf:li xml:lang="x-default">© The Author(s) 2024. Published by Oxford University Press on behalf of the ERA. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com</rdf:li>
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               <rdf:li>Rodríguez Arévalo Olga L.</rdf:li>
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               <rdf:li>Ambühl Patrice M.</rdf:li>
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               <rdf:li>ten Dam Marc A. G. J.</rdf:li>
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               <rdf:li xml:lang="x-default">DOI: 10.1093/ndt/gfae240 Nephrology Dialysis Transplantation, 40, 5, 6-11-2024. Abstract: 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&#xA;(PRD) category &amp;#x2018;miscellaneous&amp;#x2019; 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.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.In 2019, IKD-CAKUT was the fourth most common cause of kidney failure among incident KRT patients, accounting for 8.9&amp;#x0025; of cases &amp;#x005B;IKD 7.4&amp;#x0025; (including 5.0&amp;#x0025; autosomal dominant polycystic kidney disease), CAKUT 1.5&amp;#x0025;&amp;#x005D;, behind diabetes (23.0&amp;#x0025;), hypertension (14.4&amp;#x0025;) and glomerulonephritis (10.6&amp;#x0025;). IKD-CAKUT was the most common cause of kidney failure among patients &amp;#x003C;20&amp;#x00A0;years of age (41.0&amp;#x0025; of cases), but their incidence rate was highest among those ages 45&amp;#x2013;74&amp;#x00A0;years (22.5 per million age-related population). Among prevalent KRT patients, IKD-CAKUT (18.5&amp;#x0025;) and glomerulonephritis (18.7&amp;#x0025;) were the two most common causes of kidney failure overall, while IKD-CAKUT was the most common cause in women (21.6&amp;#x0025;) and in patients &amp;#x003C;45&amp;#x00A0;years of age (29.1&amp;#x0025;).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.&#xA;&#xA;</rdf:li>
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