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

Alberto Ortiz1,2, Anneke Kramer3,4, Gema Ariceta5,6

  • 1Department of Nephrology and Hypertension, IIS-Fundacion Jimenez Diaz UAM, Madrid, Spain.

Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association
|November 7, 2024
PubMed

Insights

Inherited kidney diseases (IKDs) and congenital anomalies of the kidney and urinary tract (CAKUT) are significant causes of kidney failure. Reclassifying these conditions improves understanding of chronic kidney disease (CKD) causes and emphasizes genetic testing.

Area of Science:

  • Nephrology
  • Genetics
  • Epidemiology

Background:

  • Inherited kidney diseases (IKDs) and congenital anomalies of the kidney and urinary tract (CAKUT) are often miscategorized in renal registries.
  • This miscategorization obscures the true prevalence of IKDs and may hinder the use of genetic testing for diagnosis.

Purpose of the Study:

  • To re-evaluate the etiology of kidney replacement therapy (KRT) by creating distinct categories for IKD and CAKUT.
  • To improve the characterization of chronic kidney disease (CKD) causes and highlight the role of genetic testing.

Main Methods:

  • Utilized data from the European Renal Association (ERA) Registry.
  • Composed separate primary renal disease (PRD) groups for IKD and CAKUT to analyze KRT aetiology.

Main Results:

  • In 2019, IKD-CAKUT was the fourth leading cause of kidney failure (8.9%), and the most common in patients under 20 years old.
  • Among prevalent KRT patients, IKD-CAKUT (18.5%) was a leading cause, particularly in women and individuals under 45.
  • IKD-CAKUT incidence was highest in the 45-74 age group (22.5 per million population).

Conclusions:

  • IKD and CAKUT are substantial contributors to kidney failure requiring KRT.
  • Distinct categorization of IKD and CAKUT provides a more accurate epidemiological picture of CKD.
  • This reclassification underscores the importance of genetic testing in diagnosing CKD.
Abstract

Keywords:
CAKUTaetiologyepidemiologygenetic kidney diseaseinherited kidney diseasekidney failurekidney replacement therapy

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