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International audienceDozens of inherited diseases affect the kidney; collectively, they account for at least 10% of patients with end-stage renal disease in Europe [1]. The underlying genetic defects may affect all structures and cell types of the nephron and may therefore compromise all aspects of kidney function. In addition, extra-renal involvement is frequently associated. In terms of frequency, inherited kidney disorders vary from relatively frequent diseases, such as autosomal dominant polycystic kidney disease (ADPKD, that arguably affects one in every 1000 person), to 'rare' diseases that, by definition, affect less than five persons in every 10 000

The ERA-EDTA Working Group on inherited kidney disorders