Alport's syndrome as a cause of renal failure in Europe

Abstract

We studied the geographical distribution, male to female ratio, and age at the start of renal replacement therapy (RRT) for end-stage renal failure (ESRF) in 600 patients with hereditary nephritis with nerve deafness (Alport's syndrome) reported to the European Dialysis and Transplant Association Registry since 1975. Annual age- and sex-specific acceptance rates for RRT showed a variable peak incidence according to country, ranging between, 0 and 2.4 patients per million population in males aged 15-24 years, but with only about half this incidence in females. In Scandinavian countries there were very few females who started RRT, and males were older than in the rest of Europe. The overall male to female ratio was 4:1. The median age at the start of RRT was: males (n = 479) 24.3 years (1st quartile 19.5 years; 3rd quartile 31.5 years); females (n = 121) 31.5 years (1st quartile 23.0 years; 3rd quartile 43.2 years). Our study provided confirmation that males reach ESRF earlier than females. In addition, we detected previously unrecognized geographical differences.