Autosomal-dominant PKD (ADPKD) occurs worldwide and in all races. Prevalence in the US is estimated to be between 1 in 400 (including observed and estimated autopsy cases) and 1 in 1000 (clinically diagnosed cases only). [6] [7] [8] [9] [10] Approximately 600,000 Americans are affected with the disease, with over 2000 patients starting renal replacement therapy reported due to cystic kidney disease every year. [11]

In Copenhagen, prevalence is estimated to be 1 in 1000; [7] however, lower figures have been reported in France (1 per 1111), [12] Wales (1 per 2459), [13] and Japan (1 per 4033). [14] In the Seychelles, prevalence in the white population was found to be 1 in 544, but the disease was rare in black people. [15]

ESRD due to ADPKD is less common among black people than among white people because of the higher incidence of ESRD from other causes in black people. Annual US incidence rates for ESRD caused by ADPKD (1998-2001) was 8.7 million in men and 6.9 million for women; in Europe (1998-1999) the rates for men were 7.8 million and 6 million for women; [16] and in Japan, 5.6 million for men (1999-2000) and 4 million women. [17] Age-adjusted sex ratios greater than unity (1.2 to 1.3) suggest a more progressive disease in men than in women.

Autosomal-recessive PKD is far less common with an incidence of 1 in 10,000 to 1 in 40,000. [18] [19]

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