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).
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Gabow PA. Autosomal dominant polycystic kidney disease. N Engl J Med. 1993;329:332-342.
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Kelleher CL, McFann KK, Johnson AM, et al. Characteristics of hypertension in young adults with autosomal dominant polycystic kidney disease compared with the general U.S. population. Am J Hypertens. 2004;17:1029-1034.
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Torres VE, Holley KE, Offord KP. Epidemiology. In: Grantham JJ, Gardner KD, eds. Problems in diagnosis and management of polycystic kidney disease. Kansas City, MO: PKR Foundation; 1985:49-69.
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.
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U.S. Renal Data System. USRDS 2007 annual data report. B: Prevalence of reported ESRD. Bethesda, MD: NIH, National Institute of Diabetes and Digestive and Kidney Diseases; 2007:17.
http://www.usrds.org/2007/ref/B_prevalence_07.pdf
In Copenhagen, prevalence is estimated to be 1 in 1000;
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Dalgaard OZ. Bilateral polycystic disease of the kidneys: a follow-up of two hundred and eighty-four patients and their families. Acta Med Scand Suppl. 1957;328:1-255.
http://www.ncbi.nlm.nih.gov/pubmed/13469269?tool=bestpractice.com
however, lower figures have been reported in France (1 per 1111),
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Simon P, Le Goff JY, Ang KS, et al. Epidemiologic data, clinical and prognostic features of autosomal dominant polycystic kidney disease in a French region [in French]. Nephrologie. 1996;17:123-130.
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Wales (1 per 2459),
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Davies F, Coles GA, Harper PS, et al. Polycystic kidney disease re-evaluated: a population-based study. Q J Med. 1991;79:477-485.
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and Japan (1 per 4033).
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Higashihara E, Nutahara K, Kojima M, et al. Prevalence and renal prognosis of diagnosed autosomal dominant polycystic kidney disease in Japan. Nephron. 1998;80:421-427.
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In the Seychelles, prevalence in the white population was found to be 1 in 544, but the disease was rare in black people.
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Yersin C, Bovet P, Wauters JP, et al. Frequency and impact of autosomal dominant polycystic kidney disease in the Seychelles (Indian Ocean). Nephrol Dial Transplant. 1997;12:2069-2074.
http://ndt.oxfordjournals.org/content/12/10/2069.full.pdf+html
http://www.ncbi.nlm.nih.gov/pubmed/9351067?tool=bestpractice.com
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;
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Stengel B, Billon S, Van Dijk PC, et al. Trends in the incidence of renal replacement therapy for end-stage renal disease in Europe, 1990-1999. Nephrol Dial Transplant. 2003;18:1824-1833.
http://ndt.oxfordjournals.org/content/18/9/1824.full
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and in Japan, 5.6 million for men (1999-2000) and 4 million women.
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Wakai K, Nakai S, Kikuchi K, et al. Trends in incidence of end-stage renal disease in Japan, 1983-2000: age-adjusted and age-specific rates by gender and cause. Nephrol Dial Transplant. 2004;19:2044-2052.
http://ndt.oxfordjournals.org/content/19/8/2044.full
http://www.ncbi.nlm.nih.gov/pubmed/15173379?tool=bestpractice.com
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.
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Cole BR, Conley SB, Stapleton FB. Polycystic kidney disease in the first year of life. J Pediatr. 1987;111:693-699.
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Kääriäinen H. Polycystic kidney disease in children: a genetic and epidemiological study of 82 Finnish patients. J Med Genet. 1987;24:474-481.
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