Prevalence and renal prognosis of diagnosed autosomal dominant polycystic kidney disease in Japan

The prevalence and renal prognosis of diagnosed autosomal dominant polycyst ic kidney disease (ADPKD) in Japan were estimated. Hospital-based nationwid e surveys were conducted in 1995. The number of ADPKD patients who visited hospitals but were not on chronic dialysis was estimated to be 10,000 (95% confidence interval: 8,200- 11,900) and that of ADPKD patients on dialysis was 4,590, yielding a prevalence of ADPKD of 117 per million population at the end of 1994 (95% confidence interval: 102-1.32). The prevalence increas ed with age and reached a peak value of 261 per million population at the a ge group of 55-59 years. The rate of end-stage renal disease among living p atients was calculated based on the assumption that the prevalence of ADPKD in the population under the age of 55 years was 261 per million population . The rate of end-stage renal disease increased with the progression of the patients' age, reaching 49% at the age of 65-69 years and declining therea fter. Conclusion: The hospital-based prevalence of ADPKD is lower than the autopsy-based prevalence, suggesting that a fairly large number of these pa tients do not receive medical care in their lifetime. The probability of en dstage renal disease is at most 50% among ADPKD patients who visit a hospit al.