Higashihara E, Nutahara K, Kojima M, Tamakoshi A, Yoshiyuki O, Sakai H, Kurokawa K
Department of Urology, Kyorin University School of Medicine, Tokyo, Japan.
Nephron. 1998 Dec;80(4):421-7. doi: 10.1159/000045214.
The prevalence and renal prognosis of diagnosed autosomal dominant polycystic kidney disease (ADPKD) in Japan were estimated. Hospital-based nationwide 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: 102-132). The prevalence increased with age and reached a peak value of 261 per million population at the age group of 55-59 years. The rate of end-stage renal disease among living patients 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 thereafter.
The hospital-based prevalence of ADPKD is lower than the autopsy-based prevalence, suggesting that a fairly large number of these patients do not receive medical care in their lifetime. The probability of end-stage renal disease is at most 50% among ADPKD patients who visit a hospital.
对日本确诊的常染色体显性多囊肾病（ADPKD）的患病率及肾脏预后进行了评估。1995年开展了基于医院的全国性调查。据估计，前往医院就诊但未接受慢性透析的ADPKD患者数量为10,000例（95%置信区间：8,200 - 11,900），接受透析的ADPKD患者数量为4,590例，1994年末ADPKD的患病率为每百万人口117例（95%置信区间：102 - 132）。患病率随年龄增长而升高，在55 - 59岁年龄组达到峰值，为每百万人口261例。基于55岁以下人群中ADPKD患病率为每百万人口261例这一假设，计算了存活患者中终末期肾病的发生率。终末期肾病的发生率随患者年龄增长而升高，在65 - 69岁时达到49%，此后下降。
基于医院的ADPKD患病率低于基于尸检的患病率，这表明相当一部分此类患者一生中未接受医疗护理。在前往医院就诊的ADPKD患者中，终末期肾病的概率最高为50%。
