Chan K W
Department of Pathology, University of Hong Kong.
Pathology. 1993 Jul;25(3):229-32. doi: 10.3109/00313029309066577.
To appraise the frequency, clinical features and prognosis of adult polycystic kidney disease (APKD) in Hong Kong, 41 Chinese patients who had an autopsy diagnosis of APKD were studied retrospectively. The APKD patients constituted 0.295% of 13,890 (or one in 339) autopsies performed during the study period (1964-1991). The frequency of APKD in autopsied populations in Western countries varies from one in 261 to one in 1,019 with an average of one in 503. Nineteen (46%) patients reached end-stage renal disease (ESRD) at the time of death. Ruptured berry aneurysm caused death in 3 patients while 3 others had spontaneous intracerebral hemorrhage. Left ventricular hypertrophy was found in 24 (59%) patients. Polycystic liver was present in 26 (63%) patients. This frequency was higher than the 4% to 60% (average 47%) reported in similar studies conducted in Western countries. The probability of patients being alive and without ESRD, estimated using a life table analysis, was 83%, 54%, 32% and 12% by the age of 40, 50, 60 and 70 respectively. No difference in frequency, clinical features and survival between male and female APKD patients could be demonstrated.
为评估香港成人多囊肾病（APKD）的发病率、临床特征及预后，我们对41例经尸检诊断为APKD的中国患者进行了回顾性研究。在研究期间（1964年至1991年）进行的13,890例尸检中，APKD患者占0.295%（即339例中有1例）。西方国家尸检人群中APKD的发病率从261例中有1例至1019例中有1例不等，平均为503例中有1例。19例（46%）患者在死亡时已发展至终末期肾病（ESRD）。3例患者因破裂的浆果样动脉瘤死亡，另有3例发生自发性脑出血。24例（59%）患者发现左心室肥厚。26例（63%）患者存在多囊肝。这一比例高于西方国家类似研究报道的4%至60%（平均47%）。使用生命表分析估计，40岁、50岁、60岁和70岁时患者存活且无ESRD的概率分别为83%、54%、32%和12%。未发现男性和女性APKD患者在发病率、临床特征及生存率方面存在差异。
