成人多囊肾病肉眼血尿的临床意义

Clinical significance of gross hematuria in autosomal dominant polycystic kidney patients

目的 探讨成人多囊肾病 (ADPKD)肉眼血尿的临床意义。方法 分析总结 4 98例ADPKD患者肉眼血尿的发生率、发作的相关因素及其与临床病程和生存时间的相关性。结果 本组ADPKD患者肉眼血尿的发生率为 38.4 %。首次发作肉眼血尿的平均年龄为 32岁。肉眼血尿 5次以下和 6次以上者分别占 72 .8%和 2 7.2 %。与血尿相关的因素有高血压 (5 2 .1% )、泌尿系感染 (45 .0 % )、上尿路结石 (2 4 .6 % )、运动或用力过度(2 0 .2 % )和外伤 (2 .6 % )。肾脏体积越大、肾功能越差 ,则肉眼血尿发作越严重。血尿组和无血尿组患者总的生存时间平均为 (10 .9± 2 .6 )年和 (2 0 .2± 4 .7)年 ,差异有显著性 (P <0 .0 0 1) ,手术可明显延长生存时间。结论 长期和多次发作的肉眼血尿可影响肾脏功能 ,缩短患者生存时间。对ADPKD患者应及时控制或消除血尿有关因素 ,及时施行囊肿去顶减压术 ,有利于控制血尿 ,缓解症状 ,延缓肾功能损害的进程 ,提高生活质量 ,延长生存时间。

Objective To evaluate the clinical significance of gross hematuria in patients with autosomal dominant polycystic kidney disease(ADPKD). Methods Retrospective studies were performed in 498 cases of ADPKD from June 1960 to May 2002, emphasizing on the incidence, related factors of gross hematuria, and the effects on renal function and survival. Results 38.4%(191/498)cases had single or more episodes of gross hematuria. The mean age at the initial episode was 32 years. Patients experienced less than 5 and more than 6 episodes of gross hematuria were 72.8% and 27.2%,respectively. The related factors of gross hematuria were hypertension(52.1%), urinary tract infection(45.0%), upper urinary tract stones(24.6%), sports or strenuous activity(20.2%)and trauma(2.6%). Those subjects with more episodes of gross hematuria showed larger renal size and higher serum creatinine. The mean survival period in gross hematuria group and nonhematuria group were (10.9±2.6) years and (20.2±4.7) years( P <0.001). Conclusion Long term and more episodes of gross hematuria may have unfavorable impacts on renal function and survival. Controlling and elimination of harmful elements of gross hematuria, and performing cyst decapitative decompression operation at proper time in selective patients may have the advantages of relieving symptoms,preserving renal functions,improving quality of life and prolonging the survival period.