常染色体显性多囊肾病患者并发肉眼血尿治疗方法的回顾研究

A retrospective study on management of gross hematuria in autosomal dominant polycystic kidney disease patients

目的 寻找治疗常染色体显性多囊肾病（ADPKD）并发肉眼血尿的理想疗法。方法1993年以来曾在我科住院治疗以及目前在我科多囊肾病专科门诊定期就诊随访的ADPKD患者为对象。收集ADPKD患者出现肉眼血尿时的平均年龄、性别构成、肾功能水平、诱发因素、治疗方案、症状持续时间、血小板计数、凝血参数、肾脏囊肿大小等资料，分别以不同的肉眼血尿诱发因素及治疗方案进行分组，比较其各指标间的差异。结果共筛选出ADPKD患者905例。279例（男150例，女129例）曾有肉眼血尿病史，其中146例能提供完整的病史和治疗经过，而只有101例能提供相关的实验室检查结果。在这101例中，肉眼血尿可出现在慢性肾脏病（CKD）任何一期；GFR为（56．4±44．1）ml·min·（1．73m2）-1；症状持续时间（8．8±8．0）d；男、女患者症状持续时间差异无统计学意义[（8．2±7．3）d比（9．5±8．8）d，P=0．426）；凝血参数均在正常参考范围内，其中91例患者血小板计数正常。不同诱发因素导致的肉眼血尿持续时间差异有统计学意义（P〈0．05）。卧床休息组症状持续时间显著短于其他组患者（P〈0．05）。各组间血小板计数、凝血酶时间和国际标准化比值等差异无统计学意义。结论对出现肉眼血尿的ADPKD患者应首先明确其诱因。卧床休息应作为核心治疗措施。在考虑使用止血药物时建议使用抗纤维蛋白溶解类药物，不需要预防性使用抗生素。

Objective To search the ideal dominant polycystic kidney disease （ADPKD）. management for gross hematuria in autosomal Methods ADPKD patients who were ever hospitalized and followed up in our department since 1993 were enrolled in the study. Demographic and clinical data were colloected, such as gender, age of gross hematuria, level of renal function, causative factors, management strategies, duration of gross hematuria, blood platelet count, activated partial thromboplastin time, prothrombin time, international normalized ratio, size of kidney cyst and so on. ADPKD patients were divided into different groups according to causative factors or management. The clinical data were compared among groups. Results A total of 905 ADPKD patients were screened, among whom 279 patients ever had gross hematuria （male/female：150/129）. One hundred and forty-six patients had integrated therapeutic process records, while only 101 patients could provide relevant laboratory examination results. In these 101 patients, gross hematuria was found in any stage of chronic kidney disease （CKD）; the average eGFR was （56.4±44.1） mml.min-1 （1.73 m2）-1; the duration of gross hematuria was （8.8 ±8.0） d; no significant difference between male and female in duration of gross hematuria existed [（8.2±7.3） d vs （9.5±8.8） d, P= 0.426]; coagulation parameters were all normal. The platelet count was also normal in 91 patients. Duration of gross hematuria among groups divided according to different causative factors was significantly different （P〈0.05）. The patients in bed rest group had significantly shorter duration of gross hematuria compared with other groups （P〈0.05）. The platelet count, prothrombin time and international normalized ratio were all at similar level in different groups. Conclusions The causative factors in ADPKD patients with gross hematuria should be confirmed as the first step of management strategies. Bed rest is the key point in management. Antifibrinolytie agent is a proper choice in the cases receiving hemostatic drugs. It is unnecessary to use antibiotic agent for prevention.