继发于肾源性尿崩症的巨输尿管-巨膀胱综合征(附一例报告并文献复习)

目的 探讨肾源性尿崩症所致巨输尿管－巨膀胱综合征的临床特点及诊治措施。方法 分析1999年12月收治的1列病历资料，结合文献复习讨论。结果 病人为重度尿路积水，血生化、血电解质正常，尿比重1．000。尿动力学检查和明动态显影未见尿路梗阻表现。利钠利水剂等药物有助于减少患者尿量。治疗后随访6个月，患者尿量5000－6000ml。B超复查肾盂积水较前有所减轻，膀胱剩余尿量明显减少。结论 肾源性尿崩症导致巨输尿管－巨膀胱综合征合并尿路破裂者罕见，药物治疗可明显减少尿量，减轻和阻止尿路积水的发生。临床早期诊断采取防治措施十分重要。

Megaureter-megacystis syndrome secondary to congenital nephrogenic diabets insipidus (a case report and literature review)

Objective To study the clinical features and the urological complications of congenital diabetes insipidus. Methods A case of congenital diabetse insipidus was presented with review of the li- terature.The urodynamics and the isotopic renal dynamic imaging of the patient were studied. Results The patient had megahydronephrosis,megaureter and megacystis with very low specific gravity of the urine (1.000) yet with normal blood chemistry,normal blood electrolytes and with no urinary tract outflow obstruction.The urine volume could be decreased by medication. Conclusions Early diagnosis of congenital diabetes insipidus and the prevention of urological complications are important.Automatic urinary tract rupture secondary to congenital diabetes insipidus is rarely seen.