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目的:探讨中枢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:分析我院于2006年～2007年收治的2例病例资料,并结合文献复习讨论。结果:2位患者均为中枢性尿崩症致泌尿系重度积水,肾功能、电解质正常,例1尿比重1.000,尿渗透压40 mmol/L;例2尿比重1.002,尿渗透压79 mmol/L。影像尿流动力学检查和肾血流肾功能显像均未见尿路梗阻表现。激素替代治疗(右旋精氨酸血管加压素)后患者尿量明显减少。治疗后随访18个月以上,患者尿量持续稳定小于4 000 ml/d。B超复查肾积水均较治疗前明显减轻,膀胱剩余尿量明显减少。结论:中枢性尿崩症致巨输尿管-巨膀胱综合征少见,激素替代治疗可明显减少尿量,减轻和阻止尿路积水的发生,改善和保护肾功能。临床早期诊断采取防治措施十分重要,对于严重的膀胱扩大造成的大量剩余尿及继发输尿管梗阻危害肾功能者可行输尿管膀胱再植+膀胱造瘘。
Objective: To investigate the clinical features, diagnosis and treatment of giant ureteral giant snake syndrome caused by central diabetes insipidus. Methods: The data of 2 cases admitted in our hospital from 2006 to 2007 were analyzed and discussed in the literature review. Results: Both of the two patients had severe hydronephrosis, renal function and electrolytes in central diabetes insipidus. The urine specific gravity was 1.000 and the urine osmolality was 40 mmol / L. Example 2 Urinary specific gravity was 1.002, urinary osmotic pressure was 79 mmol / L. Imaging urodynamics and renal blood flow imaging renal function were not seen in urinary tract obstruction. Patients with hormone replacement therapy (L-arginine vasopressin) significantly reduced urine output. Follow-up after treatment for 18 months or more, the patient’s urine output remained stable less than 4 000 ml / d. B-ultrasound hydronephrosis were significantly reduced compared with before treatment, bladder residual urine output decreased significantly. Conclusion: Central uremic disorder caused by giant ureteral giant bladder syndrome is rare, hormone replacement therapy can significantly reduce urine output, reduce and prevent the occurrence of urinary tract hydronephrosis, improve and protect renal function. Early clinical diagnosis and control measures taken is very important for the serious expansion of the bladder caused by a large number of residual urine and secondary ureteral obstruction hazards of renal function feasible ureter bladder replantation + bladder fistula.