Aboseif S, Gasparini M, Schmidt R, Tanagho E
Department of Urology, University of California School of Medicine, San Francisco.
Br J Urol. 1993 Jul;72(1):106-11. doi: 10.1111/j.1464-410x.1993.tb06469.x.
We report 2 patients with DIDMOAD syndrome (diabetes insipidus, diabetes mellitus, optic atrophy and neural deafness), with emphasis on the urological aspects and their management. Both patients underwent thorough radiological endoscopic and urodynamic evaluation, in addition to detailed evaluation of other systems involved. Each had the characteristic hyper-reflexive neurogenic bladder with sphincteric dyssynergia, which resulted in severe urinary tract dilation. One patient was diagnosed at this institution and managed conservatively with clean intermittent catheterisation and anticholinergic medication; the second patient was referred to us after several attempts at surgical correction. The presentation, details of the urological evaluation with special emphasis on the urodynamic findings, and the outcome of different means of management are discussed.
我们报告了2例患有尿崩症、糖尿病、视神经萎缩和神经性耳聋综合征（DIDMOAD综合征）的患者，重点关注泌尿系统方面及其管理。除了对其他受累系统进行详细评估外，两名患者均接受了全面的放射学、内镜和尿动力学评估。每位患者都有特征性的高反射性神经源性膀胱伴括约肌协同失调，导致严重的尿路扩张。一名患者在本机构确诊，并采用清洁间歇性导尿和抗胆碱能药物进行保守治疗；第二名患者在多次手术矫正尝试后转诊至我们这里。本文讨论了病例介绍、泌尿系统评估细节（特别强调尿动力学检查结果）以及不同治疗方法的结果。
