Soler Fernández J M, Caravaca Magariños F, Domínguez Bravo C, Murillo Mirat J, Herrera Puerto J, Sanz Cueva J
Servicio de Urología, Hospital Infanta Cristina, Badajoz.
Actas Urol Esp. 1990 Sep-Oct;14(5):374-7.
Massive polyuria existing in congenital nephrogenic diabetes insipidus can cause a more or less severe dilatation of the urinary tract in absence of obstruction. Clinical and diagnostic aspects of this pathology are presented relating then with other types of diabetes insipidus. One case of bilateral severe dilatation with evolution towards renal atrophia is presented. Mechanical obstruction was discarded. The disease was refractory to urinary concentration tests and therapy to reduce urine volume. The possible etiopathological mechanisms of functional obstruction and surgical alternatives directed to preserve the kidney function are explained and discussed. The current literature is reviewed but the cases reported are few due to the low incidence of urological affectation. Presence of kidney atrophia is exceptional.
先天性肾性尿崩症中存在的大量多尿在无梗阻情况下可或多或少导致尿路严重扩张。本文介绍了该疾病的临床和诊断方面，并与其他类型的尿崩症进行了关联。文中呈现了一例双侧严重扩张并进展为肾萎缩的病例。排除了机械性梗阻。该疾病对尿液浓缩试验及减少尿量的治疗均无效。文中解释并讨论了功能性梗阻可能的病因病理机制以及旨在保留肾功能的手术选择。对当前文献进行了综述，但由于泌尿系统受累的发生率较低，报道的病例较少。肾萎缩的情况较为罕见。
