梗阻性尿路病假醛固酮增多症(PHA),文献中已经报道了尿路感染和先天性泌尿生殖道畸形;然而,由于常见的泌尿生殖道,没有PHA与积水相关的报道。Hydrometrocolpos是一种由于分泌物(血液除外)的积聚而导致阴道和子宫扩张的疾病,由雌激素刺激增加和阴道流出道阻塞引起。我们报告了一例新生儿PHA病例,该病例是由复发性水生动物引起的,表现为呕吐和体重增加不良。有严重的低钠血症,高钾血症,和贫血需要在手术前进行医疗稳定。PHA在阻塞中发生的机制涉及由于肾积水的压力和肾内细胞因子的释放而导致的肾小管功能障碍。此外,婴儿期对醛固酮有不成熟或耐药的肾小管反应。临床医生应该意识到这种罕见但严重的表现。
Pseudohypoaldosteronism (PHA) due to obstructive uropathy, urinary tract infections and congenital urogenital malformations has been reported in the literature; however, there are no
reports of PHA associated with hydrometrocolpos due to a common urogenital tract. Hydrometrocolpos is a condition resulting in distension of the vagina and uterus due to accumulation of secretions (other than blood), caused by increased oestrogenic stimulation and vaginal outflow obstruction. We report on a neonatal
case of PHA caused by recurrent hydrometrocolpos presenting with vomiting and poor weight gain. There was significant hyponatraemia, hyperkalaemia, and anaemia requiring medical stabilisation prior to surgery. The mechanism by which PHA occurs in obstruction involves renal tubular dysfunction due to pressure from hydronephrosis and the release of intrarenal cytokines. In addition, there is an immature or resistant renal tubular responsiveness to aldosterone during infancy. Clinicians should be aware of this uncommon but serious presentation.