Levin T L, Abramson S J, Burbige K A, Connor J P, Ruzal-Shapiro C, Berdon W E
Department of Pediatric Radiology, Columbia Presbyterian Medical Center, Babies Hospital, New York.
Pediatr Radiol. 1991;21(6):413-5. doi: 10.1007/BF02026675.
Salt losing nephropathy, occurring predominantly in male infants, has been reported in association with a spectrum of urologic diseases including obstructive uropathy and massive, infected vesicoureteral reflux (VUR). This has been called pseudo-hypoaldosteronism (PHA) or alternatively, pseudo salt-losing congenital adrenal hyperplasia (CAH), and is thought to reflect a tubular unresponsiveness to aldosterone. We report our experience with six cases, discuss one case in detail and review the 39 cases previously reported. A one month old male infant presented with a left upper quadrant mass. Signs and symptoms included vomiting, dehydration, hyponatremia and hyperkalemia. This suggested the diagnosis of CAH for which therapy was instituted. Ultrasonographic examination subsequently revealed the mass to be a urinoma in an infant with posterior urethral valve (PUV) and obstructive hydronephrosis.
失盐性肾病主要发生于男婴，据报道与一系列泌尿系统疾病相关，包括梗阻性尿路病以及重度感染性膀胱输尿管反流（VUR）。这被称为假性醛固酮减少症（PHA），或者称为假性失盐性先天性肾上腺增生症（CAH），被认为反映了肾小管对醛固酮无反应。我们报告6例病例的经验，详细讨论1例病例，并回顾之前报道的39例病例。一名1个月大的男婴出现左上腹肿块。体征和症状包括呕吐、脱水、低钠血症和高钾血症。这提示了CAH的诊断并开始进行治疗。随后超声检查显示该肿块为一名患有后尿道瓣膜（PUV）和梗阻性肾积水婴儿的尿囊肿。
