Kari Jameela A, Bamashmous Hussain A, Al-Agha Abdulmoein E, Mosli Hisham A
Pediatrics Department, King Abdul-Aziz University Hospital, PO Box 80215, Jeddah 21589, Kingdom of Saudi Arabia.
Saudi Med J. 2002 Jul;23(7):863-5.
Pseudo-hypoaldosteronism occurring predominately in male infants has been reported in association with a spectrum of urologic diseases including obstructive uropathy. This is thought to reflect tubule unresponsiveness to aldosterone. We report a case, which was misdiagnosed as a case of congenital adrenal hyperplasia and treated inappropriately with hydrocortisone and fludrocortisone for 12-months before he had a urinary tract infection and was discovered to have obstructive uropathy on ultrasound. He presented with vomiting, dehydration, hyperkalemia, hyponatremia and metabolic acidosis. His initial 17 hydroxyprogestrone was high. His electrolytes improved to normal after relieving the obstruction by vesicostomy and his treatment weaned slowly without complications. This case demonstrates the importance of urine culture and ultrasound examination in suspected cases of pseudo-hypoaldosteronism.
主要发生于男婴的假性醛固酮减少症已被报道与一系列泌尿系统疾病相关，包括梗阻性尿路病。这被认为反映了肾小管对醛固酮无反应。我们报告一例，该病例最初被误诊为先天性肾上腺皮质增生症，并接受了12个月的氢化可的松和氟氢可的松不恰当治疗，之后他发生了尿路感染，超声检查发现有梗阻性尿路病。他表现为呕吐、脱水、高钾血症、低钠血症和代谢性酸中毒。他最初的17-羟孕酮水平较高。通过膀胱造口术解除梗阻后，他的电解质恢复正常，治疗逐渐减量，未出现并发症。该病例证明了在疑似假性醛固酮减少症病例中进行尿培养和超声检查的重要性。
