Secondary Pseudohypoaldosteronism Associated With Mild Hydronephrosis in a Newborn

Neonatal hyponatremia with hyperkalemia is a rare but potentially life-threatening occurrence. Aldosterone deficiency secondary to congenital adrenal hyperplasia (CAH) is often suspected in these cases, although it is not easy to accurately diagnose it initially. We report the case of a 12-day-old female infant presenting with poor sucking, hyperkalemia, and hyponatremia. Plasma renin activity (PRA) and aldosterone levels were markedly elevated, and mild hydronephrosis [Society for Fetal Urology (SFU) grade 1] was noted. We then suspected secondary pseudohypoaldosteronism (S-PHA); however, her serum potassium level remained elevated despite sodium infusion. Because we could not rule out a diagnosis of adrenal insufficiency caused by CAH, we cautiously initiated hydrocortisone. After reviewing the results of a mass screening test and a urine steroid profile analysis, adrenal diseases were ruled out and we diagnosed the patient with S-PHA. This report aims to illustrate that mild hydronephrosis can cause S-PHA by inducing renal tubular resistance to aldosterone. Because the symptoms of S-PHA are similar to those of CAH, we recognize that further studies are needed to clarify their differences.