Abstract
We present a case of a female infant diagnosed with secondary pseudohypoaldosteronism (PHA) who exhibited weight loss, hyponatremia, hyperkalemia, and metabolic acidosis without the presence of urinary tract anomalies (UTA) or urinary tract infections (UTI). She was born at 35 weeks gestation and developed electrolyte abnormalities. Initially managed for transient tachypnea of the newborn, she developed respiratory distress requiring mechanical ventilation. Subsequently, she exhibited persistent hyponatremia, hyperkalemia, and metabolic acidosis despite adequate fluid therapy, prompting consideration of adrenal insufficiency and congenital adrenal hyperplasia (CAH). Treatment with hydrocortisone and fludrocortisone was initiated empirically until hormonal analyses excluded CAH. Further evaluation excluded UTA and UTI as underlying causes, implicating secondary PHA. The infant responded well to saline and electrolyte replacement therapy, with normalization of electrolyte levels and clinical improvement. Follow-up assessments demonstrated resolution of electrolyte imbalances, and the patient was discharged after 27 days, at an adjusted age of 38 weeks and 6 days, without further complications. Secondary PHA, characterized by renal tubular resistance to aldosterone, typically presents with severe electrolyte disturbances in infancy. It can occur independently of UTA or UTI, highlighting the importance of considering this diagnosis in neonates and infants presenting with hyponatremia, hyperkalemia, and metabolic acidosis that do not respond to conventional therapies. Early recognition and appropriate management, including fluid-electrolyte correction, and hormone replacement if indicated, are crucial to prevent life-threatening complications associated with salt-wasting syndromes in this vulnerable population.
Keywords: Secondary pseudohypoaldosteronism, newborn, no urinary tract anomaly or infection
Read the full text
3 sections · 1 table · PDF
Create your free account to read the full text.
- Full text with figures and tables
- PDF and supplementary files
- Access on every Galenos journal
One Galenos account works on every Galenos journal site.
References
Ellison DH. Pseudohypoaldosteronism Type II. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, eds. GeneReviews ®. Seattle (WA): University of Washington, Seattle; November 10, 2011.
Memoli E, Lava SAG, Bianchetti MG, Vianello F, Agostoni C, Milani GP. Prevalence, diagnosis, and management of secondary pseudohypoaldosteronism. Pediatr Nephrol. 2020;35:713-714. Epub 2019 Dec 20
Krishnappa V, Ross JH, Kenagy DN, Raina R. Secondary or transient pseudohypoaldosteronism associated with urinary tract anomaly and urinary infection: a case report. Urol Case Rep. 2016;8:61-62.
Betti C, Lavagno C, Bianchetti MG, Kottanattu L, Lava SAG, Schera F, Lacalamita MC, Milani GP. Transient secondary pseudo-hypoaldosteronism in infants with urinary tract infections: systematic literature review. Eur J Pediatr. 2024;183:4205-4214. Epub 2024 Jul 10
Ranjith G, Uthup S, Satish B, Jain N. Salt wasting disorder in the newborn. Indian J Pediatr. 2006;73:95-96.
Klingenberg C, Hagen IJ. Transient pseudohypoaldosteronisme hos spedbarn med vesikoureteral refluks [Transient pseudohypoaldosteronism in infants with vesicoureteral reflux]. Tidsskr Nor Laegeforen. 2006;126:315-317. Norwegian.
Delforge X, Kongolo G, Cauliez A, Braun K, Haraux E, Buisson P. Transient pseudohypoaldosteronism: a potentially severe condition affecting infants with urinary tract malformation. J Pediatr Urol. 2019;15:265.e1-265.e7. Epub 2019 Mar 9
Kocaoglu C, Akturk S. Analysis of patients presenting with serum electrolyte imbalance in terms of the differential diagnosis of pseudohypoaldosteronism. North Clin Istanb. 2023;10:754-760.
Günay F, Şıklar Z, Berberoğlu M. Difficulties in the diagnosis and management of eight infants with secondary pseudohypoaldosteronism. Turk J Pediatr. 2022;64:490-499.
Melzi ML, Guez S, Sersale G, Terzi F, Secco E, Marra G, Tirelli AS, Assael BM. Acute pyelonephritis as a cause of hyponatremia/hyperkalemia in young infants with urinary tract malformations. Pediatr Infect Dis J. 1995;14:56-59.
Rodríguez-Soriano J, Vallo A, Oliveros R, Castillo G. Transient pseudohypoaldosteronism secondary to obstructive uropathy in infancy. J Pediatr. 1983;103:375-380.
Kumar S, McDermott H, Kamupira S, Agwu JC. Rare case of pseudohypoaldosteronism in a neonate secondary to congenital hydrometrocolpos. BMJ Case Rep. 2020;28;13:e234813.
Martinerie L, Pussard E, Yousef N, Cosson C, Lema I, Husseini K, Mur S, Lombès M, Boileau P. Aldosterone-signaling defect exacerbates sodium wasting in very preterm neonates: the premaldo study. J Clin Endocrinol Metab. 2015;100:4074-4081.
Watanabe T, Yamazaki A. Pneumothorax and transient pseudohypoaldosteronism in an infant with hydronephrosis. Pediatr Nephrol Berl Ger. 2013;18:62-64.


