Ewha Med J.  2019 Jan;42(1):6-9. 10.12771/emj.2019.42.1.6.

Transient Pseudohypoaldosteronism in a 5-Month-old Infant Manifested as a Failure to Thrive

• Lee JW
• Cho SJ
• Kim HS

• KMID: 2433550
• DOI: http://doi.org/10.12771/emj.2019.42.1.6

Abstract

Pseudohypoaldosteronism (PHA) in infants is manifested by presence of hyperkalemia, hyponatremia, and metabolic acidosis. At initial stages, PAH is generally suspected as congenital adrenal hyperplasia. Transient PHA has been reported in infants with urinary tract infection and urinary tract malformation. We report a case of 5-month-old infant with failure to thrive and finally diagnosed with transient PHA due to urinary tract infection with vesicoureteral reflux.

Keyword

Pseudohypoaldosteronism; Failure to thrive; Urinary tract infections; Vesico-ureteral reflux

MeSH Terms

Acidosis
Adrenal Hyperplasia, Congenital
Failure to Thrive*
Humans
Hyperkalemia
Hyponatremia
Infant*
Pseudohypoaldosteronism*
Urinary Tract
Urinary Tract Infections
Vesico-Ureteral Reflux

Figure

• 

  Fig. 1 The technetium-99m-dimercaptosuccinic acid (DMSA) renal scan findings show multiple cortical defects in both the kidneys and generalized areas of diminished uptake in right kidney. (A) Anterior view of DMSA. (B) Posterior view of DMSA. RT, right; ANT, anterior; LT, left; POST, posterior.

• 

  Fig. 2 Voiding cystourethrography findings show vesicoureteral reflux (right, grade V; left, grade IV).

Reference

1. Geller DS. Mineralocorticoid resistance. Clin Endocrinol (Oxf). 2005; 62:513–520.

2. Bogdanovic R, Stajic N, Putnik J, Paripovic A. Transient type 1 pseudo-hypoaldosteronism: report on an eight-patient series and literature review. Pediatr Nephrol. 2009; 24:2167–2175.

3. Dolezel Z, Starha J, Novotna D, Dostalkova D. Secondary pseudohypoaldosteronism in an infant with pyelonephritis. Bratisl Lek Listy. 2004; 105:435–437.

4. Hanukoglu A. Type I pseudohypoaldosteronism includes two clinically and genetically distinct entities with either renal or multiple target organ defects. J Clin Endocrinol Metab. 1991; 73:936–944.

5. Zennaro MC, Lombes M. Mineralocorticoid resistance. Trends Endocrinol Metab. 2004; 15:264–270.

6. Pujo L, Fagart J, Gary F, Papadimitriou DT, Claes A, Jeunemaitre X, et al. Mineralocorticoid receptor mutations are the principal cause of renal type 1 pseudohypoaldosteronism. Hum Mutat. 2007; 28:33–40.

7. Rodriguez-Soriano J, Vallo A, Oliveros R, Castillo G. Transient pseudohypoaldosteronism secondary to obstructive uropathy in infancy. J Pediatr. 1983; 103:375–380.

8. Proctor G, Linas S. Type 2 pseudohypoaldosteronism: new insights into renal potassium, sodium, and chloride handling. Am J Kidney Dis. 2006; 48:674–693.

9. Watanabe T. Reversible secondary pseudohypoaldosteronism. Pediatr Nephrol. 2003; 18:486.

10. Belot A, Ranchin B, Fichtner C, Pujo L, Rossier BC, Liutkus A, et al. Pseudohypoaldosteronisms, report on a 10-patient series. Nephrol Dial Transplant. 2008; 23:1636–1641.

11. Melzi ML, Guez S, Sersale G, Terzi F, Secco E, Marra G, et al. Acute pyelonephritis as a cause of hyponatremia/hyperkalemia in young infants with urinary tract malformations. Pediatr Infect Dis J. 1995; 14:56–59.

12. Bowden SA, Cozzi C, Hickey SE, Thrush DL, Astbury C, Nuthakki S. Autosomal dominant pseudohypoaldosteronism type 1 in an infant with salt wasting crisis associated with urinary tract infection and obstructive uropathy. Case Rep Endocrinol. 2013; 2013:524647.

13. Fujinaga S, Ohtomo Y, Someya T, Shimizu T, Yamashiro Y. Transient pseudohypoaldosteronism complicating acute renal failure in an infant with vesico-ureteral reflux and pyelonephritis. Pediatr Int. 2009; 51:744–746.

14. Delhikumar CG, Narayanan P, Mahadevan S. Pseudohypoaldosteronism masquerading as congenital adrenal hyperplasia. Indian J Pediatr. 2012; 79:115–116.