Transient pseudohypoaldosteronism – „puzzle“ of pediatric nephrology and urology

Authors: Z. Doležel 1;  P. Jabandžiev 1;  Z. Ráčilová 2;  J. Štarha 1;  D. Dostálková 1;  P. Zerhau 3
Authors‘ workplace: Pediatrická klinika LF MU a FN Brnopřednosta prof. MUDr. Z. Doležel, CSc. 1;  Klinika dětské radiologie LF MU a FN Brnopřednosta doc. MUDr. J. Skotáková, CSc. 2;  Klinika dětské chirurgie, ortopedie a traumatologie LF MU a FN Brnopřednosta prof. MUDr. L. Plánka, Ph. D. 3
Published in: Čes-slov Pediat 2014; 69 (1): 3-11.
Category: Original Papers


In childhood the majority of changes in serum electrolytes concerns dysbalance of sodium and potassium. Hyponatremia accompanied by hyperkalemia is usually associated with disorders of the adrenal glands, but may also occur in the course of kidney diseases or disorders of gastrointestinal tract. Pseudohypoaldosteronism is an example of salt-losing syndrome and is characterized by hyponatremia, hyperkalemia, metabolic acidosis, high serum aldosterone and high plasma renin. One type of this condition is transient pseudohypoaldosteronism type I. Its development occurs most frequently in neonates or infants in association with urinary tract infections and/or urinary tract anomalies.

We present a group of 14 children (6 girls, 8 boys, mean age 5.6 months) with transient pseudohypoaldosteronism type I, in 12 of them were cause of this type pseudohypoaldosteronism urinary tract anomalies accompanied by urinary infection, in 2 patients were cause acute pyelonephritis without obstructive uropathy. In all children the blood chemistry values were normalized after treatment of urinary infection, 4 patients with serious urinary tract anomalies were required urologic surgery. It is important that urinalysis and renal ultrasonography should be performed in any newborn or infant presenting with salt-wasting with hyperkalemia to exclude urinary tract anomalies or urinary infection as the cause of the dyselectrolytemias.

Key words:
transient pseudohypoaldosteronism, urinary tract infection, obstructive uropathy


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Neonatology Paediatrics General practitioner for children and adolescents
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