Introduction. Infants with urinary tract malformations (UTM) presenting with
urinary tract infection (UTI) are prone to develop transient type 1
pseudohypoaldosteronism (THPA1). Objective. Report on patient series with
characteristics of THPA1, UTM and/or UTI and suggestions for the diagnosis
and therapy. Methods. Patients underwent blood and urine electrolyte and
acid-base analysis, serum aldosterosterone levels and plasma rennin activity
measuring; urinalysis, urinoculture and renal ultrasound were done and
medical and/or surgical therapy was instituted. Results. Hyponatraemia
(120.9?5.8 mmol/L), hyperkalaemia (6.9?0.9 mmol/L), metabolic acidosis
(plasma bicarbonate, 11?1.4 mmol/L), and a rise in serum creatinine levels
(145?101 ?mol/L) were associated with inappropriately high urinary sodium
(51.3?17.5 mmol/L) and low potassium (14.1?5.9 mmol/L) excretion. Elevated
plasma aldosterone concentrations (170.4?100.5 ng/dL) and the very high
levels of the plasma aldosterone to potassium ratio (25.2?15.6) together with
diminished urinary K/Na values (0.31?0.19) indicated tubular resistance to
aldosterone. After institution of appropriate medical and/or surgical
therapy, serum electrolytes, creatinine, and acid-base balance were
normalized. Imaging studies showed ureteropyelic or ureterovesical junction
obstruction in 3 and 2 patients, respectively, posterior urethral valves in
3, and normal UT in 1 patient. According to our knowledge, this is the first
report on THPA1 in the Serbian literature. Conclusion. Male infants with
hyponatraemia, hyperkalaemia and metabolic acidosis have to have their urine
examined and the renal ultrasound has to be done in order to avoid both, the
underdiagnosis of THPA1 and the inappropriate medication.