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Updated: Jun 2, 2026

A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
[Transient pseudohypoaldosteronism]
Insights
Infants with urinary tract malformations and infections may develop transient type 1 pseudohypoaldosteronism (THPA1). Early diagnosis via urine and ultrasound is crucial to prevent underdiagnosis and incorrect treatment.
Area of Science:
- Pediatric Endocrinology
- Nephrology
- Urology
Context:
- Urinary tract malformations (UTM) and infections (UTI) in infants are associated with transient type 1 pseudohypoaldosteronism (THPA1).
- THPA1 presents with electrolyte and acid-base imbalances, mimicking other conditions.
Purpose:
- To report on a patient series with THPA1, UTM, and/or UTI.
- To provide suggestions for diagnosis and therapy of THPA1 in infants.
Summary:
- Patients exhibited hyponatremia, hyperkalemia, metabolic acidosis, and elevated creatinine, with characteristic urinary electrolyte findings indicating tubular resistance to aldosterone.
- Elevated aldosterone and plasma aldosterone-to-potassium ratio, along with low urinary K/Na, confirmed tubular resistance.
- Medical and/or surgical interventions normalized patient parameters. Imaging revealed various urinary tract obstructions.
Impact:
- Highlights the importance of considering THPA1 in infants with UTM/UTI and electrolyte disturbances.
- Emphasizes the necessity of urine and renal ultrasound for accurate THPA1 diagnosis.
- Aims to prevent underdiagnosis and inappropriate treatment of THPA1 in male infants.
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 micromol/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.
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