Aim To review the clinical features, presentation, investigations undertaken, and outcome of infantile salt-wasting presenting in the setting of urinary tract infection (UTI) and/or urinary tract malformation (UTM) over a two-year surveillance period on the island of Ireland. To estimate a population incidence based on the results and to make recommendations on the approach to management of this condition.
Methods A two-year (2013–14) prospective surveillance undertaken for the island of Ireland via the Irish and Ulster Paediatric Surveillance Units. Monthly-prepaid postcards were circulated to Consultant Paediatricians (n = 260) at all Paediatric Units on the island of Ireland.
Infants under one year of age presenting for the first time with hyponatremia (serum sodium < 130 mmol/L) and/or hyperkalemia (serum potassium > 5.0 mmol/L) associated with urosepsis/UTM were included.
Results Seven patients (six male), all aged younger than five months (3 weeks to 20 weeks) were reported during the study period. All had culture-proven UTI and five (71%) also had an underlying UTM (one diagnosed antenatally). Four (57%) patients had a documented elevated serum aldosterone supporting secondary pseudohypoaldosteronism (PHA) as the underlying diagnosis. Data on aldosterone was not reported in the other three patients but clinical features were suggestive of secondary PHA. All had an excellent outcome with full resolution of the electrolyte disturbance. No cases of primary PHA were submitted during the surveillance period. The estimated incidence for the Irish population of transient pseudohypoaldosteronism was 1 per 13,200 live births per year for the study duration.
Conclusions Salt-wasting is a rare complication of UTI, especially if associated with underlying UTM. There is a similar annual incidence rate to the previously reported incidence of congenital adrenal hyperplasia in Ireland. Boys appear to be at particular risk. Prognosis is good if the condition is recognised and managed promptly.
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