The incidence of transient infantile pseudohypoaldosteronism in Ireland: A prospective study

Abstract

Aim To review the clinical course, outcome and incidence of infantile salt wasting associated with urinary tract infection (UTI) and/or urinary tract malformation (UTM) over a two‐year surveillance period on the island of Ireland. Methods A two‐year (2013‐14) prospective surveillance undertaken 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 hyponatraemia (Na < 130 mmol/L) and/or hyperkalaemia (K > 5.0 mmol/L) associated with urosepsis/UTM were reported. Results All 7 reported patients (6 male) had culture‐proven UTI, and 5 (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 were not reported in the other 3 patients, but clinical features were suggestive of secondary PHA. The estimated incidence for the Irish population of transient PHA is 1 per 13,200 total live births per year. Conclusions Salt wasting is a rare complication of UTI, especially if associated with underlying UTM. Boys appear to be at particular risk.

Neonatal Pseudohypoaldosteronism Type-1 in Japan

“…Thus, we may conclude that preterm infants are predisposed to developing PHA-1. Contrary to previous findings regarding infantile PHA-1, no male predominance was found in our neonatal PHA-1 cases [ 7 , 18 ].…”

“…They concluded that the risk of transient PHA-1 was the greatest in boys with underlying urinary tract malformations, with or without concurrent urinary tract infection, within the first 3 months of life. Similar to the previous case series, only 2 out of 7 cases in their study had data on both renin and aldosterone levels at onset [ 7 ]. Likewise, Abraham et al reported a case series of five patients with PHA-1 secondary to UTI from a single center retrospective study in Australia.…”

“…In this regard, Storey et al proposed the existence of transient aldosterone resistance in newborns without renal complications [ 19 ]. In general, the term “transient PHA-1” has often been used for secondary PHA caused by UTI and/or UTM [ 7 , 18 ]. However, our study revealed cases that met the biochemical and hormonal definition of PHA-1 very transiently, including having no renal complications.…”

“…The occurrence of secondary PHA-1 due to obstructive uropathies with and without infection during infancy and lateris well known. Kaninde et al conducted a two-year prospective surveillance in Ireland and speculated the incidence of infantile secondary PHA-1 associated with urinary tract infection (UTI) and/or urinary tract malformation (UTM) to be 1 per 13,200 total live births per year [ 7 ].…”

“…Both primary and secondary PHA-1 include symptoms of sodium loss (aldosterone deficiency) from the neonatal period to infancy; however, they often do not require treatment after childhood [ 2 , 7 , 8 ]. It has been postulated that a developmental increase in tubular responsiveness to aldosterone might account for the improvement of PHA-1 [ 9 ].…”