Aldosterone-Signaling Defect Exacerbates Sodium Wasting in Very Preterm Neonates: The Premaldo Study

Abstract

Very preterm infants have a previously unrecognized defective aldosterone secretion but conserved renal aldosterone sensitivity in the neonatal period, which modifies the current view of sodium balance in these infants and suggests alternative management approaches.

Temporal and spatial distribution of mast cells and steroidogenic enzymes in the human fetal adrenal

“…Our results indicate that neonate salt wasting syndrome could also be the consequence of a primary defect in aldosterone secretion in preterm infants, as suggested by low level of adrenal CYP11B2 expression before 28 WG. This hypothesis is in accordance with the recent report by Martinerie et al showing unrecognized defective aldosterone secretion but conserved renal aldosterone sensitivity in the neonatal period of extreme preterm infants (Martinerie et al, 2015).…”

“…On the basis of the results obtained in the present study, it could be suggested that cortisol and aldosterone secretions respectively arise from 18-20 WG and 22e24 WG. This interpretation is supported by Bagnoli et al who detected cortisol in cord plasma of preterm children from 21 WG (Bagnoli et al, 2013) and Martinerie et al, who reported measurable aldosterone levels in cord blood of preterm infants born between 26 and 32 WG (Martinerie et al, 2015). The comprehension of the mechanisms involved in the establishment of mineralocorticoid synthesis is extremely important for the management of the salt wasting disorders frequently observed in extreme preterm neonates (Barrington, 2014).…”

Alterations of adrenal steroidomic profiles in preterm infants at birth

“…Umbilical cord blood samples of 152 newborns enrolled from the Premaldo cohort were assessed 17. Briefly, inclusion criteria were mothers aged 18–45 years old, with no history of type 1 or 2 diabetes and none of the following maternal treatment prior to pregnancy (systemic or inhaled glucocorticoid therapy, hormonal treatment for adrenal or pituitary insufficiency and antihypertensive drugs).…”

A novel frameshift mutation in <i>NR3C2</i> leads to decreased expression of mineralocorticoid receptor: a family with renal pseudohypoaldosteronism type 1

“…These findings suggest that MR function during early infancy is more important than it is during adulthood, and has independent affects at each stage of life. Martinerie et al demonstrated that human MR expression is significantly decreased during infancy, and increases from 11 months old [21,22]. These findings suggest that increased MR expression with advancing age may compensate for the effect of mutations on MR function, although it is reported that adult PHA1 patients present with life-long increased plasma renin and aldosterone levels, together with an increased salt appetite, which allows them to maintain a normal salt balance with normal potassium levels and blood pressure [23].…”