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DIFFERENTIATION OF PSEUDOHYPOALDOSTERONISM (PHA) AND ADRENAL BIOSYNTHETIC DEFECTS

Adrenal salt-wasting diseases in the neonatal period and early infancy can be confirmed by the pattern of steroids excreted in urine. The presence or absence of cortisol, aldosterone and corticosterone metabolites, plus certain precursors, is established in a single analysis of steroids using gas chromatography with capillary columns and mass spectrometry.In infants (n=5) with the Type II biosynthetic defect of aldosterone, the excretion of corticosterone and 18-hydroxycorticosterone metabolites were elevated. In PHA (n=8), both 18-hydroxycorticosterone and aldosterone metabolites were found at high concentrations in urine. When salt loss was controlled by replacement therapy or sodium supplements respectively, and plasma renin activity (pRA) were normalised, the excretion rates of the metabolites were lower but the ratios of excretion for 18-hydroxycorticosterone to aldosterone metabolites were greater than 30 to 1 in the biosynthetic defect but nearer unity in PHA. The interpretation of the overall pattern of steroid excretion also requires consideration of the changes in the nature of steroid metabolites in newborns through infancy, since conjugates of tetrahydro-metabolites replace hydroxylated and free compounds. These studies are complementary to determinations of pRA and plasma steroid concentrations in defining the diagnosis of infantile salt wasting disease.