Analysis of Steroids in Urine for Differentiation of Pseudohypoaldosteronism and Aldosterone Biosynthetic Defect

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Abstract
The salt-losing syndromes in the neonatal period and early infancy due to adrenal disease can be differentiated bythe pattern of excretion of steroids in urine. The presence orabsence of metabolites of cortisol, aldosterone, and corticosteroneas well as certain precursors can be established in a singleanalysis of steroids in urine by using gas chromatography withopen tubular capillary columns. The profiles of steroid excretion in the urine of 8 infants withrenal tubular insensitivity to aldosterone were compared with those in 5 infants with isolated aldosterone biosynthetic defects.The excretion in urine of 18 hydroxytetrahydro-compound Awas elevated in all 13 children, but relative to the excretion oftetrahydroaldosterone, a high ratio was found for the biosyntheticdefect and clearly distinguished the 2 conditions. Agerelatedchanges in steroid metabolism are described. The diagnosisin each case was supported by clinical investigation togetherwith determinations of PRA and aldosterone concentrations.