Janner M, Sommer G, Groessl M, et al. - In this prospective observational study, researchers sought to compare the urinary steroid metabolome and enzyme activities of girls with PA (n = 23) to age-matched control girls (n = 22) and to published steroid values of girls with normal adrenarche and of women with polycystic ovary syndrome (PCOS) and their newborn daughters. The authors discovered that girls with PA had a higher BMI (mean SDS 0.9 vs -0.3). In girls with PA, activities of 17β-hydroxysteroid-dehydrogenase and of 17,20-lyase were higher. Activities of 3β-hydroxysteroid-dehydrogenase, 21-hydroxylase and 5α-reductase activity were not different in groups, compared with reported findings for girls with normal adrenarche or PCOS females. Metabolites and enzymes that participate in alternate androgen pathways tend to be PA markers. Prospective studies should determine whether steroid production in PA also varies from adrenarche at normal timing and continues into adulthood.