Premature Adrenarche - A Common Condition with Variable Presentation

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Premature Adrenarche - A Common Condition with Variable Presentation Horm Res Paediatr 2015;83:221-231 - DOI:10.1159/000369458 Fig. 1. Steroidogenesis in the ZR of the human adrenal cortex (left panel) and peripheral conversion of AAPs DHEA and DHEAS prior to the final AR-mediated action in peripheral cells (right panel). The middle panel depicts the variably altered steroid pattern in the circulation of children with PA compared to that of age-matched control children [2,5]. (1) The expression and activity of the steroidogenic enzymes and their cofactors in ZR influence the adrenal androgen (precursor) secretion; high 17,20-lyase activity of the 17-hydroxylase/17,20-lyase enzyme (CYP17) and expression of POR and cytochrome b5 (b5) are essential [18]. (2) Possible minor T secretion from ZR [19], gonads or peripheral tissues may contribute to circulating T levels. (3) The expression and activity of the steroid-converting enzymes in peripheral tissues determine the formation of biologically active androgens from AAPs. (4) An alternative backdoor pathway to DHT formation [30]. (5) An alternative pathway from 11β-hydroxyandrostenedione (11OHA4) via 11β-hydroxytestosterone (11OHT) to a bioactive androgen 5α,11β-hydroxytestosterone (5,11OHT) [19]. (6) The activity of AR is influenced by AR gene polymorphisms, epigenetic modulations, and expression of its cofactors/comodulators [70,71,72]. A4 = Androstenedione; StAR = steroidogenic acute regulatory protein; 17OHPreg = 17OH-pregnenolone; 3βHSD = 3β-hydroxysteroid dehydrogenase (HSD3B); 17βHSD = 17β-hydroxysteroid dehydrogenase (HSD17B); ↑ = increased steroid concentration; ↔ = no reported change in the circulation of children with PA. © 2015 S. Karger AG, Basel

Premature Adrenarche - A Common Condition with Variable Presentation Horm Res Paediatr 2015;83:221-231 - DOI:10.1159/000369458 Fig. 2. Management of children with PA. 17OHP = 17-hydroxyprogesterone; DM2 = type 2 diabetes mellitus; SD = standard deviation; OGTT = oral glucose tolerance test; WC = waist circumference. © 2015 S. Karger AG, Basel