17-Hydroxyprogesterone in children, adolescents and adults

Abstract

17-Hydroxyprogesterone (17-OHP) is an intermediate steroid in the adrenal biosynthetic pathway from cholesterol to cortisol and is the substrate for steroid 21-hydroxylase. An inherited deficiency of 21-hydroxylase leads to greatly increased serum concentrations of 17-OHP, while the absence of cortisol synthesis causes an increase in adrenocorticotrophic hormone. The classical congenital adrenal hyperplasia (CAH) presents usually with virilisation of a girl at birth. Affected boys and girls can have renal salt loss within a few days if aldosterone production is also compromised. Diagnosis can be delayed in boys. A non-classical form of congenital adrenal hyperplasia (NC-CAH) presents later in life usually with androgen excess. Moderately raised or normal 17-OHP concentrations can be seen basally but, if normal and clinical suspicion is high, an ACTH stimulation test will show 17-OHP concentrations (typically >30 nmol/L) above the normal response. NC-CAH is more likely to be detected clinically in females and may be asymptomatic particularly in males until families are investigated. The prevalence of NC-CAH in women with androgen excess can be up to 9% according to ethnic background and genotype. Mutations in the 21-hydroxylase genes in NC-CAH can be found that have less deleterious effects on enzyme activity. Other less-common defects in enzymes of cortisol synthesis can be associated with moderately elevated 17-OHP. Precocious puberty, acne, hirsutism and subfertility are the commonest features of hyperandrogenism. 17-OHP is a diagnostic marker for CAH but opinions differ on the role of 17OHP or androstenedione in monitoring treatment with renin in the salt losing form. This review considers the utility of 17-OHP measurements in children, adolescents and adults.

Multiple adrenocortical steroid response to administration of exogenous adrenocorticotropic hormone to hospitalized foals

“…11 There are increased progesterone and 17α-OH-progesterone responses to T A B L E 2 Area under the curve and Delta 0-30 for adrenocortical steroids, ACTH/cortisol ratio, and laboratory findings in surviving and non-surviving hospitalized foals (median and ranges) ACTH stimulation in dogs with adrenal-and pituitary-dependent hyperadrenocorticism, in children with congenital adrenal hyperplasia, and in healthy intact and ovariohysterectomized cats and cows. [27][28][29][30] In the current study, the progestogen response to ACTH was lower in foals that died. The reason for the lower 17α-OH-progesterone secretion in non-surviving foals after ACTH administration remains unclear.…”

“…The reason for the lower 17α‐OH‐progesterone secretion in non‐surviving foals after ACTH administration remains unclear. Both progesterone and 17α‐OH‐progesterone are adrenocortical precursors of cortisol; progesterone is synthesized from pregnenolone by 3β‐hydroxysteroid dehydrogenase to be further converted into 17α‐OH‐progesterone by 17‐α‐hydroxylase, and ultimately to cortisol . A deficiency of these enzymes or a shift in adrenal steroidogenesis might reduce progesterone and 17α‐OH‐progesterone concentrations, indirectly resulting in inappropriate glucocorticoid synthesis .…”

Total testosterone to dihydrotestosterone ratio assessed by LC-MS/MS predicts a worse metabolic profile not only in PCOS patients

“…As liquid chromatography coupled with mass spectrometry (LC-MS/MS) is indicated as the method with the highest accuracy, we have used this method to measure androgens concentrations in order to look for relations between androgen levels, TT/DHT ratio and the metabolic profile in PCOS patients and controls [6,7]. [1].…”

The hunt for a selective 17,20 lyase inhibitor; learning lessons from nature

“…Once again, while produced as an intermediate in the adrenal ZF itself, circulating levels of 17-hydroxyprogesterone are not normally a reflection of adrenal production, but of both ovarian pre-ovulatory theca and post-ovulatory luteinized theca cell function, particularly from the latter during the post-ovulatory luteal phase of the ovarian or menstrual cycle 35; 36 . Only in conditions of CYP21A1 deficiency will circulating levels of 17-hydroxyprogesterone reflect adrenal activity 6; 37; 38 and so are used as diagnostic criteria for congenital adrenal hyperplasia 39; 40; 41; 42 .…”