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17-Hydroxyprogesterone in children, adolescents and adults.
Ann Clin Biochem. 2014 Jul; 51(Pt 4):424-40.AC

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.

Authors+Show Affiliations

Institute of Women's Health, University College London, London, UK jwhonour@ntlworld.com.

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

24711560

Citation

Honour, John W.. "17-Hydroxyprogesterone in Children, Adolescents and Adults." Annals of Clinical Biochemistry, vol. 51, no. Pt 4, 2014, pp. 424-40.
Honour JW. 17-Hydroxyprogesterone in children, adolescents and adults. Ann Clin Biochem. 2014;51(Pt 4):424-40.
Honour, J. W. (2014). 17-Hydroxyprogesterone in children, adolescents and adults. Annals of Clinical Biochemistry, 51(Pt 4), 424-40. https://doi.org/10.1177/0004563214529748
Honour JW. 17-Hydroxyprogesterone in Children, Adolescents and Adults. Ann Clin Biochem. 2014;51(Pt 4):424-40. PubMed PMID: 24711560.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 17-Hydroxyprogesterone in children, adolescents and adults. A1 - Honour,John W, Y1 - 2014/04/07/ PY - 2014/4/9/entrez PY - 2014/4/9/pubmed PY - 2015/7/4/medline KW - Steroid hormones KW - endocrinology KW - evaluation of new methods SP - 424 EP - 40 JF - Annals of clinical biochemistry JO - Ann Clin Biochem VL - 51 IS - Pt 4 N2 - 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. SN - 1758-1001 UR - https://www.unboundmedicine.com/medline/citation/24711560/17_Hydroxyprogesterone_in_children_adolescents_and_adults_ L2 - https://journals.sagepub.com/doi/10.1177/0004563214529748?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -