Serum levels of 5-androstene-3 beta,17 beta-diol sulphate, 5 alpha-androstane-3 alpha, 17beta-diol sulphate and glucuronide, in late onset 21-hydroxylase deficiency.

Abstract

Serum sulphates of 5-androstene-3 beta,17 beta-diol (5-ADIOL-S), 5 alpha-androstane-3 alpha,17 beta-diol (3 alpha-DIOL-S) and dehydroepiandrosterone (DHEA-S), as well as 5 alpha-androstane-3 alpha,17 beta-diol glucuronide (3 alpha-DIOL-G) and unconjugated androstenedione (AD) and testosterone (T), sex hormone binding globulin (SHBG), free androgen index (FAI) and 17 alpha-hydroxyprogester-one (17OHP) were measured by specific radioimmunoassays (RIA) in 14 women with late-onset 21-hydroxylase deficiency (LOCAH), and in normal women (n = 73). The diagnosis of LOCAH was made on the finding of a (17OHP) response level greater than 30 nmol/l following ACTH stimulation, and/or an elevation of urinary metabolites of 17OHP. Mean values for serum concentrations of all steroids measured and the free androgen index (100 X T nmol/l divided by SHBG nmol/l) were significantly elevated, and SHBG levels depressed in patients with LOCAH. These studies show that in LOCAH, in addition to the unconjugated steroids AD and T, the sulphoconjugated steroids DHEA-S, 5-ADIOL-S and 3 alpha-DIOL-S are increased, as is the glucuronide conjugate 3 alpha-DIOL-G and the index of bioavailable testosterone (FAI), and that mean SHBG levels are depressed. These data suggest that as well as AD, 5-ADIOL-S and DHEA-S may act as pro-hormones for more potent steroids (T and 5 alpha-dihydrotestosterone) in peripheral tissues, while 3 alpha-DIOL-S and 3 alpha-DIOL-G may both reflect peripheral androgen metabolism in patients with LOCAH.

• Authors: J Montalto, J W Funder, A B Yong, C B Whorwood, J F Connelly
• Journal: The Journal of steroid biochemistry and molecular biology (J Steroid Biochem Mol Biol) Vol. 37 Issue 4 Pg. 593-8 (Nov 30 1990) ISSN: 0960-0760 [Print] England
• PMID: 2177625 (Publication Type: Journal Article)

Chemical References

• Glucuronates
• Hydroxyprogesterones
• Sex Hormone-Binding Globulin
• Sulfates
• Androstane-3,17-diol
• Testosterone
• Androstenedione
• Dehydroepiandrosterone
• Dehydroepiandrosterone Sulfate
• 17-alpha-Hydroxyprogesterone
• Adrenocorticotropic Hormone
• Androstenediol

Topics

• 17-alpha-Hydroxyprogesterone
• Adrenal Hyperplasia, Congenital
• Adrenocorticotropic Hormone
• Adult
• Androstane-3,17-diol (blood)
• Androstenediol (blood)
• Androstenedione (blood)
• Dehydroepiandrosterone (analogs & derivatives, blood)
• Dehydroepiandrosterone Sulfate
• Female
• Glucuronates (blood)
• Hirsutism (blood)
• Humans
• Hydroxyprogesterones (blood, urine)
• Sex Hormone-Binding Globulin (metabolism)
• Sulfates (blood)
• Testosterone (blood)