Serum 17-hydroxypregnenolone and 17-hydroxypregnenolone sulfate concentrations in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Abstract

Serum concentrations of 17-hydroxypregnenolone, 17-hydroxypregnenolone sulfate and 17-hydroxyprogesterone were measured simultaneously in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency, using a combined radioimmunoassay method. All these precursor steroids were found to be markedly elevated in the sera of untreated patients with a salt-losing form of the disease, whereas, in untreated patients with a simple virilizing form, only the concentration of unconjugated steroids was increased and the 17-hydroxypregnenolone sulfate concentration remained within the normal range. Among the patients with a salt-losing form under maintenance therapy, these steroids were all still significantly increased in those on insufficient control, whereas only 17-hydroxyprogesterone was significantly but slightly increased in those on adequate control. Although the mechanism whereby the serum 17-hydroxypregnenolone sulfate concentration is not increased in the untreated simple virilizers is unknown, both a milder degree of 21-hydroxylase deficiency and a role of 17-hydroxypregnenolone sulfate in adrenal steroid production as a kind of supplier are suggested as possible explanations, especially in the neonatal period and early infancy. Thus, this study showed the serum concentrations of 17-hydroxypregnenolone and its sulfate together with 17-hydroxyprogesterone in patients with 21-hydroxylase deficiency in various conditions.

• Authors: K Shimozawa, S Saisho, J Yata, A Kambegawa
• Journal: Endocrinologia japonica (Endocrinol Jpn) Vol. 35 Issue 1 Pg. 11-8 (Feb 1988) ISSN: 0013-7219 [Print] Japan
• PMID: 3260857 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Hydroxyprogesterones
• 17-hydroxypregnenolone sulfate
• 17-alpha-Hydroxypregnenolone
• 17-alpha-Hydroxyprogesterone
• Steroid Hydroxylases
• Cortisone
• Hydrocortisone

Topics

• 17-alpha-Hydroxypregnenolone (analogs & derivatives, blood)
• 17-alpha-Hydroxyprogesterone
• Administration, Oral
• Adolescent
• Adrenal Hyperplasia, Congenital (drug therapy, etiology, metabolism)
• Aging
• Child
• Child, Preschool
• Cortisone (analogs & derivatives, therapeutic use)
• Humans
• Hydrocortisone (therapeutic use)
• Hydroxyprogesterones (blood)
• Infant
• Infant, Newborn
• Radioimmunoassay
• Steroid Hydroxylases (deficiency)