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[21-deoxycortisol. A new marker of virilizing adrenal hyperplasia caused by 21-hydroxylase deficiency].
- Source :
-
Presse medicale (Paris, France : 1983) [Presse Med] 1989 Dec 02; Vol. 18 (40), pp. 1965-9. - Publication Year :
- 1989
-
Abstract
- 21-deoxycortisol is a steroid produced mainly by the adrenal gland. Its normal plasma baseline concentrations (0.03 to 0.30 n/ml) and its concentrations after tetracosactide injection (0.15 to 0.76 ng/ml) do not significantly vary with age, sex and phases of the menstrual cycle. 21-deoxycortisol was assayed in plasma by a specific radioimmunological method and its values were compared with those of 17-OH progesterone in heterozygous subjects with the classical and non-classical forms of 21-hydroxylase deficiency, and in the amniotic fluid of foetuses with this deficiency. Baseline concentrations of 21-deoxycortisol in the classical forms of 21-hydroxylase deficiency (n = 12; 55.36 to 186.6 ng/ml) and post-tetracosactide concentrations in non-classical late onset forms (n = 31; 4.04 to 47 ng/ml) were much higher than in normal subjects, thus making this steroid as sensitive as, or even more sensitive than 17-OH progesterone in diagnosing 21-hydroxylase deficiency. Post-tetracosactide assays of 21-deoxycortisol in 84 heterozygous subjects with 21-hydroxylase deficiency (0.70 to 5.40 ng/ml) enabled these subjects to be detected with a more than 90 percent sensitivity, which cannot be obtained with 17-OH progesterone assays. 21-deoxycortisol concentrations in the amniotic fluid of foetuses with 21-hydroxylase deficiency (n = 11; 0.391 to 0.930 ng/ml) were constantly superior to those observed in normal foetuses (n = 38; 0.034 to 0.221 ng/ml), so that the deficiency can be diagnosed with the steroid as easily as with 17-OH progesterone.
- Subjects :
- 17-alpha-Hydroxyprogesterone
Adult
Amniotic Fluid analysis
Biomarkers analysis
Biomarkers blood
Child
Cortodoxone blood
Cosyntropin
Female
Heterozygote
Humans
Infant, Newborn
Male
Prenatal Diagnosis
Radioimmunoassay
17-Hydroxycorticosteroids analysis
Adrenal Hyperplasia, Congenital blood
Cortodoxone analysis
Hydroxyprogesterones blood
Mixed Function Oxygenases deficiency
Subjects
Details
- Language :
- French
- ISSN :
- 0755-4982
- Volume :
- 18
- Issue :
- 40
- Database :
- MEDLINE
- Journal :
- Presse medicale (Paris, France : 1983)
- Publication Type :
- Academic Journal
- Accession number :
- 2531882