Plasma 17-Hydroxyprogesterone, 21-Deoxycortisol and Cortisol in Congenital Adrenal Hyperplasia*
Abstract
Plasma concentrations of 17-hydroxyprogesterone, 21-deoxycortisol and cortisol were determined in 12 patients aged 3 days to 40 yr with congenital adrenal hyperplasia (21-hydroxylase defect) and in 4 normal controls aged 4 days to 18 yr administered ACTH. Levels of 17-hydroxyprogesterone were 90 to 528 ng/ml in the untreated patients and 2.4 to 4.8 ng/ml in the controls. 21-Deoxycortisol was not detectable in plasma of controls and levels in patients were 20 to 169 ng/ml. Levels of cortisol were 20 to 107 ng/ml in the patients and 193 to 393 ng/ml in the ACTH-treated normal controls. In the 4 patients with the sodium-losing variant the mean concentrations of 17-hydroxyprogesterone and 21-deoxycortisol were higher and cortisol lower than those for the 8 patients with the simplevirilizing disorder. In untreated congenital adrenal hyperplasia plasma 17-OHP levels exceeded by 2.2-to 5.7-fold those of 21-deoxycortisol without relation to the age of the patient.
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17-hydroxyprogesterone is a substrate for adrenal 21-hydroxylase and is markedly increased in the serum of untreated and insufficiently controlled patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
“It is widely recognized that 17-hydroxy- a substrate for adrenal 21-hydroxylase is progesterone (17-OH-progesterone) which is markedly increased in the serum of untreated and insufficiently controlled patients”
From Serum 17-hydroxypregnenolone and 17-hydroxypregnenolone sulfate concentrations in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. (1988) · cites this paper as Franks, 1974
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- Authors
- FRANKS, R. C.
- Affiliations
- The University of Texas at San Antonio Health Science Center (US)
- Corresponding author
- FRANKS, ROBERT C.
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 1974-12
- Volume
- 39
- Issue
- 6
- Pages
- 1099-1102
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W2055842299
- Cited by
- 53
- References
- 0
- Citations by year
- 2022: 2 · 2019: 1 · 2015: 1
- FWCI
- 1.18
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Reproductive Medicine › Ovarian function and disorders
- Keywords
- congenital adrenal hyperplasia, 21-hydroxylase deficiency, 17-hydroxyprogesterone, 21-deoxycortisol, cortisol, ACTH stimulation test, plasma steroid levels
- MeSH terms
- Adolescent, Adrenocortical Hyperfunction, Adrenal Hyperplasia, Congenital, Adrenocorticotropic Hormone, Adult, Child, Child, Preschool, Cortisone, Cortodoxone, Female, Humans, Hydrocortisone, Mixed Function Oxygenases, Hydroxyprogesterones, Immune Sera, Infant, Male, Prednisone, Pregnadienediols, Radioimmunoassay, Tritium
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