Abstract
In CAH with deficient 21-hydroxylation there is impaired cortisol (F) biosynthesis resulting in elevated plasma levels of 17-hydroxyprogesterone(17-OHP) and urinary excretion of pregnanetriol. Increased urinary excretion of 11-ketopregnanetriol also occurs, but data concerning plasma levels of the precursor 21-deoxyF are limited, and the purpose of the current study was the measurement of plasma levels of 17-OHP, 21-deoxyF and F in patients with CAH.
Plasma samples were obtained from twelve patients (3d-40yr) with CAH before and three after treatment, and from four normal controls (4d-18yrs) following ACTH administration. Steroids were isolated by Sephadex LH-20 column chromotography of plasma extracts and quantitated by RIA.
Plasma 17-OHP was 2.2-5.7 fold higher than 21-deoxyF in each patient. In all untreated patients, the levels of both steroids were elevated, without relation to age or variant of CAH, and decreased with therapy. Mean values for patients were significantly different from those for normal controls.
Conclusion: Patients with untreated CAH have elevated plasma levels of not only 17-OHP but also 21-deoxyF, and the concentration of 17-OHP exceeds that of 21-deoxyF in individual patients of all ages.
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Franks, R. PLASMA 17-HYDROXYPROGESTERONE, 21-DEOXYCORTISOL AND CORTISOL IN CONGENITAL ADRENAL HYPERPLASIA (CAH). Pediatr Res 8, 368 (1974). https://doi.org/10.1203/00006450-197404000-00170
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DOI: https://doi.org/10.1203/00006450-197404000-00170