Abstract
Patients with late-onset congenital adrenal hyperplasia(LOCAH)due to partial 21-hydroxylase deficiency have no clinical evidence of hypocortisolism, unlike patients with classical forms of CAR, who have elevated plasma ACTH and low serum cortisol levels. In order to study the interactions of the various components of the hypothalamic-pituitary-adrenal axis in LOCAH. we administered ovine corticotropin releasing hormone(oCRH), 1 ug/kg, at 20:00h to 10 untreated patients with LOCAH (8 females, 2 males, ages 7-35y). Baseline(basal, B) and oCRH-stimulated(peak, P) ACTH and cortisol responses did not differ from normal and the integrated ACTH:cortisol response ratio was also normal. Stimulated serum 17-hydroxyprogesterone responses were significantly greater than normal (p<0.005). Stimulated serum Δ 4-androstenedione responses in 5 postpubertal female patients moderately exceeded those of 5 age-matched control females(p=NS). [Data shown as mean + SBM]
We conclude that patients with LOCAH compensate for the cortisol biosynthetic defect at the expense of a moderate increase in adrenal androgen secretion. The normal ACTH and cortisol responses to oCRH suggest that a new equilibium has been established between the pituitary and the adrenal gland.
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Feuillan, P., Avgerinos, P., Schürmeyer, T. et al. ADAPTATION OF THE HYPOTHALAMIC-P ITU ITARY-ADRLNAL AXIS IN LATE-ONSET CONGENITAL ADRENAL HYPERPLASIA. Pediatr Res 21 (Suppl 4), 247 (1987). https://doi.org/10.1203/00006450-198704010-00478
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DOI: https://doi.org/10.1203/00006450-198704010-00478