Abstract
Results obtained from ten prepubertal patients (6 girls, 4 boys) suffering of CVAH were compared over 12-41 months periods before and over 14-52 months periods during CA therapy (63 ± 21 (SD)mg/m2/day). At the beginning of treatment with CA, growth parameters were as follow : chronological (Ch) ages 5 to 10 years, heights (H) 102.5 ± 9.2% of normal H for Ch ages, bone (B) ages 6 to 92 (mean 49) months over Ch ages. CA induced decreases in growth velocity (6.9 ± .66 (SEN) to 4.3 ± .54 cm/year, p < .01), in bone maturation (2.4 ± .76 to .35 ± .11 years/Ch year p < .05) and in B/H ages ratios (1.42 ± .09 to 1.23 ± .06, p < .05). In spite of reduced cortisol therapy from 15.4 ± 1.23, before, to 12.24 ± .72 mg/m2/day, during CA treatment, (p < .05), 17 hydroxyprogesterone (17OHP) and testosterone (T) plasma levels decreased from 62 ± 9.8 to 22.7 ± 7 (p < .01) and from .42 ± .07 to .17 ± .03 (p < .05) respectively. When compared to normal values for B ages, most plasma DHA values were normal before (9/10) and during (7/10), most Δ4 androstenedione (Δ4A) values were elevated before (9/10) and normal (9/10) during CA treatment, while Δ4A/T ratios increased significantly from 3.7 ± .32 to 6.4 ± .86 (p < .01). In conclusion, CA appears to be of interest in the treatment of prepubertal patients with CVAH. The antiandrogenic effect is demonstrated by the improvement of bone maturation, the inhibition of ACTH production by the decreases in 17OHP levels. In addition there is evidence of a decrease in the conversion rates of adrenal androgens to testosterone.
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Raux-Demay, M., Pierret, T. & Girard, F. CONGENITAL VIRILIZING ADRENAL HYPERPLASIA (CVAH) DUE TO 21 HYDROXYLASE DEFICIENCY, RESULTS OF THERAPY WITH CYPROTERONE ACETATE (CA). Pediatr Res 20, 1201 (1986). https://doi.org/10.1203/00006450-198611000-00167
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DOI: https://doi.org/10.1203/00006450-198611000-00167