Abstract
Conventional therapy of CAH does not yet achieve normal adult height (NEJM 299:1392, '78). We postulated that standard hydrocortisone (F) dose schedules might yield periods of undertreatment or overtreatment throughout the circadian cycle, and that an optimal dose schedule might improve the results of treatment. We therefore evaluated the adrenal suppressive effect of 5 dose schedules of F in 6 patients with 21-hydroxylase deficiency. The dose was 12.5/m2/d, given in a random sequence for 6 weeks according to each of the following schedules: I, 3/3(AM); II, 2/3 (AM), 1/3(HS); III, 1/3 (AM), 1/3 (NOON), 1/3 (HS); IV, 1/3 (AM), 2/3 (HS); V, 3/3 (HS). We collected blood Q2H×24 hrs during the last day of each schedule to measure plasma 17 hydroxyprogesterone (170HP) and F. We collected a 24-hr urine to measure pregnanetriol (PTRIOL), 17 ketosteroids (17KS) and urine free F (UFC) (mean ± SEM):
None of the schedules caused a significant improvement of plasma 170HP or its urinary metabolites. We conclude that the total daily dose of hydrocortisone appears to determine the degree of adrenal suppression irrespective of the dose schedule.
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Winterer, J., Chrousos, G., Loriaux, D. et al. THE ADRENAL SUPPRESSIVE EFFECT OF HYDROCORTISONE IN CONGENITAL ADRENAL HYPERPLASIA (CAH) IS NOT AFFECTED BY VARYING THE DOSE SCHEDULE. Pediatr Res 18 (Suppl 4), 180 (1984). https://doi.org/10.1203/00006450-198404001-00521
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DOI: https://doi.org/10.1203/00006450-198404001-00521