Abstract
Most studies have shown that patients with virilizing CAH are ultimately shorter than their peers. Although great progress has been made in the treatment of CAH, standard criteria for the management of this condition have not been developed. To assess current treatment practice for children with CAH with respect to drug, dose, dose schedule and criteria for dose adjustment, we polled by questionnaire all members of the LWPES. This summary is based on a response of 35%.
Most respondents use hydrocortisone (76%), Some prefer cortisone acetate (17%) and a few use prednisone (7%). Of those using hydrocortisone, 1% give 5-10 mg/m2/day, 63% give 10-20 and 36% give 20-30 mg/m2/day. Treatment is given on a tid schedule by the majority (66%), the rest use a bid schedule. None give hydrocortisone or cortisone once daily. Many distribute drug evenly over the day (52%), while others prefer a larger pm dose (30%) or a larger am dose (18%). The main indications for using Florinef are an elevated PRA and salt-wasting. Some, (15%)give it to all non-hypertensive patients.
Most respondents adjusted the dose of glucocorticoid on the basis of plasma (17-OH P &/or delta-4 A) and urine (17KS &/or P triol) measures together with growth and maturation rates. Some however, rely only on serum levels (10%) or on urine measurements (5%). A few prefer to depend solely on rates of growth and maturation.
This survey supports the hypothesis that regimens used to treat CAH vary widely, particularly with regard to scheduling of medication. Thus, studies comparing different approaches to management with objective measures of outcome, such as growth rates and ultimate stature, seem indicated.
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Feuillan, P., Cutler, G., Lynn Loriaux, D. et al. 110 CURRENT MANAGEMENT OF CONGENITAL ADRENAL HYPERPLASIA (CAH): A SURVEY OF THE LAWS0N WILKINS PEDIATRIC ENDOCRINE SOCIETY (LWPES). Pediatr Res 19, 621 (1985). https://doi.org/10.1203/00006450-198506000-00130
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DOI: https://doi.org/10.1203/00006450-198506000-00130