Abstract

Previous studies have demonstrated the usefulness of serum 17-OHP determinations in children with CAH. Recent reports have suggested that serum levels of DS, an adrenal androgen, might better reflect degree of control and be simpler to measure. Hourly serum samples x 24 hr were assayed for 17-OHP and DS in 6 patients aged 10-19 yrs with 21-hydroxylase deficiency (salt losing, N=4; non-salt losing, N=2) during therapy with Prednisone (P) and after glucocorticoid treatment had been withdrawn for 3 days. After withdrawal of P the 24 hr mean serum 17-OHP levels increased in all patients (64% to 900%) and ranged from 3 mcg/dl to 28 mcg/dl. Urinary 17-ketosteroids increased in 5 of 6 patients. There was no consistent change in mean DS levels which ranged from 1 mcg/dl to 114 mcg/dl on Rx and 6 mcg/dl to 82 mcg/dl off Rx. DS levels increased in 4 patients and decreased in 2 while off Rx. All DS levels on and off Rx were in the normal range for age. Patients considered poorly controlled by urinary 17-ketosteroids and serum 17-OH-P did not have elevated DS. No diurnal changes in DS levels were evident. Serum DS levels from 101 patients aged 2-19 yrs without adrenal pathology were consistent with other reports and showed an increase with age of patient: (X ± S.D.: 2-8 yrs - 29 ± 32; 9-12 yrs = 57 ± 49; 13-19 yrs = 131 ± 78). Our data indicate” that DS concentrations do not become abnormally high after acute withdrawal of glucocorticoid and would not be a reliable indicator of control in children with CAH.

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