La. Lopes et al., SHOULD WE MONITOR MORE CLOSELY THE DOSAGE OF 9-ALPHA-FLUOROHYDRO-CORTISONE IN SALT-LOSING CONGENITAL ADRENAL-HYPERPLASIA, Journal of pediatric endocrinology & metabolism, 11(6), 1998, pp. 733-737
In salt-losing congenital adrenal hyperplasia (CAH), continuous therap
y with glucocorticoids and 9 alpha-fluorohydrocortisone (9 alpha-F) re
mains the golden rule. Previous reports showed a growth promoting effe
ct of 9 alpha-F therapy. In addition, 9 alpha-F seemed to have a negli
gible glucocorticoid action. To confirm these facts, we analyzed the c
linical data and the biological markers of control of therapy in two g
roups of patients with salt-losing CAH aged from 2 to 12 years: group
I: before (time 0) and 6 months after the increase in 9 alpha-F dosage
(time +6); group II: at time 0 and time +6 but without change in 9 al
pha-F dosage, Groups were similar in terms of mean age, bone age and h
ydrocortisone dose. The mean dose of 9 alpha-F was 68.2+/-5.0 mu g/m(2
)/d at time 0 and was increased to 98.6+/-7.7 mu g/m(2)/d at time +6 i
n group I; it remained similar in group II. In group I, height velocit
y decreased significantly from 8.1+/-0.6 at time 0 to 6.3+/-0.3 cm/yr
at time +6 (p<0.01) while in group II there was no significant change.
In group I, plasma renin activity decreased from 10.4 +/- 1.6 at time
0 to 3.9+/-1.1 ng/ml/h at time +6 (p<0.005) and showed no change in g
roup II. These preliminary results suggest that careful monitoring of
9 alpha-F is essential to control a proper growth rate.