Effect of the dose of oral hydrocortisone on growth rate during long-term treatment of children with salt losing congenital adrenal hyperplasia

RESUMO

The effect of the dose of oral hydrocortisone on stature growth rate of patients with the salt losing form of congenital adrenal hyperplasia and adequate electrolyte balance was here assessed. Thirty patients (21 girls and 9 boys) were followed longitudinally for 0.52 to 8.64 years, between chronological ages 0.35 and 8.64 years. Nine consecutive periods (Ps) of follow up were defined in order to compare two auxological parameters, height (H) at the end of a follow up P and DH standard deviation score (SDS). According to growth rate during a particular P, two types of Ps were defined Ps with DH SDS > -0.5 (Group 1, satisfactory growth rate) and Ps with DH SDS = or < -0.5 (Group 2, poor growth rate). A cut off value of 18.5 mg/m 2 /day of oral hydrocortisone (95% CI upper limit of group 1) was defined to separate acceptable from excessive doses. In P2, mean (± SD) H SDS (-1.81 ± 1.15) was significantly lower than in any of the other Ps (p < 0.001). In P1 and in P2, DH SDS was negative, but it was positive in P3 and in P4. Hydrocortisone dose in P1 and in P2 was significantly higher than in the rest of the Ps. All patients in P1 and most patients in P2 , but not in other Ps, received excessive doses. Predicted adult H, calculated in 9 patients was not statistically different from their respective target H. It is concluded that,during the first year of life, our patients received an excess of oral hydrocortisone (> 18 mg/m 2 /day) and grew poorly, but they were able to recover, at least temporarily, when the dose was adjusted during the following years