Growth Potential in Congenital Adrenal Hyperplasia(Cah) Due To 21-Hydroxylase Deficiency Treated With Cortisone Acetate(Ca) and Fludrohidrocortisone(Fh)

Optimal therapy for CAH includes replacement of glucocorticoids, mineralocorticoids in salt-wasters(SW), control of hyperandrogenism and skeletal maturation allowing normal growth. Reported final heights in patients treated since infancy have been compromised compared to parental height. Additional benefit from association with FH for nonsalt-wasters has been controversial. AC is known to have the least inhibitory effect on growth and was given in a dose of 18-22 mg/m²/day po divided in three doses, associated to FH, 0.1 -0.15 mg/day during the first year of life and 0.05 - 0.1 mg/day thereafter, to 11 patients with CAH due to 21-hydroxylase deficiency, classical form (10 girls, 1 boy; 8 simple virilizing form (SV) and 3 SW form). Age at diagnosis and start of therapy was 23 days to 2.66 y(median (M)=0.58 y.) and duration of therapy 1.75 to 11 years (M= 7 y.). Biochemical control was considered good (G) when Δ4-androstenedione and testosterone were normal in 3 out of 4 quarterly steroid measurements and regular (R) when 2 out of 4 were normal. We evaluated growth potential by expressing height in standard deviation scores (SDS) for bone age, before and at the last evaluation(LEH) and compared to target height (TH) also expressed in SDS. Table

Table 1