The Effect of Therapy on Mature Height in Congenital Adrenal Hyperplasia*

The effects of therapy on the final height of children with congenital adrenal hyperplasia (CAH) were evaluated. Forty-six children with the 21-hydroxylase defect, 30 of whom had defects in electrolyte metabolism, were followed to maturity. In group A, there were 19 children (11 females and 8 males) who were diagnosed and adequately treated at less than 3 yr of age. In group B, there were 9 children (8 females and 1 male) who were adequately treated, but treatment was begun after 3 yr of age. In group C, there were 15 children (10 females and 5 males) whose treatment was inadequate. In group D, there were 3 female pseudohermaphrodites reared as males. The mean ages at diagnosis were: group A, 0.60 yr (±0.79 sd); group B, 6.43 yr (±2.96 sd); and group C, 4.96 yr (±5.19 sd). The group A males (final height, 171.9 cm ± 2.8 se) and females (final height, 162.5 cm ± 1.6 se) were significantly taller than the corresponding group C children (males, 160.14 cm ± 2.4 se, P < 0.01; females, 145.64 cm ± 2.89 se, P < 0.005) as regards mean final height. Group B females (159.86 cm ± 2.5 se) did not differ significantly from group A females in mean final height, but group B females were different from the U.S mean for females (18.25–19 yr; P < 0.05). Group A males and group A females were not significantly different from the U.S. mean for males and females, respectively (18.25–19 yr; P > 0.05 and P > 0.1, respectively). Group C males and females were significantly different from the U.S. mean for respective males or females 18.25–19 yr (P < 0.001). Group A females were not significantly different from group D subjects (162.35 cm ± 3.8 se). Anticipated final height of the females in Group B may reflect the different treatment method or the possibility that they may have a milder form of the disease. The patients who were adequately treated had greater final heights than those who were inadequately treated.