Growth and Reproductive Outcomes in Congenital Adrenal Hyperplasia

The pediatric endocrinologist has the difficult task of carefully adjusting medications in actively growing children with CAH so as to avoid overtreatment as well as undertreatment. Glucocorticoid excess may result in poor linear growth, weight gain, hypertension, and other unwanted side effects. On the other hand, undertreatment results in excess androgen production and advanced skeletal maturation.An important goal in treating children with CAH is to achieve a normal FH. Until recently, it was generally perceived that children with CAH would ultimately be short as adults and invariably below their genetic potential. However, studies over the last ten years have contrasted this idea (Table 1), suggesting that most children with CAH will have a FH that is below their target height but within 2 standard deviations (SD) of the mean.Studies from 1977 through 1998 [1–17] describing height outcomes in patients with CAH were examined in a meta-analysis with respect to FH, impact of gender, and time of diagnosis [18]. Overall, mean weighted FH SD score (SDS) for all studies was 1.37. In regards to gender, mean FH SDS was 1.57 for males and 1.24 for females. When time at diagnosis was analyzed, mean FH was 1.11 SDS for patients identified early (less than 1 year old) as compared to 1.61 for patients indentified late (after 1 year old). Although not all reports are in agreement, more recent studies [18–33] also indicate that a FH within 2 SD of the mean can be achieved with males tending to have a slightly worse outcome than females (Table 1). Final adult height nearly equal to target height following strict compliance and regular clinic visits every three months has also been reported [34]. Therefore, there is now substantial evidence that with appropriate clinical management, most children with CAH can obtain a FH that is within their genetic potential [18, 35].In growing children, it is well known that an elevated cumulative daily dose of glucocorticoids adversely affects