Dexamethasone treatment of virilizing congenital adrenal hyperplasia: the ability to achieve normal growth.
OBJECTIVE
To assess whether treatment of virilizing congenital adrenal hyperplasia (CAH) with long-acting glucocorticoids is associated with favorable growth outcomes.


METHODS
We examined the long-term growth of 17 boys and 9 girls with CAH treated with dexamethasone (.27 +/-.01 mg/m(2)/day).


RESULTS
For individuals with comparable bone age (BA) and chronological age (CA) at the onset of dexamethasone therapy, males were 2.8 +/-.8 years (mean +/- standard error of the mean; n = 13) and females were 2.4 +/- 1.0 years (n = 6). Males were treated for 7.3 +/- 1.1 years (DeltaCA) over which time the change in BA (DeltaBA) was 7.0 +/- 1.3 years, and the change in height age (DeltaHA) was 6.9 +/- 1.1 years. Females were treated for 6.8 +/- 1.3 years, over which time the DeltaBA was 6.5 +/- 1.0 years, and the DeltaHA was 6.3 +/-.8 years. During treatment 17 ketosteroid excretion rates were normal for age and 17-hydroxyprogesterone values were 69.6 +/- 18 ng/dL. Testicular enlargement was first detected at 10.7 +/-.8 years and breast tissue at 9.9 +/- 1.2 years. Three boys and 1 girl had final heights of 171. 8 +/- 6 cm and 161 cm, respectively, compared with midparental heights of 176.1 +/- 4.1 cm and 160 cm. Predicted adult heights for 6 other boys and 5 girls were 176.8 +/- 2.0 cm and 161.4 +/- 2.8 cm, respectively, compared with midparental heights of 174.6 +/- 1.4 cm and 158.2 +/- 2.0 cm. Statural outcomes were less favorable for 7 children started on dexamethasone when BAs were considerably advanced, although height predictions increased during therapy.


CONCLUSIONS
These observations show that children treated with dexamethasone for CAH can achieve normal growth with the convenience of once-a-day dosing in most cases.congenital adrenal hyperplasia, dexamethasone, growth.
Rivkees
S A
SA
Section of Pediatric Endocrinology, Department of Pediatrics, Yale University Medical School, New Haven, Connecticut, USA. scott.rivkees@yale.edu
Crawford
J D
JD
D015068:17-Ketosteroids / Q000652:urine; D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000365:Age Determination by Skeleton; D001846:Bone Development / Q000187:drug effects; D002675:Child, Preschool; D003907:Dexamethasone / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D005938:Glucocorticoids / Q000494:pharmacology / Q000627:therapeutic use*; D006128:Growth / Q000187:drug effects*; D006801:Humans; D008297:Male
D015068:17-Ketosteroids; D005938:Glucocorticoids; D019326:17-alpha-Hydroxyprogesterone; D003907:Dexamethasone; D000324:Adrenocorticotropic Hormone