Indicators of adult height outcome in classical 21-hydroxylase deficiency congenital adrenal hyperplasia.
OBJECTIVE
To obtain objective information on the relationship between adult height (AH), glucocorticoid (GC) dose, and degree of hormonal suppression in a population of patients with 21-hydroxylase deficiency congenital adrenal hyperplasia (21-OHD CAH) to optimize treatment regimes.


METHODS
Multicenter retrospective chart review of patients with salt wasting 21-OHD CAH diagnosed in the first 6 months of life, and who had reached AH (n = 54). The data were compiled into a single database.


RESULTS
Mean adult height standard deviation score - midparental height standard deviation score was -1.1 for both sexes. Growth velocity was normal during childhood but compromised during infancy and puberty. Onset and tempo of puberty were normal-to-delayed. Bone age was closely correlated with chronologic age (r = 0.93). AH was negatively correlated with androstenedione in infancy (r -0.68; P =.03) and childhood (-0.66; P <.01) and with testosterone in childhood (r -0.44; P =.01), but not with dehydroepiandrosterone or 17-hydroxyprogesterone. GC dose was not associated with AH.


CONCLUSIONS
Mean AH was in the lower range of genetic potential in this group of persons with 21-OHD CAH. Androgen levels should be used in conjunction with growth velocity measurements to optimize GC dosing in persons with 21-OHD CAH.
Department of Pediatrics, Children's Hospital of Eastern Ontario, University of Ottawa, Ontario, Canada.
Sarah
S
Muirhead
Elizabeth A C
EA
Sellers
Harvey
H
Guyda
D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000201:enzymology* / Q000503:physiopathology; D000328:Adult; D000365:Age Determination by Skeleton; D015415:Biomarkers / Q000097:blood; D001827:Body Height / Q000187:drug effects / Q000502:physiology*; D002170:Canada; D002648:Child; D002669:Child Welfare; D002675:Child, Preschool; D004305:Dose-Response Relationship, Drug; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006128:Growth / Q000187:drug effects; D006801:Humans; D007223:Infant; D007229:Infant Welfare; D007231:Infant, Newborn; D008297:Male; D008901:Mineralocorticoids / Q000627:therapeutic use; D012189:Retrospective Studies; D013223:Statistics as Topic; D013255:Steroid 21-Hydroxylase / Q000187:drug effects; D013739:Testosterone / Q000097:blood; D016896:Treatment Outcome
D015415:Biomarkers; D005938:Glucocorticoids; D008901:Mineralocorticoids; D013739:Testosterone; D019326:17-alpha-Hydroxyprogesterone; D013255:Steroid 21-Hydroxylase