Reduced bone mineral density and increased bone metabolism rate in young adult patients with 21-hydroxylase deficiency.
BACKGROUND
Patients with congenital adrenal hyperplasia (CAH) receive glucocorticoids as replacement therapy. Glucocorticoid therapy is the most frequent cause of drug-induced osteoporosis.


OBJECTIVE
The objective of the study was to evaluate bone mineral density (BMD) and bone metabolism in CAH patients.


METHODS
This was a cross-sectional observational study.


METHODS
The study was conducted at a referral center for pediatric endocrinology.


METHODS
Thirty young patients with the classical form of CAH (aged 16.4-29.7 yr) treated with glucocorticoid from diagnosis (duration of treatment 16.4-29.5 yr) and 138 healthy controls (aged 16.0-30.0 yr) were enrolled.


METHODS
BMD was measured in the lumbar spine and whole body by dual-energy x-ray absorptiometry. Bone formation and resorption rates were estimated by serum measurements of bone-specific alkaline phosphatase and C-terminal telopeptide of type I collagen, respectively.


RESULTS
CAH patients were shorter than controls (women -6.8 and men -13.3 cm). Therefore, several methods were used to account for the effect of this difference on bone measurements. Whole-body BMD measurements were significantly lower, compared with controls (P < 0.03), after controlling for height (on average -2.5% in females and -9.3% in male patients). No differences were found in lumbar spine measurements. Bone-specific alkaline phosphatase and C-terminal telopeptide of type I collagen serum concentrations were higher in CAH patients than control subjects (P < 0.04). BMD measurements and bone metabolism markers did not correlate with the actual glucocorticoid dose or mean dose over the previous 7 yr.


CONCLUSIONS
Young adult patients with the classical form of CAH have decreased bone density values, compared with healthy controls. This may put them at risk of developing osteoporosis early in life.
Sciannamblo
Mariateresa
M
Laboratory of Pediatric Endocrinology and Department of Pediatrics, San Raffaele Scientific Institute, Vita-Salute S. Raffaele University, Milan, Italy.
Russo
Gianni
G
Cuccato
Debora
D
Chiumello
Giuseppe
G
Mora
Stefano
S
D015502:Absorptiometry, Photon / Q000379:methods; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy / Q000378:metabolism*; D000328:Adult; D015415:Biomarkers / Q000032:analysis; D015519:Bone Density; D001842:Bone and Bones / Q000378:metabolism*; D016022:Case-Control Studies; D003430:Cross-Sectional Studies; D005260:Female; D005938:Glucocorticoids / Q000009:adverse effects / Q000627:therapeutic use; D020249:Hormone Replacement Therapy / Q000009:adverse effects; D006801:Humans; D008297:Male; D010024:Osteoporosis / Q000139:chemically induced
D015415:Biomarkers; D005938:Glucocorticoids