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Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada T6G 2R7
Address all correspondence and requests for reprints to: Rose Girgis, Pediatric Endocrinology Fellow, Division of Endocrinology and Metabolism, Department of Pediatrics 2C3 WMC, University of Alberta, 8440–112 Street, Edmonton, Alberta, Canada T6G 2R7.
Even with current so called physiologic doses of glucocorticoid
replacement therapy, children with congenital adrenal hyperplasia (CAH)
often show relative short stature and delayed bone maturation, an
observation that suggests possible long-term effects on bone metabolism
of daily transient post-absorptive hypercortisolemia. In 28 patients
with 21-hydroxylase or 17 
-hydroxylase deficiency (16 females and 12
males, ages 4.9–22 yr) who had received oral cortisol 10–15
mg/M2/day for 4.7–22 yr, we studied cortisol
bioavailability, growth, bone maturation, vertebral bone mineral
density, and various markers of bone formation and resorption. Patients
were grouped according to mean on-therapy serum 170H-progesterone or
progesterone levels as tight control (170HP < 10 nmol/L), fair
control (170HP 10–40 nmol/L or progesterone 1.0–1.5 nmol/L), or poor
control (170HP > 40 nmol/L). There was no difference in peak
post-absorptive serum cortisol or area under the concentration-time
curve, and only three patients had a peak serum cortisol of more than
700 nmol/L. There was no difference in present height Z-score (-0.96;
-0.24; -0.6), height Z-score at age 2 yr (-1.5; +0.4; -1.3), or
current growth velocity Z-score (-0.1; +1.2; -2.2) between the
groups, but bone maturation Z-score was significantly delayed (-1.63)
in the tight control group and advanced (+0.8) in the poor control
group. Present height was highly correlated (r = 0.8) with height
at age 2 yr. Serum calcium, phosphorus, alkaline phosphatase,
parathormone, and 25OH-vitamin D levels were all normal. There was no
difference between the groups in age-corrected vertebral bone mineral
density, and no difference in serum osteocalcin, procollagen peptide,
or collagen C-terminal telopeptide, nor in urinary amino-terminal
telopeptide. The data suggest that current methods of cortisol
replacement do not significantly influence bone formation, resorption
or density during childhood and therefore should not contribute to
adult osteoporosis. The possibility remains that hypercortisolemia
during infancy produces the short stature and delayed bone maturation
that are present by the age 2 yr.
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