LISBON -- Women who receive long-term glucocorticoid treatment for congenital adrenal hyperplasia due to 21-hydroxylase deficiency are at risk for decreased bone mineral density, Jeremy A. King, M.D., reported in a poster presentation at the 12th International Congress of Endocrinology.
The risk of decreased bone mineral density (BMD) is particularly high among postmenopausal women with the salt-losing form of 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH). The best way to prevent the problem is to avoid oversuppression of adrenal androgens via careful monitoring, said Dr. King of Johns Hopkins University, Baltimore.
Morphologic measurements, serum hormone assays, and BMD assessments were performed in 11 adult women with the salt-losing (SL) form of CAH who had been receiving glucocorticoid replacement therapy since infancy, and in 15 women with the simple virilizing (SV) form of CAH, who had begun glucocorticoid treatment at ages varying from infancy to 22 years. The measurements were also performed in 15 controls, 9 of whom were unaffected sisters (US) of the subjects, and 6 with polycystic ovarian syndrome (PCOS).
Subjects from both CAH groups were significantly shorter than the controls. (See table.) All bone parameters, including Z-score, T-score, and L-spine, were lower in the CAH group.
In addition, levels of 17-hydroxyprogesterone, androstenedione, dehydroepiandrosterone (DHEA) and dehydroepiandrosterone sulfate (DHEAS) were also all significantly lower in the two CAH groups than in the controls. Dr. King reported.
Not surprisingly, adrenal androgens and bone parameters were lower among the 12 postmenopausal CAH patients, compared with the 14 still premenopausal women, he said.
Osteopenia, defined as a T-score between -1 and -2.5, was present in 5 of the 11 SL patients (45%) and 2 of the 15 SV patients (13%), compared with just 1 of the 15 controls (7%). There were no differences in age, menopausal status, or cortisol equivalents between the CAH patients with and without osteopenia. However, adrenal androgens were more suppressed in the osteopenic group, he noted.
Body mass index also did not differ between the osteopenic and nonosteopenic CAH patients, suggesting that increased BMI alone does not confer protection from low BMD in this population. The SL group had a mean BMI less than that of the controls with PCOS but greater than the US controls. Yet, the SL group still had lower lumbar BMD and lower Z-scores compared with both control groups.
BY MIRIAM E. TUCKER
COPYRIGHT 2004 International Medical News Group
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