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a Adrenal Steroid Disorders Program, Division of Pediatric Endocrinology, Mount Sinai School of Medicine, New York, New York, USA
Key Words: CAH • 21-hydroxylase deficiency • glucocorticoid • bone mineral density • adrenal steroids • osteoprotegerin • RANK-L
Address for correspondence: Karen Lin-Su, M.D., Mount Sinai School of Medicine, One Gustave L. Levy Place, Box 1198, New York, NY 10029. Voice: 212-241-8210; fax: 212-241-5405. karen.su{at}mssm.edu
The primary treatment for patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is glucocorticoid replacement therapy, which at supraphysiologic levels can result in diminished bone accrual and lead to osteopenia and osteoporosis. Unlike other diseases treated with chronic glucocorticoid therapy, previous studies of patients with 21OHD have not demonstrated a detrimental effect of glucocorticoid treatment on bone mineral density (BMD). It has been postulated that the elevated androgens typically found in these patients have a protective effect on bone integrity, but the precise mechanism remains unknown. We propose that the inhibitory effect of corticosteroid therapy on bone formation is counteracted by estrogen's effect on bone resorption through the RANK-L/osteoprotegerin (OPG) system. A better understanding of the mechanism by which patients with 21OHD are protected against bone loss may lead to novel therapeutic measures to prevent or treat osteopenia and osteoporosis in other conditions, including postmenopausal women.
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