6533b86dfe1ef96bd12c936d

RESEARCH PRODUCT

Bone mineral density and bone turnover in Romanian children and young adults with classical 21-hydroxylase deficiency are influenced by glucocorticoid replacement therapy

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Summary Objective  It remains controversial if glucocorticoid replacement therapy impairs bone mineral density (BMD) in young patients with 21-hydroxylase deficiency. We aimed to analyze the impact of treatment variables, phenotype and genotype on BMD and bone metabolism in these patients. Design  Cross-sectional study. Measurements  Twenty-eight Caucasian patients with classical 21-hydroxylase deficiency (5–39 years). Clinical parameters, hormonal status, osteocalcin (OC), C-terminal telopeptide of type I collagen (CTX), genotype and lumbar BMD (Z-scores) were assessed. Cumulative and mean hydrocortisone equivalent doses were calculated for the entire treatment period. Results  Patients with severely reduced BMD Z-scores (≤–2·5 SD) had significantly higher mean/cumulative glucocorticoid doses compared to patients with moderately reduced (P = 0·003/P = 0·026) and normal Z-scores (> –1 SD) (P = 0·005/P = 0·011). Mean hydrocortisone equivalent doses > 20 mg/m2/day led to significantly lower lumbar BMD Z-scores (–2·16 ± 1·4 SD) vs. doses ≤ 20 mg/m2/day (–0·59 ± 1·25 SD) (P = 0·008). BMD correlated negatively with mean/cumulative glucocorticoid doses and treatment duration. OC (86·45 ± 37·45 ng/ml) and CTX (1·45 ± 0·43 ng/ml) were significantly increased compared to an age- and sex-matched control group in patients with active growth; only CTX was slightly increased in patients who completed growth. Conclusions  High cumulative and mean glucocorticoid doses negatively impact on BMD in children and young adults with classical 21-hydroxylase deficiency. Substitution therapy should be adapted particularly at this life period to prevent bone loss.