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Impact of total cumulative glucocorticoid dose on bone mineral density in patients with 21-hydroxylase deficiency.

¹ AP-HP, Department of Endocrinology and Reproductive Medicine, Groupe Hospitalier Pitié-Salpêtrière, 47-83 boulevard de l'Hôpital, 75651 Paris Cedex 13, France² Department of Pediatric Endocrinology,, Hôpital Necker-Enfants Malades, Paris, France³ Department of Endocrinology,, Hôpital Saint-Antoine, Paris, France⁴ Department of Pediatric Endocrinology,, Hôpital Saint-Vincent de Paul, Paris, France⁵ Department of Pediatric Endocrinology,, Hôpital Trousseau Malades, Paris, France⁶ Departments of Pediatric Endocrinology,, Hôpital Robert Debré, Paris, France and ⁷ Inserm U845,, University Paris-Descartes, Faculty of Medicine René Descartes, 75015 Paris, France; on behalf of Centre des Maladies Endocriniennes Rares de la Croissance and Association Surrénales

(Correspondence should be addressed to P Touraine; Email: philippe.touraine{at}psl.aphp.fr)

Objective: It remains controversial whether long-term glucocorticoids are charged of bone demineralization in patients with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The aim of this study was to know whether cumulative glucocorticoid dose from the diagnosis in childhood to adulthood in patients with CAH had a negative impact on bone mineral density (BMD).

Design: This was a retrospective study.

Methods: Thirty-eight adult patients with classical and non-classical CAH were included. BMD was measured in the lumbar spine and femoral neck. Total cumulative glucocorticoid (TCG) and total average glucocorticoid (TAG) doses were calculated from pediatric and adult files.

Results: We showed a difference between final and target heights (–0.82±0.92 S.D. for women and –1.31±0.84 S.D. for men; P<0.001). Seventeen patients (44.7%) had bone demineralization (35.7% of women and 70% of men). The 28 women had higher BMD than the 10 men for lumbar (–0.26±1.20 vs –1.25±1.33 S.D.; P=0.02) and femoral T-scores (0.21±1.30 S.D. versus –1.08±1.10 S.D.; P=0.007). In the salt-wasting group, women were almost significantly endowed with a better BMD than men (P=0.053). We found negative effects of TCG, TAG on lumbar (P<0.001, P=0.002) and femoral T-scores (P=0.006, P<0.001), predominantly during puberty. BMI was protective on BMD (P=0.006).

Conclusion: The TCG is an important factor especially during puberty for a bone demineralization in patients with 21-hydroxylase deficiency. The glucocorticoid treatment should be adapted particularly at this life period and preventive measures should be discussed in order to limit this effect.