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

Abstract

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. In the salt-wasting group, women were almost significantly endowed with a better BMD than men (PZ0.053). We found negative effects of TCG, TAG on lumbar (P!0.001, PZ0.002) and femoral T-scores (PZ0.006, P!0.001), predominantly during puberty. BMI was protective on BMD (PZ0.006). 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.European Journal of Endocrinology 158 879-887

Determining clinical and biological indicators for health outcomes in adult patients with childhood onset of congenital adrenal hyperplasia

“…In fact, 60% of the participants had bone demineralization according to the WHO criteria, which is superior to a Gaussian repartition, in which 16% of the general population is under K1 S.D. (15). We recently conducted a trial to establish the role of the glucocorticoid TCD on BMD (15).…”

“…To determine the total cumulative doses (TCDs) of glucocorticoid and mineralocorticoid, all daily treatment and annual heights and weights were noted for each patient from day 1 of the diagnosis to the day of clinical, hormonal, and anatomical evaluation based on both pediatric and adult files, as previously described (15). Doses of the various glucocorticoids were converted to growth-retarding cortisol equivalents (1 mg of dexamethasoneZ16 mg of prednisoneZ80 mg of hydrocortisone) (15,16,17).…”

“…Doses of the various glucocorticoids were converted to growth-retarding cortisol equivalents (1 mg of dexamethasoneZ16 mg of prednisoneZ80 mg of hydrocortisone) (15,16,17). Each year, we summed every daily dose of treatment and divided the total by the corresponding body area to obtain the annual cumulative doses of hydrocortisone (mg/m 2 per year).…”

Non-classic congenital adrenal hyperplasia

“…Thus, DXA can underestimate bone mineral density in shorter individuals [64]. Available data, derived from outcome reports for individuals with classic CAH, are inconsistent due to varying glucocorticoid doses, potential compliance issues, and subject heterogeneity [65,66]. In theory, inadequate treatment would lead to androgen excess that would be anticipated, in turn, to increase BMD.…”

An Evidence-Based Model of Multidisciplinary Care for Patients and Families Affected by Classical Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency

“…A review of data reveals contradictory results of the potentially adverse effects of chronic glucocorticoid replacement on bone health in both children and adults with CAH. Several studies in adults reveal decreased bone mineral density (BMD) with treatment [50–53], while others report no effect [54, 55]. Similarly, in young adults and children with CAH some studies fail to detect an effect of glucocorticoid replacement [56, 57] while others observe decreased BMD [58].…”