The congenital adrenal hyperplasia population seems to have an intrinsic tendency to a high frequency of low bone mass. However in this single-center and long-term evaluated cohort, the simplified corticoid regimen, with exclusive dexamethasone single dose reposition during adulthood, did not represent a risk factor for decrease in bone health. INTRODUCTION The impact of long-term and supposedly physiological doses of gluco and mineralocorticoid (GC/MC) on bone mineral density (BMD) in congenital adrenal hyperplasia (CAH) remains discordant among studies, which contain different clinical forms and corticoid regimens. Our aim was to evaluate the BMD in CAH adults receiving similar GC regimen since childhood and to correlate it with GC/MC cumulative doses. METHODS Only patients with good compliance, who used cortisone acetate (CA) during childhood and dexamethasone after the final height achievement. Cumulative GC/MC doses were calculated from diagnosis until last evaluation. BMD was analyzed by the first and last energy X-ray absorptiometry (DXA) scans performed. RESULTS Twenty simple virilizing (SV) and 14 salt wasting (WS) whose mean age was 26 ± 6 years, mean CA, dexamethasone, and fludrocortisone cumulative doses were 63,813 ± 32,767, 812 ± 558, and 319 ± 325 mg/m2, respectively. Based on the last DXA, low BMD was observed in 11% of patients, total hip Z-score was lower in the SW than SV form (p = 0.04). Cumulative CA dose had an inverse correlation with femoral neck Z-score (p < 0.01). Total cumulative GC and MC doses had an inverse correlation with total hip Z-score (p < 0.01). In the analysis of sequential BMD during dexamethasone therapy, no association was observed among cumulative GC/MC doses, clinical forms, sex, and lumbar Z-score delta. CONCLUSIONS Even though a low CA regimen during growth periods in addition to MC replacement appears to have an influence on BMD at femoral sites, interestingly a low dexamethasone one does not seem to be deleterious for bone health in adulthood.

中文翻译：

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糖皮质激素替代疗法对骨矿物质密度的现实影响：跨越24年的大型单中心CAH队列的回顾性经验。

先天性肾上腺增生人群似乎具有固有的趋势，即高频率出现低骨量。但是，在这一单中心且经过长期评估的队列中，简化的皮质类固醇疗法以及成年期独家地塞米松单剂量重新定位并不代表降低骨骼健康的危险因素。简介长期和假定生理剂量的葡萄糖和盐皮质激素（GC / MC）对先天性肾上腺皮质增生（CAH）中骨矿物质密度（BMD）的影响在研究中仍然不一致，这些研究包含不同的临床形式和皮质类固醇疗法。我们的目的是评估自儿童期以来接受类似GC方案的CAH成人的BMD，并将其与GC / MC累积剂量相关联。方法只有依从性好的患者，在儿童期使用醋酸可的松（CA），在达到最终身高后使用地塞米松。从诊断到最后一次评估，计算累积的GC / MC剂量。通过执行的第一个和最后一个能量X射线吸收仪（DXA）扫描分析BMD。结果二十次单纯性杀菌（SV）和14次盐消耗（WS），平均年龄为26±6岁，平均CA，地塞米松和氟可的​​松累积剂量分别为63,813±32,767、812±558和319±325 mg / m2 。根据上一次DXA，在11％的患者中观察到低BMD，SW中的总髋部Z评分低于SV形式（p = 0.04）。累积CA剂量与股骨颈Z分数呈负相关（p <0.01）。总累积GC和MC剂量与总髋部Z分数呈负相关（p <0.01）。在地塞米松治疗期间的顺序BMD分析中，未观察到累积的GC / MC剂量，临床形式，性别和腰Z评分增量之间的关联。结论尽管在MC置换过程中生长期间低CA方案似乎对股骨骨密度有影响，但有趣的是，低地塞米松似乎对成年后的骨骼健康无害。