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Evaluation of impact of steroid replacement treatment on bone health in children with 21-hydroxylase deficiency

机译:评估类固醇替代治疗对21-羟化酶缺乏症患儿骨骼健康的影响

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摘要

There are conflicting data regarding the potential impact of chronic glucocorticoid (GC) therapy on the bone mineral density of patients with congenital adrenal hyperplasia (CAH). Previous studies performed by dual-energy X-ray absorptiometry reported conflicting results. The purpose of this study was to assess the impact of chronic GC replacement treatment in children with classical and non classical CAH due to 21-hydroxylase deficiency (21-OHD) by quantitative ultrasonometry (QUS), an easy, cheap, and radiation-free technique. The study population consisted of nineteen 21-OHD patients (nine males) on lifelong GC treatment. Anthropometric, hormonal, and treatment data were recorded for each patient, and bone quality was assessed by QUS measurements. QUS findings (amplitude-dependent speed of sound and bone transmission time) were normal in 21-OHD patients and did not correlate with duration of treatment, daily, total, and yearly hydrocortisone dose. Furthermore, no significant correlation was found between QUS findings and 17 alpha-hydroxy progesterone, Delta 4-androstenedione, and testosterone levels. In conclusion, our results provide reassurance that currently used replacement doses of GC do not have a major impact on bone in patients with CAH. QUS seems to be a reliable tool for screening of bone health in children with 21-OHD.
机译:关于慢性糖皮质激素(GC)治疗对先天性肾上腺皮质增生(CAH)患者的骨矿物质密度的潜在影响存在相互矛盾的数据。以前通过双能X射线吸收仪进行的研究报告了相互矛盾的结果。这项研究的目的是通过定量超声检查(QUS)评估21-羟化酶缺乏症(21-OHD)导致的经典和非经典CAH儿童的慢性GC替代治疗的影响,该超声简便易行,便宜且无辐射技术。研究人群包括19位接受终生GC治疗的21-OHD患者(9位男性)。记录每位患者的人体测量,激素和治疗数据,并通过QUS测量评估骨质量。 QUS的发现(与声音有关的速度和骨骼的传播时间)在21-OHD患者中是正常的,并且与治疗持续时间,每日,总剂量和年度氢化可的松剂量无关。此外,在QUS结果与17个α-羟基孕酮,δ4-雄烯二酮和睾丸激素水平之间未发现显着相关性。总之,我们的结果可以保证,目前使用的GC替代剂量对CAH患者的骨骼没有重大影响。 QUS似乎是筛查21-OHD儿童骨骼健康的可靠工具。

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