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在对受21-羟化酶缺乏影响的患者的护理中,骨骼健康应是一个重要关注点。

Bone health should be an important concern in the care of patients affected by 21 hydroxylase deficiency.

作者信息

Bachelot Anne, Chakhtoura Zeina, Samara-Boustani Dinane, Dulon Jérome, Touraine Philippe, Polak Michel

机构信息

AP-HP, Department of Endocrinology and Reproductive Medicine, Groupe Hospitalier Pitié-Salpétrière, 47-83 boulevard de l'Hôpital, 75013 Paris Cedex 13, France.

出版信息

Int J Pediatr Endocrinol. 2010;2010. doi: 10.1155/2010/326275. Epub 2010 Sep 28.

Abstract

Osteoporosis has been an understandable concern for children and adult patients with congenital adrenal hyperplasia (CAH) who may receive or have received supraphysiological doses of glucocorticoids. Some previous reports on bone mineral density (BMD) in adult CAH patients showed no significant differences in BMD between patients with CAH and controls, but others have found lower BMD in CAH patients. In reports documenting the BMD reduction, this outcome has been attributed to an accumulated effect of prolonged exposure to excess glucocorticoids during infancy and childhood. We recently conducted a trial to establish the role of the total cumulative glucocorticoid dose on BMD. We established for the first time that there was a negative relationship between total cumulative glucocorticoid dose and lumbar and femoral BMD. Women might benefit from the preserving effect of estrogens compared to men. BMI (Body Mass Index) also appeared to protect patients from bone loss. In light of this, physicians should bear in mind the potential consequences of glucocorticoids on bone and therefore adjust the treatment and improve clinical and biological surveillance from infancy. Furthermore, preventive measures against corticosteroid-induced osteoporosis should be discussed right from the beginning of glucocorticoid therapy.

摘要

骨质疏松症一直是患有先天性肾上腺皮质增生症(CAH)的儿童和成年患者的一个可理解的担忧,他们可能已经接受或正在接受超生理剂量的糖皮质激素治疗。之前一些关于成年CAH患者骨密度(BMD)的报告显示,CAH患者与对照组之间的骨密度没有显著差异,但其他研究发现CAH患者的骨密度较低。在记录骨密度降低的报告中,这一结果归因于婴儿期和儿童期长期暴露于过量糖皮质激素的累积效应。我们最近进行了一项试验,以确定糖皮质激素总累积剂量对骨密度的作用。我们首次确定,糖皮质激素总累积剂量与腰椎和股骨骨密度之间存在负相关关系。与男性相比,女性可能从雌激素的保护作用中受益。体重指数(BMI)似乎也能保护患者避免骨质流失。鉴于此,医生应牢记糖皮质激素对骨骼的潜在影响,因此从婴儿期就应调整治疗方案并加强临床和生物学监测。此外,应从糖皮质激素治疗开始就讨论预防皮质类固醇诱导的骨质疏松症的措施。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fba6/2948879/a1f1d6002f70/IJPE2010-326275.001.jpg

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