Bone mineral density in Marfan syndrome. A large case-control study
Bone mineral density in Marfan syndrome. A large case-control study
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DOI：
10.1016/j.jbspin.2006.01.026
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发表时间：
2006-12-01
期刊：
影响因子：
4.2
通讯作者：
Le Parc, Jean-Marie
中科院分区：
文献类型：
--
作者：
Moura, Bertrand;Tubach, Florence;Le Parc, Jean-Marie
目的：测量符合马凡综合征 Gand 标准的一组患者的骨密度 (BMD)，并与一组健康对照进行比较。方法：使用双能 X 射线骨密度测定法 (DEXA) 测量法国巴黎多学科马凡诊所就诊的 130 名患者的髋部和腕部骨密度。将结果与吸收测定仪 (Hologic QDR100) 数据库中的值以及 72 名身高匹配的健康对照者的值进行比较，其中 35 名患者的体重指数 (BMI) 值与患者相似。结果：32 名 (24.6%) 患者有骨折史。与 Hologic 数据库值相比，患者股骨颈 (-1.190 +/- 0.098，P < 0.0001) 和腕部 (-1.403 +/- 1.06；P < 0.001) 的 Z 评分值显着降低。与身高匹配的对照组相比，患者股骨颈的 BMD 值显着较低（0.841 +/- 0.15 与 1.010 +/- 0.0 17；P < 0.0001）。与身高和体重指数相似的对照组相比，患者的 BMD 值也显着较低。 BMD 值与骨折史或髋臼前突无关。在患者中，BMD 值低于 -2.5 与硬脑膜扩张的存在相关。结论：患有马凡综合征的男性和女性有明显的骨质减少，与 BMI 无关。 (C) 2006 Elsevier Masson SAS。版权所有。
Objective: To measure bone mineral density (BMD) in a group of patients meeting Gand criteria for Marfan syndrome, comparatively with a group of healthy controls.Methods: Dual-energy X-ray absorptiometry (DEXA) was used to measure BMD at the hip and wrist in 130 patients seen at the Multidisciplinary Marfan Clinic, Paris, France. Results were compared to values in the database of the absorptiometry machine (Hologic QDR100) and to values in 72 healthy height-matched controls including 35 whose body mass index (BMI) values were similar to those in the patients.Results: A history of fractures was noted in 32 (24.6%) patients. Z-score values were significantly decreased in the patients compared to the Hologic database values at the femoral neck (-1.190 +/- 0.098, P < 0.0001) and wrist (-1.403 +/- 1.06; P < 0.001). Patients had significantly lower BMD values at the femoral neck compared to the height-matched controls (0.841 +/- 0.15 versus 1.010 +/- 0.0 17; P < 0.0001). BMD values were also significantly lower in the patients compared to the controls of similar height and BMI. BMD values did not correlate with history of fractures or acetabular protrusion. In the patients, BMD values lower than -2.5 correlated with presence of dural ectasia.Conclusion: Men and women with Marfan syndrome have significant osteopenia independent from BMI. (C) 2006 Elsevier Masson SAS. All rights reserved.