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Higher fracture prevalence and smaller bone size in patients with hEDS/HSD—a prospective cohort study
Osteoporosis International ( IF 4.2 ) Pub Date : 2019-12-24 , DOI: 10.1007/s00198-019-05269-z
T. Banica , M. Coussens , C. Verroken , P. Calders , I. De Wandele , F. Malfait , H.-G. Zmierczak , S. Goemaere , B. Lapauw , L. Rombaut

Summary

Increased fracture risk in patients with Ehlers-Danlos syndromes has been reported, but the reasons for it are incompletely understood. We aimed to investigate possible determinants of this increased risk and found that hEDS/HSD patients present with a cortical bone size deficit compared with control subjects, possibly related to lower mechanical loading.

Introduction

The Ehlers-Danlos syndromes (EDS) comprise a group of heritable connective tissue disorders caused by defects in the biosynthesis, secretion, and/or organization of fibrillar collagens which might impair bone strength. Our aim was to compare fracture prevalence, volumetric and areal bone mineral density (BMD), bone geometry, muscle size and the muscle-bone interaction, body composition and longitudinal changes therein between patients with hypermobile EDS (hEDS) or hypermobility spectrum disorder (HSD), and healthy control subjects.

Methods

Cross-sectional data comprised 39 female hEDS/HSD patients (age 41 ± 11 years) and 43 age-matched controls. After 8 years, 27 hEDS/HSD and 17 control subjects were re-evaluated. Tibial trabecular and cortical volumetric BMD, bone mineral content (BMC), cortical bone geometry, and lower leg muscle cross-sectional area (CSA) were measured using pQCT. Body composition, areal BMD, and BMC were determined by DXA.

Results

At baseline, patients with hEDS/HSD presented with a smaller cortical bone area, smaller cortical thickness and muscle CSA, and a higher fracture prevalence than control subjects (all p < 0.05). No differences in areal or volumetric BMD were found. Longitudinally, muscle CSA decreased in both groups and muscle density decreased in the hEDS/HSD group (p < 0.001) whereas all bone parameters remained unchanged.

Conclusion

hEDS/HSD patients have a cortical bone size deficit compared with controls, possibly contributing to their increased fracture risk. They presented with decreased muscle CSA but normal bone/muscle area ratio, suggesting that this bone size deficit is likely secondary to decreased mechanical loading. Further, there were no arguments for accelerated bone loss in hEDS/HSD subjects.



中文翻译:

hEDS / HSD患者较高的骨折发生率和较小的骨尺寸—前瞻性队列研究

概要

据报道,Ehlers-Danlos综合征患者的骨折风险增加,但其原因尚不完全清楚。我们旨在研究这种风险增加的可能决定因素,并发现与对照组相比,hEDS / HSD患者存在皮质骨大小不足,可能与较低的机械负荷有关。

介绍

Ehlers-Danlos综合征(EDS)包括一组可遗传的结缔组织疾病,这些疾病是由可能损害骨骼强度的原纤维胶原蛋白的生物合成,分泌和/或组织缺陷引起的。我们的目的是比较高活动性EDS(hEDS)或高活动性谱系障碍(HSD)患者之间的骨折患病率,体积和面积骨矿物质密度(BMD),骨骼几何形状,肌肉大小以及肌肉与骨骼的相互作用,身体组成和纵向变化)和健康对照对象。

方法

横断面数据包括39名女性hEDS / HSD患者(41±11岁)和43个年龄匹配的对照。8年后,对27名hEDS / HSD和17名对照受试者进行了重新评估。使用pQCT测量胫骨小梁和皮质体积BMD,骨矿物质含量(BMC),皮质骨几何形状和小腿肌肉横截面积(CSA)。身体成分,面积BMD和BMC由DXA确定。

结果

在基线时,与对照组相比,hEDS / HSD患者表现出较小的皮质骨面积,较小的皮质厚度和肌肉CSA以及较高的骨折发生率(所有p  <0.05)。没有发现面积或体积的骨密度差异。纵向上,两组的肌肉CSA均降低,而hEDS / HSD组的肌肉密度降低(p  <0.001),而所有骨骼参数均保持不变。

结论

与对照组相比,hEDS / HSD患者的皮质骨大小不足,可能导致骨折风险增加。他们表现出肌肉CSA降低,但骨骼/肌肉面积比正常,表明这种骨骼尺寸不足很可能是由于机械负荷降低所致。此外,在hEDS / HSD受试者中没有关于加速骨质流失的争论。

更新日期:2020-04-22
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