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获得性和先天性异位骨化:新的发病机制见解和治疗机会。

Acquired and congenital forms of heterotopic ossification: new pathogenic insights and therapeutic opportunities.

机构信息

Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

出版信息

Curr Opin Pharmacol. 2018 Jun;40:51-58. doi: 10.1016/j.coph.2018.03.007. Epub 2018 Mar 31.

Abstract

Heterotopic ossification (HO) involves the formation and accumulation of extraskeletal bone tissue at the expense of local tissues including muscles and connective tissues. There are common forms of HO that are triggered by extensive trauma, burns and other bodily insults, and there are also rare congenital severe forms of HO that occur in children with Fibrodysplasia Ossificans Progressiva or Progressive Osseous Heteroplasia. Given that HO is often preceded by inflammation, current treatments usually involve anti-inflammatory drugs alone or in combination with local irradiation, but are not very effective. Recent studies have provided novel insights into the pathogenesis of acquired and genetic forms of HO and have used the information to conceive and test new and more specific therapies in animal models. In this review, I provide salient examples of these exciting and promising advances that are undoubtedly paving the way toward resolution of this debilitating and at times fatal disease.

摘要

异位骨化(HO)涉及到在牺牲局部组织(包括肌肉和结缔组织)的情况下形成和积累骨骼外的骨组织。HO 有常见的形式,是由广泛的创伤、烧伤和其他身体损伤引发的,也有罕见的先天性严重形式,发生在患有纤维性骨发育不良或进行性骨异质增生的儿童中。由于 HO 通常是在炎症之前发生的,目前的治疗方法通常是单独使用抗炎药物或与局部放射治疗相结合,但效果不是很好。最近的研究为获得性和遗传性 HO 的发病机制提供了新的见解,并利用这些信息在动物模型中构思和测试新的、更具特异性的治疗方法。在这篇综述中,我提供了这些令人兴奋和有前途的进展的突出例子,这些进展无疑为解决这种使人衰弱且有时致命的疾病铺平了道路。

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