首页> 美国卫生研究院文献>Journal of the Korean Association of Oral and Maxillofacial Surgeons >Temporomandibular joint ankylosis in Williams syndrome patient: an insight on the function of elastin in temporomandibular joint disorder
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Temporomandibular joint ankylosis in Williams syndrome patient: an insight on the function of elastin in temporomandibular joint disorder

机译:Williams 综合征患者的颞下颌关节强直:弹性蛋白在颞下颌关节疾病中的作用

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摘要

Williams–Beuren syndrome (WS) is a rare genetic disorder that results from microdeletion at chromosome 7, which harbors the elastin gene. Clinical findings include arteriopathy, aortic stenosis, hypertension, and laxities and contractures in different joints throughout the body. While many components of the temporomandibular joint (TMJ) normally contain elastin, there are few reports on TMJ manifestations of WS. This study reports a TMJ ankylosis case in a WS patient and shares insight on a possible link between development of TMJ ankylosis and elastin deficiency in WS patients. A WS patient presented with bilateral TMJ ankylosis and was successfully treated with TMJ gap arthroplasty. Hypermobility of TMJ and lack of elastin in retrodiscal tissue can induce anterior disc displacement without reduction. Due to lack of elastin, which has a significant role in the compensatory and reparatory mechanism of TMJ, WS patients might be prone to TMJ ankylosis.
机译:Williams-Beuren 综合征 (WS) 是一种罕见的遗传性疾病,由含有弹性蛋白基因的 7 号染色体微缺失引起。临床表现包括动脉病变、主动脉瓣狭窄、高血压以及全身不同关节的松弛和挛缩。虽然颞下颌关节 (TMJ) 的许多成分通常含有弹性蛋白,但关于 WS 的 TMJ 表现的报道很少。本研究报告了 WS 患者的 TMJ 强直病例,并分享了对 WS 患者 TMJ 强直发展与弹性蛋白缺乏之间可能联系的见解。一名 WS 患者表现为双侧 TMJ 强直,并成功接受了 TMJ 间隙关节置换术治疗。TMJ 过度活动和椎间盘后组织中弹性蛋白的缺乏可诱导椎间盘前移位而不复位。由于缺乏在 TMJ 的代偿和修复机制中起重要作用的弹性蛋白,WS 患者可能容易发生 TMJ 强直。

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