Hypermobile Ehlers-Danlos Syndrome (a.k.a. Ehlers-Danlos Syndrome Type III and Ehlers-Danlos Syndrome Hypermobility Type): Clinical Description and Natural History
Hypermobile Ehlers-Danlos Syndrome (a.k.a. Ehlers-Danlos Syndrome Type III and Ehlers-Danlos Syndrome Hypermobility Type): Clinical Description and Natural History
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DOI：
10.1002/ajmg.c.31538
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发表时间：
2017-03-01
影响因子：
3.1
通讯作者：
Levy, Howard
中科院分区：
文献类型：
--
作者：
Tinkle, Brad;Castori, Marco;Levy, Howard
Ehlers-Danlos综合征（hEDS）的高活动型可能是最常见的结缔组织遗传性疾病。它主要在肌肉骨骼主诉患者中描述，包括关节过度活动、关节半脱位/脱位以及皮肤和软组织表现。许多患者报告与活动有关的疼痛，有些人继续每天疼痛。两个未分化的综合征已被用来描述这些表现-关节过度活动综合征和hEDS。两者都是在没有其他原因的情况下的临床诊断。目前的医学文献进一步复杂化的分化和描述多种相关的症状和障碍。目前的EDS疾病分类将这两种实体合并为超机动型EDS。在此，我们回顾和总结文献，作为一个更好的临床描述这种类型的结缔组织疾病。(C)2017 Wiley Periodicals，Inc.
The hypermobile type of Ehlers-Danlos syndrome (hEDS) is likely the most common hereditary disorder of connective tissue. It has been described largely in those with musculoskeletal complaints including joint hypermobility, joint subluxations/dislocations, as well as skin and soft tissue manifestations. Many patients report activity-related pain and some go on to have daily pain. Two undifferentiated syndromes have been used to describe these manifestations-joint hypermobility syndrome and hEDS. Both are clinical diagnoses in the absence of other causation. Current medical literature further complicates differentiation and describes multiple associated symptoms and disorders. The current EDS nosology combines these two entities into the hypermobile type of EDS. Herein, we review and summarize the literature as a better clinical description of this type of connective tissue disorder. (C) 2017 Wiley Periodicals, Inc.