hypermobile spectrum disorder

  • 文章类型: Journal Article
    背景:高移动Ehlers-Danlos综合征(hEDS)和高移动频谱障碍(HSD)是以全身性关节过度移动为特征的结缔组织疾病,与慢性疼痛和一些症状有关,比如疲劳,自主神经失调,以及精神病合并症。在系统的感觉测试过程中对异常表现的临床观察提出了可能与功能性神经系统疾病(FND)共存的问题。因此,本研究旨在评估hEDS/HSD患者队列中是否存在积极的功能性神经体征(FNS).
    方法:回顾性分析hEDS/HSD患者(N=24)的临床资料,并与前瞻性招募的年龄/性别匹配的健康对照组(N=22)进行比较。评估了四个马达和三个感觉阳性FNS。
    结果:22例患者(92%)出现至少一种运动或感觉FNS。五名患者(21%)仅出现单一FNS,14个在2到4个FNS之间(58%),3例患者出现5个或更多FNS(12%)。没有健康对照显示运动FNS,只有两个人出现了感官FNS。
    结论:hEDS/HSD中FNS的存在值得FND更好的临床检测和正式诊断,以便在共病情况下提供更充分的护理。事实上,FND会严重干扰hEDS/HSD的康复工作,FND靶向物理治疗可能应与EDS/HSD特异性方法相结合。
    The hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobility spectrum disorders (HSD) are connective tissue disorders characterized by generalized joint hypermobility, associated with chronic pain and several symptoms, such as fatigue, dysautonomia, as well as psychiatric co-morbidities. Clinical observations of unusual manifestations during systematic sensory testing raised the question of a possible co-existence with a functional neurological disorder (FND). Hence, this study aimed to assess the presence of positive functional neurological signs (FNS) in a cohort of patients with hEDS/HSD.
    The clinical data of hEDS/HSD patients (N = 24) were retrospectively analyzed and compared to a prospectively recruited age-/sex-matched healthy control group (N = 22). Four motor- and three sensory-positive FNS were assessed.
    Twenty-two patients (92%) presented at least one motor or sensory FNS. Five patients (21%) presented only a single FNS, 14 presented between 2 and 4 FNS (58%), and 3 patients presented 5 or more FNS (12%). None of the healthy controls presented motor FNS, and only two presented a sensory FNS.
    The presence of FNS in hEDS/HSD deserves better clinical detection and formal diagnosis of FND to offer more adequate care in co-morbid situations. In fact, FND can severely interfere with rehabilitation efforts in hEDS/HSD, and FND-targeted physical therapy should perhaps be combined with EDS/HSD-specific approaches.
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  • 文章类型: Journal Article
    物理治疗(PT)在治疗患有广泛性高移动性频谱障碍(G-HSD)和高移动性Ehlers-Danlos综合征(hEDS)的个体中起着核心作用。然而,描述这些人的PT管理的研究有限。这篇综述旨在系统地绘制PT干预治疗该患者人群的证据。
    PubMed的系统文献检索,CINAHL,从2000年1月到2023年4月进行了Embase。筛选过程之后,根据所使用的PT干预类型对研究进行了评估和分类.五名审稿人独立评估了这些文章。
    搜索产生了757篇文章。28人符合纳入标准。研究包括630名参与者,主要是女性,平均年龄为26.2岁(2至69岁)。使用的PT干预措施是治疗性锻炼,患者指导,运动功能训练,自适应设备,手动治疗,和功能训练。
    证据表明,治疗性运动和运动功能训练是治疗患有G-HSD和hEDS的个体的有效方法。使用自适应设备的证据也很薄弱,患者指导,手动治疗,和功能训练。最近的研究强调多学科护理和对G-HSD/hEDS心理影响的理解。需要进一步的研究来确定PT干预的有效性和剂量。对康复的意义缺乏关于物理治疗(PT)干预措施的指南和共识,以治疗和恢复患有广泛性高移动性频谱障碍(G-HSD)和高移动性Ehlers-Danlos综合征(hEDS)的人的功能,对临床医生提出了挑战。这篇综述支持治疗性锻炼和运动功能训练以改善功能,幸福,G-HSD和hEDS患者的生活质量。使用自适应设备的证据薄弱,患者指导,手动治疗,和功能训练。我们已经收集了现有的证据,评估质量,并得出了该人群最受支持的PT干预措施的结论。
    UNASSIGNED: Physical therapy (PT) plays a central role in treating individuals with Generalized Hypermobility Spectrum Disorder (G-HSD) and Hypermobile Ehlers-Danlos Syndrome (hEDS). However, there is limited research describing these individuals\' PT management. This review aims to systematically map the evidence on PT interventions to treat this patient population.
    UNASSIGNED: A systematic literature search of PubMed, CINAHL, and Embase from January 2000 to April 2023 was performed. After the screening process, studies were appraised and classified based on the type of PT interventions used. Five reviewers independently assessed the articles.
    UNASSIGNED: The search produced 757 articles. Twenty-eight met the inclusion criteria. The studies included 630 participants, mostly female, with a mean age of 26.2 (ranging from 2 to 69). The PT interventions used were therapeutic exercise, patient instruction, motor function training, adaptive equipment, manual therapy, and functional training.
    UNASSIGNED: The evidence indicates that therapeutic exercise and motor function training are efficacious methods to treat individuals with G-HSD and hEDS. There is also weak evidence for using adaptive equipment, patient instruction, manual therapy, and functional training. Recent studies emphasize multidisciplinary care and understanding of the psychological impact of G-HSD/hEDS. Additional research is needed to determine the effectiveness and dosage of PT interventions.
    The lack of guidelines and consensus on physical therapy (PT) interventions to treat and restore function in people with Generalized Hypermobility Spectrum Disorder (G-HSD) and hypermobile Ehlers-Danlos Syndrome (hEDS) challenges clinicians.This review supports therapeutic exercise and motor function training to improve function, well-being, and quality of life in people with G-HSD and hEDS.There is weak evidence for using adaptive equipment, patient instruction, manual therapy, and functional training.We have gathered existing evidence, appraised the quality, and drawn conclusions on this population’s most supported PT interventions.
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  • 文章类型: Journal Article
    UNASSIGNED: The aim was to ascertain occupational therapist (OT) and physiotherapist (PT) experiences of managing hypermobility spectrum disorders (HSDs) and hypermobile Ehlers-Danlos syndrome (hEDS) patients, specifically the training and confidence levels of therapists, use of evidence-based practice, accessibility of pain management and clinical psychology services, and perceived usefulness of a specialist centre in Scotland.
    UNASSIGNED: A mixed-method survey was distributed to Rheumatology OTs/PTs in Scotland. It included multiple choice and open text questions, which were analysed to reflect therapists\' experiences and perception of service need.
    UNASSIGNED: We found that therapists in Scotland do have expertise in the management of HSD/hEDS patients; however, this expertise tends to be concentrated in secondary care, which makes it difficult for patients who are managed in primary care to access. The majority of respondents reported lack of access to external training (80%). There was difficulty in referral to pain management services (55%) and clinical psychology (28%) among adult therapists. Paediatric services provided considerably better access to these disciplines. Of note, the majority of respondents were in favour of a specialist centre in Scotland for the training and education of therapists (94.7%) and the diagnosis and management of complex HSD/hEDS patients (73.7%).
    UNASSIGNED: More research is needed urgently to evaluate the effectiveness of therapy interventions to underpin a national guideline in order that we can improve outcomes for HSD/hEDS patients. A specialist centre with expert and engaged clinicians would be a valuable asset in coordinating patient-focused research and conducting good-quality clinical trials.
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