关键词: Ehler-Danlos syndrome Joint hypermobility syndrome gastrointestinal motility disorder gastroparesis

Mesh : Humans Female Adult Male Joint Instability / complications diagnosis epidemiology Irritable Bowel Syndrome / complications Retrospective Studies Gastrointestinal Diseases / complications epidemiology Postural Orthostatic Tachycardia Syndrome

来  源:   DOI:10.1177/10815589231210486

Abstract:
Joint hypermobility syndrome (JHS) is a non-inflammatory hereditary disorder of connective tissue with varied clinical presentations, including frequent joint dislocations, hyperextensible skin, easy bruising, and abnormal paper-thin scar formation. Many of these patients have unexplained gastrointestinal (GI) symptoms. Our aim was to evaluate the prevalence of JHS in a tertiary gastroenterology motility clinic and the spectrum of functional bowel disorders in JHS patients. In this retrospective case series, we screened the medical records of 277 patients seen over 4 years at an academic GI Motility Center. The patients who met the criteria for JHS by Beighton hypermobility score were evaluated for the presence of functional GI disorders by Rome IV criteria. They also underwent gastric emptying study and glucose breath testing for small intestinal bacterial overgrowth. The prevalence of JHS in the study population was 9.7%. The mean age was 27 years, and 92.5% were female. The symptoms experienced by these patients include nausea/vomiting (89%), abdominal pain (70%), constipation (48%), and bloating (18.5%). The disorders associated with JHS include gastroparesis (52%), irritable bowel syndrome (55.5%), and gastroesophageal reflux disease (30%). Also, 10 patients (37%) were diagnosed with postural hypotension tachycardia syndrome secondary to autonomic dysfunction. Approximately 10% of patients with suspected functional bowel disorders have hypermobility syndrome. Hence, it is crucial to familiarize gastrointestinal practitioners with the criteria utilized to diagnose JHS and the methods to identify physical examination findings related to this condition.
摘要:
关节过度活动综合征(JHS)是一种非炎性遗传性结缔组织疾病,临床表现多样,包括频繁的关节脱位,过度伸展的皮肤,容易擦伤,和异常的薄纸疤痕形成。这些患者中的许多具有无法解释的胃肠道(GI)症状。我们的目的是评估三级胃肠病学动力诊所中JHS的患病率以及JHS患者的功能性肠道疾病谱。在这项回顾性队列研究中,我们筛选了在学术胃肠动力中心超过4年的277例患者的医疗记录.通过罗马IV标准评估通过Beighton过度活动评分符合JHS标准的患者是否存在功能性胃肠病。患者还接受了胃排空研究(GES)和葡萄糖呼气测试,以进行小肠细菌过度生长。研究人群中JHS的患病率为9.7%。平均年龄是27岁,92.5%为女性。这些患者的症状包括恶心/呕吐(89%),腹痛(70%),便秘(48%),和腹胀(18.5%)。与JHS相关的疾病包括胃轻瘫(52%),肠易激综合征(55.5%),胃食管反流病(30%)。此外,10例患者(37%)被诊断为继发于自主神经功能障碍的体位性低血压性心动过速综合征(POTS)。本质上,10%的疑似功能性肠病患者患有过度活动综合征。GI从业者应该熟悉JHS的所有标准,以及如何引出具体的体检结果。特发性胃轻瘫患者应高度怀疑JHS。
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