Alomari Mohammad, Hitawala Asif, Chadalavada Pravallika, Covut Fahrettin, Al Momani Laith, Khazaaleh Shrouq, Gosai Falgun, Al Ashi Suleiman, Abushahin Ashraf, Schneider Alison
Internal Medicine, Cleveland Clinic Foundation, Cleveland, USA.
Internal Medicine, Cleveland Clinic - Fairview Hospital, Cleveland, USA.
Cureus. 2020 Apr 29;12(4):e7881. doi: 10.7759/cureus.7881.
Introduction Ehlers-Danlos syndrome (EDS), specifically the hypermobility type (hEDS), is associated with a variety of gastrointestinal (GI) conditions. This study aims to evaluate the prevalence of and factors associated with gut dysmotility in patients with hEDS. Methods This is a retrospective study of hEDS patients conducted at the Cleveland Clinic's Center for Personalized Genetic Healthcare between January 2007 and December 2017. Demographics, GI motility testing, endoscopic, and imaging data were extracted from the patients' charts. Results A total of 218 patients with hEDS were identified. Among them, 136 (62.3%) patients had at least one GI symptom at the time of EDS diagnosis. Motility testing was performed and reported in 42 (19.2%) patients. Out of them, five (11.9%) had esophageal dysmotility, 18 (42.8%) had gastroparesis, five (11.9%) had small bowel/colon altered transit time, and four (9.5%) had global dysmotility. In univariable analysis, patients with postural orthostatic tachycardia syndrome (POTS) [odds ratio (OR): 8.88, 95% CI: 3.69-24.9, p<0.0001], fibromyalgia (OR: 4.43, 95% CI: 2.04-10.1, p=0.0002), history of irritable bowel syndrome (OR: 5.01, 95% CI: 2.31-11.2, p<0.0001), and gastroesophageal reflux disease (OR: 3.33, 95% CI: 1.55-7.44, p=0.002) were more likely to be diagnosed with GI dysmotility. On multivariable analysis, only POTS (OR: 5.74, 95% CI: 2.25-16.7, p=0.0005) was significantly associated with an increased likelihood of GI dysmotility. Conclusions This study suggests that GI symptoms are relatively common among patients with hEDS. Of the patients tested for dysmotility, 76.2% were found to have some form of dysmotility. POTS was found to be an independent predictive factor for GI dysmotility.
引言 埃勒斯-当洛综合征（EDS），特别是高活动型（hEDS），与多种胃肠道（GI）疾病相关。本研究旨在评估hEDS患者肠道动力障碍的患病率及相关因素。方法 这是一项对2007年1月至2017年12月在克利夫兰诊所个性化基因医疗中心进行的hEDS患者的回顾性研究。从患者病历中提取人口统计学、GI动力测试、内镜检查和影像学数据。结果 共识别出218例hEDS患者。其中，136例（62.3%）患者在EDS诊断时至少有1种GI症状。42例（19.2%）患者进行了动力测试并报告结果。其中，5例（11.9%）有食管动力障碍，18例（42.8%）有胃轻瘫，5例（11.9%）小肠/结肠转运时间改变，4例（9.5%）有整体动力障碍。单因素分析显示，体位性直立性心动过速综合征（POTS）患者[比值比（OR）：8.88，95%置信区间（CI）：3.69 - 24.9，p<0.0001]、纤维肌痛患者（OR：4.43，95%CI：2.04 - 10.1，p = 0.0002）、肠易激综合征病史患者（OR：5.01，95%CI：2.31 - 11.2，p<0.0001）和胃食管反流病患者（OR：3.33，95%CI：1.55 - 7.44，p = 0.002）更有可能被诊断为GI动力障碍。多因素分析显示，只有POTS（OR：5.74，95%CI：2.25 - 16.7，p = 0.0005）与GI动力障碍可能性增加显著相关。结论 本研究表明，GI症状在hEDS患者中相对常见。在进行动力障碍测试的患者中，76.2%被发现有某种形式的动力障碍。POTS被发现是GI动力障碍的独立预测因素。
