Gastrointestinal Involvement in the Ehlers-Danlos Syndromes
Gastrointestinal Involvement in the Ehlers-Danlos Syndromes
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DOI：
10.1002/ajmg.c.31546
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发表时间：
2017-03-01
影响因子：
3.1
通讯作者：
Aziz, Qasim
中科院分区：
文献类型：
--
作者：
Fikree, Asma;Chelimsky, Gisela;Aziz, Qasim
目前的证据表明，埃勒斯-当洛斯综合征 (EDS) 等结缔组织非炎症性遗传性疾病与胃肠道 (GI) 症状之间存在关联。 EDS 患者既可能出现结构性问题，如食管裂孔疝、内脏下垂、直肠前突和直肠脱垂，也可能出现功能性问题，如肠道蠕动紊乱。最近的研究表明，过度活动性 EDS (hEDS) 患者会出现与前肠和后肠相关的胃肠道症状，这些患者通常符合功能性胃肠道疾病的标准，例如功能性消化不良和肠易激综合征。 EDS 患者出现胃肠道症状会影响他们的生活质量。目前尚不存在针对 EDS 患者胃肠道症状管理的具体循证管理指南，这些患者通常接受对症治疗。然而，人们认识到对于接受手术治疗的患者需要采取某些预防措施。未来的研究需要确定导致 EDS 患者出现胃肠道症状的机制，并且需要更具体的治疗指南。 (C) 2017 年 Wiley 期刊公司。
Current evidence suggests that an association exists between non-inflammatory hereditary disorders of connective tissue such as the Ehlers-Danlos syndromes (EDS) and gastrointestinal (GI) symptoms. Patients with EDS can present with both structural problems such as hiatus hernias, visceroptosis, rectoceles, and rectal prolapse as well as functional problems such as disordered gut motility. It has recently been demonstrated that patients with hypermobile EDS (hEDS) present with GI symptoms related to the fore and hind-gut and these patients frequently meet the criteria for functional gastrointestinal disorders such as functional dyspepsia and irritable bowel syndrome. Presence of GI symptoms in EDS patients influences their quality of life. Specific evidence based management guidelines for the management of GI symptoms in EDS patients do not exist and these patients are often treated symptomatically. There is, however, recognition that certain precautions need to be taken for those patients undergoing surgical treatment. Future studies are required to identify the mechanisms that lead to GI symptoms in patients with EDS and more specific treatment guidelines are required. (C) 2017 Wiley Periodicals, Inc.