Solomon J A, Abrams L, Lichtenstein G R
Department of Radiology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania, Philadelphia, USA.
Am J Gastroenterol. 1996 Nov;91(11):2282-8.
Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue that is distinguished not only by the triad of skin hyperextensibility, articular hypermobility, and tissue fragility but also by its heterogeneity on clinical, genetic, and biochemical grounds. The phenotypical variance that characterizes this syndrome often makes its recognition difficult, and failure to recognize the disease despite a classic course is not uncommon. Diagnosis is paramount, however, so life-threatening associations can be searched for and unique principles of management can be instituted. Patients are prone to GI catastrophes such as perforation and massive bleeding, which can be compounded by grave surgical and vascular complications. A thorough knowledge of the GI manifestations of EDS and their management is mandatory to prevent unnecessary morbidity and mortality.
埃勒斯-当洛综合征（EDS）是一种遗传性结缔组织疾病，其特征不仅在于皮肤过度伸展、关节活动过度和组织脆弱三联征，还在于其在临床、遗传和生化方面的异质性。该综合征典型的表型差异常常使其难以识别，即便病程典型但仍未被识别的情况并不罕见。然而，诊断至关重要，这样才能查找危及生命的关联因素并制定独特的治疗原则。患者容易出现诸如穿孔和大出血等胃肠道灾难，严重的手术和血管并发症可能会使情况更加复杂。全面了解EDS的胃肠道表现及其治疗方法对于预防不必要的发病和死亡至关重要。
