中国普通外科杂志2011，Vol.20Issue(6)：594-598,5.
血管型Ehlers-Danlos综合征2例及一家系报道并文献复习
Vascular type Ehlers-Danlos syndrome: a report of 2 cases and a family study with literature review
王宪伟 1刘光强 1王伟 1欧阳洋 1黄建华1
作者信息
- 1. 中南大学湘雅医院普通外科,湖南长沙410008
- 折叠
摘要
Abstract
Objective To study the pathogenesis, diagnosis and treatment of vascular Ehlers-Danlos syndrome ( EDS). Methods Analysis of the clinical data of 2 patients with EDS and the EDS patients occurred in a fomily. The characteristics of pathogenesis, diagnosis and surgical treatment of EDS were reveiwed. Results One of the EDS patients had spontaneous rupture of aneurysm. At operation, the vessel was found to be fragile and the operative field had marked oozing of blood. Postoperative bleeding led to the patient death. Another case of EDS underwent interventional treatment several times for thigh hematoma, but bleeding recurred quickly.Pedigree study of case 2 family showed autosomal dominant characteristics. Conclusions Vascular type EDS is a very rare kind of congenital disease. Its vascular complications often lead to disastrous consequences. Surgical therapy has a high surgical complication and mortality rate.关键词
Ehlers-Danlos综合征/诊断/Ehlers-Danlos综合征/治疗/家族性疾病/综述文献Key words
Ehlers-Danlos Syndrome/diag/ Ehlers- Danlos Syndrome/ther/ Familial Disease/ Review Literature as Topic分类
医药卫生引用本文复制引用
王宪伟,刘光强,王伟,欧阳洋,黄建华..血管型Ehlers-Danlos综合征2例及一家系报道并文献复习[J].中国普通外科杂志,2011,20(6):594-598,5.
