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遗传出血性毛细血管扩张症(HHT)又名Rendu-Os1er-Weber氏病,为单常染色体显性遗传,临床可见多发性毛细血管扩张。病理表现为单层内皮细胞组成异常的表皮下动静脉瘘,在鼻中隔后部可见粘膜下曲张的毛细血管,易受损伤出血。其发病率难以确定。有关此病的文献及学术会议专题报告也极少。临床表现为单或双侧反复鼻衄,一天可多次发生,甚至严重的出血可危及生命,对患者生活带来严重危害。S(?)unders(1960)首先提出植皮术,即在鼻窥镜下,刮除中隔病变粘膜,暴露并保留软骨膜血管床,植以游离皮片,填塞固定,可双侧一次完成。范围广泛者可应用“袖套状”游离皮
Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Os1er-Weber’s disease, is a single autosomal dominant inheritance, multiple visible capillary dilation. Pathological manifestations of abnormal monolayer of endothelial cells of the subcortical arteriovenous fistula in the posterior nasal septum visible submucosal varicose capillaries, vulnerable to bleeding. Its incidence is difficult to determine. Literature on the disease and academic conference special reports are rare. Clinical manifestations of repeated single or double epistaxis, multiple occurrences a day, and even severe bleeding can be life-threatening, serious harm to the lives of patients. S (?) Unders (1960) first proposed skin grafting, which under the endoscopic nasal mirror, the removal of mucosal lesions of the septum, exposed and retained the perichondrial vascular bed, planted with free skin flap, fixed by packing, can be completed at both sides. A wide range of people can apply “sleeve-like” free skin