马凡氏综合征(Marfan Syndrome)是一种遗传性结缔组织疾病。主要特点是蜘蛛样指趾和晶状体脱位。常发生心血管合并症,而且大多数患者死于主动脉根部进行性改变所致的主动脉功能不全或破裂。在一组72例的报告中死亡年龄平均为32岁。本病的地区和种族分布很广。其发病率在北爱尔兰约为10万分之1.5。按此数字估计英国至少有750名病人,美国约有4,000人患病。 Marfan Syndrome is a hereditary connective tissue disease. The main features are spider-like digit and lens dislocation. Cardiovascular complications often occur, and most patients die of aortic insufficiency or rupture caused by progressive changes in the aortic root. The average age of death in a group of 72 patients was 32 years. The area and ethnic distribution of the disease is very wide. Its incidence is about 1.5 in 100,000 in Northern Ireland. According to this figure, there are at least 750 patients in the United Kingdom and about 4,000 in the United States.