Gray J R, Bridges A B, West R R, McLeish L, Stuart A G, Dean J C, Porteous M E, Boxer M, Davies S J
Institute of Medical Genetics, University Hospital of Wales, Cardiff, UK.
Clin Genet. 1998 Aug;54(2):124-8. doi: 10.1111/j.1399-0004.1998.tb03714.x.
A total of 206 patients with Marfan syndrome were ascertained throughout genetic clinics in Wales and Scotland during the period 1970-1990. There were 45 deaths representing 22% of the cohort. Mean age at death was 45.3+/-16.5 years. 50% median cumulative survival in the total cohort (n=206) was 53 years for males and 72 years for females. Multivariate analysis confirmed severity as the best independent indicator of survival. These findings and survival curves will assist in the counselling of British families and individuals with Marfan syndrome.
1970年至1990年期间，在威尔士和苏格兰的基因诊所共确诊了206例马凡综合征患者。其中45人死亡，占该队列的22%。平均死亡年龄为45.3±16.5岁。整个队列（n = 206）中，男性的中位累积生存率为50%时是53岁，女性为72岁。多变量分析证实病情严重程度是生存的最佳独立指标。这些研究结果和生存曲线将有助于为患有马凡综合征的英国家庭和个人提供咨询。
