儿童和青少年马凡综合征：主动脉根部生长对筛查主动脉并发症的预测和预后价值。
Marfan syndrome in children and adolescents: predictive and prognostic value of aortic root growth for screening for aortic complications.
作者信息
Groenink M, Rozendaal L, Naeff M S, Hennekam R C, Hart A A, van der Wall E E, Mulder B J
机构信息
Department of Cardiology, Academic Medical Centre, Amsterdam, Netherlands.
出版信息
Heart. 1998 Aug;80(2):163-9. doi: 10.1136/hrt.80.2.163.
OBJECTIVE
To assess and measure the diagnostic and prognostic value of individual aortic root growth in children and adolescents with Marfan syndrome.
DESIGN
From 1983 to 1996, 250 children were screened for Marfan syndrome. Serial echocardiographic aortic root measurements of 123 children (57 Marfan, 66 control) were available for evaluation of aortic root growth. Aortic root diameters were correlated with body surface area. Based on individual growth of the aortic root a discrimination formula was derived to distinguish Marfan patients from control subjects.
RESULTS
Sensitivity and specificity of this method, which were dependent on the number of measurements, were 84% and 73%, respectively, for three serial measurements. The discrimination score also predicted a five year complication free survival in all patients.
CONCLUSIONS
In children and adolescents with Marfan syndrome, aortic root growth differs significantly from subjects in whom Marfan syndrome was definitely ruled out during screening. Measurement of individual aortic root growth may serve to establish diagnosis at an early stage and is of prognostic value for occurrence of aortic complications.
目的
评估并测量马凡综合征儿童及青少年个体主动脉根部生长情况的诊断和预后价值。
设计
1983年至1996年期间，对250名儿童进行马凡综合征筛查。可获得123名儿童（57名马凡综合征患者，66名对照者）的系列超声心动图主动脉根部测量数据，用于评估主动脉根部生长情况。主动脉根部直径与体表面积相关。基于主动脉根部的个体生长情况，推导了一个判别公式，以区分马凡综合征患者和对照者。
结果
该方法的敏感性和特异性（取决于测量次数），对于三次系列测量分别为84%和73%。判别分数还可预测所有患者五年无并发症生存率。
结论
在马凡综合征儿童及青少年中，主动脉根部生长情况与筛查期间明确排除马凡综合征的个体显著不同。测量个体主动脉根部生长情况有助于早期诊断，并对主动脉并发症的发生具有预后价值。
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