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儿童和青少年急性主动脉夹层:对患者及其家庭的诊断及事件后的随访责任

Acute aortic dissection in children and adolescents: diagnostic and after-event follow-up obligation to the patient and family.

作者信息

Fikar Charles R

机构信息

Woods Health Sciences Library, St. Vincent Catholic Medical Centers, Jamaica, New York 11432, USA.

出版信息

Clin Cardiol. 2006 Sep;29(9):383-6. doi: 10.1002/clc.4960290903.

Abstract

Acute aortic dissection in childhood and adolescence is a rare but life-threatening condition with many known risk factors, most of which are heritable. The importance of determining the presence of any predisposing disorders is stressed, since all close relatives can then be screened for any such inherited trait that would make them at risk for a similar catastrophic event. Even with no obvious predisposing disorder, it may still be prudent to evaluate close kin for the presence of aortic root dilatation, since some genetic disorders have no manifestation other than acute aortic dissection secondary to aortic medial pathology. Close monitoring for at-risk family members, including the index patient, may need to be a life-long process.

摘要

儿童和青少年急性主动脉夹层是一种罕见但危及生命的疾病,有许多已知的风险因素,其中大多数是遗传性的。强调了确定是否存在任何易患疾病的重要性,因为这样就可以对所有近亲进行筛查,以寻找任何可能使他们面临类似灾难性事件风险的遗传特征。即使没有明显的易患疾病,评估近亲是否存在主动脉根部扩张可能仍属谨慎之举,因为有些遗传疾病除了继发于主动脉中层病变的急性主动脉夹层外没有其他表现。对包括索引患者在内的高危家庭成员进行密切监测可能需要成为一个终身过程。

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