大脑AVM紧度得分在患有遗传性出血性telangiectasia的儿童
Lauren A Beslow1, Arastoo Vossough2, Helen Kim3
1Neurology and Pediatrics, Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Penn HHT Center of Excellence and Children's Hospital of Philadelphia Hereditary Hemorrhagic Telangiectasia Program, 3401 Civic Center Boulevard, Philadelphia, 19104, PA, USA. beslow@chop.edu.
概括
患有遗传性出血性疏通症 (HHT) 和脑动脉静脉形 (BAVM) 的儿童可能比患有零星BAVM的儿童具有更紧的巢穴. 巢穴紧密度得分 (CS) 可能有助于预测HTT相关的BAVM中的出血风险.
科学领域:
- 神经学 神经学
- 放射学 放射学是一门学科.
- 遗传学 遗传学 是一个
背景情况:
- 大脑动脉静脉形形 (BAVMs) 是一种复杂的血管病变.
- 血管学上的阴茎紧密度评分 (CS) 预测了零星BAVM切除后的复发.
- 遗传性出血端膜炎 (HHT) 是一种遗传性疾病,可以导致BAVMs.
研究的目的:
- 测量患有HHT的儿童中BAVM的血管学CS.
- 为了比较HHT相关的BAVM与零星的BAVM中的CS特征.
- 探索 CS 作为 HHT 中出血的预测因素的潜力.
主要方法:
- 一位儿科干预神经放射学家审查了HHT儿童的血管图.
- 根据体和周周血管的紧密度来确定CS.
- CS被分为分散的 (1),中间的 (2) 或紧的 (3).
主要成果:
- 在47名HHT儿童中,分析了54名BAVM.
- 与零星BAVM (CS 3: 8.7%,p=0.045) 相比,HHT BAVM显示了紧巢的更高流行率 (CS 3: 33.3%).
- 七名患有HHT的儿童经历了内出血,CS得分各不相同.
结论:
- 在HHT BAVM中存在一系列的CS,需要进一步调查.
- 与HHT相关的BAVM可能比零星的BAVM表现出更紧的巢穴.
- 在HHT中,CS可能是BAVM复发和出血风险的宝贵血管学标志物.
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