...
  • 主题
高级搜索  >
历史搜索 清空历史
首页> 外文期刊>British Journal of Haematology >Magnetic resonance angiography-defined intracranial vasculopathy is associated with silent cerebral infarcts and glucose-6-phosphate dehydrogenase mutation in children with sickle cell anaemia
【24h】

Magnetic resonance angiography-defined intracranial vasculopathy is associated with silent cerebral infarcts and glucose-6-phosphate dehydrogenase mutation in children with sickle cell anaemia

机译：磁共振血管造影定义的颅内血管病变与镰状细胞贫血儿童的沉默脑梗死和葡萄糖-6-磷酸脱氢酶突变有关

获取原文
获取原文并翻译 | 示例
   

获取外文期刊封面封底 >>

       
页面导航
  • 摘要
  • 著录项
  • 相似文献
  • 相关主题

摘要

Silent cerebral infarct (SCI) is the most commonly recognized cause of neurological injury in sickle cell anaemia (SCA). We tested the hypothesis that magnetic resonance angiography (MRA)-defined vasculopathy is associated with SCI. Furthermore, we examined genetic variations in glucose-6-phosphate dehydrogenase (G6PD) and HBA (α-globin) genes to determine their association with intracranial vasculopathy in children with SCA. Magnetic resonance imaging (MRI) of the brain and MRA of the cerebral vasculature were available in 516 paediatric patients with SCA, enrolled in the Silent Infarct Transfusion (SIT) Trial. All patients were screened for G6PD mutations and HBA deletions. SCI were present in 41·5% (214 of 516) of SIT Trial children. The frequency of intracranial vasculopathy with and without SCI was 15·9% and 6·3%, respectively (P < 0·001). Using a multivariable logistic regression model, only the presence of a SCI was associated with increased odds of vasculopathy (P = 0·0007, odds ratio (OR) 2·84; 95% Confidence Interval (CI) = 1·55-5·21). Among male children with SCA, G6PD status was associated with vasculopathy (P = 0·04, OR 2·78; 95% CI = 1·04-7·42), while no significant association was noted for HBA deletions. Intracranial vasculopathy was observed in a minority of children with SCA, and when present, was associated with G6PD status in males and SCI.
机译：沉默的脑梗死（SCI）是镰状细胞贫血（SCA）中最常见的神经损伤原因。我们测试了磁共振血管造影（MRA） - 定义血管病与SCI相关的假设。此外，我们检查了葡萄糖-6-磷酸脱氢酶（G6PD）和HBA（α-珠蛋白）基因的遗传变异，以确定其与SCA儿童颅内血管病变的关联。脑血管系统的大脑和MRA的磁共振成像（MRI）可在516名与SCA患者中获得，注册了沉默的梗死输血（SIT）试验。所有患者均被筛选用于G6PD突变和HBA缺失。 SCI存在于41·5％（214分中516年）坐试验儿童。具有和不含SCI的颅内血管病变的频率分别为15·9％和6·3％（P <0·001）。使用多变量的逻辑回归模型，只有SCI的存在与血管病变的增加有关（P = 0·0007，差距（或）2·84; 95％置信区间（CI）= 1·55-5· 21）。在SCA的雄性儿童中，G6PD状态与血管病变有关（P = 0·04，或2·78; 95％CI = 1·04-7·42），而HBA缺失则没有注意到重大关联。在少数SCA的儿童中观察到颅内血管病变，当存在时，与男性和SCI的G6PD状态有关。

著录项

  • 来源
    《British Journal of Haematology》 |2012年第3期|共8页
  • 作者

    ThangarajhM.; YangG.; FuchsD.; PonisioM.R.; MckinstryR.C.; JajuA.; NoetzelM.J.; CasellaJ.F.; Barron-CasellaE.; HooperW.C.; BouletS.L.; BeanC.J.; PyleM.E.; PayneA.B.; DriggersJ.; TrauH.A.; VendtB.A.; RodeghierM.; DebaunM.R.;

  • 作者单位

    Department of Neurology and Pediatrics Washington University School of Medicine Saint Louis MO;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Pediatric Radiology and Neuroradiology Sections Washington University School of Medicine Saint;

    Pediatric Radiology and Neuroradiology Sections Washington University School of Medicine Saint;

    Pediatric Radiology and Neuroradiology Sections Washington University School of Medicine Saint;

    Pediatric Radiology and Neuroradiology Sections Washington University School of Medicine Saint;

    Department of Neurology and Pediatrics Washington University School of Medicine Saint Louis MO;

    Division of Hematology Department of Pediatrics Johns Hopkins University School of Medicine;

    Division of Hematology Department of Pediatrics Johns Hopkins University School of Medicine;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Clinical and Molecular Hemostasis Laboratory Branch Division of Blood Disorders National Center;

    Electronic Radiology Laboratory Mallinckrodt Institute of Radiology Washington University School;

    Statistical Collaborator Chicago IL United States;

    Department of Pediatrics Vanderbilt Meharry Sickle Cell Disease Center of Excellence Vanderbilt;

  • 收录信息
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类 血液及淋巴系疾病;
  • 关键词

    α-thalassaemia; Glucose-6-phosphate dehydrogenase; Sickle cell anaemia; Silent cerebral infarcts; Vasculopathy;

    机译：α-地中海贫血;葡萄糖-6-磷酸脱氢酶;镰状细胞贫血;无声脑梗塞;血管病变;

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱：kefu@zhangqiaokeyan.com

京公网安备：11010802029741号 ICP备案号：京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有

  • 客服微信

  • 服务号