The severity of anaemia depletes cerebrovascular dilatory reserve in children with sickle cell disease: a quantitative magnetic resonance imaging study

The severity of anaemia depletes cerebrovascular dilatory reserve in children with sickle cell disease: a quantitative magnetic resonance imaging study
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DOI:
10.1111/bjh.14424
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发表时间:
2017-01-01
影响因子:
6.5
通讯作者:
Kassner, Andrea
Kassner, Andrea
中科院分区:
医学2区
文献类型:
--
作者:
Kosinski, Przemyslaw D.;Croal, Paula L.;Kassner, Andrea

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明显的缺血性中风是镰状细胞病(SCD)儿童最具破坏性的并发症之一。SCD患者对贫血的代偿反应包括通过进入脑血管扩张储备增加脑血流量(CBF)。继发于贫血应激的舒张储备耗竭可导致脑缺血。本研究的目的是调查脑血流和脑血管反应性(CVR)使用磁共振成像(MRI)与SCD儿童,并与贫血的血液学标志物。使用动脉自旋标记测量基线CBF。使用响应于CO2刺激的血氧水平依赖性MRI来获取CVR。总共有28名SCD儿童(23名未接受任何疾病改善治疗,5名接受慢性输血)和22名健康对照者使用MRI进行成像。在两个时间点对输血患者进行成像,以评估输血周期后红细胞压积变化的影响。在SCD儿童中,与健康对照组相比,CBF显著升高,而CVR显著降低。这两项措施显着相关的红细胞压积。对于输血患者,在一个输血周期后,CBF降低,CVR增加。最后,在SCD儿童和健康对照组中,观察到CBF和CVR之间存在显著相关性。
Overt ischaemic stroke is one of the most devastating complications in children with sickle cell disease (SCD). The compensatory response to anaemia in SCD includes an increase in cerebral blood flow (CBF) by accessing cerebrovascular dilatory reserve. Exhaustion of dilatory reserve secondary to anaemic stress may lead to cerebral ischaemia. The purpose of this study was to investigate CBF and cerebrovascular reactivity (CVR) using magnetic resonance imaging (MRI) in children with SCD and to correlate these with haematological markers of anaemia. Baseline CBF was measured using arterial spin labelling. Blood-oxygen level-dependent MRI in response to a CO2 stimulus was used to acquire CVR. In total, 28 children with SCD (23 not on any disease-modifying treatment, 5 on chronic transfusion) and 22 healthy controls were imaged using MRI. Transfusion patients were imaged at two time points to assess the effect of changes in haematocrit after a transfusion cycle. In children with SCD, CBF was significantly elevated compared to healthy controls, while CVR was significantly reduced. Both measures were significantly correlated with haematocrit. For transfusion patients, CBF decreased and CVR increased following a transfusion cycle. Lastly, a significant correlation was observed between CBF and CVR in both children with SCD and healthy controls.