通过 MRI 对儿科镰状细胞病患者的血脑屏障通透性进行成像：一项可行性研究。
Imaging Blood-Brain Barrier Permeability Through MRI in Pediatric Sickle Cell Disease: A Feasibility Study.
机构信息
The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Department of Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland, USA.
出版信息
J Magn Reson Imaging. 2022 May;55(5):1551-1558. doi: 10.1002/jmri.27965. Epub 2021 Oct 22.
BACKGROUND
Blood-brain barrier (BBB) disruption may lead to endothelium dysfunction and inflammation in sickle cell disease (SCD). However, abnormalities of BBB in SCD, especially in pediatric patients for whom contrast agent administration less than optimal, have not been fully characterized.
PURPOSE
To examine BBB permeability to water in a group of pediatric SCD participants using a non-invasive magnetic resonance imaging technique. We hypothesized that SCD participants will have increased BBB permeability.
STUDY TYPE
Prospective cross-sectional.
POPULATION
Twenty-six pediatric participants (10 ± 1 years, 15F/11M) were enrolled, including 21 SCD participants and 5 sickle cell trait (SCT) participants, who were siblings of SCD patients.
FIELD STRENGTH/SEQUENCE: 3 T. Water extraction with phase-contrast arterial spin tagging with echo-planer imaging, phase-contrast and T -weighted magnetization-prepared rapid acquisition of gradient echo.
ASSESSMENT
Water extraction fraction (E), BBB permeability-surface area product (PS), cerebral blood flow, hematological measures (hemoglobin, hematocrit, hemoglobin S), neuropsychological scores (including domains of intellectual ability, attention and executive function, academic achievement and adaptive function, and a composite score). Regions of interest were drawn by Z.L. (6 years of experience).
STATISTICAL TESTS
Wilcoxon rank sum test and chi-square test for group comparison of demographics. Multiple linear regression analysis of PS with diagnostic category (SCD or SCT), hematological measures, and neuropsychological scores. A two-tailed P value of 0.05 or less was considered statistically significant.
RESULTS
Compared with SCT participants, SCD participants had a significantly higher BBB permeability to water (SCD: 207.0 ± 33.3 mL/100 g/minute, SCT: 171.2 ± 27.2 mL/100 g/minute). SCD participants with typically more severe phenotypes also had a significantly leakier BBB than those with typically milder phenotypes (severe: 217.3 ± 31.7 mL/100 g/minute, mild: 193.3 ± 31.8 mL/100 g/minute). Furthermore, more severe BBB disruption was associated with worse hematological symptoms, including lower hemoglobin concentrations (β = -8.84, 95% confidence interval [CI] [-14.69, -3.00]), lower hematocrits (β = -2.96, 95% CI [-4.84, -1.08]), and higher hemoglobin S fraction (β = 0.77, 95% CI [0.014, 1.53]).
DATA CONCLUSION
These findings support a potential role for BBB dysfunction in SCD pathogenesis of ischemic injury.
LEVEL OF EVIDENCE
2 TECHNICAL EFFICACY: Stage 2.
背景
血脑屏障（BBB）破坏可导致镰状细胞病（SCD）中的内皮功能障碍和炎症。然而，SCD 中 BBB 的异常，特别是对于那些不太适合使用造影剂的儿科患者，尚未得到充分描述。
目的
使用一种非侵入性磁共振成像技术检查一组儿科 SCD 参与者的 BBB 水通透性。我们假设 SCD 参与者的 BBB 通透性会增加。
研究类型
前瞻性横断面研究。
人群
共纳入 26 名儿科参与者（10±1 岁，15 名女性/11 名男性），包括 21 名 SCD 参与者和 5 名镰状细胞特征（SCT）参与者，他们是 SCD 患者的兄弟姐妹。
磁场强度/序列：3T。相位对比动脉自旋标记与回波平面成像、相位对比和 T1 加权快速获取梯度回波，用于水提取分数（E）、BBB 表面积渗透率产物（PS）、脑血流、血液学指标（血红蛋白、血细胞比容、血红蛋白 S）、神经心理学评分（包括智力能力、注意力和执行功能、学业成绩和适应功能以及综合评分等领域）。感兴趣区域由 Z.L.（6 年经验）绘制。
统计学检验
Wilcoxon 秩和检验和卡方检验用于组间比较人口统计学数据。使用诊断类别（SCD 或 SCT）、血液学指标和神经心理学评分对 PS 进行多元线性回归分析。双侧 P 值<0.05 被认为具有统计学意义。
结果
与 SCT 参与者相比，SCD 参与者的 BBB 对水的通透性明显更高（SCD：207.0±33.3 mL/100 g/min，SCT：171.2±27.2 mL/100 g/min）。通常具有更严重表型的 SCD 参与者的 BBB 也比通常具有更轻微表型的参与者更漏（严重：217.3±31.7 mL/100 g/min，轻度：193.3±31.8 mL/100 g/min）。此外，更严重的 BBB 破坏与更严重的血液学症状相关，包括更低的血红蛋白浓度（β=-8.84，95%置信区间[CI] [-14.69，-3.00]）、更低的血细胞比容（β=-2.96，95% CI [-4.84，-1.08]）和更高的血红蛋白 S 分数（β=0.77，95% CI [0.014，1.53]）。
数据结论
这些发现支持 BBB 功能障碍在 SCD 缺血性损伤发病机制中的潜在作用。
证据水平
2 技术功效：第 2 阶段。
Zotero 插件