MRI-based Quantitative Brain Perfusion Mapping for Sickle Cell Disease

基于 MRI 的镰状细胞病定量脑灌注图

• 批准号: 9187493
• 负责人: Qin Qin
• 金额: $ 17.64万
• 依托单位: HUGO W. MOSER RES INST KENNEDY KRIEGER
• 依托单位国家: 美国
• 财政年份: 2013
• 资助国家: 美国
• 起止时间: 2013-12-20 至 2018-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/9187493
• 关键词: Abnormal Hemoglobins; Adolescent; Adult; Affect; Age; Angiography; Blood; Blood Circulation; Blood Transfusion; Bolus Infusion; Brain; Brain Injuries; Cause of Death; Cerebral Infarction; Cerebrovascular Circulation; Cerebrum; Characteristics; Child; Childhood; Clinical; Contrast Media; Detection; Disease; Early identification; Etiology; Evaluation; Failure; Focal Neurologic Deficits; Genetic; Goals; Half-Life; Hematocrit procedure; Hematological Disease; Homeostasis; Image; Imaging Techniques; Impairment; Ionizing radiation; Label; Longitudinal Studies; Magnetic Resonance Imaging; Measurement; Measures; Microvascular Dysfunction; Monitor; Morbidity - disease rate; Neurologic; Oxygen; Patients; Perfusion; Perfusion Weighted MRI; Phase; Population; Positron-Emission Tomography; Predictive Value; Predisposition; Property; Protocols documentation; Radiation; Recording of previous events; Recurrence; Relaxation; Reproducibility; Research; Risk; Risk Factors; Role; Sickle Cell Anemia; Sickle Hemoglobin; Slice; Spin Labels; Stroke; Stroke prevention; Techniques; Testing; Time; Transfusion; Validation; Water; base; cerebral artery; cerebrovascular; clinical efficacy; disability; healthy volunteer; hemodynamics; high risk; improved; mortality; neurovascular; non-invasive monitor; perfusion imaging; prevent; public health relevance; research study; response; screening; single photon emission computed tomography

DESCRIPTION (provided by applicant): SCD is a genetic blood disorder characterized by abnormal hemoglobin S (HbS) synthesis with sickle cell anemia (SCA) as the most common subtype. Stroke is the most frequent cause of death and long-term disability in both children and adults with SCD. Studies of patients with focal neurologic deficits have revealed abnormalities of both large and small vessels in SCD. Currently, the main clinical screening test is the use of transcranial Doppler (TCD) for neurologically asymptomatic children with SCD. The TCD test has a low positive predictive value and is not effective to screen stroke risk in adult SCD patients. Cerebral blood flow (CBF), a measure of the microvascular perfusion of the brain, has been recognized as a potentially more sensitive and specific indicator of early cerebrovascular impairment in both children and adults with SCD. CBF measurement based on the arterial spin labeling (ASL) MRI technique is promising as it does not require a contrast agent and is free of radiation. However, the existing ASL techniques often acquire 2D multi-slice perfusion-weighted MRI at a single post-labeling delay and, for CBF calculation, make assumptions on a number of parameters that are related to blood properties, such as arterial arrival time (AAT), longitudinal relaxation time (T1) of blood and labeling efficiency. The overall goal of the research proposed is to develop and validate a multi-delay 3D ASL protocol combined with per-subject measurements of blood T1 and labeling efficiency, which provides accurate and reliable perfusion metrics (CBF and AAT) for SCD patients. The measured CBF and AAT are expected to allow detection of both small and large vessel disease simultaneously for SCD. The specific aims of this study is first to develop a comprehensive and fast ASL-based perfusion protocol and demonstrate its reliability and reproducibility in healthy volunteers (Aim 1), then to validate the measured CBF and AAT with phase-contrast MR Angiography (PC-MRA) and dynamic susceptibility contrast (DSC) MRI in a group of SCA patients without a history of stroke (Aim 2), and finally to monitor CBF and AAT in patients with SCD and a history of stroke to explore the association between CBF and AAT and recurrent brain injury (Aim 3).

描述（由申请人提供）：SCD是一种遗传性血液疾病，其特征是与镰状细胞贫血（SCA）合成异常的血红蛋白S（HBS）作为最常见的亚型。中风是SCD儿童和成人的死亡和长期残疾原因。对局灶性神经系统缺陷的患者的研究表明，SCD中大和小血管的异常。目前，主要的临床筛查测试是使用SCD的神经学无症状儿童的经颅多普勒（TCD）使用。 TCD测试的阳性预测值低，并且在成年SCD患者中筛选中风风险无效。脑血流（CBF）是大脑微血管灌注的一种度量，已被认为是SCD儿童和成人的早期脑血管障碍的可能更敏感和更具体的指标。基于动脉自旋标记（ASL）MRI技术的CBF测量很有希望，因为它不需要造影剂，并且没有辐射。但是，现有的ASL技术通常以单个标签后的延迟获得2D多切片灌注加权MRI，并且对于CBF计算，对与血液特性相关的许多参数（例如动脉到达时间（AAT），longudinal Helightable Sleasation timational和Babeeling Alld和Babeeling and Blood and BabeLecring和BabeLED效率）做出了假设。提出的研究的总体目标是开发和验证一种多延迟3D ASL方案，并结合了血液T1和标记效率的人均测量结果，该协议为SCD患者提供了准确可靠的灌注指标（CBF和AAT）。预计测得的CBF和AAT将允许同时检测出SCD的小血管疾病。这项研究的具体目的首先是开发一种全面，快速的基于ASL的灌注方案，并证明其在健康志愿者中的可靠性和可重复性（AIM 1），然后验证 在没有中风病史（AIM 2）的一组SCA患者中，具有相对比较MR血管造影（PC-MRA）和动态易感性对比（DSC）MRI的CBF和AAT（AIM 2），最终在SCD患者中监测CBF和AAT，并在SCD患者中监测CBF和AAT，并探索CBF和AAT和AAT和AAT和Recurent and Brain Inlucter and aat and Recurrent and Recurent and Recurrent and Recurrent and Recurrent and aT sai的史。