Hyperpolarized (129)Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.
Hyperpolarized (129)Xe for investigation of mild cystic fibrosis lung disease in pediatric patients.
复制标题
超极化（129）XE用于研究小儿患者的轻度囊性纤维化肺疾病。
DOI：
10.1016/j.jcf.2016.07.008
复制
发表时间：
2017-03
期刊：
影响因子：
--
通讯作者：
Woods JC
中科院分区：
文献类型：
--
作者：
Thomen RP;Walkup LL;Roach DJ;Cleveland ZI;Clancy JP;Woods JC
囊性纤维化（CF）是一种由肺功能衰退引起的高发病率和死亡率的遗传性疾病。监测年轻患者的疾病进展和治疗反应是可取的，但通过CT进行连续成像通常被认为是禁止的，并且使用常规成像技术无法获得详细的功能信息。超极化129Xe磁共振成像（MRI）可以描述和量化局部通气，但尚未在儿科进行研究。我们假设129Xe MRI是可行的，并且可以比FEV1更敏感地显示轻度CF肺部疾病的通气缺陷。本研究招募了11名健康对照（6-16岁）和11名轻度CF患者（8-16岁，用力呼气量（FEV1）百分比预测bbb70 %）。9例CF患者FEV1发生率为85%。每个受试者通过超极化129Xe MRI成像，并测量通风缺陷百分比（VDP）。比较各组近期FEV1和VDP。对照组FEV1为100.3%±8.5% (mean±sd)， CF患者FEV1为97.9%±16.0% （p=0.67）。对照组VDP为6.4%±2.8%，CF为18.3%±8.6% （p<0.001）。9例FEV1正常（bb0 85%）的CF患者，平均FEV1为103.1%±12.3% (p=0.57)， VDP为15.4%±6.3% （p=0.002）。超极化129Xe MRI显示FEV1正常的CF患者存在通气缺陷，比FEV1更有效地区分CF与对照组。因此，129Xe可能是检测轻度CF肺病、调查小儿肺部疾病的局部肺功能和跟踪疾病进展的有用结果指标。
Cystic fibrosis (CF) is a genetic disease which carries high morbidity and mortality from lung-function decline. Monitoring disease progression and treatment response in young patients is desirable, but serial imaging via CT is often considered prohibitive, and detailed functional information cannot be obtained using conventional imaging techniques. Hyperpolarized 129Xe magnetic resonance imaging (MRI) can depict and quantify regional ventilation, but has not been investigated in pediatrics. We hypothesized that 129Xe MRI is feasible and would demonstrate ventilation defects in mild CF lung disease with greater sensitivity than FEV1. 11 healthy controls (age 6–16 years) and 11 patients with mild CF (age 8–16 years, Forced Expiratory Volume (FEV1) percent predicted >70%) were recruited for this study. Nine CF patients had an FEV1>85%. Each subject was imaged via hyperpolarized 129Xe MRI, and the ventilation defect percentage (VDP) was measured. Recent FEV1 and VDP were compared between the groups. FEV1 for controls was 100.3%±8.5% (mean ± sd) and for CF patients was 97.9%±16.0% (p=0.67). VDP was 6.4%±2.8% for controls and 18.3%±8.6% for CF (p<0.001). When considering the 9 CF patients with normal FEV1 (>85%), the mean FEV1 was 103.1%±12.3% (p=0.57 compared to controls) and VDP was 15.4% ± 6.3% (p=0.002). Hyperpolarized 129Xe MRI demonstrated ventilation defects in CF patients with normal FEV1 and more effectively discriminated CF from controls than FEV1. Thus 129Xe may be a useful outcome measure to detect mild CF lung disease and to investigate regional lung function in pediatric lung diseases and to follow disease progression.