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肺磁共振成像生物标志物在α-1 抗胰蛋白酶缺乏症中的区域性气腔扩大。

Pulmonary He Magnetic Resonance Imaging Biomarkers of Regional Airspace Enlargement in Alpha-1 Antitrypsin Deficiency.

机构信息

Robarts Research Institute, 1151 Richmond Street North, London, ON, Canada N6A 5B7; Department of Medical Biophysics, The University of Western Ontario, 1151 Richmond St North, London, ON, Canada N6A 5C1.

Robarts Research Institute, 1151 Richmond Street North, London, ON, Canada N6A 5B7.

出版信息

Acad Radiol. 2017 Nov;24(11):1402-1411. doi: 10.1016/j.acra.2017.05.008. Epub 2017 Jun 20.

Abstract

RATIONALE AND OBJECTIVES

Thoracic x-ray computed tomography (CT) and hyperpolarized He magnetic resonance imaging (MRI) provide quantitative measurements of airspace enlargement in patients with emphysema. For patients with panlobular emphysema due to alpha-1 antitrypsin deficiency (AATD), sensitive biomarkers of disease progression and response to therapy have been difficult to develop and exploit, especially those biomarkers that correlate with outcomes like quality of life. Here, our objective was to generate and compare CT and diffusion-weighted inhaled-gas MRI measurements of emphysema including apparent diffusion coefficient (ADC) and MRI-derived mean linear intercept (L) in patients with AATD, chronic obstructive pulmonary disease (COPD) ex-smokers, and elderly never-smokers.

MATERIALS AND METHODS

We enrolled patients with AATD (n = 8; 57 ± 7 years), ex-smokers with COPD (n = 8; 77 ± 6 years), and a control group of never-smokers (n = 5; 64 ± 2 years) who underwent thoracic CT, MRI, spirometry, plethysmography, the St. George's Respiratory Questionnaire, and the 6-minute walk test during a single 2-hour visit. MRI-derived ADC, L, surface-to-volume ratio, and ventilation defect percent were generated for the apical, basal, and whole lung as was CT lung area ≤-950 Hounsfield units (RA), low attenuating clusters, and airway count.

RESULTS

In patients with AATD, there was a significantly different MRI-derived ADC (P = .03), L (P < .0001), and surface-to-volume ratio (P < .0001), but not diffusing capacity of carbon monoxide, residual volume or total lung capacity, or CT RA (P > .05) compared to COPD ex-smokers with a significantly different St. George's Respiratory Questionnaire.

CONCLUSIONS

In this proof-of-concept demonstration, we evaluated CT and MRI lung emphysema measurements and observed significantly worse MRI biomarkers of emphysema in patients with AATD compared to patients with COPD, although CT RA and diffusing capacity of carbon monoxide were not significantly different, underscoring the sensitivity of MRI measurements of AATD emphysema.

摘要

背景与目的

胸部 X 射线计算机断层扫描(CT)和超极化氦磁共振成像(MRI)可提供肺气肿患者的气腔扩大的定量测量。对于由于α-1 抗胰蛋白酶缺乏(AATD)引起的全小叶型肺气肿患者,很难开发和利用疾病进展和治疗反应的敏感生物标志物,尤其是那些与生活质量等结果相关的生物标志物。在这里,我们的目的是生成并比较 AATD、慢性阻塞性肺疾病(COPD)戒烟者和老年从不吸烟者的 CT 和扩散加权吸入性气体 MRI 测量值,包括肺气肿的表观扩散系数(ADC)和 MRI 衍生的平均线性截距(L)。

材料与方法

我们纳入了 8 名 AATD 患者(57±7 岁)、8 名 COPD 戒烟者(77±6 岁)和 5 名从不吸烟者的对照组(64±2 岁),他们在单次 2 小时就诊期间接受了胸部 CT、MRI、肺量计、体积描记法、圣乔治呼吸问卷和 6 分钟步行测试。为了获得顶部、底部和整个肺的 MRI 衍生 ADC、L、表面积与体积比以及通气缺陷百分比,我们还生成了 CT 肺区≤-950 个 Hounsfield 单位(RA)、低衰减簇和气道计数。

结果

与 COPD 戒烟者相比,AATD 患者的 MRI 衍生 ADC(P=0.03)、L(P<0.0001)和表面积与体积比(P<0.0001)存在显著差异,但一氧化碳弥散量、残气量或肺总量或 CT RA(P>0.05)无差异,圣乔治呼吸问卷结果也存在显著差异。

结论

在本概念验证性研究中,我们评估了 CT 和 MRI 肺部肺气肿测量值,结果发现 AATD 患者的 MRI 肺气肿生物标志物明显比 COPD 患者更差,尽管 CT RA 和一氧化碳弥散量无明显差异,但 MRI 测量 AATD 肺气肿的敏感性较高。

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