Lung T1 mapping magnetic resonance imaging in the assessment of pulmonary disease in children with cystic fibrosis: a pilot study.,Pediatric Radiology

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Lung T1 mapping magnetic resonance imaging in the assessment of pulmonary disease in children with cystic fibrosis: a pilot study.
Pediatric Radiology ( IF 2.3 ) Pub Date : 2020-03-11 , DOI: 10.1007/s00247-020-04638-9
Fatima Neemuchwala ₁ , Maryam Ghadimi Mahani _{2,

3} , Yuxi Pang ₄ , Eunjee Lee ₅ , Timothy D Johnson ₆ , Craig J Galbán ₄ , Aleksa B Fortuna ₄ , Ramon Sanchez-Jacob ₇ , Chris A Flask ₈ , Samya Z Nasr ₉

Affiliation

Division of Pediatric Pulmonology, Department of Pediatrics, University of California San Francisco, San Francisco, CA, USA.
Section of Pediatric Radiology, C. S. Mott Children's Hospital, Department of Radiology, University of Michigan, 1540 E. Hospital Drive, SPC 4252, Ann Arbor, MI, 48109, USA.
Division of Cardiothoracic Radiology, Department of Radiology, University of Michigan, Ann Arbor, MI, USA.
Department of Radiology, University of Michigan, Ann Arbor, MI, USA.
Department of Information and Statistics, Chungnam National University, Daejeon, Republic of Korea.
Department of Biostatistics, University of Michigan, Ann Arbor, MI, USA.
Department of Radiology, George Washington University Medical Center, Children's National Health System, Washington, DC, USA.
Departments of Radiology, Biomedical Engineering, and Pediatrics, Case Western Reserve University, Cleveland, OH, USA.
Division of Pediatric Pulmonology, C. S. Mott Children's Hospital, Department of Pediatrics, University of Michigan, Ann Arbor, MI, USA.

Background

Assessment tools for early cystic fibrosis (CF) lung disease are limited. Detecting early pulmonary disease is crucial to increasing life expectancy by starting interventions to slow the progression of the pulmonary disease with the many treatment options available.

Objective

To compare the utility of lung T1-mapping MRI with ultrashort echo time (UTE) MRI in children with cystic fibrosis in detecting early stage lung disease and monitoring pulmonary exacerbations.

Materials and methods

We performed a prospective study in 16 children between September 2017 and January 2018. In Phase 1, we compared five CF patients with normal spirometry (mean 11.2 years) to five age- and gender-matched healthy volunteers. In Phase 2, we longitudinally evaluated six CF patients (median 11 years) in acute pulmonary exacerbation. All children had non-contrast lung T1-mapping and UTE MRI and spirometry testing. We compared the mean normalized T1 value and percentage lung volume without T1 value in CF patients and healthy subjects in Phase 1 and during treatment in Phase 2. We also performed cystic fibrosis MRI scoring. We evaluated differences in continuous variables using standard statistical tests.

Results

In Phase 1, mean normalized T1 values of the lung were significantly lower in CF patients in comparison to healthy controls (P=0.02) except in the right lower lobe (P=0.29). The percentage lung volume without T1 value was also significantly higher in CF patients (P=0.006). UTE MRI showed no significant differences between CF patients and healthy volunteers (P=0.11). In Phase 2, excluding one outlier case who developed systemic disease in the course of treatment, the whole-lung T1 value increased (P=0.001) and perfusion scoring improved (P=0.02) following therapy. We observed no other significant changes in the MRI scoring.

Conclusion

Lung T1-mapping MRI can detect early regional pulmonary CF disease in children and might be helpful in the assessment of acute pulmonary exacerbations.

中文翻译：

肺T1映射磁共振成像在囊性纤维化患儿肺部疾病评估中的初步研究。

背景

早期囊性纤维化（CF）肺部疾病的评估工具有限。通过采用多种可用治疗方法开始干预以减缓肺部疾病的进展，发现早期肺部疾病对于延长预期寿命至关重要。

目的

为了比较肺T1映射MRI与超短回声时间（UTE）MRI在囊性纤维化患儿中检测早期肺疾病和监测肺病情加重的实用性。

材料和方法

我们在2017年9月至2018年1月期间对16名儿童进行了一项前瞻性研究。在第一阶段，我们将5例肺活量正常的CF患者（平均11.2岁）与5名年龄和性别相匹配的健康志愿者进行了比较。在第2阶段，我们纵向评估了6例CF患者（中位年龄为11岁），他们患有急性肺部加重。所有儿童均进行了非对照肺T1映射和UTE MRI和肺活量测定。我们比较了第一阶段和第二阶段治疗期间CF患者和健康受试者的平均标准化T1值和无T1值的肺体积百分比。我们还进行了囊性纤维化MRI评分。我们使用标准统计检验评估了连续变量的差异。

结果

在第1阶段，除右下叶（P = 0.29）外，CF患者的平均标准化T1值与健康对照组相比（P = 0.02）显着降低。CF患者中没有T1值的肺容量百分比也显着更高（P = 0.006）。UTE MRI显示CF患者与健康志愿者之间无显着差异（P = 0.11）。在阶段2中，除了一名在治疗过程中发生全身性疾病的异常病例外，治疗后全肺T1值升高（P = 0.001），灌注评分得到改善（P = 0.02）。我们在MRI评分中没有观察到其他显着变化。