1 Department of Diagnostic and Interventional Radiology.
Am J Respir Crit Care Med. 2014 Apr 15;189(8):956-65. doi: 10.1164/rccm.201309-1659OC.
Studies demonstrating early structural lung damage in infants and preschool children with cystic fibrosis (CF) suggest that noninvasive monitoring will be important to identify patients who may benefit from early therapeutic intervention. Previous studies demonstrated that magnetic resonance imaging (MRI) detects structural and functional abnormalities in lungs from older patients with CF without radiation exposure.
To evaluate the potential of MRI to detect abnormal lung structure and perfusion in infants and preschool children with CF, and to monitor the response to therapy for pulmonary exacerbation.
MRI studies were performed in 50 children with CF (age, 3.1 ± 2.1 yr; range, 0-6 yr) in stable clinical condition (n = 40) or pulmonary exacerbation before and after antibiotic treatment (n = 10), and in 26 non-CF control subjects (age, 2.9 ± 1.9 yr). T1- and T2-weighted sequences before and after intravenous contrast and first-pass perfusion imaging were acquired, and assessed on the basis of a dedicated morphofunctional score.
MRI demonstrated bronchial wall thickening/bronchiectasis, mucus plugging, and perfusion deficits from the first year of life in most stable patients with CF (global score, 10.0 ± 4.0), but not in non-CF control subjects (score, 0.0 ± 0.0; P < 0.001). In patients with exacerbations, the global MRI score was increased to 18.0 ± 2.0 (P < 0.001), and was significantly reduced to 12.0 ± 3.0 (P < 0.05) after antibiotic therapy.
MRI detected abnormalities in lung structure and perfusion, and response to therapy for exacerbations in infants and preschool children with CF. These results support the development of MRI for noninvasive monitoring and as an end point in interventional trials for early CF lung disease. Clinical trial registered with www.clinicaltrials.gov (NCT00760071).
研究表明，囊性纤维化（CF）患儿和学龄前儿童存在早期肺部结构性损伤，这提示非侵入性监测对于发现可能受益于早期治疗干预的患者非常重要。先前的研究表明，磁共振成像（MRI）可检测无辐射暴露的年长 CF 患者的肺部结构和功能异常。
评估 MRI 检测 CF 患儿和学龄前儿童肺部结构和灌注异常的潜力，并监测对肺部恶化的治疗反应。
对 50 名 CF 患儿（年龄 3.1 ± 2.1 岁；范围 0-6 岁）进行 MRI 研究，这些患儿处于稳定的临床状态（n = 40）或在抗生素治疗前（n = 10）和治疗后（n = 10）发生肺部恶化，另外还纳入了 26 名非 CF 对照者（年龄 2.9 ± 1.9 岁）。获取静脉内对比前后的 T1-和 T2 加权序列，并根据专门的形态功能评分进行评估。
在大多数稳定的 CF 患儿中，MRI 从生命的第一年开始就显示出支气管壁增厚/支气管扩张、黏液栓和灌注不足（整体评分 10.0 ± 4.0），而非 CF 对照者无此表现（评分 0.0 ± 0.0；P < 0.001）。在恶化的患者中，整体 MRI 评分增加至 18.0 ± 2.0（P < 0.001），抗生素治疗后显著降低至 12.0 ± 3.0（P < 0.05）。
MRI 检测到 CF 患儿和学龄前儿童肺部结构和灌注异常，以及对恶化的治疗反应。这些结果支持 MRI 用于非侵入性监测以及作为早期 CF 肺部疾病介入试验的终点。该临床试验已在 www.clinicaltrials.gov 注册（NCT00760071）。
