Longitudinal Magnetic Resonance Imaging of Changes in Lung Morphology and Perfusion in Children with Cystic Fibrosis From Infancy through Adolescence.

IF 6.8 2区 医学 Q1 RESPIRATORY SYSTEM Annals of the American Thoracic Society Pub Date : 2024-09-10 DOI:10.1513/annalsats.202404-396oc
Mark O Wielpütz,Mirjam Stahl,Simon M F Triphan,Lena Wucherpfennig,Patricia Leutz-Schmidt,Sonja Gestewitz,Eva Steinke,Simon Y Graeber,Hans-Ulrich Kauczor,Monika Eichinger,Michael U Puderbach,Abdulsattar Alrajab,Jens-Peter Schenk,Olaf Sommerburg,Marcus A Mall
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Abstract

RATIONALE The progression of lung changes in cystic fibrosis (CF) from infancy through adolescence remains poorly understood due to limited longitudinal imaging data. OBJECTIVES To assess changes in lung morphology and perfusion in children with CF through the pediatric age range by longitudinal chest magnetic resonance imaging (MRI). METHODS 1112 annual chest MRI were performed in 226 patients with CF aged 0-18yr. MRI was assessed using a validated MRI scoring system. MEASUREMENTS AND MAIN RESULTS The MRI global score continuously increased from 5.5±4.6 at infancy (0yr) to 17.9±8.4 at adolescence (range 12-18yr), and the MRI morphology score from 5.0±3.9 to 12.4±6.0 (P<0.001). Bronchiectasis/wall thickening prevalence increased from 89.1% at infancy to approx. 100% from preschool age (1-5yr), and the subscore increased from 3.1±1.9 at infancy to 6.6±2.1 at adolescence (P<0.001). Mucus plugging prevalence increased from 55.4% at infancy to 83.5% at adolescence, and the subscore increased from 1.2±1.6 to 3.7±2.5 in the same period (P<0.001). Perfusion abnormalities were found in 44.4% at infancy, and increased to approx. 90% from preschool age (P<0.001). The MRI perfusion score increased from 1.1±1.6 at infancy to 5.6±3.0 at adolescence (P<0.001). Chronic Pseudomonas aeruginosa infection was associated with higher MRI scores from school age (6-11yr, P<0.05-0.001). CONCLUSIONS This is the first study assessing longitudinal changes in lung morphology and perfusion in CF throughout the pediatric age range, providing percentiles as age-specific reference for lung disease severity. Our data may facilitate the use of MRI as an endpoint in clinical trials in children with CF.
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囊性纤维化儿童从婴儿期到青春期肺形态和灌注变化的纵向磁共振成像。
目的通过纵向胸部磁共振成像(MRI)评估囊性纤维化(CF)患儿肺部形态和灌注的变化。测量和主要结果MRI总体评分从婴儿期(0yr)的5.5±4.6分持续上升到青春期(12-18yr)的17.9±8.4分,MRI形态评分从5.0±3.9分上升到12.4±6.0分(P<0.001)。支气管扩张/管壁增厚的患病率从婴儿期的89.1%增加到学龄前(1-5岁)的约100%,子分数从婴儿期的3.1±1.9增加到青春期的6.6±2.1(P<0.001)。黏液堵塞发生率从婴儿期的 55.4% 增加到青春期的 83.5%,同期的子分数从 1.2±1.6 增加到 3.7±2.5(P<0.001)。婴儿期发现灌注异常的比例为 44.4%,学龄前期增加到约 90%(P<0.001)。核磁共振灌注评分从婴儿期的 1.1±1.6 增加到青春期的 5.6±3.0(P<0.001)。结论:这是第一项评估 CF 儿童整个年龄段肺部形态和灌注纵向变化的研究,提供了百分位数作为肺部疾病严重程度的年龄特异性参考。我们的数据有助于将磁共振成像作为CF患儿临床试验的终点。
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来源期刊
Annals of the American Thoracic Society
Annals of the American Thoracic Society Medicine-Pulmonary and Respiratory Medicine
CiteScore
9.30
自引率
3.60%
发文量
0
期刊介绍: The Annals of the American Thoracic Society (AnnalsATS) is the official international online journal of the American Thoracic Society. Formerly known as PATS, it provides comprehensive and authoritative coverage of a wide range of topics in adult and pediatric pulmonary medicine, respiratory sleep medicine, and adult medical critical care. As a leading journal in its field, AnnalsATS offers up-to-date and reliable information that is directly applicable to clinical practice. It serves as a valuable resource for clinical specialists, supporting their formative and continuing education. Additionally, the journal is committed to promoting public health by publishing research and articles that contribute to the advancement of knowledge in these fields.
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