Martínez Tanya M, Llapur Conrado J, Williams Tamica H, Coates Cathy, Gunderman Richard, Cohen Mervyn D, Howenstine Michelle S, Saba Osama, Coxson Harvey O, Tepper Robert S
Department of Pediatrics, Section of Pediatric Pulmonology, James Whitcomb Riley Hospital for Children, 702 Barnhill Drive, Room 4270, Indianapolis, IN 46202, USA.
Am J Respir Crit Care Med. 2005 Nov 1;172(9):1133-8. doi: 10.1164/rccm.200412-1665OC. Epub 2005 Jul 28.
The development of early lung disease in patients with cystic fibrosis (CF) remains poorly defined.
Determine whether asymptomatic infants with CF have evidence for changes in airway structure when assessed by high-resolution computed tomography, and whether airway structure correlates with airway function in this age group.
Thirteen infants with CF (8-33 mo) and 13 control infants (7-25 mo) were evaluated. Airway wall and lumen areas were measured from three 1-mm-thick cross-sectional images obtained from upper, middle, and lower lobes during a respiratory pause with the lungs inflated to an airway pressure of 20 cm H2O. Lung tissue density was measured from images obtained during a respiratory pause at FRC. Forced expiratory flows were measured by the rapid thoracic compression technique in 11 infants with CF.
Airway wall area increased more per unit increase in airway size, whereas airway lumen area increased less per unit increase in airway size in the CF than in the control group. Among infants with CF, a greater ratio of wall to lumen area correlated with lower airway function. In addition, lung density at relaxed (passive) FRC was lower for infants with CF than for control infants (0.38 vs. 0.43 g/ml; p < 0.02).
Our results indicate that infants with CF have thickened airway walls, narrowed airway lumens, and air trapping, when assessed by high-resolution computed tomography, and measurements of airway structure correlated with airway function.
囊性纤维化（CF）患者早期肺部疾病的发展仍未明确界定。
确定通过高分辨率计算机断层扫描评估时，无症状的CF婴儿是否有气道结构变化的证据，以及该年龄组气道结构与气道功能是否相关。
对13名CF婴儿（8 - 33个月）和13名对照婴儿（7 - 25个月）进行评估。在呼吸暂停期间，将肺部充气至气道压力20 cm H2O，从取自上叶、中叶和下叶的三张1毫米厚的横截面图像中测量气道壁和管腔面积。在功能残气量（FRC）呼吸暂停期间获得的图像中测量肺组织密度。通过快速胸廓压缩技术对11名CF婴儿进行用力呼气流量测量。
与对照组相比，CF组气道壁面积随气道大小单位增加的增加幅度更大，而气道管腔面积随气道大小单位增加的增加幅度更小。在CF婴儿中，气道壁与管腔面积的更大比例与较低的气道功能相关。此外，CF婴儿在放松（被动）FRC时的肺密度低于对照婴儿（0.38 vs. 0.43 g/ml；p < 0.02）。
我们的结果表明，通过高分辨率计算机断层扫描评估时，CF婴儿气道壁增厚、气道管腔变窄并有气体潴留，且气道结构测量值与气道功能相关。
