Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets
Air Trapping and Airflow Obstruction in Newborn Cystic Fibrosis Piglets
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DOI：
10.1164/rccm.201307-1268oc
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发表时间：
2013-12-15
影响因子：
24.7
通讯作者：
Stoltz, David A.
中科院分区：
文献类型：
--
作者：
Adam, Ryan J.;Michalski, Andrew S.;Stoltz, David A.
理论基础：囊性纤维化婴儿的空气滞留和气流阻塞的病例越来越多。这些发现通常归因于呼吸道感染、炎症和粘液堆积。目的：了解在囊性纤维化的呼吸道感染和炎症发生之前是否存在空气滞留和气流阻塞。方法：出生时，囊性纤维化的仔猪没有呼吸道感染和炎症。因此，我们使用新生的野生型仔猪和囊性纤维化仔猪，通过吸气和呼气X射线计算机断层扫描来评估空气滞留、呼吸道大小和肺容量。使用微型计算机断层扫描来评估更远端的呼吸道大小。用机械呼吸机测定气道阻力。测量和主要结果：出生当天，囊性纤维化仔猪比野生型仔猪更容易出现空气滞留(分别为75%和12.5%)。此外，患有囊性纤维化的新生仔猪的呼吸道阻力增加，并伴有气管、主干支气管和近端呼吸道的管腔缩小。结论：囊性纤维化新生仔猪在发生呼吸道感染、炎症和粘液蓄积之前，存在空气滞留、气流阻塞和气道内径缩小，提示囊性纤维化对呼吸道发育有影响。我们的研究结果表明，囊性纤维化婴儿早期的气流阻塞和空气滞留可能部分是由先天性呼吸道异常引起的。
Rationale: Air trapping and airflow obstruction are being increasingly identified in infants with cystic fibrosis. These findings are commonly attributed to airway infection, inflammation, and mucus buildup.Objectives: To learn if air trapping and airflow obstruction are present before the onset of airway infection and inflammation in cystic fibrosis.Methods: On the day they are born, piglets with cystic fibrosis lack airway infection and inflammation. Therefore, we used newborn wild-type piglets and piglets with cystic fibrosis to assess air trapping, airway size, and lung volume with inspiratory and expiratory X-ray computed tomography scans. Micro-computed tomography scanning was used to assess more distal airway sizes. Airway resistance was determined with a mechanical ventilator. Mean linear intercept and alveolar surface area were determined using stereologic methods.Measurements and Main Results: On the day they were born, piglets with cystic fibrosis exhibited air trapping more frequently than wild-type piglets (75% vs. 12.5%, respectively). Moreover, newborn piglets with cystic fibrosis had increased airway resistance that was accompanied by luminal size reduction in the trachea, mainstem bronchi, and proximal airways. In contrast, mean linear intercept length, alveolar surface area, and lung volume were similar between both genotypes.Conclusions: The presence of air trapping, airflow obstruction, and airway size reduction in newborn piglets with cystic fibrosis before the onset of airway infection, inflammation, and mucus accumulation indicates that cystic fibrosis impacts airway development. Our findings suggest that early airflow obstruction and air trapping in infants with cystic fibrosis might, in part, be caused by congenital airway abnormalities.