Respiratory Medicine, Princess Margaret Hospital for Children, Perth, Australia.
PLoS One. 2011;6(8):e23932. doi: 10.1371/journal.pone.0023932. Epub 2011 Aug 19.
In school-aged children with cystic fibrosis (CF) structural lung damage assessed using chest CT is associated with abnormal ventilation distribution. The primary objective of this analysis was to determine the relationships between ventilation distribution outcomes and the presence and extent of structural damage as assessed by chest CT in infants and young children with CF.
Data of infants and young children with CF diagnosed following newborn screening consecutively reviewed between August 2005 and December 2009 were analysed. Ventilation distribution (lung clearance index and the first and second moment ratios [LCI, M(1)/M(0) and M(2)/M(0), respectively]), chest CT and airway pathology from bronchoalveolar lavage were determined at diagnosis and then annually. The chest CT scans were evaluated for the presence or absence of bronchiectasis and air trapping.
Matched lung function, chest CT and pathology outcomes were available in 49 infants (31 male) with bronchiectasis and air trapping present in 13 (27%) and 24 (49%) infants, respectively. The presence of bronchiectasis or air trapping was associated with increased M(2)/M(0) but not LCI or M(1)/M(0). There was a weak, but statistically significant association between the extent of air trapping and all ventilation distribution outcomes.
These findings suggest that in early CF lung disease there are weak associations between ventilation distribution and lung damage from chest CT. These finding are in contrast to those reported in older children. These findings suggest that assessments of LCI could not be used to replace a chest CT scan for the assessment of structural lung disease in the first two years of life. Further research in which both MBW and chest CT outcomes are obtained is required to assess the role of ventilation distribution in tracking the progression of lung damage in infants with CF.
在学龄期囊性纤维化(CF)患儿中,胸部 CT 评估的结构性肺损伤与通气分布异常有关。本分析的主要目的是确定通气分布结果与胸部 CT 评估的结构性损伤的存在和程度之间的关系,这些结果来自于 CF 婴儿和幼儿。
分析了 2005 年 8 月至 2009 年 12 月连续接受新生儿筛查诊断的 CF 婴儿和幼儿的数据。在诊断时以及随后每年确定通气分布(肺清除指数和第一和第二时刻比[LCI、M(1)/M(0)和 M(2)/M(0)])、胸部 CT 和支气管肺泡灌洗液中的气道病理学。胸部 CT 扫描评估支气管扩张和空气潴留的存在或不存在。
在 49 例(31 例男性)有支气管扩张和空气潴留的婴儿中,有匹配的肺功能、胸部 CT 和病理学结果。13 例(27%)和 24 例(49%)婴儿存在支气管扩张或空气潴留。支气管扩张或空气潴留的存在与 M(2)/M(0)增加有关,但与 LCI 或 M(1)/M(0)无关。空气潴留的程度与所有通气分布结果之间存在弱但有统计学意义的关联。
这些发现表明,在早期 CF 肺部疾病中,通气分布与胸部 CT 评估的肺损伤之间存在微弱的关联。这些发现与在年龄较大的儿童中报告的结果不同。这些发现表明,在生命的头两年,LCI 的评估不能代替胸部 CT 扫描来评估结构性肺病。需要进一步研究,同时获得 MBW 和胸部 CT 结果,以评估通气分布在跟踪 CF 婴儿肺损伤进展中的作用。