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感染后闭塞性细支气管炎患儿肺和气道的发育异常

Dysanaptic growth of lung and airway in children with post-infectious bronchiolitis obliterans.

作者信息

Mosquera Ricardo A, Hashmi Syed S, Pacheco Susan E, Reverdin Alexandra, Chevallier Justyna, Colasurdo Giuseppe N

机构信息

Division of Pulmonary Medicine, Department of Pediatrics, University of Texas Medical School at Houston, Houston, TX, USA.

出版信息

Clin Respir J. 2014 Jan;8(1):63-71. doi: 10.1111/crj.12037. Epub 2013 Oct 1.

DOI:10.1111/crj.12037
PMID:23800208
Abstract

RATIONALE

Post-infectious bronchiolitis obliterans (PBO) is a rare form of chronic obstructive lung disease associated with small airway fibrosis following a severe insult to the lower respiratory tract. It has been suggested that PBO is a non-progressive disease. However, evidence supporting this statement is limited. In this case series, we sought to determine the changes of pulmonary function tests (PFT) over time in children with PBO.

METHODS

Seven children with PBO, ages 6-15 years old, were retrospectively studied between 1994 and 2012. Spirometry and lung volumes tests were performed in accordance with American Thoracic Society (ATS) guidelines and were monitored over time. The average rate of change was calculated using generalized linear mixed models.

RESULTS

The median baseline values for forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), the FEV1/FVC ratio and forced expiratory flow 25%-75% (FEF25%-75%) were 57%, 50%, 87% and 29%, respectively. FVC increased at a rate of 1.8% per year (P = 0.008). There was no significant change in FEV1 over time (P = 0.112). However, the FEV1/FVC ratio decreased by 2.6% per year (P < 0.001).

CONCLUSION

PFT in childhood PBO was characterized by significant airway obstruction. Over time, FVC (lung parenchyma) increased and FEV1 (airway) remained stable, but FEV1/FVC ratio declined more than expected, suggesting a mismatch in the growth of the airway and lung parenchyma (dysanaptic growth). Further studies in larger populations are needed to validate these observations.

摘要

原理

感染后闭塞性细支气管炎(PBO)是一种罕见的慢性阻塞性肺疾病,与下呼吸道严重损伤后的小气道纤维化有关。有人提出PBO是一种非进行性疾病。然而,支持这一说法的证据有限。在这个病例系列中,我们试图确定PBO患儿肺功能测试(PFT)随时间的变化。

方法

对1994年至2012年间7名年龄在6至15岁的PBO患儿进行回顾性研究。根据美国胸科学会(ATS)指南进行肺活量测定和肺容积测试,并随时间进行监测。使用广义线性混合模型计算平均变化率。

结果

用力肺活量(FVC)、第1秒用力呼气量(FEV1)、FEV1/FVC比值和25%-75%用力呼气流量(FEF25%-75%)的基线中位数分别为57%、50%、87%和29%。FVC以每年1.8%的速度增加(P = 0.008)。FEV1随时间无显著变化(P = 0.112)。然而,FEV1/FVC比值每年下降2.6%(P < 0.001)。

结论

儿童PBO的PFT表现为明显的气道阻塞。随着时间的推移,FVC(肺实质)增加,FEV1(气道)保持稳定,但FEV1/FVC比值下降幅度超过预期,提示气道和肺实质生长不匹配(发育异常生长)。需要在更大的人群中进行进一步研究以验证这些观察结果。

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