Ultra-Small Lung Cysts Impair Diffusion Without Obstructing Air Flow in Lymphangioleiomyomatosis.
Ultra-Small Lung Cysts Impair Diffusion Without Obstructing Air Flow in Lymphangioleiomyomatosis.
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DOI：
10.1016/j.chest.2021.01.077
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发表时间：
2021-07
期刊：
影响因子：
9.6
通讯作者：
Wen H
中科院分区：
文献类型：
--
作者：
Matthew BP;Hasani AM;Chen YC;Pirooznia M;Stylianou M;Rollison SF;Machado TR;Quade NM;Jones AM;Julien-Williams P;Taveira-DaSilva A;Chen MY;Moss J;Wen H
淋巴管平滑肌瘤病（LAM）是一种罕见的肺部疾病，主要见于育龄妇女，其特征是形成充满空气的囊肿，这可能与肺功能下降有关。一项实验性的区域超高分辨率CT扫描发现，相对于标准胸部CT成像，囊肿的体积增加，主要由超小囊肿组成。这些超小囊肿对LAM患者肺功能有何影响？103例LAM患者在同一次就诊中接受了肺功能检查和CT检查。使用美国食品和药物管理局批准的商业软件测量囊肿评分，即囊肿占肺体积的百分比。通过统计分析对人口统计学变量进行调整，评估囊肿评分与肺功能测试（肺一氧化碳弥散能力（Dlco）（预测%）、FEV1（预测%）和FEV1/FVC（预测%））之间的关系。观察患者平均囊肿大小和超小囊肿比例的分布。通过实验，高分辨率扫描发现的额外囊肿体积平均由2.2±0.8 mm直径的囊肿组成，因此被标记为“超小囊肿部分”。占患者总囊肿体积的27.9±19.0%。结果调整后的全肺囊肿评分更好地解释了Dlco的差异（经多次比较调整后P < 0.001），但不能解释FEV1和FEV1/FVC （P = 1.00）。超小的囊肿分数有助于Dlco的降低（P < .001），但对FEV1和FEV1/FVC没有影响（P = .760和P = .760）。575年,分别)。超小囊肿分数和平均囊肿大小与囊肿负荷、FEV1、FEV1/FVC相关，与Dlco相关性较小。超小囊肿主要有助于Dlco的降低，对FEV1和FEV1/FVC的影响很小。囊肿负荷较轻、FEV1和FEV1/FVC较好的患者，其平均囊肿大小较小，超小囊肿比例较高。ClinicalTrials.gov;否。: NCT00001465;URL: www.clinicaltrials.gov
Lymphangioleiomyomatosis (LAM) is a rare lung disease found primarily in women of childbearing age, characterized by the formation of air-filled cysts, which may be associated with reductions in lung function. An experimental, regional ultra-high resolution CT scan identified an additional volume of cysts relative to standard chest CT imaging, which consisted primarily of ultra-small cysts. What is the impact of these ultra-small cysts on the pulmonary function of patients with LAM? A group of 103 patients with LAM received pulmonary function tests and a CT examination in the same visit. Cyst score, the percentage lung volume occupied by cysts, was measured by using commercial software approved by the US Food and Drug Administration. The association between cyst scores and pulmonary function tests of diffusing capacity of the lungs for carbon monoxide (Dlco) (% predicted), FEV1 (% predicted), and FEV1/FVC (% predicted) was assessed with statistical analysis adjusted for demographic variables. The distributions of average cyst size and ultra-small cyst fraction among the patients were evaluated. The additional cyst volume identified by the experimental, higher resolution scan consisted of cysts of 2.2 ± 0.8 mm diameter on average and are thus labeled the “ultra-small cyst fraction.” It accounted for 27.9 ± 19.0% of the total cyst volume among the patients. The resulting adjusted, whole-lung cyst scores better explained the variance of Dlco (P < .001 adjusted for multiple comparisons) but not FEV1 and FEV1/FVC (P = 1.00). The ultra-small cyst fraction contributed to the reduction in Dlco (P < .001) but not to FEV1 and FEV1/FVC (P = .760 and .575, respectively). The ultra-small cyst fraction and average cyst size were correlated with cyst burden, FEV1, and FEV1/FVC but less with Dlco. The ultra-small cysts primarily contributed to the reduction in Dlco, with minimal effects on FEV1 and FEV1/FVC. Patients with lower cyst burden and better FEV1 and FEV1/FVC tended to have smaller average cyst size and higher ultra-small cyst fraction. ClinicalTrials.gov; No.: NCT00001465; URL: www.clinicaltrials.gov
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影响因子：
19.7
作者：
Avila, NA;Chen, CC;Moss, J
通讯作者：
Moss, J
影响因子：
3.9
作者：
Taveira-DaSilva AM;Moss J
通讯作者：
Moss J
DOI：
10.1152/ajplung.00176.2007
发表时间：
2007-09-01
影响因子：
4.9
作者：
Steagall, Wendy K.;Glasgow, Connie G.;Moss, Joel
通讯作者：
Moss, Joel
影响因子：
9.6
作者：
Balasubramanian, Aparna;Kolb, Todd M.;Mathai, Stephen C.
通讯作者：
Mathai, Stephen C.
影响因子：
9.6
作者：
Gopalakrishnan, Vissaagan;Yao, Jianhua;Moss, Joel
通讯作者：
Moss, Joel