Abstract：
Reticular basement membrane (RBM) thickening may occur in children with allergic bronchial asthma (BA), cystic fibrosis (CF), and primary ciliary dyskinesia (PCD). Its functional consequences remain unknown. We investigated the relationship between baseline RBM thickness and subsequent spirometry. In our cohort follow-up study, patients aged 3-18 yr with BA, CF, and PCD and controls underwent baseline lung clearance index (LCI) measurement, spirometry, and endobronchial biopsy sampling. Total RBM and collagen IV-positive layer thickness were measured. Trends in forced vital capacity (FVC), forced expired volume in 1 s (FEV1), and FEV1/FVC were analyzed during follow-up, and their relationship to baseline characteristics was studied using univariate analysis and multiple regression models. Complete baseline data were available in 19 patients with BA, 30 patients with CF, 25 patients with PCD, and 19 controls. The RBM was thicker in patients with BA (6.33 ± 1.22 μm), CF (5.60 ± 1.39 μm), and PCD (6.50 ± 1.87 μm) than in controls (3.29 ± 0.55 μm) (all P < 0.001). The LCI was higher in patients with CF (15.32 ± 4.58, P < 0.001) and PCD (10.97 ± 2.46, P = 0.002) than in controls (7.44 ± 0.43). The median follow-up times were 3.6, 4.8, 5.7, and 1.9 years in patients with BA, CF, PCD, and controls, respectively. The z-scores of FEV1 and FEV1/FVC deteriorated significantly in all groups except in controls. In patients with CF and PCD, trends in FEV1 z-scores correlated with baseline LCI and RBM; in BA, it correlated with collagen IV. In multiple regression models, RBM morphology and ventilation inhomogeneity could predict up to 84.4% of variability in spirometry trends. In conclusion, baseline LCI value and RBM morphology may predict trends in subsequent spirometry.NEW & NOTEWORTHY This paper deals with the relationship between reticular basement membrane (RBM) morphology at baseline and follow-up spirometry in children with asthma, cystic fibrosis, and primary ciliary dyskinesia. For the first time, to our knowledge, the possibility to predict subsequent lung function development using selected baseline characteristics (reticular basement membrane morphology from endobronchial biopsy and ventilation inhomogeneity from nitrogen multiple breath washout test) is proposed. Corresponding predictive models are presented.
摘要：
网状基底膜(RBM)增厚可发生在过敏性支气管哮喘(BA)患儿中,囊性纤维化（CF），和原发性纤毛运动障碍（PCD）。其功能后果仍然未知。我们调查了基线RBM厚度与随后的肺活量测定之间的关系。在我们的队列随访研究中,年龄在3-18岁的BA患者，CF,PCD和对照组进行基线肺清除指数（LCI）测量，肺活量测定,和支气管活检取样。测量总RBM和胶原IV阳性层厚度。强迫肺活量（FVC）的趋势，在1秒内强制过期卷(FEV1)，随访期间分析FEV1/FVC，使用单变量分析和多元回归模型研究了它们与基线特征的关系。完整的基线数据在19BA中可用，30CF,25例PCD患者，19个控制RBM在BA中较厚(6.33±1.22μm)，CF(5.60±1.39μm),和PCD（6.50±1.87μm）比对照组（3.29±0.55μm）（所有p<0.001）。CF（15.32±4.58，p<0.001）和PCD（10.97±2.46，p=0.002）的LCI高于对照组（7.44±0.43）。BA的中位随访时间为3.6、4.8、5.7和1.9年，CF,PCD,和控件分别。除对照组外，所有组的FEV1和FEV1/FVC的Z评分均显着下降。在CF和PCD中，FEV1z分数与基线LCI和RBM相关的趋势；在BA中，它与胶原蛋白IV相关。在多元回归模型中，RBM形态和通气不均匀性可以预测肺活量测定趋势的变异性高达84.4％。总之,基线LCI值和RBM形态学可以预测随后肺活量测定的趋势。
