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Updated: Jul 27, 2025
Determining Ciliary Function and Membrane Impermeability of the Pseudostratified Lung Airway Epithelium
Published on: February 21, 2025
Baseline reticular basement membrane morphology is related to subsequent spirometry deterioration in pediatric
Václav Koucký1,2, Jiří Uhlík2, Lenka Hoňková1,2
1Department of Paediatrics, Second Faculty of Medicine, Charles University and University Hospital Motol, Prague, Czech Republic.
Insights
Reticular basement membrane (RBM) thickening in children with asthma, cystic fibrosis, and primary ciliary dyskinesia predicts future lung function decline. Baseline RBM morphology and ventilation inhomogeneity help predict spirometry trends.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Cellular Biology
Background:
- Reticular basement membrane (RBM) thickening is observed in pediatric allergic bronchial asthma (BA), cystic fibrosis (CF), and primary ciliary dyskinesia (PCD).
- The functional impact of RBM thickening on lung function progression in these conditions is not well understood.
Purpose of the Study:
- To investigate the relationship between baseline RBM thickness and subsequent spirometry trends in children with BA, CF, and PCD.
- To determine if RBM morphology and ventilation inhomogeneity can predict future lung function decline.
Main Methods:
- A cohort follow-up study involving pediatric patients (3-18 years) with BA, CF, PCD, and healthy controls.
- Baseline measurements included lung clearance index (LCI), spirometry, and endobronchial biopsy for RBM and collagen IV thickness.
- Longitudinal spirometry data (FVC, FEV1, FEV1/FVC) were analyzed using univariate and multiple regression models.
Main Results:
- RBM was significantly thicker in patients with BA, CF, and PCD compared to controls (P < 0.001).
- LCI was elevated in CF and PCD patients versus controls (P < 0.001, P = 0.002).
- Spirometry z-scores (FEV1, FEV1/FVC) deteriorated significantly in all patient groups but not controls.
- Baseline RBM morphology and LCI correlated with FEV1 trends in CF and PCD; collagen IV correlated in BA.
- RBM morphology and ventilation inhomogeneity predicted up to 84.4% of spirometry trend variability.
Conclusions:
- Baseline RBM morphology and LCI are significant predictors of subsequent spirometry trends in children with BA, CF, and PCD.
- These findings suggest that RBM characteristics and ventilation patterns may play a crucial role in the progression of lung disease in these pediatric populations.
- The study proposes novel predictive models for lung function development based on endobronchial biopsy and lung clearance index measurements.
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.
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