Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study

Abstract Background Non-cystic fibrosis (non-CF) bronchiectasis (BE) is defined as a clinical syndrome of recurrent, persistent wet cough and abnormal bronchial dilatation on chest High Resolution Computed Tomography (HRCT) scans. The aims of this study were to characterize the pattern of the trajec...

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Main Authors: Rossella Lamberti, Simona Ferraro, Andrea Farolfi, Michele Ghezzi, Salvatore Zirpoli, Alice Marianna Munari, Sai Spandana Adivishnu, Giuseppe Marano, Elia Biganzoli, Gian Vincenzo Zuccotti, Enza D’Auria
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Language:English
Published: BMC 2024-11-01
Series:Italian Journal of Pediatrics
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Online Access:https://doi.org/10.1186/s13052-024-01799-3
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author Rossella Lamberti
Simona Ferraro
Andrea Farolfi
Michele Ghezzi
Salvatore Zirpoli
Alice Marianna Munari
Sai Spandana Adivishnu
Giuseppe Marano
Elia Biganzoli
Gian Vincenzo Zuccotti
Enza D’Auria
author_facet Rossella Lamberti
Simona Ferraro
Andrea Farolfi
Michele Ghezzi
Salvatore Zirpoli
Alice Marianna Munari
Sai Spandana Adivishnu
Giuseppe Marano
Elia Biganzoli
Gian Vincenzo Zuccotti
Enza D’Auria
author_sort Rossella Lamberti
collection DOAJ
description Abstract Background Non-cystic fibrosis (non-CF) bronchiectasis (BE) is defined as a clinical syndrome of recurrent, persistent wet cough and abnormal bronchial dilatation on chest High Resolution Computed Tomography (HRCT) scans. The aims of this study were to characterize the pattern of the trajectories of lung function parameters and to consider the relationship between the lung function and radiological severity according to the modified Reiff score. Methods The study retrospectively considered 86 children (46.5% male, median age of 4 years) with non-CF BE, admitted at the Paediatric Pneumology Unit of Buzzi Children’s Hospital from January 2015 to December 2022. The diagnosis of BE was made according to the presence of a suggestive clinical history and symptoms and key features of BE evidenced on chest HRCT scans. The modified Reiff score was adapted to quantify the severity of BE. Spirometry (COSMED MicroQuark spirometer) was performed at median age of 5.78 years (baseline or T0) and after 1 and 2 years from the baseline (T1 and T2, respectively). The general trends of lung function parameters were estimated by ANOVA models for repeated measurements. For each lung function parameter, a longitudinal regression model was fitted. The analysis was performed with the software R release 4.2.3. The statistical significance was deemed when the p-value resulted lower than 0.05. Results The general trends of lung function parameters showed a statistically significant variation of forced vital capacity (FVC%) and forced expiratory volume in 1s (FEV1%) from T0 to T1 (p = 0.0062, 0.0009) and no significant change for FVC%, FEV1% and forced expiratory flow 25–75% of VC (FEF25/75%) from T1 to T2 (p = 0.145, 0.210, 0.600, respectively). Notably, we found no correlation between the age at diagnosis and the lung function parameters at T0 (r = 0.149, 0.103 and 0.042 for FVC%, FEV1% and FEF25/75%, respectively). Instead, a poor negative correlation resulted between the Reiff score and FVC%, FEV1% e FEF25/75% at baseline (Spearman coefficients: rho=-0.156, -0.204, -0.103, respectively). Conclusions A stable pulmonary function is detectable within 2 years follow up from baseline spirometry. The modified Reiff score should be considered as a good tool not only to quantify the radiological lung involvement but also the degree of pulmonary function impairment.
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spelling doaj-art-7a77d8f2657643f1b550db4f6abe4dfd2025-08-20T02:20:48ZengBMCItalian Journal of Pediatrics1824-72882024-11-0150111010.1186/s13052-024-01799-3Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational studyRossella Lamberti0Simona Ferraro1Andrea Farolfi2Michele Ghezzi3Salvatore Zirpoli4Alice Marianna Munari5Sai Spandana Adivishnu6Giuseppe Marano7Elia Biganzoli8Gian Vincenzo Zuccotti9Enza D’Auria10Department of Pediatrics, Buzzi Children’s HospitalDepartment of Pediatrics, Buzzi Children’s HospitalDepartment of Pediatrics, Buzzi Children’s HospitalDepartment of Pediatrics, Buzzi Children’s HospitalRadiology Department, “Vittore Buzzi” Children’s HospitalRadiology Department, “Vittore Buzzi” Children’s HospitalDepartment of Biomedical and Clinical Sciences, Buzzi Children’s Hospital, University of MilanDepartment of Biomedical and Clinical Sciences, Buzzi Children’s Hospital, University of MilanDepartment of Biomedical and Clinical Sciences, Buzzi Children’s Hospital, University of MilanDepartment of Pediatrics, Buzzi Children’s HospitalDepartment of Pediatrics, Buzzi Children’s HospitalAbstract Background Non-cystic fibrosis (non-CF) bronchiectasis (BE) is defined as a clinical syndrome of recurrent, persistent wet cough and abnormal bronchial dilatation on chest High Resolution Computed Tomography (HRCT) scans. The aims of this study were to characterize the pattern of the trajectories of lung function parameters and to consider the relationship between the lung function and radiological severity according to the modified Reiff score. Methods The study retrospectively considered 86 children (46.5% male, median age of 4 years) with non-CF BE, admitted at the Paediatric Pneumology Unit of Buzzi Children’s Hospital from January 2015 to December 2022. The diagnosis of BE was made according to the presence of a suggestive clinical history and symptoms and key features of BE evidenced on chest HRCT scans. The modified Reiff score was adapted to quantify the severity of BE. Spirometry (COSMED MicroQuark spirometer) was performed at median age of 5.78 years (baseline or T0) and after 1 and 2 years from the baseline (T1 and T2, respectively). The general trends of lung function parameters were estimated by ANOVA models for repeated measurements. For each lung function parameter, a longitudinal regression model was fitted. The analysis was performed with the software R release 4.2.3. The statistical significance was deemed when the p-value resulted lower than 0.05. Results The general trends of lung function parameters showed a statistically significant variation of forced vital capacity (FVC%) and forced expiratory volume in 1s (FEV1%) from T0 to T1 (p = 0.0062, 0.0009) and no significant change for FVC%, FEV1% and forced expiratory flow 25–75% of VC (FEF25/75%) from T1 to T2 (p = 0.145, 0.210, 0.600, respectively). Notably, we found no correlation between the age at diagnosis and the lung function parameters at T0 (r = 0.149, 0.103 and 0.042 for FVC%, FEV1% and FEF25/75%, respectively). Instead, a poor negative correlation resulted between the Reiff score and FVC%, FEV1% e FEF25/75% at baseline (Spearman coefficients: rho=-0.156, -0.204, -0.103, respectively). Conclusions A stable pulmonary function is detectable within 2 years follow up from baseline spirometry. The modified Reiff score should be considered as a good tool not only to quantify the radiological lung involvement but also the degree of pulmonary function impairment.https://doi.org/10.1186/s13052-024-01799-3ChildrenNon-cystic fibrosis bronchiectasisLung function
spellingShingle Rossella Lamberti
Simona Ferraro
Andrea Farolfi
Michele Ghezzi
Salvatore Zirpoli
Alice Marianna Munari
Sai Spandana Adivishnu
Giuseppe Marano
Elia Biganzoli
Gian Vincenzo Zuccotti
Enza D’Auria
Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study
Italian Journal of Pediatrics
Children
Non-cystic fibrosis bronchiectasis
Lung function
title Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study
title_full Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study
title_fullStr Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study
title_full_unstemmed Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study
title_short Lung function trajectories in children with early diagnosis of non-cystic fibrosis bronchiectasis: a retrospective observational study
title_sort lung function trajectories in children with early diagnosis of non cystic fibrosis bronchiectasis a retrospective observational study
topic Children
Non-cystic fibrosis bronchiectasis
Lung function
url https://doi.org/10.1186/s13052-024-01799-3
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