Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome
Aim of the study: To evaluate clinical outcome and respiratory function in patients with congenital lobar/segmental emphysema (CLE/CSE) treated conservatively. Methods: In this single-centre retrospective study we included patients ≥5-year-old with a diagnosis of CLE/CSE, treated conservatively (dia...
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Format: | Article |
Language: | English |
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Elsevier
2023-04-01
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Series: | Journal of Pediatric Surgery Open |
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Online Access: | http://www.sciencedirect.com/science/article/pii/S2949711623000060 |
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author | Alba Ganarin Valentina Agnese Ferraro Alessandra Rancan Silvia Carraro Stefania Zanconato Piergiorgio Gamba |
author_facet | Alba Ganarin Valentina Agnese Ferraro Alessandra Rancan Silvia Carraro Stefania Zanconato Piergiorgio Gamba |
author_sort | Alba Ganarin |
collection | DOAJ |
description | Aim of the study: To evaluate clinical outcome and respiratory function in patients with congenital lobar/segmental emphysema (CLE/CSE) treated conservatively. Methods: In this single-centre retrospective study we included patients ≥5-year-old with a diagnosis of CLE/CSE, treated conservatively (diagnosis from 1990). We assessed demographic/anthropometric data, clinical outcome, pulmonary function tests (PFT) analyzed according to Global Lung Initiative. GraphPadPrism® (California, USA) was used for statistical analysis. Main results: Sixteen patients were enrolled (median age 9.3y; range 5-17): 13 had a prenatal diagnosis, 3 showed symptoms at birth. On chest computed tomography (CT), performed in the first 6 months of life, 9 children had lobar emphysema and 7 segmental emphysema. All patients showed a good growth, 14 were asymptomatic, 1 had recurrent respiratory infections and wheezing, 1 had allergic asthma. In 14 patients we performed PFT (median value): FVC 93.87%pred (IQR: 85.24; 107.69), Z-Score -0.52 (IQR: -1.39; 0.77); FEV1 93%pred (IQR: 78.75; 102.23), Z-Score -0.58 (IQR: -1.92; 0.34); RV 112.48%pred (IQR: 102.26; 137.70), Z-Score 0.25 (IQR: 0.05; 0.79); FRC 106.55%pred (IQR: 94.20; 111.04), Z-Score 0.31 (IQR: -0.29; 0.67); TLC 101.44%pred (IQR: 86.98; 113.25), Z-Score 0.11 (IQR: -0.96; 0.97), RV/TLC 122.94%pred (IQR: 113.52; 144.16), Z-Score 0.54 (IQR: 0.34; 1.01). Conclusions: Our data show that patients with CLE/CSE treated conservatively have a good clinical and functional outcome. Nonetheless, due to the short follow-up, further larger studies are needed to evaluate outcome into adulthood. |
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institution | Directory Open Access Journal |
issn | 2949-7116 |
language | English |
last_indexed | 2024-03-13T01:34:50Z |
publishDate | 2023-04-01 |
publisher | Elsevier |
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spelling | doaj.art-61db864ade944c238efaf81bac13dead2023-07-04T05:12:28ZengElsevierJournal of Pediatric Surgery Open2949-71162023-04-011100008Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcomeAlba Ganarin0Valentina Agnese Ferraro1Alessandra Rancan2Silvia Carraro3Stefania Zanconato4Piergiorgio Gamba5Unit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, Italy; Corresponding author at: Unit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, Italy.Unit of Pediatric Allergy and Respiratory Medicine, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Allergy and Respiratory Medicine, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Allergy and Respiratory Medicine, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyUnit of Pediatric Surgery, Department of Women's and Children's Health, University of Padova, 35128 Padova, ItalyAim of the study: To evaluate clinical outcome and respiratory function in patients with congenital lobar/segmental emphysema (CLE/CSE) treated conservatively. Methods: In this single-centre retrospective study we included patients ≥5-year-old with a diagnosis of CLE/CSE, treated conservatively (diagnosis from 1990). We assessed demographic/anthropometric data, clinical outcome, pulmonary function tests (PFT) analyzed according to Global Lung Initiative. GraphPadPrism® (California, USA) was used for statistical analysis. Main results: Sixteen patients were enrolled (median age 9.3y; range 5-17): 13 had a prenatal diagnosis, 3 showed symptoms at birth. On chest computed tomography (CT), performed in the first 6 months of life, 9 children had lobar emphysema and 7 segmental emphysema. All patients showed a good growth, 14 were asymptomatic, 1 had recurrent respiratory infections and wheezing, 1 had allergic asthma. In 14 patients we performed PFT (median value): FVC 93.87%pred (IQR: 85.24; 107.69), Z-Score -0.52 (IQR: -1.39; 0.77); FEV1 93%pred (IQR: 78.75; 102.23), Z-Score -0.58 (IQR: -1.92; 0.34); RV 112.48%pred (IQR: 102.26; 137.70), Z-Score 0.25 (IQR: 0.05; 0.79); FRC 106.55%pred (IQR: 94.20; 111.04), Z-Score 0.31 (IQR: -0.29; 0.67); TLC 101.44%pred (IQR: 86.98; 113.25), Z-Score 0.11 (IQR: -0.96; 0.97), RV/TLC 122.94%pred (IQR: 113.52; 144.16), Z-Score 0.54 (IQR: 0.34; 1.01). Conclusions: Our data show that patients with CLE/CSE treated conservatively have a good clinical and functional outcome. Nonetheless, due to the short follow-up, further larger studies are needed to evaluate outcome into adulthood.http://www.sciencedirect.com/science/article/pii/S2949711623000060Congenital lobar emphysemaCongenital segmental emphysemaChildrenConservative treatmentRespiratory function tests |
spellingShingle | Alba Ganarin Valentina Agnese Ferraro Alessandra Rancan Silvia Carraro Stefania Zanconato Piergiorgio Gamba Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome Journal of Pediatric Surgery Open Congenital lobar emphysema Congenital segmental emphysema Children Conservative treatment Respiratory function tests |
title | Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome |
title_full | Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome |
title_fullStr | Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome |
title_full_unstemmed | Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome |
title_short | Conservative approach in lobar and segmental congenital emphysema: Lung function and clinical outcome |
title_sort | conservative approach in lobar and segmental congenital emphysema lung function and clinical outcome |
topic | Congenital lobar emphysema Congenital segmental emphysema Children Conservative treatment Respiratory function tests |
url | http://www.sciencedirect.com/science/article/pii/S2949711623000060 |
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