Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data

Abstract Background This study aimed to investigate the relationship between lung volumetry, thoracic scoliosis, and anthropometric data (height, weight, BMI) in patients with Osteogenesis Imperfecta (OI) Type 3. Three hypotheses were tested: H1 predicted lower lung volumes in patients with OI Type...

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Main Authors: Jorge Lopez-Beneyto, Elisabetta Ponte, Vicente Martínez-Sanjuan, Maria Fernandez-Velilla, Federico Mata-Escolano, Maribel Torres-Sanchez, Francisco García-Río, Shahed Nalla, Ilker Ercan, Juan A. Sanchis-Gimeno
Format: Article
Language:English
Published: BMC 2025-06-01
Series:Orphanet Journal of Rare Diseases
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Online Access:https://doi.org/10.1186/s13023-025-03797-y
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author Jorge Lopez-Beneyto
Elisabetta Ponte
Vicente Martínez-Sanjuan
Maria Fernandez-Velilla
Federico Mata-Escolano
Maribel Torres-Sanchez
Francisco García-Río
Shahed Nalla
Ilker Ercan
Juan A. Sanchis-Gimeno
author_facet Jorge Lopez-Beneyto
Elisabetta Ponte
Vicente Martínez-Sanjuan
Maria Fernandez-Velilla
Federico Mata-Escolano
Maribel Torres-Sanchez
Francisco García-Río
Shahed Nalla
Ilker Ercan
Juan A. Sanchis-Gimeno
author_sort Jorge Lopez-Beneyto
collection DOAJ
description Abstract Background This study aimed to investigate the relationship between lung volumetry, thoracic scoliosis, and anthropometric data (height, weight, BMI) in patients with Osteogenesis Imperfecta (OI) Type 3. Three hypotheses were tested: H1 predicted lower lung volumes in patients with OI Type 3 compared to controls, H2 predicted differences between right and left lung volumes in patients with OI Type 3 due to chest deformities, and H3 predicted a correlation between lung volumes in patients with OI Type 3 and their thoracic scoliosis and anthropometric data. Methods Age, biological sex, weight, height, body mass index (BMI), Cobb angle of thoracic scoliosis, left and right lung volumes, and total lung volume were recorded. CT scans were performed on all participants, and lung volumetry was analysed using specialised software. The intraclass correlation coefficient was used to assess measurement reliability, and statistical analysis was conducted to examine correlations between variables. Results Patients with OI had significantly lower total lung volumes than controls (p < 0.001). However, no significant correlation was found between lung volumetry and scoliosis (r =− 0.406; p = 0.244), age (r = 0.201; p = 0.578), height (r = 0.479; p = 0.162), weight (r = 0.358; p = 0.310), or BMI (r = − 0.042; p = 0.907) in OI patients. In the control group, significant correlations were observed between lung volume and height (r = 0.756; p = 0.011) and weight (r = 0.638; p = 0.047). Conclusion OI type 3 patients have lower lung volumes than healthy subjects, but have no left and right lung volume differences. In addition, they did not present any correlation between lung volumes and scoliosis, height, weight, and body mass index.
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spelling doaj-art-9e34683ee7ce40c0bc1bd4f33014e8692025-08-20T02:30:59ZengBMCOrphanet Journal of Rare Diseases1750-11722025-06-012011810.1186/s13023-025-03797-yLung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric dataJorge Lopez-Beneyto0Elisabetta Ponte1Vicente Martínez-Sanjuan2Maria Fernandez-Velilla3Federico Mata-Escolano4Maribel Torres-Sanchez5Francisco García-Río6Shahed Nalla7Ilker Ercan8Juan A. Sanchis-Gimeno9Facultat de Medicina i Odontologia, Universitat de ValènciaComplejo Hospitalario de ToledoASCIRES Biomedical GroupLa Paz University Hospital Biomedical Research FoundationASCIRES Biomedical GroupLa Paz University Hospital Biomedical Research FoundationLa Paz University Hospital Biomedical Research FoundationFacultat de Medicina i Odontologia, Universitat de ValènciaBursa Uludağ ÜniversitesiFacultat de Medicina i Odontologia, Universitat de ValènciaAbstract Background This study aimed to investigate the relationship between lung volumetry, thoracic scoliosis, and anthropometric data (height, weight, BMI) in patients with Osteogenesis Imperfecta (OI) Type 3. Three hypotheses were tested: H1 predicted lower lung volumes in patients with OI Type 3 compared to controls, H2 predicted differences between right and left lung volumes in patients with OI Type 3 due to chest deformities, and H3 predicted a correlation between lung volumes in patients with OI Type 3 and their thoracic scoliosis and anthropometric data. Methods Age, biological sex, weight, height, body mass index (BMI), Cobb angle of thoracic scoliosis, left and right lung volumes, and total lung volume were recorded. CT scans were performed on all participants, and lung volumetry was analysed using specialised software. The intraclass correlation coefficient was used to assess measurement reliability, and statistical analysis was conducted to examine correlations between variables. Results Patients with OI had significantly lower total lung volumes than controls (p < 0.001). However, no significant correlation was found between lung volumetry and scoliosis (r =− 0.406; p = 0.244), age (r = 0.201; p = 0.578), height (r = 0.479; p = 0.162), weight (r = 0.358; p = 0.310), or BMI (r = − 0.042; p = 0.907) in OI patients. In the control group, significant correlations were observed between lung volume and height (r = 0.756; p = 0.011) and weight (r = 0.638; p = 0.047). Conclusion OI type 3 patients have lower lung volumes than healthy subjects, but have no left and right lung volume differences. In addition, they did not present any correlation between lung volumes and scoliosis, height, weight, and body mass index.https://doi.org/10.1186/s13023-025-03797-yAnthropometric dataComputed tomographyLung volumetryOsteogenesis imperfectaScoliosis
spellingShingle Jorge Lopez-Beneyto
Elisabetta Ponte
Vicente Martínez-Sanjuan
Maria Fernandez-Velilla
Federico Mata-Escolano
Maribel Torres-Sanchez
Francisco García-Río
Shahed Nalla
Ilker Ercan
Juan A. Sanchis-Gimeno
Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
Orphanet Journal of Rare Diseases
Anthropometric data
Computed tomography
Lung volumetry
Osteogenesis imperfecta
Scoliosis
title Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
title_full Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
title_fullStr Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
title_full_unstemmed Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
title_short Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
title_sort lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
topic Anthropometric data
Computed tomography
Lung volumetry
Osteogenesis imperfecta
Scoliosis
url https://doi.org/10.1186/s13023-025-03797-y
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