TY - JOUR
T1 - Lung volumetry of osteogenesis imperfecta type 3 subjects is not correlated with thoracic scoliosis and anthropometric data
AU - Lopez-Beneyto, Jorge
AU - Ponte, Elisabetta
AU - Martínez-Sanjuan, Vicente
AU - Fernandez-Velilla, Maria
AU - Mata-Escolano, Federico
AU - Torres-Sanchez, Maribel
AU - García-Río, Francisco
AU - Nalla, Shahed
AU - Ercan, Ilker
AU - Sanchis-Gimeno, Juan A.
N1 - Publisher Copyright:
© The Author(s) 2025.
PY - 2025/12
Y1 - 2025/12
N2 - 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.
AB - 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.
KW - Anthropometric data
KW - Computed tomography
KW - Lung volumetry
KW - Osteogenesis imperfecta
KW - Scoliosis
UR - https://www.scopus.com/pages/publications/105007145502
U2 - 10.1186/s13023-025-03797-y
DO - 10.1186/s13023-025-03797-y
M3 - Article
AN - SCOPUS:105007145502
SN - 1750-1172
VL - 20
JO - Orphanet Journal of Rare Diseases
JF - Orphanet Journal of Rare Diseases
IS - 1
M1 - 265
ER -