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. 2025 Jun 2;20(1):265.
doi: 10.1186/s13023-025-03797-y.

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

Jorge Lopez-Beneyto  1 , Elisabetta Ponte  2 , Vicente Martínez-Sanjuan  3 , Maria Fernandez-Velilla  4 , Federico Mata-Escolano  3 , Maribel Torres-Sanchez  4 , Francisco García-Río  4 , Shahed Nalla  1   5 , Ilker Ercan  6 , Juan A Sanchis-Gimeno  7
Affiliations

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

Jorge Lopez-Beneyto et al. Orphanet J Rare Dis. .

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.

Keywords: Anthropometric data; Computed tomography; Lung volumetry; Osteogenesis imperfecta; Scoliosis.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: The study was conducted according to the guidelines of the Declaration of Helsinki and approved by the Institutional Review Board (or Ethics Committee) of the University of Valencia (protocol code H1417174744011). Consent for publication: Written informed consent was obtained from all subjects involved in the study. Written informed consent was obtained from the patients to publish this paper. Competing interests: The authors declare no conflict of interest. The funders had no role in the study's design, in the collection, analyses, or interpretation of data, in the writing of the manuscript, or in the decision to publish the results.

Figures

Fig. 1
Fig. 1
3D volumetric computed tomography reconstruction showing the lung parenchyma, trachea and pulmonary vascularization. A: Osteogenesis Imperfecta. B: Control group
Fig. 2
Fig. 2
Axial computed tomography slice (inferior view) showing a rotated vertebral column protrusion into the right thoracic cavity in an Osteogenesis Imperfecta patient
See this image and copyright information in PMC

References

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