Scoliosis and Cardiopulmonary Outcomes in Osteogenesis Imperfecta Patients.
Adolescent
Adult
Child
Female
Fractures, Compression
/ complications
Humans
Lung
/ physiopathology
Lung Diseases
/ etiology
Male
Osteogenesis Imperfecta
/ complications
Quality of Life
Radiography
Respiratory Function Tests
Respiratory Insufficiency
/ etiology
Retrospective Studies
Scoliosis
/ complications
Spinal Fractures
/ complications
Thoracic Wall
/ physiopathology
Vital Capacity
Young Adult
Journal
Spine
ISSN: 1528-1159
Titre abrégé: Spine (Phila Pa 1976)
Pays: United States
ID NLM: 7610646
Informations de publication
Date de publication:
01 Aug 2019
01 Aug 2019
Historique:
entrez:
24
7
2019
pubmed:
25
7
2019
medline:
18
12
2019
Statut:
ppublish
Résumé
Retrospective clinical study of individuals with osteogenesis imperfecta (OI). To assess the relationship between severity of scoliosis and pulmonary function, and to assess the relationship between restrictive lung disease and self-reported quality of life in individuals with OI. OI is a heritable connective tissue disorder characterized by osteopenia and a predisposition to fracture. Respiratory insufficiency is a leading cause of mortality. Literature on pulmonary function in this population has shown a negative correlation between percent-predicted vital capacity and severity of scoliosis. However, it has been suggested that decreased pulmonary function in OI may be due to intrinsic pulmonary disease, in addition to the impact of vertebral compression fractures and scoliosis. Anterior-posterior spine radiographs and pulmonary function tests from 30 individuals with OI were reviewed. Radiographs were evaluated for scoliosis, defined as a curve ≥ 10°. If more than one curve was present, the largest curve was used. Pulmonary function was defined as the forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio. Restrictive pulmonary disease was defined as FEV1/FVC > 80%, while obstructive disease was defined as FEV1/FVC < 70%. Bivariate correlation analysis was performed, using Spearman rho correlation coefficient (P < 0.05). Quality of life was assessed by SF-36. The mean age was 27.6 years (range: 12-42 yrs). 57.6% were female. OI type IV was the most common (46.7%), followed by OI type III (33.3%), OI type I (10%), OI type IX (6.67% each), and OI type VIII (3.33%). Pulmonary comorbidity was present in 40% of individuals, while 6.67% had a cardiac comorbidity. The correlation between scoliosis and pulmonary function was weak and not significant (R = -0.059, P = 0.747). Pulmonary function is not significantly correlated with scoliosis, supporting the hypothesis that decreased pulmonary function is intrinsic to OI and/or chest wall deformities, rather than secondary to scoliosis. 4.
Sections du résumé
STUDY DESIGN
METHODS
Retrospective clinical study of individuals with osteogenesis imperfecta (OI).
OBJECTIVE
OBJECTIVE
To assess the relationship between severity of scoliosis and pulmonary function, and to assess the relationship between restrictive lung disease and self-reported quality of life in individuals with OI.
SUMMARY OF BACKGROUND DATA
BACKGROUND
OI is a heritable connective tissue disorder characterized by osteopenia and a predisposition to fracture. Respiratory insufficiency is a leading cause of mortality. Literature on pulmonary function in this population has shown a negative correlation between percent-predicted vital capacity and severity of scoliosis. However, it has been suggested that decreased pulmonary function in OI may be due to intrinsic pulmonary disease, in addition to the impact of vertebral compression fractures and scoliosis.
METHODS
METHODS
Anterior-posterior spine radiographs and pulmonary function tests from 30 individuals with OI were reviewed. Radiographs were evaluated for scoliosis, defined as a curve ≥ 10°. If more than one curve was present, the largest curve was used. Pulmonary function was defined as the forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio. Restrictive pulmonary disease was defined as FEV1/FVC > 80%, while obstructive disease was defined as FEV1/FVC < 70%. Bivariate correlation analysis was performed, using Spearman rho correlation coefficient (P < 0.05). Quality of life was assessed by SF-36.
RESULTS
RESULTS
The mean age was 27.6 years (range: 12-42 yrs). 57.6% were female. OI type IV was the most common (46.7%), followed by OI type III (33.3%), OI type I (10%), OI type IX (6.67% each), and OI type VIII (3.33%). Pulmonary comorbidity was present in 40% of individuals, while 6.67% had a cardiac comorbidity. The correlation between scoliosis and pulmonary function was weak and not significant (R = -0.059, P = 0.747).
CONCLUSION
CONCLUSIONS
Pulmonary function is not significantly correlated with scoliosis, supporting the hypothesis that decreased pulmonary function is intrinsic to OI and/or chest wall deformities, rather than secondary to scoliosis.
LEVEL OF EVIDENCE
METHODS
4.
Identifiants
pubmed: 31335789
doi: 10.1097/BRS.0000000000003012
pii: 00007632-201908010-00009
doi:
Substances chimiques
Osteogenesis Imperfecta, Type IV
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
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