[Osteogenesis imperfecta: quality of life in children]
Virginia Fano1, Mariana del Pino, Mercedes Rodríguez Celin
1Servicio de Crecimiento y Desarrollo, Hospital Nacional de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. virginiafano@gmail.com
Insights
Osteogenesis imperfecta significantly impacts children's quality of life, particularly physical and social domains. Treatment compliance and pamidronate dosage correlate with better outcomes in pediatric patients.
Area of Science:
- Pediatric Health
- Genetics
- Quality of Life Research
Context:
- Osteogenesis imperfecta (OI) significantly affects children's quality of life, yet research in this area is limited.
- This study evaluated 65 children with OI in Argentina using the PedsQL 4.0 questionnaire.
- OI types I and III-IV were compared to assess differences in quality of life.
Purpose:
- To investigate the impact of osteogenesis imperfecta on children's quality of life.
- To identify factors associated with improved quality of life in pediatric OI patients.
Summary:
- A total of 65 children (38 males, median age 7.76 years) with OI were assessed.
- Significant differences in physical quality of life were found between OI types I and III-IV, reported by both children and parents.
- Parents also reported significant differences in the social domain for different OI types.
- Multivariate analysis revealed that treatment compliance was associated with better quality of life scores in children's self-reports.
- Parental reports linked better quality of life to higher pamidronate doses, less height compromise, and fewer fractures.
Impact:
- This research highlights the significant impact of osteogenesis imperfecta on pediatric quality of life.
- Findings suggest that treatment compliance, pamidronate therapy, and fracture reduction are crucial for improving the well-being of children with OI.
- The study provides valuable data for clinicians and researchers focusing on pediatric bone disorders and quality of life assessments.
Abstract:
The impact produced by osteogenesis imperfecta in childrens' quality of life almost has not been reported; 65 children, 38 males, were evaluated according to the questionnaire PedsQL 4.0 Argentinean Spanish version. Median age was 7.76 years; 35 children with OI type I were compared with 30 type III-IV (according to Sillence Classification) finding significant difference in the physical domain in both visions, children and parents, and only in parents' vision in the social area. Multivariate analysis showed an association between better PedQL scores and treatment compliance (coef. β= 19.41 p= 0.03) in children's vision. In parental report on the other hand, the association was found with greater pamidronate doses (coef. β 1.44 p=0.037), lower height compromise (coef. β= 3.8; p= 0.039) and less number of fractures (coef. β= 0.69; p= 0.003).
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