Abstract
Background: Osteogenesis imperfecta（OI）is a chronic, non-infectious disease that accompanies the patient for life. OI will result in reduced bone mass, fractures, thereby leading to bone deformities and reduced patient the health-related quality of life (HRQoL). With the endocrine therapy and surgical orthopedic had been recommended, the disease was effectively controlled. The disease status is discrepancy in different regions. This study aims to assess the diagnosis and treatment status, evaluate the HRQoL of children and adolescents with OI in southwest China, and analyze the influencing factors, so as to provide reference for health care consideration for OI population in southwest China.Methods: This cross-sectional study was performed using the Pediatric Quality of Life InventoryTM (PedsQL TM) to assess the HRQoL of children and adolescents with OI. The demographic and clinical data were collected. This study was conducted in Children’s Hospital of Chongqing Medical University, the National Children's Regional Medical Centre (SOUTH-WEST) and the National Clinical Research for child health. Healthy children and adolescents aged 2 to 18 years lived in southwest China as a reference.Results: A total of 27 children and adolescents with OI were included in this study. The current status of diagnosis and treatment such as the treatment, education, family, BMI, pain, steep walking and friends and so on had been investigated. The PedsQLTM of OI population was lower in all dimensions, and they were significantly different in physiological and social functioning and the total score, compared with 54 healthy children and adolescents. Factors associated with HRQoL of OI patients were the steep walking and fixing a playmate at less.Conclusions: The HRQoL of children and adolescents with OI had been improved with the endocrine therapy and surgical orthopedic. But there was an imbalance around the world for the less effective management. Health care education should be more professional, effectively and continuously. Training patients to gain steep walking ability and peer companionship could be a way to improve the HRQoL of children and adolescents with OI in southwest China. The medical staff also should pay attention to their life and growth after discharge.
Health-related quality of life in healthy children and adolescents of HIV-infected parents in Alexandria, Egypt
