Assessment of health-related quality of life in children with osteogenesis imperfecta (OI).

Abstract

Objective: To assess the health-related quality of life (HRQoL) in children with different types of osteogenesis imperfecta (OI). Study Design: Cross-sectional study. Setting: Department of Endocrinology, National Institute of Child Health, Karachi, Pakistan. Period: July 2023 to December 2023. Methods: Children of either gender, aged 5-18 years and already diagnosed with clinical diagnoses of OI type I, III, IV, or V were analyzed. At the time of enrollment, demographic as well as clinical information were gathered. HRQoL was evaluated using Pediatric Quality of life Inventory (PEDSQL ^TM 4.0 generic core). Results: In a total of 52 children, 27 (51.9%) were male. The mean age was 10.19±4.15 years (ranging between 5-18 years). The most common type of OI were Type-I, and Type-III, noted in 22 (42.3%), and 13 (25.0%) children respectively. It was found that total scores (p<0.001), psycho-social health (p<0.001), physical functioning (p<0.001), social functioning (p<0.001), and school functioning (p=0.013) were having distinct relationship with the types of OI. Bivariate analysis applying pearson correlation showed that there were no significant correlation that existed between age, weight, and PedsQL scores. Conclusion: Children with OI Type-III exhibited lower HRQoL across multiple domains, emphasizing the need for tailored interventions addressing the specific challenges associated with the severity of the condition.