Functional status of individuals with osteogenesis imperfecta: data from a reference center

Afiliação

(1)    Programa de Pós-graduação em Medicina e Saúde, Faculdade de Medicina da Bahia, Universidade Federal da Bahia, Salvador, BA, Brasil; Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, BA, Brasil.

(2)    Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, BA, Brasil.

(3)    Universidade Federal da Bahia, Salvador, BA, Brasil.

(4)    Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, BA, Brasil; Universidade Federal do Recôncavo da Bahia, BA, Brasil.

(5)    Programa de Pós-graduação em Medicina e Saúde, Faculdade de Medicina da Bahia, Universidade Federal da Bahia, Salvador, BA, Brasil; Hospital Universitário Professor Edgard Santos, Universidade Federal da Bahia, Salvador, BA, Brasil; Departamento de Pediatria da Faculdade de Medicina da Bahia, Universidade Federal da Bahia, Salvador, BA, Brasil.

Resumo

Objective: To evaluate the functional status of individuals with Osteogenesis Imperfecta (OI) followed up at a reference center in the state of Bahia. Materials and methods: This is an observational, cross-sectional, descriptive study, which evaluated individuals with OI, based on a non-probabilistic sampling. To assess motor function, the Motor Function Measure (MFM) score was used, in addition to the measurement of muscle strength using the Medical Research Council (MRC) score. Functional performance was measured using the Pediatric Assessment of Disability Inventory, Computerized Adaptive Testing (PEDI-CAT). Results: Thirty-one individuals aged between two and 18 years old were evaluated. The overall score of MFM was 74.2%, and the lowest score was found in participants with type III OI (56.3%). The median of the MRC index was 80. The mobility domain was the most affected in the PEDI-CAT evaluation, with a mean T score of 23.9, (14.2 in type III OI). Conclusions: Among the evaluated individuals, functional alterations were identified, reduced global gross motor functionality and muscle strength, impacting the mobility domain, with the most relevant findings in individuals with type III OI.