Bone Density

Objective: Osteogenesis imperfecta (01) is a heritable type 1 collagen disorder characterized by weakness, fractures, bony malalignment, and short stature. Functional capability ranges from the ability to run, to the need for powered locomotion. As a group, children with 0 1 appear to be intelligent, and socially very skillful. However, it is unclear whether this is related to advanced social skills, or small stature makes these children appear more capable than they actually are. We asked how children with 0 1 perceive themselves, compared with nondisabled peers, in several domains of competence. Design: Prospective study to compare self-perceived competence of children with 0 1 with normative data for nondisabled age peers. Setting: Federal research hospital Participants: Culturally diverse cohort of 55 children (30 females, 25 males) with types I11 (26) and lV(29) osteogenesis imperfecta, aged 4-18 years, who complete annual assessments as part of an osteogenesis imperfecta research program for children from birth to 18 years. Measurements and main results: Self-perceived competence was assessed with three standardized, age-appropriate, selfreport instruments: Harter Pictorial Scale of Perceived Competence and Social Acceptance forYoung Children (age 4 grade 2); Harter Self-Perception Profile for Children (grade 3 -age 12); and Harter Self-Perception Profile for Adolescents (age 13-18 years). Gross motor skill level was documented with the Brief Assessment of Motor Function (Cintas et al 2003). Independent t-tests compared children with 0 1 and normative values. Spearman correlations and multiple regression identified significant relationships and predictors of total competence. Young children ( 4 7 years), and adolescents (13-18 years) consistently reported less athletic competene @= 0.02-0.001) than nondisabled peers. Middle children and adolescents reported themselves as being more competent than nondisabled children in cognitive competence @= O.Os-O.OOl), conduct (p= 0.05-0.001), and global self-worth (p=0.02-0.001). Significant relationships were identified between motor performance and total competence (r=O.44, p < 0.OOOl). Mated attachment p d c t e d total competence for young children (1"= 0.68,p< 0.001). Global self-worth predicted total competence for middle children (?= 0.73,p< O.OOl), and adolescents (?= 0.79,p< 0.001). Conclusion: Younger children and adolescents with 01 clearly perceive their motor skill limitations, but middle children remain undeclared. Reported higher cognitive competence, conduct, and self-worth suggest that maturing children with 0 1 view themselves more positively in these domains than nondisabled peers. Although significant relationships exist between motor skill and total competence, maternal attachment and self-worth are the most important predictors of total competence.