Abstract

RODRIGUEZ CELIN, Mercedes  and  FANO, Virginia. Osteogenesis imperfecta. Arch. argent. pediatr. [online]. 2016, vol.114, n.3, pp.248-251. ISSN 0325-0075.  http://dx.doi.org/10.5546/aap.2016.248.

Introduction. Osteogenesis imperfecta is a group of hereditary connective tissue disorders that cause bone fragility, with a wide clinical variability resulting in varying degrees of motor disability. Objectives. To describe the level of independence and of social, recreational and sports participation among adolescents with osteogenesis imperfecta. Population and methods. Descriptive, analytical and crosssectional study conducted in patients with osteogenesis imperfecta older than 15 years old attending the Skeletal Dysplasia Office of Hospital "Prof. Dr. Juan P. Garrahan" (May 2013 through December 2014). Self-administered survey. Short stature was an outcome measure that indicated severity. Results. There were 18 patients; age: 19.17 (±3.4 sDE); 83% had moderate-severe forms of OI; median height: -7.9 sDE; 50% used a wheelchair. Average education years: 12.2; 56% participated in sporting activities; and 78% were involved in recreational and social activities. A high level of independence was observed. We found a correlation between short stature and use of wheelchair (r: -0.77) and between short stature and participation in sporting activities (r: 0.66). No correlation was observed with years of education (r: -0.15), participation in social activities (r: -0.22) or recreational activities (r: 0.35).

Keywords : Osteogenesis imperfecta; Adolescent; Social participation; Recreational activities.

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