Abstract Treatment of moderate and severe forms of osteogenesis imperfecta (OI) with cyclic pamidronate at the Reference Center for OI Treatment in Southern Brazil was studied. A retrospective cohort study was conducted from 2002 to 2012. Data were obtained during inpatient (drug infusion) and outpatient care. Clinical data, including the presence of blue sclerae, dentinogenesis imperfecta, history and site of the fractures, biochemical data, including calcium, phosphorus, and alkaline phosphatase levels, were systematically collected. Bone mineral density (BMD) was measured using dual energy X-ray absorptiometry (DXA). Forty-five patients (26 females) were included in the study, and the age of the patients at the time of diagnosis ranged from 1 to 144 months, with a median age (p25-p75) of 38 (5-96) months. Most cases presented OI-4 (51.1%), and the median age of the patients at the start of treatment was 3.3 years (25-75 percentiles: 0.5 - 8.7 years). Twenty-four patients (54.5%) had some adverse events or intercurrences during treatment, and the treatment compliance mean was 92.3% (± 10.7). The treatment with intravenous pamidronate has shown to be safe, well-tolerated, and effective in regard to the improvement of BMD and the reduction of the number of fractures in children and adolescents with OI.

BACKGROUND: The assessment of strength and its relationships with functional capacity could contribute to more specific and effective disability management of stroke survivors. OBJECTIVE: To compare and investigate associations between measures of strength and functional capacity of 98 chronic stroke survivors, stratified into three groups, according to their physical activity levels. METHOD: The physical activity levels were classified as impaired, moderately active, and active, based on their Human Activity Profile (HAP) scores. Strength was assessed by the maximal inspiratory (MIP) and expiratory (MEP) pressures and by the residual deficits (RDs) of work of the lower limb and trunk muscles, whereas functional capacity was evaluated by the distance covered during the six-minute walking test (6MWT). RESULTS: One-way analyses of variance revealed significant differences between the groups, except between the active and moderately active groups regarding the RDS of the hip and knee flexors/extensors and ankle dorsiflexors (2.91<F<8.62; 0.001<p<0.01). Differences between the groups were found for the 6MWT (F=10.75; p<0.001), but no differences were found for the MIP and MEP measures (0.92<F<2.13; 0.13<p<0.40). Significant, negative, and fair correlations were observed between the RDS of the hip and knee muscles and the 6MWT (0.30<r<-0.43; p<0.01) and the HAP (-0.28<r<-0.41; p<0.01). Moderate to good correlations were found between the 6MWT and the HAP (r=0.50; p<0.0001). There were no significant correlations between measures of respiratory strength and any of the investigated variables (-0.11<r<0.12; 0.26<p<0.56). CONCLUSIONS: Lower strength deficits and higher functional capacity were associated with higher physical activity levels. However, the moderately active and active groups demonstrated similar strength deficits.