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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.

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BACKGROUND/AIMS: Osteogenesis Imperfecta (OI) is a bone disease characterized by bone fragility, deformities, and multiple fractures. The aim of this study was to compare the different methods of measuring the basal metabolic rate (BMR) and body composition (BC) in pediatric patients with OI. METHODS: This cross-sectional study included 52 individuals with a median age of 9 (5.25-12.7) years. BMR was calculated by bioelectrical impedance analyses (BIA), predictive values according to age from the World Health Organization (WHO), a kcal/cm formula, and indirect calorimetry (IC). BC was assessed using the anthropometric calculation of percentage body fat (%BF) and lean mass (kg), BIA, and dual-energy X-ray absorptiometry (DEXA). Agreement among the methods was assessed using the Bland-Altman technique. RESULTS: IC estimates of BMR were greater than BIA and lower than values obtained using the WHO and kcal/cm methods. Better agreement was observed using the WHO values for mild forms of OI and the kcal/cm formula for moderate-to-severe forms. For BC, DEXA estimates of %BF were higher and the lean mass was lower than the values obtained using BIA and anthropometry. Neither method agreed with the DEXA method results. CONCLUSIONS: Significant differences exist among the various methods used for measuring BMR and BC with regard to phenotypic differences between OI types.

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OBJECTIVE: Vitamin D is essential to the development and maintenance of the skeleton, especially for children with bone disorders such as osteogenesis imperfecta (OI). We evaluated serum 25-hydroxyvitamin D (25-OHD) levels to assess the relationship between determinants of vitamin D status in pediatric patients with OI. METHODS: This cross-sectional study evaluated sex, age, weight, height, body mass index, OI type, sunscreen use, season of assessment, sun exposure, vitamin D and calcium supplementation, bisphosphonate treatment, bone mineral density (BMD), milk and soda consumption, mobility, and time of sedentary activity. Levels of serum 25-OHD, calcium, parathyroid hormone (PTH), phosphorus, and alkaline phosphatase (ALP) were analyzed. Serum levels of 25-OHD were classified according to sufficient (>30 ng/ml or 75 nmol/L), insufficient (20-30 ng/ml or 50-75 nmol/L), moderately deficient (20-10 ng/ml or 50-25 nmol/L), and severely deficient (<10 ng/ml or 25 nmol/L). RESULTS: Fifty-two patients were included and 46 (88.4%) were classified as having insufficient or deficient 25-OHD. An inverse correlation between serum 25-OHD and time of sedentary activity (r = -0.597, p < 0.001) and a positive correlation with height (r = 0.521, p = 0.046) and whole body BMD (r = 0.586, p = 0.022) were observed. A significant difference between the number of glasses of milk consumed (p = 0.010) was observed. CONCLUSION: To optimize bone health, patients with OI need to be educated regarding habits that can improve serum 25-OHD levels, such as a reduction in periods of inactivity, the importance of sun exposure, and increasing consumption of milk and fortified dairy products.

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OBJECTIVE: The aim of the present study was to assess anthropometric measurements, nutritional status, dietary intake, and body fat percentage of pediatric patients with osteogenesis imperfecta (OI). METHOD: A cross-sectional study evaluated 63 OI patients from 0 to 19 years of age. We analyzed anthropometric measurements, mobility, bisphosphonate treatment, body fat percentage (by dual-energy x-ray absorptiometry [DEXA] and sum of skinfold thickness), nutritional status, and dietary intake (using World Health Organization [WHO] and dietary reference intake recommendations for macronutrients and calcium intake, respectively). Participants' energy requirements were calculated using both kilocalorie per centimeter measurements and WHO methods. RESULTS: Patients with different types of OI had different anthropometric measurements (p < 0.05), where OI type III had severely limited stature and poor mobility. Nutritional status was correlated with measurements of arm circumference and body fat. We also found a strong correlation between the 2 methods used to calculate percentage of body fat (r = 0.803). OI type III had a higher percentage of energy intake. We observed that 75% of subjects had a calcium intake below 95% of recommended daily value and there was an inverse correlation between age and calcium intake. CONCLUSIONS: This study showed that stature was compromised mainly in OI type III. Skinfold thickness and arm circumference correlated to nutritional status and also to body fat calculated by DEXA. Daily calcium intake was below the recommended levels in pediatric patients with OI. These findings are important for the management of OI subjects.

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