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Serum tartrate-resistant acid phosphatase (TRAP) and total body bone mineral content (TBBM) were determined in a group of 16 children with osteogenesis imperfecta (OI) aged 5-14 years, 9 of whom suffered from type I and 7 from type III OI. TRAP and TBBM were also determined in a group of 26 normal
At the ultrastructural level alkaline phosphatase has been studied in calcifying cartilage but not in bone. The aim of this study was to assess if there is an osteoblast dysfunction in Osteogenesis Imperfecta (OI) with respect to alkaline phosphatase activity. Specimens from three OI type II foetal
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
A case is introduced with osteogenesis imperfecta, suffered from left femoral fracture and a consecutive hypertrophic callus formation, with extreme swelling on the thigh. Concerning the laboratory tests the only significant difference was the elevation in alkaline phosphatase activity and a
An iliac bone biopsy from an adult male, 58 years of age, with osteogenesis imperfecta type I was studied by bone histomorphometry after double-fluorescence labeling with tetracycline. Low bone mineral density (BMD) of the radius, measured by dual-energy X-ray absorptiometry (DXA) was associated
Purpose: The study was aimed at monitoring vertebral bodies changes with the use of Vertebral Fracture Assessment (VFA) in children and adolescents affected by osteogenesis imperfecta (OI) during treatment with intravenous
Markers of bone formation [C-terminal and N-terminal propeptides of procollagen I (PICP, PINP), osteocalcin and alkaline phosphatase] and bone resorption [C-terminal cross-linked telopeptide of collagen I (ICTP) and hydroxypyridinium cross-links, pyridinoline (Pyr) and deoxypyridinoline (Dpyr)] were
The metabolic defect(s) in bone cells which manifests itself in the disease osteogenesis imperfecta (OI) type 1 is not known. Since this form of the disease displays both a variable tissue involvement and the possibility of remission it is difficult to attribute its expression to a primary mutation
A prospective open study was performed to determine the efficacy and safety of pamidronate in improving bone mineralisation and reducing fracture incidence in osteogenesis imperfecta (OI). Intravenous pamidronate was administered at 1.5 mg/kg bi-monthly to six children with OI, over 12-23 months.