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Benefits of Pamidronate Treatment in Osteogenesis Imperfecta

Source: www.fmshk.com.hk
Topic: Osteogenesis imperfecta
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Sort Desciption: Previous studies demonstrated that cyclical pamidronate therapy is effective in increasing bone density and improving clinical outcomes in children with osteogenesis imperfecta. ...

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Osteogenesis imperfecta is a hereditary disorder of connective tissues characterised by low bone mass and bone fragility. Previous studies demonstrated that cyclical pamidronate therapy is effective in increasing bone density and improving clinical outcomes in children with osteogenesis imperfecta. We report our experience in treating two children with cyclical intravenous pamidronate infusion. The benefits and problems of the treatment are discussed. Key words Osteogenesis imperfecta; Paediatric; Pamidronate treatment Introduction Osteogenesis imperfecta (OI) is a genetic disorder of synthesis of collagen. This results in low bone mass, fragile bones and recurrent fractures. The phenotype is heterogeneous and is currently classified into seven types. The classical types I-IV of Sillence classification 1 are associated with mutation of type 1 collagen gene while the genetic basis of the newly identified types V, VI and VII remains to be determined. 2,3 Children with more severe forms of OI have multiple fractures and progressive deformities thus affecting their quality of life. The impairment in bone formation in OI shifted the balance towards bone resorption. The patients thus have thin bones with few trabeculae, thin cortices and high remodeling activity. 4 Bisphosphonates adversely affect the function of osteoclast and also cause osteoclast apoptosis. 5 Pamidronate belongs to the second generation bisphosphonate and is a potent inhibitor of bone resorption. Prior studies showed that cyclical intravenous ...

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