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- Juha-Jaakko Sinikumpu, Marja Ojaniemi, Petri Lehenkari, and Willy Serlo.
- Division of Paediatric Surgery and orthopaedics, Oulu University Hospital; Oulu Cranifacial Centre, Oulu; Medical Research Center Oulu; PEDEGO Research Center, Finland. Electronic address: juha-jaakko.sinikumpu@ppshp.fi.
- Injury. 2015 Aug 1; 46 (8): 1440-6.
AbstractOsteogenesis imperfecta (OI) is a group of genetic disorders, of which Type III is the most severe among survivors. The disease is characterised in particular by bone fragility, decreased bone mass and increased incidence of fractures. Other usual findings are muscle hypotonia, joint hypermobility and short stature. Fractures and weak bones may consequently cause limb and spinal deformity and chronic physical disability. Bisphosphonates have revolutionised the treatment of newborn children with severe OI type III. Surgery is still needed in most patients due to high frequency of the fractures. In this systematic review we describe the present state-of-art in treating the most severe type of OI in newborn and preschool children with their bone fractures.Copyright © 2015 Elsevier Ltd. All rights reserved.
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