Osteogenesis imperfecta: lifelong management is imperative and feasible

Osteogenesis imperfecta is a group of inherited diseases responsible for va rying degrees of skeletal fragility. Minimal trauma is sufficient to cause fractures and bone deformities. The classification of osteogenesis imperfec ta has recently been improved by the inclusion of additional clinical and h istomorphometric data. The diagnosis is often readily made in infancy; some cases, however, go unrecognized until adulthood. Lifelong multidisciplinar y management is imperative. Pamidronate therapy in childhood is the most ex tensively studied treatment and has been proved beneficial. Other bisphosph onates are being evaluated, particularly in adults. Prevention of vitamin D and calcium deficiency is essential throughout life. Pain is common and sh ould be given adequate attention. Joint Bone Spine 2001 ; 68 : 125-9. (C) 2 001 Editions scientifiques et medicales Elsevier SAS.