[Children with osteogenesis imperfecta. An infrequent but important disease]

Imperfect osteogenesis is a disease which is included in the group of the osseous dysplasias having a heterogeneous genetic character and whose basic defect is an alteration in the synthesis of Procollagen I. This leads to a serious fragility in skeletal structures as well as in exoskeletal structures, causing multiple fractures and deformities. The absence of a truly effective medical, surgical or orthopedic treatment makes correctly planned nursing care acquire vital importance in order to succeed in avoiding, and diminishing, fractures and deformities due to an inadequate handling of these patients; while to the contrary contributing to success in integrating these patients into society in the best possible conditions. This is the first of two articles which the authors will dedicate to this disease; this disease will be described in this first article, while the second one will concentrate exclusively on nursing treatments recommended for patients suffering from this disease.