title: Current and emerging treatments for the management of osteogenesis imperfecta.
abstract: Osteogenesis imperfecta (OI) is the most common bone genetic disorder and it is characterized by bone brittleness and various degrees of growth disorder. Clinical severity varies widely; nowadays eight types are distinguished and two new forms have been recently described although not yet classified. The approach to such a variable and heterogeneous disease should be global and therefore multidisciplinary. For simplicity, the objectives of treatment can be reduced to three typical situations: the lethal perinatal form (type II), in which the problem is survival at birth; the severe and moderate forms (types III-IX), in which the objective is 'autonomy'; and the mild form (type I), in which the aim is to reach 'normal life'. Three types of treatment are available: non-surgical management (physical therapy, rehabilitation, bracing and splinting), surgical management (intramedullary rod positioning, spinal and basilar impression surgery) and medical-pharmacological management (drugs to increase the strength of bone and decrease the number of fractures as bisphosphonates or growth hormone, depending on the type of OI). Suggestions and guidelines for a therapeutic approach are indicated and updated with the most recent findings in OI diagnosis and treatment.
authors.affiliation: Department of Life Sciences and Reproduction, Pediatric Clinic University of Verona, Verona, Italy;
authors.forename: Elena
authors.initials: E
authors.lastname: Monti
authors.forename: Monica
authors.initials: M
authors.lastname: Mottes
authors.forename: Paolo
authors.initials: P
authors.lastname: Fraschini
authors.forename: Piercarlo
authors.initials: P
authors.lastname: Brunelli
authors.forename: Antonella
authors.initials: A
authors.lastname: Forlino
authors.forename: Giacomo
authors.initials: G
authors.lastname: Venturi
authors.forename: Francesco
authors.initials: F
authors.lastname: Doro
authors.forename: Silvia
authors.initials: S
authors.lastname: Perlini
authors.forename: Paolo
authors.initials: P
authors.lastname: Cavarzere
authors.forename: Franco
authors.initials: F
authors.lastname: Antoniazzi