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.
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Authors | Elena Monti, Monica Mottes, Paolo Fraschini, Piercarlo Brunelli, Antonella Forlino, Giacomo Venturi, Francesco Doro, Silvia Perlini, Paolo Cavarzere, Franco Antoniazzi |
Journal | Therapeutics and clinical risk management
(Ther Clin Risk Manag)
Vol. 6
Pg. 367-81
(Sep 07 2010)
ISSN: 1178-203X [Electronic] New Zealand |
PMID | 20856683
(Publication Type: Journal Article)
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