Current and Emerging Treatments for the Management of Osteogenesis Imperfecta

Overview

Journal Ther Clin Risk Manag

Publisher Dove Medical Press

Date 2010 Sep 22

PMID 20856683

Citations 49

Authors

Elena Monti

Monica Mottes

Paolo Fraschini

Piercarlo Brunelli

Antonella Forlino

Giacomo Venturi

Francesco Doro

Silvia Perlini

Paolo Cavarzere

Franco Antoniazzi

Affiliations

Soon will be listed here.

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.

Citing Articles

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