Bifosfonates to Osteogenesis imperfect

Background: Osteogenesis imperfect is a disease of genetic origin, which causes a defect in the formation of collagen type I, with the common feature of presenting congenital bone fragility and heterogeneous clinical manifestations that can range from a small number of fractures or osteopenia, to se...

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Main Authors: Camilo Garcés-Constain, E. Beltrán-Zúñiga, Maria Amparo Acosta-Aragón
Format: Article
Language:Spanish
Published: Universidad del Cauca 2012-06-01
Series:Revista de la Facultad de Ciencias de la Salud
Subjects:
Online Access:http://revistas.unicauca.edu.co/index.php/rfcs/article/view/96
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author Camilo Garcés-Constain
E. Beltrán-Zúñiga
Maria Amparo Acosta-Aragón
author_facet Camilo Garcés-Constain
E. Beltrán-Zúñiga
Maria Amparo Acosta-Aragón
author_sort Camilo Garcés-Constain
collection DOAJ
description Background: Osteogenesis imperfect is a disease of genetic origin, which causes a defect in the formation of collagen type I, with the common feature of presenting congenital bone fragility and heterogeneous clinical manifestations that can range from a small number of fractures or osteopenia, to severe skeletal deformities with fatal results. It’s incidence is variable and there is no clear data in Colombia, equally affects all ethnicities and genders, with primary commitment to the long bones of the limbs, and sequels such as deformities, bone pain and functional limitation unwieldy for everyday activities, the personal and social development. It is classified into nine major subtypes according to the genetic, radiological and clinical characteristics, the last ones being the most important to discriminate between mild, moderate or severe and lethal disease, and determining prognosis and therapeutic interventions. Objective: Describe the current concepts of etiology, pathophysiology, emphasizing on therapeutic measures. Conclusions: It needs an interdisciplinary approach, which has been limited to conservative and surgical correction of deformities, without touching the intrinsic fragility of the disease. Bisphosphonates are effectives. It improves the quality of life and chances of surgical correction. Zoledronic acid is the most effective.
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spelling doaj.art-0dbe7e90f994437e9eceee47a12e80142022-12-22T03:11:19ZspaUniversidad del CaucaRevista de la Facultad de Ciencias de la Salud0124-308X2538-99712012-06-01142232897Bifosfonates to Osteogenesis imperfectCamilo Garcés-Constain0E. Beltrán-Zúñiga1Maria Amparo Acosta-Aragón2Universidad del CaucaHospital Universitario San José de PopayánUniversidad del Cauca Departamento de PediatríaBackground: Osteogenesis imperfect is a disease of genetic origin, which causes a defect in the formation of collagen type I, with the common feature of presenting congenital bone fragility and heterogeneous clinical manifestations that can range from a small number of fractures or osteopenia, to severe skeletal deformities with fatal results. It’s incidence is variable and there is no clear data in Colombia, equally affects all ethnicities and genders, with primary commitment to the long bones of the limbs, and sequels such as deformities, bone pain and functional limitation unwieldy for everyday activities, the personal and social development. It is classified into nine major subtypes according to the genetic, radiological and clinical characteristics, the last ones being the most important to discriminate between mild, moderate or severe and lethal disease, and determining prognosis and therapeutic interventions. Objective: Describe the current concepts of etiology, pathophysiology, emphasizing on therapeutic measures. Conclusions: It needs an interdisciplinary approach, which has been limited to conservative and surgical correction of deformities, without touching the intrinsic fragility of the disease. Bisphosphonates are effectives. It improves the quality of life and chances of surgical correction. Zoledronic acid is the most effective.http://revistas.unicauca.edu.co/index.php/rfcs/article/view/96Osteogénesis imperfecta, bifosfonatos, fracturas, niños
spellingShingle Camilo Garcés-Constain
E. Beltrán-Zúñiga
Maria Amparo Acosta-Aragón
Bifosfonates to Osteogenesis imperfect
Revista de la Facultad de Ciencias de la Salud
Osteogénesis imperfecta, bifosfonatos, fracturas, niños
title Bifosfonates to Osteogenesis imperfect
title_full Bifosfonates to Osteogenesis imperfect
title_fullStr Bifosfonates to Osteogenesis imperfect
title_full_unstemmed Bifosfonates to Osteogenesis imperfect
title_short Bifosfonates to Osteogenesis imperfect
title_sort bifosfonates to osteogenesis imperfect
topic Osteogénesis imperfecta, bifosfonatos, fracturas, niños
url http://revistas.unicauca.edu.co/index.php/rfcs/article/view/96
work_keys_str_mv AT camilogarcesconstain bifosfonatestoosteogenesisimperfect
AT ebeltranzuniga bifosfonatestoosteogenesisimperfect
AT mariaamparoacostaaragon bifosfonatestoosteogenesisimperfect