Pathophysiology of respiratory failure in patients with osteogenesis imperfecta: a systematic review.

ABSTRACT:

Introduction

Respiratory failure is a major cause of death in patients with Osteogenesis Imperfecta. Moreover, respiratory symptoms seem to have a dramatic impact on their quality of life. It has long been thought that lung function disorders in OI are mainly due to changes in the thoracic wall, caused by bone deformities. However, recent studies indicate that alterations in the lung itself can also undermine respiratory health.

Objectives

Is there any intrapulmonary alteration in Osteogenesis Imperfecta that can explain decreased pulmonary function? The aim of this systematic literature review is to investigate to what extent intrapulmonary or extrapulmonary thoracic changes contribute to respiratory dysfunction in Osteogenesis Imperfecta.

Methods

A literature search

SUBMITTER: Storoni S 

PROVIDER: S-EPMC8477932 | biostudies-literature | 2021 Dec

REPOSITORIES: biostudies-literature