Rib cage deformities alter respiratory muscle action and chest wall function in patients with severe Osteogenesis imperfecta

Antonella LoMauro, Simona Pochintesta, Marianna Romei, Maria Grazia D'Angelo, Antonio Pedotti, Anna Carla Turconi, Andrea Aliverti

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Osteogenesis imperfecta (OI) is an inherited connective tissue disorder characterized by bone fragility, multiple fractures and significant chest wall deformities. Cardiopulmonary insufficiency is the leading cause of death in these patients. Methods: Seven patients with severe OI type III, 15 with moderate OI type IV and 26 healthy subjects were studied. In addition to standard spirometry, rib cage geometry, breathing pattern and regional chest wall volume changes at rest in seated and supine position were assessed by opto-electronic plethysmography to investigate if structural modifications of the rib cage in OI have consequences on ventilatory pattern. One-way or two-way analysis of variance was performed to compare the results between the three groups and the two postures. Results: Both OI type III and IV patients showed reduced FVC and FEV1 compared to predicted values, on condition that updated reference equations are considered. In both positions, ventilation was lower in OI patients than control because of lower tidal volume (p

Original languageEnglish
Article numbere35965
JournalPLoS One
Volume7
Issue number4
DOIs
Publication statusPublished - Apr 27 2012

ASJC Scopus subject areas

  • Agricultural and Biological Sciences(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • Medicine(all)

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