rib cage deformities alter respiratory muscle action and chest wall function in patients with severe osteogenesis imperfecta肋骨畸形改变患者的呼吸肌肉动作和胸壁功能严重成骨不全症.pdfVIP

Rib Cage Deformities Alter Respiratory Muscle Action and Chest Wall Function in Patients with Severe Osteogenesis Imperfecta 1 2 2 2 1 Antonella LoMauro , Simona Pochintesta , Marianna Romei , Maria Grazia D’Angelo , Antonio Pedotti , 2 1 Anna Carla Turconi , Andrea Aliverti * 1TBMLab, Dipartimento di Bioingegneria, Politecnico di Milano, Milano, Italy, 2 IRCCS E.Medea, Bosisio Parini (Lc), Italy 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,0.01). In contrast to OI type IV patients, whose chest wall geometry and function was normal, OI type III patients were characterized by reduced (p,0.01) angle