title: Use of intrapulmonary percussive ventilation (IPV) in the management of pulmonary complications of an infant with osteogenesis imperfecta.
abstract: Osteogenesis imperfecta (OI) is a genetic disorder characterized by abnormal collagen formation and short stature. These patients present with frequent vertebral, rib, and long bone fractures. There are many respiratory complications associated with OI including pneumonia, the most common cause of mortality in the severe forms of the disease. We present a case of an infant with OI (type III/IV) and significant tracheobronchomalacia who had required multiple hospitalizations for recurrent atelectasis and respiratory failure in the setting of acute respiratory infections. External chest percussion and vibration were avoided because of the risk of rib fractures. intrapulmonary percussive ventilation (IPV) was initiated during an acute illness with good effect, and continued successfully after discharge from hospital. We conclude that IPV represents a safe and effective alternative to airway clearance in infants with OI.
authors.affiliation: Division of Pulmonary Medicine, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.
authors.forename: Gustavo
authors.initials: G
authors.lastname: Nino
authors.forename: Paul
authors.initials: P
authors.lastname: McNally
authors.forename: Laura J
authors.initials: LJ
authors.lastname: Miske
authors.forename: Eileen
authors.initials: E
authors.lastname: Hickey
authors.forename: Howard B
authors.initials: HB
authors.lastname: Panitch
mesh_terms: D006801:Humans; D007223:Infant; D008297:Male; D010013:Osteogenesis Imperfecta / Q000150:complications*; D011175:Positive-Pressure Respiration / Q000379:methods*; D001261:Pulmonary Atelectasis / Q000209:etiology* / Q000628:therapy*