title: [Early use of BiPAP in the management of respiratory failure in an infant with osteogenesis imperfecta: case report].
abstract: Osteogenesis imperfecta (OI) is an heterogeneous group of genetic disorders that affect connective tissue integrity. Severe forms cause chest deformities, sometimes associated to alveolar hypoventilation. We report a 4 months old infant with OI type III, who developed respiratory failure (RF) due to a bronchiolitis and required mechanical ventilation. Weaning progressed successfully to a nasal bi-level Positive Airway Pressure (n-BiPAP) device. Clinical follow up showed a normal cognitive development and growth. Respiratory condition, blood gases and ventilation status were in normal ranges. Non invasive ventilation, associated to careful monitoring may avoid tracheostomy and its complications in infants with OI.
authors.affiliation: Sección de Respiratorio Pediátrico, Departamento de Pediatría, Pontificia Universidad Católica de Chile. LEvega@puc.cl
authors.forename: Luis
authors.initials: L
authors.lastname: Vega-Briceño
authors.forename: Ilse
authors.initials: I
authors.lastname: Contreras
authors.forename: Ignacio
authors.initials: I
authors.lastname: Sánchez
authors.forename: Pablo
authors.initials: P
authors.lastname: Bertrand
mesh_terms: D001988:Bronchiolitis / Q000628:therapy; D045422:Continuous Positive Airway Pressure; D006801:Humans; D007223:Infant; D008297:Male; D008397:Masks; D010013:Osteogenesis Imperfecta / Q000628:therapy*; D012131:Respiratory Insufficiency / Q000628:therapy*
vernacular_title: Uso precoz del BiPAP en el tratamiento de la insuficiencia respiratoria en un lactante con osteogénesis imperfecta. Caso clínico.