title: Respiratory System Disease.
abstract: Respiratory system involvement in cystic fibrosis is the leading cause of morbidity and mortality. Defects in the cystic fibrosis transmembrane regulator (CFTR) gene throughout the sinopulmonary tract result in recurrent infections with a variety of organisms including Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus, and nontuberculous mycobacteria. Lung disease occurs earlier in life than once thought and ideal methods of monitoring lung function, decline, or improvement with therapy are debated. Treatment of sinopulmonary disease may include physiotherapy, mucus-modifying and antiinflammatory agents, antimicrobials, and surgery. In the new era of personalized medicine, CFTR correctors and potentiators may change the course of disease.
authors.affiliation: Pediatric Pulmonology, Jacobs School of Medicine, Women & Children's Hospital of Buffalo, State University of New York, 219 Bryant Street, Buffalo, NY 14222, USA. Electronic address: dgoetz@upa.chob.edu.
authors.forename: Danielle M
authors.initials: DM
authors.lastname: Goetz
authors.affiliation: Pediatric Pulmonology, Jacobs School of Medicine, Women & Children's Hospital of Buffalo, State University of New York, 219 Bryant Street, Buffalo, NY 14222, USA.
authors.forename: Shipra
authors.initials: S
authors.lastname: Singh
mesh_terms: D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D006801:Humans; D007223:Infant; D012140:Respiratory Tract Diseases / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy
chemical_list: C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Bronchiectasis; CFTR; Cystic fibrosis; Lung disease; Lung function; Pulmonary exacerbation