title: Cystic fibrosis.
abstract: Cystic fibrosis is the most common lethal inherited disorder with autosomal recessive inheritance. Major progress has been made in understanding the molecular mechanisms leading to increased susceptibility to Pseudomonas aeruginosa colonization. Persistent respiratory infection with P. aeruginosa leads to progressive pulmonary inflammation and is the major cause of morbidity and mortality. Treatment and prophylaxis of respiratory infection has improved the median survival and quality of life of cystic fibrosis patients. In the future, treatment of the underlying genetic defect may be possible.
authors.affiliation: Royal Brompton Hospital, Sydney Street, London, UK.
authors.forename: Amanda L
authors.initials: AL
authors.lastname: Brennan
authors.forename: Duncan M
authors.initials: DM
authors.lastname: Geddes
mesh_terms: D000900:Anti-Bacterial Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage / Q000627:therapeutic use; D003550:Cystic Fibrosis / Q000235:genetics / Q000382:microbiology / Q000503:physiopathology / Q000628:therapy; D015916:Drainage, Postural; D016905:Gram-Negative Bacterial Infections / Q000150:complications / Q000188:drug therapy; D006801:Humans; D009154:Mutation / Q000235:genetics; D013203:Staphylococcal Infections / Q000150:complications / Q000188:drug therapy
chemical_list: D000900:Anti-Bacterial Agents; D000893:Anti-Inflammatory Agents