title: Fungi in cystic fibrosis and non-cystic fibrosis bronchiectasis.
abstract: Bronchiectasis is a pathologic bronchial dilatation with loss of function that can result from multiple inflammatory and infectious injuries to the conducting airways of the lung. Molds, particularly the filamentous fungus Aspergillus fumigatus, have been implicated as a common cause of both cystic fibrosis (CF) and non-CF bronchiectasis, the latter primarily in patients with severe asthma. The pathogenesis of mold-associated bronchiectasis is usually due to atopic sensitization to mold allergens in the presence of active chronic endobronchial fungal infection with host innate and adaptive immune deviation to a Th2-dominated inflammation, a condition known as allergic bronchopulmonary aspergillosis (ABPA) (or allergic bronchopulmonary mycosis if a non-Aspergillus mold is implicated). Diagnostic criteria of ABPA continue to evolve, while treatment relies upon downregulation of the allergic inflammatory response with immunomodulatory agents and antifungal pharmacotherapy.
authors.affiliation: Department of Pediatrics, Lucile Salter Packard Children's Hospital, Stanford University School of Medicine, Palo Alto, California.
authors.forename: Richard B
authors.initials: RB
authors.lastname: Moss
mesh_terms: D000666:Amphotericin B / Q000627:therapeutic use; D000935:Antifungal Agents / Q000627:therapeutic use; D001228:Aspergillosis / Q000188:drug therapy*; D001229:Aspergillosis, Allergic Bronchopulmonary / Q000175:diagnosis* / Q000188:drug therapy; D001232:Aspergillus fumigatus / Q000472:pathogenicity*; D001987:Bronchiectasis / Q000175:diagnosis / Q000382:microbiology* / Q000628:therapy; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000382:microbiology* / Q000628:therapy; D006801:Humans; D006967:Hypersensitivity; D007073:Immunoglobulin E / Q000097:blood; D014230:Triazoles / Q000627:therapeutic use
chemical_list: D000935:Antifungal Agents; D014230:Triazoles; D007073:Immunoglobulin E; D000666:Amphotericin B