title: Aspergillus infections in cystic fibrosis.
abstract: Patients with cystic fibrosis (CF) suffer from chronic lung infection and airway inflammation. Respiratory failure secondary to chronic or recurrent infection remains the commonest cause of death and accounts for over 90% of mortality. Bacteria as Staphylococcus aureus, Pseudomonas aeruginosa and Burkholderia cepacia complex have been regarded the main CF pathogens and their role in progressive lung decline has been studied extensively. Little attention has been paid to the role of Aspergillus spp. and other filamentous fungi in the pathogenesis of non-ABPA (allergic bronchopulmonary aspergillosis) respiratory disease in CF, despite their frequent recovery in respiratory samples. It has become more apparent however, that Aspergillus spp. may play an important role in chronic lung disease in CF. Research delineating the underlying mechanisms of Aspergillus persistence and infection in the CF lung and its link to lung deterioration is lacking. This review summarizes the Aspergillus disease phenotypes observed in CF, discusses the role of CFTR (cystic fibrosis transmembrane conductance regulator)-protein in innate immune responses and new treatment modalities.
authors.affiliation: Institute of Medical Sciences, Aberdeen Fungal Group, University of Aberdeen, UK.
authors.forename: Jill
authors.initials: J
authors.lastname: King
authors.affiliation: Institute of Medical Sciences, Aberdeen Fungal Group, University of Aberdeen, UK.
authors.forename: Shan F
authors.initials: SF
authors.lastname: Brunel
authors.affiliation: Institute of Medical Sciences, Aberdeen Fungal Group, University of Aberdeen, UK. Electronic address: a.warris@abdn.ac.uk.
authors.forename: Adilia
authors.initials: A
authors.lastname: Warris
mesh_terms: D001229:Aspergillosis, Allergic Bronchopulmonary / Q000209:etiology / Q000276:immunology / Q000382:microbiology* / Q000628:therapy; D001232:Aspergillus fumigatus / Q000302:isolation & purification / Q000472:pathogenicity; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000150:complications* / Q000276:immunology / Q000382:microbiology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism; D006801:Humans; D007113:Immunity, Innate; D008168:Lung / Q000382:microbiology / Q000503:physiopathology; D010641:Phenotype; D055732:Pulmonary Aspergillosis / Q000209:etiology / Q000276:immunology / Q000382:microbiology* / Q000628:therapy
chemical_list: C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Allergic bronchopulmonary aspergillosis; Aspergillosis; Aspergillus; Aspergillus fumigatus; CFTR; Cystic fibrosis