title: Diagnosis of Adult Patients with Cystic Fibrosis.
abstract: The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis.
authors: Department of Medicine, National Jewish Health, 1400 Jackson, Denver, CO 80206, USA; Department of Medicine, University of Colorado Denver School of Medicine, 13001 East 17th Pl, Aurora, CO 80045, USA. Electronic address: nickj@njhealth.org.
authors: Jerry A
authors: JA
authors: Nick
authors: Department of Medicine, University of Colorado Denver School of Medicine, 13001 East 17th Pl, Aurora, CO 80045, USA; Department of Pediatrics, National Jewish Health, Denver, CO 80206, USA.
authors: David P
authors: DP
authors: Nichols
mesh_terms: D000328:Adult; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000032:analysis* / Q000235:genetics; D005820:Genetic Testing / Q000379:methods*; D006801:Humans; D009154:Mutation
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Adult; Bronchiectasis; Cystic fibrosis; Diagnosis; Genotype