4.3 Article

Diagnosis of Adult Patients with Cystic Fibrosis

Journal

CLINICS IN CHEST MEDICINE
Volume 37, Issue 1, Pages 47-+

Publisher

W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.ccm.2015.11.006

Keywords

Cystic fibrosis; Diagnosis; Adult; Bronchiectasis; Genotype

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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.

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