title: Airway ion transport impacts on disease presentation and severity in cystic fibrosis.
abstract: OBJECTIVE
  Abnormal airway ion transport is a feature of cystic fibrosis. The aim of this study was to investigate whether distinct components of ion transport are associated with the clinical expression and severity of the disease.
  
  
  METHODS
  Univariate and multivariate analyses were used to study interaction effects between nasal potential difference parameters and clinical status, recorded at stable conditions, in 75 F508del homozygous young adults.
  
  
  RESULTS
  All patients demonstrated increased sodium and reduced chloride conductances. Less sodium transport abnormalities were related to better respiratory function and nutrition. Presentation with digestive symptoms at diagnosis was associated with lower chloride conductance. With an accuracy of 85% good nutritional status was linked to more preserved lung function, increasing age and more preserved chloride conductance.
  
  
  CONCLUSIONS
  Ion transport abnormalities have distinct clinical outcomes. Sodium conductance relates to respiratory function and nutrition; chloride conductance to nutrition and presentation with digestive symptoms at diagnosis.
authors.affiliation: Clinical Chemistry, Université Catholique de Louvain, Brussels, Belgium.
authors.forename: Teresinha
authors.initials: T
authors.lastname: Leal
authors.forename: Isabelle
authors.initials: I
authors.lastname: Fajac
authors.forename: Helen L
authors.initials: HL
authors.lastname: Wallace
authors.forename: Patrick
authors.initials: P
authors.lastname: Lebecque
authors.forename: Jean
authors.initials: J
authors.lastname: Lebacq
authors.forename: Dominique
authors.initials: D
authors.lastname: Hubert
authors.forename: Josette
authors.initials: J
authors.lastname: Dall'Ava
authors.forename: Daniel
authors.initials: D
authors.lastname: Dusser
authors.forename: Anusha P
authors.initials: AP
authors.lastname: Ganesan
authors.forename: Christiane
authors.initials: C
authors.lastname: Knoop
authors.forename: Jean
authors.initials: J
authors.lastname: Cumps
authors.forename: Pierre
authors.initials: P
authors.lastname: Wallemacq
authors.forename: Kevin W
authors.initials: KW
authors.lastname: Southern
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001692:Biological Transport; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000235:genetics / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000502:physiology; D004200:Diseases in Twins; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D017136:Ion Transport / Q000502:physiology*; D008297:Male; D009297:Nasal Mucosa / Q000503:physiopathology; D010477:Perfusion; D011199:Potentiometry; D012720:Severity of Illness Index; D035781:Siblings; D012964:Sodium / Q000378:metabolism
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D012964:Sodium