Airway ion transport impacts on disease presentation and severity in cystic fibrosis.
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.
Leal
Teresinha
T
Clinical Chemistry, Université Catholique de Louvain, Brussels, Belgium.
Fajac
Isabelle
I
Wallace
Helen L
HL
Lebecque
Patrick
P
Lebacq
Jean
J
Hubert
Dominique
D
Dall'Ava
Josette
J
Dusser
Daniel
D
Ganesan
Anusha P
AP
Knoop
Christiane
C
Cumps
Jean
J
Wallemacq
Pierre
P
Southern
Kevin W
KW
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
D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D012964:Sodium