Relationships between nasal potential difference and respiratory function in adults with cystic fibrosis.
This study investigated the relations between nasal transepithelial electric potential difference (PD) and the phenotype and genotype of cystic fibrosis (CF) adult patients. Basal nasal PD was measured in 95 adult CF patients who were classified into three groups of nasal PD (expressed as absolute values) according to the 10th and the 90th percentiles (28.3 and 49.2 mV, respectively), which defined group 1 (nasal PD < or =28.3 mV), group 2 (nasal PD 28.3-49.2 mV) and group 3 (nasal PD > or =49.2 mV). Patients from group 1 had a higher forced vital capacity (FVC) than patients from groups 2 and 3 (76.5+/-22.4 versus 57.4+/-21.2 and 55.7+/-21.1% predicted, respectively, p<0.05) and a higher forced expiratory volume in one second (FEV1) (69.3+/-24.0 versus 42.5+/-22.4 and 42.2+/-21.4% pred, respectively, p<0.01). Among patients with severe mutations (deltaF508 homozygotes, or one deltaF508 mutation plus another "severe" mutation, or two "severe" mutations), patients from group 1 had a higher FVC, FEV1 and arterial oxygen tension than patients from groups 2 and 3 (p<0.05 for each comparison). The results show that in adult cystic fibrosis patients a normal basal nasal potential difference is related to milder respiratory disease, irrespective of the severity of the genotype.
Fajac
I
I
Dept of Respiratory Physiology, Cochin Hospital, René Descartes University, Paris, France.
Hubert
D
D
Bienvenu
T
T
Richaud-Thiriez
B
B
Matran
R
R
Kaplan
J C
JC
Dall'Ava-Santucci
J
J
Dusser
D J
DJ
D000293:Adolescent; D000328:Adult; D003550:Cystic Fibrosis / Q000235:genetics / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008297:Male; D008564:Membrane Potentials; D008875:Middle Aged; D009154:Mutation; D009297:Nasal Mucosa / Q000503:physiopathology*; D015656:Respiratory Mechanics; D014797:Vital Capacity
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator