Reproducibility of nasal potential difference measurements in cystic fibrosis.
BACKGROUND
Nasal potential difference (NPD) measurement has been advocated as a diagnostic tool for cystic fibrosis (CF) patients and as a method for assessing the response to new therapies. The purpose of this study was to examine the reproducibility of NPD measurements performed in a single center.


METHODS
A total of 68 CF patients with a mean (+/- SD) age of 16 +/- 8 years (age range, 6 to 52 years) underwent NPD measurements on at least two occasions.


RESULTS
A total of 25 patients with classic CF (mean age, 21 +/- 8 years) and 43 patients with nonclassic CF (mean age, 14 +/- 8 years) underwent sweat tests and NPD measurements. The mean sweat chloride values were 102 +/- 18 and 54 +/- 14 mEq/L, respectively, for classic CF and nonclassic CF groups. All patients underwent repeat NPD measurements. The basal NPD and the response to amiloride (DeltaAmil) and response to Cl(-) free and isoproterenol (DeltaCl(-) free + iso) were very similar in both measurements. In the classic CF group, the basal potential difference values were -40 +/- 12 vs -39 +/- 11 mV (p = 0.57), respectively, for the first and second measurements; 27 +/- 9 vs 26 +/- 10 mV (p = 0.55), respectively, for DeltaAmil; and 2.1 +/- 3.8 vs 0.4 +/- 2.9 mV (p = 0.07), respectively, for DeltaCl(-) free + iso. In the nonclassic CF group, the values were -32 +/- 13 vs -28 +/- 10 mV (p = 0.008), respectively; 19 +/- 10 vs 17 +/- 8 mV (p = 0.388), respectively; and -3.2 +/- 4.6 vs -3.3 +/- 4.4 mV (p = 0.876), respectively.


CONCLUSIONS
When performed in a single center, NPD is a reproducible test for CF patients and thus may be a useful outcome measurement for assessment of the efficacy of new treatments.
Yaakov
Yasmin
Y
Cystic Fibrosis Center, Hadassah University Hospital, Jerusalem, Israel.
Kerem
Eitan
E
Yahav
Yaakov
Y
Rivlin
Joseph
J
Blau
Hannah
H
Bentur
Lea
L
Aviram
Micha
M
Picard
Elie
E
Bdolah-Abram
Tali
T
Wilschanski
Michael
M
D000293:Adolescent; D000328:Adult; D002648:Child; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008564:Membrane Potentials; D008875:Middle Aged; D015203:Reproducibility of Results; D012189:Retrospective Studies; D015222:Sodium Channels / Q000502:physiology
D015222:Sodium Channels