Nasal potential difference: Best or average result for CFTR function as diagnostic criteria for cystic fibrosis?

作者：Keenan Katherine; Avolio Julie; Rueckes Nilges Claudia; Tullis Elizabeth; Gonska Tanja; Naehrlich Lutz^*

来源：Journal of Cystic Fibrosis, 2015, 14(3): 310-316.

DOI：10.1016/j.jcf.2014.09.006

• Cystic fibrosis
• Diagnosis
• Nasal potential difference

Background: The current practice of averaging the nasal potential difference (NPD) results of right and left nostril measurements reduce inter-individual variability but may underestimate individual CFTR function.
Methods: Best NPD response to Cl--free and isdproterenol perfusion (=largest Delta PD0Cl/Iso) from the right and left nostril was compared to the average result in 13 cystic fibrosis (CF), 78 query-CF patients and 22 healthy controls from 2 cohorts.
Results: Despite moderate to good correlation (p < 0.001) between right and left measured Delta PD0Cl/Iso, we observed large differences in some individuals. A comparison of average versus best Delta PD0Cl/Iso showed only moderate agreement (Giessen kappa = 0.538; Toronto kappa = 0.607). Averaging Delta PD0Cl/Iso showed a lower composite chloride response compared to best Delta PD0Cl/Iso and altered diagnostic NPD interpretation in 30 of 113 (27%) subjects.
Conclusions: The current practice of averaging the NPD results of right and left nostril measurements leads to an underestimation of the individual CFTR function and should be reconsidered.

• 出版日期2015-5