comparative variability of nasal potential difference measurements in human and mice比较鼻电位差的变化测量在人类和老鼠.pdfVIP

Open Journal of Respiratory Diseases, 2012, 2, 43-56 /10.4236/ojrd.2012.22007 Published Online May 2012 (http://www.SciRP.org/journal/ojrd) Comparative Variability of Nasal Potential Difference Measurements in Human and Mice 1,2* 2* 2 2 2 Anissa Leonard , Bob Lubamba , Barbara Dhooghe , Sabrina Noel , Pierre Wallemacq , Patrick Lebecque1, Teresinha Leal1,2# 1Pediatric Pulmonology Cystic Fibrosis Unit, St Luc University Hospital, Brussels, Belgium 2Louvain Centre for Toxicology and Applied Pharmacology, Institut de Recherche Clinique et Expérimentale, Université Catholique de Louvain, Brussels, Belgium Email: #teresinha.leal@uclouvain.be Received February 21, 2012; revised March 20, 2012; accepted April 25, 2012 ABSTRACT Background: Nasal potential difference (NPD) test has long been used to assist in the diagnosis of Cystic Fibrosis (CF) and more recently as an outcome measure in clinical trials of new CF therapies. This test has also been adapted to the mouse nose. Objectives: We aimed at evaluating variability of the NPD measurements in CF patients displaying two severe CFTR mutations and in sex-matched healthy controls. NPD recorded from F508del-CF and normal wild-type mice were also compared. Methods and Results: In each setting, tests were performed by a single qualified operator. In the clinical setting, the latest standardized operation protocol of the CF foundation was followed. A total of 80 trac- ings were obtained from 10 patients (23.2 y; range 14 to 32) and 10 healthy subjects (34 y; range 24 to 53), each tested twice, in both nostrils. Two CF and two con