Impact of different chloride and glucose solutions on nasal potential difference

2009 ◽  
Vol 44 (7) ◽  
pp. 645-648 ◽  
Author(s):  
Hugh H. House ◽  
Peter G. Middleton
Keyword(s):  
Potential Difference ◽  
Nasal Potential Difference

scholarly journals An International Randomized Multicenter Comparison of Nasal Potential Difference Techniques

CHEST Journal ◽  
2010 ◽  
Vol 138 (4) ◽  
pp. 919-928 ◽  
Author(s):  
George M. Solomon ◽  
Michael W. Konstan ◽  
Michael Wilschanski ◽  
Joanne Billings ◽  
Isabelle Sermet-Gaudelus ◽  
...  
Keyword(s):  
Potential Difference ◽  
Nasal Potential Difference

Uncertainty in the diagnosis of cystic fibrosis: Possible role of in vivo nasal potential difference measurements

1998 ◽  
Vol 132 (4) ◽  
pp. 596-599 ◽  
Author(s):  
David C. Wilson ◽  
Lynda Ellis ◽  
Julian Zielenski ◽  
Mary Corey ◽  
Wan F. Ip ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Potential Difference ◽  
Nasal Potential Difference

scholarly journals Assessment of Lentiviral Vector Mediated CFTR Correction in Mice Using an Improved Rapid in vivo Nasal Potential Difference Measurement Protocol

2021 ◽  
Vol 12 ◽  
Author(s):  
P. Cmielewski ◽  
J. Delhove ◽  
M. Donnelley ◽  
D. Parsons
Keyword(s):  
Ion Transport ◽  
Lentiviral Vector ◽  
Chloride Transport ◽  
Diagnostic Technique ◽  
Potential Difference ◽  
Third Generation ◽  
Flow Rates ◽  
Measurement Protocol ◽  
Nasal Potential Difference

Cystic Fibrosis (CF) is caused by a defect in the CF transmembrane conductance regulator (CFTR) gene responsible for epithelial ion transport. Nasal potential difference (PD) measurement is a well established diagnostic technique for assessing the efficacy of therapies in CF patients and animal models. The aim was to establish a rapid nasal PD protocol in mice and quantify the efficacy of lentiviral (LV) vector-based CFTR gene therapy. Anaesthetised wild-type (WT) and CF mice were non-surgically intubated and nasal PD measurements were made using a range of buffer flow rates. Addition of the cAMP agonist, isoproterenol, to the buffer sequence was then examined. The optimised rapid PD technique was then used to assess CFTR function produced by second and third generation LV-CFTR vectors. V5 epitope tagged-CFTR in nasal tissue was identified by immunohistochemistry. When intubated, mice tolerated higher flow rates. Isoproterenol could discriminate between WT and CF mice. Improved chloride transport was observed for the second and third generation LV-CFTR vectors, with up to 60% correction of the cAMP-driven chloride response towards WT. V5-CFTR was located in ciliated epithelial cells. The rapid PD technique enables improved functional assessment of the bioelectrical ion transport defect for both current and potential CF therapies.

Comparison of Nasal Potential Difference and Intestinal Current Measurements as Surrogate Markers for CFTR Function

2016 ◽  
Vol 63 (5) ◽  
pp. e92-e97 ◽  
Author(s):  
Michael Wilschanski ◽  
Yasmin Yaakov ◽  
Ibrahim Omari ◽  
Munir Zaman ◽  
Camilia R. Martin ◽  
...  
Keyword(s):  
Potential Difference ◽  
Surrogate Markers ◽  
Nasal Potential Difference

scholarly journals Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis

1997 ◽  
Vol 10 (9) ◽  
pp. 2018-2022 ◽  
Author(s):  
L.P. Ho ◽  
J.M. Samways ◽  
D.J. Porteous ◽  
J.R. Dorin ◽  
A. Carothers ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Clinical Condition ◽  
Potential Difference ◽  
Nasal Potential Difference

scholarly journals 483 Nasal potential difference measurements in Turkish CF patients

2006 ◽  
Vol 5 ◽  
pp. S106
Author(s):  
G. Cinell ◽  
M. Sevgili ◽  
D. Balkancy ◽  
I. Karabulut ◽  
D. Dogru ◽  
...  
Keyword(s):  
Potential Difference ◽  
Nasal Potential Difference

scholarly journals Nasal potential difference to detect Na+channel dysfunction in acute lung injury

2011 ◽  
Vol 300 (3) ◽  
pp. L305-L318 ◽  
Author(s):  
R. Mac Sweeney ◽  
H. Fischer ◽  
D. F. McAuley
Keyword(s):  
Acute Lung Injury ◽  
Lung Injury ◽  
Ion Channel ◽  
Pulmonary Edema ◽  
Potential Difference ◽  
Na Channel ◽  
Alveolar Epithelial ◽  
Nasal Potential Difference ◽  
Respiratory Epithelial

Pulmonary fluid clearance is regulated by the active transport of Na+and Cl−through respiratory epithelial ion channels. Ion channel dysfunction contributes to the pathogenesis of various pulmonary fluid disorders including high-altitude pulmonary edema (HAPE) and neonatal respiratory distress syndrome (RDS). Nasal potential difference (NPD) measurement allows an in vivo investigation of the functionality of these channels. This technique has been used for the diagnosis of cystic fibrosis, the archetypal respiratory ion channel disorder, for over a quarter of a century. NPD measurements in HAPE and RDS suggest constitutive and acquired dysfunction of respiratory epithelial Na+channels. Acute lung injury (ALI) is characterized by pulmonary edema due to alveolar epithelial-interstitial-endothelial injury. NPD measurement may enable identification of critically ill ALI patients with a susceptible phenotype of dysfunctional respiratory Na+channels and allow targeted therapy toward Na+channel function.

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