Locally deposited but not inhaled frusemide reduces nasal potential difference in healthy subjects

1993 ◽  
Vol 45 (4) ◽  
Author(s):  
P. Mialon ◽  
R. Charfi ◽  
J. Regnard ◽  
A. Lockhart ◽  
A.T. Dinh-Xuan
2015 ◽  
Vol 2015 ◽  
pp. 1-11 ◽  
Author(s):  
Ronny Tah Yen Ng ◽  
Fernando Augusto de Lima Marson ◽  
Jose Dirceu Ribeiro ◽  
Antonio Fernando Ribeiro ◽  
Carmen Silvia Bertuzzo ◽  
...  

The gold standard for diagnosing cystic fibrosis (CF) is a sweat chloride value above 60 mEq/L. However, this historical and important tool has limitations; other techniques should be studied, including the nasal potential difference (NPD) test.CFTRgene sequencing can identifyCFTRmutations, but this method is time-consuming and too expensive to be used in all CF centers. The present study compared CF patients with two classes I-IIICFTRmutations (10 patients) (G1), CF patients with classes IV-VICFTRmutations (five patients) (G2), and 21 healthy subjects (G3). The CF patients and healthy subjects also underwent the NPD test. A statistical analysis was performed using the Mann-Whitney, Kruskal-Wallis,χ2, and Fisher’s exact tests,α=0.05. No differences were observed between the CF patients and healthy controls for the PDMax, Δamiloride, and Δchloride + free + amiloride markers from the NPD test. For the finger value, a difference between G2 and G3 was described. The Wilschanski index values were different between G1 and G3. In conclusion, our data showed that NPD is useful for CF diagnosis when classes I-IIICFTRmutations are screened. However, if classes IV-VI are considered, the NPD test showed an overlap in values with healthy subjects.


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

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

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

2021 ◽  
Vol 12 ◽  
Author(s):  
P. Cmielewski ◽  
J. Delhove ◽  
M. Donnelley ◽  
D. Parsons

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.


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

1997 ◽  
Vol 10 (9) ◽  
pp. 2018-2022 ◽  
Author(s):  
L.P. Ho ◽  
J.M. Samways ◽  
D.J. Porteous ◽  
J.R. Dorin ◽  
A. Carothers ◽  
...  

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

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