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1

Uwaifo, Omotola, Penelope Bamford, Pamela L. Zeitlin, and Carol J. Blaisdell. "Acidic pH Hyperpolarizes Nasal Potential Difference." Pediatric Pulmonology 41, no. 2 (2006): 151–57. http://dx.doi.org/10.1002/ppul.20328.

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2

Sermet-Gaudelus, I., D. Roussel, F. Huet, et al. "369* Nasal potential difference in hypertrypsinemic children." Journal of Cystic Fibrosis 6 (June 2007): S90. http://dx.doi.org/10.1016/s1569-1993(07)60339-7.

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3

Vermeulen, F., N. Feyaerts, M. Proesmans, and K. De Boeck. "31 Modified nasal catheter for measurement of nasal potential difference improves reproducibility." Journal of Cystic Fibrosis 10 (June 2011): S8. http://dx.doi.org/10.1016/s1569-1993(11)60052-0.

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4

Vermeulen, F., J. Ophoff, M. Proesmans, L. J. Dupont, and K. De Boeck. "49 Modified nasal catheter for measurement of nasal potential difference improves repeatability." Journal of Cystic Fibrosis 11 (June 2012): S68. http://dx.doi.org/10.1016/s1569-1993(12)60218-5.

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5

Bronsveld, Inez, François Vermeulen, Dorotha Sands, et al. "Influence of perfusate temperature on nasal potential difference." European Respiratory Journal 42, no. 2 (2012): 389–93. http://dx.doi.org/10.1183/09031936.00097712.

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6

Chung, Nancy C., Beate Illek, Jonathan H. Widdicombe, and Horst Fischer. "Measurement of Nasal Potential Difference in Mild Asthmatics." Chest 123, no. 5 (2003): 1467–71. http://dx.doi.org/10.1378/chest.123.5.1467.

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7

Domingo-Ribas, C., and M. Bosque-García. "Nasal Potential Difference Test to Diagnose Cystic Fibrosis." Archivos de Bronconeumología ((English Edition)) 42, no. 1 (2006): 33–38. http://dx.doi.org/10.1016/s1579-2129(06)60111-0.

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8

Ng, Ronny Tah Yen, Fernando Augusto de Lima Marson, Jose Dirceu Ribeiro, et al. "Nasal Potential Difference in Cystic Fibrosis considering SevereCFTRMutations." Disease Markers 2015 (2015): 1–11. http://dx.doi.org/10.1155/2015/306825.

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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 subject
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9

Schüler, Daniel, Isabelle Sermet-Gaudelus, Michael Wilschanski, et al. "Basic protocol for transepithelial nasal potential difference measurements." Journal of Cystic Fibrosis 3 (August 2004): 151–55. http://dx.doi.org/10.1016/j.jcf.2004.05.032.

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10

Graham, Scott M., Shaun N. Scott, Janice Launspach, and Joseph Zabner. "The Effects of Fluticasone Propionate on Nasal Epithelial Potential Difference." American Journal of Rhinology 16, no. 3 (2002): 145–49. http://dx.doi.org/10.1177/194589240201600304.

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Background Human airway epithelium maintains homeostasis of the fluid and salt composition at the airway surface by a regulated transport of sodium and chloride ions. The volume and composition of airway surface liquid have been shown to be important in the pathogenesis of cystic fibrosis, nasal inflammatory disease, and nasal polyposis. The presence of functional epithelial sodium and chloride channels in the airway epithelium can be evaluated electrically by measuring the voltage across the nasal epithelium (Vt). Because fluticasone propionate is commonly used to treat nasal inflammatory dis
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11

STEWART, ELEANOR J., MICHAEL J. CINNAMOND, PAUL NICHOLLS, and C. FRED STANFORD. "The effects of diuretics on nasal transmucosal potential difference." Clinical Otolaryngology 17, no. 4 (1992): 351–53. http://dx.doi.org/10.1111/j.1365-2273.1992.tb01011.x.

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12

Cohen, M., Y. Yaakov, H. Friedman, et al. "26 Nasal potential difference in young children is feasible." Journal of Cystic Fibrosis 16 (June 2017): S70. http://dx.doi.org/10.1016/s1569-1993(17)30391-0.

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13

Cinell, G., M. Sevgili, D. Balkancy, et al. "483 Nasal potential difference measurements in Turkish CF patients." Journal of Cystic Fibrosis 5 (2006): S106. http://dx.doi.org/10.1016/s1569-1993(06)80407-8.

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14

Yaakov, Yasmin, Eitan Kerem, Yaakov Yahav, et al. "Reproducibility of Nasal Potential Difference Measurements in Cystic Fibrosis." Chest 132, no. 4 (2007): 1219–26. http://dx.doi.org/10.1378/chest.06-2975.

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15

Mairbäurl, Heimo, Jörn Weymann, Andreas Möhrlein, et al. "Nasal Epithelium Potential Difference at High Altitude (4,559 m)." American Journal of Respiratory and Critical Care Medicine 167, no. 6 (2003): 862–67. http://dx.doi.org/10.1164/rccm.200208-864oc.

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16

Baker, E., X. Jeunemaitre, A. J. Portal, et al. "Abnormalities of nasal potential difference measurement in Liddle's syndrome." Journal of Clinical Investigation 102, no. 1 (1998): 10–14. http://dx.doi.org/10.1172/jci1795.

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17

Procianoy, Elenara da Fonseca Andrade, Fernando Antônio de Abreu e Silva, Paulo José Cauduro Maróstica, and Paul M. Quinton. "Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology." Lung 198, no. 1 (2019): 151–56. http://dx.doi.org/10.1007/s00408-019-00293-6.

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18

Peckham, D. G., A. Conn, C. Chotai, S. Lewis, and A. J. Knox. "Effect of Oral Digoxin, Topical Ouabain and Salbutamol on Transepithelial Nasal Potential Difference in Patients with Cystic Fibrosis." Clinical Science 89, no. 3 (1995): 277–84. http://dx.doi.org/10.1042/cs0890277.

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1. Airway epithelium in cystic fibrosis is characterized by a defect in chloride secretion across the apical membrane and an increase in sodium absorption. The increased rate of sodium absorption can be inhibited in vitro by ouabain, a Na+-K+-ATPase inhibitor, and in cystic fibrosis patients the number and activity of nasal epithelial Na+-K+-ATPase pumps is increased. 2. We have performed a series of studies to determine whether drugs which modify airway epithelial Na+-K+-ATPase activity in vitro can modify nasal potential in cystic fibrosis patients in vivo. As transepithelial nasal potential
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19

Wilschanski, M., H. Famini, N. Strauss-Liviatan, et al. "Nasal potential difference measurements in patients with atypical cystic fibrosis." European Respiratory Journal 17, no. 6 (2001): 1208–15. http://dx.doi.org/10.1183/09031936.01.00092501.

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20

Höfmann, T., O. Bohmer, G. Hüls, et al. "Conventional and modified nasal potential-difference measurement in cystic fibrosis." American Journal of Respiratory and Critical Care Medicine 155, no. 6 (1997): 1908–13. http://dx.doi.org/10.1164/ajrccm.155.6.9196094.

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21

Solomon, George M., Michael W. Konstan, Michael Wilschanski, et al. "An International Randomized Multicenter Comparison of Nasal Potential Difference Techniques." Chest 138, no. 4 (2010): 919–28. http://dx.doi.org/10.1378/chest.10-0179.

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22

Tridello, Gloria, Laura Menin, Emily Pintani, Gabriella Bergamini, Baroukh Maurice Assael, and Paola Melotti. "Nasal potential difference outcomes support diagnostic decisions in cystic fibrosis." Journal of Cystic Fibrosis 15, no. 5 (2016): 579–82. http://dx.doi.org/10.1016/j.jcf.2016.06.009.

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23

Solomon, George M., Bo Liu, Isabelle Sermet-Gaudelus, et al. "A multiple reader scoring system for Nasal Potential Difference parameters." Journal of Cystic Fibrosis 16, no. 5 (2017): 573–78. http://dx.doi.org/10.1016/j.jcf.2017.04.011.

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24

Alton, EW, D. Currie, R. Logan-Sinclair, JO Warner, ME Hodson, and DM Geddes. "Nasal potential difference: a clinical diagnostic test for cystic fibrosis." European Respiratory Journal 3, no. 8 (1990): 922–26. http://dx.doi.org/10.1183/09031936.93.03080922.

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Patients with cystic fibrosis (CF) demonstrate a markedly more negative potential difference (PD) across respiratory epithelia than normal or "diseased" controls. A technique is described for the measurement of nasal PD in both children and adults. 145 non-CF subjects showed a mean PD of -19.0 mV (range -2 to -36) in comparison to 60 patients with cystic fibrosis with mean of -46.0 mV (range -32 to -77). Amongst the latter group those with more severe disease had a more negative PD. Measurement of nasal PD is easily learnt and rapidly performed and may provide an additional means of diagnosis
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25

Kersting, U., A. Schwab, and A. Hebestreit. "Measurement of human nasal potential difference to teach the theory of transepithelial fluid transport." Advances in Physiology Education 275, no. 6 (1998): S72. http://dx.doi.org/10.1152/advances.1998.275.6.s72.

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We describe a novel student course in membrane physiology in which students record their own nasal potential difference, i.e., the transepithelial potential difference of the respiratory mucosa in the nose. The nasal potential difference monitors directly, and in vivo, changes in the apical cell membrane potential of the respiratory mucosa induced by activators and inhibitors of ion channel activities. Basic principles of transepithelial fluid transport are taught by applying an appropriate perfusion protocol to the respiratory epithelium to either depolarize or hyperpolarize the membrane pote
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26

Leonard, Anissa, Bob Lubamba, Barbara Dhooghe, et al. "Comparative Variability of Nasal Potential Difference Measurements in Human and Mice." Open Journal of Respiratory Diseases 02, no. 02 (2012): 43–56. http://dx.doi.org/10.4236/ojrd.2012.22007.

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27

Delmarco, A., U. Pradal, G. Cabrini, A. Bonizzato, and G. Mastella. "Nasal potential difference in cystic fibrosis patients presenting borderline sweat test." European Respiratory Journal 10, no. 5 (1997): 1145–49. http://dx.doi.org/10.1183/09031936.97.10051145.

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28

Durie, P. R., L. Ellis, M. Corey, et al. "NASAL TRANSEPITHELIAL POTENTIAL DIFFERENCE IN PATIENTS WITH DEFINED CFTR GENE MUTATIONS." Journal of Pediatric Gastroenterology and Nutrition 21, no. 3 (1995): 359. http://dx.doi.org/10.1097/00005176-199510000-00150.

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29

PRADAL, UGO, CARLO CASTELLANI, ANTONELLA DELMARCO, and GIANNI MASTELLA. "Nasal Potential Difference in Congenital Bilateral Absence of the Vas Deferens." American Journal of Respiratory and Critical Care Medicine 158, no. 3 (1998): 896–901. http://dx.doi.org/10.1164/ajrccm.158.3.9711029.

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30

Sermet-Gaudelus, Isabelle, Emmanuelle Girodon, Frédéric Huet, et al. "Nasal potential difference in cystic fibrosis diagnosis of very young children." Journal of Pediatrics 150, no. 3 (2007): e34-e35. http://dx.doi.org/10.1016/j.jpeds.2006.11.055.

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31

Middleton, P. G., and J. R. Bishop. "368 Nasal potential difference testing in the diagnostic algorithm of CF." Journal of Cystic Fibrosis 6 (June 2007): S90. http://dx.doi.org/10.1016/s1569-1993(07)60338-5.

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32

Sinaasappel, M., H. De Jonge, and I. Bronsveld. "P0289 NASAL POTENTIAL DIFFERENCE VERSUS INTESTINAL CURRENT MEASUREMENTS IN CF DIAGNOSIS." Journal of Pediatric Gastroenterology and Nutrition 39, Supplement 1 (2004): S167. http://dx.doi.org/10.1097/00005176-200406001-00413.

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33

Segal, I., E. Kerem, Y. Yaacov, et al. "P0811 THE USE OF NASAL POTENTIAL DIFFERENCE MEASUREMENTS IN RECURRENT PANCREATITIS." Journal of Pediatric Gastroenterology and Nutrition 39, Supplement 1 (2004): S365. http://dx.doi.org/10.1097/00005176-200406001-00935.

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34

Vermeulen, François, Marijke Proesmans, Mieke Boon, and Kris De Boeck. "Improved repeatability of nasal potential difference with a larger surface catheter." Journal of Cystic Fibrosis 14, no. 3 (2015): 317–23. http://dx.doi.org/10.1016/j.jcf.2014.08.006.

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35

Aalbers, Bente L., Yasmin Yaakov, Nico Derichs, et al. "Nasal potential difference in suspected cystic fibrosis patients with 5T polymorphism." Journal of Cystic Fibrosis 19, no. 4 (2020): 627–31. http://dx.doi.org/10.1016/j.jcf.2019.07.001.

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36

House, Hugh H., and Peter G. Middleton. "Impact of different chloride and glucose solutions on nasal potential difference." Pediatric Pulmonology 44, no. 7 (2009): 645–48. http://dx.doi.org/10.1002/ppul.21032.

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37

Dias, Michael A., Pathmajani Sukumar, Thomas K. Fitzgerald, Perry W. Alexion, and Rebecca Bascom. "A Sub-Irritant Pulse of Air to the Nasal Mucosa Alters Nasal Transepithelial Potential Difference in Humans." Otolaryngology–Head and Neck Surgery 113, no. 2 (1995): P136. http://dx.doi.org/10.1016/s0194-5998(05)80807-1.

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38

Vermeulen, F., F. Vermeulen, N. Feyaerts, M. Proesmans, and K. De Boeck. "Comparison of nasal potential difference measurements done on the nasal floor and under the inferior turbinate: repeatability." Journal of Cystic Fibrosis 8 (June 2009): S10. http://dx.doi.org/10.1016/s1569-1993(09)60043-6.

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39

Valiulis, Arūnas, Iveta Skurvydienė, Valdonė Misevičienė, Jūratė Kasnauskienė, Laimutė Vaidelienė, and Algirdas Utkus. "Relevance of Nasal Potential Difference in Diagnosis of Cystic Fibrosis Among Children." Medicina 49, no. 4 (2013): 29. http://dx.doi.org/10.3390/medicina49040029.

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Objective. The aim of this study was to estimate the significance of nasal potential difference (NPD) in the diagnosis of cystic fibrosis (CF) in children with clinical symptoms suggestive of the disease, positive sweat test results, and/or genetically confirmed diagnosis. Material and Methods. NPD measurements according to the modifications by Alton were performed in 50 children with clinical CF symptoms supported by positive sweat test results, 50 children with other obstructive lung diseases, and 50 healthy children. A subgroup of 17 children with the diagnosis confirmed by 2 identified mut
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40

Griesenbach, Uta, Stephen N. Smith, Raymond Farley, Charanjit Singh, and Eric W. F. W. Alton. "Validation of Nasal Potential Difference Measurements in Gut-Corrected CF Knockout Mice." American Journal of Respiratory Cell and Molecular Biology 39, no. 4 (2008): 490–96. http://dx.doi.org/10.1165/rcmb.2007-0385oc.

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41

Mac Sweeney, R., H. Fischer, and D. F. McAuley. "Nasal potential difference to detect Na+channel dysfunction in acute lung injury." American Journal of Physiology-Lung Cellular and Molecular Physiology 300, no. 3 (2011): L305—L318. http://dx.doi.org/10.1152/ajplung.00223.2010.

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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 HAP
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42

Southern, Kevin W., Peadar G. Noone, Darin G. Bosworth, Vicky A. LeGrys, Michael R. Knowles, and Pierre M. Barker. "A modified technique for measurement of nasal transepithelial potential difference in infants." Journal of Pediatrics 139, no. 3 (2001): 353–58. http://dx.doi.org/10.1067/mpd.2001.116278.

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43

Gowen, C. W., E. E. Lawson, J. Gingras-Leatherman, J. T. Gatzy, R. C. Boucher, and M. R. Knowles. "Increased nasal potential difference and amiloride sensitivity in neonates with cystic fibrosis." Journal of Pediatrics 108, no. 4 (1986): 517–21. http://dx.doi.org/10.1016/s0022-3476(86)80825-3.

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44

Tümmler, B., A. Schulz, J. Rademacher, T. Welte, and F. C. Ringshausen. "ePS01.9 Nasal potential difference in adults with idiopathic non-cystic fibrosis bronchiectasis." Journal of Cystic Fibrosis 15 (June 2016): S38—S39. http://dx.doi.org/10.1016/s1569-1993(16)30196-5.

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45

Yaakov, Y., R. Jaron, J. Rivlin, et al. "Nasal potential difference in non classic cystic fibrosis – long term follow up." Journal of Cystic Fibrosis 7 (June 2008): S10. http://dx.doi.org/10.1016/s1569-1993(08)60039-9.

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46

Middleton, P. G., and H. H. House. "Nasal potential difference testing – the effect of different glucose and chloride concentrations." Journal of Cystic Fibrosis 7 (June 2008): S21. http://dx.doi.org/10.1016/s1569-1993(08)60080-6.

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47

Schüler, P., S. Luigart, S. Prange-Schmidt, et al. "Duration of decreasing the nasal potential difference by amiloride-mannitol dry powder." Journal of Cystic Fibrosis 7 (June 2008): S23. http://dx.doi.org/10.1016/s1569-1993(08)60089-2.

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48

Gowen, C. W., E. E. Lawson, J. Gingras-Leatherman, R. C. Boucher, and M. R. Knowles. "1399 NASAL ELECTRIC POTENTIAL DIFFERENCE AND RESPONSE TO AMILORIDE SUPERFUSION IN NEONATES." Pediatric Research 19, no. 4 (1985): 344A. http://dx.doi.org/10.1203/00006450-198504000-01423.

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49

Saussereau, Emilie Lyne, Delphine Roussel, Siradiou Diallo, Laurent Debarbieux, Aleksander Edelman, and Isabelle Sermet-Gaudelus. "Characterization of Nasal Potential Difference in cftr Knockout and F508del-CFTR Mice." PLoS ONE 8, no. 3 (2013): e57317. http://dx.doi.org/10.1371/journal.pone.0057317.

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50

Naehrlich, Lutz, Manfred Ballmann, Jane Davies, et al. "Nasal potential difference measurements in diagnosis of cystic fibrosis: An international survey." Journal of Cystic Fibrosis 13, no. 1 (2014): 24–28. http://dx.doi.org/10.1016/j.jcf.2013.08.006.

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