Increased nasal potential difference and amiloride sensitivity in neonates with cystic fibrosis |
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| Authors: | C W Gowen E E Lawson J Gingras-Leatherman J T Gatzy R C Boucher M R Knowles |
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| Affiliation: | 1. Section of Interventional Cardiology, MedStar Washington Hospital Center, Washington, DC, United States of America;2. Cardiovascular Branch, Division of Intramural Research, National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, MD, United States of America;3. Department of Cardiac Surgery, MedStar Washington Hospital Center, Washington, DC, United States of America;1. Terrence Donnelly Heart Centre, St Michael''s Hospital, University of Toronto, Toronto, ON, Canada;2. Duke Clinical Research Institute, Durham, NC, USA;3. Canadian Heart Research Centre, Toronto, ON, Canada;4. Eli Lilly Canada Inc., Toronto, ON, Canada;5. Mazankowski Alberta Heart Institute, University of Alberta, Canadian VIGOUR Centre, Edmonton, AB, Canada;6. Institut Universitaire de Cardiologie et de Pneumologie de Quebec, Quebec, QC, Canada;7. Eli Lilly and Company, Indianapolis, IN, United States;8. Prairie Vascular Research Network, Regina Qu''Appelle Health Region, Regina, SK, Canada;9. Rouge Valley Health System, Toronto, ON, Canada;10. Cardiac Sciences Program, St Boniface General Hospital, University of Manitoba, Winnipeg, MB, Canada;11. Royal Alexandra Hospital, Edmonton, AB, Canada;12. St. Mary''s General Hospital, Kitchener, ON, Canada;13. Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada;14. Hôpital du Sacré-Coeur de Montréal, Université de Montréal, QC, Canada;15. Dalhousie University, Queen Elizabeth II Health Sciences Centre, Halifax, NS, Canada;p. Population Health Research Institute, McMaster University and Hamilton Health Sciences, Hamilton, ON, Canada |
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| Abstract: | ![]()
Patients with cystic fibrosis (CF) have an increased nasal transepithelial potential difference (PD) which reflects increased sodium absorption across epithelium relatively impermeable to chloride. To evaluate nasal epithelial function in neonates with CF, the PD was recorded and the voltage response to superfusion of 10(-5M) amiloride, an inhibitor of sodium transport, measured between a Ringer perfused bridge on the nasal mucosa and a reference electrode in the subcutaneous space. We studied three neonates with CF with meconium ileus and compared the results with those in 24 term healthy neonates, including one obligate heterozygote for CF, and 27 control neonates with disease. All three CF neonates had raised sweat chloride values (mean 100 mEq/L) at 2 months. The CF neonates had higher PDs (-64.0 +/- 8.4 mV) than those in normal (-24.4 +/- 2.0 mV) or control (-25.8 +/- 2.0 mV) neonates. Superfusion with amiloride induced a 72% reduction in PD in the CF neonates as compared with healthy (37.5 +/- 1.0%) and diseased (36.0 +/- 1.3%) neonates. The PD and amiloride response in CF neonates are similar to those in CF infants (2.24 months), older CF children (greater than 6 years), and CF adults (-64.9 +/- 9.3 mV; 77.7 +/- 1.8%, n = 51). These results suggest that (1) nasal epithelial dysfunction is present in patients with CF shortly after birth, and (2) the nasal PD may be a diagnostic adjunct to the sweat test in the early diagnosis of CF. |
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