TY - JOUR
T1 - Increased nasal potential difference and amiloride sensitivity in neonates with cystic fibrosis
AU - Gowen, C. W.
AU - Lawson, E. E.
AU - Gingras-Leatherman, J.
AU - Gatzy, J. T.
AU - Boucher, R. C.
AU - Knowles, M. R.
N1 - Funding Information:
Cystic fibrosis is an inherited disease that is manifest in many organ systems. The respiratory tract dysfunction appears to reflect abnormal airway secretions, which Supported by Grants HL00787, HL22924, and HDI9117, and Research Career Development Award 5-K04-HD00475-03 from the National Institutes of Health, and by RRDP Grant R003 6-03 from the Cystic Fibrosis Foundation. Presented in part at the Annual Meeting of the Society for Pediatric Research, May 1985. Submitted for publication Aug. 27, 1985; accepted Oct. 29, 1985. Reprint requests: Michael R. Knowles, M.D., Division of Pulmonary Diseases, 724 Burnett-Womack BuildingS'2 29H, University of North Carolina, Chapel Hill, NC 27514.
PY - 1986/4
Y1 - 1986/4
N2 - 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 (>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.
AB - 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 (>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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U2 - 10.1016/S0022-3476(86)80825-3
DO - 10.1016/S0022-3476(86)80825-3
M3 - Article
C2 - 3958823
AN - SCOPUS:0022638101
SN - 0022-3476
VL - 108
SP - 517
EP - 521
JO - The Journal of pediatrics
JF - The Journal of pediatrics
IS - 4
ER -