Биоэлектрические свойства эпителия дыхательных путей у больных муковисцидозом

Cystic fibrosis (OF) is characterized by disorders of chloride secretion and sodium absorption in exocrine epithelium. A crucial location of these ion disorders is the respiratory epithelium. Such ion pathology forms a transepithelial electric potential difference. It is hard to measure tracheobronchial electric potential difference, so a method for measuring nasal potential difference (NPD) was created. We measured baseline values of NPD in 100 patients (including 45 OF patients) and in 15 healthy volunteers. More significant negative values of the average baseline NPD were registered in the OF patients (42.2±1.4 mV) compared with healthy and COPD persons (-18.3±1.8 and 19.2±0.6 mV accordingly, p <0.0001). NPD values in 6 (13%) OF patients with typical clinical features, normal or boundary sweat test results and CF gene confirmation were compatible with CF bioelectric profile. Meantime 3 COPD patients had increased sweatiest results and a low NPD level. Under amiloride hydrochloride blocking sodium channels the basal NPD was inhibited greatly (up to 66%) in CF patients, whereas the same value in COPD patients was 36.7%.Therefore, the NPD reflects the principal CF disorder. Its increase under the amiloride influence more than 60% is thought to be used as an additional diagnostic test.