Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium

摘要: New treatments for cystic fibrosis (CF), including gene therapy, are currently being assessed. These aim to correct the basic defects of increased sodium absorption and decreased chloride secretion in airway epithelia. Assessment these bioelectric parameters, particularly nasal epithelium, is likely be used as a measure treatment efficacy. However, optimal vivo protocol discriminate from non-cystic subjects unclear. We have, therefore, compared three protocols measurement ion transport epithelium. Sodium was measured using both baseline potential difference response channel blocker, amiloride. Chloride assessed presence amiloride, perfusion with isoprenaline, or terbutaline, low solution followed by isoprenaline. Baseline (PD) absolute amiloride clearly differentiated subjects. The responses terbutaline (delta PD: non-CF: -0.8 (SEM 0.7) mV; CF: -3.6 (0.5) mV) isoprenaline (non-CF: 1.5 (0.6) -2.9 two groups subjects, but there considerable overlap values. Perfusion 12.6 (1.2) 0.6 (0.4) mV), well subsequent 10.0 (1.1) -1.4 allowed clear separation groups, no Some CF showed transient hyperpolarization stimuli, which could sustained seen subjects.(ABSTRACT TRUNCATED AT 250 WORDS)