摘要
囊性纤维化(CF)的新疗法,包括基因疗法,目前正在评估中。这些目的是纠正基本缺陷增加钠吸收和减少氯在气道上皮分泌。评估这些生物电参数,特别是在鼻上皮,很可能被用作治疗效果的一种措施。然而,区分囊性纤维化和非囊性纤维化的最佳体内方案尚不清楚。因此,我们比较了三种测量鼻腔上皮细胞囊性纤维化离子转运缺陷的方法。通过基线电位差和对钠通道阻滞剂阿米洛利的反应来测量钠吸收。在存在阿米洛利、灌注异丙肾上腺素、特布他林或低氯溶液后灌注异丙肾上腺素时评估氯离子分泌。基线电位差(PD)和对阿米洛利的绝对反应清楚地区分了囊性纤维化受试者钠吸收的增加。对特布他林(δ PD:非cf: -0.8 (SEM 0.7) mV;CF: -3.6 (0.5) mV)和异丙肾上腺素(非CF: 1.5 (0.6) mV; CF: -2.9 (0.6) mV) differentiated the two groups of subjects, but there was considerable overlap of values. Perfusion with a low chloride solution (non-CF: 12.6 (1.2) mV; CF: 0.6 (0.4) mV), as well as subsequent perfusion with isoprenaline (non-CF: 10.0 (1.1) mV; CF: -1.4 (0.4) mV) allowed clear separation of the two groups, with no overlap of values. Some CF subjects showed a transient hyperpolarization to these stimuli, which could clearly be differentiated from the sustained responses seen in non-cystic fibrosis subjects.(ABSTRACT TRUNCATED AT 250 WORDS)