Alton E W, Rogers D F, Logan-Sinclair R, Yacoub M, Barnes P J, Geddes D M
Department of Thoracic Medicine, National Heart and Lung Institute, London.
Thorax. 1992 Dec;47(12):1010-4. doi: 10.1136/thx.47.12.1010.
The basic defect in cystic fibrosis centres on abnormal ion transport in affected tissues such as the respiratory tract. Heart-lung transplantation provides a limited supply of native lower airways from these patients. The feasibility of in vitro studies of bioelectric properties and ion fluxes in lower airways, obtained at heart-lung transplantation from patients with cystic fibrosis, has been assessed. Comparison was made with airways from patients without cystic fibrosis.
Tissue segments were mounted in Ussing chambers under open circuit conditions. The basal potential difference in tissues from nine patients with cystic fibrosis was -3.6 mV (SE 0.3 mV), not different from tissues from 12 patients without cystic fibrosis of -3.6 mV (0.5 mV). Amiloride (10 microM) caused a significantly greater fall in potential difference in bronchi from patients with cystic fibrosis (83.5% (SE 2.9%)) than in those from controls (55.1% (7.1%)). Isoprenaline (100 microM) produced no significant change in bioelectric properties in non-cystic fibrosis tissues, but induced a 26.2% (6.3%) increase in potential difference in cystic fibrosis airways. The latter response was reduced by amiloride pretreatment. Mucosal chloride substituted Krebs-Henseleit solution caused no change in bioelectric properties in cystic fibrosis airways. Sodium substituted Krebs solution produced a substantial fall in potential difference similar in magnitude to that seen after amiloride. Isotropic flux measurements showed no significant differences between non-cystic fibrosis and cystic fibrosis airways. No net movement of Na+ or Cl- was detected under open circuit conditions in either group.
Cystic fibrosis bronchi obtained at heart-lung transplantation provide a viable source of tissue for in vitro studies of bioelectric properties. The increased response to amiloride characteristic of the upper airways in cystic fibrosis is retained in these tissues, as is the reduced chloride conductance. Although no differences in isotopic fluxes were seen between non-cystic fibrosis and cystic fibrosis tissues, heavily infected airways from patients with cystic fibrosis may not be suitable for ion flux measurements.
囊性纤维化的基本缺陷集中在诸如呼吸道等受影响组织中的异常离子转运。心肺移植提供的来自这些患者的天然下呼吸道数量有限。已评估了对来自囊性纤维化患者心肺移植时获取的下呼吸道生物电特性和离子通量进行体外研究的可行性。并与无囊性纤维化患者的气道进行了比较。
将组织片段置于开路条件下的尤斯灌流小室中。9例囊性纤维化患者组织的基础电位差为-3.6 mV（标准误0.3 mV），与12例无囊性纤维化患者组织的-3.6 mV（0.5 mV）无差异。氨氯吡脒（10 μM）使囊性纤维化患者支气管的电位差下降幅度（83.5%（标准误2.9%））显著大于对照组（55.1%（7.1%））。异丙肾上腺素（100 μM）对非囊性纤维化组织的生物电特性无显著影响，但使囊性纤维化气道的电位差增加了26.2%（6.3%）。氨氯吡脒预处理可降低后一种反应。用氯化物替代Krebs-Henseleit溶液的黏膜溶液对囊性纤维化气道的生物电特性无影响。用钠替代Krebs溶液使电位差大幅下降，幅度与氨氯吡脒处理后相似。同位素通量测量显示非囊性纤维化和囊性纤维化气道之间无显著差异。在开路条件下，两组均未检测到Na⁺或Cl⁻的净移动。
心肺移植时获取的囊性纤维化支气管为生物电特性的体外研究提供了可行的组织来源。这些组织保留了囊性纤维化上呼吸道对氨氯吡脒反应增加的特征，以及降低的氯电导。尽管在非囊性纤维化和囊性纤维化组织之间未观察到同位素通量的差异，但来自囊性纤维化患者严重感染的气道可能不适合进行离子通量测量。
