App E M, King M, Helfesrieder R, Köhler D, Matthys H
Pulmonary Defense Group, University of Alberta, Edmonton, Canada.
Am Rev Respir Dis. 1990 Mar;141(3):605-12. doi: 10.1164/ajrccm/141.3.605.
Cystic fibrosis (CF) is the most common inherited fatal disorder among Caucasians. Bronchial mucus in CF contains more potassium and less sodium, which may be due to increased sodium absorption, resulting in a reduced airway water content. We studied 23 patients with CF after inhalation of normal saline or amiloride (10(-3) M), a sodium transport blocker. Mucociliary clearance (MC) and cough clearance (CC) were determined with a gamma camera that traced the movement of 99mTc-labeled, hardened erythrocytes over a 1-h period after the patients inhaled these particles as an aerosol. Before and after each investigation pulmonary function tests (PFT) and blood pressure (BP) were measured. Sputum thread formation was measured by means of a filancemeter. Six of the patients also completed a 3-wk trial of amiloride inhalation therapy. MC increased significantly (p less than 0.001) after acute amiloride inhalation (bronchial deposition, 0.07 mg amiloride) compared with that in the saline control. CC also increased, but not as much as MC. After 3 wk of amiloride inhalation (2 times a day) clearance values (both MC and CC) were markedly enhanced (p less than 0.01); after a similar period of saline inhalation, clearance values were not different from baseline. Sputum filance values also decreased significantly after amiloride inhalation. There were no adverse effects of the amiloride inhalation compared with saline. We conclude that amiloride inhalation administered as a single dose or as long-term therapy is able to increase MC and CC in CF airways and that the effect of 10(-3) M amiloride inhalation on MC lasts at least 40 min. (ABSTRACT TRUNCATED AT 250 WORDS)
囊性纤维化（CF）是白种人中最常见的遗传性致命疾病。CF患者的支气管黏液含钾量更高而含钠量更低，这可能是由于钠吸收增加，导致气道含水量减少。我们对23例CF患者吸入生理盐水或氨氯吡咪（10⁻³ M）（一种钠转运阻滞剂）后的情况进行了研究。通过γ相机测定黏液纤毛清除率（MC）和咳嗽清除率（CC），该相机追踪患者吸入作为气雾剂的99mTc标记的硬化红细胞在1小时内的移动情况。每次检查前后均测量肺功能测试（PFT）和血压（BP）。通过细丝测量仪测量痰液细丝形成情况。其中6例患者还完成了为期3周的氨氯吡咪吸入治疗试验。与生理盐水对照组相比，急性吸入氨氯吡咪（支气管沉积量为0.07 mg氨氯吡咪）后MC显著增加（p＜0.001）。CC也增加了，但增幅不如MC。吸入氨氯吡咪3周（每日2次）后，清除率值（MC和CC）均显著提高（p＜0.01）；吸入生理盐水相似时间段后，清除率值与基线无差异。吸入氨氯吡咪后痰液细丝值也显著降低。与生理盐水相比，氨氯吡咪吸入无不良反应。我们得出结论，单剂量或长期吸入氨氯吡咪能够增加CF气道的MC和CC，且吸入10⁻³ M氨氯吡咪对MC的作用至少持续40分钟。（摘要截选至250字）
