Galey W R, Gillon M J, Murphy S
Pediatr Res. 1980 Nov;14(11):1269-71. doi: 10.1203/00006450-198011000-00023.
The abnormally high concentrations of electrolytes and organic solutes found in the glandular secretions of individuals with cystic fibrosis may arise from decreased water content rather than perturbed solute transport. Thus, it is possible that the abnormalities insolute concentrations are a result of a reduced water movement into the secretory product. It is our hypothesis that if an abnormality exists in the transport of water across secretory cell membranes, this defect may also be expressed in nonsecretory cell membrane permeability to water. We report here osmotic water permeability measurements on red cells and 11 cystic fibrosis patients ranging in age from 2 to 20 years and eight normal individuals. The mean hydraulic conductivity for the patients of 1.23 +/- 0.13 cm3/dyne sec is significantly less (P < 0.001) than that of normal individuals who have permeabilities of 1.45 +/- 0.11 cm3/dyne sec. Analysis showed that the osmotic water permeability correlates well with the Shwachman score (r = 0.76). Patients who are in the poorest clinical condition show the lowest hydraulic conductivities.
在囊性纤维化患者的腺分泌物中发现的电解质和有机溶质异常高浓度，可能源于水含量降低而非溶质转运紊乱。因此，溶质浓度异常可能是水进入分泌产物的运动减少的结果。我们的假设是，如果跨分泌细胞膜的水转运存在异常，这种缺陷也可能表现在非分泌细胞膜对水的通透性上。我们在此报告了对2至20岁的11名囊性纤维化患者和8名正常个体的红细胞进行的渗透水通透性测量。患者的平均水力传导率为1.23±0.13 cm³/达因·秒，显著低于正常个体的1.45±0.11 cm³/达因·秒（P<0.001）。分析表明，渗透水通透性与施瓦赫曼评分密切相关（r = 0.76）。临床状况最差的患者显示出最低的水力传导率。
