Suggestion of an Abnormal Anion Exchange Mechanism in Sweat Glands of Cystic Fibrosis Patients
Suggestion of an Abnormal Anion Exchange Mechanism in Sweat Glands of Cystic Fibrosis Patients
复制标题
囊性纤维化患者汗腺阴离子交换机制异常的提示
作者：
P. Quinton
总结：囊性纤维化（CF）汗腺中NaCl重吸收缺陷的生理基础尚不清楚。我们系统地跟踪了CF患者和正常人汗液中三种主要无机电解质的浓度与单腺体出汗率的关系。结果表明，（1）在低出汗率下，CF个体的汗腺可以将NaCl浓度降低到正常水平，以及（2）CF汗液中有机阴离子和/或HCO 3的浓度显著低于正常汗液。目前的数据和其他研究结果表明，Na/K转运和假设的Na/H交换是正常的，但假定的阴离子交换可能在囊性纤维化汗腺中是有缺陷的。这些观察结果表明，潜在的运输遗传异常，这可能会解释观察到的囊性纤维化外分泌腺分泌异常一般。
Summary: The physiological basis for the defect in NaCl reabsorption in cystic fibrosis (CF) sweat glands is not known. We have systematically followed the concentrations of the three major inorganic electrolytes in sweat as a function of single gland sweat rate in patients with CF and normal individuals. The result demonstrates that (1) at low sweat rates, sweat glands of CF individuals can reduce NaCl concentrations to normal levels and (2) that the concentration of organic anions and/or HCO3 in CF sweat is substantially less than in normal sweat.Speculation: Present data and other findings suggest that Na/K transport and a postulated Na/H exchange are normal, but that a postulated anion exchange may be defective in cystic fibrosis sweat glands. These observations suggest an underlying transport genetic abnormality, which might explain observed abnormalities in cystic fibrosis exocrine gland secretions in general.