Quinton P M
Nature. 1983 Feb 3;301(5899):421-2. doi: 10.1038/301421a0.
Cystic fibrosis is the most common fatal genetic disease affecting caucasians and is perhaps best characterized as an exocrinopathy involving a disturbance in fluid and electrolyte transport. A high NaCl concentration in the sweat is characteristic of patients with this disease; the basic physiological reason for this abnormality is unknown. We have microperfused isolated sweat ducts from control subjects and cystic fibrosis patients, and report here results which suggest that abnormally low Cl- permeability in cystic fibrosis leads to poor reabsorption of NaCl in the sweat duct, and hence to a high concentration of NaCl in the sweat.
囊性纤维化是影响白种人的最常见致命性遗传病，或许最典型的特征是一种涉及体液和电解质转运紊乱的外分泌腺病。汗液中高氯化钠浓度是该疾病患者的特征；这种异常的基本生理原因尚不清楚。我们对来自对照受试者和囊性纤维化患者的离体汗腺进行了微量灌注，并在此报告结果，这些结果表明囊性纤维化中异常低的氯离子通透性导致汗腺中氯化钠重吸收不良，进而导致汗液中氯化钠浓度升高。
