Quinton P M, Bijman J
N Engl J Med. 1983 May 19;308(20):1185-9. doi: 10.1056/NEJM198305193082002.
Patients with cystic fibrosis have characteristic disturbances in the electrolyte composition of their sweat, saliva, and pancreatic secretions. We studied the electrical properties of sweat glands in eight patients with cystic fibrosis and in seven normal volunteers to determine the basis of the well-documented inhibition of sodium absorption in this disease. The average electrical potential across 47 sweat glands in the patients was -66.3 +/- 2.1 mV, as compared with -29.8 +/- 3.2 mV for 39 glands in the normal controls (P less than 0.001). The average sweat-secretion rate in 33 glands from six patients was not significantly different from that in 34 glands from six controls, but average concentrations of sodium, chloride, and potassium were significantly higher in sweat droplets from the patients. Calculated rates of both sodium and chloride reabsorption were lower in sweat glands of patients than of normal controls, but chloride reabsorption was more markedly reduced than sodium reabsorption. We conclude that a decrease in epithelial permeability to chloride may explain the characteristic changes in sweat electrolytes in cystic fibrosis and could be a generalized abnormality in the disease.
囊性纤维化患者的汗液、唾液和胰腺分泌物的电解质组成存在特征性紊乱。我们研究了8例囊性纤维化患者和7名正常志愿者汗腺的电学特性,以确定该疾病中钠吸收受到充分记录的抑制的基础。患者47个汗腺的平均跨膜电位为-66.3±2.1 mV,而正常对照组39个汗腺的平均跨膜电位为-29.8±3.2 mV(P<0.001)。6例患者33个汗腺的平均汗液分泌率与6名对照者34个汗腺的平均汗液分泌率无显著差异,但患者汗液滴中钠、氯和钾的平均浓度显著更高。患者汗腺中钠和氯的重吸收率均低于正常对照组,但氯的重吸收比钠的重吸收减少得更明显。我们得出结论,上皮对氯的通透性降低可能解释了囊性纤维化患者汗液电解质的特征性变化,并且可能是该疾病的一种普遍异常。