CAMP STIMULATED SWEAT RATE IN CYSTIC FIBROSIS

该项目的目标是开发一种环状AMP(CAMP)- 刺激性“出汗率”测试，将完全区分 功能性、部分功能性和非功能性囊性病变 纤维化跨膜电导调节剂(CFTR)。出汗 可被胆碱能和肾上腺素能激动剂刺激。集合 胆碱能刺激性汗液&随后的汗液测量 胆碱浓度是标准诊断试验的基础。 囊性纤维化(CF)，匹罗卡品离子导入汗液试验。 毛果芸香碱刺激排汗导致初始 汗腺产生等渗性分泌物。在非CF中 病人，当分泌物穿过汗管时，氯化物是 重吸收导致汗液中的氯化物浓度较低 出现在皮肤上。这种氯离子再吸收依赖于 功能正常的CFTR的存在。在缺乏功能性CFTR的CF患者中， 汗液的氯化物浓度仍然很高，-最能区分，但 不是全部，而是来自非CF患者的CF。然而，这项技术不能 区分CFTR突变的杂合子携带者和非携带者， 汗液中氯化物的浓度也与疾病无关。 严肃性。

The goal of this project is the development of a cyclic-AMP(cAMP)- stimulated "sweat rate" test that will discriminate between fully functional, partially functional, and non-functional forms of the Cystic Fibrosis Transmembrane Conductance Regulator(CFTR). Sweat production can be stimulated by both cholinergic & adrenergic agonists. Collection of cholinergic stimulated sweat & the subsequent measurement of sweat cholride concentration is the basis of a standard diagnostic test for cystic fibrosis (CF), the pilocarpine iontophoresis sweat test. Stimulation of sweat production with pilocarpine leads to initial production of an isotonic secretion in the sweat gland. In non-CF patients, as the secretion traverses the sweat duct, chloride is reabsorbed leading to low concentrations of chloride in sweat as it appears on the skin. This chloride resorption is dependent on the presence of functional CFTR. In CF patients, who lack functional CFTR, the sweat chloride concentration remains high, & distinguishes most, but not all, CF from non-CF patients. However, this technique does not distinguish heterozygote carriers of CFTR mutations from non-carriers, nor does the sweat chloride concentration correlate with disease severity.