Department of Medicine, Section of General Pathology, University of Verona, Verona, Italy.
Cystic Fibrosis Centre, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy.
J Cyst Fibros. 2018 Mar;17(2):186-189. doi: 10.1016/j.jcf.2017.12.003. Epub 2017 Dec 29.
We have simplified the published procedure (5) for measuring sweat rates in individual human sweat glands. Sweat secretion rates were obtained from sweat drops secreted on the forearm by multiple individual glands. We computed a ratio between CFTR-dependent (by intradermal microinjection of a β adrenergic cocktail) and CFTR-independent (by methacoline as cholinergic stimulus) sweat secretion rates. We obtained a reproducible, approximately linear readout of CFTR function with measurements performed by two different independent teams. We considered three groups (CF subjects, CF carriers and non-CF controls, n=22 in each group); their mean ratios was respectively 0.000, 0.104 and 0.205 The average ratio of CF subjects was consistent with diagnosis in 3 additional cases clinically resembling CF. All groups were clearly discriminated, with sensibility and specificity ranging from 82% to 100%. A software was developed for detecting sweat droplets. This bioassay is suitabile for multicentre studies focusing on CFTR targeted therapies, controversial diagnosis and functional relevance of rare CFTR mutations.
我们简化了已发表的个体人汗腺汗液速率测量程序（5）。汗液分泌速率是通过前臂多个单个汗腺分泌的汗液滴获得的。我们计算了 CFTR 依赖性（通过皮内注射β肾上腺素鸡尾酒）和 CFTR 非依赖性（通过烟碱作为胆碱能刺激）汗液分泌速率之间的比率。我们通过两个不同的独立团队进行的测量获得了 CFTR 功能的可重复、近似线性的读数。我们考虑了三组（CF 患者、CF 携带者和非 CF 对照，每组 22 人）；他们的平均比率分别为 0.000、0.104 和 0.205。CF 患者的平均比率与另外 3 例临床类似于 CF 的病例的诊断一致。所有组均明显区分，敏感性和特异性范围为 82%至 100%。我们开发了一种用于检测汗液滴的软件。这种生物测定法适用于专注于 CFTR 靶向治疗、有争议的诊断和罕见 CFTR 突变的功能相关性的多中心研究。
