Salinas Danieli Barino, Kang Lucia, Azen Colleen, Quinton Paul
Department of Pediatrics-Pediatric Pulmonology, Children's Hospital Los Angeles, Keck School of Medicine-University of Southern California (USC), Los Angeles, California.
Southern California Clinical and Translational Science Institute, Children's Hospital Los Angeles, University of Southern California, Los Angeles, California.
Pediatr Allergy Immunol Pulmonol. 2017 Mar 1;30(1):2-6. doi: 10.1089/ped.2016.0662.
β-adrenergically stimulated sweat secretion depends on the function of the cystic fibrosis transmembrane conductance regulator (CFTR) and discriminates between cystic fibrosis (CF) patients and healthy controls. Therefore, we sought to determine the feasibility, safety, and efficacy of assaying β-adrenergic sweating in children identified by CF newborn screening to help determine prognoses for individuals with CFTR-related metabolic syndrome (CRMS). Preschool age children with a positive newborn screening test for CF participated in this cross-sectional study. Sweat rates were measured by evaporimetery (cyberDERM, inc.) as transepidermal water losses (g HO/m/h) before and after selectively stimulating sweat glands either cholinergically or β-adrenergically. Net peak sweat responses assayed as evaporation rates were compared between CF and CRMS cohorts. After a pilot test in adults, children between 4 and 6 years of age were evaluated (CF, = 16; CRMS, = 10). The test protocol was well tolerated; electrocardiograms and vital signs were within normal range for all subjects. The mean evaporative sweat rates in both groups in response to cholinergic stimulation were similar (CF, 60.3 ± 23.8; CRMS, 57.7 ± 13.9; = 0.72) as well as to β-adrenergic stimulation (CF, 1.1 ± 1.7; CRMS, 2.0 ± 2.0; = 0.14). The β-adrenergic sweat test is safe and well tolerated by young children. However, the β-adrenergic sweat secretion rates as measured by evaporimetery did not discriminate between CF and CRMS cohorts.
β-肾上腺素能刺激的汗液分泌取决于囊性纤维化跨膜传导调节因子(CFTR)的功能,可区分囊性纤维化(CF)患者与健康对照者。因此,我们试图确定对通过CF新生儿筛查确诊的儿童进行β-肾上腺素能发汗检测的可行性、安全性和有效性,以帮助确定CFTR相关代谢综合征(CRMS)个体的预后。CF新生儿筛查呈阳性的学龄前儿童参与了这项横断面研究。通过蒸发计(cyberDERM公司)测量汗液速率,作为选择性地以胆碱能或β-肾上腺素能刺激汗腺前后的经皮水分流失(g HO/m/h)。比较CF和CRMS队列中作为蒸发速率测定的净峰值汗液反应。在对成人进行预试验后,对4至6岁的儿童进行了评估(CF组,n = 16;CRMS组,n = 10)。试验方案耐受性良好;所有受试者的心电图和生命体征均在正常范围内。两组对胆碱能刺激的平均蒸发汗液速率相似(CF组,60.3±23.8;CRMS组,57.7±13.9;P = 0.72),对β-肾上腺素能刺激的平均蒸发汗液速率也相似(CF组,1.1±1.7;CRMS组,2.0±2.0;P = 0.14)。β-肾上腺素能发汗试验对幼儿是安全的且耐受性良好。然而,通过蒸发计测量的β-肾上腺素能汗液分泌速率无法区分CF和CRMS队列。