非过敏性哮喘作为一种与囊性纤维化跨膜传导调节因子相关的疾病。
Non-allergic asthma as a CFTR-related disorder.
作者信息
Schulz Angela, Tümmler Burkhard
机构信息
Clinic for Paediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease (BREATH), German Center for Lung Research, Hannover, Germany.
出版信息
J Cyst Fibros. 2016 Sep;15(5):641-4. doi: 10.1016/j.jcf.2015.10.011. Epub 2015 Oct 31.
BACKGROUND
CFTR dysfunction can be involved in CBAVD, pancreatitis or bronchiectasis.
METHODS
Subjects with cystic fibrosis-like disease, equivocal sweat chloride concentrations and no or one disease-causing CFTR mutation were investigated by intestinal current and/or nasal potential difference measurements.
RESULTS
A subgroup of female patients who had been diagnosed to suffer from non-allergic asthma showed intermediary chloride concentrations in sweat test, normal chloride secretory responses in the intestine and an abnormal nasal potential difference with Sermet scores in the cystic fibrosis range.
CONCLUSION
Non-allergic asthma is a clinical entity that may be associated with CFTR dysfunction of the respiratory epithelium.
背景
囊性纤维化跨膜传导调节因子（CFTR）功能障碍可能与先天性双侧输精管缺如（CBAVD）、胰腺炎或支气管扩张有关。
方法
对患有囊性纤维化样疾病、汗液氯化物浓度不明确且无或仅有一个致病CFTR突变的受试者进行肠道电流和/或鼻电位差测量。
结果
一组被诊断患有非过敏性哮喘的女性患者汗液试验中氯化物浓度处于中间水平，肠道氯化物分泌反应正常，鼻电位差异常，塞梅特评分在囊性纤维化范围内。
结论
非过敏性哮喘是一种可能与呼吸道上皮CFTR功能障碍相关的临床实体。
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