Bagheri-Hanson Azadeh, Nedwed Sebastian, Rueckes-Nilges Claudia, Naehrlich Lutz
Department of Pediatrics, Justus-Liebig-University Giessen, Feulgenstrasse 12, 35385 Giessen, Germany.
BMC Pulm Med. 2014 Oct 4;14:156. doi: 10.1186/1471-2466-14-156.
Nasal potential difference (NPD) and intestinal current measurement (ICM) are functional CFTR tests that are used as adjunctive diagnostic tools for cystic fibrosis (CF). Smoking has a systemic negative impact on CFTR function. A diagnostic comparison between NPD and ICM and the impact of smoking on both CFTR tests has not been done.
The sweat chloride test, NPD, and ICM were performed in 18 patients with CF (sweat chloride >60 mmol/l), including 6 pancreatic sufficient (PS) patients, and 13 healthy controls, including 8 smokers. The NPD CFTR response to Cl-free and isoproterenol perfusion (Δ0Cl- + Iso) was compared to the ICM CFTR response to forskolin/IBMX, carbachol, and histamine (ΔIsc, forskolin/IBMX+ carbachol+histamine).
The mean NPD CFTR response and ICM CFTR response between patients with CF and healthy controls was significantly different (p <0.001), but not between patients with CF who were PS and those who were pancreatic insufficient (PI). Smokers have a decreased CFTR response measured by NPD (p = 0.049). For ICM there is a trend towards decreased CFTR response (NS). Three healthy control smokers had NPD responses within the CF-range. In contrast to NPD, there was no overlap of the ICM response between patients with CF and controls.
ICM is superior to NPD in distinguishing between patients with CF who have a sweat chloride > 60 mmol/l and healthy controls, including smokers. Neither NPD nor ICM differentiated between patients with CF who were PS from those who were PI. Smoking has a negative impact on CFTR function in healthy controls measured by NPD and challenges the diagnostic interpretation of NPD, but not ICM.
鼻电位差（NPD）和肠道电流测量（ICM）是用于囊性纤维化（CF）辅助诊断的功能性CFTR检测方法。吸烟对CFTR功能有全身性负面影响。尚未对NPD和ICM进行诊断比较，也未研究吸烟对这两种CFTR检测的影响。
对18例CF患者（汗液氯化物>60 mmol/l）进行汗液氯化物检测、NPD和ICM，其中包括6例胰腺功能正常（PS）的患者，以及13例健康对照者，包括8名吸烟者。将NPD中CFTR对无氯和异丙肾上腺素灌注的反应（Δ0Cl- + Iso）与ICM中CFTR对福司可林/异丁基甲基黄嘌呤、卡巴胆碱和组胺的反应（ΔIsc福司可林/异丁基甲基黄嘌呤+卡巴胆碱+组胺）进行比较。
CF患者与健康对照者之间的平均NPD CFTR反应和ICM CFTR反应存在显著差异（p<0.001），但PS的CF患者与胰腺功能不全（PI）的CF患者之间无显著差异。吸烟者通过NPD测量的CFTR反应降低（p = 0.049）。对于ICM，CFTR反应有降低趋势（无统计学意义）。3名健康对照吸烟者的NPD反应在CF范围内。与NPD不同，CF患者与对照者之间的ICM反应没有重叠。
在区分汗液氯化物>60 mmol/l的CF患者与包括吸烟者在内的健康对照者方面，ICM优于NPD。NPD和ICM均无法区分PS的CF患者与PI的CF患者。吸烟对通过NPD测量的健康对照者的CFTR功能有负面影响，并对NPD的诊断解释提出挑战，但对ICM没有影响。
