Intestinal current measurement versus nasal potential difference measurements for diagnosis of cystic fibrosis: a case–control study

BMC Pulmonary Medicine

Lutz Naehrlich¹  Claudia Rueckes-Nilges¹  Sebastian Nedwed¹  Azadeh Bagheri-Hanson¹ 

[1] Department of Pediatrics, Justus-Liebig-University Giessen, Feulgenstrasse 12, 35385 Giessen, Germany

关键词: Smoking;    Diagnosis;    Sweat test;    Sweat chloride;    Intestinal current measurement;    Nasal potential difference;    (3–10): Cystic fibrosis;   

Others  :  1091966
DOI  :  10.1186/1471-2466-14-156

 received in 2014-06-18, accepted in 2014-09-29,  发布年份 2014

【 摘 要 】

Background

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.

Methods

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 (ΔI_{sc, forskolin/IBMX+ carbachol+histamine}).

Results

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.

Conclusions

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.

【 授权许可】

   
2014 Bagheri-Hanson et al.; licensee BioMed Central Ltd.

【 图 表 】

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