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

Bagheri-Hanson A, Nedwed S, Rueckes-Nilges C, Naehrlich L - BMC Pulm Med (2014)

Bottom Line: 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).Smokers have a decreased CFTR response measured by NPD (p = 0.049).For ICM there is a trend towards decreased CFTR response (NS).

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Justus-Liebig-University Giessen, Feulgenstrasse 12, 35385 Giessen, Germany. lutz.naehrlich@paediat.med.uni-giessen.de.

ABSTRACT

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 (ΔIsc, 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.

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Related in: MedlinePlus

Average Δ0Cl- + Iso (NPD) in healthy controls according to smoking status. The normal range is shown below the dotted line and the intermediate range is shown between the solid and dotted lines.
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Fig5: Average Δ0Cl- + Iso (NPD) in healthy controls according to smoking status. The normal range is shown below the dotted line and the intermediate range is shown between the solid and dotted lines.

Mentions: In healthy controls, smoking had no influence on sweat chloride (NS), but decreased CFTR function as measured by NPD (p = 0.049) (Table 2 and Figure 5), and resulted in intermediate or even abnormal NPD results, but not ICM or sweat chloride results. There is a trend in ICM measurements indicative that smoking might not only affect CFTR in the respiratory tract, but also in the intestine (Table 2 and Figure 6).Table 2


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

Bagheri-Hanson A, Nedwed S, Rueckes-Nilges C, Naehrlich L - BMC Pulm Med (2014)

Average Δ0Cl- + Iso (NPD) in healthy controls according to smoking status. The normal range is shown below the dotted line and the intermediate range is shown between the solid and dotted lines.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4199064&req=5

Fig5: Average Δ0Cl- + Iso (NPD) in healthy controls according to smoking status. The normal range is shown below the dotted line and the intermediate range is shown between the solid and dotted lines.
Mentions: In healthy controls, smoking had no influence on sweat chloride (NS), but decreased CFTR function as measured by NPD (p = 0.049) (Table 2 and Figure 5), and resulted in intermediate or even abnormal NPD results, but not ICM or sweat chloride results. There is a trend in ICM measurements indicative that smoking might not only affect CFTR in the respiratory tract, but also in the intestine (Table 2 and Figure 6).Table 2

Bottom Line: 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).Smokers have a decreased CFTR response measured by NPD (p = 0.049).For ICM there is a trend towards decreased CFTR response (NS).

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Justus-Liebig-University Giessen, Feulgenstrasse 12, 35385 Giessen, Germany. lutz.naehrlich@paediat.med.uni-giessen.de.

ABSTRACT

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 (ΔIsc, 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.

Show MeSH
Related in: MedlinePlus