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

Bagheri-Hanson, Azadeh ; Nedwed, Sebastian ; Rueckes-Nilges, Claudia ; Naehrlich, Lutz

Originalveröffentlichung: (2014) BMC Pulmonary Medicine 14(1):156 doi:10.1186/1471-2466-14-156
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URN: urn:nbn:de:hebis:26-opus-114015

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Freie Schlagwörter (Englisch): cystic fibrosis , nasal potential difference , intestinal current measurement , sweat chloride , sweat test
Sammlung: Open Access - Publikationsfonds
Universität Justus-Liebig-Universität Gießen
Institut: Department of Pediatrics
Fachgebiet: Medizin
DDC-Sachgruppe: Medizin
Dokumentart: Aufsatz
Sprache: Englisch
Erstellungsjahr: 2014
Publikationsdatum: 25.03.2015
Kurzfassung auf Englisch: 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 >60mmol/l), including 6 pancreatic sufficient (PS) patients, and 13 healthy controls, including 8 smokers. The NPD CFTR response to Cl-free and isoproterenol perfusion (Delta0Cl-+Iso) was compared to the ICM CFTR response to forskolin/IBMX, carbachol, and histamine (DeltaIsc, 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>60mmol/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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