Intestinal current measurement versus nasal potential difference measurements for diagnosis of cystic fibrosis: a case–control study
نویسندگان
چکیده
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.
منابع مشابه
Intestinal current measurement versus nasal potential difference measurements for diagnosis
متن کامل
Measurement of nasal potential difference in adult cystic fibrosis, Young's syndrome, and bronchiectasis.
Previous work confirmed the abnormal potential difference between the undersurface of the inferior nasal turbinate and a reference electrode in cystic fibrosis, but the technique is difficult and the results show overlap between the cystic fibrosis and the control populations. In the present study the potential difference from the floor of the nose has therefore been assessed in normal subjects...
متن کاملPositive Expiratory Pressure (PEP) versus Conventional Chest Physiotherapy in Pediatric Patients with Acute Exacerbation of Cystic Fibrosis
Background Pulmonary involvement is the main cause of mortality in cystic fibrosis (CF). Airway clearance techniques are non-pharmacological complement options for CF patients. The aim of this study was to evaluate the short-term outcome of airway cleaning treatment in patients with cystic fibrosis in a children's hospital. Materials and Methods This clinical trial study conducted on 40 CF pati...
متن کاملUsing computer based data acquisition and analysis system for nasal potential difference measurement in cystic fibrosis.
Nasal potential difference (PD) measurement has been used as a diagnostic test for cystic fibrosis (CF). It has been shown that large differences in reproducibility of nasal PD measurement can exist between different study sites. These differences reduce the validity of studies. In our study we tried to measure nasal PD values for a group of Turkish CF patients by using a computer based data ac...
متن کاملCFTR biomarkers: time for promotion to surrogate end-point.
In patients with cystic fibrosis, cystic fibrosis transmembrane conductance regulator (CFTR) biomarkers, such as sweat chloride concentration and/or nasal potential difference, are used as end-points of efficacy in phase-III clinical trials with the disease modifying drugs ivacaftor (VX-770), VX809 and ataluren. The aim of this project was to review the literature on reliability, validity and r...
متن کامل