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- W2010873479 abstract "Introduction: In atypical patients without characteristic clinical features of CF, our clinic operates the nasal potential difference (NPD) and intestinal current measurement(ICM). In previous studies, we found that patients with abnormal intestinal chloride transport as identified by ICM could show normal (<30 mmol/L), borderline (30–60 mmol/L) or positive (>60 mmol/L) sweat chloride levels. The goal of this study was to compare outcome of NPD and ICM, in patients with and without conclusive CFTR mutation analysis. Methods: The NPD determines sodium and chloride conductances in respiratory epithelium by superfusing the inferior nasal turbinate with a saline solution. The baseline PD is more negative in CF patients than in non CF individuals. For ICM a rectal suction biopsy is mounted in an Ussing chamber. Short circuit chloride currents are recorded in response to secretagogues and inhibitors of chloride channels. Hence, ICM is able to distinguish normal intestinal chloride transport in controls from anomalous chloride transport in CF patients Results: We tested a group of 43 patients suspected for CF by clinical symptoms. In NPD measurements 19 out of 43 exhibited a nasal basal PD >35 mV. The intestinal carbachol response of 9 of these 19 patients was <10 microA/cm2 suggestive for CF. Sweat chloride levels in these 9 patients ranged from 64 to 106 mmol/L. The 10 other patients showing a nasal basal PD >35 mV displayed intestinal carbachol responses >10 microA/cm2 indicating normal CFTR function in their intestinal tissues. Sweat Cl levels in this group were between 22 and 52 mmol/L. CFTR mutation analysis discovered 16 CF alleles in the first group, while in the second group only one deltaF508, two d 110h and one R 117H mutation were detected in 20 alleles. Twenty four patients showed intestinal carbachol responses >10 microA/cm2 with nasal baseline PDs <35 mV, indicating normal CFTR function in intestinal and nasal epithelia. Conclusion: Patients identified with CF like basal PDs > 35 mV in NPD could be divided by ICM in patients with high and low intestinal chloride transport. Diminished intestinal chloride transport correlated with a high incidence of CFTR mutations. In the group of 9 patients with a carbochol response <10 micro A/cm2, CFTR mutation analysis identified CF in 80% (7/9) by lesions in both CFTR alleles. CFTR mutation analysis was not able to confirm CF diagnosis in any of the 10 patients with nasal basal PD >35 mV and intestinal carbachol responses >10 microA/cm2. This, suggests that ICM can be implemented in the CF diagnostic process as an effective method to distinguish CF from non CF when other electrophysiological tests, like NPD and the sweat test, are unsuccessful to complete, the diagnosis." @default.
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- W2010873479 date "2004-06-01" @default.
- W2010873479 modified "2023-09-24" @default.
- W2010873479 title "P0289 NASAL POTENTIAL DIFFERENCE VERSUS INTESTINAL CURRENT MEASUREMENTS IN CF DIAGNOSIS" @default.
- W2010873479 doi "https://doi.org/10.1097/00005176-200406001-00413" @default.
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