Abstract
Background: Nasal potential difference (NPD) measurement is an electrophysiological test that assesses cystic fibrosis transmembrane conductance regulator (CFTR) activity and is a recognized diagnostic tool in CF. The aim of this study is to assess in the long term the role of NPD in patients whose diagnosis is questionable. Methods: We performed follow up study on 70 patients with questionable CF (QCF) who were divided previously into two groups according to their NPD results: patients who likely have CF (QCF-CF) (n = 24), and those who likely do not have CF (QCF-non-CF) (n = 46). Results: Sixty out of 70 patients were available for study. Sixteen patients in the QCF-CF group were being followed up at CF Centers as opposed to 1 in the QCF-non-CF group (P < 0.01). Seven patients from the QCF-CF group developed sinusitis during the follow up years compared to none from the QCF-non-CF group. During the years of the follow up, 17 QCF-non-CF patients were diagnosed with other medical conditions that could explain their previous symptoms. On repeated NPD measurement in the QCF-CF group, the results were similar to the original test. Conclusions: This study supports the diagnostic role of NPD measurement. Larger cohort studies are required for confirmation.
Original language | English |
---|---|
Pages (from-to) | 545-549 |
Number of pages | 5 |
Journal | Pediatric Pulmonology |
Volume | 43 |
Issue number | 6 |
DOIs | |
State | Published - 1 Jun 2008 |
Externally published | Yes |
Keywords
- Long term follow-up
- Nasal potential difference
- Non-classic cystic fibrosis
- Questionable cystic fibrosis
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Pulmonary and Respiratory Medicine