Nasal potential difference in non-classic cystic fibrosis - Long term follow up

Ranit Jaron, Yasmin Yaakov, Joseph Rivlin, Hannah Blau, Lea Bentur, Yakov Yahav, Eitan Kerem, Haim Bibi, Elie Picard, Michael Wilschanski

Research output: Contribution to journalArticlepeer-review

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 languageEnglish
Pages (from-to)545-549
Number of pages5
JournalPediatric Pulmonology
Volume43
Issue number6
DOIs
StatePublished - Jun 2008
Externally publishedYes

Keywords

  • Long term follow-up
  • Nasal potential difference
  • Non-classic cystic fibrosis
  • Questionable cystic fibrosis

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