Basic protocol for transepithelial nasal potential difference measurements

Abstract

Transepithelial nasal potential difference (NPD) measurements assess ion conductance in the upper respiratory epithelium. NPD is useful in assisting in the diagnosis of classical and atypical cystic fibrosis (CF) and of cystic fibrosis transmembrane regulator (CFTR)-related disorders, as well as for monitoring the effect of pharmacological agents and gene transfer approaches to correct the abnormalities of ion transport in CF. The article summarizes the objectives and the principle of NPD measurements, describes a hands-on protocol of the procedure and provides quality control measures, practical hints and troubleshooting.

Cystic fibrosis, aminoglycoside treatment and acute renal failure: the not so gentle micin

“…Part of this improvement comes from the establishment of early and improved diagnostics [32,33] and the aggressive treatment of chronic lung infections, often using AGs. The study by Smyth et al highlights that this approach is not without risk.…”

Pancreatitis in Cystic Fibrosis and CFTR-Related Disorder

“…This test is based on the characteristic bioelectric properties of amiloridedependent sodium (Na + ) absorption (via ENaC) and CFTR-mediated Cl¯ diffusion (Schuler et al, 2004). NPD has been used in CF research for decades but due to a lack of validated reference values and standardisation, it has only been cautiously applied in clinical practice as an adjunctive test to assist in clarifying the diagnosis of CF, especially for individuals with intermediate sweat chloride values (Farrell et al, 2008).…”

Biochemical and Molecular Genetic Testing Used in the Diagnosis and Assessment of Cystic Fibrosis

“…There have been several reports on the usefulness of NPD measurements for diagnosing CF (Hubert et al, 2004;Schüler et al, 2004). Nasal PD measurement has been widely validated in adults (Knowles et al, 1995) and provides an easy, quick and painless tool to discriminate between adults with atypical CF and those presenting some CF symptoms without CF (Delmarco et al, 1997;Wilschanski et al, 2001).…”