Nasal potential difference to detect Na+ channel dysfunction in acute lung injury - PubMed (original) (raw)

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Nasal potential difference to detect Na+ channel dysfunction in acute lung injury

R Mac Sweeney et al. Am J Physiol Lung Cell Mol Physiol. 2011 Mar.

Abstract

Pulmonary fluid clearance is regulated by the active transport of Na(+) and Cl(-) through respiratory epithelial ion channels. Ion channel dysfunction contributes to the pathogenesis of various pulmonary fluid disorders including high-altitude pulmonary edema (HAPE) and neonatal respiratory distress syndrome (RDS). Nasal potential difference (NPD) measurement allows an in vivo investigation of the functionality of these channels. This technique has been used for the diagnosis of cystic fibrosis, the archetypal respiratory ion channel disorder, for over a quarter of a century. NPD measurements in HAPE and RDS suggest constitutive and acquired dysfunction of respiratory epithelial Na(+) channels. Acute lung injury (ALI) is characterized by pulmonary edema due to alveolar epithelial-interstitial-endothelial injury. NPD measurement may enable identification of critically ill ALI patients with a susceptible phenotype of dysfunctional respiratory Na(+) channels and allow targeted therapy toward Na(+) channel function.

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Figures

Fig. 1.

Fig. 1.

Schematic diagram of alveolar type 1 and 2 cells showing distribution of ion channels, pumps, aquaporins (AQP), and β-receptors (see text, under Mechanism of alveolar Na + transport and fluid absorption, for explanations of circled numbers in figure). HSC, highly selective cation channel; PSC, poorly selective cation channel; NSC, nonselective cation channel; CNG, cyclic nucleotide-gated channel; CFTR, cystic fibrosis transmembrane conductance regulator.

Fig. 2.

Fig. 2.

Measurement of nasal potential difference (NPD).

Fig. 3.

Fig. 3.

Typical NPD tracing from a healthy volunteer (see text for details). 1 = Perfusion with Ringers solution; 2 = perfusion with amiloride solution; vertical line indicates onset of amiloride perfusion.

Fig. 4.

Fig. 4.

Schematic representation of respiratory potential differences with associated height of respiratory liquid layers. Data adapted from Refs. , , , , .

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