经上皮鼻腔电位差测量的基本方案。

Basic protocol for transepithelial nasal potential difference measurements.

作者信息

Schüler Daniel, Sermet-Gaudelus Isabelle, Wilschanski Michael, Ballmann Manfred, Dechaux Michèle, Edelman Aleksander, Hug Martin, Leal Teresinha, Lebacq Jean, Lebecque Patrick, Lenoir Gérard, Stanke Frauke, Wallemacq Pierre, Tümmler Burkhard, Knowles Michael R

机构信息

CF Center, Children Hospital of Justus Liebig University, 35385 Giessen, Germany.

出版信息

J Cyst Fibros. 2004 Aug;3 Suppl 2:151-5. doi: 10.1016/j.jcf.2004.05.032.

DOI:10.1016/j.jcf.2004.05.032

PMID:15463949

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.

摘要

经上皮鼻腔电位差（NPD）测量可评估上呼吸道上皮中的离子传导。NPD有助于诊断典型和非典型囊性纤维化（CF）以及囊性纤维化跨膜传导调节因子（CFTR）相关疾病，还可用于监测药物和基因转移方法对纠正CF中离子转运异常的效果。本文总结了NPD测量的目的和原理，描述了该操作的实际步骤方案，并提供了质量控制措施、实用提示和故障排除方法。