Basic protocol for transepithelial nasal potential difference measurements.

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.

• Publication year

  2004

• Venue

  Journal of Cystic Fibrosis

• Publication date

  2004-08-01

• Fields of study

  Medicine

• Identifiers
  DOI 10.1016/J.JCF.2004.05.032PMID 15463949
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Standardized procedure for measurement of nasal potential difference: An outcome measure in multicenter cystic fibrosis clinical trials

  2004cited by this paper
• Gentamicin-induced correction of CFTR function in patients with cystic fibrosis and CFTR stop mutations.

  2003cited by this paper
• A multicenter study of the effect of solution temperature on nasal potential difference measurements.

  2003cited by this paper
• Modified Method to Measure Nasal Potential Difference

  2003cited by this paper
• Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis * *

  2002cited by this paper
• Conventional and modified nasal potential-difference measurement in cystic fibrosis.

  1997cited by this paper
• In Vivo Nasal Potential Difference: Techniques and Protocols for Assessing Efficacy of Gene Transfer in Cystic Fibrosis

  1995cited by this paper
• Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium.

  1994cited by this paper
• Nasal potential difference: a clinical diagnostic test for cystic fibrosis.

  1990cited by this paper
• Measurements of nasal transepithelial electric potential differences in normal human subjects in vivo.

  1981cited by this paper

• Peptide CIGB‐552 has a synergistic effect on CFTR‐F508del when combined with elexacaftor/tezacaftor/ivacaftor triple therapy for cystic fibrosis

  2025cites this paper
• Progress of personalized medicine of cystic fibrosis in the times of efficient CFTR modulators

  2025cites this paper
• Enhancement of chloride transport via Ca2+ mobilization in airway epithelial cells by DS-1039, a novel selective GPR39 agonist.

  2025cites this paper
• Assessing accuracy of testing and diagnosis in cystic fibrosis

  2023cites this paper
• Assays of CFTR Function In Vitro, Ex Vivo and In Vivo

  2022cites this paper
• Cystic fibrosis transmembrane conductance regulator function in patients with chronic pancreatitis

  2022cites this paper
• Towards harmonization of solutions used for cystic fibrosis diagnosis by nasal potential difference measurements: a formulation approach with CHESS® software.

  2021cites this paper
• Optical Nanosensors for in vivo Physiological Chloride Detection for Monitoring Cystic Fibrosis Treatment.

  2020cites this paper
• Diagnosis and Presentation of Cystic Fibrosis

  2019cites this paper
• EXPERIENCE OF EVALUATING FUNCTIONALITY OF ANIONIC CFTR CHANNEL METHODS APPLICATION IN PATIENTS WITH CYSTIC FIBROSIS DIAGNOSED AND SUPPOSED

  2019cites this paper
• Insights into the variability of nasal potential difference, a biomarker of CFTR activity.

  2019cites this paper
• Salivary Thiocyanate as a Biomarker of Cystic Fibrosis Transmembrane Regulator Function.

  2019cites this paper
• Diagnostic evaluation of bronchiectasis

  2019cites this paper
• Using a systems approach to identify the mechanism of action of correctors

  2018cites this paper
• Physiological study of pulmonary involvement in adults with cystic fibrosis through simulated modeling of different clinical scenarios

  2018cites this paper
• Diagnosis and Treatment of Cystic Fibrosis: A (Not-so) Simple Recessive Condition

  2017cites this paper
• A multiple reader scoring system for Nasal Potential Difference parameters.

  2017cites this paper
• A semi-blinded study comparing 2 methods of measuring nasal potential difference: Subcutaneous needle versus dermal abrasion.

  2016cites this paper
• IN SILICO PREDICTION OF DELETERIOUS AND NON-DELETERIOUS nsSNPs IN CFTR GENE VARIANTS

  2016cites this paper
• Combination antimicrobial susceptibility testing of Burkholderia cepacia complex: significance of species.

  2016cites this paper
• Systematic review and meta-analysis of nasal potential difference in hypoxia-induced lung injury

  2016influential citation
• Nasal Potential Difference in Cystic Fibrosis considering Severe CFTR Mutations

  2015cites this paper
• Nasal potential difference measurements in diagnosis of cystic fibrosis: an international survey.

  2014cites this paper
• Multicenter Intestinal Current Measurements in Rectal Biopsies from CF and Non-CF Subjects to Monitor CFTR Function

  2013cites this paper
• Misdiagnosis of cystic fibrosis: experience from Germany.

  2013cites this paper
• S2-Konsensus-Leitlinie „Diagnose der Mukoviszidose“

  2013cites this paper
• The CF-modifying gene EHF promotes p.Phe508del-CFTR residual function by altering protein glycosylation and trafficking in epithelial cells

  2013cites this paper
• From pipeline to patient: new developments in cystic fibrosis therapeutics

  2013cites this paper
• Pancreatitis in Cystic Fibrosis and CFTR-Related Disorder

  2012cites this paper
• Recovery of ΔF508‐CFTR function by analogs of hyaluronan disaccharide

  2012cites this paper
• La protéine CFTR : Implication et cible thérapeutique dans la maladie osseuse chez les patients atteints de mucoviscidose.

  2012cites this paper
• Der Effekt von intravenösem Salbutamol auf das elektrische Potential der Nasenschleimhaut von Frühgeborenen

  2012cites this paper
• Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy.

  2012cites this paper
• Der Effekt von intravenösem Salbutamol auf das elektrische Potential der Nasenschleimhaut von Frühgeborenen

  2012influential citation
• Combination Antimicrobial Susceptibility Testing of Multidrug-Resistant Stenotrophomonas maltophilia from Cystic Fibrosis Patients

  2012cites this paper
• Colistin: an update on the antibiotic of the 21st century

  2012cites this paper
• CLCA4 variants determine the manifestation of the cystic fibrosis basic defect in the intestine

  2012cites this paper
• An association study on contrasting cystic fibrosis endophenotypes recognizes KRT8 but not KRT18 as a modifier of cystic fibrosis disease severity and CFTR mediated residual chloride secretion

  2011cites this paper
• Nasal potential measurements on the nasal floor and under the inferior turbinate: Does it matter?

  2011cites this paper
• Studies as a basis for a possible introduction of newborn screening for cystic fibrosis in Sweden

  2011cites this paper
• Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis

  2011cites this paper
• Comparable Population Pharmacokinetics and Pharmacodynamic Breakpoints of Cefpirome in Cystic Fibrosis Patients and Healthy Volunteers

  2011cites this paper
• STUDIES AS A BASIS FOR A POSSIBLE INTRODUCTION OF NEWBORN SCREENING FOR CYSTIC FIBROSIS IN SWEDEN

  2011cites this paper
• Defective CFTR Expression and Function Are Detectable in Blood Monocytes: Development of a New Blood Test for Cystic Fibrosis

  2011cites this paper
• Genes that determine immunology and inflammation modify the basic defect of impaired ion conductance in cystic fibrosis epithelia

  2010cites this paper
• Combination testing of multidrug-resistant cystic fibrosis isolates of Pseudomonas aeruginosa: use of a new parameter, the susceptible breakpoint index.

  2010cites this paper
• Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis.

  2010cites this paper
• Cystic Fibrosis gene therapy: methods for the optimisation of CFTR gene delivery.

  2010cites this paper
• Ataluren (PTC124) Induces CFTR Protein Expression and Activity in Children with Nonsense Mutation Cystic Fibrosis

  2010cites this paper
• Towards gene therapy for cystic fibrosis airway disease: development of single-dose lentiviral gene transfer for lifetime airway expression.

  2010cites this paper
• Mutations in the amiloride‐sensitive epithelial sodium channel in patients with cystic fibrosis‐like disease

  2009cites this paper
• Transient correction of the basic defect in sweat glands in an individual with cystic fibrosis carrying the complex CFTR allele F508del-R553Q

  2009cites this paper
• Pathogenetic mechanisms involved in viral-induced exacerbations of COPD

  2009cites this paper
• Physiological measurements confirming the diagnosis of cystic fibrosis: the sweat test and measurements of transepithelial potential difference.

  2009cites this paper
• Cystic fibrosis, aminoglycoside treatment and acute renal failure: the not so gentle micin

  2009cites this paper
• CFTR mutation in an Arab patient: clinical and functional features of 875+1G-->A/875+1G-->A genotype.

  2009cites this paper
• Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders

  2008cites this paper
• A novel computational and structural analysis of nsSNPs in CFTR gene

  2008cites this paper
• Atypical Cystic Fibrosis and CFTR-Related Diseases

  2008cites this paper
• Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis.

  2008cites this paper
• algorithmsfibrosis: terminology and diagnostic

  2008cites this paper
• Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial.

  2008cites this paper
• Advancing outcome measures for the new era of drug development in cystic fibrosis.

  2007cites this paper
• Procedimientos diagnósticos, características clínicas y asesoramiento en la fibrosis quística

  2007cites this paper
• Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials.

  2007cites this paper
• Clinical trials in cystic fibrosis.

  2007cites this paper
• Diagnostische Verfahren, klinische Merkmale und Beratung bei Mukoviszidose (cystischer Fibrose, CF)

  2007cites this paper
• Diversity of the basic defect of homozygous CFTR mutation genotypes in humans

  2007cites this paper
• Diagnostik der zystischen Fibrose

  2007cites this paper
• Reproducibility of nasal potential difference measurements in cystic fibrosis.

  2007cites this paper
• Diagnosis of Cystic Fibrosis, CFTR-Related Disease and Screening

  2006cites this paper
• Diagnostic Procedures, Clinical Characteristics and Counseling in Cystic Fibrosis

  2006cites this paper
• Procedimientos diagnósticos, características clínicas y asesoramiento en la fibrosis quística

  2006cites this paper
• Acidic pH Hyperpolarizes Nasal Potential Difference

  2006cites this paper
• Diagnostische Verfahren, klinische Merkmale und Beratung bei Mukoviszidose (cystischer Fibrose, CF)

  2006cites this paper
• Diagnostic Procedures, Clinical Characteristics and Counseling in Cystic Fibrosis

  2006cites this paper
• Méthodes diagnostiques, caractéristiques cliniques et conseil dans la mucoviscidose

  2006cites this paper
• Cystic fibrosis: terminology and diagnostic algorithms

  2005cites this paper
• In-vivo and ex-vivo functional assessment.

  2004cites this paper
• Genes that determine immunology and inflammation modify the basic defect of impaired ion conductance in cystic fibrosis epithelia

  year unknowncites this paper
• Genes that determine immunology and inflammation modify the basic defect of impaired ion conductance in cystic fibrosis epithelia

  year unknowncites this paper