Document Detail

Correlation between nasal potential difference measurements, genotype and clinical condition in patients with cystic fibrosis.
MedLine Citation:
PMID:  9311495     Owner:  NLM     Status:  MEDLINE    
In cystic fibrosis (CF), the clinical condition of patients correlates poorly with genotype. One possible explanation is that clinical status is influenced by net preserved chloride secretion rather than the CF mutation. We tested the relationships between residual chloride secretion, as measured by nasal potential difference (PD) and the type of mutation (genotypes expressing apical cystic fibrosis transmembrane conductance regulator (CFTR) protein versus those that do not) and clinical status. Twenty two CF patients (mean age 25.7 yrs, 11 females and 11 males, mean forced expiratory volume in one second (FEV1) 53.1% of predicted) with defined genotypes were recruited. Nasal PD was measured using a standard protocol involving the perfusion of the nasal epithelium with a sodium channel blocker (amiloride), followed by a solution of low chloride and finally with isoprenaline. Patients with apical CFTR protein showed higher residual chloride secretion than those without (amiloride to isoprenaline value of 4.59 and 0.56 mV, respectively, p = 0.01). There was no correlation between mutation type and clinical condition. When these patients were recategorized as "high" (> 10 mV amiloride to isoprenaline response) or "low" (10 mV or less) chloride secretors, we found that the former group had a significantly higher FEV1 (67.7 versus 48.3% pred) and a better pulmonary radiological score (4.14 versus 7.07, by Northern scoring system). These results suggest that some cystic fibrosis patients, regardless of genotype, have an ability to secrete chloride when stimulated with chloride secretatagogues, and this is correlated with a better lung function. These results also have implications for the use of potential difference measurements in novel cystic fibrosis transmembrane conductance regulator replacement trials.
L P Ho; J M Samways; D J Porteous; J R Dorin; A Carothers; A P Greening; J A Innes
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The European respiratory journal     Volume:  10     ISSN:  0903-1936     ISO Abbreviation:  Eur. Respir. J.     Publication Date:  1997 Sep 
Date Detail:
Created Date:  1997-12-01     Completed Date:  1997-12-01     Revised Date:  2013-05-23    
Medline Journal Info:
Nlm Unique ID:  8803460     Medline TA:  Eur Respir J     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  2018-22     Citation Subset:  IM    
Scottish Adult Cystic Fibrosis Unit Western General Hospital, Edinburgh, UK.
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MeSH Terms
Amiloride / pharmacology
Chlorides / metabolism
Cystic Fibrosis / genetics,  physiopathology*
Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
Forced Expiratory Volume
Isoproterenol / pharmacology
Membrane Potentials
Nasal Mucosa / drug effects,  physiology*
Reg. No./Substance:
0/CFTR protein, human; 0/Chlorides; 126880-72-6/Cystic Fibrosis Transmembrane Conductance Regulator; 2609-46-3/Amiloride; 7683-59-2/Isoproterenol

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