Published ahead of print on June 26, 2003, doi:10.1164/rccm.200211-1302OC Am. J. Respir. Crit. Care Med., Volume 168, Number 5, September 2003, 594-600 A more recent version of this article appeared on September 1, 2003
Submitted on November 9, 2002 Nasal airway ion transport and lung function in young people with Cystic FibrosisHelen L Wallace1*,1 Institute of Child Health, University of Liverpool, Liverpool, Merseyside, United Kingdom, 2 Division of Pediatric Pulmonology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA * To whom correspondence should be addressed. E-mail: hwallace{at}liv.ac.uk.
There is strong evidence that abnormal airway ion transport is the primary defect that initiates the pathophysiology of lung disease in cystic fibrosis. To examine the relationship between airway ion transport abnormality and severity of lung disease, we measured nasal potential difference in 51 young people with cystic fibrosis using a validated modified technique. There was no correlation between any component of the ion transport measurement and clinical condition (respiratory function, chest radiograph score or Shwachman clinical score). 30 subjects, homozygous for the Key words: young people, nasal potential difference, chloride secretion, delta F508, FEV1
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F508 mutation, were divided into those above and those below average respiratory function for their age. There was no significant difference in any of the ion transport parameters between those with above and below average pulmonary function. Of the 51 subjects, 10 had significant hyperpolarization following perfusion with a zero Cl- solution (>5mV). This Cl- secretory capacity did not correlate with above average lung function. These data do not support the assertion that the extent of lung disease in cystic fibrosis reflects the degree of ion transport abnormality. We suggest that whilst an ion transport abnormality initiates lung disease, other factors (e.g environmental and genetic modifiers) are more influential in determining disease severity.
