Normal nasal mucociliary clearance in CF children: evidence against a CFTR-related defect.
Studies on mucociliary clearance (MCC) in cystic fibrosis (CF) have produced conflicting results. This study aimed to differentiate primary (ion transport-related) from secondary (inflammatory) causes of delayed MCC in CF. Nasal MCC was measured in 50 children (CF, primary ciliary dyskinesia (PCD) and no respiratory disease). Nasal lavage fluid was analysed for interleukin (IL)-8 and tumour necrosis factor-alpha. Similar measurements were obtained in adult CF patients with and without chronic sinusitis (CS). Children with CF had neither delayed MCC nor increased levels of cytokines. Conversely, children with PCD had prolonged MCC times (all >30 min) and significantly raised levels of IL-8. CS-positive CF adults had significantly slower MCC than CS-negative subjects, but IL-8 levels were low and similar in both groups. Decreased airway surface liquid and delayed mucociliary clearance are the postulated primary mechanisms in cystic fibrosis. However, the current study reports that cystic fibrosis children have normal nasal mucociliary clearance. Abnormalities appeared in cystic fibrosis adults with symptoms of chronic sinus disease, suggesting a secondary rather than primary phenomenon. Studies to explore this mechanism in the distal, more sparsely-ciliated airways could aid an understanding of pathogenesis and the development of new treatments.
Dept of Gene Therapy, Imperial College at the National Heart and Lung Institute, London, UK.
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McShane
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Davies
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Wodehouse
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Bush
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Geddes
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Alton
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002925:Ciliary Motility Disorders / Q000175:diagnosis* / Q000453:epidemiology; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D016209:Interleukin-8 / Q000032:analysis*; D008297:Male; D008875:Middle Aged; D009079:Mucociliary Clearance / Q000502:physiology*; D017817:Nasal Lavage Fluid / Q000737:chemistry; D009297:Nasal Mucosa / Q000502:physiology; D011336:Probability; D012016:Reference Values; D018570:Risk Assessment; D012720:Severity of Illness Index; D012737:Sex Factors; D018709:Statistics, Nonparametric; D014409:Tumor Necrosis Factor-alpha / Q000032:analysis*
D016209:Interleukin-8; D014409:Tumor Necrosis Factor-alpha