Cystic Fibrosis Clinical Trial
Official title:
Epidemiology of the Nasal Flora at the Reference Center for Cystic Fibrosis of Queen Fabiola Children's University Hospital.
Cystic fibrosis (CF) is the most common autosomal recessive inherited genetic disorder in
North America, Australia and Europe.
CF is due to cystic fibrosis transmembrane conductance regulator gene mutation (CFTR) coding
for a chloride channel located at the apical membrane of epithelial cells. The most common
mutation is the deletion of the amino acid phenylalanine at the codon 508 (ΔF508) affecting
70% of the patients.
The CFTR channel participates in the regulation of the volume and composition of exocrine
secretions. At the level of the lungs, this results in a thickening of the mucus with a
dysfunction of the mucociliary clearance promoting colonization of pathogenic microorganisms.
Patients with cystic fibrosis therefore have a natural susceptibility to develop acute and
then chronic respiratory infections, gradually leading to irreversible respiratory tract
lesions called bronchiectasis. Different germs such as Haemophilus influenzae and
Staphylococcus aureus colonize the airways early in life. The progression of the disease
causes furthermore a colonization by opportunistic germs such as Pseudomonas aeruginosa and
Burkholderia cepacia, which are associated with higher mortality.
Pulmonary exacerbation is a common complication of CF requiring administration of
antibiotics. The choice of these antibiotics depends on the germs that the patient carries in
his respiratory tract.
The type of sampling and the conditions under which they are taken are therefore very
important. Sputum and oropharyngeal smear are used in adolescents and children respectively
to collect respiratory secretions in clinical routine. The recent literature describes
induced sputum, obtained after a physiotherapy session and a hypertonic serum aerosol, as
superior to the oropharyngeal smear alone and equivalent to bronchoalveolar lavage for the
evaluation of the microbiological profile of patients who cannot expectorate. However, this
technique takes time and requires the presence of a physiotherapist.
Bronchoalveolar lavage is reserved for complex cases that do not respond to standard
treatments.
Finally, the nasal flora appears to be involved in the colonization of the lower respiratory
tract. Sinuses are described as reservoirs of germs that can induce a recolonization of the
lungs despite eradication of the germ (for example after a pulmonary transplantation) .
To our knowledge, no study has investigated the involvement of nasal flora in the clinical
course of children with CF.
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