Abstract
Early acquisition of Pseudomonas aeruginosa is associated with a poorer prognosis in patients with cystic fibrosis. We investigated whether polymorphisms in CD14, the lipopolysaccharide receptor, increase the risk of early infection. Forty-five children with cystic fibrosis were investigated with annual bronchoalveolar lavage (BAL) and plasma sCD14 levels. Plasma sCD14 levels were significantly lower in children from whom P.aeruginosa was subsequently isolated (492.75 μg/ml vs. 1339.43 μg/ml, p = 0.018). Those with the CD14 -159CC genotype had a significantly increased risk of early infection with P.aeruginosa suggesting that CD14 C-159T plays a role in determining the risk of early infection with P.aeruginosa.
Highlights
Cystic fibrosis (CF) is the most common serious, monogenic, autosomal recessive disease in Caucasians and results from mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR)
There was no significant difference between CFTR mutation (p = 0.74), gender (p = 0.38), nutritional status (p = 0.39) or socio-economic status (p = 0.29) in those who did and did not isolate P.aeruginosa
We found no significant association between plasma sCD14 levels and CD14 C-159T (p = 0.38)
Summary
Cystic fibrosis (CF) is the most common serious, monogenic, autosomal recessive disease in Caucasians and results from mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CF is characterised by variable phenotypic expression, which is not entirely explained by the allelic heterogeneity of the pathogenic CFTR mutations, and there is accumulating evidence that much of this phenotypic diversity is due to the effect of modifier genes [1]. Pseudomonas aeruginosa, an environmental organism, is the most important pathogen in patients with CF as chronic infection results in a more rapid decline in lung function and reduced survival [2]. CD14, a key gene of the innate immune system, functions as a receptor for lipopolysaccharide (LPS), a constitutive element of the P.aeruginosa cell wall, and is a potential modifier of severity in patients with CF. The -159C allele is associated with lower circulating levels of sCD14 in healthy (page number not for citation purposes)
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