Introduction. Cystic Fibrosis . Cystic Fibrosis (Cf) Is

1323 WordsApr 28, 20176 Pages
Introduction Cystic fibrosis Cystic fibrosis (CF) is a lethal autosomal recessive disorder with a clinical incidence of 1 in every 3500 newborns1. Currently, over 4000 Canadians have CF, with 60% being adults2. In the past, CF was considered a fatal disease with a life expectancy of less than two years, however, advances in enzymatic and antibiotic therapy have greatly improved the life expectancy of patients afflicted with CF3. Currently, the median age of Canadian CF patients is 50 years2. CF is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CTFR) gene, which encodes a chloride ion channel that regulates osmotic balance across the epithelium through the transport of sodium ions and water4. Mutations…show more content…
However, P. aeruginosa is the archetypal pathogen infecting the CF lungs since approximately 80% of adult CF patients are chronically infected with this bacteria1. P. aeruginosa is a ubiquitous Gram-negative bacterium that thrives in moist environmental reservoirs such as in the soil, water and plants8, 9. P. aeruginosa is an opportunistic human pathogen that infects immunocompromised individuals, lending to its association with life-threatening illnesses10. In addition to pulmonary infections in CF patients, P. aeruginosa is frequently found in nosocomial infections11. As such, P. aeruginosa is recognized for its medical importance in clinical infections. Early infection with P. aeruginosa resembles acute non-CF infections within the airways, however the bacterium can evolve genotypically and phenotypically during infection, and adapt to its human host1. Adaptive mutations resulting in loss of motility and acquired antibiotic resistance support a sessile lifestyle that is associated with persistent infection within the CF lungs9, 12. Chronic infection is characterized by immunity-mediated inflammation from increased recruitment of polymorphonuclear leukocytes to the lungs2. Furthermore, P. aeruginosa utilizes an assortment of virulence factors such as proteases, exotoxin A, and phospholipases to destroy host tissue, by which the symptoms of CF patients are

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