Cystic fibrosis Pseudomonas aeruginosa

The use of the aerosol route for delivery of antibiotics for pulmonary infections remains controversial. The majority of pediatric studies have been conducted in children with cystic fibrosis. In these patients distribution of the antibiotic to the desired tissue site is impeded because of the viscosity of the sputum in patients with acute exacerbations of their pulmonary infections [91,92], Long-term studies have demonstrated preventive benefits of aerosolized antibiotics in children with cystic fibrosis who are colonizing Pseudomonas aeruginosa in their lungs but are not acutely ill [93,94], Cyclic administration of tobramycin administered by nebulizer has received FDA approval [95],... 

Hodson, M.E., Penketh, A.R. and Batten, J.C. (1981). Aerosol carbenicillin and gentamicin treatment of Pseudomonas aeruginosa infection I patients with cystic fibrosis. Lancet 2 1137-1139. 

Casaulta C, Sdioni MH, Weichel M, Crameri R, Jutel M, Daigle I, et al IL-10 controls Aspergillus fumigatus- and Pseudomonas aeruginosa-specHic T-cell response in cystic fibrosis. Pediatr Res 2003 53 313-319. 

A 1-year-old toddler with cystic fibrosis (CF) is seen by his physician for an upper respiratory infection with Pseudomonas aeruginosa. He is started on oral norfloxacin and referred to a CF center as i potential candidate for gene therapy. Prior genetic testing of the patient identified the mutation causing cystic fibrosis as a 3-base-pair deletion in esan 10 of the CF gene. The nucleotide sequences f codons 506-511 in this region of the normal and mutant alleles are compared below. 

Newer examples of aminoglycoside antibiotics include amikacin, neomycin (Neosporin, Cortisporin), and tobramycin (TOBI, TobraDex). Injectable tobramycin is used in the treatment of serious infections at many body sites. It has also been formulated in an inhalable dosage form that has a very specific use to treat cystic fibrosis patients having Pseudomonas aeruginosa lung infections. In the form suitable for inhalation by the patient, it delivers the antibiotic directly to the site of infection. 

McCoy KS, Quitmer AL, Oermann CM, Gibson RL, Retsch-Bogart GZ, Montgomery AB. (2008) Inhaled aztreonam lysine for chronic airway Pseudomonas Aeruginosa in cystic fibrosis. Am J Respir Crit Care Med 178 921-928. 

Retsch-Bogart GZ, Bums JL, Otto KL, Liou TG McCoy K, Oennann C, Gibson RL. (2008) A phase 2 study of aztreonam lysine for inhalation to treat patients with cystic fibrosis and Pseudomonas Aeruginosa infection. Pediatr Pulmonol 43 47-58. 

A 16-year-old girl, a cystic fibrosis patient, is diagnosed with a ciprofloxacin-resistant Pseudomonas aeruginosa lower respiratory tract infection. 

Cheer SM, Waugh J, Noble S Inhaled tobramycin (TOBI) A review of its use in the management of Pseudomonas aeruginosa infections in patients with cystic fibrosis. Drugs 2003 63 2501. [PMID 14609360]... 

An eight-year-old girl with cystic fibrosis is treated with ciprofloxacin for a Pseudomonas aeruginosa infection in her lungs. Which of the following enzymatic activities is most directly affected by this drug ... 

Children with cystic fibrosis lose excessive amounts of salt in perspiration and become dehydrated readily. A salty taste of the skin and an elevated chloride concentration of sweat are traditional diagnostic symptoms.3 More serious problems arise from progressive respiratory failure and inadequate pancreatic secretion. Lung infections with Pseudomonas aeruginosa are the major cause of death. The CFTR gene is expressed in many tissues, especially those of the mucous membranes. 

Pseudomonas aeruginosa is another major human pathogen that causes disease in patients with impaired host defenses.27 Ps. aeruginosa is primarily associated with ultimately fatal chronic respiratory infection in cystic fibrosis and other forms of bronchiectasis.28 Despite strong inflammatory... 

Canton R, Cobos N, de Gracia J, et al. Antimicrobial therapy for pulmonary pathogenic colonisation and infection by Pseudomonas aeruginosa in cystic fibrosis patients. Clin Microbiol Infect. 2005 11 690-703. 

Deretic, V., M. J. Schurr, J. C. Boucher, and D. W. Martin 1994. Conversion of Pseudomonas aeruginosa to mucoidy in cystic fibrosis Environmental stress and regulation of bacterial virulence by alternative sigma factors. Journal of Bacteriology 176 2773-2780. 

Oberhardt MA, Goldberg JB, Hogardt M et al (2010) Metabolic network analysis of Pseudomonas aeruginosa during chronic cystic fibrosis lung infection. J Bacteriol 192 5534-5548... 

Matsui H., Wagner V.E., Hill D.B., Schwab U.E., Rogers T.D., Buttom B., Taylor R.M., Superfine R., Rubinstein M., Iglewski R.H. and Boucher R.C. (2006) A physical linkage between cystic fibrosis airway surface dehydration and Pseudomonas aeruginosa biofilms. Proc Natl Acad Sci USA 103, 18131-18136... 

The outer region of the core oligosaccharide affects the innate immune responses in the normal host. For example, patients with cystic fibrosis are hypersusceptible to chronic airway infections, particularly with Pseudomonas aeruginosa [127], It was shown that the outer-core region of the LPS of P. aeruginosa influences a critical step... 

Fomsgaard, A., Conrad, R.S., Galanos, C., Shand, G.H., Hoiby, N. Comparativeimmunochemistry of lipopolysaccharides from typable and polyagglutinable Pseudomonas aeruginosa strains isolated from patients with cystic fibrosis. J Clin Microbiol 26 (1988) 821-826. 

Keywords Pseudomonas aeruginosa Cystic fibrosis Lipopolysaccharide Lipid A Chronic infection Antibiotic resistance Innate immunity inflammation... 

Boucher, J.C., Yu, H., Mudd, M.H., Deretic, V. Mucoid Pseudomonas aeruginosa in cystic fibrosis characterization of muc mutations in clinical isolates and analysis of clearance in a mouse model of respiratory infection. Infect Immun 65 (1997) 3838-3846. 

Hancock, R.E.W., Mutharia, L.M., Chan, L., Darveau, R.P., Speert, D.P., Pier, G.B. Pseudomonas aeruginosa isolates from patients with cystic fibrosis a class of serum sensitive, nontypable strains deficient in lipopolysaccharide side chains. Infect Immun 42 (1983) 170-177. 

Henry, R.L., Mellis, C.M., Petrovic, L. Mucoid Pseudomonas aeruginosa is a marker of poor survival in cystic fibrosis. Pediatr Pulmonol 12 (1992) 158-161. 

Li, Z., Kosorok, M.R., Farrell, P.M., Laxova, A., West, S.E., Green, C.G., Collins, J., Rock, M.J., Splaingard, M.L. Longitudinal development of mucoid Pseudomonas aeruginosa infection and lung disease progression in children with cystic fibrosis. Jama 293 (2005) 581-588. 

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