Cystic fibrosis lung disease

Boucher RC. 1999. Status of gene therapy for cystic fibrosis lung disease. J Clin Inv. 103 441-445. 

Rosenecker J, Huth S, Rudolph C. 2006. Gene therapy for cystic fibrosis lung disease Current status and future perspectives. Curr Opin Mol Ther. 8 439 145. 

ChmielJL, Konstan MW. Anti-inflammatory medications for cystic fibrosis lung disease selecting the most appropriate agent. Treat Respir Med. 2005 ... 

Moskowitz SM, Gibson RL, EfEmann EL. Cystic fibrosis lung disease genetic influences, microbial interactions, and radiological assessment. Pediatr Radiol. 2005 35 739-757. 

Thomas, S.R., Ray, A., Hodson, M.E., Pitt, T.L. Increased sputum amino acid concentrations and auxotrophy of Pseudomonas aeruginosa in severe cystic fibrosis lung disease. Thorax 55 (2000) 795-797. 

Bragonzi A, Conese M (2002) Non-viral approach toward gene therapy of cystic fibrosis lung disease. Curr Gene Ther 2 295-305... 

Grasemann H, van s Gravesande KS, Buscher R, ECnauer N, Silverman ES, Pahner LJ, et al. Endothelial nitric oxide synthase variants in cystic fibrosis lung disease. Am J Respir Crit Care Med 2003 167 390-4. 

Rubin B. Emerging therapies for cystic fibrosis lung disease. Chest 1999, 115, 1120-1126. 

Boucher RC (2002) An overview of the pathogenesis of cystic fibrosis lung disease. Adv Drug Deliv Rev 54(11)4359-1371... 

Cowley EA, Linsdell P (2002) Oxidant stress stimulates anion secretion from the human airway epithelial cell line Calu-3 implications for cystic fibrosis lung disease. J Physiol 543(Pt 1) 201-209... 

Bronsveld I, Bijman J, Mekus F, Ballmann M, Veeze HJ, Tummler B. Clinical presentation of exclusive cystic fibrosis lung disease. Thorax 1999 54 278-281. 

Brody AS, Tiddens HA, Castile RG et al. (2005) Computed tomography in the evaluation of cystic fibrosis lung disease. Am J Respir Grit Care Med 172 1246-1252 Carden KA, Boiselle PM, Waltz DA, Ernst A (2005) Tracheomalacia and tracheobronchomalacia in children and adults an in-depth review. Chest 127 984-1005 Cartier Y, Kavanagh PV, Johkoh T, Mason AC, Muller NL (1999) Bronchiectasis accuracy of high-resolution CT in the differentiation of specific diseases. AJR Am J Roentgenol 173 47-52... 

S. Onoue, et al. New treatments for chronic obstructive pulmonary disease and viable for-mulation/device options for inhalation therapy. Expert Opin DrugDeliv 6,793-811,2009. B.M. Ibrahim, et al. Challenges and advances in the development of inhalable drug formulations for cystic fibrosis lung disease. Expert Opin DrugDeliv 8, 451-466, 2011. 

Biofilms play key roles in several chronic human infections including infectious kidney stones, bacterial endocarditis, and cystic fibrosis lung infections, and the roles of biofilm formation in these diseases have been reviewed [27]. Biofilms also serve as environmental reservoirs for... 

CLC. A classic example of an inherited channelo-pathy is a mutation in CFTR leading to cystic fibrosis, a disease that causes the human body to produce unusually thick and sticky mucus, which clogs the lungs and leads to life-threatening lung infections. Cystic fibrosis affects about 30,000 people in the United States and about 70,000 worldwide. 

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