Pulmonary infections are the leading cause of morbidity and mortality in patients with cystic fibrosis. Empiric broad-spectrum antibiotics are often used for prolonged periods and repeatedly for exacerbation of bronchiectasis. Hence, as a consequence this select group of patients has the highest incidence of multi-resistant bacteria causing respiratory colonization or infection second to none. It is estimated that 25–45% of adults with cystic fibrosis are chronically infected with multi-resistant bacteria in their airways.1
Moreover, these bacteria usually cannot be eradicated and persist in the respiratory tract despite cycles of different combination of antibiotics.
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References
Lehtzin, N., John, M., Irizarry, R., Merlo, C., Diette, G., Boyle, M., (2006), Outcomes of adults with cystic fibrosis infected with antibiotic-resistant Pseudomonas aeruginosa. Respiration 73:27–33.
Boucher, R., (2005), Cystic fibrosis, in: Harrison's Principles of Internal Medicine, 16th Edition, Kasper, D.C., Braunwald, E., Fauci, A.S., Hauser, S.L., Longo, D.L., Jameson, J. L., (eds), McGraw-Hill, New York, pp. 1543–1546.
Brennan, A.L., Geddes, D.M., (2002), Cystic fibrosis. Curr. Opin. Infect. Dis. 15:175–182.
Fredericksen, B., Koch, C., Hoiby, N., (1997), Antibiotic treatment of initial colonization with Pseudomonas aeruginosa postpones chronic infection and prevents deterioration of pulmonary function in cystic fibrosis. Pediatr. Pulmonol. 23:330–335.
Starner, T.D., McCray, P.B., Jr. (2005), Pathogenesis of early lung disease in cystic fibrosis: A window of opportunity to eradicate bacteria. Ann. Intern. Med. 14:816–822.
Li, Z., Kosork, M.R., Farrell, P.M., Laxova, A., West, S.E.H., Green, C.G., Collins, J., Rock, M.J., Splaingord, M.L., (2005), Longitudinal development of mucoid Pseudomonas aerugi- nosa infection and lung disease progression in children with cystic fibrosis. JAMA 293: 581–588.
Goldman, M.J., Anderson, G.M., Stolzenberg, E.D., Kari, U.P., Zasloff, M., Wilson, J.M., (1997), Human beta-defensins is a salt-sensitive antibiotic in lung that is inactivated in cystic fibrosis. Cell 88:553–560.
Smith, J.J., Travis, S.M., Greenberg, E.P., Welsh, M.J., (1996), Cystic fibrosis airway epithe- lia fail to kill bacteria because of abnormal airway surface fluid. Cell 85:229–236.
Matsui, H., Grubb, B.R., Torran, R., Randell, S.H., Gatzy, J.T., Davis, C.W., Boucher, R.C., (1998), Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 95:1005–1015.
Poschet, J.F., Boucher, J.C., Tatterson, L., Skidmore, J., VanDyke, R.W., Deretic, V., (2001), Molecular basis for defective glycosylation and Pseudomonas pathogenesis in cystic fibrosis lung. Proc. Natl. Acad. Sci USA 98:13972–13977.
Bryan, R., Kube, D., Perez, A., Davis, P., Prince, A., (1998), Overproduction of the CFTR, R domain leads to increased levels of asialoGMI, and increased Pseudomonas aeruginosa binding by epithelial cells. Am. J. Respir. Cell Mol. Biol. 19:269–277.
De Bentzmann, S., Roger, P., Dupuit, F., Bajolet-Laudinat, O., Fuchey, C., Plotkowski, M.C., Puchelle, E., (1996), Asialo-GMI is a receptor for Pseudomonas aeruginosa adherence to regenerating respiratory epithelial cells. Am. J. Respir. Cell Mol. Biol. 64:1582–1588.
Schroeder, T.H., Reiniger, N., Meluleni, G., Grout, M., Colemon, F.T., Pier, G.B., (2001), Transgenic cystic fibrosis mice exhibit reduced early clearance of Pseudomonas aeruginosa from the respiratory tract. J. Immunol. 166:7410–7418.
Meng, O., Springall, D.R., Bishop, A.E., (1998), Lack of inducible nitric oxide synthase in bronchial epithelium: a possible mechanism of susceptibility to infection in cystic fibrosis. J. Pathol. 164:323–331.
Sturgess, J., Imrie, J., (1982), Quantitative evaluation of the development of tracheal submu- cosal glands in infants with cystic fibrosis and control infants. Am. J. Pathol. 106:303–311.
Inglis, S.K., Wilson, S.M., (2005), Cystic fibrosis and airway submucosal glands. Pediatr. Pulmon. 40:279–284.
Aichele, D., Schnare, M., Saake, M., Rollinghoff, M., Gessner, A., (2006), Expression and antimicrobial function of bactericidal permeability increasing protein in cystic fibrosis patients. Infect. Immun. 74:4708–4714.
Miller, M.B., Gilligan, P.H., (2003), Laboratory aspects of management of chronic pulmonary infections in patients with cystic fibrosis. J. Clin. Microbiol. 41:4009–4015.
Cystic Fibrosis Foundation, (2002), Patient registry 2001 annual report. Cystic Fibrosis Foundation, Washington, DC.
Jarry, T.M., Cheung, A.L., (2006), Staphylococcus aureus escapes more efficiently from the phagosome of a cystic fibrosis bronchial epithelial cell line than from it's normal counterpart.Infect. Immun. 74:2565–2577.
Lyczak, J.B., Cannon, C.C., Pier, G.B., (2002), Lung infections associated with cystic fibrosis. Clin. Microbiol. Rev. 15:194–222.
Thomas, S.R., Ray, A., Hodson, M.E., Pitt, T.L., (2000), Increased sputum amino-acid concentration and auxotrophy of Pseudomonas aeruginosa in severe cystic fibrosis lung disease. Thorax 55:795–797.
Costerton, W., Veeh, R., Shirtliff, M., Paomore, M., Post, C., Ehrlich, G., (2003), The application of biofilm science to the study and control of chronic bacterial infections. J. Clin. Invest. 112:1466–1477.
Hoiby, N., (2002), Understanding bacterial biofilms in patients with cystic fibrosis: Current and innovative approaches to potential therapies. J. Cystic Fibrosis. 1:249–254.
Gibson, R.L., Burns, J.L., Ramsey, B.W., (2003), Pathophysiology and management of pulmonary infections in cystic fibrosis. Am. J. Respir. Crit. Care Med. 168:918–951.
Oliver, A., Canton, R., Campo, P., Baquero, F., Blazquez, J., (2000), High frequency of hypermutable Pseudomonas aeruginosa in cystic fibrosis lung infection. Science 288: 1251–1254.
Soong, G., Muir, A., Gomez, M.I., Waks, J., Reddy, B., Planet, P., Singh, P.K., Kanoko, Y., Wolfgang, M.C., Hsiao, Y.S., Tony, L., Prince, A., (2006), Bacterial neuramidases faciliatates mucosal infection by participating in biofilm production. J. Clin. Invest. 116:2297–2305.
Van Alst, N.E., Picardo, K.F., Iglewski, B.H., Haidaris, C.G., (2007) Nitrate sensing and metabolism modulate motility, biofilm formation, and virulence in Pseudomonas aeruginosa. Infect. Immun. 75:3780–3790.
MacEachran, D.P., YE, S., Bomberger, J.M., Hogan, D.A., Swiatecka-Urban, A., Stanton, B. A., O'Toole, G.A., (2007), The Pseudomonas aeruginosa secreted protein PA2934 decreases apical membrane expression of the Cystic Fibrosis Trans-membrane Conductance Regulator.Infect. Immune 75:3902–3912.
Isles, A., Maclusky, I., Corey, M., Gold, R., Prober, C., Flemig, P., Levison, H., (1984), Pseudomonas cepacia infection in cystic fibrosis: an emerging problem. J. Pediatr. 104: 206–210.
Heath, D.G., Hohreker, K., Carriker, C., Smith, K., Routh, J., LiPuma, J.J., Aris, R.M., Weber,, D., Gilligan, P.H., (2002), Six-year molecular analysis of Burkholderia cepacia complex isolates among cystic fibrosis patients at a referral center for lung transplantation. J. Clin. Microbiol. 40:1188–1193.
Chen, J.S., Witzmann, K.A., Spilker, T., Fink, R.J., LiPuma, J.J., (2001), Endemicity and inter-city spread of Burkholderia cepacia genomovar III in cystic fibrosis. J. Pediatr. 139: 643–641.
Chaparro, C., Maurer, J., Gutierrez, C., Krajden, M., Chan, C., Winton, T., Keshavjce, S., Scavuzzo, M., Tullis, E., Hutcheon, M., Kesten, S.E., (2001), Infection with Burkholderia cepacia complex isolates among cystic fibrosis: outcome following lung transplantation. Am. Rev. Respir. Crit. Care Med. 163:43–48.
Aris, R.M., Routh, J.C., LiPuma, J.J., Heath, D.G., Gilligan, P.H., (2001), Lung transplantation for cystic fibrosis patients with Burkholderia cepacia complex. Survival linked to genomovar type. Am. J. Respir. Crit. Care Med. 164:2102–2106.
Henry, D., Campbell, M., Mc Gimpsey, C., Clarke, A., Louden, L., Burns, J.L., Roe, M.H., Vandamme, P, Speert, D., (1999), Comparison of isolation media for recovery of Burkhol- deria cepacia complex from respiratory secretions of patients with cystic fibrosis. J. Clin. Microbiol. 37:1004–1007.
Sun, L., Jiang, R.Z., Stainbach, S., Holmes, A., Campanelli, C., Forstner, J., Sajjan, U., Tan, Y., Riley, M., Goldstein, R., (1995), The emergence of a highly transmissible lineage of Cbl+ Pseudomonas (Burkholderia) cepacia causing CF centre epidemics in North America and Britain. Nat. Med. 7:626–627.
Graff, G.R., Burns, J.L., (2002), Factor affecting the incidence of Stentotrophomonas mal- tophilia isolation in cystic fibrosis. Chest 121:1754–1760.
Saiman, L., Chen, Y., Tabibi, S., San Gibriel, R., Zhou, J., Lui, Z., Lai, L., Whittier., S., (2001), Identification and antimicrobial susceptibility of Alcaligenes xylosoxidans isolated from patients with cystic fibrosis. J. Clin. Microbiol. 39:3942–3945.
Goss, C.H., Otto, K.L., AiKen, M.L., Rubenfeld, G.D., (2002), Detecting Stentotrophomonas maltophilia does not reduce survival of patients with cystic fibrosis. Am. J. Respir. Crit Care Med. 166:356–361.
Tan, K.K., Conway, S.P., Brownlee, K.G., Etherington, C., Peckham, G., (2002), Alcaligenesinfection in cystic fibrosis. Pediatr. Pulmonol. 34:101–104.
McMenamin, J.D., Zaccone, T.M., Coenye, T., Vandamme, P., LiPuma, (2000), Misidentifi- cation of Burkholderia cepacia in US cystic fibrosis treatment centers: an analysis of 1,051 recent sputum isolates. Chest 177:1661–1665.
Krzewinski, J.W., Nguyen: C.D., Foster, J.M., Burns, J.L., (2001), Use of random amplified polymorphic DNA PCR to examine epidemiology of Stentotrophomonas maltophilia and Achromobactor (Alcaligenes) xylosoxidans from patients with cystic fibrosis. J. Clin. Micro- biol. 39:3597–3602.
Kilby, J.M., Gilligan, P.H., Yankaskas, J.R., Highsmith. W.E. Jr., Edwards, L.J., Knowles, M. R., (1992), Nontuberculous mycobacteria in adult patients with cystic fibrosis. Chest 102: 70–75.
Oliver, K.N., Weber, D.J., Wallace, R.J. Jr., Faiz, A.R., Lee, J.H., Zhang, Y., Brown-Elliot, B. A., Handler, A., Wilson, R.W., Schechter, M.S., Edwards, L.J., Chakraborti, S., Knowles, M. R., (2003), Nontuberculous mycobacteria I: multicenter prevalence study in cystic fibrosis. Am. J. Respir. Crit. Care Med. 167:828–834.
Sermet-Gaudelus, I., Le Bourgeois, M., Pierre-Audigier, C., Offredo, C., Guillemot, D., Halley, S., Akoua-Koffi, C., Vincent, V., Sivadon-Tardy, V., Ferroni, A., Berche, P., Schein- mann, P., Lenoir, G., Gaillard, J.-L., (2003), Mycobacterium abscessus and children with cystic fibrosis. Emerg. Infect. Dis. 9:1587–1591.
Pierre-Audigier, V., Ferroni, A., Sermet – Gaudelius, I., Le Bourgeois, M., Offredo, C., Vu- Tien, H., Fauroux, B., Mariani, P., Munck, A., Binger, E., Guillemot, D., Quesne, G., Vincent, V., Berche, P., Gaillard, J.L., (2005), Age-related prevalence and distribution of nontubercu- lous mycobacterial species among patients with cystic fibrosis. J. Clin. Microbiol. 43: 3467–3470.
Olivier, K.N., Weber, D.J., Lee, J.H., Handler, A., Tuder, G, Molina, P.L., Tomashefski, J., Knowles, M.R., (2003), Nontuberculous mycobacteria: II. Nested-cohort study of impact on cystic fibrosis lung disease. Am. J. Respir. Crit. Care Med. 167:833–840.
Ferroni, A., Vu-Thien, H., Lanotte, P., Le Bourgeois, M., Sermet-Gaudelus, I., Fauroux, B., Marchand, S., Varaigne, F., Berche, P., Gaillard, J.L., Offredo, C., (2006), Value of Chorhex- idine decontamination method for recovery of nontuberculous mycobacteria from sputum samples of patients with cystic fibrosis. J. Clin. Microbiol. 44:2237–2239.
Bakore, N., Rickerts, V., Bargon, J., Just-Nubbling, G., (2003), Prevalance of Aspergillus fumigatus and other fungal species in the sputum of adult patients with cystic fibrosis. Mycoses 46:19–23.
Stevens, D.A., Moss, R.B., Kurup, V.P., Knutsen, A.P., Greenberger, P., Judson, M.A, Denning, D.W., Crameri, R., Brody, A.S., Light, M., Skove, M., Maish, W., Mastella, G., and Participants in the Cystic Fibrosis Foundation Consensus Conference, (2003), Allergic bronchopulmonary aspergillosis in cystic fibrosis state of the art: Cystic Fibrosis Foundation Consensus Conference. Clin. Infect. Dis. 37(Suppl. 3):S225–S264.
Taccetti, G., Procopio, E., Morianelti, C., Campama, S., (2000), Allergic bronchopulmonary aspergillosis in Italian cystic fibrosis patients: prevalence and percentage of positive tests in the employed diagnostic criteria. Eur. J. Epidemiol. 16:837–842.
Mastella, G., Rainisio, M., Harmes, H.K., Hodson, M., Koch, C., Navarro, J., Strandvik, B., McKenzie, S.G., (2001), Epidemiologic Registry of Cystic Fibrosis: allergic bronchopulmon- ary aspergillosis in cystic fibrosis, a European epidemiologic study. Eur. Respir. J. 16: 464–471.
Cimon, B., Carrere, J., Vinatier, J.F., Chazalette, J.P., Chabasse, D., Bouchara, J.P., (2001), Clinical significance of Scedosporium apiospermum in patients with cystic fibrosis. Eur. J. Clin. Microbiol Infect. Dis. 19:53–56.
Diemert, D., Kunimoto, D., Sard, C., Rennie, R., (2001), Sputum isolation of Wangiella dematitidis in patients with cystic fibrosis. Scand. J. Infect. Dis. 33:777–779.
Cimon, B., Carrere, J., Chazalette, J.P., Vinatier, J.F., Chabasse, D., Bouchara, J.P., (1999), Chronic airway colonization by Penicillium emesonii in a patient with cystic fibrosis. Med. Mycol. 37:291–293.
Prober, C.G., (1991), The impact of respiratory viral infections in patients with cystic fibrosis. Clin. Rev. Allergy 9:87–102.
Hiatt, P.W., Grace, S.C., Kozinetz, C.A., Raboudi, S.H., Treece, D.G., Taber, L.H., Piedra, P. A., (1999), Effects of viral lower respiratory tract infection on lung function in infants with cystic fibrosis. Pediatr. 103:619–626.
Thomassen, M.J., Demko, C.A., Doershuk, C., (1987), Cystic Fibrosis: a review of pulmonary infections and interventions. Pediatr. Pulmonol. 3:334–351.
Ratjen. F., Comes, G., Paul, K., Posselt, H.G., Wagner, T.O., Harmes, K., (2001), German Board of the European Registry for Cystic Fibrosis (ERCF): effect of continuous antistaphy- lococcal therapy on the rate of P. aeruginosa acquisition in patients with cystic fibrosis. Pediatr. Pulmonol. 31:13–16.
Stutman, H.R., Lieberman, J.M., Nussbaum, E., Marks, M.I., (2002), Antibiotic prophylaxis in infants and children with cystic fibrosis: a randomized controlled trial. J. Pediatr. 140: 299–305.
Valerius, N.H., Koch, C., Hoiby, N., (1991), Prevention of chronic Pseudomonas aeruginosa colonization in cystic fibrosis by early treatment. Lancet 338:725–726.
Burns, J.L., Gibson, R.L., McNamara, S., Yim, D., Emerson, J., Rosenfeld, M., Hiatt, P., McCoy, K., Castile, R., Smith, A.L., Ramsey, B.W., (2001), Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis. J. Infect. Dis. 183:444–452.
Munck, A., Bonacorsi, S., Mariani-Kurkdijian, P., Lebourgeois, M., Gérardin, M., Brahimi, N., Navarro, J., Bingen, E., (2001), Genotype characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis after initial and subsequent colonization. Pediatr. Pulmonol. 32:288–292.
Gibson, R.L., Emerson, J., McNamara, S., Burns, J.L., Rosenfeld, M., Yunker, A., Hamblett, N., Accurso, F., Dovey, M., Hiatt, P., Korstan, M.W., Moss, R., Retsch-Bogert, G, Wagener, J., Waltz, D., Wilmott, R., Zeitlin, P.L., Ramsey, B,: Cystic Fibrosis Therapeutic Development Network Study Group, (2003), Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am. J. Respir. Crit. Care Med. 167:841–849.
Frederiksen, H.K., Norregaard, L., Gotzche, P.C., Pressler, T., Koch, C., Hoiby, N., (1999), Changing epidemiology of Pseudomonas aeruginosa infection in Danish cystic fibrosis patients (1974–1995). Pediatr Pulmonol. 28:159–166.
Johansen, H.K., Norregaard, L., Gotzche, P.C., Presster, T., Koch, C., Hoiby, N., (2004), Antibody response to Pseudomonas aeruginosa in cystic fibrosis patients: a marker of therapeutic success? A 30year cohort study of survival in Danish CF patients after onset of chronic P. aeruginosa lung infection. Pediatr. Pumonol. 37:427–432.
Hoiby, N., Frederiksen, B., Pressler, T., (2005), Eradication of early Pseudomonas aeruginosa infection. J. Cyst. Fibrosis. 4 (Suppl. 2):49–54.
Armstrong, D.S., Grimwood, K., Carlin, J.B., Carzino, R., Gutierrez, J.P., Hull, J., Olinsky, A., Phelan, E.M., Robertson, C.F., Phelan, P.D., (1997), Lower airway inflammation in infants and young children with cystic fibrosis. Am. J. Respir. Crit. Care. 156:1197–11204.
Chiron-Corporation. Chiron Announces Launch of ELITE Trial. Accessed at http: 11phx. corporate-ir/phoenix:zhtml?C=105850&p=irol-news Article&ID=552967&highlight=on18October2005.
Early Pseudomonas Infection Control (EPIC) Trial. National Heart, Lung and Blood Institute (NHLBI). Accessed at www.clinicaltrials.gov./ct/gui/show/NCT00097773.
Illowite, J.S., Gorvoy, J.D., Smaldowe, G.C., (1987), Quantitative deposition of aerolized gentamicin in cystic fibrosis. Am. Rev. Respir. Dis. 136:1445–1449.
Geller, D.E., Pitlick, W.H., Nardella, P.A., Tracewell, W.G., Ramsey, B.W., (2002), Pharma- mcokinetics and bioavailability of aerosolized tobramycin in cystic fibrosis. Chest 122: 219–226.
Ramsey, B.W., Pepe, M.S., Quan, J.M., Otto, K.L., Montgomery, A.B., Williams-Warren, J.,Vansiljev, K.M., Borowitz, D., Bowman, C.M., Marshall, B.C., Marshall, S., Smith, A.L., (1999), Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group. N. Engl. J. Med. 340:23–30.
Moss, R.B., (2001), Administration of aerolized antibiotic in cystic fibrosis patients. Chest 120(Suppl. 3):107S–113S.
Cystic Fibrosis Foundation Patient Registry, (2002), 2001 Annual Data Report to the Center Directors. Cystic Fibrosis Foundation, Bethesda, MD.
Cystic Fibrosis Foundation Patient Registry, (2005), 2004 Annual Data Report to the Center Directors. Cystic Fibrosis Foundation, Bethesda, MD.
Littlewood, J.M., Mitter, M.G., Ghoneim, A.T., Ramsden, C.H., (1985), Nebulized colomycin for early Pseudomonas colonization in cystic fibrosis. Lancet 1:865.
Hodson, M.E., Gallagher, C.G., Govan, J.R., (2002), A randomized trial of nebulized tobra- mycin or colistin in cystic fibrosis. Eur. Respir. J. 20:658–664.
Cunningham, S., Prasad, A., Collyer, L., Carr, S., Lynn, I.B., Wallis, C., (2001), Broncho- constriction following nebulized colistin in cystic fibrosis. Arch. Dis. Child. 84:432–433.
Fernandez, J.D., Santiago, R.T., Matacon, M.P., Mayo, R.C., Sańchez, G.T., (1994), Inhaled aztreonam therapy in patients with cystic fibrosis colonized with Pseudomonas aeruginosa. An. Esp. Pediatr. 40:185–188.
Cystic Fibrosis Foundation Clinical Trials & Studies, (2006), Anti-Infection therapies. Cystic Fibrosis Foundation, Besthesda, MD, http:/www.cff.org/research/clinical-trials/ongoing- trials/anti-infection/#IV_VS/Inhaled-Antibiotics.
Reed, M.D., Stern, R.C., Myers, C.M., Yamashita, T.S., Blumer, J.L., (1988), Lack of unique ciprofloxacin pharmacokinetic characteristics in patients with cystic fibrosis. J. Clin. Pharmacol. 28:691–699.
Ball, P., (1990), Emergent resistance to ciprofloxacin amongst Pseudomonas aeruginosa and Staphylococcus aureus: clinical significance and therapeutic approaches. J. Antimicrob. Che- mother 26(Suppl. F):165–179.
Chysky, V., Kapila, K., Hullman, R., Arcieri, G., Schacht, P., Echols, R., (1991), Safety of ciprofloxacin in children: worldwide clinical experience based on compassionate use: emphasis on joint evaluation. Infections 19:289–296.
Jaffe, A., Bush, A., (2001), Antiinflammatory effects of macrolides in lung disease. Pediatr. Pulmonol. 31:464–473.
Tateda, K., Ishii, Y., Matsumoto, T., Furuya, N., Nagasluma, M., Matsunaga, T., Ohno, A., Miyazaki, S., Yomaguchi, K., (1996), Direct evidence for antipseudomonal activity of macrolides: exposure-dependent bactericidal activity and inhibition of protein synthesis by erythromycin, clarithromycin, and azithromycin. Antimicrob. Agents Chemother. 40: 2271–2275.
Wozniak, D.J., Keyser, R., (2004), Effects of subinhibitory concentration of macrolide antibiotics on Pseudomonas aeruginosa. Chest 125(Suppl. 2):62S–69S.
Labro, M.T., (1998), Antiinflammatory effects of macrolides: a new therapeutic potential? J. Antimicrob. Chemother. 41:37–46.
Equi, A., Balfour-Lynn, I.M., Bush, A., Rosenthal, M., (2002), Long term azithromycin in children with cystic fibrosis: a randomized, placebo-controlled crossover trial. Lancet 360:978–984.
Wolter, J., Seeney, S, Bell, S., Bowler, S., Masel, P., McCormach, J., (2002), Effect of long nterm treatment with azithromycin on disease parameters in cystic fibrosis: a randomized trial. Thorax 57:212–216.
Saiman, L., Marshall, B.C., Mayer-Hamblatt, N., Burns, J.L., Quittner, A.L., Cibene, D.A., Coquillette, S., Fieberg, A.Y., Accurso, F.J., Campbell, P.W.3rd, (2003), The Macrolide Study Group: a multicenter, randomized, placebo controlled, double-blind trial of azithro- mycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa. JAMA 290:1749–1756.
Elphick, H.E., Tan, A., (2006), Single versus combination therapy for people with cystic fibrosis. Cochrane Database of Systematic Reviews; accessed at: 00075320–100000000– 01458.
Aaron, S.D., Vandemheen, K.L., Ferris, W., Fergusson, D., Tullis, E., Haase, D., Berthiaume, Y., Brown, N., Wilcox, P., Yizghatlion, V., Bye, P., Bell, S., Chan, F., Rose, B., Jeanneret, A., Stephenson, A., Noseworthy, M., Freitag, A., Paterson, N., Doucette, S., Harbour, C., Ruel, M., MacDonald, N., (2005), Combination antibiotic susceptibility testing to treat exacerbation of cystic fibrosis associated with multiresistant bacteria: a randomized, double-blind controlled trial. Lancet 366:463–471.
Etherington, C., Hall, M., Conway, S., Peckham, D., Denton, M., (2008), Clinical impact of reducing routine susceptibility testing in chronic Pseudomonas aeruginosa infections in cystic fibrosis. J. Antimicrob. Chemother. 61:425–427.
Lechtzin, N., John, M., Irizarry, R., Merlo, C., Diette, G.B., Boyle, M.P., (2006), Outcomes of adults with cystic fibrosis infected with antibiotic-resistant Pseudomonas aeruginosa. Respiration 73:27–33.
Burkhardt, O., Lehmann, C., Madabushi, R., Kumar, V., Derendorf, H., Welte, T., (2006), Once-daily tobramycin in cystic fibrosis: better for clinical outcome than thrice daily but more resistance development. J. Antimicrob. Chemother. 58:822–829.
Lewin, C., Doherty, C., Gowan, J., (1993), Invitro activities of meropenem, PD127391, PD131628, ceftazidime, chloramphenical, co-trimoxazole, and ciprofloxacin against Pseu- domonas cepacia. Antimicrob. Agents. Chemother. 37:123–125.
Burns, J., Saiman, L., (1999), Burkholderia cepacia infection in cystic fibrosis. Pediatr. Infect. Dis. J. 18:123–125.
Vartivarian, S., Anaissie, E., Bodey, G., Spingg, H., Rolston, K., (1994), A changing pattern of susceptibility of Xanthomonas maltophilia to antimicrobial agents; implications for therapy. Antimicrob. Agents Chemother. 38:624–627.
Saiman, L., Chen, Y., Tabibi, S., San Gabriel, P., Zhou, J., Liu, Z., Lai, L., Whittier, S., (2001), Identification and antimicrobial susceptibility of Alcaligenes xylosoxidans isolated from patients with cystic fibrosis. J. Clin. Microbiol 39:3942–3945.
Maiz-Carro, L., Navas-Elorza, E., (2002), Nontubercilous mycobacterial pulmonary infection in patients with cystic fibrosis. Am. J. Resp. Med. 1:107–117.
Petrini, B, (2006), Mycobacterium abscessus: an emerging-rapid-growing potential pathogen. APMIS 114:319–328.
Langford, D.T., Hiller, J., (1984), Prospective, controlled study of a polyvalent Pseudomonas vaccine in cystic fibrosis: three year results. Arch. Dis. Child 59:1131–1134.
Lang, A.B., Schaad, U.B., Rudeberg, A., Wedgwood, J., Que J.U., Furer, E., Cryz, S.J., Jr., (1995), Effect of high-affinity anti-Pseudomonas aeruginosa lypopolysaccharide antibodies induced by immunization on the rate of Pseudomonas aeruginosa infection in patients with cystic fibrosis. J. Pediatr. 127:711–717.
Doring G, Dorner, F., (1997), A multicenter vaccine trial using the Pseudomonas aeruginosa flagella vaccine immunization in patients with cystic fibrosis. Behring Inst. Mitteilungen 98:338–344.
Wark, P.A.B., McDonald, V., Jones, A.P., (2006), Nebulized hypertonic saline for cystic fibrosis. Cochrane Database of Systematic Review. Access: 00075320–100000000–00452.
Donaldson, S.H., Bennett, W.D., Zeman, K.L., Knowles, M.R., Tarran, R., Boucher, R.C., (2006), Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N. Engl. J. Med. 354:241–250.
Elkins, M.R., Robinson, M., Rose, B.R., Harbour, C., Moriarty, C.P., Marks, G.B., Belou- sova, E.G., Xuan, W., Bye, P.T.P, for the National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group, (2006), A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis N. Engl. J. Med. 354:229–240.
Ratjen, F., (2006), Restoring airway surface liquid in cystic fibrosis. (Editorial), N. Engl. J. Med. 354:291–293.
Fuchs H.J., Borowitz, D.S., Christiansen, D.H., Morris, E.M., Nash, M.L., Ramsey, B.W.,m Rosenstein, B.J., Smith, A.L., Wohl, M.E., (1994), The Pulmozyme Study Group: Effect of aerolized recombinant human DNASE on exacerbations of respiratory symptoms and pulmonary function in patients with cystic fibrosis. N. Engl. J. Med. 331:637–642.
Quan, J.M., Tiddens, H.A., Sy, J.P., McKenzie, S.G., Montgomery, M.D., Robinson, P.J., Wohl, M.E., Konstan, M.-W., (2001), The Pulmozyme Early Intervention Trial Study Group: a two year randomized, placebo-controlled trail of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J. Pediatr. 139:813–820.
Jones, A.P., Wallis, C.E., Kearney, C.E., Kearney, C.E., (2006), Recombinant human deoxyribonuclease for cystic fibrosis. Cochrane Database of Systematic Reviews. Access no: 00075320–100000000–00909.
Suri, R., Metcalf, C., Lees, B., Grieve, R., Flather, M., Normand, C., Thompson, S., Bush, A., Wallis, C., (2001), Comparison of hypertonic saline and alternate-day or daily recombinant human deoxy-ribonuclease in children with cystic fibrosis: a randomized trial. Lancet 358:1316–1321.
Korstan, M.W., Davis, P.B., (2002), Pharmacological approaches for discovery and development of new anti-inflammatory agents for the treatment of cystic fibrosis. Adv. Drug Deliv. Rev. 54:1409–1423.
Lands, L.C., Desateux, C., Crighton, A., (2006), Oral non-steroidal anti-inflammatory drug therapy for cystic fibrosis. Cochrane Database Systematic Reviews. Access no: 00075320– 100000000–003494.
Rich, D.P., Anderson, M.P., Gregory, R.J., Chery, S.H., Paul, S., Jefferson, D.M., McCann, J. D., Klirger, K.W., Smith, A.E., Welsh, M.J., (1990), Expression of cystic fibrosis transmem- brane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells. Nature 347:358–363.
Cystic Fibrisis Foundation, (2004), Annual Report: Research. Cystic Fibrosis Foundation, Bethesda, MD access: no:http://www.cff.org/uploadedfiles/publications/files/2004Annual- ReportFinal.pdf.
Borghouthi, S., Guerdoud, L.M., Speert, D.P., (1996), Inhibition by dextran of Pseudomonas aeruginosa adherence to epithelial cells: Am. J. Respir. Crit. Care Med. 154:1788–1793.
Chiu, C.H., Wong, S., Hancock, R.E., Speert, D.P., (2001), Adherence of Burkholderia cepacia to respiratory tract epithelial cells and inhibition with dextrans. Microbiol. 147:2651–2658.
Bryan, R., Feldman, M., Jawetz, S.C., Rajan, S., DiMargo Tang, H.B., Scheffler, L., Speert, D.P., Prince, A., (1999), The effects of aerolized dextran in a mouse model of Pseudomonas aeruginosa pulmonary infection. J. Infect. Dis. 179:1449–1458.
Feng, W., Garrett, H., Speert, D.P., King, M., (1998), Improved clearability of cystic fibrosis sputum with dextran treatment in vitro. Am. J. Respir. Care Med. 184:29–32.
Cole, A.M., Liao, H., Stuchlik, O., Tilan, J., Pohl, J., Ganz, T., (2002), Cationic polypeptide are required for antibacterial activity of human airway fluid. J. Immunol. 169:6985–6991.
Goldman, M.J., Anderson, G.M., Stolzenberg, E.D., Kari, U.P., Zasloff, M., Wilson, J.M., (1997), Human beta-defensin-1 is a salt-sensitive antibiotic that is inactivated in cystic fibrosis. Cell 88:553–560.
Ramsey, B, Rodman, D., Redman, R., Haeslsen, M., Johnson, C., Hamblett, N., Fugii, C., Loury, D., (2001), Phase I safety and tolerability study of ascending multiple doses of aerolized isegaran HCL solution (1B-367) in adults with cystic fibrosis. Pediatr. Pulmonol. 32:A263.
Ganz, T., (2001), Fatal attraction evaded: how pathogenic bacteria resist cationic polypep- tides. J. Exp. Med. 193:F31–F34.
Hoffman, N., Lee, B., Rasmussen, T.B., Song, Z., Johansen, H.K., Givskow, M., Hoiby, N., (2007), Azithromycin block quorum sensing and alginate polymer formation and increases sensitivity to serum and stationary-growth-phase killing of Pseudomonas aeruginosa and attenuates chronic P. aeruginosa lung infection in CFTR(−/−) mice. Antimicrob. Agents Chemother. 51:3677–3687.
Overhage, J., Campisano, A., Bains, M., Torfs, ECW, Rehm, BHA, Hancock, REW, (2008), Human host defense peptide LL-37 prevents bacterial biofilm formation. Infect. Immun. 76:4176–4182.
van der Plas, M.J., Jukema, G.N., Wai, S.W., Dogterom-Ballering, H.C., Lagendijk, E.L., van Gulpen, C., van Dissel, J.T., Bloemberg, G.V., Nibbesring, P.H., (2008), Maggot excretions/secretions are differentially effective against biofilms of Staphylococcus aureus and Pseudomonas aeruginosa. J. Antimicrob. Chemother. 61:117–122.
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(2009). Emerging Issues in Pulmonary Infections of Cystic Fibrosis. In: Fong, I.W. (eds) Emerging Issues and Controversies in Infectious Disease. Emerging Infectious Diseases of the 21st Century. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84841-9_4
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