Treatment of lung infection in patients with cystic fibrosis: Current and future strategies

Journal of Cystic Fibrosis

Volumn 11, Issue 6, 2012, Pages 461-479

  Döring, Gerd ^a   Flume, Patrick ^b   Heijerman, Harry ^c   Elborn, J Stuart ^d  

^a UNIVERSITY OF TÜBINGEN   (Germany)

^b MEDICAL UNIVERSITY OF SOUTH CAROLINA   (United States)

^c LEYENBURG HOSPITAL   (Netherlands)

^d QUEEN'S UNIVERSITY BELFAST   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

AMIKACIN; AMOXICILLIN PLUS CLAVULANIC ACID; ANTIBIOTIC AGENT; ARIKACE; AZLI; AZTREONAM; AZTREONAM LYSINE; BRAMITOB; CEFTAZIDIME; CEFUROXIME AXETIL; CIPROFLOXACIN; CLINDAMYCIN; COLISTIMETHATE; COLISTIN; COTRIMOXAZOLE; DICLOXACILLIN; DOXYCYCLINE; FLUCLOXACILLIN; FOSFOMYCIN; FOSFOMYCIN PLUS TOBRAMYCIN; FUSIDIC ACID; LEVOFLOXACIN; LINEZOLID; LIPOSOMAL AMIKACIN; LIPOSOME; MEROPENEM; MINOCYCLINE; PLACEBO; RIFAMPICIN; TEICOPLANIN; TOBRAMYCIN; UNCLASSIFIED DRUG; UNINDEXED DRUG; VANCOMYCIN;

ACHROMOBACTER XYLOSOXIDANS; AEROSOL; ANAEROBIC BACTERIUM; ANTIBIOTIC PROPHYLAXIS; ANTIBIOTIC SENSITIVITY; ANTIBIOTIC THERAPY; BACTERIAL PNEUMONIA; BACTERIUM CULTURE; BURKHOLDERIA CEPACIA COMPLEX; CONSENSUS DEVELOPMENT; COST EFFECTIVENESS ANALYSIS; COUGHING; DISEASE EXACERBATION; DRUG CAPSULE; DRUG DOSE ESCALATION; DRUG EFFICACY; DRUG FORMULATION; DRUG SAFETY; DRUG TREATMENT FAILURE; ECONOMIC EVALUATION; FORCED EXPIRATORY VOLUME; HEALTH CARE COST; HUMAN; LUNG FIBROSIS; LUNG INFECTION; MEDICAL NEBULIZER; MULTIPLE CYCLE TREATMENT; MYCOBACTERIUM; MYCOBACTERIUM ABSCESSUS; NONHUMAN; PARTICLE SIZE; PATHOPHYSIOLOGY; PATIENT CARE; PATIENT MONITORING; POWDER INHALER; PSEUDOMONAS AERUGINOSA; PSEUDOMONAS PNEUMONIA; RANDOMIZED CONTROLLED TRIAL (TOPIC); RECOMMENDED DRUG DOSE; REVIEW; STAPHYLOCOCCAL PNEUMONIA; STAPHYLOCOCCUS AUREUS; STENOTROPHOMONAS MALTOPHILIA; TREATMENT DURATION; TREATMENT OUTCOME; TREATMENT RESPONSE;

ANTI-BACTERIAL AGENTS; CYSTIC FIBROSIS; HUMANS; PNEUMONIA, BACTERIAL; PSEUDOMONAS AERUGINOSA; PSEUDOMONAS INFECTIONS;

EID: 84869120178     PISSN: 15691993     EISSN: 18735010     Source Type: Journal    
DOI: 10.1016/j.jcf.2012.10.004     Document Type: Review

References (172)

1. Rosenfeld M., Gibson R.L., McNamara S., Emerson J., Burns J.L., Castile R., et al. Early pulmonary infection, inflammation, and clinical outcomes in infants with cystic fibrosis. Pediatr Pulmonol 2001, 32:356-366.
2. Emerson J., Rosenfeld M., McNamara S., Ramsey B., Gibson R.L. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol 2002, 34:91-100.
3. Cystic fibrosis foundation patient registry 2009 annual data report 2010, Cystic Fibrosis Foundation, Bethesda, MD, USA.
4. Schechter M.S., McColley S.A., Silva S., Haselkorn T., Konstan M.W., Wagener J.S., et al. Association of socioeconomic status with the use of chronic therapies and healthcare utilization in children with cystic fibrosis. J Pediatr 2009, 155:634-639.
5. McCormick J., Mehta G., Olesen H.V. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet 2010, 375:1007-1013.
6. Taylor-Robinson D., Whitehead W., Diderichsen F., Vebert Olesen H., Pressler T., Smyth R.S., Diggle P. Understanding the natural progression in %FEV1 decline in patients with cystic fibrosis: a longitudinal study. Thorax 2012, 67:860-866.
7. Döring G., Conway S.P., Heijerman H.G., Hodson M.E., Hoiby N., Smyth A., et al. Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus. Eur Respir J 2000, 16:749-767.
8. Döring G., Hoiby N., for the Consensus Study Group Early intervention and prevention of lung disease in cystic fibrosis: a European consensus. J Cyst Fibros 2004, 3:67-91.
9. Heijerman H., Westerman Elsbeth, Conway S., Touw D., Döring G. Inhaled medication and inhalation devices for lung disease in patients with cystic fibrosis: a European consensus. J Cyst Fibros 2009, 8:295-315.
10. Döring G., Gulbins E. Cystic fibrosis and innate immunity: how chloride channel mutations provoke lung disease. Cell Microbiol 2009, 11:208-216.
11. Burns J.L., Gibson R.L., McNamara S., Yim D., Emerson J., Rosenfeld M., et al. Longitudinal assessment of Pseudomonas aeruginosa in young children with cystic fibrosis. J Infect Dis 2001, 183:444-452.
12. Wiesemann H.G., Steinkamp G., Ratjen F., Bauernfeind A., Przyklenk B., Döring G., et al. Placebo-controlled, double-blind, randomized study of aerolized tobramycin for early treatment of Pseudomonas aeruginosa infection in cystic fibrosis. Pediatr Pulmonol 1998, 25:88-92.
13. Gibson R.L., Emerson J., McNamara S., Burns J.L., Rosenfeld M., Yunker A., et al. Significant microbiological effect of inhaled tobramycin in young children with cystic fibrosis. Am J Respir Crit Care Med 2003, 167:841e9.
14. Van Ewijk B.E., Wolfs T.F.W., Fleer A., Kimpen J.L.L., Van der Ent C.K. High Pseudomonas aeruginosa acquisition rate during acute respiratory infection in healthy and cystic fibrosis children. Thorax 2006, 61:641-642.
15. Lee T.W.R., Brownlee K.G., Conway S.P., Denton M., Littlewood J.M. Evaluation of a new definition for chronic Pseudomonas aeruginosa infection in cystic fibrosis patients. J Cyst Fibros 2003, 2:29-34.
16. Proesmans M., Balinska-Miskiewicz W., Dupont L., Bossuyt X., Verhaegen J., Høiby N., et al. Evaluating the "Leeds criteria" for Pseudomonas aeruginosa infection in a cystic fibrosis centre. Eur Respir J 2006, 27:937-943.
17. Taccetti G., Campana S., Festini F., Mascherini M., Döring G. Early eradication therapy against Pseudomonas aeruginosa in cystic fibrosis patients. Eur Respir J 2005, 26:1-4.
18. Kozlowska W.J., Bush A., Wade A., Aurora P., Carr S.B., Castle R.A., et al. Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis. Am J Respir Crit Care Med 2008, 178:42-49.
19. Ratjen F., Munck A., Kho P., Angyalosi G. ELITE Study Group. Treatment of early Pseudomonas aeruginosa infection in patients with cystic fibrosis: the ELITE trial. Thorax 2010, 65:286-291.
20. Treggiari M.M., Retsch-Bogart G., Mayer-Hamblett N., Khan U., Kulich M., Kronmal R., et al. Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis. Arch Pediatr Adolesc Med 2011, 165:847-856.
21. Amin R., Lam M., Dupuis A., Ratjen F. The effect of early Pseudomonas aeruginosa treatment on lung function in pediatric cystic fibrosis. Pediatr Pulmonol 2011, 46:554-558.
22. Mayer-Hamblett N., Kronmal R.A., Gibson R.L., Rosenfeld M., Retsch-Bogart G., Treggiari M.M., et al. Initial Pseudomonas aeruginosa treatment failure is associated with exacerbations in cystic fibrosis. Pediatr Pulmonol 2012, 47:125-134.
23. Tramper-Stranders G.A., van der Ent C.K., Molin S., Yang L., Hansen S.K., Rau M.H., et al. Initial Pseudomonas aeruginosa infection in patients with cystic fibrosis: characteristics of eradicated and persistent isolates. Clin Microbiol Infect 2011, 10.1111/j.1469-0691.2011.03627.x.
24. Döring G., Meisner C., Stern M. A double-blind randomized placebo-controlled phase III study of a Pseudomonas aeruginosa flagella vaccine in cystic fibrosis patients. PNAS 2007, 104:11020-11025. for the Flagella Vaccine Trial Study Group.
25. Cantón R., Cobos N., De Gracia J., Baquero F., Honorato J., Gartner S. Antimicrobial therapy of pulmonary pathogenic colonisation and infection by Pseudomonas aeruginosa in cystic fibrosis patients. Clin Microbiol Infect 2005, 11:690-703.
26. Valerius N.H., Koch C., Høiby N. Prevention of chronic Pseudomonas aeruginosa colonisation in cystic fibrosis by early treatment. Lancet 1991, 338:725-726.
27. Ratjen F., Döring G., Nikolaizik W.H. Effect of inhaled tobramycin on early Pseudomonas aeruginosa colonisation in patients with cystic fibrosis. Lancet 2001, 358:983-984.
28. Gibson R.L., Emerson J., Mayer-Hamblett N., Burns J.L., McNamara S., Accurso F.J., et al. Duration of treatment effect after tobramycin solution for inhalation in young children with cystic fibrosis. Pediatr Pulmonol 2007, 42:610-623.
29. Taccetti G., Bianchini E., Cariani L., Buzzetti R., Costantini D., Trevisan F. Early antibiotic treatment for Pseudomonas aeruginosa eradication in patients with cystic fibrosis: a randomised multicentre study comparing two different protocols. Thorax 2012, 67:853-859.
30. Høiby N., Frederiksen B., Pressler T. Eradication of early Pseudomonas aeruginosa infection. J Cyst Fibros 2005, 4(Suppl. 2):49-54.
31. Proesmans M. Comparison of two treatment regimens for eradication of P aeruginosa infection in children with cystic fibrosis. J Cyst Fibros in press.
32. Lillquist Y.P., Cho E., Davidson A.G. Economic effects of an eradication protocol for first appearance of Pseudomonas aeruginosa in cystic fibrosis patients: 1995 vs. 2009. J Cyst Fibros 2011, 10:175-180.
33. Noah T.L., Ivins S.S., Abode K.A., Stewart P.W., Michelson P.H., Harris W.T., et al. Inhaled versus systemic antibiotics and airway inflammation in children with cystic fibrosis and Pseudomonas. Pediatr Pulmonol 2010, 45:281-290.
34. Kumana C.R., Yuen K.Y. Parenteral aminoglycoside therapy. Selection, administration and monitoring. Drugs 1994, 47:902-913.
35. Rosenfeld M., Gibson R., McNamara S., Emerson J., McCoyd K.S., Shell R., et al. Serum and lower respiratory tract drug concentrations produced by tobramycin for inhalation in young children with cystic fibrosis. J Pediatr 2001, 139:572-577.
36. Hansen C.R., Pressler T., Høiby N. Early aggressive eradication therapy for intermittent Pseudomonas aeruginosa airway infection in cystic fibrosis patients: 15 years experience. J Cyst Fibros 2008, 7:523-530.
37. Treggiari M.M., Rosenfeld M., Retsch-Bogart G., Gibson R., Ramsey B. Approach to eradication of initial Pseudomonas aeruginosa infection in children with cystic fibrosis. Pediatr Pulmonol 2007, 42:751-756.
38. Rogers G.B., Hoffman L.R., Döring G. Novel concepts in evaluating antimicrobial therapy for bacterial lung infections in patients with cystic fibrosis. J Cyst Fibros 2011, 10:387-400.
39. Høiby N., Ciofu O., Bjarnsholt T. Pseudomonas aeruginosa biofilms in cystic fibrosis. Future Microbiol 2010, 5:1663-1674.
40. del Pozo J.L., Patel R. The challenge of treating biofilm-associated bacterial infections. Clin Pharmacol Ther 2007, 82:204-209.
41. Smith E.E., Buckley D.G., Wu Z., Saenphimmachak C., Hoffman L.R., D'Argenio D.A., et al. Genetic adaptation by Pseudomonas aeruginosa to the airways of cystic fibrosis patients. Proc Natl Acad Sci USA 2006, 3:8487-8492.
42. Foweraker J.E., Laughton C.R., Brown D.F., Bilton D. Comparison of methods to test antibiotic combinations against heterogeneous populations of multiresistant Pseudomonas aeruginosa from patients with acute infective exacerbations in cystic fibrosis. Antimicrob Agents Chemother 2009, 53:4809-4815.
43. Döring G., Parameswaran G.I., Murphy T.F. Differential adaptation of microbial pathogens to airways of patients with cystic fibrosis and chronic obstructive pulmonary disease. FEMS Microbiol Rev 2010, [28 MAY 2010]. 10.1111/j.1574-6976.2010.00237.
44. Mulcahy L.R., Burns J.L., Lory S., Lewis K. Emergence of Pseudomonas aeruginosa strains producing high levels of persister cells in patients with cystic fibrosis. J Bacteriol 2010, 192:6191-6199.
45. Høiby N., Bjarnsholt T., Givskov M., Molin S., Ciofu O. Antibiotic resistance of bacterial biofilms. Int J Antimicrob Agents 2010, 35:322-332.
46. Bjarnsholt T., Østrup Jensen P., Jakobsen T.H., Phipps R., Nielsen A.K., Rybtke M.T., et al. Quorum sensing and virulence of Pseudomonas aeruginosa during lung infection of cystic fibrosis patients. PLos One 2010, 5:e10115. [doi:10.1371].
47. Mowat E., Paterson S., Fothergill J.L., Wright E.A., Ledson M.J., Walshaw M.J., et al. Pseudomonas aeruginosa population diversity and turnover in cystic fibrosis chronic infections. Am J Respir Crit Care Med 2011, 183:1674-1679.
48. Ordonez C.L., Henig N.R., Mayer-Hamblett N., Accurso F.J., Burns J.L., Chmiel J.F., et al. Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis. Am J Respir Crit Care Med 2003, 168:1471-1475.
49. Ciofu O., Mandsberg L., Bjarnsholt T., Wassermann T., Høiby N. Genetic adaptation of P. aeruginosa during chronic lung infection of patients with cystic fibrosis: strong and weak mutators with heterogenous genetic backgrounds emerge in mucA and/or lasR mutants. Microbiology 2010, 156:1108-1119.
50. Yang L., Jelsbak L., Marvig R.L., Damkiæra S., Workmana C.T., Rau M.H., et al. Evolutionary dynamics of bacteria in a human host environment. PNAS 2011, 108:7481-7486.
51. Muhlebach M.S., Miller M.B., Moore C., Wedd J.P., Drake A.F., Leigh M.W. Are lower airway or throat cultures predictive of sinus bacteriology in cystic fibrosis?. Pediatr Pulmonol 2006, 41:445-451.
52. Mainz J.G., Hentschel J., Schien C., Cramer N., Pfister W., Beck J.F., et al. Sinonasal persistence of Pseudomonas aeruginosa after lung transplantation. J Cyst Fibros 2012, 11:158-161.
53. Hansen S.K., Rau M.H., Johansen H.K., Ciofu O., Jelsbak L., Yang L., et al. Evolution and diversification of Pseudomonas aeruginosa in the paranasal sinuses of cystic fibrosis children have implications for chronic lung infection. ISME J 2011, 10.1038/ismej.2011.83.
54. Johansen HK, Aanaes K, Pressler T, et al. Colonization and infection of the paranasal sinuses in cystic fibrosis patients is accompanied by a reduced PMN response. J Cyst Fibros in press, http://dx.doi.org/10.1016/j.jcf.2012.04.011. http://dx.doi.org/10.1016/j.jcf.2012.04.011.
55. Munck A., Bonacorsi S., Mariani-Kurkdjian P., Lebourgeois M., Gérardin M., Brahimi N., et al. Genotypic characterization of Pseudomonas aeruginosa strains recovered from patients with cystic fibrosis afterinitial and subsequent colonization. Pediatr Pulmonol 2001, 32:288-292.
56. Anstead, M, Heltshe S, Khan U, et al. Pseudomonas aeruginosa serology and risk for re-isolation in the EPIC trial. J Cyst Fibros in press, http://dx.doi.org/10.1016/j.jcf.2012.08.001. http://dx.doi.org/10.1016/j.jcf.2012.08.001.
57. Bellis G., Cazes M.H., Parant A., Gaimard M., Travers C., Le Roux E., et al. Cystic fibrosis mortality trends in France. J Cyst Fibros 2007, 6:179-186.
58. Guidelines to the ECFSPR http://www.ecfs.eu/projects/efcs-patient-registry/guidelines.
59. Flume P.A., O'Sullivan B.P., Robinson K.A., Goss C.H., Mogayzel P.J., Willey-Courand D.B., et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007, 176:957-969. [Epub 2007 Aug 29].
60. Flume P.A., Mogayzel P.J., Robinson K.A., Rosenblatt R.L., Quittell L., Marshall B.C., et al. Cystic fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med 2009, 180:802-808.
61. Ryan G., Singh M., Dwan K. Inhaled antibiotics for long-term therapy in cystic fibrosis. Cochrane Database Syst Rev 2011, 3:CD001021.
62. Chuchalin A., Csiszér E., Gyurkovics K., Bartnicka M.T., Sands D., Kapranov N., et al. Formulation of aerosolized tobramycin (Bramitob) in the treatment of patients with cystic fibrosis and Pseudomonas aeruginosa infection: a double-blind, placebo-controlled, multicenter study. Paediatr Drugs 2007, 9(Suppl. 1):21-31.
63. Geller D.E., Konstan M.W., Smith J., Noonberg S.B., Conrad C. Novel tobramycin inhalation powder in cystic fibrosis subjects: pharmacokinetics and safety. Pediatr Pulmonol 2007, 42:307-313.
64. Konstan M.W., Flume P.A., Kappler M., Chirond R., Higgins M., Brockhaus F., et al. Safety, efficacy and convenience of tobramycin inhalation powder in cystic fibrosis patients: the EAGER trial. J Cyst Fibros 2011, 10:54-61.
65. Konstan M.W., Geller D.E., Minić P., Brockhaus F., Zhang J., Angyalosi G. Tobramycin inhalation powder for P. aeruginosa infection in cystic fibrosis: the EVOLVE trial. Pediatr Pulmonol 2011, 46:230-238.
66. McCoy K.S., Quittner A.L., Oermann C.M., Gibson R.L., Retsch-Bogart G.Z., Montgomery A.B. Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis. Am J Respir Crit Care Med 2008, 178:921-928.
67. Retsch-Bogart G.Z., Quittner A.L., Gibson R.L., Oermann C.M., McCoy K.S., Montgomery A.B., et al. Efficacy and safety of inhaled aztreonam lysine for airway pseudomonas in cystic fibrosis. Chest 2009, 135:1223-1232.
68. Oermann C.M., Retsch-Bogart G.Z., Quittner A.L., Gibson R.L., McCoy K.S., Montgomery A.B., et al. An 18-month study of the safety and efficacy of repeated courses of inhaled aztreonam lysine in cystic fibrosis. Pediatr Pulmonol 2010, 45:1121-1134.
69. Wainwright C.E., Vidmar S., Armstrong D.S., Byrnes C.A., Carlin J.B., Cheney J., et al. Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial. JAMA 2011, 306:163-171.
70. Assael BM, Pressler T, Bilton D, Fayond M, Fischer R, Chiron R, et al. Inhaled aztreonam lysine vs. inhaled tobramycin in cystic fibrosis: A comparative efficacy trial. J Cyst Fibros in press, http://dx.doi.org/10.1016/j.jcf.2012.07.006. http://dx.doi.org/10.1016/j.jcf.2012.07.006.
71. Schuster A, Haliburn C, Döring G, et al. Safety, efficacy and convenience of colistimethate sodium dry powder for inhalation (Colobreathe DPI) in cystic fibrosis patients: a randomised study Thorax in press.
72. Meers P., Neville M., Malinin V., Scotto A.W., Sardaryan G., Kurumunda R., et al. Biofilm penetration, triggered release and in vivo activity of inhaled liposomal amikacin in chronic Pseudomonas aeruginosa lung infections. J Antimicrob Chemother 2008, 61:859-868.
73. Okusanya O.O., Bhavnani S.M., Hammel J., Minic P., Dupont L.J., Forrest A., et al. Pharmacokinetic and pharmacodynamic evaluation of liposomal amikacin for inhalation in cystic fibrosis patients with chronic pseudomonal infection. Antimicrob Agents Chemother 2009, 53:3847-3854.
74. Clancy J.P., Minic P., Dupont L., Goss C.H., Quittner A.L., Lymp J.F., et al. Full analyses of data from two phase II blinded and placebo-controlled studies of nebulized liposomal amikacin for inhalation (Arikace™) in the treatment of cystic fibrosis patients with chronic Pseudomonas aeruginosa lung infection. Pediatr Pulmonol 2010, 45:299-300.
75. Dorkin H., Criollo M., Reimnitz P., Alder J., Hampel B. Randomized, double-blind, placebo-controlled, multicenter study to evaluate the safety and efficacy of inhaled ciprofloxacin compared with placebo in patients with cystic fibrosis. Pediatr Pulmonol 2011, (Suppl. 34):A235.
76. Geller D.E., Flume P.A., Staab D., Fischer R., Loutit J.S., Conrad D.J. Levofloxacin inhalation solution (MP-376) in patients with cystic fibrosis with Pseudomonas aeruginosa. Am J Respir Crit Care Med 2011, 183:1510-1516. [Epub 2011 Feb 25].
77. Trapnell B.C., McColley S.A., Kissner D.G., Rolfe M.W., Rosen J.M., McKevitt M., et al. Fosfomycin/tobramycin for inhalation in patients with cystic fibrosis with pseudomonas airway infection. Am J Respir Crit Care Med 2012, 185:171-178.
78. Ramsey B.W., Pepe M.S., Quan J.M., Otto K.L., Montgomery A.B., Williams-Warren J., et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. N Engl J Med 1999, 340:23-30.
79. Pamp S.J., Gjermansen M., Johansen H.K., Tolker-Nielsen T. Tolerance to the antimicrobial peptide colistin in Pseudomonas aeruginosa biofilms is linked to metabolically active cells, and depends on the pmr and mexAB-oprM genes. Mol Microbiol 2008, 68:223-240.
80. Herrmann G., Yang L., Wu H., Song Z., Wang H., Høiby N., et al. Colistin/tobramycin combinations are superior to monotherapy concerning the killing of biofilm P. aeruginosa. J Infect Dis October 13 2010, 202. 10.1086/656788.
81. Smyth A., Elborn J.S. Exacerbations in cystic fibrosis: 3-Management. Thorax 2008, 63:180-184.
82. Amadori A., Atonellia A., Balteria I., Schreiber A., Bugianib M., De Rose V. Recurrent exacerbations affect FEV1 decline in adult patients with cystic fibrosis. Respir Med 2009, 103:407-413.
83. Moskowitz S., Sliva S., Mayer-Hamblett N., Pasta D.J., Mink D.R., Mabie J.A., et al. Shifting patterns of inhaled antibiotic use in cystic fibrosis. Pediatr Pulmonol 2008, 43:874-881.
84. Razvi S., Saiman L. Microbiology of cystic fibrosis: role of the clinical microbiology laboratory, susceptibility and synergy studies and infection control. Cystic Fibrosis 2007, 123-133. Hodder Arnold, London. 3rd ed. M. Hodson, D. Geddes, A. Bush (Eds.).
85. Ciofu O., Fussing V., Bagge N., Koch C., Høiby N. Characterization of paired mucoid/non-mucoid Pseudomonas aeruginosa isolates fom Danish cystic fibrosis patients: antibiotic resistance, beta-lactamase activity and riboprinting. J Antimicrob Chemother 2001, 48:391-396.
86. Foweraker J.E., Laughton C.R., Brown D.F., Bilton D. Phenotypic variability of Pseudomonas aeruginosa in sputa from patients with acute infective exacerbation of cystic fibrosis and its impact on the validity of antimicrobial susceptibility testing. J Antimicrob Chemother 2005, 55:921-927.
87. Aaron S.D., Vandemheen K.L., Ferris W., Fergusson D., Tullis E., Haase D., et al. Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria: a randomised, double-blind, controlled clinical trial. Lancet 2005, 366:463-471.
88. Moskowitz S.M., Emerson J.C., McNamara S., Shell R.D., Orenstein D.M., Rosenbluth D., et al. Randomized trial of biofilm testing to select antibiotics for cystic fibrosis airway infection. Pediatr Pulmonol 2010, 6:184-192.
89. Smith A.L., Fiel S.B., Mayer-Hamblett N., Ramsey B., Burns J.L. Susceptibility testing of Pseudomonas aeruginosa isolates and clinical response to parenteral antibiotic administration: lack of association in cystic fibrosis. Chest 2003, 123:1495-1502.
90. Stenbit A.E., Flume P.A. Pulmonary exacerbations in cystic fibrosis. Curr Opin Pulm Med 2011, 17:442-447.
91. Sanders D.B., Bittner R.C., Rosenfeld M., Hoffman L.R., Redding G.J., Goss C.H. Failure to recover to baseline pulmonary function after cystic fibrosis pulmonary exacerbation. Am J Respir Crit Care Med 2010, 182:627-632.
92. Parkins M.D., Rendall J.C., Elborn J.S. Incidence and risk factors for pulmonary exacerbation treatment failures in cystic fibrosis patients chronically infected with Pseudomonas aeruginosa. Chest Aug 11 2011, [Epub ahead of print].
93. VanDevanter D.R., O'Riordan M.A., Blumer J.L., Konstan M.W. Assessing time to pulmonary function benefit following antibiotic treatment of acute cystic fibrosis exacerbations. Respir Res 2010, 11:137.
94. Ratjen F., Walter H., Haug M., Meisner C., Grasemann H., Döring G. Diagnostic value of serum antibodies in early Pseudomonas aeruginosa infection in cystic fibrosis patients. Pediatr Pulmonol 2007, 42:249-255.
95. Pressler T., Karpati F., Granström M., Knudsen P.K., Lindblad A., Hjelte L., et al. Diagnostic significance of measurements of specific IgG antibodies to Pseudomonas aeruginosa by three different serological methods. J Cyst Fibros 2009, 8:37-42.
96. Regelmann W.E., Elliott G.R., Warwick W.J., Clawson C.C. Reduction of sputum Pseudomonas aeruginosa density by antibiotics improves lung function in cystic fibrosis more than do bronchodilators and chest physiotherapy alone. Am Rev Respir Dis 1990, 141:914-921.
97. Tunney M.M., Klem E.R., Fodor A.A., Gilpin D.F., Moriarty T.F., McGrath S.J., et al. Use of culture and molecular analysis to determine the effect of antibiotic treatment on microbial community diversity and abundance during exacerbation in patients with cystic fibrosis. Thorax 2011, 10.1136/thx.2010.137281.
98. Meyer K.C., Lewandoski J.R., Zimmerman J.J., Nunley D., Calhoun W.J., Dopico G.A. Human neutrophil elastase and elastase/alpha1-antiprotease complex in cystic fibrosis - comparison with interstitial lung disease and evaluation of the effect of intravenously administered antibiotic therapy. Am Rev Respir Dis 1991, 144:580-585.
99. Tunney M.M., Field T.R., Moriarty T.F., Patrick S., Doering G., Muhlebach M.S., et al. Detection of anaerobic bacteria in high numbers in sputum from patients with cystic fibrosis. Am J Respir Crit Care Med 2008, 177:995-1001.
100. Rogers G.B., Marsh P., Stressmann A.F., Allen C.E., Daniels T.V.W., Carroll M.P., et al. The exclusion of dead bacterial cells is essential for accurate molecular analysis of clinical samples. Clin Microbiol Infect 2010, 16:1656-1658.
101. Fodor A.A., Klem E.R., Gilpin D.F., Elborn J.S., Boucher R.C., Tunney M.M., et al. The adult cystic fibrosis airway microbiota is stable over time and infection type, and highly resilient to antibiotic treatment of exacerbations. PLoS One 2012, 7:e45001. [Epub 2012 Sep 26]. 10.1371/journal.pone.0045001.
102. Blainey P.C., Milla C.E., Cornfield D.N., Quake S.R. Quantitative analysis of the human airway microbial ecology reveals a pervasive signature for cystic fibrosis. Sci Transl Med 2012, 4:153ra130.
103. Smith A.L., Doershuk C., Goldmann D., Gore E., Hilman B. Comparison of a beta-lactam alone versus beta-lactam and an aminoglycoside for pulmonary exacerbation in cystic fibrosis. J Pediatr 1999, 134:413-421.
104. Zhao J., Schloss P.D., Kalikin L.M., et al. Decade-long bacterial community dynamics in cystic fibrosis airway. PNAS 2012, 109:5809-5814.
105. Fibrosis Trust Cystic Antibiotic Treatment for Cystic Fibrosis - Report of the UK Cystic Fibrosis Antibiotic Working Group 2009, [http://www.cftrust.org.uk/aboutcf/publications/consensusdoc/Antibiotic_ treatment_for_Cystic_Fibrosis.pdf]. 3rd ed.
106. Sagel S.D., Gibson R.L., Emerson J., McNamara S., Burns J.L., Wagener J.S., et al. Impact of Pseudomonas and staphylococcus infection on inflammation and clinical status in young children with cystic fibrosis. J Pediatr 2009, 154:183-188.
107. Gangell C., Gard S., Douglas T., Park J., de Klerk N., Keil T., et al. Inflammatory responses to individual microorganisms in the lungs of children with cystic fibrosis. Clin Infect Dis 2011, 53:425-432.
108. Smyth A., Walters S. Prophylactic antibiotics for cystic fibrosis. The Cochrane Library 2001, 3. Update Software, Oxford.
109. Stutman H.R., Lieberman J.M., Nussbaum E., Marks M.I. Antibiotic prophylaxis in infants and young children with cystic fibrosis: a randomized controlled trial. J Pediatr 2002, 140:299-305.
110. Miall L.S., McGinley N.T., Brownlee K.G., Conway S.P. Methicillin resistant Staphylococcus aureus (MRSA) infection in cystic fibrosis. Arch Dis Child 2001, 84:160-162.
111. Dasenbrook E.C., Merlo C.A., Diener-West M., Lechtzin N., Boyle M.P. Persistent methicillin-resistant Staphylococcus aureus and rate of FEV1 decline in cystic fibrosis. Am J Respir Crit Care Med 2008, 178:814-821.
112. Sawicki G.S., Rasouliyan L., Pasta D.J., Regelmann W.E., Wagener J.S., Waltz D.A., et al. The impact of incident methicillin resistant Staphylococcus aureus detection on pulmonary function in cystic fibrosis. For the investigators and coordinators of the epidemiologic study of cystic fibrosis. Pediatr Pulmonol 2008, 43:1117-1123.
113. Dasenbrook E.C., Checkley W., Merlo C.A., Konstan M.W., Lechtzin N., Boyle M.P. Association between respiratory tract methicillin-resistant Staphylococcus aureus and survival in cystic fibrosis. JAMA 2010, 303:2386-2392.
114. Muhlebach M.S., Miller M., Lavange L.M., Mayhew G., Goodrich J.S., Miller M.B. Treatment intensity and characteristics of MRSA infection in CF. J Cyst Fibros 2011, 10:201-206.
115. Goss C.H., Muhlebach M.S. Staphylococcus aureus and MRSA in cystic fibrosis. J Cyst Fibros 2011, 10:298-306.
116. Vanderhelst E., De Meirleir L., Verbanck S., Pierard D., Vincken W., Malfroot A. Prevalence and impact on FEV1 decline of chronic methicillin-resistant Staphylococcus aureus (MRSA) infection in patients with cystic fibrosis. A single-center, case-control study of 165 patients. J Cyst Fibros 2012, 11:2-7.
117. Dutch CF database http://www.ncfs.nl/index.php?id=002546.
118. Talmaciu I., Varlotta L., Mortensen J., Schidlow D.V. Risk factors for emergence of Stenotrophomonas maltophilia in cystic fibrosis. Pediatr Pulmonol 2000, 30:10-15.
119. De Vrankrijker A.M.M., Wolfs T.F.W., Van der Ent C.K. Challenging and emerging pathogens in cystic fibrosis. Paediatr Respir Rev 2010, 11:246-254.
120. Karpati F., Malmborg A.-S., Alfredsson H., Hjelte L., Strandvik B. Bacterial colonisation with Xanthomonas maltophilia - A retrospective study in a cystic fibrosis patient population. Infection 1994, 22:258-263.
121. Goss C.H., Otto K., Aitken M.L., Rubenfeld G.D. Detecting Stenotrophomonas maltophilia does not reduce survival of patients with cystic fibrosis. Am J Respir Crit Care Med 2002, 166:356-361.
122. Waters V., Yau Y., Prasad S., Lu A., Atenafu E., Crandall I., et al. Stenotrophomonas maltophilia in cystic fibrosis: serologic response and effect on lung disease. Am J Respir Crit Care Med 2011, 183:635-640.
123. LiPuma J. The changing microbial epidemiology in CF. Clin Microbiol Rev 2010, 23:299-323.
124. Nørskov-Lauritsen N., Johansen H.K., Fenger M.G., Nielsen X.C., Pressler T., Olesen H.V., et al. Unusual distribution of Burkholderia cepacia complex species in Danish cystic fibrosis clinics may stem from restricted transmission between patients. J Clin Microbiol 2010, 48:2981-2983.
125. Tullis E., Burns J., Retsch-Bogart G., Bresnik M., Henig N., Lewis S., et al. Aztreonam for inhalation solution (AZLI) in cystic fibrosis (CF) patients with chronic Burkholderia species (BURK) infection: final results from a randomized, placebo-controlled trial. J Cyst Fibros 2012, 11(S1):S11.
126. Olivier K.N., Weber D.J., Lee J.H., Handler A., Tudor G., Molina P.L., et al. Nontuberculous mycobacteria. Am J Respir Crit Care Med 2003, 167:835-840.
127. Fauroux B., Delaisi B., Clement A., Saizou C., Moissenet D., Truffot-Pernot C., et al. Mycobacterial lung disease in cystic fibrosis: a prospective study. Pediatr Infect Dis J 1997, 16:354-358.
128. Griffith D.E., Aksamit T., Brown-Elliott B.A., Catanzaro A., Daley C., Gordin F., et al. An official ATS/IDSA statement: diagnosis, treatment, and prevention of nontuberculous mycobacterial diseases. Am J Respir Crit Care Med 2007, 175:367-416.
129. Esther C.R., Henry M.M., Molina P.L., Leigh M.W. Nontuberculous mycobacterial infection in young children with cystic fibrosis. Pediatr Pulmonol 2005, 40:39-44.
130. Feldman C., Anderson R., Theron A., Mokgobu I., Cole P.J., Wilson R. The effects of ketolides on bioactive phospholipid-induced injury to human respiratory epithelium in vitro. Eur Respir J 1999, 13:1022-1028.
131. Yousef A.A., Jaffe A. The role of azithromycin in patients with cystic fibrosis. Paediatr Respir Rev 2010, 11:108-114.
132. Saiman L., Anstead M., Mayer-Hamblett N., Lands L.C., Kloster M., Hocevar-Trnka J., et al. Effect of azithromycin on pulmonary function in patients with cystic fibrosis uninfected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 2010, 303:1707-1715.
133. Tateda K., Comte R., Pechere J.C., Kohler T., Yamaguchi K., Van Delden C. Azithromycin inhibits quorum sensing in Pseudomonas aeruginosa. Antimicrob Agents Chemother 2001, 45:1930-1933.
134. Pérez-Martínez I., Haas D. Azithromycin inhibits expression of the GacA-dependent small RNAs RsmY and RsmZ in Pseudomonas aeruginosa. Antimicrob Agents Chemother 2011, 55:3399-3405.
135. Renna M., Schaffner C., Brown K., Shang S., Henao Tamayo M., Hegyi K., et al. Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection. J Clin Invest 2011, 121:3554-3563.
136. Fleet J, Guha K, Piper S, Banya W, Bilton D, Hodson ME. A retrospective analysis of the impact of azithromycin maintenance therapy on adults attending a UK cystic fibrosis clinic. J Cyst Fibros in press, http://dx.doi.org/10.1016/j.jcf.2012.05.010. http://dx.doi.org/10.1016/j.jcf.2012.05.010.
137. Rogers G.B., Hart C.A., Mason J.R., Hughes M., Walshaw M.J., Bruce K.D. Bacterial diversity in cases of lung infection in cystic fibrosis patients: 16S ribosomal DNA (rDNA) length heterogeneity PCR and 16S rDNA terminal restriction fragment length polymorphism profiling. J Clin Microbiol 2003, 41:3548-3558.
138. Rogers G.B., Carroll M.P., Serisier D.J., Hockey P.M., Jones G., Bruce K.D. Characterization of bacterial community diversity in cystic fibrosis lung infections by use of 16s ribosomal DNA terminal restriction fragment length polymorphism profiling. J Clin Microbiol 2004, 42:5176-5183.
139. Rogers G.B., Carroll M.P., Serisier D.J., Hockey P.M., Jones G., Kehagia V., et al. Use of 16S rRNA gene profiling by terminal restriction fragment length polymorphism analysis to compare bacterial communities in sputum and mouthwash samples from patients with cystic fibrosis. J Clin Microbiol 2006, 44:2601-2604.
140. Sibley C.D., Duan K., Fischer C., Parkins M.D., Storey D.G., Rabin H.R., et al. Discerning the complexity of community interactions using a Drosophila model of polymicrobial infections. PLoS Pathog 2008, 4:e1000184.
141. Worlitzsch D., Rintelen C., Bohm K., Wollschläger B., Merkel N., Borneff-Lipp M., et al. Antibiotic-resistant obligate anaerobes during exacerbations of cystic fibrosis patients. Clin Microbiol Infect 2009, 15:4544-4560.
142. Cox M.J., Allgaier M., Taylor B., Baek M.S., Huang Y.J., Daly R.A., et al. Airway microbiota and pathogen abundance in age-stratified cystic fibrosis patients. PLoS One 2010, 5:e11044. 10.1371/journal.pone.0011044.
143. Klepac-Ceraj V., Lemon K.P., Martin T.R., Allgaier M., Kembel S.V., Knapp A.A., et al. Relationship between cystic fibrosis respiratory tract bacterial communities and age, genotype, antibiotics and Pseudomonas aeruginosa. Environ Microbiol 2010, 12:1293-1303.
144. Ulrich M., Beer I., Braitmaier P., Dierkes M., Kummer F., Krismer B., et al. Relative contribution of Prevotella intermedia and Pseudomonas aeruginosa to lung pathology in airways of cystic fibrosis patients. Thorax 2010, 65:978-984.
145. De Vrankrijker A.M., van der Ent C.K., van Berkhout F.T., Stellato R.K., Willems R.J., Bonten M.J., et al. Aspergillus fumigatus colonization in cystic fibrosis: implications for lung function?. Clin Microbiol Infect 2011, 17:1381-1386.
146. Geller D.E., Kaplowitz H., Light M.J., Colin A.A. Allergic bronchopulmonary aspergillosis in cystic fibrosis: reported prevalence, regional distribution, and patient characteristics. Chest 1999, 116:639-646.
147. Mastella G., Rainisio M., Harms H.K., Hodson M.E., Koch C., Navarro J., et al. Allergic bronchopulmonary aspergillosis in cystic fibrosis. A European epidemiological study. Eur Respir J 2000, 16:464-471.
148. Sibley C.D., Parkins M.D., Rabin H.R., Duan K., Norgaard J.C., Surette M.G. A polymicrobial perspective of pulmonary infections exposes an
149. Eakin M.N., Bilderback A., Boyle M.P., Mogayzel P.J., Riekert K.A. Longitudinal association between medication adherence and lung health in people with cystic fibrosis. J Cyst Fibros 2011, 10:258-264. [Epub 2011 Mar 31].
150. Modi A.C., Quittner A.L. Barriers to treatment adherence for children with cystic fibrosis and asthma: what gets in the way?. J Pediatr Psychol 2006, 31:846-858.
151. Sawicki G.S., Rasouliyan L.R., McMullen A.H., Wagener J.S., McColley S.A., Pasta D.J., et al. Longitudinal assessment of health-related quality of life in an observational cohort of patients with cystic fibrosis. Pediatr Pulmonol 2011, 46:36-44.
152. Briesacher B.A., Quittner A.L., Saiman L., Sacco P., Fouayzi H., Quittell L.M. Adherence with tobramycin inhaled solution and health care utilization. BMC Pulm Med 2011, 11:5.
153. CHMP Guideline on the clinical development of medicinal products for the treatment of CF http://www.ema.europa.eu/docs/en_GB/document_library/Scientific_guidelin e/2009/12/WC500017055.pdf.
154. U.S. Department of Health and Human Services, Food and Drug Administration. Guidance for Industry: Developing Antimicrobial Drugs - General Considerations for Clinical Trials (draft guidance) http://www.fda.gov/downloads/Drugs/GuidanceComplianceRegulatoryInformati on/Guidances/ucm070983.pdf.
155. Que C., Cullinan P., Geddes D. Improving rate of decline of FEV1 in young adults with cystic fibrosis. Thorax 2006, 61:155-157.
156. Quittner A.L., Alpern A.N., Kimberg C.I. Integrating patient-reported outcomes into rearch and clinical practice. Kendig & Chernick's Disorders of the Respiratory Tract in Children 2012, 251-260. UK: Elsevier Inc., Oxford. 8th edition. R.W. Wilmott, T. Boat, A. Bush, V. Chernick, R. Deterding, F. Ratjen (Eds.).
157. Abbott J., Hart A., Havermans T., Matossian A., Goldbeck L., Barreto C., et al. Measuring health-related quality of life in clinical trials in cystic fibrosis. J Cyst Fibros 2011, 10:82-85.
158. Goss C.H., Quittner A.L. Patient-reported outcomes in cystic fibrosis. Proc Am Thorac Soc 2007, 4:378-386.
159. Bambha K., Kim W.R. Cost-effectiveness analysis and incremental cost-effectiveness ratios: uses and pitfalls. Eur J Gastroenterol Hepatol 2004, 16:519-526.
160. Simoens S. Factors affecting the cost effectiveness of antibiotics. Chemother Res Pract 2011, 10.1155/2011/249867.
161. Annemans L. Health economics for non-economists: an introduction to the concepts, methods and pitfalls of health economic evaluations 2011, Academia Press.
162. Drummond M., O'Brien B., Stoddart G., Torrance G., Drummond M., O'Brien B., et al. Methods for the economic evaluation of health care programmes 1987, Oxford University Press.
163. National Institute for Clinical Excellence Guide to the methods of technology appraisal 2004, NICE, London.
164. Sox H.C., Greenfield S. Comparative effectiveness research: a report from the Institute of Medicine. Ann Intern Med 2009, 151:203-205.
165. Thornton J., Elliot R.A., Tully M.P. Clinical and economic choices in the treatment of respiratory infections in cystic fibrosis: comparing hospital and home care. J Cyst Fibros 2005, 4:239-247.
166. Strandvik B., Hjelte L., Malmborg A.-S., Widén B. Home intravenous antibiotic treatment in cystic fibrosis. Acta Paediatr Scand 1992, 81:340-344.
167. Knudsen P.K., Olesen H.V., Høiby N., Johannesson M., Karpati F., Laerum B.N., et al. Differences in prevalence and treatment of Pseudomonas aeruginosa in cystic fibrosis centres in Denmark, Norway and Sweden. J Cyst Fibros 2009, 8:135-142.
168. Iles R., Legh-Smith J., Drummond M., Prevost A., Vowler S. Economic evaluation of tobramycin nebuliser solution in cystic fibrosis. J Cyst Fibros 2003, 2:120-128.
169. LeLorier J., Perreault S., Birnbaum H., Greenberg P., Sheehy O. Savings in direct medical costs from the use of tobramycin solution for inhalation in patients with cystic fibrosis. Clin Ther 2000, 22:140-151.
170. Schechter M.S. Benchmarking to improve the quality of cystic fibrosis care. Curr Opin Pulm Med 2012, 18:596-601. 10.1097/MCP.0b013e328358d533.
171. Baumann U., Stocklossa C., Greiner W., Graf von der Schulenburg J.-M., von der Hardt H. Cost of care and clinical condition in paediatric cystic fibrosis patients. J Cyst Fibros 2003, 2:84-90.
172. Dalbøge C., Pressler T., Høiby N., Nielsen K., Johansen H.K. A cohort study of the Copenhagen CF centre eradication strategy against Staphylococcus aureus in patients with CF. J Cyst Fibros 2012, 10.1016/j.cf.2012.06.005.