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1. Cystic Fibrosis Foundation patient registry: annual data report 2011. Cystic Fibrosis Foundation. 2012. http://www.cff.
Registry.pdf. Accessed March 26, 2014.
2. Doring G, Flume P, Heijerman H, et al. Treatment of lung
infection in patients with cystic fibrosis: current and future
strategies. J Cyst Fibros 2012;11:461–79.
3. Rosenfeld M, Gibson RL, McNamara S, et al. Early pulmonary infection, inflammation, and clinical outcomes
in infants with cystic fibrosis. Pediatr Pulmonol 2001;32:
4. Emerson J, Rosenfeld M, McNamara S, et al. Pseudomonas aeruginosa and other predictors of mortality and
morbidity in young children with cystic fibrosis. Pediatr
5. Sagel SD, Gibson RL, Emerson J, et al. Impact of
Pseudomonas and Staphylococcus infection on inflammation
and clinical status in young children with cystic fibrosis. J
6. Stenbit AE, Flume PA. Pulmonary exacerbations in cystic
fibrosis. Curr Opin Pulm Med 2011;17:442–7.
7. Kraynack NC, Gothard MD, Falletta LM, McBride JT. Approach to treating cystic fibrosis pulmonary exacerbations
varies widely across US CF care centers. Pediatr Pulmonol 2011;6:870–81.
8. Bhatt JM. Treatment of pulmonary exacerbations in cystic
fibrosis. Eur Respir Rev 2013;22:205–16.
9. Bilton D, Canny G, Conway S, et al., Pulmonary exacerbation: towards a definition for use in clinical trials. Report from the EuroCareCF Working Group on outcome
parameters in clinical trials. J Cyst Fibros 2011; 10 Suppl
10. Flume PA, Mogayzel PJ Jr, Robinson KA, et al. Cystic
fibrosis pulmonary guidelines: treatment of pulmonary exacerbations. Am J Respir Crit Care Med 2009;180:802–8.
11. Abbott J, Holt A, Hart A, et al. What defines a pulmonary
exacerbation? The perceptions of adults with cystic fibrosis. J Cyst Fibros 2009;8:356–9.
12. Roderfeld M, Rath T, Schulz R, et al. Serum matrix metal-loproteinases in adult CF patients: Relation to pulmonary
exacerbation. J Cyst Fibros 2009;8:338–47.
13. Reid DW, Misso N, Aggarwal S, et al. Oxidative stress and
lipid-derived inflammatory mediators during acute exacer-
bations of cystic fibrosis. Respirology 2007;12:63–9.
14. Robroeks CM, Rosias PP, van Vliet D, et al. Biomarkers in
exhaled breath condensate indicate presence and severity of cystic fibrosis in children. Pediatr Allergy Immunol
15. Laguna TA, Wagner BD, Starcher B, et al. Urinary desmo-sine: a biomarker of structural lung injury during CF pulmonary exacerbation. Pediatr Pulmonol 2012;47:856–63.
16. Harris WT, Muhlebach MS, Oster RA, et al. Plasma TGF-beta( 1) in pediatric cystic fibrosis: potential biomarker of
lung disease and response to therapy. Pediatr Pulmonol
17. Gray RD, et al. Sputum and serum calprotectin are useful biomarkers during CF exacerbation. J Cyst Fibros
18. Liou TG, Adler FR, Fitzsimmons SC, et al. Predictive
5-year survivorship model of cystic fibrosis. Am J Epide-miol 2001;153:345–52.
19. Simmonds NJ, D’Souza L, Roughton M, et al. Cystic fibrosis and survival to 40 years: a case-control study. Eur
Respir J 2010;36:1277–83.
20. Britto MT, Kotagal UR, Hornung RW, et al. Impact of recent
pulmonary exacerbations on quality of life in patients with
cystic fibrosis. Chest 2002;121:64–72.
21. Dobbin CJ, Bartlett D, Melehan K, et al. The effect of infective exacerbations on sleep and neurobehavioral function
in cystic fibrosis. Am J Respir Crit Care Med 2005;172:
22. Lieu TA, Ray GT, Farmer G, Shay GF. The cost of medical
care for patients with cystic fibrosis in a health maintenance organization. Pediatrics 1999;103:e72.
23. Ouyang L, Grosse SD, Amendah DD, Schechter MS.
Healthcare expenditures for privately insured people with
cystic fibrosis. Pediatr Pulmonol 2009;44:989–96.
24. Konstan MW, Wagener JS, Vandevanter DR, et al. Risk
factors for rate of decline in forced expiratory volume in one
second in children and adolescents with cystic fibrosis. J
Pediatr 2007;151:134–9, 139 e1.
25. Sanders DB, Bittner RC, Rosenfeld M, et al. Pulmonary
exacerbations are associated with subsequent FEV1 decline in both adults and children with cystic fibrosis. Pediatr
26. Amadori A, Antonell A, Balteri I, et al. Recurrent exacerbations affect FEV( 1) decline in adult patients with cystic
fibrosis. Respir Med 2009;103:407–13.
27. Waters V, Stanojevic S, Atenafu EG, et al. Effect of pulmonary exacerbations on long-term lung function decline in
cystic fibrosis. Eur Respir J 2012;40:61–6.
28. Sanders DB, Bittner RC, Rosenfeld M, et al. Failure to
recover to baseline pulmonary function after cystic fibro-
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