71
13. Castellani C, Southern K, et al. European best practice
guidelines for cystic fibrosis neonatal screening. J Cyst Fibros
2009; 8:153-173.
14. Button BM, Wilson C, et al. Physiotherapy for Cystic Fibrosis
in Australia and New Zealand: a Clinical Practice Guideline.
Respirology 2016; 21: 656-667.
15. Fuchs HJ, Borowitz DS, Christiansen DH, et al. Effect of
aerosolized recombinant human DNase on exacerbations of
respiratory symptoms and on pulmonary function in patients
with cystic fibrosis. The Pulmozyme Study Group. N Engl J Med
1994; 331:637-42.
16. Flume PA, O’Sullivan BP, Robinson KA, et al. Cystic fibrosis
pulmonary guidelines: chronic medications for maintenance
of lung health. Am J Respir Crit Care Med 2007; 176:957-69.
17. Suri R, Grieve R, Normand C, et al. Effects of hypertonic saline,
alternate day and daily rhDNase on healthcare use, costs
and outcomes in children with cystic fibrosis. Thorax 2002;
57:841-46.
18. Elkins MR, Robinson M, Rose BR, et al. A controlled trial of
long-term inhaled hypertonic saline in patients with cystic
fibrosis. N Engl J Med 2006; 354:229-40.
19. Rosenfeld M, Ratjen F, Brumback L, et al. Inhaled hypertonic
saline in infants and childrenyounger than 6 years with
cystic fibrosis: the ISIS randomized controlled trial. JAMA
2012;307:2269-77.
20. Rosenfeld M, Gibson RL, McNamara S, et al. Early pulmonary
infection, inflammation, and clinical outcomes in infants with
cystic fibrosis. Pediatr Pulmonol 2001; 32:356-366.
21. Gibson RL, Burns JL, Ramsey BW. Pathophysiology and
management of pulmonary infections in cystic fibrosis. Am J
Respir Crit Care Med 2003; 168:918-51.
22. Emerson J, Rosenfeld M, McNamara S, et al. Pseudomonas
aeruginosa and other predictors of mortality and morbidity in
young children with cystic fibrosis. Pediatr Pulmonol 2002;
34:91-100.
23. Ratjen F, Munck A, Kho P, et al. Treatment of early Pseudomonas
aeruginosa infection in patients with cystic fibrosis: the ELITE
trial. Thorax 2010; 65:286-91.
24. Treggiari MM, Retsch-Bogart G, 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- 56
25. Konstan MW, Flume PA, et al. One-year safety and efficacy
of tobramycin powder for inhalation in patients with cystic
fibrosis. Pediatr Pulmonol 2016; 51:372-78.
26. Sanders DB, Bittner RC, et al. Pulmonary exacerbations are
associated with subsequent FEV1 decline in both adults and
children with cystic fibrosis. Pediatr Pulmonol 2011;46:393-
400.
27. Rosenfeld M, Emerson J, et al. Defining a pulmonary
exacerbation in cystic fibrosis. J Pediatr 2001; 139:359-65.
28. Borowitz D, Robinson KA, et al. Cystic Fibrosis Foundation
evidence-based guidelines for management of infants with
cystic fibrosis. J Pediatr 2009;155:S73-93.
29. Lahiri T, Hempstead SE, Brady C, et al. Clinical Practice
Guidelines From the Cystic Fibrosis Foundation for Preschoolers
With Cystic Fibrosis. Pediatrics 2016;137 (4):e20151784.
30. Mogayzel PJ, Edward T, Cystic Fibrosis Pulmonary Guidelines,
Chrinic Medications for Maintenance of Lung Health. Am J
Respir Crit Care Med 2013; 187,680–689.
31. Smyth AR, Bell SC, et al. European Cystic Fibrosis Society
Standards of Care: Best Practice guidelines. Journal of Cystic
Fibrosis 2014; 13 S23–S42.
32. Equi A, Balfour-Lynn IM, Long term azithromycin in children
with cystic fibrosis: a randomised, placebo-controlled
crossover trial. Lancet 2002; 360:978-84.
33. Johnson C, Butler SM, et al. Factors influencing outcomes
in cystic fibrosis: a centerbased analysis. Chest 2003;123:
20–27.
[MANEJO ACTUAL DE LA FIBROSIS QUÍSTICA - Dr. Óscar Fielbaum]