Cystic Fibrosis Reading List

Outcomes of intensive care unit care in adults with cystic fibrosis. Sood et al. Am J Respir Crit Care Med 2001; 163: 335-338.
A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. Elkins et al. NEJM 2006; 354: 229-40.
Lung transplantation and end of life issues in cystic fibrosis. Aurora et al. Pediatric Respiratory Reviews 2000; 1: 114-120.
Cystic Fibrosis since 1938. P Davis. Am J of Repir Crit Care Med 2006; 173: 475-482.
Diagnostic sweat testing: the Cystic Fibrosis Foundation guidelines. LeGrys et al. The Journal of Pediatrics 2007; 151: 85-90.
Effect of high dose ibuprofen in patients with Cystic Fibrosis. Konstan et al. NEJM 1995; 332: 848-854.
Cystic Fibrosis Adult Care: Consensus Conference Report. Yankaskas et al. Chest 2004; 125: 1S-39S.
Cystic Fibrosis Pulmonary Guidelines: Pulmonary complications – hemoptysis and pneumothorax. Flume et al. Am J Respir Crit Care Med 2010; 182: 298-306.
Cystic Fibrosis Pulmonary Guidelines: Chronic medications for maintenance of lung health. Flume et al. Am J Respir Crit Care Med 2007; 176: 957-969.
Cystic Fibrosis – Mechanisms of Disease. Rave et al. NEJM 2005; 352: 1992-2001.
Seminar – Cystic Fibrosis. Ratjan et al. Lancet 2003; 361: 681-689.
Aztreonam Lysine for chronic airway Pseudomonas Aeruginosa in cystic fibrosis. McCoy et al. Am J Respir Crit Care Med 2008; 178: 921-928.
Susceptibility testing of Pseudomonas Aeruginosa isolates and clinical response to parenteral antibiotic administration: lack of association in cystic fibrosis. Smith et al. Chest 2003; 123: 1495-1502.
Aminoglycoside prescribing and surveillance in cystic fibrosis. Tan et al. Am J Respir Crit Care Med 2003; 167: 819-823.
Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. Fuchs et al. NEJM 1994; 331: 637-642.
Intermittent administration of inhaled TOBI in patients with cystic fibrosis. Ramsey et al. NEJM 1999; 340: 23-30.
Effects of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. Accurso et al. NEJM 2010; 363: 1991-2003.
Azithromycin in patients with CF chronically infected with Pseudomonas Aeruginosa. Saiman et al. JAMA 2003; 290:1749-1756.
Cystic fibrosis transmembrane conductance regulator. MH Akabas. The J of Biological Chemistry 2000; 275: 3729-3732.
Mucus clearance and lung function in cystic fibrosis with hypertonic saline. Donaldson et al. NEJM 2006; 354: 241-250.
Efficacy of Alendronate in adults with cystic fibrosis with low bone density. Aris et al. Am J of Respir Crit Care Med 2004; 169: 77-82.
Nontuberculous Mycobacterium. Olivier et al. Am J Respir Crit Care Med 2003; 167: 835-840.
Management of non-tuberculous mycobacteria in patients with cystic fibrosis. LM Quittell. Paediatr Respir Rev 2004; 5 Suppl A: S217-219.
Survival effect of lung transplantation among patient with cystic fibrosis. Liou et al. JAMA 2001; 286: 2683-2689.
Update in cystic fibrosis 2006. FJ Accurso. Am J Respir Crit Care Med 2007; 175: 754-757.
Pathogenesis of early lung disease in cystic fibrosis: a window of opportunity to eradicate bacteria. Starner et al. Ann Intern Med. 2005; 143: 816-822.

Cystic Fibrosis Reading List

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