Further reading

Data sources


  • Allen J, Panitch H, Rubenstein R. Cystic Fibrosis. Lung Biology in Health and Disease. Vol 242. New York, Informa Healthcare USA, 2010.
  • Hodson M, Geddes D, Bush A, eds. Hodson and Geddes’s Cystic Fibrosis. 4th Edn. London, Hodder Arnold, 2013.
  • Mogayzel PJ, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2013; 187: 680–689.

Diagnosis of CF

  • De Boeck K, Wilschanski M, Castellani C, et al. Cystic fibrosis: terminology and diagnostic algorithms. Thorax 2006; 61: 627–635.
  • Farrell PM, Rosenstein BJ, White TB, et al. Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation Consensus Report. J Pediatr 2008; 153: S4–S14.
  • Ooi CY, Dupuis A, Ellis L, et al. Comparing the American and European diagnostic guidelines for cystic fibrosis: same disease, different language? Thorax 2012; 67: 618–624.
  • Simmonds NJ, Bush A. Diagnosing cystic fibrosis: what are we sweating about? Thorax 2012; 67: 571–573.


  • Nagano Y, Elborn JS, Millar BC, et al. Development of a novel PCR assay for the identification of the black yeast, Exophiala (Wangiella) dermatitidis from adult patients with cystic fibrosis (CF). J Cyst Fibros 2008; 7: 576–580.
  • Rogers GB, Daniels TW, Tuck A, et al. Studying bacteria in respiratory specimens by using conventional and molecular microbiological approaches. BMC Pulm Med 2009; 9: 14.
  • Tunney MM, Field TR, Moriarty TF, 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.


  • Bobadilla JL, Macek M, Fine JP, et al. Cystic fibrosis: a worldwide analysis of CFTR mutations – correlation with incidence data and applicability to screening. Hum Mutat 2002; 19: 575–606.
  • Dodge JA, Lewis PA, Stanton M, et al. Cystic fibrosis mortality and survival in the UK: 1947–2003. Eur Respir J 2007; 29: 522–526.
  • McCormick J, Mehta G, Olesen HV, et al. Comparative demographics of the European cystic fibrosis population: a cross-sectional database analysis. Lancet 2010; 375: 1007–1013.
  • Sims EJ, Mugford M, Clark A, et al. Economic implications of newborn screening for cystic fibrosis: a cost of illness retrospective cohort study. Lancet 2007; 369: 1187–1195.
  • Verma N, Bush A, Buchdahl R. Is there still a gender gap in cystic fibrosis? Chest 2005; 128: 2824–2834.

CF-related diabetes

  • Milla CE, Billings J, Moran A. Diabetes is associated with dramatically decreased survival in female but not male subjects with cystic fibrosis. Diabetes Care 2005; 28: 2141–2144.
  • Moran A, Dunitz J, Nathan B, et al. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care 2009; 32: 1626–1631.

Novel therapies

  • Bush A, Simmonds NJ. Hot off the breath: ‘I’ve a cost for’ – the 64 million dollar question. Thorax 2012; 67: 382–384.
  • Clancy JP, Rowe SM, Accurso FJ, et al. Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax 2012; 67: 12–18.
  • Elborn JS. Personalised medicine for cystic fibrosis: treating the basic defect. Eur Respir Rev 2013; 22: 3–5.
  • Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 1663–1672.
  • Rosenfeld M, Ratjen F, Brumback L, et al. Inhaled hypertonic saline in infants and children younger than 6 years with cystic fibrosis: the ISIS randomized controlled trial. JAMA 2012; 307: 2269–2277.

See the entire Cystic Fibrosis Chapter