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Cystic Fibrosis: Evolution from a Fatal Disease of Infancy with a Clear Phenotype to a Chronic Disease of Adulthood with Diverse Manifestations

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Abstract

Cystic fibrosis (CF) is the most common life-threatening genetic condition in the Caucasian population. Since the complete pathologic description of the disease in 1938, tremendous progress has been made in identifying its genetic basis, the pathophysiology of CF lung disease, and the development of new therapies. This issue of CRAI will review several areas of progress in our understanding and treatment of CF.

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References

  1. Andersen D (1938) Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathological study. Am J Dis Child 56:344–399

    Google Scholar 

  2. Davis PB (2001) Cystic fibrosis. Pediatr Rev 22:257–264

    Article  PubMed  CAS  Google Scholar 

  3. Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z et al (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245:1066–1073

    Article  PubMed  CAS  Google Scholar 

  4. Davis PB (2006) Cystic fibrosis since 1938. Am J Respir Crit Care Med 173:475–482

    Article  PubMed  Google Scholar 

  5. Cantin A (1995) Cystic fibrosis lung inflammation: early, sustained, and severe. Am J Respir Crit Care Med 151:939–941

    PubMed  CAS  Google Scholar 

  6. Khan TZ, Wagener JS, Bost T, Martinez J, Accurso FJ, Riches DW (1995) Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med 151:1075–1082

    PubMed  CAS  Google Scholar 

  7. Ferkol T, Rosenfeld M, Milla CE (2006) Cystic fibrosis pulmonary exacerbations. J Pediatr 148:259–264

    Article  PubMed  Google Scholar 

  8. Liou TG, Adler FR, FitzSimmons SC, Cahill BC, Hibbs JR, Marshall BC (2001) Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol 153:345–352

    Article  PubMed  CAS  Google Scholar 

  9. Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB et al (2006) A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 354:229–240

    Article  PubMed  CAS  Google Scholar 

  10. Quan JM, Tiddens HA, Sy JP, McKenzie SG, Montgomery MD, Robinson PJ et al (2001) A two-year randomized, placebo-controlled trial of dornase alfa in young patients with cystic fibrosis with mild lung function abnormalities. J Pediatr 139:813–820

    Article  PubMed  CAS  Google Scholar 

  11. Ramsey BW, Pepe MS, Quan JM, Otto KL, Montgomery AB, Williams-Warren J et al (1999) Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. Cystic Fibrosis Inhaled Tobramycin Study Group. N Engl J Med 340:23–30

    Article  PubMed  CAS  Google Scholar 

  12. Saiman L, Marshall BC, Mayer-Hamblett N, Burns JL, Quittner AL, Cibene DA et al (2003) Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA 290:1749–1756

    Article  PubMed  CAS  Google Scholar 

  13. Cystic Fibrosis Foundation Patient Registry (2006) Cystic Fibrosis Foundation, Bethesda, MD

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Correspondence to Clement L. Ren.

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Ren, C.L. Cystic Fibrosis: Evolution from a Fatal Disease of Infancy with a Clear Phenotype to a Chronic Disease of Adulthood with Diverse Manifestations. Clinic Rev Allerg Immunol 35, 97–99 (2008). https://doi.org/10.1007/s12016-008-8077-y

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  • DOI: https://doi.org/10.1007/s12016-008-8077-y

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