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Review
. 2007 Dec 15;335(7632):1255-9.
doi: 10.1136/bmj.39391.713229.AD.

Cystic fibrosis

Jane C Davies  1 , Eric W F W Alton, Andrew Bush
Affiliations
Review

Cystic fibrosis

Jane C Davies et al. BMJ. .
No abstract available

PubMed Disclaimer

Conflict of interest statement

Competing interests: JCD and EWFWA are members of the UK Cystic Fibrosis Gene Therapy Consortium.

Figures

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Fig 1 Severe bronchiectasis in end stage cystic fibrosis shown in chest radiograph (top) and computed tomogram (bottom). For reasons that are not fully understood, the upper lobes are often most severely affected, although the patient has severe bronchiectasis throughout the whole of the right lung. Note presence of indwelling intravenous catheter (a “port-a-cath”) on the right lateral chest wall
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Fig 2 Finger clubbing indicates advance suppurative lung disease. It is not characteristic of asthma, with which older patients have sometimes been misdiagnosed
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Fig 3 Child’s arm during sweat test with the macroduct system. After pilocarpine iontophoresis to stimulate sweating, the closed capillary collecting system is applied to the skin of the forearm. Sweat can be seen entering the tubing (blue); electrolyte analysis can be reliably performed on as little as 50 μl of sweat
See this image and copyright information in PMC

References

    1. Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003. Eur Respir J 2007;29:522-6. - PubMed
    1. Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989;245:1066-73. - PubMed
    1. Matsui H, Grubb BR, Tarran R, Randell SH, Gatzy JT, Davis CW, et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell 1998;95:1005-15. - PubMed
    1. 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. - PubMed
    1. Davis PB. Cystic fibrosis since 1938. Am J Respir Crit Care Med 2006;173:475-82. - PubMed

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