BMJ 2007;335:1255-1259 (15 December), doi:10.1136/bmj.39391.713229.AD
Clinical review
Clinical Review
Cystic fibrosis
Jane C Davies, senior lecturer and honorary consultant1,2,
Eric W F W Alton, professor of gene therapy and professor of respiratory medicine1,3,
Andrew Bush, professor of paediatric respirology2
1 Department of Gene Therapy, Imperial College, London ,
2 Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London,
3 Department of Adult Respiratory Medicine, Royal Brompton Hospital, London
Correspondence to: J C Davies, Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London SW3 6NP j.c.davies@ic.ac.uk
| The first 150 words of the full text of this article appear below. |
Summary points
- Cystic fibrosis is the commonest inherited disease in white populations, with an incidence of 1 in 2500 newborns; over 7000 people in the United Kingdom currently have the disease
- Until recently, the diagnosis has been largely clinical, although the widespread implementation of a screening programme for newborns is now complete in the UK
- Cystic fibrosis is a multiorgan disease best managed in a multidisciplinary setting in conjunction with a specialist centre for cystic fibrosis, with treatment tailored to the individual
- The cornerstones of management are proactive treatment of airway infection and encouragement of good nutrition and an active lifestyle
- Conventional treatment has improved greatly over the past few decades; however, current treatments at best slow the decline in lung function. Newer approaches such as gene and small molecule based treatments may have more potential to halt disease progression
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Over 7000 people have cystic fibrosis in the United Kingdom. It is . . . [Full text of this article]
What is the cause of cystic fibrosis?
What are the clinical features and when should the diagnosis be considered?
How is cystic fibrosis diagnosed?
Management
Lung disease Extrapulmonary diseasePsychological issues
Sources and selection criteriaOngoing researchAdditional educational resources

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