Cystic fibrosis

doi:10.1136/bmj.39391.713229.AD

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 inheriteddisease in white populations, with an incidence of 1 in 2500newborns; over 7000 people in the United Kingdom currently havethe disease

Until recently, the diagnosis has been largelyclinical, although the widespread implementation of a screeningprogramme for newborns is now complete in the UK

Cystic fibrosisis a multiorgan disease best managed in a multidisciplinarysetting in conjunction with a specialist centre for cystic fibrosis,with treatment tailored to the individual

The cornerstonesof management are proactive treatment of airway infection andencouragement of good nutrition and an active lifestyle

Conventionaltreatment 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 treatmentsmay have more potential to halt disease progression

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 disease

Psychological issues

Sources and selection criteria
Ongoing research
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