Data from multiple sources - Curated by EPG Health - Last updated 06 June 2017

Cystic fibrosis

Cystic fibrosis (CF) is an autosomal recessive, life-limiting, genetically inherited disease. It is a multi-system disorder and results from one of several identified


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Latest news articles

Added 6 days ago Drug news

FDA approves Symdeko to treat cystic fibrosis F508del mutation,- Vertex.

Vertex Pharmaceuticals Incorporated announced that the FDA approved Symdeko (tezacaftor/ivacaftor and ivacaftor) for treating the underlying cause of cystic fibrosis...

Added 17 days ago Drug news

Triple combination regimens for next generation treatment of cystic fibrosis.

VX 659 and VX 445 enter phase III trials as triple therapies to treat cystic fibrosis withF508del gene mutation.

Added 1 month ago Drug news

First medicine for treatment of underlying cause cystic fibrosis approved.

EU approval for Orkambi to treat cystic fibrosis (F508del mutation) in children ages 6 through 11.

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Learning Zones

An Learning Zone (LZ) is an area of the site dedicated to providing detailed self-directed medical education about a disease, condition or procedure.

Cystic Fibrosis

Cystic Fibrosis

View disease awareness information, treatment options and European Cystic Fibrosis Society best practice guidelines.

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Hyperammonaemia can result in serious neurological damage or death. Could you recognise the signs and symptoms?

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European Cystic Fibrosis Society Standards of Care: Best Practice guidelines

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above...

Added 3 years ago

Culture-based diagnostic microbiology in cystic fibrosis: Can we simplify the complexity?

This review summarizes state-of-the art culture methods and makes recommendations for addition of non-culture based methods in the diagnostic laboratory.

Added 4 years ago

Consensus statement for inert gas washout measurement using multiple- and single-breath tests

Inert gas washout tests, performed using the single- or multiple-breath washout technique, were first described over 60 years ago. As...

Added 5 years ago

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Journal articles

Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles.

Background: To determine in vivo effects of CFTR modulators on mutation S945L.

Added 24 days ago

Pseudomonas aeruginosa in cystic fibrosis patients with c.1652G›A (G551D)-CFTR treated with ivacaftor-Changes in microbiological parameters.

The CFTR potentiator, ivacaftor (IVA), has been widely used in the treatment of cystic fibrosis (CF) patients with the G551D mutation. To date, there has been limited information on the microbiological status...

Added 24 days ago

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Cystic fibrosis: clinical review

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