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 1 month ago Drug news

Vertex Pharma initiates Phase III study for VX 659 + tezacaftor + ivacaftor to treat F508del mutation cystic fibrosis.

Vertex Pharmaceuticals Incorporated announced that it is initiating the first Phase III study of VX 659, tezacaftor and ivacaftor as...

Added 2 months 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 2 months 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.

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Learning Zones

An epgonline.org 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

Hyperammonaemia

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

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Guidelines

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 what was seen in the general population over the same period.

Added 3 years ago

End of life care for patients with cystic fibrosis

Palliative care is an approach that improves quality of life for patients and their families facing problems associated with a life-threatening illness. Care planning is particularly important in CF...

Added 6 years ago

Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease

Approximately 5–10% of cystic fibrosis (CF) patients develop multilobular cirrhosis during the first decade of life. Most CF patients later develop signs of portal hypertension with complications, mainly variceal bleeding.

Added 6 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 2 months 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 2 months ago

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CME

Cystic fibrosis: clinical review

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