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 13 days ago Drug news

Phase III ARRIVAL data on Kalydeco in cystic fibrosis published in The Lancet Respiratory Medicine.- Vertex Pharma.

Vertex Pharmaceuticals announced data from the ongoing Phase III, open-label ARRIVAL study and published online in The Lancet Respiratory Medicine...

Added 1 month ago Drug news

VX 445 + tezacaftor + ivacaftor combination for cystic fibrosis patients with a F508del mutation enters Phase III studies.- Vertex

Vertex Pharmaceuticals Incorporated announced that it is initiating two Phase III studies of VX 445, tezacaftor and ivacaftor as an...

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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 Knowledge Centre

Cystic Fibrosis Knowledge Centre

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

Hyperammonaemia

Hyperammonaemia

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

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Guidelines

Cystic fibrosis: diagnosis and management

This guideline covers diagnosing and managing cystic fibrosis. It specifies how to monitor the condition and manage the symptoms to improve quality of life.

Added 10 days ago

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 4 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 7 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 4 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 4 months ago

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CME

Cystic fibrosis: clinical review

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