Data from multiple sources - Curated by Marshall Pearce - 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 17 days ago Drug news

Phase III studies of VX 661+ Kalydeco in cystic fibrosis published in NEJM.- Vertex Pharma.

Vertex Pharmaceuticals announced that the New England Journal of Medicine (NEJM) published two articles with results from two Phase III...

Added 6 months ago Drug news

Kalydeco is FDA approved for use in cystic fibrosis patients ages 2 and older who have one of 23 residual function mutations in the CFTR gene.- Vertex Pharma

Vertex Pharmaceuticals Incorporated announced that the FDA has approved Kalydeco (ivacaftor) for use in people with cystic fibrosis (CF) ages...

Added 7 months ago Drug news

Two Phase III studies reported of tezacaftor (VX-661) / ivacaftor combination of cystic fibrosis transmembrane conductance regulator (CFTR) gene affected patients.-Vertex

Vertex Pharmaceuticals Incorporated announced results from two Phase III studies of the tezacaftor (VX-661) / ivacaftor combination treatment that showed...

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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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Airway Disease

Airway Disease

The Airway Disease Learning Zone is an area where you can access the information about VisionAir and see video presentations from the forum. The Inhalation Technology page features a video presentation from the live broadcast presented by key faculty.

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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

Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis.

Background:  Cystic fibrosis is an autosomal recessive disease caused by mutations in the CFTR gene that lead to progressive respiratory decline. Some mutant CFTR proteins show residual function and...

Added 13 days ago

The safety of lumacaftor and ivacaftor for the treatment of cystic fibrosis.

Introduction: Lumacaftor-ivacaftor is indicated for treatment of cystic fibrosis (CF) in patients homozygous for the Phe-508del cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations.

Added 1 month ago

Lumacaftor/ivacaftor, a novel agent for the treatment of cystic fibrosis patients who are homozygous for the F580del CFTR mutation.

Introduction: Cystic Fibrosis (CF) is an autosomal recessive disease affecting up to 90,000 people worldwide. Approximately 73% of patients are homozygous for the F508del cystic fibrosis transmembrane conductance regulator...

Added 1 month ago

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Cystic fibrosis: clinical review

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