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 2 days ago Drug news

FDA approves Symdeko for use in children with cystic fibrosis who have two copies of the F508del-CFTR mutation or who at least one mutation in the CFTR gene.- Vertex

Vertex Pharmaceuticals Incorporated announced the FDA approved Symdeko (tezacaftor/ivacaftor and ivacaftor) for use in children with cystic fibrosis ages 6...

Added 25 days ago Drug news

Vertex has selected the triple combination of VX-445 (elexacaftor), tezacaftor and ivacaftor to submit for potential regulatory approvals for people ages 12 and older with cystic fibrosis.

Vertex Pharmaceuticals Incorporated announced that it has selected the triple combination of the next-generation corrector VX-445 (elexacaftor), tezacaftor and ivacaftor...

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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.

Inflammatory Bowel Disease Knowledge Centre

Inflammatory Bowel Disease Knowledge Centre

The Inflammatory Bowel Disease (IBD) Knowledge Centre contains key information relating to the epidemiology and pathophysiology of Crohn’s disease and ulcerative colitis, highlighting prevalence, impact and unmet needs and the underlying inflammatory processes that drive IBD, considering some of the key inflammatory pathways.

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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 1 year 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 5 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 8 years ago

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Journal articles

Inhaled hypertonic saline in preschool children with cystic fibrosis (SHIP): a multicentre, randomised, double-blind, placebo-controlled trial.

Background: Inhaled hypertonic saline enhances mucociliary clearance, improves lung function, and reduces pulmonary exacerbations in people with cystic fibrosis older than age 6 years. We aimed to assess the effect of inhaled...

Added 5 days ago

Augmentation of CFTR function in human bronchial epithelial cells via SLC6A14-dependent amino acid uptake: Implications for treatment of Cystic Fibrosis.

Rationale: SLC6A14 mediated L-arginine transport has been shown to augment the residual anion channel activity of the major mutant, F508del-CFTR, in the murine gastrointestinal tract. It is not yet known if this transporter...

Added 6 days ago

Disease progression in patients with cystic fibrosis treated with ivacaftor: Data from national US and UK registries.

Background: Ivacaftor is the first in a class of drugs, CFTR modulators, that target the underlying defect in cystic fibrosis (CF). This long-term observational safety study evaluated CF disease progression in patients treated...

Added 6 days ago

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Clinical trials

Saline Hypertonic in Preschoolers (SHIP)

The purpose of this study is to assess whether inhalation of 7% hypertonic saline (HS) twice daily for 48 weeks improves the lung clearance index by multiple breath nitrogen washout in comparison with inhalation of 0.9% isotonic...

Added 5 days ago

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CME

Cystic fibrosis: clinical review

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