Cystic Fibrosis News and Research

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Cystic fibrosis is a life threatening, inherited disease of the exocrine glands. The condition primarily affects the digestive and respiratory systems which become clogged with a thick, sticky mucus.

Cystic fibrosis is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene which results in an excess of salt and water passing into cells, causing a thick, sticky mucus to build up in bodily passageways.

In the airways, this mucus causes a persistent cough, wheezing, breathlessness, and repeated lung infections. In the digestive tract, the tubes that carry digestive enzymes from the pancreas into the small intestine become blocked, preventing the proper absorption of food nutrients. This can lead to poor weight gain, intestinal blockage (particularly in newborns), and foul-smelling, greasy stools.

There is no cure for cystic fibrosis, so treatment aims to ease symptoms and make the condition easier to live with. Bronchodilators may be used to help expand the airways, antibiotics to treat chest infections and physiotherapy can help expel mucus from the lungs.
The condition is most common in white people of northern European descent and is estimated to occurs in 1 in every 2,500 babies born in the UK, where babies are screened at birth for cystic fibrosis as part of the National Health Service newborn screening programme.
ERT’s Centralized LCI Services improve data quality to measure disease progression in respiratory clinical trials

ERT’s Centralized LCI Services improve data quality to measure disease progression in respiratory clinical trials

Study provides new understanding of silent gene changes and genetic complexity of cystic fibrosis

Study provides new understanding of silent gene changes and genetic complexity of cystic fibrosis

FDA expands approval for cystic fibrosis drug to treat additional rare gene mutations

FDA expands approval for cystic fibrosis drug to treat additional rare gene mutations

Scientists grow and purify earliest lung progenitors from human stem cells

Scientists grow and purify earliest lung progenitors from human stem cells

Shire and Parion Sciences to collaborate on P-321 for ophthalmic indications

Shire and Parion Sciences to collaborate on P-321 for ophthalmic indications

New technique dramatically accelerates search for new cancer treatments

New technique dramatically accelerates search for new cancer treatments

Researchers discover molecular compound that targets nucleus of allergen-sensitized cells

Researchers discover molecular compound that targets nucleus of allergen-sensitized cells

TSRI scientists develop easy method for synthesizing compounds with potential to treat lung disorders

TSRI scientists develop easy method for synthesizing compounds with potential to treat lung disorders

New strategy in antimicrobial peptides holds promise for combating multi-drug resistant bacteria

New strategy in antimicrobial peptides holds promise for combating multi-drug resistant bacteria

Researchers discover how fat soluble vitamins may offer viable solution for treating cystic fibrosis

Researchers discover how fat soluble vitamins may offer viable solution for treating cystic fibrosis

New UBC research reveals clues to how bacteria proliferates in lungs of cystic fibrosis patients

New UBC research reveals clues to how bacteria proliferates in lungs of cystic fibrosis patients

Stem cell therapy can reduce lung inflammation in mouse model of COPD and cystic fibrosis

Stem cell therapy can reduce lung inflammation in mouse model of COPD and cystic fibrosis

Scientists map three-dimensional structure of protein that causes cystic fibrosis

Scientists map three-dimensional structure of protein that causes cystic fibrosis

Researchers uncover major clue to how mucus becomes abnormal in CF airways

Researchers uncover major clue to how mucus becomes abnormal in CF airways

Phage therapy could offer safe and effective treatment option for Cystic Fibrosis lung infections

Phage therapy could offer safe and effective treatment option for Cystic Fibrosis lung infections

McMaster researchers find new way to combat world's worst infectious diseases

McMaster researchers find new way to combat world's worst infectious diseases

Innovative ways to target antibiotic resistance in short term

Innovative ways to target antibiotic resistance in short term

Researcher examines scarring process in hope of preventing chronic lung transplant rejection

Researcher examines scarring process in hope of preventing chronic lung transplant rejection

New report expresses ethical concerns about use of non-invasive prenatal testing

New report expresses ethical concerns about use of non-invasive prenatal testing

Diagnosing infections through molecules in patient breath

Diagnosing infections through molecules in patient breath