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.
New research pinpoints how immigrant microbes get to the lungs

New research pinpoints how immigrant microbes get to the lungs

New method that detects NMD efficiency could help in treatment of many diseases

New method that detects NMD efficiency could help in treatment of many diseases

Researchers developing compact respiratory assist device for children with lung failure

Researchers developing compact respiratory assist device for children with lung failure

UNC scientists isolate protein that could play role in asthmatics

UNC scientists isolate protein that could play role in asthmatics

Experts update cystic fibrosis guidelines for better diagnosis and personalized treatment

Experts update cystic fibrosis guidelines for better diagnosis and personalized treatment

First stem cell study could lead to development of therapy to reduce inflammation caused by CF

First stem cell study could lead to development of therapy to reduce inflammation caused by CF

Scientists identify potential therapeutic solutions to combat age-related fibrosis

Scientists identify potential therapeutic solutions to combat age-related fibrosis

GUMC researchers describe lab technique to fill gaps in clinical care and research

GUMC researchers describe lab technique to fill gaps in clinical care and research

Researchers explore ins and outs of genome sequencing in newborns

Researchers explore ins and outs of genome sequencing in newborns

HUB and Dutch health insurance companies to test use of organoid technology for CF treatment

HUB and Dutch health insurance companies to test use of organoid technology for CF treatment

UNC Catalyst aims to provide knowledge and research tools to tackle rare diseases

UNC Catalyst aims to provide knowledge and research tools to tackle rare diseases

Giving polycystic kidney disease the bump

Giving polycystic kidney disease the bump

Study reports volume and cost of in-home care for children with special medical conditions

Study reports volume and cost of in-home care for children with special medical conditions

Study tracks parents' unpaid time assisting children with special health care needs

Study tracks parents' unpaid time assisting children with special health care needs

Researchers identify protein that degrades and inhibits infectious biofilms

Researchers identify protein that degrades and inhibits infectious biofilms

New drug treatment can override genetic fault that causes choroideremia

New drug treatment can override genetic fault that causes choroideremia

Elucigene collaborates with Congenica to develop new NGS kit for cystic fibrosis

Elucigene collaborates with Congenica to develop new NGS kit for cystic fibrosis

New UGA research finds pathogen's motility activates immune response

New UGA research finds pathogen's motility activates immune response

New three-dimensional map of cystic fibrosis protein offers new insights to treating fatal disease

New three-dimensional map of cystic fibrosis protein offers new insights to treating fatal disease

MILabs to provide Adaptive X-ray CT system to UAB for advancing pulmonary research

MILabs to provide Adaptive X-ray CT system to UAB for advancing pulmonary research