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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.
Antibiotic-resistant bacteria share resources to cause chronic infections, show studies

Antibiotic-resistant bacteria share resources to cause chronic infections, show studies

Antibiotic-resistant bacteria can share resources to cause chronic infections, Vanderbilt University investigators have discovered. [More]
Major scientific breakthrough in understanding retinal degenerative diseases that cause blindness

Major scientific breakthrough in understanding retinal degenerative diseases that cause blindness

An important scientific breakthrough by a team of IRCM researchers led by Michel Cayouette, PhD, is being published today by The Journal of Neuroscience. The Montréal scientists discovered that a protein found in the retina plays an essential role in the function and survival of light-sensing cells that are required for vision. [More]
Tannic acid may help ease impact of bacterial lung infections in CF patients

Tannic acid may help ease impact of bacterial lung infections in CF patients

By screening over 2,000 approved drugs and natural products, scientists have shown that tannic acid may help ease the impact of bacterial lung infections in cystic fibrosis patients. [More]
International Biophysics files patent protection for revolutionary AffloVest

International Biophysics files patent protection for revolutionary AffloVest

International Biophysics Corporation of Austin, Texas announces its filing of U.S. patent protection for its revolutionary AffloVest. The company has also recently confirmed exclusive ownership rights concerning the manufacture, sale and distribution of the AffloVest in its markets. [More]
Childhood eating disorders could have underlying psychological issues, researchers warn

Childhood eating disorders could have underlying psychological issues, researchers warn

Researchers at the University of Montreal and its affiliated CHU Sainte-Justine children's hospital are warning parents that difficult eaters could have underlying psychological issues, as they have found that restrictive behaviours can appear before puberty. [More]
IRCM researchers discover mechanism that promotes progression of medulloblastoma

IRCM researchers discover mechanism that promotes progression of medulloblastoma

Scientists at the IRCM discovered a mechanism that promotes the progression of medulloblastoma, the most common brain tumour found in children. The team, led by Fr-d-ric Charron, PhD, found that a protein known as Sonic Hedgehog induces DNA damage, which causes the cancer to develop. [More]
Allakos secures additional $10M investment to expand its development portfolio

Allakos secures additional $10M investment to expand its development portfolio

Allakos Inc. announced today that it has secured an additional $10 million investment from current investors to fund development of an additional therapeutic antibody. [More]
Leading Cystic Fibrosis experts call for greater research on antibacterial resistance

Leading Cystic Fibrosis experts call for greater research on antibacterial resistance

World leading Cystic Fibrosis experts, from Queen's University Belfast, have called for greater research to address the major concern of antibacterial resistance. [More]
Study demonstrates longer-term benefits of Lung Flute for COPD patients

Study demonstrates longer-term benefits of Lung Flute for COPD patients

Patients with chronic obstructive pulmonary disease (COPD) report improved symptoms and health status when they use a hand-held respiratory device called the Lung Flute-, according to a new study by the University at Buffalo. Usually caused by smoking, COPD, which includes chronic bronchitis and emphysema, is the third leading cause of death in the U.S. [More]

US children with cystic fibrosis have better lung function compared to UK counterparts

There are significant differences in the lung function of children and young adults in the United States (US) who have cystic fibrosis compared with those in the United Kingdom (UK). [More]

Sheffield Teaching Hospitals NHS Foundation Trust nominated twice for Health Service Journal Awards

SHEFFIELD Teaching Hospitals NHS Foundation Trust has been nominated twice for outstanding ideas improving patient care in the Health Service Journal Awards 2014 – one of the most coveted awards in healthcare. [More]
New method helps reveal secret dance of proteins

New method helps reveal secret dance of proteins

Staying clear of diseases requires that the proteins in our cells cooperate with one another. But, it has been a well-guarded secret how tens of thousands of different proteins find the correct dancing partners as they degrade and build up the human body, brain and nervous system. [More]
Parion Sciences receives $3 million CFFT award to support development of new CF treatment

Parion Sciences receives $3 million CFFT award to support development of new CF treatment

Parion Sciences today announced it has received a $3 million award from Cystic Fibrosis Foundation Therapeutics to support the development of P-1037, Parion's new investigational treatment for cystic fibrosis (CF). [More]
Researchers find new method to measure modified protein structures in biological sample

Researchers find new method to measure modified protein structures in biological sample

Cells regulate protein functions in a wide variety of ways, including by modifying the protein structure. In an instant, a protein can take on another form and perform no or even the "wrong" function: in humans, proteins that fold wrongly can cause serious diseases such as Alzheimer's, Parkinson's or cystic fibrosis. [More]

Scientists aim to develop rapid test system for immune dysfunction to treat cystic fibrosis

Cystic fibrosis (CF) is a frequent genetic disease affecting the lung and the gastrointestinal tract. Scientists from the Helmholtz Zentrum München now have shown that many of the adult patients with CF in addition lack a cell surface molecule, which is important for immune defence. The results have been published recently in the 'Journal of Molecular Medicine'. [More]
Researchers discover pattern of infection of bacterium responsible for causing severe lung infections

Researchers discover pattern of infection of bacterium responsible for causing severe lung infections

Researchers at the University of Liverpool's Institute of Infection and Global Health have discovered the pattern of infection of the bacterium responsible for causing severe lung infections in people with cystic fibrosis. [More]
White pill may help scientists learn why patients with cystic fibrosis have less exercise capacity

White pill may help scientists learn why patients with cystic fibrosis have less exercise capacity

A little white pill may help scientists learn why patients with cystic fibrosis have less exercise capacity than their peers, even if their lungs are relatively healthy. [More]
Young people with chronic illnesses face uncertainty when transitioning from pediatric to adult care

Young people with chronic illnesses face uncertainty when transitioning from pediatric to adult care

Young people with chronic illnesses face uncertainty when their care is transferred from pediatrics to adult-oriented health professionals. A new study in the Journal of Adolescent Health has found that one in five such young adults said the transfer of their care was unsatisfactory. [More]
AbbVie CF Scholarship program presents annual academic awards to 40 student scholars

AbbVie CF Scholarship program presents annual academic awards to 40 student scholars

AbbVie today announced the 40 outstanding students with cystic fibrosis (CF) who will each receive $2,500 to pursue their undergraduate or graduate school education during the 2014-2015 school year through the AbbVie CF Scholarship program. [More]
UI scientists discover that CF mucus abnormality is present at birth

UI scientists discover that CF mucus abnormality is present at birth

Mucus is key to keeping our lungs clean and clear of bacteria, viruses, and other foreign particles that can cause infection and inflammation. When we inhale microbes and dust, they are trapped in the mucus and then swept up and out of the lungs via a process called mucociliary transport. [More]