Cystic Fibrosis News and Research RSS Feed - Cystic Fibrosis News and Research

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 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 M√ľnchen 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]
New x-ray imaging system helps monitor effectiveness of treatment for cystic fibrosis

New x-ray imaging system helps monitor effectiveness of treatment for cystic fibrosis

Scientists have developed an x-ray imaging system that enables researchers to see 'live' how effective treatments are for cystic fibrosis. [More]
Researchers develop new lung-on-chip model for laboratory studies of respiratory challenges

Researchers develop new lung-on-chip model for laboratory studies of respiratory challenges

Researchers at RTI International, in collaboration with the University of North Carolina at Chapel Hill, have developed a new lung-on-chip microdevice for laboratory studies of respiratory challenges and therapeutics. The microdevice includes multiple vertically stacked cellular layers that mimic the structure of the airway tissue. [More]
Researchers create synthetic ion transporter that triggers apoptosis in cancer cells

Researchers create synthetic ion transporter that triggers apoptosis in cancer cells

Researchers from the University of Southampton are part of an international team that has helped to create a molecule that can cause cancer cells to self-destruct by carrying sodium and chloride ions into the cells. [More]
Researchers create synthetic molecule that can cause cancer cells to self-destruct

Researchers create synthetic molecule that can cause cancer cells to self-destruct

Researchers from The University of Texas at Austin and five other institutions have created a molecule that can cause cancer cells to self-destruct by ferrying sodium and chloride ions into the cancer cells. [More]
Research report on global market for respiratory devices

Research report on global market for respiratory devices

The worldwide respiratory device market continues to grow despite economic downturns mainly due to the aging of the world population and increasing incidence of COPD. [More]
Experts explain pros and cons of over-the-counter enzymes

Experts explain pros and cons of over-the-counter enzymes

Enzyme supplements available without a prescription are becoming increasingly popular, but should everyone add them to their shopping list? Brent Bauer, M.D., director of the Mayo Clinic Complementary and Integrative Medicine Program, is co-author of a new paper in the medical journal Mayo Clinic Proceedings on the pros and cons of over-the-counter enzymes. [More]
Computational biologists develop program for easy diagnosis of hereditary illnesses

Computational biologists develop program for easy diagnosis of hereditary illnesses

In the case of a cough or a sore throat, the doctor can usually diagnose a common cold immediately. However, the diagnosis of hereditary illnesses like cystic fibrosis, which affects the metabolism, or Huntington's disease, which leads to cognitive decline, is much more complex. [More]

NC Senate plan to overhaul state Medicaid program draws fire from doctors and hospitals

The North Carolina plan was presented by state senators during a Wednesday meeting of the Senate Rules and Operations Committee. [More]
First Edition: July 17, 2014

First Edition: July 17, 2014

Today's headlines include reports about how Capitol Hill politics are impacting health care policy. Houston Public Media's Carrie Feibel, working in partnership with Kaiser Health News and NPR, reports: "Primary care doctors have reported problems making referrals for patients who have purchased some of the cheaper plans from the federal insurance marketplace. [More]
PTC Therapeutics initiates reimbursed expanded access program for Translarna

PTC Therapeutics initiates reimbursed expanded access program for Translarna

PTC Therapeutics, Inc. today announced the initiation of a reimbursed expanded access program (EAP). PTC's EAP program is intended to make Translarna (ataluren) available to patients before commercial availability in certain countries. [More]
Children who undergo simple emergency surgeries on weekends are more likely to die

Children who undergo simple emergency surgeries on weekends are more likely to die

Children who undergo simple emergency surgeries, such as hernia repairs or appendix removals, on weekends are more likely to suffer complications and even die than children getting the same kind of treatment during the week, according to results of a Johns Hopkins Children's Center study. [More]