http://www.sciencedaily.com/news/health_medicine/cystic_fibrosis/ Cystic Fibrosis (CF) Research News. Read the latest advances in treatment of cystic fibrosis. en-us Sat, 26 May 2012 13:05:01 EDT Sat, 26 May 2012 13:05:01 EDT 60 http://www.sciencedaily.com/images/logosmall.gif http://www.sciencedaily.com/news/health_medicine/cystic_fibrosis/ For more science articles, visit ScienceDaily. http://www.sciencedaily.com/releases/2012/05/120524092750.htm Researchers have discovered why females with cystic fibrosis do worse than males. The study is the first to show that the female hormone estrogen promotes the presence of a particular form of bacteria which results in more severe symptoms for female cystic fibrosis patients. In addition, females who were taking the oral contraceptive pill, which decreases the amount of naturally occurring estrogen in their bodies, were found to have lower levels of the problematic bacteria. Thu, 24 May 2012 09:27:27 EDT http://www.sciencedaily.com/releases/2012/05/120524092750.htm http://www.sciencedaily.com/releases/2012/05/120521104625.htm Inhaling concentrated saline (salt water) mist does not reduce how often infants and young children with cystic fibrosis need antibiotics for respiratory symptoms, according to new findings. This trial is the largest study of concentrated, or hypertonic, saline therapy in infants and preschoolers. Mon, 21 May 2012 10:46:46 EDT http://www.sciencedaily.com/releases/2012/05/120521104625.htm http://www.sciencedaily.com/releases/2012/04/120422162413.htm The bacterium Burkholderia cenocepacia is harmless in healthy people but causes a severe and persistent lung infection in cystic fibrosis patients and is resistant to nearly all known antibiotics. The bacterium interferes with an important survival process in cells whose job is to fight infection. This phenomenon is even stronger in CF patients, new research shows. Sun, 22 Apr 2012 16:24:24 EDT http://www.sciencedaily.com/releases/2012/04/120422162413.htm http://www.sciencedaily.com/releases/2012/04/120419191707.htm By developing software that uses 3-D models of proteins involved in cystic fibrosis, a team of scientists has identified several new molecules that may ease the symptoms of the disease. Thu, 19 Apr 2012 19:17:17 EDT http://www.sciencedaily.com/releases/2012/04/120419191707.htm http://www.sciencedaily.com/releases/2012/04/120401134927.htm The discovery by cystic fibrosis researchers offers the possibility of developing therapies to intervene in utero. Some of these genes may influence disease in other cystic fibrosis-affected organs. Sun, 01 Apr 2012 13:49:49 EDT http://www.sciencedaily.com/releases/2012/04/120401134927.htm http://www.sciencedaily.com/releases/2012/03/120312114121.htm Restoring normal function to the mutant gene product responsible for cystic fibrosis (CF) requires correcting two distinct structural defects, according to new research. This finding could point to more effective therapeutic strategies for CF in the future. Mon, 12 Mar 2012 11:41:41 EDT http://www.sciencedaily.com/releases/2012/03/120312114121.htm http://www.sciencedaily.com/releases/2012/01/120131171816.htm The FDA's approval of ivacaftor for treatment of cystic fibrosis will provide substantial benefit for CF patients with the G551D mutation in CFTR, say investigators who conducted clinical trials on the drug. Tue, 31 Jan 2012 17:18:18 EST http://www.sciencedaily.com/releases/2012/01/120131171816.htm http://www.sciencedaily.com/releases/2011/11/111102190402.htm A new study has confirmed that the drug, ivacaftor (VX-770), significantly improves lung function in some people with cystic fibrosis. The oral medicine targets the defective protein produced by the gene mutation called G551D that causes CF. Researchers found that patients carrying G551D -- approximately four per cent of all CF patients -- who were treated with VX-770 showed a 17 per cent relative improvement in lung function that was sustained over the course of 48 weeks. Wed, 02 Nov 2011 19:04:04 EDT http://www.sciencedaily.com/releases/2011/11/111102190402.htm http://www.sciencedaily.com/releases/2011/10/111012113355.htm If you smoke cigarettes, you have more in common with someone who has cystic fibrosis than you think. A new research report shows that smoking cigarettes affects the lungs in a way that is very similar to cystic fibrosis, a life threatening disease affecting the lungs and other organs. Wed, 12 Oct 2011 11:33:33 EDT http://www.sciencedaily.com/releases/2011/10/111012113355.htm http://www.sciencedaily.com/releases/2011/10/111005172645.htm A new study may lead to a way to prevent the progression, or induce the regression, of lung injury that results from use of the anti-cancer chemotherapy drug bleomycin. Pulmonary fibrosis caused by this drug, as well as idiopathic pulmonary fibrosis from unknown causes, affect nearly five million people worldwide. No therapy is known to improve the health or survival of patients. Wed, 05 Oct 2011 17:26:26 EDT http://www.sciencedaily.com/releases/2011/10/111005172645.htm http://www.sciencedaily.com/releases/2011/09/110919113838.htm Cystic fibrosis is caused by a genetic defect in a chloride channel called cystic fibrosis transmembrane conductase regulator. Although scientists do not fully understand how or why this defect occurs, researchers have found a promising clue: a protein called ubiquitin ligase Nedd4L. Mon, 19 Sep 2011 11:38:38 EDT http://www.sciencedaily.com/releases/2011/09/110919113838.htm http://www.sciencedaily.com/releases/2011/09/110919104805.htm When researchers discovered the primary genetic defect that causes cystic fibrosis (CF) back in 1989, they opened up a new realm of research into treatment and a cure for the disease. Since then, scientists have been able to clone the defective gene and study its effects in animals. Now researchers have developed a technique for observing the defects at work in human tissue donated by patients with CF. Mon, 19 Sep 2011 10:48:48 EDT http://www.sciencedaily.com/releases/2011/09/110919104805.htm http://www.sciencedaily.com/releases/2011/09/110901134638.htm Researchers have identified an unconventional path that may correct the defect underlying cystic fibrosis, according to a new study. This new treatment dramatically extends the lives of mice carrying the disease-associated mutation. Thu, 01 Sep 2011 13:46:46 EDT http://www.sciencedaily.com/releases/2011/09/110901134638.htm http://www.sciencedaily.com/releases/2011/08/110801122952.htm The clinical outcome is improved if patients with chronic lung diseases such as cystic fibrosis are treated long-term with the antibiotic azithromycin. However, azithromycin treatment in patients with cystic fibrosis as recently associated with increased infection with nontuberculous mycobacteria. Now, researchers have confirmed that long-term use of azithromycin by adults with cystic fibrosis is associated with infection with nontuberculous mycobacteria and identified an underlying mechanism. Mon, 01 Aug 2011 12:29:29 EDT http://www.sciencedaily.com/releases/2011/08/110801122952.htm http://www.sciencedaily.com/releases/2011/07/110712162818.htm Compared to a standard diagnostic procedure, infants with cystic fibrosis who received treatment based on a diagnostic procedure involving obtaining and culturing fluid samples from the lungs did not have a lower prevalence of lung-damaging infection or structural lung injury at 5 years of age, according to a new study. Tue, 12 Jul 2011 16:28:28 EDT http://www.sciencedaily.com/releases/2011/07/110712162818.htm http://www.sciencedaily.com/releases/2011/06/110627183934.htm Researchers have identified a new stem cell that participates in the repair of the large airways of the lungs, which play a vital role in protecting the body from infectious agents and toxins in the environment. Mon, 27 Jun 2011 18:39:39 EDT http://www.sciencedaily.com/releases/2011/06/110627183934.htm http://www.sciencedaily.com/releases/2011/06/110620095242.htm Scientists have developed a new treatment for cystic fibrosis. The new drug will benefit sufferers who have the ‘Celtic Gene’, a genetic mutation which is particularly common in Ireland. Mon, 20 Jun 2011 09:52:52 EDT http://www.sciencedaily.com/releases/2011/06/110620095242.htm http://www.sciencedaily.com/releases/2011/05/110527101233.htm A bacteria which infects people with cystic fibrosis could help combat other antibiotic-resistant microbes, according to new research. Scientists have discovered antibiotics from Burkholderia are effective against MRSA and even other cystic fibrosis infecting bacteria. Fri, 27 May 2011 10:12:12 EDT http://www.sciencedaily.com/releases/2011/05/110527101233.htm http://www.sciencedaily.com/releases/2011/05/110523121339.htm Scientists have pinpointed regions of the genome that contribute to the debilitating lung disease that is the hallmark of cystic fibrosis. Mon, 23 May 2011 12:13:13 EDT http://www.sciencedaily.com/releases/2011/05/110523121339.htm http://www.sciencedaily.com/releases/2011/05/110503132656.htm A small study of children and teens with cystic fibrosis (CF) shows that simple exercise, individually tailored to each patient's preference and lifestyle, can help improve lung function and overall fitness. Tue, 03 May 2011 13:26:26 EDT http://www.sciencedaily.com/releases/2011/05/110503132656.htm http://www.sciencedaily.com/releases/2011/04/110430133119.htm Video games controlled by the player's breath can encourage youths with cystic fibrosis to use techniques that can help keep their airways clearer, according to a new study. Sat, 30 Apr 2011 13:31:31 EDT http://www.sciencedaily.com/releases/2011/04/110430133119.htm http://www.sciencedaily.com/releases/2011/04/110424152447.htm An over-the-counter drug used to treat diarrhea combined with minocycline, an antibiotic used to treat bacterial infections, could one day change the lives of those living with cystic fibrosis. Sun, 24 Apr 2011 15:24:24 EDT http://www.sciencedaily.com/releases/2011/04/110424152447.htm http://www.sciencedaily.com/releases/2011/03/110316152947.htm Using a newly created pig model that genetically replicates the most common form of cystic fibrosis, researchers have now shown that the CF protein is "misprocessed" in the pigs and does not end up in the correct cellular location. This glitch leads to disease symptoms, including gastrointestinal abnormalities and lung disease in the pigs, which mimic CF in humans. Wed, 16 Mar 2011 15:29:29 EDT http://www.sciencedaily.com/releases/2011/03/110316152947.htm http://www.sciencedaily.com/releases/2011/02/110222092654.htm Scientists in the UK are using the latest DNA sequencing technology to understand the diversity of a bacterium that causes severe lung infection in cystic fibrosis patients. Tue, 22 Feb 2011 09:26:26 EST http://www.sciencedaily.com/releases/2011/02/110222092654.htm http://www.sciencedaily.com/releases/2011/01/110125160850.htm Researchers have discovered a new method to fight bacterial infections associated with contact lenses. The method may also have applications for bacterial infections associated with severe burns and cystic fibrosis. Tue, 25 Jan 2011 16:08:08 EST http://www.sciencedaily.com/releases/2011/01/110125160850.htm http://www.sciencedaily.com/releases/2010/12/101229142158.htm Researchers have discovered that a protein involved in cystic fibrosis also regulates inflammation and cell death in emphysema and may be responsible for other chronic lung diseases. The findings pave the way toward new treatments to prevent lung damage caused by infections or cigarette smoke in emphysema. Wed, 29 Dec 2010 14:21:21 EST http://www.sciencedaily.com/releases/2010/12/101229142158.htm http://www.sciencedaily.com/releases/2010/12/101217145643.htm Cystic fibrosis (CF) patients with normal to mildly impaired lung function may benefit from a new investigational drug designed to help prevent formation of the sticky mucus that is a hallmark of the disease, according to researchers involved in a phase 3 clinical trial of the drug. Called denufosol, the investigational medication can be given early in the CF disease process, and may help delay the progression of lung disease in these patients, the researchers found. Fri, 17 Dec 2010 14:56:56 EST http://www.sciencedaily.com/releases/2010/12/101217145643.htm http://www.sciencedaily.com/releases/2010/11/101117184405.htm An investigational drug targeting a defective protein that causes cystic fibrosis has been shown to improve lung function in a small study of CF patients. The drug, VX-770, appeared to improve function of what is known as CFTR -- the faulty protein responsible for CF. It is among the first compounds being developed for CF that specifically targets the root cause of cystic fibrosis. Wed, 17 Nov 2010 18:44:44 EST http://www.sciencedaily.com/releases/2010/11/101117184405.htm http://www.sciencedaily.com/releases/2010/11/101116181955.htm A common transmissible strain of the bacteria Pseudomonas aeruginosa has been identified among cystic fibrosis (CF) patients in Canada, suggesting that cross-infection has occurred widely between CF centers in the United Kingdom and Canada, according to a new study. Infection with this strain among Canadian CF patients has been associated with an increased risk of death or lung transplantation. Tue, 16 Nov 2010 18:19:19 EST http://www.sciencedaily.com/releases/2010/11/101116181955.htm http://www.sciencedaily.com/releases/2010/11/101113165437.htm Researchers have demonstrated that the gene mutated in cystic fibrosis not only controls traffic on the chloride highway, but also keeps the sodium highway from being overused. Sat, 13 Nov 2010 16:54:54 EST http://www.sciencedaily.com/releases/2010/11/101113165437.htm http://www.sciencedaily.com/releases/2010/10/101028113612.htm Researchers have generated 100 new lines of human induced pluripotent stem cells (iPSC) from individuals with lung diseases, including cystic fibrosis and emphysema. The new stem cell lines could possibly lead to new treatments for these debilitating diseases. The findings demonstrate the first time lung disease-specific iPSC have been created in a lab. Thu, 28 Oct 2010 11:36:36 EDT http://www.sciencedaily.com/releases/2010/10/101028113612.htm http://www.sciencedaily.com/releases/2010/10/101012114043.htm New research may move scientists closer to a cure for cystic fibrosis, one of the most common fatal genetic diseases. Tue, 12 Oct 2010 11:40:40 EDT http://www.sciencedaily.com/releases/2010/10/101012114043.htm http://www.sciencedaily.com/releases/2010/09/100917151848.htm Researchers have found a possible new target for fighting cystic fibrosis (CF) that could compensate for the lack of a functioning ion channel in affected CF-related cells. Fri, 17 Sep 2010 15:18:18 EDT http://www.sciencedaily.com/releases/2010/09/100917151848.htm http://www.sciencedaily.com/releases/2010/05/100520092938.htm An emerging population of middle-aged cystic fibrosis patients contains significantly more females and includes a large proportion of patients who lived for decades without a diagnosis or specialized care, according to new research. Thu, 20 May 2010 09:29:29 EDT http://www.sciencedaily.com/releases/2010/05/100520092938.htm http://www.sciencedaily.com/releases/2010/04/100428142250.htm Aided by a new experimental model, scientists are a step closer to understanding how cystic fibrosis (CF) causes lung disease in people with the condition. The findings could help improve treatments for lung disease, which causes most of the deaths and disability among people with CF. Wed, 28 Apr 2010 14:22:22 EDT http://www.sciencedaily.com/releases/2010/04/100428142250.htm http://www.sciencedaily.com/releases/2010/04/100426131435.htm Genetic mutation can disrupt the way human cells make proteins, which in turn leads to inherited disease. According to one researcher, scientists are closer than ever to producing drugs that fix this disrupted-protein pathway and drastically improving treatment of genetic disease. Mon, 26 Apr 2010 13:14:14 EDT http://www.sciencedaily.com/releases/2010/04/100426131435.htm http://www.sciencedaily.com/releases/2010/04/100419102916.htm A research team from Italy reported a case of a 23-year-old patient suffering from cystic fibrosis (CF) and Crohn's disease who was successfully treated with infliximab. This case report was thought to be one of the first regarding the use of biological therapy in patients with CF. Mon, 19 Apr 2010 10:29:29 EDT http://www.sciencedaily.com/releases/2010/04/100419102916.htm http://www.sciencedaily.com/releases/2010/03/100311202721.htm New research reveals Small Colony Variants (SCVs) of P. aeruginosa to be a hallmark of chronic infection in cystic fibrosis (CF) patients. Results suggest that SCV-mediated persistence might be a good target for antimicrobial chemotherapy. Thu, 11 Mar 2010 20:27:27 EST http://www.sciencedaily.com/releases/2010/03/100311202721.htm http://www.sciencedaily.com/releases/2010/02/100214143133.htm In a study that could lead to new therapeutic targets for patients with the cystic fibrosis, scientists have identified a defective signaling pathway that contributes to disease severity. Sun, 14 Feb 2010 14:31:31 EST http://www.sciencedaily.com/releases/2010/02/100214143133.htm http://www.sciencedaily.com/releases/2010/02/100209183238.htm Mutations of a common environmental pathogen that causes chronic lung disease in patients with cystic fibrosis are able to survive in oxygen-poor, nitrate-rich airway secretions. This same survival mechanism also enables the mutate bacteria to resist the effects of certain antibiotics -- even without any previous exposure to antibiotics. Tue, 09 Feb 2010 18:32:32 EST http://www.sciencedaily.com/releases/2010/02/100209183238.htm http://www.sciencedaily.com/releases/2010/01/100104114547.htm Researchers have created biodegradable nanosized particles that can easily slip through the body's sticky and viscous mucus secretions to deliver a sustained-release medication cargo. Mon, 04 Jan 2010 11:45:45 EST http://www.sciencedaily.com/releases/2010/01/100104114547.htm http://www.sciencedaily.com/releases/2009/12/091221130216.htm Researchers have clarified the structure of an enzyme that disturbs the communication processes between bacteria. By doing so they have laid the foundations for a new method of tackling bacterial infections such as cystic fibrosis. Mon, 21 Dec 2009 13:02:02 EST http://www.sciencedaily.com/releases/2009/12/091221130216.htm http://www.sciencedaily.com/releases/2009/12/091218083550.htm Scientists studying the protein that, when defective or absent, causes cystic fibrosis has made an important discovery about how that protein is normally controlled and under what circumstances it might go awry. Fri, 18 Dec 2009 08:35:35 EST http://www.sciencedaily.com/releases/2009/12/091218083550.htm http://www.sciencedaily.com/releases/2009/12/091206162959.htm In an encouraging new development, scientists have restored partial function to lung cells collected from patients with cystic fibrosis. While there is still much work to be done before the therapy can be tested in humans, the discovery opens the door to a new class of therapies for this and a host of other chronic diseases. Sun, 06 Dec 2009 16:29:29 EST http://www.sciencedaily.com/releases/2009/12/091206162959.htm http://www.sciencedaily.com/releases/2009/11/091104191823.htm Scientists in Belgium have successfully differentiated human embryonic stem cells (hESC) into major cell types of lung epithelial tissue using a convenient air-liquid interface. The technique could provide an alternative to lung transplants for patients with lung injury due to chronic pulmonary disease and inherited genetic diseases such as cystic fibrosis. Wed, 04 Nov 2009 19:18:18 EST http://www.sciencedaily.com/releases/2009/11/091104191823.htm http://www.sciencedaily.com/releases/2009/10/091022153646.htm The American Thoracic Society has released new clinical guidelines for the treatment of exacerbations in cystic fibrosis based on a review of the literature on current clinical practices. Thu, 22 Oct 2009 15:36:36 EDT http://www.sciencedaily.com/releases/2009/10/091022153646.htm http://www.sciencedaily.com/releases/2009/09/090914172332.htm Scientists have identified the main genetic switch that causes excessive mucus in the lungs, a discovery that one day could ease suffering for people with chronic lung disease or just those fighting the common cold. The discovery sheds light on the precise biological reasons the lungs in people with asthma, cystic fibrosis and other respiratory ailments clog with thick mucus. Mon, 14 Sep 2009 17:23:23 EDT http://www.sciencedaily.com/releases/2009/09/090914172332.htm http://www.sciencedaily.com/releases/2009/09/090910091342.htm Researchers are trying to invent a novel noninvasive lung test for cystic fibrosis sufferers. The goal is to find a method that requires a patient only to breath into a machine instead of undergo an invasive procedure. Thu, 10 Sep 2009 09:13:13 EDT http://www.sciencedaily.com/releases/2009/09/090910091342.htm http://www.sciencedaily.com/releases/2009/09/090908193426.htm New research could lead to earlier detection and diagnosis of cystic fibrosis liver disease and better treatment options for the patients affected by the disease. Tue, 08 Sep 2009 19:34:34 EDT http://www.sciencedaily.com/releases/2009/09/090908193426.htm http://www.sciencedaily.com/releases/2009/08/090825085954.htm A hallmark of cystic fibrosis, a disease caused by mutations in the CTFR gene, is the accumulation of abnormally thick and sticky mucus in the lung, intestine, and various other organs. Although the accumulation of this mucus is thought likely to play a central role in the development of disease, how mutations in the CTFR gene lead to mucus accumulation have not been determined. Scientists have now provided insight into this issue by studying mouse small intestine segments ex vivo. Tue, 25 Aug 2009 08:59:59 EDT http://www.sciencedaily.com/releases/2009/08/090825085954.htm http://www.sciencedaily.com/releases/2009/07/090730200632.htm Patients with cystic fibrosis usually experience significant muscle loss, a symptom traditionally considered to be a secondary complication of the devastating genetic disease. However, a recent study reverses the equation: new results show that muscle atrophy and weakness may be a primary symptom caused by the effects of CFTR gene mutations on the muscle itself. Thu, 30 Jul 2009 20:06:06 EDT http://www.sciencedaily.com/releases/2009/07/090730200632.htm http://www.sciencedaily.com/releases/2009/07/090721090134.htm Miglustat is a drug currently under phase 2 clinical trials on patients suffering from cystic fibrosis. Its potential for treating the disease was discovered in 2006 and researchers can now show that daily, long-term treatment of human cystic fibrosis cells with low doses of miglustat corrects the main pathological abnormalities. They are therefore extremely hopeful that miglustat will prove effective with patients, and become the first drug able to treat the disease rather than the symptoms. Tue, 21 Jul 2009 09:01:01 EDT http://www.sciencedaily.com/releases/2009/07/090721090134.htm http://www.sciencedaily.com/releases/2009/07/090720202603.htm Scientists have worked for 20 years to perfect gene therapy for the treatment of cystic fibrosis, which causes the body to produce dehydrated, thicker-than-normal mucus that clogs the lungs and leads to life threatening infections. Now scientists have found what may be the most efficient way to deliver a corrected gene to lung cells collected from cystic fibrosis patients. Mon, 20 Jul 2009 20:26:26 EDT http://www.sciencedaily.com/releases/2009/07/090720202603.htm http://www.sciencedaily.com/releases/2009/07/090716113243.htm Cystic fibrosis medicines that help to break down mucus in the lungs may carry an unexpected long-term benefit, a study suggests. The treatments not only help breathing in the short term -- they may also make lung infections develop to be less harmful in the long run, research shows. Thu, 16 Jul 2009 11:32:32 EDT http://www.sciencedaily.com/releases/2009/07/090716113243.htm http://www.sciencedaily.com/releases/2009/07/090701082704.htm An otherwise effective treatment for cystic fibrosis places patients at a high risk of sensorineural hearing loss, according to new research. Wed, 01 Jul 2009 08:27:27 EDT http://www.sciencedaily.com/releases/2009/07/090701082704.htm http://www.sciencedaily.com/releases/2009/04/090427102225.htm Three reports describing advances in cystic fibrosis genetic testing appear in the latest issue of The Journal of Molecular Diagnostics. Mon, 27 Apr 2009 10:22:22 EDT http://www.sciencedaily.com/releases/2009/04/090427102225.htm http://www.sciencedaily.com/releases/2009/04/090426082452.htm Researchers have identified Notch1 as a mediator of lung fibrosis. Scar tissue, or fibrosis, can accumulate in the lungs, restricting the flow of oxygen and leading to end-stage lung disease, respiratory failure, and eventually death. Sun, 26 Apr 2009 08:24:24 EDT http://www.sciencedaily.com/releases/2009/04/090426082452.htm http://www.sciencedaily.com/releases/2009/04/090424114653.htm Researchers have devised a new way to thwart the relentless bacterial infections that thrive in the lungs of people with cystic fibrosis, unlocking new possibilities against a tenacious and toxic hallmark of the common genetic disease. Fri, 24 Apr 2009 11:46:46 EDT http://www.sciencedaily.com/releases/2009/04/090424114653.htm http://www.sciencedaily.com/releases/2009/03/090323143910.htm Researchers have discovered a promising strategy for destroying the molecular scaffolding that can make Pseudomonas bacterial infections extremely difficult to treat in cystic fibrosis patients. Mon, 23 Mar 2009 14:39:39 EDT http://www.sciencedaily.com/releases/2009/03/090323143910.htm http://www.sciencedaily.com/releases/2009/03/090316173220.htm Immune cells once thought to be innocent bystanders in cystic fibrosis may hold the key to stopping patients' fatal lung disease. New findings show that white blood cells called neutrophils respond strongly to conflicting signals from cystic fibrosis patients' lungs, setting up a molecular fracas that may explain the patients' severe lung damage. Mon, 16 Mar 2009 17:32:32 EDT http://www.sciencedaily.com/releases/2009/03/090316173220.htm