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Current Cystic Fibrosis News and Events, Cystic Fibrosis News Articles.
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Pulmonary artery thrombosis a complication of radiation therapy
According to ARRS' American Journal of Roentgenology, the imaging findings of in situ pulmonary artery thrombosis (PAT) associated with radiation therapy (RT) are different from those of acute pulmonary emboli and do not appear to embolize. Due to the differences in clinical prognosis and subsequent management strategies, in situ PAT associated with RT -- which has not previously been described in the English literature -- must be distinguished from pulmonary embolism. (2020-10-09)

Mechanical forces of biofilms could play role in infections
Studying bacterial biofilms, EPFL scientists have discovered that mechanical forces within them are sufficient to deform the soft material they grow on, e.g. biological tissues, suggesting a ''mechanical'' mode of bacterial infection. (2020-10-08)

Treating cystic fibrosis with mRNA therapy or CRISPR
The potential for treating cystic fibrosis (CF) using mRNA therapies or CRISPR gene editing is possible regardless of the causative mutation. CF clinical trials showing that a genotype-agnostic gene therapy for CF is possible (2020-10-08)

Igniting the synthetic transport of amino acids in living cells
Researchers from ICIQ's Ballester group and IRBBarcelona's Palacín group have published a paper in Chem showing how a synthetic carrier calix[4]pyrrole cavitand can transport amino acids across liposome and cell membranes bringing future therapies a step closer. (2020-10-06)

Gene expression altered by direction of forces acting on cell
Tissues and cells in the human body are subjected to a constant push and pull - strained by other cells, blood pressure and fluid flow, to name a few. The type and direction of the force on a cell alters gene expression by stretching different regions of DNA, researchers at University of Illinois Urbana-Champaign and collaborators in China found in a new study. (2020-10-01)

Landmark clinical trial shows effectiveness of oral antibiotics in treating cystic fibrosis condition
A major national study led by experts from Bristol and Nottingham has found that oral antibiotics are just as effective as intravenous antibiotics in killing a common germ that causes dangerous complications in cystic fibrosis (CF) patients. (2020-09-29)

A step toward helping patients breathe deeply
In a new study, researchers at La Jolla Institute for Immunology (LJI) report that a protein called TL1A drives fibrosis in several mouse models, triggering tissue remodeling, and making it harder for lungs and airways to function normally. (2020-09-24)

BU researchers discover how COVID-19 may trigger fatal levels of lung inflammation
Responding to the COVID-19 pandemic caused by the novel coronavirus, SARS-CoV-2, requires models that can duplicate disease development in humans, identify potential targets and enable drug testing. Specifically, access to primary human lung in vitro model systems is a priority since a variety of respiratory epithelial cells are the proposed targets of viral entry. (2020-09-21)

New high-speed test shows how antibiotics combine to kill bacteria
Researchers at Uppsala University have developed a new method to determine - rapidly, easily and cheaply - how effective two antibiotics combined can be in stopping bacterial growth. The new method is simple for laboratories to use and can provide greater scope for customising treatment of bacterial infections. The study is published in PLOS Biology. (2020-09-17)

New treatments for deadly lung disease could be revealed by 3D modeling
A 3D bioengineered model of lung tissue built by University of Michigan researchers is poking holes in decades worth of flat, Petri dish observations into how the deadly disease pulmonary fibrosis progresses. (2020-09-14)

Excessive lung release of neutrophil DNA traps may explain severe complications in Covid-19 patients
Researchers from the University of Liège (Belgium) has detected significant amounts of DNA traps in distinct compartments of the lungs of patients who died from Covid-19. These traps, called NETs, are released massively into the airways, the lung tissue and the blood vessels. Such excessive release could be a major contributor to severe disease complications leading to in-hospital death. These results are published this week in the Journal of Experimental Medicine. (2020-09-14)

Drugging the undruggable: Yale finds treatment path for muscular dystrophy
Researchers at Yale have identified a possible treatment for Duchenne muscular dystrophy (DMD), a rare genetic disease for which there is currently no cure or treatment, by targeting an enzyme that had been considered ''undruggable.'' The finding appears in the Aug. 25 edition of Science Signaling. (2020-09-11)

UNC researchers publish striking images of SARS-CoV-2 infected cells
The UNC School of Medicine laboratory of Camille Ehre, PhD, Assistant Professor of Pediatrics, produced striking images in respiratory tract cultures of the infectious form of the SARS-CoV-2 virus produced by infected respiratory epithelial cells. The New England Journal of Medicine featured this work in its 'Images in Medicine' section. (2020-09-10)

Rare immune cells drive gut repair
Scientists from King's College London have discovered an unexpected tissue reparative role for a rare immune cell type in the gut that could tip toward fibrosis or cancer if dysregulated. The breakthrough will have important implications for treating patients who suffer from inflammatory bowel diseases (IBD) such as Crohn's disease and ulcerative colitis. (2020-09-07)

New method of detecting illnesses including coronavirus and cystic fibrosis
A new and quicker method of diagnosing diseases in patients has been created by researchers. The team has developed a system of examining individual molecules to detect the presence of disease in blood. (2020-09-02)

Immune protein IL-17A responsible for lethal side effects of gastric cancer
The formation of scar tissue, or fibrosis, as gastric cancer disseminates throughout the peritoneum can be more lethal than the cancer itself and can interfere with chemotherapy. Researchers from Kanazawa University have now found that proinflammatory cytokine IL-17A from mast cells heavily influences the degree of fibrosis and causes structural changes in peritoneal cells. Preventing mast cells from releasing IL-17A may therefore be a promising treatment strategy for gastric cancer patients with peritoneal dissemination. (2020-08-26)

New insights into mechanism of therapy to reduce liver fat and prevent fibrosis
A team led by researchers at Massachusetts General Hospital (MGH) has taken an important step forward in the goal of developing a potential treatment for non-alcoholic fatty liver disease. (2020-08-26)

Researchers show children are silent spreaders of virus that causes COVID-19
Researchers provide critical data showing that children play a larger role in the community spread of COVID-19 than previously thought. (2020-08-20)

Research reveals cilia's role in cardiovascular functions and genetic diseases
Research from Chapman University discover ciliary extracellular-like vesicles (cELVs). Released by fluid-shear, cELVs act as nano-compartments within a cilium. Proteomic identification reveals the role of cELVs in human genetic diseases and cardiovascular functions. (2020-08-20)

Electronic consultations between primary providers and radiologists improve patient care
According to ARRS' American Journal of Roentgenology (AJR), the eConsult electronic consultation system allowed primary care providers to easily consult with radiologists, was perceived as high value by primary care providers, resulted in altered patient management, and avoided unnecessary imaging tests. ''We identified follow-up imaging of cystic lesions and imaging workup of pain in patients as opportunities for continuing medical education for primary care providers,'' the AJR authors wrote. (2020-08-19)

Protein produced by the nervous system may help treatments for inflammatory diseases
A Rutgers-led team discover a protein produced by nervous system may be key to treating inflammatory diseases like asthma, allergies, chronic fibrosis and chronic obstructive pulmonary disease (COPD) (2020-08-17)

Exercise induces secretion of biomarkers into sweat
The aim was to reveal the potential of microRNAs in sweat extracellular vesicles in monitoring exercise performance. (2020-08-12)

The CNIO pave the way for a future gene therapy to reverse pulmonary fibrosis associated with ageing
''Our results indicate that a new therapy may be developed to prevent the development of pulmonary fibrosis associated with ageing,'' says CNIO's Maria Blasco, principal investigator of the study * Lung tissue of patients with pulmonary fibrosis does not regenerate because the cells involved in lung generation have damaged telomeres, the ends of the chromosomes. The new study describes a gene therapy that activates the enzyme that repairs telomeres (2020-08-10)

Dietary control of the healing of injury-induced inflammation
The purpose of this review is to describe the molecular components of the Resolution Response and how different dietary factors can either optimize or inhibit their actions. (2020-08-10)

Researchers characterize important regulators of tissue inflammation, fibrosis and regeneration
Although macrophages (cells involved in the detection and destruction of bacteria and other harmful organisms as well as dead cells) are classified as immune cells functioning in the activation and resolution of tissue inflammation, it is now clear that they are critically involved in a variety of disease processes, such as chronic inflammatory diseases, tumor growth and metastasis and tissue fibrosis. (2020-08-10)

Change isn't a good thing for Japanese rheumatoid arthritis patients
Interstitial lung disease (ILD) is a common complication of rheumatoid arthritis (RA) that dramatically increases the risk of death. In a recent study led by Osaka University, researchers identified a genetic variant that increases the risk of ILD among Japanese RA patients, most likely by altering the expression of the RPA3 gene and triggering scarring of lung tissue. Identifying risk variants associated with RA-ILD will help unravel the underlying causes of disease in different populations. (2020-08-09)

Scientists discover curious clues in the war between cf bacteria
Several different kinds of bacteria can cause lung infections in people with cystic fibrosis. Pseudomonas aeruginosa, which can cause pneumonia, typically infects infants or young children and persists for life, while Burkholderia cepacia complex species only infect teenagers and adults. Although Burkholderia infections are rare, when they do take hold, they are deadly. Now, UNC School of Medicine scientists have discovered a reason for this pathogen's apparent age discrimination. (2020-08-07)

Pulmonary fibrosis treatment shows proof of principle
A pre-clinical study led by scientists at Cincinnati Children's demonstrates that in mice the drug barasertib reverses the activation of fibroblasts that cause dangerous scar tissue to build up in the lungs of people with idiopathic pulmonary fibrosis (IPF). (2020-08-06)

Osteopontin, a protein not always as bad as it is made out to be
In an article published in the prestigious journal Aging Cell, the UPV/EHU's Lipids & Liver research group shows that contrary to expectations, physiological levels in the liver of osteopontin, a multifunctional protein linked to various serious diseases, need to be maintained during ageing to prevent the progression of ageing-related, non-alcoholic fatty liver disease. (2020-08-05)

New insights into wound healing
Research from a multidisciplinary team led by Washington University may provide new insights into wound healing, scarring and how cancer spreads (2020-07-29)

Sweat science: Engineers detect health markers in thread-based, wearable sweat sensors
Engineers at Tufts University have created a first-of-its-kind, flexible electronic sensing patch that can be sewn into clothing to analyze sweat for multiple markers. The patch could be used to to diagnose and monitor acute and chronic health conditions or to monitor athletic performance. (2020-07-28)

Mix and match: New 3D cell culture model replicates fibrotic elements of pancreatic cancer
Pancreatic cancer is a deadly cancer characterized by prominent fibrosis, which plays a crucial role in disease progression and therapeutic resistance. Despite various efforts, experimental models to date have generally failed to fully recapitulate the extent of fibrosis in human tissue. Now, scientists at Okayama University, Japan, have reported a new method that uses a 3D cell culture technique to generate pancreatic cancer tissues in the laboratory with any given amount of fibrosis. (2020-07-22)

'Bystander' Cs meet their match in gene-editing technique
Biomolecular engineers at Rice University have developed new tools to increase the accuracy of CRISPR single-base editing to treat genetic diseases. (2020-07-15)

Cystic fibrosis: why so many respiratory complications?
Cystic fibrosis, one of the most common genetic diseases in Switzerland, causes severe respiratory and digestive disorders. Despite considerable therapeutic advances, this disease still reduces life expectancy, in particular due to life-threatening respiratory infections. Scientists from the University of Geneva (UNIGE) have discovered the reason for this large number of lung infections: a protein, Vav3. (2020-07-13)

Yale, Baylor study reveals new cell types in lethal lung disease
A research team from Yale and Baylor College of Medicine has completed the largest single-cell analysis to date of lungs affected by Idiopathic Pulmonary Fibrosis (IPF), revealing how cells change in response to the disease and identifying previously unknown cell types. The findings, published in the July 8 issue of Science Advances, deepen understanding of IPF and could improve treatment of it and other fibrotic lung diseases. (2020-07-12)

World-first technology breathes new life into cystic fibrosis detection and treatment
World-first research, led by Monash University, has developed radical non-invasive X-ray technology aimed at helping diagnose, treat and manage people with cystic fibrosis. (2020-07-12)

Researchers find promising therapy to fight epidemic of liver disease
In an effort to combat a growing worldwide epidemic of Nonalcoholic Fatty Liver Disease (NAFLD), scientists have discovered a new target and a new therapy that has shown promising results in preclinical mouse models, according to researchers at the University of Colorado Anschutz Medical Campus. (2020-07-08)

New clues to lung-scarring disease may aid treatment
Scientists at Vanderbilt University Medical Center (VUMC) and the Translational Genomics Research Institute (TGen) in Phoenix, Arizona, have discovered previously unreported genetic and cellular changes that occur in the lungs of people with pulmonary fibrosis (PF). (2020-07-08)

TGen-led study identifies unique cells that may drive lung fibrosis
This is one of the first comprehensive looks at lung cells using a technology called single-cell RNA sequencing. Instead of examining a mash-up of many cells from a tissue sample, single-cell sequencing allowed researchers in this study to closely examine the individual cells that make up the lungs; to identify their function, and ultimately understand the molecular changes that may be driving the disease. (2020-07-08)

Largest source of AATD stem cells collected
Researchers from the Center for Regenerative Medicine (CReM) at Boston University and Boston Medical Center (BMC) have assembled the largest repository of patient derived stem cells (iPSCs) from patients with alpha-1 antitrypsin deficiency (AATD). (2020-07-02)

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