Respiratory Problems
Study shows treatment for genetically caused emphysema is effective
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A landmark clinical study in the Lancet provides convincing evidence that a frequently overlooked therapy for genetically-caused emphysema is effective and slows the progression of lung disease.
Alpha-1 antitrypsin deficiency is an inherited disorder that can cause emphysema even without exposure to tobacco smoke. Alpha-1 antitrypsin (AAT) is a protein made in the liver that protects the lungs. With this disorder, the AAT protein builds up in liver cells and doesn’t reach the lungs to protect them. Augmentation therapy involves regular infusions of purified AAT protein to raise the level of the protein in the blood and lungs. Although the therapy has been available for more than 25 years, it has seen limited use because doctors have been unsure that it works.
The study, “Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trail,” published online today will change how clinicians understand this treatment and encourage them to consider its early use before the condition causes severe emphysema.
New method enables drug target validation for COPD treatment
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Together with clinical partners, a team led by Dr. Dr. Melanie Königshoff and the doctoral student Franziska Uhl at the Comprehensive Pneumology Center of Helmholtz Zentrum München have investigated, for the first time, the suitability of Wnt/beta-catenin
activation to initiate repair in patient-derived COPD lung tissue. To achieve this, the researchers used a variety of chemical, biological and imaging techniques.
“In our study we showed that activation of the Wnt / beta-catenin ** - signaling pathway induces lung tissue repair, depending on the patient’s stage of COPD,” said Königshoff. The method developed by her team represents a powerful new tool for pathological assessment, drug validation, and mechanistic studies in patient-derived lung tissue, which will open novel avenues for successful clinical translation and precision medicine.
New method represents important advancement
“Previously, studies largely relied on animal models, and cell cultures in the petri dish were limited to two dimensions and individual time points,” said Uhl.
Garlic extract could help cystic fibrosis patients fight infection
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A chemical found in garlic can kill bacteria that cause life-threatening lung infections in people with cystic fibrosis, research suggests.
The study is the first to show that the chemical - known as allicin - could be an effective treatment against a group of infectious bacteria that is highly resistant to most antibiotics.
Allicin is produced naturally by garlic bulbs to ward off a closely-related group of plant pathogens found in soil and water habitats. In the 1980s, the bacteria - known as the Burkholderia cepacia complex (Bcc) - emerged as a cause of serious and transmissible lung infections in people with cystic fibrosis.
Measures to limit the spread of Bcc infections among people with cystic fibrosis have brought the number of cases down considerably. However, current therapies available to treat infections - that are potentially fatal - are limited and require the use of combinations of three to four antibiotics at a time.
New technique provides novel approach to diagnosing ciliopathies
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Cilia, the cell’s tails and antennas, are among the most important biological structures. They line our windpipe and sweep away all the junk we inhale; they help us see, smell and reproduce. When a mutation disrupts the function or structure of cilia, the effects on the human body are devastating and sometimes lethal.
The challenge in diagnosing, studying and treating these genetic disorders, called ciliopathies, is the small size of cilia—about 500-times thinner than a piece of paper. It’s been difficult to examine them in molecular detail until now.
Professor Daniela Nicastro and postdoctoral fellow Jianfeng Lin have captured the highest-resolution images of human cilia ever, using a new approach developed jointly with Lawrence Ostrowski and Michael Knowles from the University of North Carolina School of Medicine. They reported on the approach in a recent issue of Nature Communications.
About 20 different ciliopathies have been identified so far, including primary ciliary dyskinesia (PCD) and polycystic kidney disease (PKD), two of the most common ciliopathies. They are typically diagnosed through genetic screening and examination of a patient’s cilia under a conventional electron microscope.
Innovative Experiment Aims to Boost Lung Transplants
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The pair of lungs sits inside a clear dome, gently inflating as doctors measure how well they’ll breathe if implanted into a patient who desperately needs a new set.
It’s a little-known twist of nature - your lungs can live on for a while after you die. The air left inside keeps them from deteriorating right away as other organs do.
An innovative experiment now aims to use that hour or more window of time to boost lung transplants by allowing donations from people who suddenly collapse and die at home instead of in a hospital.
“There aren’t enough lungs. We’re burying them,” said Dr. Thomas Egan of the University of North Carolina, Chapel Hill, who is leading the project. “It turns out your lungs don’t die when you do.”
CPAP improves work productivity for sleep apnea patients
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The study will be presented today (11 April 2013) at the Sleep and Breathing Conference in Berlin, organised by the European Respiratory Society and the European Sleep Research Society.
Previous research has demonstrated that people with sleep apnoea are less productive at work, usually due to excessive daytime sleepiness. This study aimed to assess whether continuous positive airway pressure (CPAP) improved productivity at work.
The researchers used the Endicott Work Productivity Scale, a questionnaire designed to assess productivity at work, and the Epworth Sleepiness Scale, a globally accepted questionnaire which assesses daytime sleepiness. Included were 45 patients with sleep apnoea of working age (between 40 and 56 years), who completed the questionnaires at the outset and after 3 months of CPAP treatment.
The results showed that 35 of the patients who had good adherence to CPAP treatment showed significant improvement in their productivity at work and in their daytime sleepiness. The 10 patients who did not follow the treatment programme had no significant improvement in symptoms or work productivity.
New Mayo Software Identifies and Stratifies Risk Posed by Lung Nodules
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A multidisciplinary team of researchers at Mayo Clinic has developed a new software tool to noninvasively characterize pulmonary adenocarcinoma, a common type of cancerous nodule in the lungs. Results from a pilot study of the computer-aided nodule assessment and risk yield (CANARY) are published in the Journal of Thoracic Oncology.
“Pulmonary adenocarcinoma is the most common type of lung cancer and early detection using traditional computed tomography (CT) scans can lead to a better prognosis,” says Tobias Peikert, M.D., a Mayo Clinic pulmonologist and senior author of the study. “However, a subgroup of the detected adenocarcinomas identified by CT may grow very slowly and may be treatable with less extensive surgery.”
CANARY can noninvasively stratify the risk lung adenocarcinomas pose by characterizing the nodule as aggressive or indolent with high-sensitivity, specificity and predictive values.
Researchers explore PKC role in lung disease
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New research examines the role of PKC in airway smooth muscle contraction and raises the possibility that this enzyme could be a therapeutic target for treating asthma, COPD, and other lung diseases.
In the lungs, pathological increases in the contraction of the smooth muscle cells (SMCs) lining airway walls- a process that decreases airflow - contribute to the chain of events leading to asthma and COPD, two common lung diseases. Jose Perez-Zoghbi and colleagues from Texas Tech University Health Sciences Center designed a series of experiments to investigate the role of the enzyme PKC in this process. The results, which appear in The Journal of General Physiology, provide new insight into the mechanisms involved in regulating luminal diameter of small airways and reveal PKC as a potential target for drug therapies.
The researchers used phase-contrast video microscopy, confocal microscopy, Western blot analysis, and pharmacological activators and inhibitors to investigate the role of PKC in airway SMC contraction in mouse lung slices.
Breathing program may held save newborns’ lives: studies
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Training midwives and other birth attendants to help babies start breathing immediately after birth if they need help may prevent stillbirths and newborn deaths in the developing world, according to two U.S. studies.
So-called birth asphyxia - when babies are born not breathing - is one of the major causes of newborn death in regions with limited resources, said researchers whose work appeared in Pediatrics.
Reducing infant mortality in the developing world is one of the United Nations Millennium Development Goals - but progress has been slow, according to Jeffrey Perlman from Weill Cornell Medical College in New York, who helped implement the Helping Babies Breathe program in Tanzania.
Vitamin D Improves Exercise Outcomes in Patients with COPD
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Vitamin D supplements may help patients with chronic obstructive pulmonary disease (COPD) get more from their pulmonary rehabilitation programs, according to a study conducted by researchers from Belgium.
The study results will be presented at the ATS 2011 International Conference in Denver.
“Our study shows that high doses of vitamin D supplementation on top of a standard rehabilitation program improve the outcome in terms of exercise capacity and respiratory muscle strength,” said Miek Hornikx, physiotherapist and doctoral student in the department of pneumology at the Katholieke Universiteit Leuven in Leuven, Belgium.
CPAP improves daytime sleepiness even in patients with low levels of symptoms
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Continuous positive airway pressure, or CPAP, can increase alertness and even improve quality of life for sufferers of obstructive sleep apnea (OSA), even if their symptoms are minimal, according to a study conducted by researchers in Europe. Patients enrolled in the study reported an improvement in daytime sleepiness within six months of beginning CPAP treatment.
The study will be presented at the ATS 2011 International Conference in Denver.
“Treatment with CPAP clearly reduces daytime sleepiness and improves quality of life in patients with very limited symptoms, at a rate of about half the improvement seen in patients with more severe symptoms,” said Sonya Craig, research fellow at Churchill Hospital, Oxford.
Anti-Clotting Agent Does Not Improve Outcomes of Patients with Severe Pneumonia
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Use of the blood clot-inhibiting medication tifacogin does not appear to improve outcomes of patients with severe community-acquired pneumonia (sCAP), according to a study conducted by researchers from North and South America, Europe, Asia and Africa. The drug had shown some potential benefit in the sCAP subgroup of an earlier trial involving sepsis patients.
The findings were published online ahead of the print edition of the American Thoracic Society’s American Journal of Respiratory and Critical Care Medicine.
“Administration of tifacogin showed no treatment benefit in this large population of patients with severe CAP,”said Richard Wunderink, MD, professor of pulmonary and critical care medicine at Northwestern University’s Feinberg School of Medicine. “This result was consistent across a range of disease severity indices.”
New test announced for major killer of lung transplant patients
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A lung transplant can mean a new chance at life. But many who receive one develop a debilitating, fatal condition that causes scar tissue to build up in the lungs and chokes off the ability to breathe.
University of Michigan researchers hope a new diagnostic tool they developed to predict bronchiolitis obliterans syndrome (BOS) will allow doctors to intervene earlier and, ultimately, to provide life-saving treatments.
BOS is the leading cause of death for those who survive one year after lung transplantation and more than half of recipients will develop BOS within five years. There is currently no cure.
Acid suppressive medication may increase risk of pneumonia
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Using acid suppressive medications, such as proton pump inhibitors and histamine2 receptor antagonists, may increase the risk of developing pneumonia, states an article in CMAJ (Canadian Medical Association Journal).
Acid suppressive drugs are the second leading medication worldwide, totaling over US$26 billion in sales in 2005. Recently, medical literature has looked at unrecognized side effects in popular medications and their impact on public health.
This systematic review, which incorporated all relevant studies on the association of acid suppressive medications and pneumonia that could be identified to August 2009, showed that out of every 200 inpatients being treated with acid suppressive medication one will develop pneumonia.
Cystic Fibrosis Gene Typo Is a Double Whammy
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An imbalance of salt and water in patients with cystic fibrosis makes their lungs clog up with sticky mucus that is prone to infection. The cause of the offending imbalance is a well-known genetic error, one that blocks the molecular expressway for tiny chloride ions to move across the surface of the lungs.
But how does that same gene mutation upset a parallel roadway controlling the flow of the other component of salt, sodium ions? Now, researchers at the University of North Carolina at Chapel Hill School of Medicine have found the answer, demonstrating that the gene mutated in cystic fibrosis not only controls traffic on the chloride highway, but also keeps the sodium highway from being overused.
The finding suggests that the infamous mutation – in a gene called CFTR – is a double whammy, affecting the flow of two different ions that are important to keep the mucus on the surfaces of the airways hydrated. Clarifying this link between the genetic defect and the thick sticky mucus in cystic fibrosis lungs could help researchers develop better therapies.
