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Cystic fibrosis

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35 papers 0 to 25 followers
Kamal Hamed, Laurie Debonnett
Chronic airway infection with Pseudomonas aeruginosa is a major cause of increased morbidity and mortality in patients with cystic fibrosis (CF). The development and widespread use of nebulized antibacterial therapies, including tobramycin inhalation solution (TIS), has led to improvements in lung function and quality of life. However, the use of nebulizers is associated with various challenges, including extended administration times and the need for frequent device cleaning and disinfection. Multiple therapies are required for patients with CF, which poses a considerable burden to patients, and adherence to the recommended treatments remains a challenge...
May 2017: Therapeutic Advances in Respiratory Disease
Sarah Averill, Meghan G Lubner, Christine O Menias, Sanjeev Bhalla, Vincent M Mellnick, Tabassum A Kennedy, Perry J Pickhardt
OBJECTIVE: There is an expanding and increasingly heterogeneous population of adult patients with cystic fibrosis (CF). Although CF is usually diagnosed in children with progressive multisystem involvement, up to 7% of CF cases are currently diagnosed de novo in adults with subtle manifestations distinct from the typical features of classic CF. The purpose of this article is to present the wide spectrum of CF in adults, including both classic and nonclassic variants, with an emphasis on the nonclassic imaging findings...
July 2017: AJR. American Journal of Roentgenology
William Flight, Andrew Jones
Respiratory viruses, such as those that cause influenza and the common cold, are a regular feature of life for the entire human population. Among people with CF, these viruses are associated with prolonged respiratory illness and show a clear association with pulmonary exacerbations which in turn are associated with lung function decline and risk of death. Human rhinovirus is the most commonly encountered respiratory viral pathogen in CF although adenovirus, bocavirus, coronavirus, influenza, parainfluenza, metapneumovirus and respiratory syncytial virus are all also responsible for infections in this population...
March 2017: Expert Review of Respiratory Medicine
Marijke Proesmans
For many years, management of cystic fibrosis (CF) lung disease was focused on symptomatic treatment of chronic lung infection, which is characterized by cough and sputum production, leading to progressive lung damage. With increasing survival and better knowledge of the pathogenesis of CF lung disease, it has become clear that treatment has to start very early because lung damage occurs in young patients, often before obvious symptoms appear. The arrival of new cystic fibrosis transmembrane conductance-regulator (CFTR)-correcting therapies will bring more opportunities to prevent the disease, apart from only treating chronic lung infection...
February 2017: Therapeutic Advances in Respiratory Disease
Sarah Jane Schwarzenberg, Sarah E Hempstead, Catherine M McDonald, Scott W Powers, Jamie Wooldridge, Shaina Blair, Steven Freedman, Elaine Harrington, Peter J Murphy, Lena Palmer, Amy E Schrader, Kyle Shiel, Jillian Sullivan, Melissa Wallentine, Bruce C Marshall, Amanda Radmer Leonard
Nutrition is integral to the care of individuals with cystic fibrosis (CF). Better nutritional status is associated with improved pulmonary function. In some individuals with CF, enteral tube feeding can be useful in achieving optimal nutritional status. Current nutrition guidelines do not include detailed recommendations for enteral tube feeding. The Cystic Fibrosis Foundation convened an expert panel to develop enteral tube feeding recommendations based on a systematic review of the evidence and expert opinion...
November 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Ruth K Michl, Harold Tabori, Julia Hentschel, James F Beck, Jochen G Mainz
Cystic fibrosis (CF) is the most frequent life-shortening autosomal recessive disorder in Caucasians. Defects or absence of the CF-transmembrane conductance regulator impair ion transport in apical membranes of exocrine glands. Leading symptoms of typical CF are exocrine pancreatic insufficiency and progressive pulmonary destruction, causing premature death. Additionally, patients can suffer from chronic rhinosinusitis, hepatic and intestinal involvement, diabetes and predominantly male infertility. Areas covered: CFTR-related disorders affect only one or several different organ systems, often to a milder degree...
October 12, 2016: Expert Review of Respiratory Medicine
Jerry A Nick, Kerstin Pohl, Stacey L Martiniano
PURPOSE OF REVIEW: The diagnosis of nontuberculous mycobacteria-pulmonary disease (NTM-PD) in cystic fibrosis (CF) is challenging, as it requires both microbiological and clinical evidence in the setting of coexisting airway infections and progressive lung disease. Although in some individuals NTM can accelerate the progression of CF lung disease, in others NTM may remain indolent for years, or appear transiently in sputum cultures. The dilemma faced by clinicians is to accurately identify those patients who are likely to benefit from therapy, while avoiding unnecessary treatment in those with indolent infection...
November 2016: Current Opinion in Pulmonary Medicine
Uta Griesenbach, Jane C Davies, Eric Alton
PURPOSE OF REVIEW: Since cloning of the disease-causing gene 27 years ago, the development of cystic fibrosis (CF) gene therapy has been pursued. Here, we will summarize key findings with a particular focus on recent developments. RECENT FINDINGS: Almost 3 decades of research have highlighted the complexity of lung gene transfer and have generated a body of data that has recently led to the completion of a large phase IIB study. This trial has, for the first time, shown that nonviral gene transfer can, albeit modestly, stabilize lung function in CF and provides the impetus for further development of more potent gene transfer agents...
November 2016: Current Opinion in Pulmonary Medicine
Daniel L Hamilos
Chronic rhinosinusitis (CRS) is highly prevalent in patients with cystic fibrosis (CF) and accounts for significant morbidity and contribution to CF lung disease. Mutations of the cystic fibrosis transmembrane regulator gene occur with increased prevalence in patients with CRS without CF, suggesting some contribution to CRS pathophysiology. Nasal polyps (NPs) occur with increased prevalence in patients with CF of all ages and have a more neutrophilic appearance with fewer eosinophils and increased submucosal glandular elements in comparison to NPs from patients without CF...
July 2016: Journal of Allergy and Clinical Immunology in Practice
Helena Lund-Palau, Andrew R Turnbull, Andrew Bush, Emmanuelle Bardin, Loren Cameron, Odel Soren, Natasha Wierre-Gore, Eric W F W Alton, Jacob G Bundy, Gary Connett, Saul N Faust, Alain Filloux, Paul Freemont, Andy Jones, Valerie Khoo, Sandra Morales, Ronan Murphy, Rishi Pabary, Ameze Simbo, Silke Schelenz, Zoltan Takats, Jeremy Webb, Huw D Williams, Jane C Davies
Pseudomonas aeruginosa is a remarkably versatile environmental bacterium with an extraordinary capacity to infect the cystic fibrosis (CF) lung. Infection with P. aeruginosa occurs early, and although eradication can be achieved following early detection, chronic infection occurs in over 60% of adults with CF. Chronic infection is associated with accelerated disease progression and increased mortality. Extensive research has revealed complex mechanisms by which P. aeruginosa adapts to and persists within the CF airway...
June 2016: Expert Review of Respiratory Medicine
J Stuart Elborn
Cystic fibrosis is a common life-limiting autosomal recessive genetic disorder, with highest prevalence in Europe, North America, and Australia. The disease is caused by mutation of a gene that encodes a chloride-conducting transmembrane channel called the cystic fibrosis transmembrane conductance regulator (CFTR), which regulates anion transport and mucociliary clearance in the airways. Functional failure of CFTR results in mucus retention and chronic infection and subsequently in local airway inflammation that is harmful to the lungs...
November 19, 2016: Lancet
R Zachary Thompson, Craig A Martin, Donna R Burgess, W Cliff Rutter, David S Burgess
BACKGROUND: Patients with cystic fibrosis (CF) exhibit increased clearance of beta-lactams. The purpose of this study was to predict the probability of beta-lactam target attainment (PTA) against Pseudomonas aeruginosa in adult CF patients based on local microbiological data. METHODS: CF-specific pharmacokinetic parameters were obtained from published data for aztreonam, cefepime, ceftazidime, meropenem and piperacillin-tazobactam. Pharmacodynamic modeling was used to determine the PTA for bolus, prolonged infusion, and continuous infusion regimens...
September 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Noel G McElvaney
Cystic fibrosis and alpha-1 antitrypsin (AAT) deficiency are two of the commonest lethal hereditary lung diseases affecting white individuals. Although having quite different phenotypic extrapulmonary presentations, the lung disease associated with these conditions is exemplified by a neutrophil-dominated inflammation in which neutrophil elastase plays a major role. In AAT deficiency the diminution of the anti-neutrophil elastase protection, due to diminished AAT levels in the lung, predisposes the lung to an unopposed neutrophil elastase attack, whereas, in cystic fibrosis, the levels of AAT and other antiproteases are normal, but the neutrophil elastase burden is so large that it overwhelms the normal anti-neutrophil elastase protection...
April 2016: Annals of the American Thoracic Society
Kris De Boeck, Margarida D Amaral
Standard follow-up and symptomatic treatment have allowed most patients with cystic fibrosis to live to young adulthood. However, many patients still die prematurely from respiratory insufficiency. Hence, further investigations to improve these therapies are important and might have relevance for other diseases-eg, exploring how to increase airway hydration, how to safely downscale the increased inflammatory response in the lung, and how to better combat lung infections associated with cystic fibrosis. In parallel, development of modulators that target the underlying dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR) is fast moving forward...
August 2016: Lancet Respiratory Medicine
Bradley S Quon, Steven M Rowe
Cystic fibrosis (CF) is a monogenic autosomal recessive disorder that affects about 70,000 people worldwide. The clinical manifestations of the disease are caused by defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The discovery of the CFTR gene in 1989 has led to a sophisticated understanding of how thousands of mutations in the CFTR gene affect the structure and function of the CFTR protein. Much progress has been made over the past decade with the development of orally bioavailable small molecule drugs that target defective CFTR proteins caused by specific mutations...
March 30, 2016: BMJ: British Medical Journal
J Stuart Elborn, Patrick A Flume, Fredric Cohen, Jeffery Loutit, Donald R VanDevanter
BACKGROUND: Levofloxacin inhalation solution (LIS) is the first aerosolized fluoroquinolone licensed for treatment of patients with cystic fibrosis (CF) and chronic Pseudomonas aeruginosa lung infection. This study evaluated the safety and efficacy of extended LIS treatment. METHODS: Patients completing a multinational, randomized study comparing LIS and tobramycin inhalation solution (TIS) were enrolled in an open-label extension in which all patients received three additional cycles of 28days of LIS 240mg twice daily followed by 28days off drug...
September 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Kimberly A Spoonhower, Pamela B Davis
Improved quality of care and rapidly emerging therapeutic strategies to restore chloride transport profoundly impact the epidemiology and pathobiology of cystic fibrosis (CF) in the twenty-first century. CF now serves as a model for chronic illness management, continuous quality improvement via registry data, and a seamless link between basic science research, translational studies, clinical trials, and outcomes research to enable rapid expansion of treatment options.
March 2016: Clinics in Chest Medicine
Marie E Egan
Cystic fibrosis (CF) is a common life-shortening autosomal recessive genetic disorder caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator protein (CFTR). Almost 2000 variants in the CFTR gene have been identified. The mutational classes are based on the functional consequences on CFTR. New therapies are being developed to target mutant CFTR and restore CFTR function. Understanding specific CF genotypes is essential for providing state-of-the art care to patients...
March 2016: Clinics in Chest Medicine
John Brewington, J P Clancy
Cystic Fibrosis (CF) is a rare, multisystem disease leading to significant morbidity and mortality. CF is caused by defects in the cystic fibrosis transmembrane conductance regulator protein (CFTR), a chloride and bicarbonate transporter. Early diagnosis and access to therapies provides benefits in nutrition, pulmonary health, and cognitive ability. Several screening and diagnostic tests are available to support a diagnosis. We discuss the characteristics of screening and diagnostic tests for CF and guideline-based algorithms using these tools to establish a diagnosis...
March 2016: Clinics in Chest Medicine
Jerry A Nick, David P Nichols
The diagnosis of cystic fibrosis (CF) is being made with increasing frequency in adults. Patients with CF diagnosed in adulthood typically present with respiratory complaints, and often have recurrent or chronic airway infection. At the time of initial presentation individuals may appear to have clinical manifestation limited to a single organ, but with subclinical involvement of the respiratory tract. Adult-diagnosed patients have a good response to CF center care, and newly available cystic fibrosis transmembrane receptor-modulating therapies are promising for the treatment of residual function mutation, thus increasing the importance of the diagnosis in adults with unexplained bronchiectasis...
March 2016: Clinics in Chest Medicine
2016-02-11 15:03:40
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