Business Briefing: Lung Transplant Device Receives Federal Approval

The Food and Drug Administration said Tuesday that the Xvivo Perfusion System could lead to more successful transplants of lungs for people with cystic fibrosis and other deadly respiratory diseases.
Source: NYT Health - Category: Consumer Health News Authors: Tags: Cystic Fibrosis Respiratory System Food and Drug Administration Source Type: news

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Authors: Graul AI, Pina P, Tracy M, Sorbera L Abstract Highlights of our annual review of new approvals and launches on global drug markets include the approval and launch of Trikafta, the most widely applicable treatment to date for cystic fibrosis; approval of the first Ebola vaccine for general (rather than emergency) use; the pilot rollout in three African countries of the world's first malaria vaccine; approval of a new treatment option for multidrug-resistant bacterial infections; and the approval and launch in China of the first new drug to treat Alzheimer's disease in more than a decade. Several new immune ...
Source: Drugs of Today - Category: Drugs & Pharmacology Tags: Drugs Today (Barc) Source Type: research
Cystic fibrosis (CF) is a life-shortening recessive disorder caused by mutations in both alleles of the cystic fibrosis transmembrane conductance regulator (CFTR) gene [1]. CFTR codes for an ion channel that mediates chloride transport across epithelial cell membranes. Mutations resulting in CFTR dysfunction cause chronic obstructive lung disease, intestinal obstruction syndromes, liver dysfunction, exocrine and endocrine pancreatic dysfunction, and male infertility [1,2]. CFTR is a complex, multidomain, membrane-spanning protein that undergoes highly regulated folding and trafficking post-biosynthesis to be functionally m...
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research
Journal Name: Clinical Chemistry and Laboratory Medicine (CCLM) Issue: Ahead of print
Source: Clinical Chemistry and Laboratory Medicine - Category: Laboratory Medicine Source Type: research
The cystic fibrosis transmembrane conductance regulator (CFTR) is an ion channel protein that is defective in individuals with cystic fibrosis (CF). To advance the rational design of CF therapies, it is important to elucidate how mutational defects in CFTR lead to its impairment and how pharmacological compounds interact with and alter CFTR. Here, using a helical-hairpin construct derived from CFTR's transmembrane (TM) helices 3 and 4 (TM3/4) and their intervening loop, we investigated the structural effects of a patient-derived CF-phenotypic mutation, E217G, located in the loop region of CFTR's membrane-spanning domain. E...
Source: Journal of Biological Chemistry - Category: Chemistry Authors: Tags: Accelerated Communications Source Type: research
Substantial improvements in diagnostics and treatments have been obtained for patients with cystic fibrosis (CF), resulting in a tremendously improved prognosis for the patients [1]. However, chronic lung infections remain the major cause for premature death in CF patients [1], especially with Pseudomonas aeruginosa. It is generally accepted that biofilm formation in the lungs of patients with CF or chronic lung disease (e.g. bronchiectasis) provides the mechanism leading to the persistent airway infections, since biofilm growth renders the bacteria tolerant to the host response and to antibiotics [2].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research
Publication date: Available online 13 February 2020Source: Pharmacology &TherapeuticsAuthor(s): Kylie B.R. Belchamber, Louise E. DonnellyAbstractChronic pulmonary conditions now account for 1 in 15 deaths in the US and mortality is increasing. Chronic obstructive pulmonary disease (COPD) is due to become the 3rd largest cause of mortality by 2030 and mortality from other respiratory conditions such as asthma, idiopathic pulmonary fibrosis and cystic fibrosis are not reducing. There is an urgent need for novel therapies to address this problem as many of the current strategies targeting inflammation are not sufficient. ...
Source: Pharmacology and Therapeutics - Category: Drugs & Pharmacology Source Type: research
Individuals with impaired immune responses, such as ventilated and cystic fibrosis patients are often infected with Pseudomonas aeruginosa (P.a) bacteria, and a co-infection with the Influenza virus (IAV) is often present. It has been known for many years that infection with IAV predisposes the host to secondary bacterial infections (such as Streptococcus pneumoniae or Staphylococcus aureus), and there is an abundance of mechanistic studies, including those studying the role of desensitization of TLR signaling, type I IFN- mediated impairment of neutrophil chemokines and antimicrobial production, attenuation of IL1β p...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
The Journal of Cystic Fibrosis (JCF) has just published the second special issue featuring current topics in basic cystic fibrosis (CF) research that were presented and discussed at the 16th ECFS Basic Science Conference held in Dubrovnik, Croatia from 27 to 30th March 2019 [1]. There have been major breakthroughs in the treatment of people with CF in recent years, largely due to the development of highly effective CFTR modulators [2 –5]. Underpinning the discovery of these modulator drugs are the Basic Science discoveries in the area that have led to greater understanding of the structure and function of CFTR and ho...
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Editorial Source Type: research
We describe a case of a staphylococcal lung abscess in a teenage girl with CF who presented with a two-week history of non-specific malaise followed by two days of left posterior chest pain and fever. A chest radiograph was consistent with a left sided pulmonary abscess, which was confirmed on a CT scan of the chest. The abscess was drained under ultrasound guidance and cultured methicillin-sensitive Staphylococcus aureus. The patient responded well to antibiotic treatment with the abscess cavity showing complete radiological resolution by six weeks post drainage.
Source: Respiratory Medicine Case Reports - Category: Respiratory Medicine Source Type: research
ConclusionOur work highlights the value of extended or targeted genotyping in selected populations. It also demonstrates the benefit of routine collaboration between national registries. This will promote identification of novel mutations; leading to greater understanding of genotype ‐phenotype associations, improved individual prognostication, and ultimately the improved availability of novel precision therapies. This collaboration is essential if we are to achieve health equality for people with CF living in resource‐limited settings.
Source: The Clinical Respiratory Journal - Category: Respiratory Medicine Authors: Tags: BRIEF REPORT Source Type: research
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