Model of mucociliary clearance in cystic fibrosis lungs
Publication date: 7 May 2015 Source:Journal of Theoretical Biology, Volume 372 Author(s): P. Kurbatova , N. Bessonov , V. Volpert , H.A.W.M. Tiddens , C. Cornu , P. Nony , D. Caudri Mucus clearance is a primary innate defense mechanism in the human airways. Cystic fibrosis (CF) is a genetic disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CF is characterized by dehydration of airway surface liquid and impaired mucociliary clearance. As a result, microorganisms are not efficiently removed from the airways, and patients experience chronic pulm...
Source: Journal of Theoretical Biology - March 19, 2015 Category: Biology Source Type: research
Children and young adults with CF in the USA have better lung function compared with the UK
Conclusions
Children and young adults with CF have better lung function in the USA compared with the UK despite similar nutritional status. (Source: Thorax)
Source: Thorax - February 16, 2015 Category: Respiratory Medicine Authors: Goss, C. H., MacNeill, S. J., Quinton, H. B., Marshall, B. C., Elbert, A., Knapp, E. A., Petren, K., Gunn, E., Osmond, J., Bilton, D. Tags: Epidemiologic studies, Cystic fibrosis Source Type: research
Enhancement of Pulmozyme activity in purulent sputum by combination with poly-aspartic acid or gelsolin
DNase (Pulmozyme) effectiveness in cystic fibrosis treatment is in some cases limited by its inability to access DNA trapped within bundles in highly viscous fluids that also contain actin. Dissociating DNA-containing bundles using actin depolymerizing agents and polyanions has potential to increase DNase efficacy. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - February 12, 2015 Category: Respiratory Medicine Authors: Robert Bucki, Katrina Cruz, Katarzyna Pogoda, Ashley Eggert, LiKang Chin, Marianne Ferrin, Giovanna Imbesi, Denis Hadjiliadis, Paul A. Janmey Tags: Original Article Source Type: research
Enzymes Approved for Human Therapy: Indications, Mechanisms and Adverse Effects
Abstract
Research and drug developments fostered under orphan drug product development programs have greatly assisted the introduction of efficient and safe enzyme-based therapies for a range of rare disorders. The introduction and regulatory approval of 20 different recombinant enzymes has enabled, often for the first time, effective enzyme-replacement therapy for some lysosomal storage disorders, including Gaucher (imiglucerase, taliglucerase, and velaglucerase), Fabry (agalsidase alfa and beta), and Pompe (alglucosidase alfa) diseases and mucopolysaccharidoses I (laronidase), II (idursulfase), IVA (elo...
Source: BioDrugs - February 4, 2015 Category: Drugs & Pharmacology Source Type: research
Effects of dornase alfa and hypertonic saline on gas mixing in lung periphery in children with cystic fibrosis
Conclusion: Treatment with DA may systematically change VI possibly arising in lung periphery. Lung function response exceeding the test variability was more frequently observed in children using DA compared to HS. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 23, 2014 Category: Respiratory Medicine Authors: Singer, F., Stanojevic, S., Yammine, S., Subbarao, P., Jensen, R., Amin, R., Ratjen, F., Latzin, P. Tags: 7.3 Cystic Fibrosis Source Type: research
Modelling and simulation of experimental designs to find the best design of randomized clinical trials in a rare disease: Cystic fibrosis
Conclusion: This in silico approach could be used to find the most effective clinical trial design, especially in rare diseases such as CF. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 23, 2014 Category: Respiratory Medicine Authors: Kurbatova, P., Bajard, A., Tiddens, H., Volpert, V., Cornu, C., Bessonov, N., Kassai, B., Chabaud, S., Nony, P., Caudri, D. Tags: 7.3 Cystic Fibrosis Source Type: research
The performance of a new vibrating mesh prototype nebulizer for inhaled biodrugs
Conclusion:Based on in vitro results, the DTF-VM nebulizer could be used as an aerosol generator to achieve rapidly high lung deposition, making it suitable for expensive biodrugs in development. (Source: European Respiratory Journal)
Source: European Respiratory Journal - December 23, 2014 Category: Respiratory Medicine Authors: Marchand, D., Maria, C., Pinel, M., Nicolini, T., Vecellio, L. Tags: 5.1 Airway Pharmacology and Treatment Source Type: research
Nasal saline as a placebo in chronic rhinosinusitis
I read with interest Mainz et al’s article in the July issue of your journal [1]. The authors concluded that; vibrating sinonasal inhalation of dornase alfa reduces rhinosinusitis symptoms in cystic fibrosis. We question the interpretation of the results. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - July 30, 2014 Category: Respiratory Medicine Authors: Ahmet Karadag, Ferhat Catal Tags: Correspondence Source Type: research
Sinonasal inhalation of dornase alfa administered by vibrating aerosol to cystic fibrosis patients: A double-blind placebo-controlled cross-over trial
Chronic rhinosinusitis significantly impairs CF patients' quality of life and overall health. The Pari-Sinus™ device delivers vibrating aerosol effectively to paranasal sinuses. After a small pilot study to assess sinonasal inhalation of dornase alfa and placebo (isotonic saline) on potential sinonasal outcome measures, we present the subsequent prospective double-blind placebo-controlled crossover-trial. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - July 1, 2014 Category: Respiratory Medicine Authors: Jochen G. Mainz, Claudia Schien, Isabella Schiller, Katja Schädlich, Assen Koitschev, Christiane Koitschev, Joachim Riethmüller, Uta Graepler-Mainka, Bärbel Wiedemann, James F. Beck Tags: Ear, Nose and Throat Source Type: research
Nebulized Therapy. SEPAR Year.
Abstract
Inhaled drugs are deposited directly in the respiratory tract. They therefore achieve higher concentrations with faster onset of action and fewer side effects than when used systemically. Nebulized drugs are mainly recommended for patients that require high doses of bronchodilators, when they need to inhale drugs that only exist in this form (antibiotics or dornase alfa) or when they are unable to use other inhalation devices. Technological development in recent years has led to new devices that optimize pulmonary deposits and reduce the time needed for treatment. In this review we focus solely on...
Source: Archivos de Bronconeumologia - July 1, 2014 Category: Respiratory Medicine Authors: Olveira C, Muñoz A, Domenech A Tags: Arch Bronconeumol Source Type: research
Sinonasal inhalation of dornase alfa administered by vibrating aerosol to cystic fibrosis patients: A double-blind placebo-controlled cross-over trial
Chronic rhinosinusitis significantly impairs CF patients' quality of life and overall health. The Pari-Sinus™ device delivers vibrating aerosol effectively to paranasal sinuses. After a small pilot study to assess sinonasal inhalation of dornase alfa and placebo (isotonic saline) on potential sinonasal outcome measures, we present the subsequent prospective double-blind placebo-controlled crossover-trial. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - July 1, 2014 Category: Respiratory Medicine Authors: Jochen G. Mainz, Claudia Schien, Isabella Schiller, Katja Schädlich, Assen Koitschev, Christiane Koitschev, Joachim Riethmüller, Uta Graepler-Mainka, Bärbel Wiedemann, James F. Beck Tags: Ear, Nose and Throat Source Type: research
Dornase Alfa for Non-Cystic Fibrosis Pediatric Pulmonary Atelectasis.
CONCLUSIONS: Dornase alfa may be considered as a therapeutic option in non-cystic fibrosis pediatric patients with pulmonary atelectasis, who require treatment intervention when conventional therapy is unsuccessful.
PMID: 24811400 [PubMed - as supplied by publisher] (Source: The Annals of Pharmacotherapy)
Source: The Annals of Pharmacotherapy - May 8, 2014 Category: Drugs & Pharmacology Authors: Thornby KA, Johnson A, Axtell S Tags: Ann Pharmacother Source Type: research
Temporal expression of agrB, cidA, and alsS in the early development of Staphylococcus aureus UAMS‐1 biofilm formation and the structural role of extracellular DNA and carbohydrates
Abstract
Extracellular DNA (eDNA) is an important component of the extracellular polymeric substance matrix and is important in the establishment and persistence of Staphylococcus aureus UAMS‐1 biofilms. The aim of the study was to determine the temporal expression of genes involved in early biofilm formation and eDNA production. We used qPCR to investigate expression of agrB, which is associated with secreted virulence factors and biofilm dispersal, cidA, which is associated with biofilm adherence and genomic DNA release, and alsS, which is associated with cell lysis, eDNA release and acid tolerance. The contribution of...
Source: FEMS Immunology and Medical Microbiology - March 11, 2014 Category: Microbiology Authors: Rossella Grande, Laura Nistico, Karthik Sambanthamoorthy, Mark Longwell, Antonio Iannitelli, Luigina Cellini, Antonio Stefano, Luanne Hall Stoodley, Paul Stoodley Tags: Research Article Source Type: research
Sinonasal inhalation of dornase alfa administered by vibrating aerosol to cystic fibrosis patients: A double-blind placebo-controlled cross-over trial
Conclusion: Vibrating sinonasal inhalation of dornase alfa reduces rhinosinusitis symptoms in CF. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - March 4, 2014 Category: Respiratory Medicine Authors: Jochen G. Mainz, Claudia Schien, Isabella Schiller, Katja Schädlich, Assen Koitschev, Christiane Koitschev, Joachim Riethmüller, Uta Graepler-Mainka, Bärbel Wiedemann, James F. Beck Tags: Ear, Nose and Throat Source Type: research
