Etiology and new treatment options for patients with plastic bronchitis
Ugaki and colleagues1 describe a case of a 4-year-old child diagnosed with hypoplastic left heart syndrome and plastic bronchitis (PB) that developed shortly after his Fontan operation. The patient failed conventional management, including nebulized tissue plasminogen activator and dornase alfa, and Fontan fenestration. Due to persistent symptoms of PB, bilateral groin intranodal lymphangiography was performed. Access to the cisterna chyli was obtained via the transhepatic approach and contrast injection demonstrated an accessory right duct perfusing the right hilar and peribronchial lymphatic vessels. (Source: The Journal...
Source: The Journal of Thoracic and Cardiovascular Surgery - May 9, 2016 Category: Cardiovascular & Thoracic Surgery Authors: Yoav Dori, Maxim Itkin Tags: Editorial commentary Source Type: research
Inhaled protein/peptide-based therapies for respiratory disease
Abstract
Asthma, chronic obstructive pulmonary disease (COPD), and cystic fibrosis (CF) are all chronic pulmonary diseases, albeit with different etiologies, that are characterized by airflow limitation, chronic inflammation, and abnormal mucus production/rheology. Small synthetic molecule-based therapies are commonly prescribed for all three diseases. However, there has been increased interest in “biologicals” to treat these diseases. Biologicals typically constitute protein- or peptide-based therapies and are often more potent than small molecule-based drugs. In this review, we shall describe the ...
Source: Molecular and Cellular Pediatrics - April 19, 2016 Category: Cytology Source Type: research
Dornase alfa for cystic fibrosis.
CONCLUSIONS: There is evidence to show that, compared with placebo, therapy with dornase alfa improves lung function in people with cystic fibrosis in trials lasting one month to two years. There was a decrease in pulmonary exacerbations in trials of six months or longer. Voice alteration and rash appear to be the only adverse events reported with increased frequency in randomised controlled trials. There is not enough evidence to firmly conclude if dornase alfa is superior to hyperosmolar agents in improving lung function.
PMID: 27043279 [PubMed - as supplied by publisher] (Source: Cochrane Database of Systematic Reviews)
Source: Cochrane Database of Systematic Reviews - April 3, 2016 Category: Journals (General) Authors: Yang C, Chilvers M, Montgomery M, Nolan SJ Tags: Cochrane Database Syst Rev Source Type: research
Nebulised dornase alfa versus placebo or hypertonic saline in adult critically ill patients: a systematic review of randomised clinical trials with meta-analysis and trial sequential analysis
Conclusions:
We found very low quantity and quality of evidence for use of nebulised dornase alfa in adult critically ill patients in this systematic review with meta-analysis.Systematic review registrationThe International Prospective Register of Systematic Reviews (PROSPERO), no. CRD442015016047. (Source: Epidemiologic Perspectives and Innovations)
Source: Epidemiologic Perspectives and Innovations - November 8, 2015 Category: Epidemiology Authors: Casper ClaudiusAnders PernerMorten Møller Source Type: research
Nebulised dornase alfa versus placebo or hypertonic saline in adult critically ill patients: a systematic review of randomised clinical trials with meta-analysis and trial sequential analysis
Conclusions
We found very low quantity and quality of evidence for use of nebulised dornase alfa in adult critically ill patients in this systematic review with meta-analysis.
Systematic review registration
The International Prospective Register of Systematic Reviews (PROSPERO), no. CRD442015016047. (Source: Systematic Reviews)
Source: Systematic Reviews - November 8, 2015 Category: Global & Universal Source Type: research
Does the lung clearance index track with disease progression in early childhood?
Conclusion: We observed associations with known risk factors for poorer lung function supporting the utility of LCI as a measure to track lung function in CF. (Source: European Respiratory Journal)
Source: European Respiratory Journal - October 30, 2015 Category: Respiratory Medicine Authors: Sonneveld, N., Stanojevic, S., Jensen, R., Subbarao, P., Ratjen, F. Tags: 7.3 Cystic Fibrosis Source Type: research
Inhaled mannitol for cystic fibrosis.
CONCLUSIONS: There is evidence to show that treatment with mannitol over a six-month period is associated with an improvement in some measures of lung function in people with cystic fibrosis compared to control. There is no evidence that quality of life is improved for participants taking mannitol compared to control; a decrease in burden of treatment was observed up to four months on mannitol compared to control but this difference was not maintained to six months. Randomised information regarding the burden of adding mannitol to an existing treatment is limited. There is no randomised evidence of improvement in lung func...
Source: Cochrane Database of Systematic Reviews - October 9, 2015 Category: Journals (General) Authors: Nolan SJ, Thornton J, Murray CS, Dwyer T Tags: Cochrane Database Syst Rev Source Type: research
Lung function comparison between two decades in cystic fibrosis children: A single centre study
ConclusionsOur results show that pulmonary function has clearly ameliorated over a decade in young CF patients, in a period during which several significant therapeutic changes have been introduced, such as dornase alfa, tobramycin and hypertonic saline. To our knowledge this is the first study showing a cohort effect in patients diagnosed after neonatal screening. Pediatr Pulmonol. 2015;9999:1–7. © 2015 Wiley Periodicals, Inc. (Source: Pediatric Pulmonology)
Source: Pediatric Pulmonology - September 30, 2015 Category: Respiratory Medicine Authors: Gloria Tridello, Sonia Volpi, Baroukh M. Assael, Ilaria Meneghelli, Marianna Passiu, Maria Circelli Tags: Original Article: Cystic Fibrosis Source Type: research
Sustained Benefit from Ivacaftor Demonstrated by Combining Clinical Trial and CF Patient Registry Data.
The objective of this analysis was to examine, over a 3-year period, whether ivacaftor therapy affects pulmonary function and nutritional measures in CF patients with a G551Dmutation compared to CF patients who are homozygous for the F508del mutation. A propensity score was used to match patients with CF ≥6 years of age who have a G551D mutation and received ivacaftor in clinical trials for up to 144 weeks with data from patients in the U.S. Cystic Fibrosis Foundation Patient Registry who are homozygous for the F508del mutation. Matching was based on variables including age, gender, weight for age, height for age, BMI fo...
Source: American Journal of Respiratory and Critical Care Medicine - July 1, 2015 Category: Respiratory Medicine Authors: Sawicki GS, McKone EF, Pasta DJ, Millar SJ, Wagener JS, Johnson CA, Konstan MW Tags: Am J Respir Crit Care Med Source Type: research
AARC Clinical Practice Guideline: Effectiveness of Pharmacologic Airway Clearance Therapies in Hospitalized Patients.
Abstract
Aerosolized medications are used as airway clearance therapy to treat a variety of airway diseases. These guidelines were developed from a systematic review with the purpose of determining whether the use of these medications to promote airway clearance improves oxygenation and respiratory mechanics, reduces ventilator time and ICU stay, and/or resolves atelectasis/consolidation compared with usual care. Recombinant human dornase alfa should not be used in hospitalized adult and pediatric patients without cystic fibrosis. The routine use of bronchodilators to aid in secretion clearance is not reco...
Source: Respiratory Care - June 28, 2015 Category: Respiratory Medicine Authors: Strickland SL, Rubin BK, Haas CF, Volsko TA, Drescher GS, O'Malley CA Tags: Respir Care Source Type: research
146 Sinonasal inhalation of isotonic vs. hypertonic saline (6.0%) in CF patients with chronic rhinosinusitis – results of a multicentre, double-blind, controlled prospective trial
Chronic rhinosinusitis significantly impairs CF patients’ quality of life. The PariSinus™ device effectively delivers vibrating aerosols to paranasal sinuses. Previously, we demonstrated a significant reduction of sinonasal symptoms by inhalation of dornase alfa with the device (JCF 2014). Bronchially inhaled hypertonic saline (3–7%) was shown to reduce pulmonary symptoms and exacerbations in CF. Aims of this multicentre, double-blind, controlled, crossover trial were to assess effects of hypertonic (6%) and isotonic saline on sinonasal outcome measures. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - June 1, 2015 Category: Respiratory Medicine Authors: J.G. Mainz, K. Schaedlich, J. Hentschel, U. Schumacher, C. Koitschev, A. Koitschev, J. Riethmüller, F. Prenzel, O. Sommerburg, D. Staab, W. Gleiber, J.F. Beck, C. Arnold, CF-sinonasal study group: H.-Eberhard Heuer, Christina Smaczny, Claudius Werner, He Tags: Posters Source Type: research
Randomized trial of efficacy and safety of dornase alfa delivered by eRapid nebulizer in cystic fibrosis patients
Dornase alfa administered via jet nebulizer is indicated as a chronic respiratory medication for cystic fibrosis (CF) patients. Efficacy and safety of dornase alfa via an electronic nebulizer with vibrating membrane technology have not been formally assessed in randomized clinical trials. (Source: Journal of Cystic Fibrosis)
Source: Journal of Cystic Fibrosis - April 25, 2015 Category: Respiratory Medicine Authors: Gregory S. Sawicki, Will Chou, Karina Raimundo, Ben Trzaskoma, Michael W. Konstan Tags: Original Article Source Type: research
The Effect of Dornase Alfa (rhDNase) on Recurrent Gram Negative Infections in Adult Cystic Fibrosis Lung Transplant Recipients
We describe our single center experience of CF lung transplant recipients who received rhDNase post-transplant. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2015 Category: Transplant Surgery Authors: L.J. Stuckey, A.M. Clark, S. Chang, K.M. Chan, C.E. Bartos, T.C. Ojo Source Type: research
Geographic variations in cystic fibrosis: An analysis of the U.S. CF Foundation Registry
ConclusionsThe U.S. has significant regional variations in CF demographics, insurance, pathogens, medication usage, and co‐morbidities, without an overall impact on regional mortality. Regional variations in care practices should be studied further based on the findings. Pediatr Pulmonol. © 2015 Wiley Periodicals, Inc. (Source: Pediatric Pulmonology)
Source: Pediatric Pulmonology - March 30, 2015 Category: Respiratory Medicine Authors: Benjamin T. Kopp, Lisa Nicholson, Grace Paul, Joseph Tobias, Chandar Ramanathan, Don Hayes Tags: Original Article: Cystic Fibrosis Source Type: research
