Cystic Fibrosis Pulmonary Guidelines

Flume, Patrick A.; O’Sullivan, Brian; Robinson, Karen A.; Goss, Christopher H.; Mogayzel, Peter J.; Willey-Courand, Donna Beth; Bujan, Janet; Finder, Jonathan D.; Lester, Mary; Quittell, Lynne M.; Rosenblatt, Randall L.; Vender, Robert L.; Hazle, Leslie; Sabadosa, Kathy; Marshall, Bruce C.

doi:10.1164/rccm.200705-664oc

Cited by 794 publications

(254 citation statements)

References 93 publications

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“…This UK CF registry analysis found that many people with CF do not use any airway clearance techniques, or use exercise as their primary airway clearance technique despite the fact that this is not recommended [2,3]. Forced expiratory techniques and airway clearance devices (PEP and oscillating PEP) were found to be the most common primary airway clearance techniques used by people with CF in the UK, while postural drainage and high-frequency chest wall oscillation are the least common techniques (used by 4% and 1%, respectively).…”

Section: Resultsmentioning

confidence: 99%

“…Chest physiotherapy to remove airway secretions is recommended by various CF organisations as a vital treatment for people with CF [2,3].…”

Section: Introductionmentioning

confidence: 99%

“…Despite various studies and meta-analyses (including five Cochrane systematic reviews), the best airway clearance technique for people with CF has yet to be identified [5]. As such, it is recommended that airway clearance techniques should be tailored according to individual needs, lifestyle and symptoms [2,3]. Most people with CF have their own preferences for a particular airway clearance technique, as shown by the drop-outs in a recent randomised controlled trial of long-term airway clearance techniques [6].…”

Section: Introductionmentioning

confidence: 99%

“…People with CF may perceive exercise to be a nonstigmatising activity performed by all healthy people, so may choose to use exercise alone as an airway clearance technique despite recommendations to the contrary [2,3]. Evidence suggests that although exercise helps increase ease of sputum expectoration, it may not actually increase solid content [35].…”

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confidence: 99%

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Airway clearance techniques used by people with cystic fibrosis in the UK

et al. 2015

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Please cite this article as: Hoo ZH, Daniels T, Wildman MJ, Teare MD, Bradley JM, Airway clearance techniques used by people with cystic fibrosis in the UK, Physiotherapy (2015), http://dx.doi.org/10.1016/j.physio. 2015.01.008 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain. Setting and participants All people with CF in the UK aged ≥11 years (n=6372). ResultsOf the 6372 people on the UK CF registry in 2011, 89% used airway clearance techniques.The most commonly used primary techniques were forced expiratory techniques (28%) and oscillating positive expiratory pressure (PEP) (23%). Postural drainage and high-frequency chest wall oscillation were used by 4% and 1% of people with CF, respectively. The male:female ratio of individuals who used exercise as their primary airway clearance technique was 2:1, compared with 1:1 for other techniques. Individuals with more severe lung disease tended to use devices such as non-invasive ventilation or high-frequency chest wall oscillation.Conclusions Forced expiratory techniques and oscillating PEP are the most common airway clearance techniques used by people with CF in the UK, and postural drainage and high-frequency chest wall oscillation are the least common techniques. This is significant in terms of planning airway clearance technique trials, where postural drainage has been used traditionally as the comparator. The use of airway clearance techniques varies between countries, but the reasons for these differences are unknown.

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Section: Resultsmentioning

confidence: 99%

“…Chest physiotherapy to remove airway secretions is recommended by various CF organisations as a vital treatment for people with CF [2,3].…”

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

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Airway clearance techniques used by people with cystic fibrosis in the UK

et al. 2015

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“…5F) while depriving the EPEC or STEC bacteria of their meal. Indeed, inhaled DNase (dornase) has been shown to improve outcomes of infection in cystic fibrosis patients (33,34), and intrapleural injection of DNase plus a fibrinolytic agent improved recovery from empyema of the chest (35). It is very plausible, therefore, that DNA NETs in the gut lumen may assist EPEC and STEC more than they hurt them and that the role of NETs in vivo may not be as simple as depicted in studies of neutrophils forming NETs on glass microscope slides.…”

Section: Discussionmentioning

confidence: 99%

Biological Activities of Uric Acid in Infection Due to Enteropathogenic and Shiga-Toxigenic Escherichia coli

2016

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In previous work, we identified xanthine oxidase (XO) as an important enzyme in the interaction between the host and enteropathogenic Escherichia coli (EPEC) and Shiga-toxigenic E. coli (STEC). Many of the biological effects of XO were due to the hydrogen peroxide produced by the enzyme. We wondered, however, if uric acid generated by XO also had biological effects in the gastrointestinal tract. Uric acid triggered inflammatory responses in the gut, including increased submucosal edema and release of extracellular DNA from host cells. While uric acid alone was unable to trigger a chloride secretory response in intestinal monolayers, it did potentiate the secretory response to cyclic AMP agonists. Uric acid crystals were formed in vivo in the lumen of the gut in response to EPEC and STEC infections. While trying to visualize uric acid crystals formed during EPEC and STEC infections, we noticed that uric acid crystals became enmeshed in the neutrophilic extracellular traps (NETs) produced from host cells in response to bacteria in cultured cell systems and in the intestine in vivo. Uric acid levels in the gut lumen increased in response to exogenous DNA, and these increases were enhanced by the actions of DNase I. Interestingly, addition of DNase I reduced the numbers of EPEC bacteria recovered after a 20-h infection and protected against EPEC-induced histologic damage. Xanthine oxidase (XO), also known as xanthine oxidoreductase (XOR), has been hailed as an important host defense against enteric pathogens for decades (1, 2). In a recent study, however, we found that XO could have deleterious as well as beneficial effects in the context of infection with Shiga-toxigenic Escherichia coli (STEC) (3). One of the deleterious effects of XO was its ability to induce production of the Shiga toxins (Stx) from STEC bacteria. Low to intermediate amounts of XO activity generated enough H 2 O 2 to induce Stx but not enough to actually kill STEC or enteropathogenic E. coli (EPEC) bacteria, resulting in an "uncanny valley" of worsened disease outcome.In addition to H 2 O 2 , XO also generates uric acid as a product. While uric acid used to be viewed as an inert waste product of purine metabolism, recent advances have highlighted the biological roles of uric acid in inflammation and innate immunity (4, 5). Although monosodium urate (MSU) is the technically more accurate term, we will use "uric acid" here, since the latter term is deeply entrenched in the biomedical literature. In a previous study, we found that uric acid crystals were formed in vivo in response to EPEC and STEC infection in the lumen of the gastrointestinal (GI) tract (6). In the present study, we focused on determining the biological effects of uric acid, as opposed to H 2 O 2 , produced by XO. We investigated the effects of uric acid on inflammatory responses, as well as secretory responses, in intestinal tissues and found that uric acid acted as a potentiator or modulator of proinflammatory or prosecretory agonists and that its crystals became enmeshed in the...

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Improving Evidence-Based Care in Cystic Fibrosis Through Quality Improvement

McPhail¹,

Weiland²,

Acton³

et al. 2010

Arch Pediatr Adolesc Med

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To increase clinician adherence to prescribing guidelines for pulmonary medications in children with cystic fibrosis (CF). Design: Quality improvement project with multiple time series design. Setting: The CF center at a tertiary care pediatric hospital in the United States. Patients: Children with CF who were eligible to receive oral azithromycin, nebulized dornase alfa, or inhaled tobramycin sulfate based on prescribing guidelines for CF lung disease. Intervention: Evidence-based prescribing guidelines were designed by a local committee to reflect consensus recommendations from the CF Foundation. Clinicians and families were educated about guidelines. Adherence to prescribing guidelines was tracked using a local CF database and record reviews. Weekly meetings were used to highlight adherence failures and promote clinician accountability. Main Outcome Measure: The rate of clinician adherence to prescribing guidelines. Results: One hundred seventy patients with CF were included. At the start of the project, the rate of clinician adherence to prescribing guidelines was 62%. After 3 months of the project, the rate of clinician adherence to prescribing guidelines was 87% (odds ratio=4.6; 95% confidence interval, 3.0-7.0). The improvements in adherence to prescribing guidelines were sustained for 21 months of follow-up. Conclusions: Educating clinicians about prescribing guidelines, sharing guidelines with families, and monitoring clinician adherence improve prescribing adherence to evidence-based recommendations.

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Cystic Fibrosis Pulmonary Guidelines

Abstract: It is hoped that the guidelines provided in this article will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis.

Cited by 794 publications

References 93 publications

Airway clearance techniques used by people with cystic fibrosis in the UK

Airway clearance techniques used by people with cystic fibrosis in the UK

Biological Activities of Uric Acid in Infection Due to Enteropathogenic and Shiga-Toxigenic Escherichia coli

Improving Evidence-Based Care in Cystic Fibrosis Through Quality Improvement

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