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Find video protocols related to scientific articles indexed in Pubmed.
Protease-antiprotease imbalances differ between Cystic Fibrosis patients' upper and lower airway secretions.
J. Cyst. Fibros.
PUBLISHED: 06-10-2014
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Balanced levels of proteases and anti-proteases are essential in host defense systems. In CF patients' lungs, elevated protease/anti-protease-ratios contribute to damage of airway tissue and premature death with the inherited disease. Little is known about upper airway protease equilibrium in CF.
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Dynamics of soluble and cellular inflammatory markers in nasal lavage obtained from cystic fibrosis patients during intravenous antibiotic treatment.
BMC Pulm Med
PUBLISHED: 04-24-2014
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In cystic fibrosis (CF) patients, the upper airways display the same ion channel defect as evident in the lungs, resulting in chronic inflammation and infection. Recognition of the sinonasal area as a site of first and persistent infection with pathogens, such as Pseudomonas aeruginosa, reinforces the "one-airway" hypothesis. Therefore, we assessed the effect of systemic antibiotics against pulmonary pathogens on sinonasal inflammation.
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Sinonasal inhalation of dornase alfa administered by vibrating aerosol to cystic fibrosis patients: a double-blind placebo-controlled cross-over trial.
J. Cyst. Fibros.
PUBLISHED: 02-07-2014
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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.
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Non-invasive assessment of upper and lower airway infection and inflammation in CF patients.
Pediatr. Pulmonol.
PUBLISHED: 01-25-2014
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The upper (UAW) and lower (LAW) airways of patients with cystic fibrosis (CF) have the same ion-channel defects, but little is known about similarities and differences in host immunological responses at the two levels.
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Sinonasal inhalation of tobramycin vibrating aerosol in cystic fibrosis patients with upper airway Pseudomonas aeruginosa colonization: results of a randomized, double-blind, placebo-controlled pilot study.
Drug Des Devel Ther
PUBLISHED: 01-01-2014
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In cystic fibrosis (CF), the paranasal sinuses are sites of first and persistent colonization by pathogens such as Pseudomonas aeruginosa. Pathogens subsequently descend to the lower airways, with P. aeruginosa remaining the primary cause of premature death in patients with the inherited disease. Unlike conventional aerosols, vibrating aerosols applied with the PARI Sinus™ nebulizer deposit drugs into the paranasal sinuses. This trial assessed the effects of vibrating sinonasal inhalation of the antibiotic tobramycin in CF patients positive for P. aeruginosa in nasal lavage.
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Influences of nasal lavage collection-, processing- and storage methods on inflammatory markers - Evaluation of a method for non-invasive sampling of epithelial lining fluid in cystic fibrosis and other respiratory diseases.
J. Immunol. Methods
PUBLISHED: 09-30-2013
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Non-invasive sampling of airway epithelial-lining-fluid by nasal lavage (NL) is an emerging method to monitor allergy, infection and inflammation in patients with respiratory diseases. However, the influences of collection-, processing- and storage-methods have not been sufficiently evaluated and standardized.
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Efficacy and safety of ivacaftor in patients aged 6 to 11 years with cystic fibrosis with a G551D mutation.
Am. J. Respir. Crit. Care Med.
PUBLISHED: 04-18-2013
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Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, has been shown to improve lung function, pulmonary exacerbation rate, respiratory symptoms, and weight gain compared with placebo in patients with cystic fibrosis aged 12 years or older with a G551D-CFTR mutation.
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Therapy of CF-patients with amitriptyline and placebo--a randomised, double-blind, placebo-controlled phase IIb multicenter, cohort-study.
Cell. Physiol. Biochem.
PUBLISHED: 03-18-2013
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Several recent studies revealed an accumulation of ceramide in bronchial, tracheal and intestinal epithelial cells of mice and patients with cystic fibrosis (CF). Normalization of ceramide concentrations in lungs of CF mice employing the functional acid sphingomyelinase inhibitor amitriptyline also normalized mucociliary clearance, chronic inflammation and infection susceptibility to pulmonary P. aeruginosa in these mice.
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Effects of ivacaftor on severely ill patients with cystic fibrosis carrying a G551D mutation.
J. Cyst. Fibros.
PUBLISHED: 02-18-2013
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Recently, ivacaftor, a CFTR-potentiator, has been shown to be effective and safe in patients with cystic fibrosis carrying a G551D mutation and moderately impaired lung function. The objective of this retrospective study was to assess efficacy and safety of ivacaftor in severely ill patients with at least one G551D mutation.
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Reduced nasal nitric oxide production in cystic fibrosis patients with elevated systemic inflammation markers.
PLoS ONE
PUBLISHED: 01-01-2013
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Nitric oxide (NO) is produced within the respiratory tract and can be detected in exhaled bronchial and nasal air. The concentration varies in specific diseases, being elevated in patients with asthma and bronchiectasis, but decreased in primary ciliary dyskinesia. In cystic fibrosis (CF), conflicting data exist on NO levels, which are reported unexplained as either decreased or normal. Functionally, NO production in the paranasal sinuses is considered as a location-specific first-line defence mechanism. The aim of this study was to investigate the correlation between upper and lower airway NO levels and blood inflammatory parameters, CF-pathogen colonisation, and clinical data.
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Sinonasal inhalation of dornase alfa in CF: A double-blind placebo-controlled cross-over pilot trial.
Auris Nasus Larynx
PUBLISHED: 09-21-2010
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The paranasal sinuses are almost always involved in cystic fibrosis, and chronic rhinosinusitis and nasal polyps are very frequent in the disease. Hereby, the patients quality of life and their overall health are relevantly impaired. Although dornase alfa, a mucolytic agent, may also be effective in the upper airways, deposition of inhaled drugs into paranasal sinuses is substantially limited. The novel PARI SINUS™ nebuliser has been shown in deposition studies to deliver aerosol into paranasal sinuses but has not yet been clinically tested. This DBPC pilot-trial applying dornase alfa aims to evaluate outcome parameters and sample sizes for a subsequent efficacy trial.
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Management of chronic rhinosinusitis in CF.
J. Cyst. Fibros.
PUBLISHED: 05-23-2009
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Routine CF management often does not include upper airway (UAW) assessment although CFTR defects equally affect the sinonasal mucosa. Up to 50% of CF patients have chronic rhinosinusitis (CRS) and/or nasal polyps, and almost 100% reveal UAW abnormalities on CT scan. CRS impairs quality of life. UAW dysfunction in filtering, humidifying, and warming inspired air affects lower airways and the UAW is a potential site of first colonization and a reservoir for opportunistic bacteria. Therefore, UAW pathology substantially affects overall health in CF. Standard treatments are scarce and mostly lack evidence. Nasal douche can remove mucus and crusts. Recently, delivery of dornase alfa using a vibrating aerosol has shown potential as treatment for CF-related CRS. Surgery is indicated when conservative approaches fail but postoperative relapse is frequent. In summary, upper airway involvement in CF is undertreated and requires prospective investigation and an interdisciplinary consensus on diagnosis and therapy.
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Substantially reduced calcaneal bone ultrasound parameters in severe untreated asthma.
Respiration
PUBLISHED: 02-21-2009
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We report the case of a 9-year-old male patient with severe bronchial asthma, who had not received inhaled corticosteroids (ICS) despite persistent asthma symptoms and 2 life-threatening asthma attacks. ICS application had been avoided for fear of systemic side effects like bone density loss. Quantitative ultrasound (QUS) is a noninvasive and ionizing radiation-free method to assess bone characteristics. In our clinic, regional age-, sex-, weight- and height-related references for calcaneal QUS were generated from 3,299 children and adolescents. Ultrasonic bone parameters from our patient were substantially reduced at the initial measurement when the patient was 6 years of age by up to -3.75 standard deviation scores. At the same time, chronic pulmonary hyperinflation led to severe thoracic deformation. Only when a second life-threatening asthma attack almost required mechanical ventilation, the family was convinced to have ICS therapy initiated. This treatment led to clinical stabilization including normalization of the thoracic shape. In parallel, QUS values improved continuously up to the normal range. Our case report emphasizes the risks of omitting ICS in severe asthma for fear for systemic side effects. On the contrary, ICS in low to medium dosages may allow regular growth, bone development and normalization of previously severe effects of the disease itself.
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Pathogenesis and management of nasal polyposis in cystic fibrosis.
Curr Allergy Asthma Rep
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Beginning in preschool age, during their lives, upto 50% of cystic fibrosis (CF) patients experience obstructing nasal polyposis (NP), which is rare in non-CF children. Pathogenetic factors of NP in general and especially in CF are still obscure. However, defective epithelial ion transport from mucosal glands plays a central role in CF, and viscous secretions impair mucociliary clearance, promoting chronic pathogen colonization and neutrophil-dominated chronic inflammation.Presently, CF-NP is not curable but can be clinically stabilized,though the large variety of proposed treatment modalities indicates a lack of standardization and of evidence of treatment efficacy. When conservative measures are exhausted, surgical intervention combining individually adapted endoscopic sinus surgery and supportive conservative treatment is performed. Topical steroids, approved as the gold standard for non-CF NP, may be beneficial, but they are discussed to be less effective in neutrophilic inflammation,and CF-specific antimicrobial and mucolytic therapy, as is true of all treatment modalities, urgently requires evaluation by controlled clinical trials within interdisciplinary networks.
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Soluble inflammation markers in nasal lavage from CF patients and healthy controls.
J. Cyst. Fibros.
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CF sinonasal and bronchial mucosa reveal identical ion channel defects. Nasal Lavage (NL) allows non-invasive repeated sampling of airway surface liquid. We compared inflammatory mediators in NL from CF-patients and healthy controls, and in CF in relation to sinonasal pathogen colonization.
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Non-invasive evaluation of cystic fibrosis related liver disease in adults with ARFI, transient elastography and different fibrosis scores.
PLoS ONE
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Cystic fibrosis-related liver disease (CFLD) is present in up to 30% of cystic fibrosis patients and can result in progressive liver failure. Diagnosis of CFLD is challenging. Non-invasive methods for staging of liver fibrosis display an interesting diagnostic approach for CFLD detection.
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Pathogenesis and management of nasal polyposis in cystic fibrosis.
Curr Allergy Asthma Rep
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Beginning in preschool age, during their lives, up to 50% of cystic fibrosis (CF) patients experience obstructing nasal polyposis (NP), which is rare in non-CF children. Pathogenetic factors of NP in general and especially in CF are still obscure. However, defective epithelial ion transport from mucosal glands plays a central role in CF, and viscous secretions impair mucociliary clearance, promoting chronic pathogen colonization and neutrophil-dominated chronic inflammation. Presently, CF-NP is not curable but can be clinically stabilized, though the large variety of proposed treatment modalities indicates a lack of standardization and of evidence of treatment efficacy. When conservative measures are exhausted, surgical intervention combining individually adapted endoscopic sinus surgery and supportive conservative treatment is performed. Topical steroids, approved as the gold standard for non-CF NP, may be beneficial, but they are discussed to be less effective in neutrophilic inflammation, and CF-specific antimicrobial and mucolytic therapy, as is true of all treatment modalities, urgently requires evaluation by controlled clinical trials within interdisciplinary networks.
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Homozygous CFTR mutation M348K in a boy with respiratory symptoms and failure to thrive. Disease-causing mutation or benign alteration?
Eur. J. Pediatr.
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We report on a 6-month-old premature boy from consanguineous parents. He presented with respiratory distress, necrotizing enterocolitis and hyperbilirubinemia shortly after birth. Persisting respiratory symptoms and failure to thrive prompted cystic fibrosis diagnostics, which showed the lack of wild-type signal for the mutation R347P suggesting a homozygous deletion or an alteration different from the known mutation at this position. Sequencing of this region revealed the homozygous substitution 1175 T?>?A (HGVS: c.1043 T?>?A) in exon 7 resulting in the homozygous amino acid change M348K. This mutation has never been reported in homozygosity before. Computational analysis tools classified M348K as presumably disease causing. In our patient, sweat testing and electrophysiological assessment of CFTR function in native rectal epithelium demonstrated normal Cl(-) secretion. Conclusion: We assume that the homozygous alteration M348K is a harmless variant rather than a CF-causing mutation.
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What is Visualize?

JoVE Visualize is a tool created to match the last 5 years of PubMed publications to methods in JoVE's video library.

How does it work?

We use abstracts found on PubMed and match them to JoVE videos to create a list of 10 to 30 related methods videos.

Video X seems to be unrelated to Abstract Y...

In developing our video relationships, we compare around 5 million PubMed articles to our library of over 4,500 methods videos. In some cases the language used in the PubMed abstracts makes matching that content to a JoVE video difficult. In other cases, there happens not to be any content in our video library that is relevant to the topic of a given abstract. In these cases, our algorithms are trying their best to display videos with relevant content, which can sometimes result in matched videos with only a slight relation.