Cystic Fibrosis
Chronic Rhinosinusitis in Patients with Cystic Fibrosis.
Chronic Rhinosinusitis in Patients with Cystic Fibrosis.
J Allergy Clin Immunol Pract. 2016 Jul-Aug;4(4):605-612
Authors: Hamilos DL
Abstract
Chronic rhinosinusitis (CRS) is highly prevalent in patients with cystic fibrosis (CF) and accounts for significant morbidity and contribution to CF lung disease. Mutations of the cystic fibrosis transmembrane regulator gene occur with increased prevalence in patients with CRS without CF, suggesting some contribution to CRS pathophysiology. Nasal polyps (NPs) occur with increased prevalence in patients with CF of all ages and have a more neutrophilic appearance with fewer eosinophils and increased submucosal glandular elements in comparison to NPs from patients without CF. Mainstays of medical treatment include isotonic saline irrigations and topical intranasal glucocorticoids, with some evidence that topical intranasal glucocorticoids reduce NP size. Although inhaled hypertonic saline (7%) has been widely studied as a mucolytic agent for CF lung disease, there are no reports of its use in CF CRS. Mucolytics have also not been studied as a treatment for CRS in CF, and most evidence does not support their use for CF lung disease. Nasally nebulized dornase alfa (recombinant human deoxyribonuclease) following sinus surgery shows promise for treatment. Other unproven therapies include addition of baby shampoo to isotonic saline to potentially thin mucus and help prevent biofilm formation. There are no data to support the use of low-dose oral macrolide antibiotics or the use of prophylactic oral antibiotics for CRS in patients with CF. However, there is some support for the use of topical antibiotics, including colistimethate sodium or tobramycin, administered as a sinus irrigation or antral lavage in patients following sinus surgery when susceptible bacteria are cultured. Key components of CF sinus surgical management include extensive surgery to ensure that the maxillary, frontal, sphenoid, and ethmoid sinuses are all widely opened with smoothing of bony overhangs to prevent mucus retention and bacterial recolonization, postoperative meticulous daily nasal irrigations, and appropriate use of culture-directed topical antibiotics. There are no data yet on whether CF-targeted therapies, including ivacaftor or ivacaftor combined with lumacaftor, have an impact on CF CRS.
PMID: 27393775 [PubMed - as supplied by publisher]
Predictors of Hospital Readmission in Patients Receiving Outpatient Parenteral Antimicrobial Therapy.
Predictors of Hospital Readmission in Patients Receiving Outpatient Parenteral Antimicrobial Therapy.
Pharmacotherapy. 2016 Jul 9;
Authors: Means L, Bleasdale S, Sikka M, Gross AE
Abstract
STUDY OBJECTIVE: Outpatient parenteral antimicrobial therapy (OPAT) is increasingly used and unfortunately, readmissions during OPAT are common. The purpose of this study was to identify predictors of hospital readmission among patients receiving OPAT.
DESIGN: Retrospective cohort study.
SETTING: Large, academic, tertiary-care hospital.
PATIENTS: A total of 216 adults who were discharged and received OPAT through a peripherally inserted central catheter for at least 2 days for treatment of an active infection, excluding patients with cystic fibrosis, between January 2012 and August 2013; of these patients, 43 had hospital readmissions and 173 did not.
MEASUREMENTS AND MAIN RESULTS: The median age of all study patients was 56 years. Common infections included bone and joint (32%), genital/urinary tract (16%), endocarditis (14%), central nervous system (9.7%), and pneumonia (9.7%). For the 43 patients (20%) who had readmissions, reasons for readmission were infection recurrence or progression (33%), adverse drug reactions (24%), central catheter-associated issues (16%), or non-OPAT-related reasons (27%). In the multivariate analysis, patients assigned to a primary care physician were less likely to be readmitted (odds ratio [OR] 0.286, 95% confidence interval [CI] 0.115-0.711), whereas factors independently associated with an increased readmission rate included previous hospital admission in the past 12 months (OR 2.588, 95% CI 1.159-5.778), medical history of malignant lymphoma (OR 25.172, 95% CI 2.311-272.209), and increased planned OPAT duration (OR 1.058, 95% CI 1.034-1.082).
CONCLUSION: Readmissions while patients received OPAT were common. Therefore, proactive interventions including primary care physician assignment to facilitate follow-up and communication should be implemented to decrease the risk of readmission, particularly in the identified high-risk populations. This article is protected by copyright. All rights reserved.
PMID: 27393717 [PubMed - as supplied by publisher]
"Cystic Fibrosis"; +6 new citations
6 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:
These pubmed results were generated on 2016/07/09
PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.
Incorporation of farnesol significantly increases the efficacy of liposomal ciprofloxacin against Pseudomonas aeruginosa biofilms in vitro.
Incorporation of farnesol significantly increases the efficacy of liposomal ciprofloxacin against Pseudomonas aeruginosa biofilms in vitro.
Mol Pharm. 2016 Jul 6;
Authors: Bandara HM, Herpin MJ, Kolacny D, Harb A, Romanovicz D, Smyth HD
Abstract
The challenge of eliminating Pseudomonas aeruginosa infections, such as in cystic fibrosis lungs, remains unchanged due to the rapid development of antibiotic resistance. Poor drug penetration into dense P. aeruginosa biofilms plays a vital role in ineffective clearance of the infection. Thus, the current antibiotic therapy against P. aeruginosa biofilms need to be revisited and alternative anti-biofilm strategies need to be invented. Fungal quorum sensing molecule (QSM), farnesol, appear to have detrimental effects on P. aeruginosa. Thus, this study aimed to co-deliver naturally occurring QSM farnesol, with the antibiotic ciprofloxacin as a liposomal formulation to eradicate P. aeruginosa biofilms. Four different liposomes (with ciprofloxacin and farnesol: Lcip+far, with ciprofloxacin: Lcip, with farnesol: Lfar, control: Lcon) were prepared using dehydration-rehydration method and characterized. Drug entrapment and release were evaluated by spectrometry and high performance liquid chromatography (HPLC). The efficacy of liposomes was assessed using standard biofilm assay. Liposome-treated 24h P. aeruginosa biofilms were quantitatively assessed by XTT reduction assay and crystal violet assay, qualitatively by confocal laser scanning microscopy (CLSM) and transmission electron microscopy (TEM). Ciprofloxacin release from liposomes was higher when encapsulated with farnesol (Lcip+far ) compared to Lcip (3.06% vs 1.48%) whereas farnesol release was lower when encapsulated with ciprofloxacin (Lcip+far ) compared to Lfar (1.81% vs 4.75%). The biofilm metabolism was significantly lower when treated with Lcip+far or Lcip compared to free ciprofloxacin (XTT, P<0.05). When administered as Lcip+far, the ciprofloxacin concentration required to achieve similar biofilm inhibition was 125-fold or 10-fold lower compared to free ciprofloxacin or Lcip respectively (P<0.05). CLSM and TEM confirmed predominant biofilm disruption, greater dead cell ratio and increased depth of biofilm killing when treated with Lcip+far compared to other liposomal preparations. Thus, co-delivery of farnesol and ciprofloxacin is likely to be a promising approach to battle antibiotic resistant P. aeruginosa biofilms by enhancing biofilm killing at significantly lower antibiotic doses.
PMID: 27383205 [PubMed - as supplied by publisher]
"Cystic Fibrosis"; +9 new citations
9 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:
These pubmed results were generated on 2016/07/07
PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.
Synthetic Cystic Fibrosis Sputum Medium Regulates Flagellar Biosynthesis through the flhF Gene in Burkholderia cenocepacia.
Synthetic Cystic Fibrosis Sputum Medium Regulates Flagellar Biosynthesis through the flhF Gene in Burkholderia cenocepacia.
Front Cell Infect Microbiol. 2016;6:65
Authors: Kumar B, Cardona ST
Abstract
Burkholderia cenocepacia belongs to the Burkholderia cepacia complex (Bcc), a group of at least 18 distinct species that establish chronic infections in the lung of people with the genetic disease cystic fibrosis (CF). The sputum of CF patients is rich in amino acids and was previously shown to increase flagellar gene expression in B. cenocepacia. We examined flagellin expression and flagellar morphology of B. cenocepacia grown in synthetic cystic fibrosis sputum medium (SCFM) compared to minimal medium. We found that CF nutritional conditions induce increased motility and flagellin expression. Individual amino acids added at the same concentrations as found in SCFM also increased motility but not flagellin expression, suggesting a chemotactic effect of amino acids. Electron microscopy and flagella staining demonstrated that the increase in flagellin corresponds to a change in the number of flagella per cell. In minimal medium, the ratio of multiple: single: aflagellated cells was 2:3.5:4.5; while under SCFM conditions, the ratio was 7:2:1. We created a deletion mutant, ΔflhF, to study whether this putative GTPase regulates the flagellation pattern of B. cenocepacia K56-2 during growth in CF conditions. The ΔflhF mutant exhibited 80% aflagellated, 14% single and 6% multiple flagellated bacterial subpopulations. Moreover, the ratio of multiple to single flagella in WT and ΔflhF was 3.5 and 0.43, respectively in CF conditions. The observed differences suggest that FlhF positively regulates flagellin expression and the flagellation pattern in B. cenocepacia K56-2 during CF nutritional conditions.
PMID: 27379216 [PubMed - as supplied by publisher]
Active cycle of breathing technique for cystic fibrosis.
Active cycle of breathing technique for cystic fibrosis.
Cochrane Database Syst Rev. 2016 Jul 5;7:CD007862
Authors: Mckoy NA, Wilson LM, Saldanha IJ, Odelola OA, Robinson KA
Abstract
BACKGROUND: People with cystic fibrosis experience chronic airway infections as a result of mucus build up within the lungs. Repeated infections often cause lung damage and disease. Airway clearance therapies aim to improve mucus clearance, increase sputum production, and improve airway function. The active cycle of breathing technique (also known as ACBT) is an airway clearance method that uses a cycle of techniques to loosen airway secretions including breathing control, thoracic expansion exercises, and the forced expiration technique. This is an update of a previously published review.
OBJECTIVES: To compare the clinical effectiveness of the active cycle of breathing technique with other airway clearance therapies in cystic fibrosis.
SEARCH METHODS: We searched the Cochrane Cystic Fibrosis Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the reference lists of relevant articles and reviews.Date of last search: 25 April 2016.
SELECTION CRITERIA: Randomised or quasi-randomised controlled clinical studies, including cross-over studies, comparing the active cycle of breathing technique with other airway clearance therapies in cystic fibrosis.
DATA COLLECTION AND ANALYSIS: Two review authors independently screened each article, abstracted data and assessed the risk of bias of each study.
MAIN RESULTS: Our search identified 62 studies, of which 19 (440 participants) met the inclusion criteria. Five randomised controlled studies (192 participants) were included in the meta-analysis; three were of cross-over design. The 14 remaining studies were cross-over studies with inadequate reports for complete assessment. The study size ranged from seven to 65 participants. The age of the participants ranged from six to 63 years (mean age 22.33 years). In 13 studies, follow up lasted a single day. However, there were two long-term randomised controlled studies with follow up of one to three years. Most of the studies did not report on key quality items, and therefore, have an unclear risk of bias in terms of random sequence generation, allocation concealment, and outcome assessor blinding. Due to the nature of the intervention, none of the studies blinded participants or the personnel applying the interventions. However, most of the studies reported on all planned outcomes, had adequate follow up, assessed compliance, and used an intention-to-treat analysis.Included studies compared the active cycle of breathing technique with autogenic drainage, airway oscillating devices, high frequency chest compression devices, conventional chest physiotherapy, and positive expiratory pressure. Preference of technique varied: more participants preferred autogenic drainage over the active cycle of breathing technique; more preferred the active cycle of breathing technique over airway oscillating devices; and more were comfortable with the active cycle of breathing technique versus high frequency chest compression. No significant difference was seen in quality of life, sputum weight, exercise tolerance, lung function, or oxygen saturation between the active cycle of breathing technique and autogenic drainage or between the active cycle of breathing technique and airway oscillating devices. There was no significant difference in lung function and the number of pulmonary exacerbations between the active cycle of breathing technique alone or in conjunction with conventional chest physiotherapy. All other outcomes were either not measured or had insufficient data for analysis.
AUTHORS' CONCLUSIONS: There is insufficient evidence to support or reject the use of the active cycle of breathing technique over any other airway clearance therapy. Five studies, with data from eight different comparators, found that the active cycle of breathing technique was comparable with other therapies in outcomes such as participant preference, quality of life, exercise tolerance, lung function, sputum weight, oxygen saturation, and number of pulmonary exacerbations. Longer-term studies are needed to more adequately assess the effects of the active cycle of breathing technique on outcomes important for people with cystic fibrosis such as quality of life and preference.
PMID: 27378490 [PubMed - as supplied by publisher]
Eradication of methicillin resistant Staphylococcus aureus detected for the first time in cystic fibrosis: A single center observational study.
Eradication of methicillin resistant Staphylococcus aureus detected for the first time in cystic fibrosis: A single center observational study.
Pediatr Pulmonol. 2016 Jul 5;
Authors: Kappler M, Nagel F, Feilcke M, Kröner C, Pawlita I, Naehrig S, Ripper J, Hengst M, von Both U, Forstner M, Hector A, Griese M
Abstract
OBJECTIVE: To retrospectively identify CF patients with methicillin resistant Staphylococcus aureus (MRSA) and to assess the long-term success of an eradication scheme introduced in 2002 for all newly colonized patients.
PATIENTS: All microbiological results from all 505 CF patients followed between 2002 and 2012 were analyzed focusing on the detection of MRSA.
METHODS: Retrospective patient record analysis of MRSA positive CF patients regarding eradication and clinical outcome.
RESULTS: We identified 57 patients with MRSA, mean age 15.3 years (range: 0.6-36.9, incidence 0.9%/year). Of these, nine patients were lost to follow-up; seven chronically colonized patients were excluded from the intervention. Eradication was suggested to all patients, 37/41 gave their consent to the following two-step approach: (i) dual iv antibiotic treatment over 3 weeks, accompanied by hygienic directives and topical therapy for 5 days followed by a 6-week period with dual oral antibiotic therapy and inhalation with vancomycin. (ii) Each new MRSA detection was treated with 6 weeks inhalation of vancomycin and topical therapy for 5 days. Long-term eradication was rated by the microbiological status in the third year after first detection. MRSA was eradicated in 31 of 37 patients (84%) whose clinical course was stable (mean FEV1 one year before MRSA 80.4%, 3 years after MRSA 81.0%).
CONCLUSIONS: MRSA colonization mandates complex and expensive hygienic measures which are not well accepted by patients. Therefore, MRSA eradication is desirable. Intensive therapy regimens may be successful in patients with CF and might help to maintain a stable clinical course. Pediatr Pulmonol. © 2016 Wiley Periodicals, Inc.
PMID: 27378061 [PubMed - as supplied by publisher]
Colonization of CF patients' upper airways with S. aureus contributes more decisively to upper airway inflammation than P. aeruginosa.
Colonization of CF patients' upper airways with S. aureus contributes more decisively to upper airway inflammation than P. aeruginosa.
Med Microbiol Immunol. 2016 Jul 4;
Authors: Janhsen WK, Arnold C, Hentschel J, Lehmann T, Pfister W, Baier M, Böer K, Hünniger K, Kurzai O, Hipler UC, Mainz JG
Abstract
In cystic fibrosis (CF) patients' airways, inflammatory processes decisively contribute to remodeling and pulmonary destruction. The aims of this study were to compare upper airway (UAW) inflammation in the context of Staphylococcus aureus and Pseudomonas aeruginosa colonization in a longitudinal setting, and to examine further factors influencing UAW inflammation. Therefore, we analyzed soluble inflammatory mediators in noninvasively obtained nasal lavage (NL) of CF patients together with microbiology, medication, and relevant clinical parameters. NL, applying 10 mL of isotonic saline per nostril, was serially performed in 74 CF patients (326 samples). Concentrations of the inflammatory mediators' interleukin (IL)-1β, IL-6, IL-8, matrix metalloproteinase (MMP)-9, and its anti-protease TIMP-1 were quantified by bead-based multiplexed assay, neutrophil elastase (NE) via ELISA. Culture-based microbiology of the upper and lower airways (LAW), as well as serological and clinical findings, were compiled. Our results indicate that UAW colonization with S. aureus significantly impacts the concentration of all measured inflammatory mediators in NL fluid except TIMP-1, whereas these effects were not significant for P. aeruginosa. Patients with S. aureus colonization of both the UAW and LAW showed significantly increased concentrations of IL-1β, IL-6, IL-8, MMP-9, and slightly elevated concentrations of NE in NL fluid compared to non-colonized patients. This work elaborates a survey on S. aureus' virulence factors that may contribute to this underestimated pathology. Serial assessment of epithelial lining fluid by NL reveals that colonization of the UAW with S. aureus contributes more to CF airway inflammatory processes than hitherto expected.
PMID: 27377929 [PubMed - as supplied by publisher]
Classification of CFTR mutation classes.
Classification of CFTR mutation classes.
Lancet Respir Med. 2016 Jul 1;
Authors: Stanke F, Tümmler B
PMID: 27377412 [PubMed - as supplied by publisher]
"Cystic Fibrosis"; +9 new citations
9 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:
These pubmed results were generated on 2016/07/05
PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.
Research and drug development activities in rare diseases: differences between Japan and Europe regarding influence of prevalence.
Research and drug development activities in rare diseases: differences between Japan and Europe regarding influence of prevalence.
Drug Discov Today. 2016 Jun 28;
Authors: Mizoguchi H, Yamanaka T, Kano S
Abstract
Orphan drug legislation has contributed enormously to promote drug development for rare diseases but further effective and sustainable approaches are required. This study focused on the difference of rare disease prevalence between Japan and Europe, classified the rare diseases comprehensively using cluster analysis and analyzed the influence of prevalence on research activity and drug development. Although overall strong correlative progress of research was found and absolute numbers of values were greater in Europe than in Japan, the regional higher prevalent diseases demonstrated more progress of research and development relatively in the region by examining clusters. Our findings suggest potential optimal drug development in consideration of regional differences. Moreover, an in-depth analysis of diseases that showed exceptional research achievements compared with prevalence speculated important determinants of progress.
PMID: 27371505 [PubMed - as supplied by publisher]
A tale of two sites: how inflammation can reshape the microbiomes of the gut and lungs.
A tale of two sites: how inflammation can reshape the microbiomes of the gut and lungs.
J Leukoc Biol. 2016 Jun 30;
Authors: Scales BS, Dickson RP, Huffnagle GB
Abstract
Inflammation can directly and indirectly modulate the bacterial composition of the microbiome. Although studies of inflammation primarily focus on its function to negatively select against potential pathogens, some bacterial species have the ability to exploit inflammatory byproducts for their benefit. Inflammatory cells release reactive nitrogen species as antimicrobial effectors against infection, but some facultative anaerobes can also utilize the increase in extracellular nitrate in their environment for anaerobic respiration and growth. This phenomenon has been studied in the gastrointestinal tract, where blooms of facultative anaerobic Gammaproteobacteria, primarily Escherichia coli, often occur during colonic inflammation. In cystic fibrosis, Pseudomonas aeruginosa, another Gammaproteobacteria facultative anaerobe, can reduce nitrogen for anaerobic respiration and it blooms in the airways of the chronically inflamed cystic fibrosis lung. This review focuses on the evidence that inflammation can provide terminal electron acceptors for anaerobic respiration and can support blooms of facultative anaerobes, such as E. coli and P. aeruginosa in distinct, but similar, environments of the inflamed gastrointestinal and respiratory tracts.
PMID: 27365534 [PubMed - as supplied by publisher]
Fungal epidemiology and diversity in cystic fibrosis patients over a 5-year period in a national reference center.
Fungal epidemiology and diversity in cystic fibrosis patients over a 5-year period in a national reference center.
Med Mycol. 2016 Jun 30;
Authors: Ziesing S, Suerbaum S, Sedlacek L
Abstract
The knowledge on prevalence rates of yeasts and moulds in patients with cystic fibrosis (CF) in Germany is scarce. The aim of this report is to give an overview of the diversity and epidemiology of fungal species in CF patients. Over a 5-year period, all fungal isolates cultured from microbiological specimen from CF patients were recorded. Beside standard bacteriological culture media two fungal media were used for cultivation. Species were identified by microscopy, biochemical profiling, MALDI-TOF analysis or DNA sequencing methods. In sum, 25,975 clinical samples from CF patients were analyzed. About 75% of CF patients were colonized by yeasts, mainly Candida albicans (38%) and Candida dubliniensis (12%). In 35% of the patients Aspergillus spp. (Aspergillus fumigatus: 29%) were detected, followed by Exophiala dermatitidis and Scedosporium/Lomentospora complex isolates (4% each). Data for other fungal species are shown. Over a 5-year period, the epidemiology of fungal species detected in CF patients was relatively constant. Clinical microbiology laboratories should carefully monitor samples from CF patients for newly occurring fungal pathogens.
PMID: 27364649 [PubMed - as supplied by publisher]
Phenotypic variability of R117H-CFTR expression within monozygotic twins.
Phenotypic variability of R117H-CFTR expression within monozygotic twins.
Paediatr Respir Rev. 2016 Jun 15;
Authors: Waller MD, Simmonds NJ
Abstract
Whilst cystic fibrosis is a monogenic condition, variation in phenotype exists for the same CFTR genotype, which is influenced by multiple genetic and non-genetic (environmental) factors. The R117H-CFTR mutation has variability directly relating to in cis poly-thymidine alleles, producing a differing spectrum of disease. This paper provides evidence of extreme phenotype variability-including fertility status - in the context of male monogenetic twins, discussing mechanisms and highlighting the diagnostic and treatment challenges.
PMID: 27364092 [PubMed - as supplied by publisher]
Promising gene therapies pose million-dollar conundrum.
Promising gene therapies pose million-dollar conundrum.
Nature. 2016 Jun 16;534(7607):305-6
Authors: Check Hayden E
PMID: 27306167 [PubMed - indexed for MEDLINE]
"Cystic Fibrosis"; +12 new citations
12 new pubmed citations were retrieved for your search. Click on the search hyperlink below to display the complete search results:
These pubmed results were generated on 2016/07/01
PubMed comprises more than 24 million citations for biomedical literature from MEDLINE, life science journals, and online books. Citations may include links to full-text content from PubMed Central and publisher web sites.
Pyrosequencing Unveils Cystic Fibrosis Lung Microbiome Differences Associated with a Severe Lung Function Decline.
Pyrosequencing Unveils Cystic Fibrosis Lung Microbiome Differences Associated with a Severe Lung Function Decline.
PLoS One. 2016;11(6):e0156807
Authors: Bacci G, Paganin P, Lopez L, Vanni C, Dalmastri C, Cantale C, Daddiego L, Perrotta G, Dolce D, Morelli P, Tuccio V, De Alessandri A, Fiscarelli EV, Taccetti G, Lucidi V, Bevivino A, Mengoni A
Abstract
Chronic airway infection is a hallmark feature of cystic fibrosis (CF) disease. In the present study, sputum samples from CF patients were collected and characterized by 16S rRNA gene-targeted approach, to assess how lung microbiota composition changes following a severe decline in lung function. In particular, we compared the airway microbiota of two groups of patients with CF, i.e. patients with a substantial decline in their lung function (SD) and patients with a stable lung function (S). The two groups showed a different bacterial composition, with SD patients reporting a more heterogeneous community than the S ones. Pseudomonas was the dominant genus in both S and SD patients followed by Staphylococcus and Prevotella. Other than the classical CF pathogens and the most commonly identified non-classical genera in CF, we found the presence of the unusual anaerobic genus Sneathia. Moreover, the oligotyping analysis revealed the presence of other minor genera described in CF, highlighting the polymicrobial nature of CF infection. Finally, the analysis of correlation and anti-correlation networks showed the presence of antagonism and ecological independence between members of Pseudomonas genus and the rest of CF airways microbiota, with S patients showing a more interconnected community in S patients than in SD ones. This population structure suggests a higher resilience of S microbiota with respect to SD, which in turn may hinder the potential adverse impact of aggressive pathogens (e.g. Pseudomonas). In conclusion, our findings shed a new light on CF airway microbiota ecology, improving current knowledge about its composition and polymicrobial interactions in patients with CF.
PMID: 27355625 [PubMed - as supplied by publisher]
Validity and Reliability Concerns Associated with Cardiopulmonary Exercise Testing Young People with Cystic Fibrosis.
Validity and Reliability Concerns Associated with Cardiopulmonary Exercise Testing Young People with Cystic Fibrosis.
Respiration. 2016 Jun 30;
Authors: Saynor ZL, Barker AR, Oades PJ, Tomlinson OW, Williams CA
PMID: 27355590 [PubMed - as supplied by publisher]
Specific phenotype and function of CD56-expressing innate immune cell subsets in human thymus.
Specific phenotype and function of CD56-expressing innate immune cell subsets in human thymus.
J Leukoc Biol. 2016 Jun 28;
Authors: Gerstner S, Köhler W, Heidkamp G, Purbojo A, Uchida S, Ekici AB, Heger L, Luetke-Eversloh M, Schubert R, Bader P, Klingebiel T, Koehl U, Mackensen A, Romagnani C, Cesnjevar R, Dudziak D, Ullrich E
Abstract
Whereas innate immune cells, such as NK and innate lymphoid cells (ILCs), have been characterized in different human tissues, knowledge on the thymic CD56-expressing cell subsets is limited. In this study, the rare subpopulations of thymic CD56(+)CD3(-) cells from samples of >100 patients have been successfully analyzed. The results revealed fundamental differences between thymic and peripheral blood (PB) CD56(+)CD3(-) cells. Thymic tissues lacked immunoregulatory CD56(high)CD16(dim) NK cells but showed two Eomes(+)CD56(dim) subsets on which common NK cell markers were significantly altered. CD56(dim)CD16(high) cells expressed high amounts of NKG2A, NKG2D, and CD27 with low CD57. Conversely, CD56(dim)CD16(dim) cells displayed high CD127 but low expression of KIR, NKG2D, and natural cytotoxicity receptors (NCRs). Thymic CD56(+)CD3(-) cells were able to gain cytotoxicity but were especially immunoregulatory cells, producing a broad range of cytokines. Finally, one population of thymic CD56(+) cells resembled conventional NK cells, whereas the other represented a novel, noncanonical NK subset.
PMID: 27354408 [PubMed - as supplied by publisher]