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  • 1
    Article
    Article
    Association between Stenotrophomonas maltophilia and lung function in cystic fibrosis
    2004
    ISSN: 0040-6376 
    Details
    Language: English
    In: Thorax, 2004-11, Vol.59 (11), p.955-959
    Description: Background:Stenotrophomonas maltophilia (SM) is a Gram-negative non-fermenting bacteria cultured from the sputum of patients with cystic fibrosis (CF). To date, no information is available regarding the effect of this organism on lung function in CF. Methods: A cohort study was conducted to assess the effect of SM on lung function among CF patients aged ⩾6 years in the CF Foundation National Patient Registry from 1994 to 1999. Repeated measures regression was used to assess the association between SM and lung function. Results: The cohort consisted of 20 755 patients with median age at entry of 13.8 years and median follow up time of 3.8 years; 2739 patients (13%) were positive at least once for SM and 18 016 (87%) were never positive. After adjusting for sex, height and age, patients with SM had a mean forced expiratory volume in 1 second which was 0.09 l less (95% CI 0.05 to 0.14) than those without SM. The mean rate of decline associated with SM positivity was 0.025 l/year (95% CI 0.012 to 0.037) but, after adjusting for confounders (sex, height, weight, intravenous antibiotic courses, hospital admissions, pancreatic insufficiency, and Pseudomonas aeruginosa and Burkholderia cepacia status), the mean rate of decline decreased to 0.008 l/year (−0.008, 95% CI −0.019 to 0.003). Conclusions: Although CF patients with SM have worse lung function at the time of positivity, no association was found between SM and increased rate of decline after controlling for confounders.
    Subject(s): Adolescent ; Adult ; Age ; Antibiotics ; Biological and medical sciences ; Burkholderia cepacia ; Child ; Child, Preschool ; Cohort Studies ; Cystic Fibrosis ; Cystic Fibrosis - microbiology ; Cystic Fibrosis - physiopathology ; Female ; Forced Expiratory Volume - physiology ; Gastroenterology. Liver. Pancreas. Abdomen ; Gram-negative bacteria ; Gram-Negative Bacterial Infections - complications ; Gram-Negative Bacterial Infections - diagnosis ; Gram-Negative Bacterial Infections - physiopathology ; Humans ; Infant ; Liver. Biliary tract. Portal circulation. Exocrine pancreas ; lung function ; Male ; Medical prognosis ; Medical sciences ; Other diseases. Semiology ; outcome ; Pathogens ; Patients ; Pediatrics ; Pneumology ; Pseudomonas aeruginosa ; Pulmonary function tests ; Research ; Respiratory system : syndromes and miscellaneous diseases ; Stenotrophomonas maltophilia ; Studies
    ISSN: 0040-6376
    E-ISSN: 1468-3296
    DOI: 10.1136/thx.2003.017707
    Source: HighWire Press (Free Journals)
    Source: Hellenic Academic Libraries Link
    Source: PubMed Central
    URL: http://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=16271570$$DView record in Pascal Francis
    URL: https://www.ncbi.nlm.nih.gov/pubmed/15516471$$D View this record in MEDLINE/PubMed
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  • 2
    Article
    Article
    713 CT Scores Are Predictive of Survival in CF Patients Awaiting Lung Transplantation
    2012
    ISSN: 1053-2498 
    Details
    Language: English
    In: The Journal of heart and lung transplantation, 2012, Vol.31 (4), p.S244-S244
    Description: Byline: M. Loeve (1)(2), W.C.J. Hop (3), M. de Bruijne (2)(4), P. Th.W. van Hal (5), P. Robinson (6), M.L. Aitken (7), J.D. Dodd (8), H.A.W.M. Tiddens (1)(2) Author Affiliation: (1) Pediatric Pulmonology, Erasmuc MC Sophia Children's Hospital, Rotterdam, Zuid Holland, Netherlands (2) Radiology, Erasmus MC, Rotterdam, Zuid Holland, Netherlands (3) Biostatistics, Erasmus MC, Rotterdam, Zuid Holland, Netherlands (4) Medical Informatics, Erasmus MC, Rotterdam, Zuid Holland, Netherlands (5) Respiratory Medicine, Erasmus MC, Rotterdam, Zuid Holland, Netherlands (6) Pediatric Pulmonology, Royal Children's Hospital Melbourne, Melbourne, VIC, Australia (7) Pulmonary and Critical Care Medicine, University of Washington, Seattle, WA (8) Radiology, Sint Vincent's Hospital, Dublin, Ireland
    Subject(s): Medical informatics ; Surgery ; Transplantation of organs, tissues, etc
    ISSN: 1053-2498
    E-ISSN: 1557-3117
    DOI: 10.1016/j.healun.2012.01.728
    Source: Alma/SFX Local Collection
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  • 3
    Article
    Article
    Implementation of a generalized actuator disk wind turbine model into the weather research and forecasting model for large-eddy simulation applications
    2014
    ISSN: 1941-7012 
    Details
    Language: English
    In: Journal of renewable and sustainable energy, 2014-01, Vol.6 (1), p.13104
    Description: A generalized actuator disk (GAD) wind turbine parameterization designed for large-eddy simulation (LES) applications was implemented into the Weather Research and Forecasting (WRF) model. WRF-LES with the GAD model enables numerical investigation of the effects of an operating wind turbine on and interactions with a broad range of atmospheric boundary layer phenomena. Numerical simulations using WRF-LES with the GAD model were compared with measurements obtained from the Turbine Wake and Inflow Characterization Study (TWICS-2011), the goal of which was to measure both the inflow to and wake from a 2.3-MW wind turbine. Data from a meteorological tower and two light-detection and ranging (lidar) systems, one vertically profiling and another operated over a variety of scanning modes, were utilized to obtain forcing for the simulations, and to evaluate characteristics of the simulated wakes. Simulations produced wakes with physically consistent rotation and velocity deficits. Two surface heat flux values of 20 W m−2 and 100 W m−2 were used to examine the sensitivity of the simulated wakes to convective instability. Simulations using the smaller heat flux values showed good agreement with wake deficits observed during TWICS-2011, whereas those using the larger value showed enhanced spreading and more-rapid attenuation. This study demonstrates the utility of actuator models implemented within atmospheric LES to address a range of atmospheric science and engineering applications. Validated implementation of the GAD in a numerical weather prediction code such as WRF will enable a wide range of studies related to the interaction of wind turbines with the atmosphere and surface.
    Subject(s): Actuators ; Atmospheric attenuation ; Atmospheric boundary layer ; Atmospheric models ; Computer simulation ; Heat flux ; Heat transfer ; Inflow ; Large eddy simulation ; Lidar ; Parameterization ; Simulation ; Stability ; Turbines ; Vortices ; Wakes ; Weather forecasting ; Wind effects ; Wind turbines
    ISSN: 1941-7012
    E-ISSN: 1941-7012
    DOI: 10.1063/1.4861061
    Source: American Institute of Physics (AIP) Publications
    Source: American Institute of Physics:Jisc Collections:Complete Collection with extended back-file:2017-2019
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  • 4
    Article
    Article
    Chest computed tomography scores are predictive of survival in patients with cystic fibrosis awaiting lung transplantation
    2012
    ISSN: 1073-449X 
    Details
    Language: English
    In: American journal of respiratory and critical care medicine, 2012-05-15, Vol.185 (10), p.1096-1103
    Description: Rationale: Up to one-third of patients with cystic fibrosis (CF) awaiting lung transplantation (LTX) die while waiting. Inclusion of computed tomography (CT) scores may improve survival prediction models such as the lung allocation score (LAS). Objectives: This study investigated the association between CT and survival in patients with CF screened for LTX. Methods: Clinical data and chest CTs of 411 patients with CF screened for LTX between 1990 and 2005 were collected from 17 centers. CTs were scored with the Severe Advanced Lung Disease (SALD) four-category scoring system, including the components infection/inflammation (INF), air trapping/hypoperfusion (AT), normal/hyperperfusion (NOR), and bulla/cysts (BUL). The volume of each componentwas computed using semiautomated software. Survival analysis included Kaplan-Meier curves and Cox regression models. Measurements and Main Results: Three hundred and sixty-six (186 males) of 411 patients entered the waiting list (median age, 23 yr; range, 5-58 yr). Subsequently, 67 of 366 (18%) died while waiting, 263 of 366 (72%) underwent LTX, and 36 of 366 (10%) were awaiting LTX at the census date. INF and LAS were significantly associated with waiting list mortality in univariate analyses. The multivariate Cox model including INF and LAS grouped in tertiles, and comparing tertiles 2 and 3 with tertile 1, showed waiting list mortality hazard ratios of 1.62 (95% confidence interval [95% CI], 0.78-3.36; P=0.19) and 2.65 (95% CI, 1.35-5.20; P = 0.005) for INF, and 1.42 (95% CI, 0.63-3.24; P=0.40), and 2.32 (95% CI, 1.17-4.60; P=0.016) for LAS, respectively. These results indicated that INF and LAS had significant, independent predictive value for survival. Conclusions: CT score INF correlates with survival, and adds to the predictive value of LAS. Copyright
    Subject(s): Abridged Index Medicus ; Adolescent ; Adult ; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy ; Biological and medical sciences ; Child ; Child, Preschool ; Cohort Studies ; Cystic fibrosis ; Cystic Fibrosis - diagnostic imaging ; Cystic Fibrosis - mortality ; Cystic Fibrosis - surgery ; Decision Support Techniques ; Female ; Humans ; Intensive care medicine ; Investigative techniques, diagnostic techniques (general aspects) ; Kaplan-Meier Estimate ; Lung disease ; Lung Transplantation ; Male ; Medical sciences ; Middle Aged ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Radiodiagnosis. Nmr imagery. Nmr spectrometry ; Respiratory system ; Severity of Illness Index ; Tomography X-ray computed ; Waiting list survival ; Waiting Lists - mortality ; Young Adult
    ISSN: 1073-449X
    E-ISSN: 1535-4970
    DOI: 10.1164/rccm.201111-2065OC
    Source: Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
    Source: ProQuest Central
    URL: http://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=25856464$$DView record in Pascal Francis
    URL: https://www.ncbi.nlm.nih.gov/pubmed/22403801$$D View this record in MEDLINE/PubMed
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  • 5
    Article
    Article
    New and Emerging Therapies for Pulmonary Complications of Cystic Fibrosis
    2001
    ISSN: 0012-6667 
    Details
    Language: English
    In: Drugs (New York, N.Y.), 2001, Vol.61 (10), p.1379-1385
    Description: In the decade since the gene for cystic fibrosis (CF) was discovered, research into potential therapeutic interventions has progressed on a number of different fronts. The vast majority of morbidity and mortality in CF results from inflammation and infection of the airways. Direct delivery of antibacterials to the airway secretions via a nebuliser is an attractive therapeutic option, and a novel formulation of tobramycin designed for such a purpose has been demonstrated to improve spirometry and decrease the need for intravenous antibacterials. In addition, early clinical trials are studying the effects of small peptides with antibiotic properties (defensins) delivered directly to the airways.Inflammation, whether secondary to infection or an independent feature of CF, leads to progressive bronchiectasis. Anti-inflammatories such as prednisone and possibly ibuprofen have been shown to decrease the rate of respiratory decline in patients with CF but have tolerability profiles that limit clinical usefulness. Macrolides also have anti-inflammatory properties and clinical trials are now ongoing to assess the efficacy of these agents in CF.Multiple agents, including uridine triphosphate (UTP), genistein, phenylbutyrate and CPX (cyclopentyl dipropylxanthine), have been demonstrated in cell culture to at least partially correct the primary defect of ion transport related to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). No agent of this class has yet demonstrated clinical effectiveness, but several are in preclinical and early clinical trials.Finally, gene therapy that allows for the incorporation and expression of wild-type CFTR in respiratory epithelial cells would be definitive therapy for CF. However, multiple barriers to delivery and expression need to be overcome. With research proceeding on these multiple fronts, new therapies for pulmonary complications promise to continue to increase the life expectancy of individuals with CF.
    Subject(s): Anti inflammatories, therapeutic use ; Anti-Bacterial Agents - therapeutic use ; Anti-Inflammatory Agents - pharmacology ; Anti-Inflammatory Agents - therapeutic use ; Antibacterials, therapeutic use ; Biological and medical sciences ; Cystic fibrosis ; Cystic Fibrosis - complications ; Cystic Fibrosis - drug therapy ; Cystic Fibrosis Transmembrane Conductance Regulator - drug effects ; Enzyme Inhibitors - pharmacology ; Enzyme Inhibitors - therapeutic use ; Errors of metabolism ; Gene Expression Regulation ; Gene therapies, therapeutic use ; Genetic Therapy ; Humans ; Inflammation ; Lung Diseases - drug therapy ; Lung Diseases - etiology ; Lung disorders, treatment ; Medical sciences ; Metabolic diseases ; Miscellaneous hereditary metabolic disorders ; Prognosis ; Steroids
    ISSN: 0012-6667
    E-ISSN: 1179-1950
    DOI: 10.2165/00003495-200161100-00001
    Source: Alma/SFX Local Collection
    URL: http://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=1106017$$DView record in Pascal Francis
    URL: https://www.ncbi.nlm.nih.gov/pubmed/11558827$$D View this record in MEDLINE/PubMed
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  • 6
    Article
    Article
    A phase I study of aerosolized administration of tgAAVCF to cystic fibrosis subjects with mild lung disease
    2001
    ISSN: 1043-0342 
    Details
    Language: English
    In: Human gene therapy, 2001, Vol.12 (15), p.1907-1916
    Description: Cystic fibrosis (CF) is one of the most common autosomal recessive disorders in North America, leading to significant morbidity and early mortality. The defect in the cystic fibrosis transmembrane conductance regulator protein (CFTR) function can be corrected in vitro by gene replacement with a wild-type gene. A Phase I, single administration, dose escalation trial was designed and executed to assess safety and delivery of tgAAVCF, an adeno-associated virus (AAV) vector encoding the human CFTR cDNA, by nebulization to the lungs of CF subjects. Four cohorts of three subjects each were administered increasing doses of the study agent, beginning with 10(10) DNase-resistant particles (DRP) and escalating in log increments up to 10(13) DRP. Sequential bronchoscopies were performed to gather analytical samples throughout the study. All 12 subjects completed the study. There were a total of 242 adverse events (AEs), six of which were defined as serious and three of which were defined as possibly being related to the study drug. A clear dose-response relationship was observed in vector gene transfer. A maximum of 0.6 and 0.1 vector copies per brushed cell were observed 14 days and 30 days, respectively, following nebulization of 10(13) DRP tgAAVCF, and this declined to nearly undetectable levels by day 90. Vector gene transfer was evenly distributed throughout the fourth airway generation following single-dose administration. RNA-specific PCR did not detect vector-derived mRNA. This Phase I trial shows that aerosolized tgAAVCF is safe and widely delivered to the proximal airways of CF subjects by nebulization.
    Subject(s): Adult ; Alleles ; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy ; Applied cell therapy and gene therapy ; Biological and medical sciences ; Biotechnology ; Cells, Cultured ; CFTR protein ; Cystic Fibrosis - genetics ; Cystic Fibrosis - therapy ; Cystic Fibrosis Transmembrane Conductance Regulator - genetics ; Cytokines - metabolism ; Dependovirus - genetics ; DNA, Complementary - metabolism ; Dose-Response Relationship, Drug ; Enzyme-Linked Immunosorbent Assay ; Female ; Fundamental and applied biological sciences. Psychology ; Gene therapy ; Gene Transfer Techniques ; Genetic Therapy - adverse effects ; Genetic Vectors ; Health. Pharmaceutical industry ; HeLa Cells ; Humans ; Immunohistochemistry ; Industrial applications and implications. Economical aspects ; Lung - physiology ; Lung Diseases - therapy ; Male ; Medical sciences ; Mutation ; Nebulizers and Vaporizers ; Polymerase Chain Reaction ; Reverse Transcriptase Polymerase Chain Reaction ; Time Factors ; Transfusions. Complications. Transfusion reactions. Cell and gene therapy
    ISSN: 1043-0342
    E-ISSN: 1557-7422
    DOI: 10.1089/104303401753153956
    Source: Alma/SFX Local Collection
    URL: http://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=14141587$$DView record in Pascal Francis
    URL: https://www.ncbi.nlm.nih.gov/pubmed/11589832$$D View this record in MEDLINE/PubMed
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  • 7
    Article
    Article
    Long-Term Inhaled Dry Powder Mannitol in Cystic Fibrosis An International Randomized Study
    2012
    ISSN: 1073-449X 
    Details
    Language: English
    In: American journal of respiratory and critical care medicine, 2012, Vol.185 (6), p.645-652
    ISSN: 1073-449X
    E-ISSN: 1535-4970
    Source: Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
    Source: ProQuest Central
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  • 8
    Article
    Article
    Sputum induction as a research tool for sampling the airways of subjects with cystic fibrosis
    2001
    ISSN: 0040-6376 
    Details
    Language: English
    In: Thorax, 2001-04, Vol.56 (4), p.306-311
    Description: BACKGROUND Sputum induction (SI) has proved to be a reliable non-invasive tool for sampling inflammatory airway contents in asthma, with distinct advantages over collection of expectorated sputum (ES) and bronchoalveolar lavage (BAL). A study was undertaken to evaluate the safety of SI and to assess if it might be an equally valuable outcome tool in patients with cystic fibrosis (CF). METHODS The safety of the procedure was examined and sample volume, cell counts, cytokine concentrations, and bacterial culture results obtained by SI, spontaneous ES, and fibreoptic bronchoscopy were compared in 10 adults with CF. RESULTS SI was well tolerated and was preferred to BAL by all subjects. The mean (SE) sample volume obtained by SI was significantly greater than ES (6.74 (1.46) ml v 1.85 (0.33) ml, p = 0.005). There was no significant difference in the number of cells per ml of sample collected. There was a difference in the mean (SD) percentage of non-epithelial, non-squamous cells collected (67 (28)%, 86 (21)%, and 99 (1)% for ES, SI, and BAL, respectively). These percentage counts were different between ES and both SI and BAL (p=0.03 and p=0.006, respectively). Cell differential counts (excluding squamous cells) from all collection methods were similar (mean (SD) 84 (9)%, 87 (7)%, and 88 (11)% polymorphonuclear cells for ES, SI, and BAL, respectively). The concentrations of interleukin (IL)-8 and tumour necrosis factor (TNF)-α were the same in all three samples when corrected for dilution using urea concentration. The test specific detection rate for recovery of bacteriological pathogens was 79% for SI, 76% for ES, and 73% for BAL. CONCLUSION SI offers safety advantages over BAL and may be a more representative airway outcome measurement in patients with CF.
    Subject(s): Adult ; Airway (Medicine) ; Analysis of Variance ; Biological and medical sciences ; Bronchoalveolar Lavage - methods ; Bronchoalveolar Lavage Fluid - chemistry ; Bronchoalveolar Lavage Fluid - cytology ; Cystic fibrosis ; Cystic Fibrosis - complications ; Cystic Fibrosis - pathology ; Cytokines - analysis ; Female ; Forced Expiratory Volume - physiology ; Gastroenterology. Liver. Pancreas. Abdomen ; Humans ; Liver. Biliary tract. Portal circulation. Exocrine pancreas ; Male ; Medical examination ; Medical sciences ; Original ; Other diseases. Semiology ; Research ; Saline Solution, Hypertonic - administration & dosage ; Sputum ; Sputum - chemistry ; Sputum - cytology ; Urea - analysis
    ISSN: 0040-6376
    E-ISSN: 1468-3296
    DOI: 10.1136/thorax.56.4.306
    Source: HighWire Press (Free Journals)
    Source: Hellenic Academic Libraries Link
    Source: PubMed Central
    URL: http://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=953524$$DView record in Pascal Francis
    URL: https://www.ncbi.nlm.nih.gov/pubmed/11254823$$D View this record in MEDLINE/PubMed
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  • 9
    Article
    Article
    Complications of indwelling catheters in cystic fibrosis : A 10-year review
    2000
    ISSN: 0012-3692 
    Details
    Language: English
    In: Chest, 2000, Vol.118 (6), p.1598-1602
    Description: Patients with cystic fibrosis (CF) frequently require recurrent courses of IV antibiotics to treat acute exacerbations of their pulmonary disease. Over time, CF patients often lose peripheral access, and indwelling central venous catheters are placed. We attempted to determine the type and incidence of catheter complications so that CF patients could be fully informed of the risks prior to placement of these catheters. The charts of all CF patients who attended the Adult Cystic Fibrosis Clinic of the University of Washington Medical Center from January 1989 through December 1998 were reviewed. Demographic information was obtained along with the type and duration of catheter, type and number of complications, and the use of anticoagulant medication. Of the 218 CF patients who attended the clinic, 65 patients (30%) had indwelling catheters in place at some time during the study period. A total of 87 catheters were placed into these 65 patients. The total number of catheter-days for first indwelling catheters was 68,220. The total number of catheter-days for all catheters was 75,660 (210 catheter-years). Thirty-five catheter-related complications were identified, occurring in 26 patients. Complications included thrombosis (n = 14), infections (n = 9), mechanical problems (n = 6), pneumothorax (n = 3), superior vena cava syndrome/stenosis (n = 2), and air embolism (n = 1), for an overall complication rate of 0. 463/1,000 catheter-days. We conclude that indwelling catheters are relatively safe in patients with CF. Good infection control policies appear to prevent most infectious complications. The most common complication is that of thrombosis, which may be recurrent in some patients. Consideration should be given to prophylactic warfarin therapy despite the potential risk of significant hemoptysis in this patient population.
    Subject(s): Abridged Index Medicus ; Adolescent ; Adult ; Biological and medical sciences ; Catheters, Indwelling - adverse effects ; Child ; Cystic Fibrosis - therapy ; Equipment Failure ; Female ; Gastroenterology. Liver. Pancreas. Abdomen ; Humans ; Infection - etiology ; Liver. Biliary tract. Portal circulation. Exocrine pancreas ; Male ; Medical sciences ; Middle Aged ; Other diseases. Semiology ; Retrospective Studies ; Risk Factors ; Thrombosis - etiology
    ISSN: 0012-3692
    E-ISSN: 1931-3543
    Source: Academic Search Ultimate
    URL: http://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=845224$$DView record in Pascal Francis
    URL: https://www.ncbi.nlm.nih.gov/pubmed/11115445$$D View this record in MEDLINE/PubMed
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  • 10
    Article
    Article
    Malignant Nerve-Sheath Tumor with Divergent and Glandular Differentiation in a Dog: A Case Report
    2002
    ISSN: 0300-9858 
    Details
    Language: English
    In: Veterinary pathology, 2002-05, Vol.39 (3), p.406-410
    Description: Malignant nerve-sheath tumor with divergent differentiation including epithelial components was diagnosed in an 8-year-old Labrador retriever. The myelographic, morphologic, and immunohistochemical findings confirmed the diagnosis. The tumor was located in the peripheral nerve roots at the first and second lumbar vertebrae. The dog survived for 161 days after resection of the tumor.
    Subject(s): Animals ; Ataxia - pathology ; Ataxia - surgery ; Ataxia - veterinary ; Cell Differentiation - physiology ; Dog Diseases - pathology ; Dog Diseases - surgery ; Dogs ; Fatal Outcome ; Female ; Immunohistochemistry - veterinary ; Nerve Sheath Neoplasms - pathology ; Nerve Sheath Neoplasms - surgery ; Nerve Sheath Neoplasms - veterinary ; Phosphopyruvate Hydratase - metabolism ; S100 Proteins - metabolism ; Vimentin - metabolism
    ISSN: 0300-9858
    E-ISSN: 1544-2217
    DOI: 10.1354/vp.39-3-406
    Source: Alma/SFX Local Collection
    URL: https://www.ncbi.nlm.nih.gov/pubmed/12014509$$D View this record in MEDLINE/PubMed
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