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  1. Article ; Online: Neutrophil respiratory burst activity is not exaggerated in cystic fibrosis.

    Kelk, Dean / Logan, Jayden / Andersen, Isabella / Gutierrez Cardenas, Diana / Bell, Scott C / Wainwright, Claire E / Sly, Peter D / Fantino, Emmanuelle

    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

    2022  Volume 21, Issue 4, Page(s) 707–712

    Abstract: Background: Exaggerated neutrophil-dominated inflammation underlies progressive cystic fibrosis (CF) lung disease. Older studies reported a defective respiratory burst in CF, but more recent studies suggest neutrophil function is normal.: Methods: We ...

    Abstract Background: Exaggerated neutrophil-dominated inflammation underlies progressive cystic fibrosis (CF) lung disease. Older studies reported a defective respiratory burst in CF, but more recent studies suggest neutrophil function is normal.
    Methods: We measured the amount and rate of reactive oxygen species (ROS) during PMA-stimulated respiratory burst activity in children [70 CF, 13 disease controls, 19 health controls] and adults [31 CF, 14 health controls] in neutrophils harvested from peripheral blood. Blood was collected from participants with CF when clinically stable (60 children, 9 adults) and on hospital admission (38 children, 24 adults) and discharge (18 children, 21 adults) for acute pulmonary exacerbations.
    Results: When clinically stable, children with CF had lower ROS production [median 318,633, 25% 136,810 - 75% 569,523 RLU] than disease controls [median 599,459, 25% 425,566 - 75% 730,527 RLU] and healthy controls [median 534,073, 25% 334,057 - 75% 738,593 RLU] (p = 0.008). The rate of ROS production was also lower (p = 0.029). In neither children nor adults with CF did ROS production increase on hospital admission for acute pulmonary exacerbation, nor fall prior to discharge. There were no associations between ROS production and high-sensitivity C-reactive protein (indicating systemic inflammation) in either children or adults with CF.
    Conclusions: Our data do not support a role for exaggerated respiratory burst activity contributing to the exaggerated neutrophil-dominated inflammation seen with CF lung disease.
    MeSH term(s) Adult ; Child ; Cystic Fibrosis ; Humans ; Inflammation/metabolism ; Neutrophils/metabolism ; Reactive Oxygen Species/metabolism ; Respiratory Burst
    Chemical Substances Reactive Oxygen Species
    Language English
    Publishing date 2022-01-03
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2084724-5
    ISSN 1873-5010 ; 1569-1993
    ISSN (online) 1873-5010
    ISSN 1569-1993
    DOI 10.1016/j.jcf.2021.12.015
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Factors in childhood associated with lung function decline to adolescence in cystic fibrosis.

    Begum, Nelufa / Byrnes, Catherine A / Cheney, Joyce / Cooper, Peter J / Fantino, Emmanuelle / Gailer, Nicholas / Grimwood, Keith / GutierrezCardenas, Diana / Massie, John / Robertson, Colin F / Sly, Peter D / Tiddens, Harm Awm / Wainwright, Claire E / Ware, Robert S

    Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

    2022  Volume 21, Issue 6, Page(s) 977–983

    Abstract: Background: Despite improvements in general health and life expectancy in people with cystic fibrosis (CF), lung function decline continues unabated during adolescence and early adult life.: Methods: We examined factors present at age 5-years that ... ...

    Abstract Background: Despite improvements in general health and life expectancy in people with cystic fibrosis (CF), lung function decline continues unabated during adolescence and early adult life.
    Methods: We examined factors present at age 5-years that predicted lung function decline from childhood to adolescence in a longitudinal study of Australasian children with CF followed from 1999 to 2017.
    Results: Lung function trajectories were calculated for 119 children with CF from childhood (median 5.0 [25%-75%=5.0-5.1]) years) to early adolescence (median 12.5 [25%-75%=11.4-13.8] years). Lung function fell progressively, with mean (standard deviation) annual change -0.105 (0.049) for forced vital capacity (FVC) Z-score (p<0.001), -0.135 (0.048) for forced expiratory volume in 1-second (FEV
    Conclusions: Action in the first 5-years of life to prevent and/or treat respiratory exacerbations and counteract neutrophilic inflammation in the lower airways may reduce lung function decline in children with CF, and these should be targets of future research.
    MeSH term(s) Child ; Adult ; Adolescent ; Humans ; Child, Preschool ; Cystic Fibrosis/complications ; Longitudinal Studies ; Lung ; Vital Capacity ; Forced Expiratory Volume ; Spirometry
    Language English
    Publishing date 2022-03-24
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 2084724-5
    ISSN 1873-5010 ; 1569-1993
    ISSN (online) 1873-5010
    ISSN 1569-1993
    DOI 10.1016/j.jcf.2022.03.008
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Personalized Transcriptomics Reveals Heterogeneous Immunophenotypes in Children with Viral Bronchiolitis.

    Jones, Anya C / Anderson, Denise / Galbraith, Sally / Fantino, Emmanuelle / Gutierrez Cardenas, Diana / Read, James F / Serralha, Michael / Holt, Barbara J / Strickland, Deborah H / Sly, Peter D / Bosco, Anthony / Holt, Patrick G

    American journal of respiratory and critical care medicine

    2018  Volume 199, Issue 12, Page(s) 1537–1549

    Abstract: Rationale: ...

    Abstract Rationale:
    MeSH term(s) Bronchiolitis, Viral/genetics ; Bronchiolitis, Viral/immunology ; Child, Preschool ; Female ; Humans ; Immunity, Innate ; Infant ; Infant, Newborn ; Leukocytes, Mononuclear/immunology ; Male ; Nasal Mucosa/immunology ; Phenotype ; Sequence Analysis, RNA ; Transcriptome
    Language English
    Publishing date 2018-12-17
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1180953-x
    ISSN 1535-4970 ; 0003-0805 ; 1073-449X
    ISSN (online) 1535-4970
    ISSN 0003-0805 ; 1073-449X
    DOI 10.1164/rccm.201804-0715OC
    Database MEDical Literature Analysis and Retrieval System OnLINE

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