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Braedon McDonald
During critical illness, dramatic alterations in neutrophil biology are observed including abnormalities of granulopoeisis and lifespan, cell trafficking and antimicrobial effector functions. As a result, neutrophils transition from powerful antimicrobial protectors into dangerous mediators of tissue injury and organ dysfunction. In this article, the role of neutrophils in the pathogenesis of critical illness (sepsis, trauma, burns and others) will be explored, including pathological changes to neutrophil function during critical illness and the utility of monitoring aspects of the neutrophil phenotype as biomarkers for diagnosis and prognostication...
March 2018: Cell and Tissue Research
J S Hui-Yuen, A M Christiano, A Askanase
OBJECTIVES: Systemic lupus erythematosus (SLE) is a chronic disease occurring up to 15 times more frequently in females than males. This bias extends to possible differences in disease flares and response to therapy. This study was initiated to investigate the differences between girls and boys with childhood-onset SLE (cSLE) at the molecular level. METHOD: We analysed the Gene Expression Omnibus National Center for Biotechnology Information (GEO NCBI) microarray data available for 88 girls and 16 boys with treatment-naïve cSLE and compared the results to those from healthy controls...
October 2016: Scandinavian Journal of Rheumatology
Jean Donadieu, Blandine Beaupain, Christine Bellanné-Chantelot
Congenital neutropenia are extremely rare diseases, defined by a permanent or cyclic decrease of blood neutrophils. Molecular basis of several congenital neutropenia has been recently determined, involving gene coding for the neutrophil elastase gene (ELA2), GFI1, WAS protein and mitochondrial HAX1 protein. These mutations, dominant (ELA2, GFI1), X-linked (WAS) and autosomal recessive (HAX1), result in instability of the contents of the granules- particularly the neutrophil elastase- or in abnormalities of the cytoskeleton, and possibly, in an increased apoptosis...
March 2008: Médecine Sciences: M/S
Jay K Kolls, Anders Lindén
IL-17A was cloned more than 10 years ago and six IL-17 family members (IL-17A-F) have subsequently been described. IL-17A is largely produced by activated memory T lymphocytes but stimulates innate immunity and host defense. IL-17A and IL-17F both mobilize neutrophils partly through granulopoeisis and CXC chemokine induction, as well as increased survival locally. IL-17A and IL-17F production by T lymphocytes is regulated by IL-23 independent of T cell receptor activation. Increasing evidence shows that IL-17 family members play an active role in inflammatory diseases, autoimmune diseases, and cancer...
October 2004: Immunity
J Donadieu
Many circumstances lead to discover a neutropenia in paediatric practice. In most of the cases, it is an acquired, transient neutropenia, related to a viral or a bacterial infection, a malignant haemopathy, or an acquired auto-immune neutropenia, also called benign chronic neutropenia. Constitutional disorder with neutropenia is more exceptional. Many complex genetic diseases include a neutropenia, among which several immunologic disorders that could be easily diagnosed by immunological tests. Other complex genetic diseases include Shwachman-Diamond syndrome, associating an external pancreatic insufficiency with bone and skin abnormalities; Glycogen storage disease type Ib, with metabolic disorder...
September 2003: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
P Mohan, P Brocklehurst
BACKGROUND: Neonatal sepsis causes significant neonatal mortality and morbidity. Neonates, especially preterm infants, have an immaturity of granulopoeisis and have a limited capacity for progenitor cell proliferation. This results in the frequent occurrence of neutropaenia in septic neonates. Neutropaenic septic neonates have a higher mortality than neonates who are septic but not neutropaenic. Transfusion of granulocytes to septic neutropaenic neonates, therefore, may help reduce mortality and morbidity...
2003: Cochrane Database of Systematic Reviews
L Walker, A Carlson, H T Tan-Pertel, G Weinmaster, J Gasson
Members of the Notch family of transmembrane receptors are found on primitive hematopoietic precursors, and Notch ligand expression has been demonstrated on the surface of stromal cells, suggesting a role for Notch signaling in mammalian blood cell development. The current report examines the expression of Notch receptors and their ligands in murine hematopoietic tissues to determine: A) which blood cell lineages in the adult are influenced by Notch activity, and B) whether fetal hematopoiesis in the embryo involves the Notch pathway...
2001: Stem Cells
S Calderwood, L Kilpatrick, S D Douglas, M Freedman, K Smith-Whitley, M Rolland, J Kurtzberg
The purpose of this study was to evaluate the efficacy and toxicity of recombinant human granulocyte colony-stimulating factor (rhG-CSF) therapy in patients with neutropenia and/or neutrophil dysfunction secondary to glycogen storage disease (GSD) type 1b. Thirteen patients with neutropenia and/or neutrophil dysfunction secondary to GSD type 1b were treated with rhG-CSF. The effects of therapy on neutrophil numbers and in vitro neutrophil function and on bone marrow cellularity and morphology were studied. The clinical status of the patients and the occurrence of adverse events associated with rhG-CSF use were monitored...
January 15, 2001: Blood
J Donadieu
Kostmann Syndrome is defined as a chronic neutropenia, dating from early childhood, characterized typically by a granulopoeisis impairment at the promyelocyte stage. The origin is not yet understood. G-CSF receptor anomaly -the intra-cellular carboxy terminal region- was noted in a few patients (6 out of 54), initially in two patients who later developed secondary leukemia. More follow-up, with other patients, led us to consider the mutation of the G-CSF receptor sometimes as a transient event, not systematically resulting in malignancy...
April 1997: Hematology and Cell Therapy
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