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Clinical Features of Cytokine Storm Syndrome
2019Cytokine storm syndrome (CSS) is a severe life-threatening condition characterized by a clinical phenotype of overwhelming systemic inflammation, hyperferritinemia, hemodynamic instability, and multiple organ failure (MOF), and, if untreated, it can potentially lead to death.
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JAK Inhibitors in Cytokine Storm Syndromes
Cytokine storm syndromes (CSSs) comprise a group of severe and often fatal hyperinflammatory conditions driven by the overproduction of pro-inflammatory cytokines by activated cells of the immune system. Many of the CSS-associated cytokines mediate their downstream effects by signaling through the Janus kinases (JAKs) and signal transducers and ...Camille, Keenan +3 more
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Cytokine Storm Syndrome as a Manifestation of Primary HIV Infection
2019HIV infection triggers an inflammatory response that manifests as acute retroviral syndrome (ARS) in most individuals infected by HIV. While this syndrome is usually self-limited, primary HIV infection sometimes triggers a fulminant inflammatory process consistent with cytokine storm syndrome (CSS).
Nathaniel, Erdmann, Sonya L, Heath
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Kawasaki Disease-Associated Cytokine Storm Syndrome
Kawasaki disease (KD) is a hyperinflammatory syndrome manifesting as an acute systemic vasculitis characterized by fever, nonsuppurative conjunctival injection, rash, oral mucositis, extremity changes, and cervical lymphadenopathy. KD predominantly affects young children and shares clinical features and immunobiology with other hyperinflammation ...Paul, Tsoukas, Rae S M, Yeung
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Murine Models of Secondary Cytokine Storm Syndromes
Hemophagocytic lymphohistiocytosis (HLH) comprises a broad spectrum of life-threatening cytokine storm syndromes, classified into primary (genetic) or secondary (acquired) HLH. The latter occurs in a variety of medical conditions, including infections, malignancies, autoimmune and autoinflammatory diseases, acquired immunodeficiency, and metabolic ...Ellen, Brisse +5 more
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Primary Immunodeficiencies and Cytokine Storm Syndromes
2019Primary immunodeficiency diseases (PIDs) are a diverse and growing category of more than 300 chronic disorders that share a susceptibility to infections. Whether the result of a genetic lesion that causes defective granule-dependent cytotoxicity, excessive lymphoproliferation, or an overwhelming infection representing a unique antigenic challenge, PIDs
David A. Hill, Neil Romberg
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Cytokines and metabolic syndrome: The perfect storm for arterial aging
Atherosclerosis, 2011N ...
RIZZA, STEFANO, FEDERICI, MASSIMO
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Inborn Errors of Immunity and Cytokine Storm Syndromes
Inborn errors of immunity (IEI) are a diverse and growing category of more than 430 chronic disorders that share susceptibilities to infections. Whether the result of a genetic lesion that causes defective granule-dependent cytotoxicity, excessive lymphoproliferation, or an overwhelming infection represents a unique antigenic challenge, IEIs can ...Whitney, Reid, Neil, Romberg
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Genetics of Acquired Cytokine Storm Syndromes
2019Secondary hemophagocytic lymphohistiocytosis (sHLH) has historically been defined as a cytokine storm syndrome (CSS) occurring in the setting of triggers leading to strong immunological activation, without any known genetic predilection. However, recent studies have suggested that existing underlying genetic factors may synergize with particular ...
Grant S. Schulert, Kejian Zhang
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Murine Models of Familial Cytokine Storm Syndromes
Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory disease caused by mutations in effectors and regulators of cytotoxicity in cytotoxic T cells (CTL) and natural killer (NK) cells. The complexity of the immune system means that in vivo models are needed to efficiently study diseases like HLH.Volkmer, Benjamin +3 more
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