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Cytokine Storm Syndromes, including HLH and MAS, are frequently fatal disorders, particularly if not recognized early and treated during presentation. The genetics of Cytokine Storm Syndromes are being defined with many of the risk alleles giving rise to mutations in the perforin-mediated cytolytic pathway used by CD8 cytotoxic T cells and natural killer cells. These are being studied using murine models. Up to 10% of the general population may carry risk alleles for developing Cytokine Storm Syndromes, and Cytokine Storm Syndromes are being increasingly recognized around the world in pediatric and adult hospitals. A variety of infectious, rheumatic, and oncologic triggers are commonly associated with Cytokine Storm Syndromes, but understanding this disorder is critical for all researchers and physicians to ensure timely and appropriate therapy. This second edition addresses all aspects of the disorder, from genetics, pathophysiology, and ongoing research, to clinical presentations, risk factors and treatment. New-to-this-edition features include dedicated chapters on severe COVID-19 CSS, and post-COVID CSS of multi-system inflammatory syndrome in children (MIS-C). In addition, novel topics including CSS associated with Castleman disease, pregnancy, therapeutics, transplantation, and cardiac bypass, as well as treatment with JAK inhibitors are addressed.

Cytokine Storm Syndromes, including HLH and MAS, are frequently fatal disorders, particularly if not recognized early and treated during presentation. The genetics of Cytokine Storm Syndromes are being defined with many of the risk alleles giving rise to mutations in the perforin-mediated cytolytic pathway used by CD8 cytotoxic T cells and natural killer cells. These are being studied using murine models. Up to 10% of the general population may carry risk alleles for developing Cytokine Storm Syndromes, and Cytokine Storm Syndromes are being increasingly recognized around the world in pediatric and adult hospitals. A variety of infectious, rheumatic, and oncologic triggers are commonly associated with Cytokine Storm Syndromes, but understanding this disorder is critical for all researchers and physicians to ensure timely and appropriate therapy. This textbook, the first of its kind, addresses all aspects of the disorder – from genetics, pathophysiology, and ongoing research,to clinical presentations, risk factors, and treatment.

Neurobiology of Cytokines, Part A provides an overview of the effects of cytokines in the brain and in the endocrine system. The book discusses the general aspects of cytokines, including the endogenous agonists and antagonists, their receptors, their second messengers, and transport mechanisms for cytokines across the blood-brain barrier. The text also describes the anatomical localization of cytokines, cytokine receptors, and their respective mRNA in brain and in endocrine tissues. The methods for evaluating the in vivo as well as in vitro actions of cytokines on hormone secretion are also considered. The book further tackles the synthesis and release of cytokines and their central nervous system actions; and the methodology for studying the role of cytokines in human neuropathological conditions.