第二版细胞因子风暴综合征

English | September 9, 2024 | ISBN-10: 3031598148 | 644 pages| Epub PDF (True) | 61 MB

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风暴综合征，包括HLH和MAS，在许多情况下都是致命的病症，尤其是如果没有早期识别并治疗。目前正通过CD8细胞毒性T细胞和自然杀伤细胞使用的基于穿孔素介导的细胞凋亡途径中的突变来定义这些病症的风险基因。研究人员正在使用小鼠模型研究这些基因。一般人群中约有10%的人可能携带引发Cytokine风暴综合征风险基因，目前世界各地的儿科医院和成人医院越来越多地开始认识Cytokine风暴综合征。 感染性、风湿性和肿瘤性触发因素通常与Cytokine风暴综合征有关联，但深入了解这种疾病对所有研究人员和医生来说至关重要，以确保及时并恰当的治疗。第二版涵盖了这一疾病的各个方面，从遗传学、病理生理学以及正在进行的研究，到临床表现、风险因素及治疗方法。本版新增内容包括专门章节探讨重症COVID-19引起的Cytokine风暴综合征，以及儿童多系统炎症综合征（MIS-C）后的Cytokine风暴综合征。此外，还涵盖了与Castleman病、妊娠、治疗、移植和心脏旁路手术相关的CSS，以及JAK抑制剂的使用等新颖话题。

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