Division of Oncology, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa; Immune Dysregulation Frontier Program, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa.
Division of Oncology, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa; Immune Dysregulation Frontier Program, Department of Pediatrics, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pa.
J Allergy Clin Immunol Pract. 2023 Jun;11(6):1636-1644. doi: 10.1016/j.jaip.2023.03.033. Epub 2023 Mar 27.
Cytokine storm syndromes (CSS) represent a diverse group of disorders characterized by severe overactivation of the immune system. In the majority of patients, CSS arise from a combination of host factors, including genetic risk and predisposing conditions, and acute triggers such as infections. CSS present differently in adults than in children, who are more likely to present with monogenic forms of these disorders. Individual CSS are rare, but in aggregate represent an important cause of severe illness in both children and adults. We present 3 rare, illustrative cases of CSS in pediatric patients that describe the spectrum of CSS.
细胞因子风暴综合征(CSS)代表了一组不同的疾病,其特征是免疫系统的严重过度激活。在大多数患者中,CSS 是由宿主因素(包括遗传风险和易患疾病)和急性触发因素(如感染)共同作用引起的。CSS 在成人和儿童中的表现不同,儿童更有可能表现出这些疾病的单基因形式。单个 CSS 较为罕见,但总体而言,它们是儿童和成人严重疾病的重要原因。我们提出了 3 例儿科 CSS 罕见病例,以描述 CSS 的范围。