MIS-C 和川崎病:不同的疾病还是同一病谱的一部分?

Angelo Ravelli
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摘要

在 COVID-19 大流行期间,儿童多系统炎症综合征(MIS-C)的发生给儿科医生带来了挑战。这种疾病的某些特征与川崎病(KD)相似,但其临床症状和体征在川崎病中并不常见,如腹泻、腹痛、神经功能障碍和心脏受累,尤其是心肌炎。许多患 MIS-C 的儿童因出现多器官衰竭和循环休克(通常是心肌源性休克)而必须入住重症监护病房。治疗以静脉注射免疫球蛋白、糖皮质激素为主,病情最严重的患儿还需要使用阿那金拉。MIS-C 和 KD 在临床上的相似性引发了一场激烈的争论,即它们是临床特征重叠的不同疾病,还是属于同一疾病谱。基于 MIS-C 和 KD 在流行病学、临床和免疫学方面的差异,大多数权威人士倾向于认为这两种疾病是不同的。另一些学者则认为,这两种疾病可能是一个连续体,其表型和严重程度的某些差异与病毒载量或毒株、免疫反应的程度或动力学有关。本文旨在批判性地分析支持第二种假设的理由和证据。
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MIS-C and Kawasaki disease: Different illnesses or part of the same spectrum?

During COVID-19 pandemic pediatricians have been challenged by the occurrence of the multisystem inflammatory syndrome in children (MIS-C). This condition displays some features similar to those of Kawasaki disease (KD), but was is characterized by clinical signs and symptoms that are infrequent in this illness, such as diarrhea, abdominal pain, neurologic dysfunction and cardiac involvement, especially myocarditis. Many children with MIS-C necessitated ICU admission due to development of multiorgan failure and circulatory shock, usually of myocardial origin. Management is based on the administration of intravenous immunoglobulin, glucocorticoids and, in the most severe forms, anakinra. The clinical similarities between MIS-C and KD have raised an intense debate about whether they represent different illnesses with overlapping clinical features or are part of the same disease spectrum. Most authorities favor the assumption that MIS-C is distinct from KD, based on epidemiological, clinical and immunological differences between the two entities. Others argue that the two disorders may represent a continuum, with some differences in phenotype and severity being related to viral load or strain or magnitude or kinetics of immune response. The present article is aimed to analyze critically the rationale and evidence in favor of the second hypothesis.

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Global pediatrics
Global pediatrics Perinatology, Pediatrics and Child Health
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