Orr William B, Elward Alexis M, Lin John C, Reich Patrick J, Scheel Janet N, Hayes Ericka V, Remy Kenneth E
Division of Cardiology, Department of Pediatrics, Washington University in St. Louis, St. Louis, MO.
Division of Infectious Diseases, Department of Pediatrics, Washington University in St. Louis, St. Louis, MO.
Crit Care Explor. 2020 Oct 1;2(10):e0236. doi: 10.1097/CCE.0000000000000236. eCollection 2020 Oct.
Although significant disease burden in the severe acute respiratory syndrome coronavirus 2 pandemic has been relatively uncommon in children, worldwide cases of a postinfectious multisystem inflammatory syndrome in children and possible atypical Kawasaki-like disease attributing to severe acute respiratory syndrome coronavirus 2 infection have arisen. Original thinking for coronavirus disease-19 disease was that an overwhelming proinflammatory response drove disease pathogenesis. Emerging reports suggest that a robust immune suppression may be more relevant and predominant. Recently reported data on children with multisystem inflammatory syndrome in children have demonstrated a heterogeneity of immune phenotypes among these patients, with concern for a strong initial proinflammatory state; however, data are lacking to support this. Likewise, understanding development of certain clinical findings to changes in the immune system is lacking.
We report a 12-year-old multiracial male with negative coronavirus disease-19 nasopharyngeal RNA polymerase chain reaction testing but positive severe acute respiratory syndrome coronavirus 2 serology, subsequent development of vasodilatory shock with myocardial depression, and subsequent delayed development of coronary artery dilatation after resolution of myocardial depression. Unlike previous reported cases of multisystem inflammatory syndrome in children, he exhibited profound lymphopenia without specific inflammatory cytokines elevations, whereas nonspecific markers (ferritin and C-reactive protein) were increased. He subsequently was discharged on day 12 of hospitalization with complete recovery.
Our representative case of a patient with coronavirus disease-19-associated multisystem inflammatory syndrome in children without robust hyperinflammation and a delayed finding of coronary artery dilatation compared with reported case series highlights the need for further mechanistic understanding of coronavirus disease-19 disease and subsequent multisystem inflammatory syndrome in children or Kawasaki disease development. This report offers a number of disease mechanisms and clinical evolution considerations for further elucidation to guide development of potential therapies.
尽管在严重急性呼吸综合征冠状病毒2大流行中，儿童的重大疾病负担相对不常见，但全球已出现儿童感染后多系统炎症综合征病例以及可能归因于严重急性呼吸综合征冠状病毒2感染的非典型川崎样疾病。最初对冠状病毒病19的认识是，压倒性的促炎反应驱动疾病发病机制。新出现的报告表明，强大的免疫抑制可能更相关且占主导地位。最近报告的关于儿童多系统炎症综合征患儿的数据显示，这些患者的免疫表型存在异质性，人们担心最初存在强烈的促炎状态；然而，缺乏支持这一点的数据。同样，对于某些临床发现与免疫系统变化之间的关系也缺乏了解。
我们报告一名12岁的多族裔男性，其冠状病毒病19鼻咽RNA聚合酶链反应检测呈阴性，但严重急性呼吸综合征冠状病毒2血清学检测呈阳性，随后出现血管舒张性休克伴心肌抑制，心肌抑制缓解后出现冠状动脉扩张延迟。与先前报道的儿童多系统炎症综合征病例不同，他表现出严重淋巴细胞减少，而特定炎症细胞因子无升高，而非特异性标志物（铁蛋白和C反应蛋白）升高。他随后在住院第12天出院，完全康复。
我们报告的这例冠状病毒病19相关儿童多系统炎症综合征患者，与报道的病例系列相比，没有强烈的炎症反应且冠状动脉扩张发现延迟，这凸显了需要进一步从机制上了解冠状病毒病19以及随后儿童多系统炎症综合征或川崎病的发展。本报告提供了一些疾病机制和临床演变方面的考虑因素，以供进一步阐明，以指导潜在治疗方法的开发。
