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生物制剂在儿童 SARS-CoV-2 相关多系统炎症综合征治疗中的作用。

Role of Biological Agents in the Treatment of SARS-CoV-2-Associated Multisystem Inflammatory Syndrome in Children.

机构信息

From the Department of Pediatric Rheumatology, Ankara Children's Hospital, University of Health Sciences.

Pediatric Intensive Care Unit, Ankara Children's Hospital, Ankara Yildirim Beyazit University.

出版信息

J Clin Rheumatol. 2022 Mar 1;28(2):e381-e387. doi: 10.1097/RHU.0000000000001734.

Abstract

OBJECTIVES

The aims of this study were to evaluate the role of biological agents in the treatment of severe multisystem inflammatory syndrome in children (MIS-C) and to assess the current application, outcomes, and adverse effects in patients who are followed up in a pediatric intensive care unit (PICU).

PATIENTS AND METHODS

This observational, descriptive, medical records review study was performed on patients with MIS-C admitted to the PICU between September 1 and November 1, 2020. Through medical records review, we confirmed that patients were positive for current or recent SARS-CoV-2 infection or for COVID-19 exposure history within the 4 weeks before the onset of symptoms.

RESULTS

A total of 33 patients with severe MIS-C were included (21 male) with a median age of 9 years. The most common signs and symptoms during disease course were fever (100%) and abdominal pain (75.5%). Clinical features of 63.6% patients were consistent with Kawasaki disease/Kawasaki disease shock syndrome, and 36.4% were consistent with secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Myocardial dysfunction and/or coronary artery abnormalities were detected in 18 patients during the PICU stay. Intravenous immunoglobulin and corticosteroids were given to 33 patients. Anakinra was administered to 23 patients (69.6%). There was a significant increase in lymphocyte and platelet counts and a significant decrease in ferritin, B-type natriuretic peptide, and troponin levels at the end of the first week of treatment in patients who were given biological therapy. Two patients were switched to tocilizumab because of an insufficient response to anakinra. The mortality rate of MIS-C patients admitted in PICU was 6.0%.

CONCLUSIONS

Management of systemic inflammation and shock is important to decrease mortality and the development of persistent cardiac dysfunction in MIS-C. The aggressive treatment approach, including biological agents, may be required in patients with severe symptoms and cardiac dysfunction.

摘要

目的

本研究旨在评估生物制剂在儿童严重多系统炎症综合征(MIS-C)治疗中的作用,并评估在儿科重症监护病房(PICU)接受随访的患者中的当前应用、结局和不良反应。

患者和方法

本观察性、描述性病历回顾研究纳入了 2020 年 9 月 1 日至 11 月 1 日期间入住 PICU 的 MIS-C 患者。通过病历回顾,我们确认患者在症状出现前 4 周内当前或近期 SARS-CoV-2 感染或 COVID-19 暴露史呈阳性。

结果

共纳入 33 例严重 MIS-C 患者(21 例男性),中位年龄为 9 岁。疾病过程中最常见的体征和症状是发热(100%)和腹痛(75.5%)。63.6%的患者临床表现符合川崎病/川崎病休克综合征,36.4%符合继发性噬血细胞性淋巴组织细胞增生症/巨噬细胞活化综合征。18 例患者在 PICU 期间发现心肌功能障碍和/或冠状动脉异常。33 例患者给予静脉注射免疫球蛋白和皮质类固醇。23 例(69.6%)患者给予阿那白滞素。接受生物治疗的患者在治疗第 1 周末淋巴细胞和血小板计数显著增加,铁蛋白、B 型利钠肽和肌钙蛋白水平显著降低。2 例患者因阿那白滞素反应不足而换用托珠单抗。入住 PICU 的 MIS-C 患者死亡率为 6.0%。

结论

管理全身炎症和休克对于降低 MIS-C 死亡率和持续性心肌功能障碍的发展至关重要。对于有严重症状和心肌功能障碍的患者,需要积极的治疗方法,包括生物制剂。

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