Avrusin Ilia S, Bregel Liudmila V, Efremova Olesya S, Kostik Mikhail M
Hospital Pediatrics, Saint Petersburg State Pediatric Medical University, Saint Petersburg 194100, Russia.
Department of Pediatrics, Irkutsk State Medical Academy of Postgraduate Education, A Branch of the Russian Medical Academy of Continuous Professional Education, Irkutsk 664049, Russia.
Biomedicines. 2024 Dec 17;12(12):2868. doi: 10.3390/biomedicines12122868.
Macrophage activation syndrome (MAS) can be regarded as a key factor determining the severity of multisystem inflammatory syndrome associated with COVID-19 in children (MIS-C), and often requires treatment in the intensive care unit (ICU) to avoid life-threatening complications. No reputable specific criteria for the diagnosis of MAS in MIS-C patients have yet been identified, and criteria currently used for the diagnosis of hemophagocytic syndromes, such as HLH-2004, MAS-2005, and MAS-2016, are not sufficient for MAS in MIS-C. Our goal in this study was to work out the criteria for the early diagnosis of MAS in MIS-C. One hundred and sixty-six (166) patients with MIS-C were assessed retrospectively. The two most experienced experts independently identified patients with MAS. The patients were divided into three cohorts: MAS ( = 19), without MAS ( = 78), and probable MAS ( = 67). The latter included patients diagnosed with MAS by only one expert, and it was excluded from the analysis. The age of patients with MAS was much higher, and they more frequently had edematous syndrome, hypotension and/or shock, splenomegaly, and CNS involvement. In their blood tests, thrombocytopenia, hypoalbuminemia, and hypertriglyceridemia occurred more often. The level of biomarkers of inflammation, such as ferritin, CRP, troponin, AST, and ALT, was also higher in this group. Increased fibrinogen and D-dimer were also found, demonstrating hypercoagulation in the MAS-MIS-C group. We chose 21 continuous and categorical variables with statistical significance, out of which 2-ferritin > 469 μg/L or platelets < 114 × 10/L-allowed us to discriminate MAS patients. Ferritin > 469 μg/L or platelets < 114 × 10/L can be regarded as key signs to differentiate MAS in MIS-C patients with a sensitivity of 100% and specificity of 94.9%, and they can be used along with other diagnostic methods.
巨噬细胞活化综合征（MAS）可被视为决定儿童新冠病毒相关多系统炎症综合征（MIS-C）严重程度的关键因素，且常需在重症监护病房（ICU）接受治疗以避免危及生命的并发症。目前尚未确定用于诊断MIS-C患者中MAS的可靠特异性标准，而目前用于诊断噬血细胞综合征的标准，如HLH-2004、MAS-2005和MAS-2016，并不适用于MIS-C中的MAS。本研究的目的是制定MIS-C中MAS的早期诊断标准。对166例MIS-C患者进行了回顾性评估。两位经验最丰富的专家独立确定患有MAS的患者。这些患者被分为三组：MAS组（n = 19）、无MAS组（n = 78）和可能MAS组（n = 67）。后者包括仅由一位专家诊断为MAS的患者，该组被排除在分析之外。MAS患者的年龄要高得多，且他们更常出现水肿综合征、低血压和/或休克、脾肿大以及中枢神经系统受累。在他们的血液检查中，血小板减少、低白蛋白血症和高甘油三酯血症更为常见。该组炎症生物标志物的水平，如铁蛋白、C反应蛋白、肌钙蛋白、谷草转氨酶和谷丙转氨酶也更高。还发现纤维蛋白原和D-二聚体增加，表明MAS-MIS-C组存在高凝状态。我们选择了21个具有统计学意义的连续和分类变量，其中铁蛋白>469μg/L或血小板<114×10⁹/L使我们能够区分MAS患者。铁蛋白>469μg/L或血小板<114×10⁹/L可被视为区分MIS-C患者中MAS的关键指标，其敏感性为100%，特异性为94.9%，可与其他诊断方法一起使用。
