Maniscalco Valerio, Niccolai Rachele, Marrani Edoardo, Maccora Ilaria, Bertini Federico, Pagnini Ilaria, Simonini Gabriele, Lasagni Donatella, Trapani Sandra, Mastrolia Maria Vincenza
Department of Health Sciences, University of Florence, 50134 Firenze, Italy.
Rheumatology Unit, ERN-ReCONNET Center, Meyer Children Hospital IRCCS, 50139 Firenze, Italy.
Children (Basel). 2023 Mar 25;10(4):618. doi: 10.3390/children10040618.
Multisystem Inflammatory Syndrome in Children (MIS-C) is a systemic hyperinflammatory disorder that is associated with a hypercoagulable state and a higher risk of thrombotic events (TEs). We report the case of a 9-year-old MIS-C patient with a severe course who developed a massive pulmonary embolism that was successfully treated with heparin. A literature review of previous TEs in MIS-C patients was conducted (60 MIS-C cases from 37 studies). At least one risk factor for thrombosis was observed in 91.7% of patients. The most frequently observed risk factors were pediatric intensive care unit hospitalization (61.7%), central venous catheter (36.7%), age >12 years (36.7%), left ventricular ejection fraction <35% (28.3%), D-dimer >5 times the upper limit of normal values (71.9%), mechanical ventilation (23.3%), obesity (23.3%), and extracorporeal membrane oxygenation (15%). TEs may concurrently affect multiple vessels, including both arterial and venous. Arterial thrombosis was more frequent, mainly affecting the cerebral and pulmonary vascular systems. Despite antithrombotic prophylaxis, 40% of MIS-C patients developed TEs. Over one-third of patients presented persistent focal neurological signs, and ten patients died, half of whom died because of TEs. TEs are severe and life-threatening complications of MIS-C. In case with thrombosis risk factors, appropriate thromboprophylaxis should be promptly administered. Despite proper prophylactic therapy, TEs may occur, leading in some cases to permanent disability or death.
儿童多系统炎症综合征(MIS-C)是一种全身性高炎症性疾病,与高凝状态和血栓形成事件(TEs)风险增加相关。我们报告了一例9岁的MIS-C重症患者,该患者发生了大面积肺栓塞,经肝素治疗成功。我们对之前MIS-C患者发生的TEs进行了文献综述(来自37项研究的60例MIS-C病例)。91.7%的患者观察到至少一种血栓形成危险因素。最常观察到的危险因素是入住儿科重症监护病房(61.7%)、中心静脉导管(36.7%)、年龄>12岁(36.7%)、左心室射血分数<35%(28.3%)、D-二聚体>正常上限5倍(71.9%)、机械通气(23.3%)、肥胖(23.3%)和体外膜肺氧合(15%)。TEs可能同时影响多个血管,包括动脉和静脉。动脉血栓形成更常见,主要影响脑和肺血管系统。尽管进行了抗血栓预防,仍有40%的MIS-C患者发生了TEs。超过三分之一的患者出现持续性局灶性神经体征,10例患者死亡,其中一半死于TEs。TEs是MIS-C严重且危及生命的并发症。对于有血栓形成危险因素的病例,应及时给予适当的血栓预防措施。尽管进行了适当的预防性治疗,仍可能发生TEs,在某些情况下会导致永久性残疾或死亡。