Senken Brooke, Whitehead Anne
Riley Hospital for Children, Indianapolis, IN, USA.
Department of Emergency Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
Case Rep Pediatr. 2022 May 13;2022:7890566. doi: 10.1155/2022/7890566. eCollection 2022.
Catastrophic antiphospholipid syndrome (CAPS) is an infrequent but feared life-threatening complication of antiphospholipid syndrome (APS). CAPS is characterized by the rapid development of numerous thromboses across multiple organs resulting in multiorgan failure. It is rare but well-documented in the adult population. In contrast, it is exceedingly uncommon in pediatric patients and therefore not yet well described in the pediatric literature. Early recognition of APS is of the utmost importance to provide timely and effective management for a positive outcome. We present the case of an 11-year-old girl with history of systemic lupus erythematosus (SLE) and hypertension (HTN) who presented with acute onset altered mental status, found to have a large ischemic middle cerebral artery (MCA) and anterior cerebral artery (ACA) stroke as well as multiple, diffuse, and smaller ischemic lesions in the frontal lobe and cerebellum. Her presentation was further complicated by thrombocytopenia and renal and splenic infarction, as well as thrombosis of the right brachial vein consistent with a diagnosis of CAPS.
灾难性抗磷脂综合征(CAPS)是抗磷脂综合征(APS)一种罕见但可怕的危及生命的并发症。CAPS的特征是多个器官迅速出现大量血栓形成,导致多器官功能衰竭。它在成人中虽罕见但有充分记录。相比之下,在儿科患者中极为罕见,因此儿科文献中对其描述尚不充分。早期识别APS对于提供及时有效的治疗以获得良好结局至关重要。我们报告一例11岁女孩,有系统性红斑狼疮(SLE)和高血压(HTN)病史,出现急性起病的精神状态改变,发现有大脑中动脉(MCA)和大脑前动脉(ACA)大面积缺血性卒中以及额叶和小脑多发、弥漫性较小缺血性病变。她的病情因血小板减少、肾和脾梗死以及右肱静脉血栓形成而进一步复杂化,符合CAPS的诊断。