Division of Hematology, Cancer and Blood Diseases Institute Cincinnati Children's Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Curr Opin Hematol. 2019 Sep;26(5):366-371. doi: 10.1097/MOH.0000000000000523.
The purpose of this review is to summarize the diagnosis and management of the antiphospholipid syndrome (APS) in children.
APS is a rare, acquired autoimmune systemic disease that can result in significant morbidity in children related to vascular thrombosis. The diagnosis and management of APS in children can be challenging due to a lack of validated diagnostic criteria and the rarity of the disease. In addition, many healthy children have transient circulating antiphospholipid antibodies without thrombotic complications. Nevertheless, epidemiological studies suggest that APS represents a greater relative proportion of thrombotic disease in children than it does in adults. Management of pediatric APS is largely inferred from adult data despite unique characteristics of pediatric APS. The current recommendations include long-term anticoagulation, which can be problematic in young, active individuals. There is little data on potential benefits of nonantithrombotic therapy in the management of pediatric APS.
Data on pediatric APS are limited, but evidence suggests that using current available diagnostic testing is valuable and, until further evidence is available, treating thrombotic complications with heparins or warfarin should be standard of care.
本文旨在总结儿童抗磷脂综合征（APS）的诊断和治疗。
APS 是一种罕见的、获得性自身免疫性系统性疾病，可导致儿童发生与血管血栓形成相关的严重疾病。由于缺乏经过验证的诊断标准和疾病的罕见性，儿童 APS 的诊断和管理具有一定挑战性。此外，许多健康儿童的循环抗磷脂抗体是短暂的，没有发生血栓并发症。然而，流行病学研究表明，APS 在儿童中的血栓性疾病比例相对高于成人。尽管儿童 APS 具有独特的特征，但儿童 APS 的管理主要是从成人数据中推断出来的。目前的建议包括长期抗凝治疗，但对于年轻、活跃的个体来说，这可能存在问题。在儿童 APS 的管理中，非抗血栓治疗的潜在益处的数据很少。
儿童 APS 的数据有限，但有证据表明，使用当前可用的诊断检测方法是有价值的，并且在进一步的证据出现之前，用肝素或华法林治疗血栓性并发症应该是标准的治疗方法。
