背景：非标准抗磷脂抗体（NC-aPL）是抗磷脂抗体（aPL）的相对未定义的亚组。成人关于NC-aPL的知识是有限的，在儿科患者中甚至更少。抗磷脂综合征（APS）的常规测试-一种以aPL与血管血栓形成相关的临床状态-通常包括狼疮抗凝药（LAC），抗心磷脂（aCL）和-β-2糖蛋白I（aβ2GPI）。LAC是血栓前aPL的功能筛选，而后者检测可识别特异性自身抗体。NC-aPL的具体目标包括，但不限于,磷脂酰乙醇胺，磷脂酰丝氨酸,和凝血酶原。
方法：我们提供了在3年期间确定的8例儿童NC-aPL患者的单中心数据。所有患者均具有引起APS怀疑的特征。大多数患者是患有原发性风湿病的女性。一名患者中风。另一名患者患有肺泡出血和肺动脉高压。雷诺现象,累及四肢远端的皮疹,头痛很常见。大多数患者LAC阳性，然而他们的常规aPL测试是阴性的，提示NC-aPL测试。
结论：我们的研究结果表明，在儿科患者中，NC-aPL与APS的典型体征和症状相关。当临床怀疑较高且常规aPL检测阴性时，儿科医生和儿科专科医师应考虑NC-aPL，特别是当LAC为正值时。虽然儿童或成人的NC-aPL指南尚不存在，这些自身抗体具有致病潜力。可行的项目可以包括评估其他（原发性）风湿性疾病的存在，并就抗凝/血小板抑制和血栓形成教育咨询血液学家和/或产科医生。关于NC-aPL的未来指南将仅通过收集更多数据来生成，理想的前瞻性。
BACKGROUND: Non-criteria antiphospholipid antibodies (NC-aPL) are a relatively undefined subgroup of antiphospholipid antibodies (aPL). Knowledge about NC-aPL in adults is limited and even less is known in pediatric patients. Routine tests for antiphospholipid syndrome (APS)-a clinical state marked by the presence of aPL in association with vascular thrombosis-usually include lupus anticoagulant (LAC), anti-cardiolipin (aCL) and -beta-2 glycoprotein I (aβ2GPI). LAC is a functional screen for prothrombotic aPL, while the latter tests identify specific autoantibodies. Specific targets of NC-aPL include, but are not limited to, phosphatidylethanolamine, phosphatidylserine, and prothrombin.
METHODS: We present single-center data from eight pediatric patients with NC-aPL identified during a three-year period. All patients had presenting features raising suspicion for APS. Most patients were female with a primary rheumatic disease. One patient had a stroke. Another patient had alveolar hemorrhage and pulmonary hypertension. Raynaud\'s phenomenon, rashes involving distal extremities, and headaches were common. Most patients had a positive LAC, yet their routine aPL tests were negative, prompting testing for NC-aPL.
CONCLUSIONS: Our findings suggest NC-aPL are associated with typical signs and symptoms of APS in pediatric patients. Pediatricians and pediatric subspecialists should consider NC-aPL when clinical suspicion is high and routine aPL tests are negative, particularly when LAC is positive. While guidelines for NC-aPL do not yet exist for children or adults, these autoantibodies have pathogenic potential. Actionable items could include evaluation for the presence of other (primary) rheumatic diseases, and consultation with hematologists and/or obstetricians regarding anticoagulation/platelet inhibition and thrombosis education. Future guidelines regarding NC-aPL will only be generated by gathering more data, ideally prospectively.
