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Abstract：
Our study aimed to evaluate the presence, clinical associations, and potential mechanistic roles of non-criteria antiphospholipid antibodies (aPL) and circulating calprotectin, a highly stable marker of neutrophil extracellular trap release (NETosis), in pediatric APS patients. We found that 79% of pediatric APS patients had at least one non-criteria aPL at moderate-to-high titer. Univariate logistic regression demonstrated that positive anti-beta-2 glycoprotein I domain 1 (anti-D1) IgG (p = 0.008), anti-phosphatidylserine/prothrombin (aPS/PT) IgG (p < 0.001), and aPS/PT IgM (p < 0.001) were significantly associated with venous thrombosis. Positive anti-D1 IgG (p < 0.001), aPS/PT IgG (p < 0.001), and aPS/PT IgM (p = 0.001) were also associated with non-thrombotic manifestations of APS, such as thrombocytopenia. Increased levels of calprotectin were detected in children with APS. Calprotectin correlated positively with absolute neutrophil count (r = 0.63, p = 0.008) and negatively with platelet count (r = -0.59, p = 0.015). Mechanistically, plasma from pediatric APS patients with high calprotectin levels impaired platelet viability in a dose-dependent manner.
摘要：
我们的研究旨在评估存在，临床关联，以及非标准抗磷脂抗体（aPL）和循环钙卫蛋白的潜在机制作用，中性粒细胞胞外诱捕网释放(NETosis)的高度稳定标志物，在儿科APS患者中。我们发现79%的儿科APS患者在中高滴度时至少有一种非标准aPL。单因素logistic回归表明抗β-2糖蛋白I结构域1（抗D1）IgG阳性（p=0.008），抗磷脂酰丝氨酸/凝血酶原（aPS/PT）IgG（p<0.001），aPS/PTIgM(p<0.001)与静脉血栓形成显著相关。抗D1IgG阳性（p<0.001），aPS/PTIgG（p<0.001），aPS/PTIgM（p=0.001）也与APS的非血栓性表现相关，如血小板减少症。在APS儿童中检测到钙卫蛋白水平升高。钙卫蛋白与中性粒细胞绝对计数呈正相关(r=0.63,p=0.008),与血小板计数呈负相关(r=-0.59,p=0.015)。机械上,高钙卫蛋白水平的儿科APS患者的血浆以剂量依赖性方式损害血小板活力。
