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57例儿童和青少年系统性红斑狼疮患者的抗磷脂抗体与抗磷脂综合征

Antiphospholipid antibodies and antiphospholipid syndrome in 57 children and adolescents with systemic lupus erythematosus.

作者信息

Campos L M A, Kiss M H, D'Amico E A, Silva C A Almeida

机构信息

Paediatric Rheumatology Unit, Children's Institute, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil.

出版信息

Lupus. 2003;12(11):820-6. doi: 10.1191/0961203303lu471oa.

Abstract

The presence of antiphospholipid (aPL) antibodies and antiphospholipid syndrome (APS) was researched in 57 children and adolescents with systemic lupus erythematosus (SLE). The frequency of aPL antibodies was 75.4% (anticardiolipin 70.2% and lupus anticoagulant 29.1%). The positivity for these antibodies fluctuated during the course of the disease. No association was found between aPL antibodies and clinical or laboratory manifestations or the autoantibodies studied, nor with the activity or gravity of the SLE. APS was diagnosed in 14% of the cases (eight patients), on average three years after the diagnosis of SLE. Four patients had arterial thrombosis (stroke, three; transient ischaemic attack, one; amaurosis fugax, two; renal, one), one presented with deep vein thrombosis (DVT) and three had involvement of small calibre vessels (osteonecrosis, two; transverse myelitis, one). Recurrences were observed in three of the eight cases (37.5%), with a mean interval of 13 months between the events. The presence of APS was associated with haemolytic anaemia, leukopenia, thrombocytopenia, coagulation abnormalities, ischaemic cerebrovascular accidents, amaurosis fugax, osteonecrosis and interstitial pneumonitis. A negative association was observed between APS and the presence of anti-Ro antibodies.

摘要

对57例患有系统性红斑狼疮(SLE)的儿童和青少年研究了抗磷脂(aPL)抗体和抗磷脂综合征(APS)的情况。aPL抗体的出现频率为75.4%(抗心磷脂抗体为70.2%,狼疮抗凝物为29.1%)。这些抗体的阳性率在疾病过程中波动。未发现aPL抗体与临床或实验室表现、所研究的自身抗体之间存在关联,也未发现与SLE的活动度或严重程度有关联。14%的病例(8例患者)被诊断为APS,平均在SLE诊断后三年。4例患者发生动脉血栓形成(中风3例;短暂性脑缺血发作1例;一过性黑矇2例;肾脏1例),1例出现深静脉血栓形成(DVT),3例有小口径血管受累(骨坏死2例;横贯性脊髓炎1例)。8例中有3例(37.5%)出现复发,事件之间的平均间隔为13个月。APS的存在与溶血性贫血、白细胞减少、血小板减少、凝血异常、缺血性脑血管意外、一过性黑矇、骨坏死和间质性肺炎有关。观察到APS与抗Ro抗体的存在呈负相关。

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