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[小儿抗磷脂综合征:一例报告]

[Antiphospholipid syndrome in Pediatrics: a case report].

作者信息

Micheletti Eugenia, Blanco Luciana, Cechinni Ana, Acerenza Marcelo, Canda Paula

机构信息

Sanatorio Sagrado Corazón, Ciudad Autónoma de Buenos Aires.

出版信息

Arch Argent Pediatr. 2017 Dec 1;115(6):e412-e415. doi: 10.5546/aap.2017.e412.

Abstract

The antiphospholipid syndrome is a multisystem autoimmune disease in which autoantibodies against a variety of phospholipids and phospholipid binding proteins are produced. It occurs in 1.8% of the population and only 2% of the cases are pediatric. The spectrum of clinical manifestations is wide from asymptomatic patients to a life-threatening disease like the catastrophic antiphospholipid syndrome. Any organ can be affected. The most frequent manifestations in pediatrics correspond to venous thrombosis in 60% of patients, arterial thrombosis in 32%, hematological disease in 38% (thrombocytopenia, leucopenia), skin alterations in 18% (livedo reticularis, Raynaud's phenomenon) and neurological signs in 16%. We describe the case of a previously healthy 14-year-old female patient diagnosed with antiphospholipid syndrome.

摘要

抗磷脂综合征是一种多系统自身免疫性疾病,可产生针对多种磷脂和磷脂结合蛋白的自身抗体。其在人群中的发病率为1.8%,仅2%的病例为儿科患者。临床表现范围广泛,从无症状患者到像灾难性抗磷脂综合征这样危及生命的疾病。任何器官都可能受累。儿科中最常见的表现为60%的患者出现静脉血栓形成,32%出现动脉血栓形成,38%出现血液系统疾病(血小板减少、白细胞减少),18%出现皮肤改变(网状青斑、雷诺现象),16%出现神经体征。我们描述了一名先前健康的14岁女性患者被诊断为抗磷脂综合征的病例。

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