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抗磷脂综合征:一项临床与实验室挑战。

Antiphospholipid syndrome: a clinical and laboratorial challenge.

作者信息

Dusse Luci Maria Santana, Silva Fernanda Dias e, Freitas Letícia Gonçalves, Rios Danyelle Romana Alves, Armond Sandra Cristina, Marcolino Milena Soriano

机构信息

Department of Clinical and Toxicological Analyses, Faculty of Pharmacy, Federal University of Minas Gerais, Belo Horizonte, MG, Brazil.

Federal University of São João Del-Rei, São João Del Rei, MG, Brazil.

出版信息

Rev Assoc Med Bras (1992). 2014 Mar-Apr;60(2):181-6. doi: 10.1590/1806-9282.60.02.016.

Abstract

Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia characterized by the presence of a heterogeneous family of antibodies that bind to plasma proteins with affinity for phospholipid surfaces. The two major protein targets of antiphospholipid antibodies are prothrombin and β2-glycoprotein I (β2GPI). APS leads to aprothrombotic state, and it is characterized by the occurrence of arterial, venous or microvascular thrombosis or recurrent fetal loss. The diagnosis of APS is based on a set of clinical criteria and the detection of lupus anticoagulant (LA), anticardiolipin antibodies (ACA) or anti-β2GPI in plasma. Although laboratory tests are essential for APS diagnosis, these tests have limitations associated with the robustness, reproducibility and standardization. The standardization of diagnostic tests for detection of APLAs has been a challenge and a variety of results have been obtained using different commercial kits and in-house techniques. An increased sensitivity of the ELISA kits for detection of ACA effectively has contributed to APS diagnosis. However, the lack of specificity associated with a high number of false-positive results is a clinical and laboratorial challenge, since such results may lead to mistaken clinical decisions, such as prescription of oral anticoagulant, leading to the risk of hemorrhaging. Furthermore, clinicians are often unfamiliar with these tests and have difficulty interpreting them, requiring interaction between clinical and laboratory professionals in order to ensure their correct interpretation.

摘要

抗磷脂综合征(APS)是一种获得性自身免疫性血栓形成倾向,其特征是存在一类异质性抗体家族,这些抗体可与对磷脂表面具有亲和力的血浆蛋白结合。抗磷脂抗体的两个主要蛋白质靶点是凝血酶原和β2-糖蛋白I(β2GPI)。APS会导致血栓形成状态,其特征为动脉、静脉或微血管血栓形成或反复流产。APS的诊断基于一系列临床标准以及血浆中狼疮抗凝物(LA)、抗心磷脂抗体(ACA)或抗β2GPI的检测。尽管实验室检测对于APS诊断至关重要,但这些检测存在与稳健性、可重复性和标准化相关的局限性。用于检测抗磷脂抗体的诊断试验标准化一直是一项挑战,使用不同的商业试剂盒和内部技术已获得了各种各样的结果。ELISA试剂盒检测ACA的灵敏度提高有效地促进了APS的诊断。然而,与大量假阳性结果相关的缺乏特异性是一个临床和实验室挑战,因为此类结果可能导致错误的临床决策,如口服抗凝剂的处方,从而导致出血风险。此外,临床医生通常不熟悉这些检测,难以对其进行解读,需要临床和实验室专业人员之间进行互动以确保正确解读。

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