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Usefulness of multiple electrode aggregometry as a screening tool for bleeding disorders in a pediatric hospital

机译：多电极聚集体作为儿科医院出血障碍的筛选工具

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摘要

Platelet function testing is a cornerstone in the diagnostic investigation of patients with a bleeding history. Multiple electrode aggregometry (MEA) has been shown to detect von Willebrand disease (VWD), platelet function disorders, and drug-induced bleeding disorders. However, there are few studies supporting its successful use in children. We have implemented and used MEA over 3 years in our hemostasis laboratory in order to study its usefulness to supplement and expedite diagnosis. This is a retrospective, single-center, cohort study of 109 hospitalized children who underwent a laboratory investigation of hemostasis and either had a reported bleeding history or an abnormal bleeding episode. Plasmatic coagulation testing, blood counts, plasmatic von Willebrand testing, platelet function analyzer (PFA-100), and impedance aggregometry (MEA) were performed in all children. Light transmission aggregometry testing was performed as needed. In 41 cases (37.6%), a working diagnosis was made; a primary hemostatic disorder was detected in 35 children (VWD (n = 16), platelet disorder (n = 15), and valproic acid therapy-induced bleeding disorder (n = 3), acetylsalicylic acid-related bleeding (n = 1). In patients diagnosed with VWD, MEA ristocetin-induced platelet aggregation test (RISTO) high test revealed abnormally low aggregation in six patients (43.8%); whereas in patients diagnosed with a platelet function disorder, abnormally low values were found by MEA in only three children (20%). Three of the four children with laboratory evidence of drug-induced platelet dysfunction had abnormalities on MEA. There were no cases in which an abnormal MEA result was used to make a previously undetermined diagnosis. Retrospectively, MEA has demonstrated limited additional diagnostic value beyond standard laboratory testing for detecting defects of primary hemostasis in children.
机译：血小板函数测试是一种基石在出血历史的诊断调查中。已经显示多电极聚集体（MEA）检测von Willebrand疾病（VWD），血小板功能障碍和药物诱导的出血障碍。但是，很少有研究支持其在儿童的成功使用。我们在我们的止血实验室中实施和使用了3年以上的MEA，以研究其对补充和加快诊断的有用性。这是回顾性，单中心，队列的109名住院儿童的研究，他们经历了对止血的实验室调查，并且有报告的出血历史或异常出血集。在所有儿童中进行了血浆凝血测试，血液计数，血浆Von Willebrand测试，血小板功能分析仪（PFA-100）和阻抗聚合物（MEA）。根据需要进行光传输聚合测试。在41例（37.6％）中，进行了工作诊断;在35名儿童（VWD（n = 16），血小板障碍（n = 15）和丙甲酸治疗诱导的出血障碍（n = 3）中检测到一次止血障碍（VWD（n = 16），乙酰胱氨酸相关出血（n = 1）。在患有VWD的患者中，MEA ristocetin诱导的血小板聚集试验（Risto）高测试显示出六名患者的异常低聚集（43.8％）;而在诊断出血小板功能障碍的患者中，MEA仅在三个患者中发现异常低的值儿童（20％）。有四个儿童有4名药物诱导的血小板功能障碍的儿童患有MEA的异常。没有病例，其中使用异常的MEA结果来制作先前未确定的诊断。回顾性，MEA已经证明了有限公司用于检测儿童原发性止血缺陷的标准实验室检测的额外诊断价值。

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