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评估有出血倾向的儿童血小板功能障碍——一项单中心研究。

Evaluating platelet function disorders in children with bleeding tendency - A single center study.

机构信息

a Pediatric Ward A , Haemek Medical Center , Afula , Israel.

b Pediatric Hematology-Oncology , Schneider Children's Medical Center of Israel , Petach Tikva , Israel.

出版信息

Platelets. 2017 Nov;28(7):676-681. doi: 10.1080/09537104.2016.1257784. Epub 2017 Jan 6.

Abstract

Platelet function disorders (PFDs) are a common cause of mild bleeding tendency. However, they cannot be recognized by standard screening studies. The gold standard test for PFD is platelet aggregation, performed by light transmission aggregometry (LTA). A newer and less validated method is the closure time (CT), performed by the platelet function Analyzer 100 (PFA-100). Data regarding the validity of these tests in children are limited. The aim of this study was to evaluate the usefulness of LTA and PFA-100 for the diagnosis of pediatric patients with bleeding tendency. This retrospective study included patients one month-18 year old that had LTA tests performed at the coagulation laboratory of Rabin Medical Center between the years 2006-2015. Bleeding severity was assessed using a pediatric bleeding score. Patients were excluded from analysis if they had thrombocytopenia, thrombocytosis or coagulation factors deficiencies. One hundred and thirty-seven (137) patients were included in the analysis. The median age was 7.5 years (range one month-18 years). Most patients (93%) had a bleeding score of 2 or more. Abnormal LTA was found in 40% and prolonged CT in 23% of the patients. Abnormal LTA was significantly more common in patients with a bleeding score of 2 or more compared to patients with a lower bleeding scores (P = 0.04). No significant correlation was found between the bleeding severity and the number of agonists which induced abnormal responses (p = 0.52) or the CT (p = 0.35). Furthermore, no correlation was found between abnormal LTA and prolonged CT. To conclude, we were able to diagnose 40% of children who presented with bleeding tendency with platelet aggregation defects by LTA. Abnormal LTA was significantly more prevalent in patients with a bleeding score of 2 and above. In contrast, CT was not found to be sensitive as a screening tool for PFD. Therefore, our data extend the validity of the use of LTA for the evaluation of pediatric patients with bleeding tendency.

摘要

血小板功能障碍(PFD)是轻度出血倾向的常见原因。然而,这些障碍不能通过标准的筛选研究来识别。血小板功能障碍的金标准测试是通过光传输聚集(LTA)进行的血小板聚集。一种较新的且验证程度较低的方法是通过血小板功能分析仪 100(PFA-100)进行的封闭时间(CT)。关于这些测试在儿童中的有效性的数据有限。本研究的目的是评估 LTA 和 PFA-100 在诊断有出血倾向的儿科患者中的作用。这项回顾性研究纳入了 2006 年至 2015 年在拉宾医学中心凝血实验室进行 LTA 检测的一个月至 18 岁的患者。出血严重程度使用儿科出血评分进行评估。如果患者有血小板减少症、血小板增多症或凝血因子缺乏症,则将其排除在分析之外。共有 137 名患者纳入分析。中位年龄为 7.5 岁(范围为一个月至 18 岁)。大多数患者(93%)的出血评分为 2 分或更高。40%的患者 LTA 异常,23%的患者 CT 延长。与出血评分较低的患者相比,出血评分 2 分或更高的患者 LTA 异常更为常见(P=0.04)。未发现出血严重程度与诱导异常反应的激动剂数量(p=0.52)或 CT(p=0.35)之间存在显著相关性。此外,也未发现 LTA 异常与 CT 延长之间存在相关性。总之,我们能够通过 LTA 诊断 40%有出血倾向的儿童存在血小板聚集缺陷。出血评分 2 分或更高的患者中 LTA 异常更为常见。相比之下,CT 作为 PFD 的筛选工具并不敏感。因此,我们的数据扩展了 LTA 用于评估有出血倾向的儿科患者的有效性。

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