Morley Sarah L
NHS Blood and Transplant and Cambridge University Hospitals NHS Trust, Cambridge Blood Centre, Long Road, Cambridge CB2 0PT, UK.
Arch Dis Child Educ Pract Ed. 2011 Apr;96(2):49-60. doi: 10.1136/adc.2007.135749. Epub 2010 Sep 27.
Acquired coagulopathy is a relatively uncommon occurrence in acute paediatrics but when it occurs is usually associated with significant underlying pathology and often with critical illness. It can be caused by a number of disease processes but infection, blood loss, iatrogenic causes and liver dysfunction are among the commonest. The blood coagulation cascade is complex and intersects with many other physiological pathways. It is also subject to developmental changes, and 'normal' coagulation and haemostasis change considerably during early life. The diagnosis of abnormal coagulation and when treatment should be initiated is influenced both by age and developmental status and limited by the range of tests routinely available to clinicians. Treatment has predominantly involved transfusion of plasma products (usually fresh frozen plasma and cryoprecipitate) but a number of pharmaceutical and human-derived options are now available. Although plasma products are less frequently transfused than red cells or platelets, their use continues to increase and has not followed the reducing usage of other blood components. This article discusses the aetiology of coagulopathy, describes the commonly available diagnostic tests and outlines the evidence available to guide paediatricians when treating acutely ill children with acquired coagulopathy.
获得性凝血病在急性儿科中相对少见，但一旦发生，通常与严重的潜在病理状况相关，且常伴有危重症。它可由多种疾病过程引起，但感染、失血、医源性因素和肝功能障碍是最常见的原因。血液凝固级联反应复杂，与许多其他生理途径相互交叉。它还会受到发育变化的影响，在生命早期，“正常”的凝血和止血会发生相当大的变化。异常凝血的诊断以及何时开始治疗既受年龄和发育状态的影响，也受临床医生常规可用检测范围的限制。治疗主要涉及输注血浆制品（通常是新鲜冰冻血浆和冷沉淀），但现在有多种药物和人源制剂可供选择。尽管血浆制品的输注频率低于红细胞或血小板，但它们的使用仍在增加，并未随着其他血液成分使用的减少而减少。本文讨论凝血病的病因，描述常用的诊断检测方法，并概述在治疗患有获得性凝血病的急性病儿童时可供儿科医生参考的证据。
