Muntean Wolfgang
Department of Pediatrics, University of Graz, Graz, Austria.
Thromb Res. 2002 Oct 31;107 Suppl 1:S29-32. doi: 10.1016/s0049-3848(02)00149-4.
Generally, the rules of good practice in transfusion medicine apply also to the pediatric age group. However, the frequency of specific diseases that might necessitate the administration of fresh frozen plasma (FFP) differs from that in adults. Physiologic differences to the later age exist in the neonatal period and in young infants, especially with respect to the hemostatic system, that must be recognized when considering administration of FFP. The plasma levels of many procoagulant factors and important anticoagulants are lower in neonates than in other age groups. Despite these findings, healthy neonates show no easy bruising, no increased bleeding during surgery, and excellent wound healing. The same discrepancy obtains between in vitro and clinical findings with primary hemostasis in neonates. The good primary hemostasis in neonates despite poor in vitro platelet function seems to be due mainly to a very high von Willebrand factor and the presence of more high-multimeric subunits of von Willebrand factor than later in life. We must assume that these particular plasma levels of procoagulant and anticoagulant proteins are essential for the correct function of neonatal hemostasis. Evidence that the hemostatic system of neonates works best with physiologic concentrations of procoagulants and anticoagulants can also be inferred from studies where the administration of clotting factor concentrates gave poor results.Since healthy neonates and young infants have excellent hemostasis, there is absolutely no indication to 'correct' these values to adult's norms prior to invasive procedures by administering FFP. Indications for FFP, met more frequently in the pediatric age group than later in life, are exchange transfusion and extracorporeal membrane oxygenation. Indications applying equally to adults are other extracorporeal life support systems, disseminated intravascular coagulation, hepatic coagulopathy, and 'complex unclear coagulopathies'. In congenital clotting factor deficiency, replacement therapy is much more easily administered using a highly specific concentrate. When FFP is used to raise the level of the congenitally deficient factor, the huge volume needed to reach sufficiently high plasma levels can frequently be a major problem. For this reason, FFP as a replacement therapy in congenital factor deficiency is only indicated when no specific concentrate is available, as is the case in factor V deficiency and factor XI deficiency.
一般来说，输血医学中的良好操作规范同样适用于儿科年龄段。然而，可能需要输注新鲜冰冻血浆（FFP）的特定疾病的发生率与成人不同。新生儿期和小婴儿与较大年龄儿童存在生理差异，尤其是在止血系统方面，在考虑输注FFP时必须认识到这些差异。许多促凝血因子和重要抗凝剂的血浆水平在新生儿中低于其他年龄组。尽管有这些发现，但健康的新生儿没有容易出现瘀伤，手术期间没有出血增加，并且伤口愈合良好。新生儿原发性止血的体外和临床结果之间也存在同样的差异。尽管新生儿体外血小板功能较差，但良好的原发性止血似乎主要是由于血管性血友病因子（vWF）水平非常高，并且vWF的高多聚体亚单位比以后的生命阶段更多。我们必须假设这些促凝血和抗凝蛋白的特定血浆水平对于新生儿止血的正确功能至关重要。从凝血因子浓缩物给药效果不佳的研究中也可以推断出，新生儿的止血系统在促凝血剂和抗凝剂的生理浓度下工作最佳。由于健康的新生儿和小婴儿具有良好的止血功能，在侵入性操作前通过输注FFP将这些值“纠正”到成人标准绝对没有指征。FFP的适应证在儿科年龄段比以后的生命阶段更常见，包括换血疗法和体外膜肺氧合。同样适用于成人的适应证是其他体外生命支持系统、弥散性血管内凝血、肝性凝血病和“复杂不明凝血病”。在先天性凝血因子缺乏症中，使用高度特异性的浓缩物进行替代治疗要容易得多。当使用FFP来提高先天性缺乏因子的水平时，达到足够高的血浆水平所需的巨大体积常常是一个主要问题。因此，FFP作为先天性因子缺乏症的替代疗法仅在没有特异性浓缩物可用时才适用，如在因子V缺乏症和因子XI缺乏症的情况下。
