National Hemophilia Center, Coagulation Unit and Amalia Biron Research Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel Hashomer and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Department of Diagnostic Imaging, Sheba Medical Center, Tel Hashomer, Israel.
Semin Thromb Hemost. 2023 Apr;49(3):217-224. doi: 10.1055/s-0042-1756704. Epub 2022 Sep 29.
Studies from the past 50 years have contributed to the expanding knowledge regarding developmental hemostasis. This is a dynamic process that begins in the fetal phase and is characterized by physiological variations in platelet counts and function, and concentrations of most coagulation factors and the native coagulation inhibitors in early life, as compared with adulthood. The developmental hemostasis studies since the 1980 to 1990s established the laboratory reference values for coagulation factors. It was only a decade or two later, that thromboelastography (TEG) or (rotational thromboelastometry [ROTEM]) as well as thrombin generation studies, provided special pediatric reference values along with the ability to evaluate clot formation and lysis. In addition, global whole blood-based clotting assays provided point of care guidance for proper transfusion support to children hospitalized in intensive care units or undergoing surgery. Although uncommon, thrombosis in children and neonates is gaining increasing recognition, typically as a secondary complication in sick children. Bleeding in children, and particularly intracerebral hemorrhage in newborns, still represent a therapeutic challenge. Notably, our review will outline the advancements in understanding developmental hemostasis and its manifestations, with respect to the pathophysiology of thrombosis and bleeding complications in young children. The changes of transfusion policy and approach to thrombophilia testing during the last decade will be mentioned. Subsequently, a brief summary of the data on anticoagulant treatments in pediatric patients will be presented. Finally, we will point out the 10 most cited articles in the field of pediatric and neonatal hemostasis.
过去 50 年的研究为不断扩展的发育性止血知识做出了贡献。这是一个始于胎儿期的动态过程，其特点是血小板计数和功能，以及大多数凝血因子和天然抗凝剂的浓度在生命早期与成年期相比存在生理性变化。20 世纪 80 年代至 90 年代的发育性止血研究建立了凝血因子的实验室参考值。仅仅过了一二十年，血栓弹力图（TEG）或（旋转血栓弹力测定法 [ROTEM]）以及凝血酶生成研究提供了特殊的儿科参考值，同时还能够评估血栓形成和溶解。此外，基于全血的全球凝血检测为重症监护病房或接受手术的住院儿童提供了适当的输血支持的即时护理指导。尽管罕见，但儿童和新生儿的血栓形成越来越受到关注，通常是患病儿童的继发并发症。儿童出血，尤其是新生儿颅内出血，仍然是一个治疗挑战。值得注意的是，我们的综述将概述对发育性止血及其表现的认识进展，以及小儿血栓形成和出血并发症的病理生理学。将提到过去十年中输血政策和血栓形成倾向检测方法的改变。随后，将简要总结儿科患者抗凝治疗的数据。最后，我们将指出儿科和新生儿止血领域中被引用最多的 10 篇文章。
