In adults with end-stage liver disease concurrent changes in pro- and antihemostatic pathways result in a rebalanced hemostasis. Children though, have a developing hemostatic system, different disease etiologies, and increased risk of thrombosis. This study aimed to assess the hemostatic state of children during and after liver transplantation. Serial blood samples were obtained from 20 children (≤16 years) undergoing primary liver transplantation (September 2017-October 2018). Routine hemostasis tests, thrombomodulin-modified thrombin generation, clot lysis times, and hemostatic proteins were measured. Reference values were established using an age-matched control group of 30 children. Thrombocytopenia was present in study patients. Von Willebrand factors were doubled and ADAMTS13 levels decreased during and after transplantation up until day 30, when platelet count had normalized. Whereas prothrombin time and activated partial thromboplastin time were prolonged during transplantation, thrombin generation was within normal ranges, except during perioperative heparin administration. Fibrinogen, factor VIII levels, and clot lysis time were elevated up until day 30. In conclusion, children with end-stage liver disease are in tight hemostatic balance. During transplantation a temporary heparin-dependent hypocoagulable state is present, which rapidly converts to a hemostatic balance with distinct hypercoagulable features that persist until at least day 30. This hypercoagulable state may contribute to the risk of posttransplant thrombosis.

中文翻译：

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儿科肝移植中重新平衡止血系统的证据：一项前瞻性队列研究。

在患有终末期肝病的成人中，促止血途径和抗止血途径的同时变化导致止血重新平衡。然而，儿童的止血系统正在发育，疾病病因不同，并且血栓形成的风险增加。本研究旨在评估儿童肝移植术中及术后的止血状态。从 20 名接受原发性肝移植（2017 年 9 月至 2018 年 10 月）的儿童（≤16 岁）中获取系列血样。测量了常规止血试验、血栓调节蛋白修饰的凝血酶生成、凝块溶解时间和止血蛋白。使用由 30 名儿童组成的年龄匹配的对照组来确定参考值。研究患者存在血小板减少症。在移植期间和移植后直至第 30 天，冯维勒布兰德因子增加了一倍，ADAMTS13 水平下降，当血小板计数恢复正常时。尽管在移植期间凝血酶原时间和活化的部分凝血活酶时间延长，但凝血酶生成在正常范围内，围手术期肝素给药期间除外。纤维蛋白原、凝血因子 VIII 水平和凝块溶解时间一直升高到第 30 天。总之，患有终末期肝病的儿童处于严格的止血平衡状态。在移植过程中，存在一种暂时的肝素依赖性低凝状态，它会迅速转变为具有明显高凝特征的止血平衡，这种状态至少持续到第 30 天。这种高凝状态可能会增加移植后血栓形成的风险。凝血酶生成在正常范围内，围手术期肝素给药期间除外。纤维蛋白原、凝血因子 VIII 水平和凝块溶解时间一直升高到第 30 天。总之，患有终末期肝病的儿童处于严格的止血平衡状态。在移植过程中，存在一种暂时的肝素依赖性低凝状态，它会迅速转变为具有明显高凝特征的止血平衡，这种状态至少持续到第 30 天。这种高凝状态可能会增加移植后血栓形成的风险。凝血酶生成在正常范围内，围手术期肝素给药期间除外。纤维蛋白原、凝血因子 VIII 水平和凝块溶解时间一直升高到第 30 天。总之，患有终末期肝病的儿童处于严格的止血平衡状态。在移植过程中，存在一种暂时的肝素依赖性低凝状态，它会迅速转变为具有明显高凝特征的止血平衡，这种状态至少持续到第 30 天。这种高凝状态可能会增加移植后血栓形成的风险。