Brown Jeffrey B, Emerick Karan M, Brown Deborah L, Whitington Peter F, Alonso Estella M
Department of Gastroenterology, Hepatology, and Nutrition, Children's Memorial Hospital, Northwestern University, Feinberg School of Medicine, Chicago, IL 60614, USA.
J Pediatr Gastroenterol Nutr. 2003 Sep;37(3):268-72. doi: 10.1097/00005176-200309000-00013.
Coagulopathy is an important cause of morbidity and mortality in patients with liver failure. The benefit of traditional therapies to correct coagulation is often limited and short-lived. Our aim is to identify indications for rFVIIa use and the outcome of treatment in children with liver failure.
A retrospective review from July 2000 to December 2001 was performed to identify consecutive patients with acute or chronic liver failure who received rFVIIa. Prothrombin times (PT) before and after therapy were compared by paired t test.
Fifteen patients were treated with rFVIIa for coagulopathy caused by liver failure. All were receiving fresh frozen plasma (mean infusion rate, 39.7 mL/kg/day) when rFVIIa therapy was started. The mean PT before rFVIIa was 32.0 +/- 7.0 seconds. One hour after infusion, the PT normalized to 13.7 +/- 2.4 seconds (P < 0.0001) and remained significantly reduced at 6 hours (19.8 +/- 5.3 seconds; P < 0.0001). A sustained improvement was maintained during the subsequent 3 days. Five of seven patients with bleeding complications improved clinically after rFVIIa treatment. Two of the bleeding patients also benefited from improved fluid balance as fresh frozen plasma support was reduced. No thrombotic events were attributed to rFVIIa therapy.
In patients with liver failure, rFVIIa therapy quickly normalizes the PT and maintains improved hemostasis, even when coagulopathy has been refractory to fresh frozen plasma. Therapy subjectively reduces clinical bleeding and can improve fluid balance, without complications.
凝血功能障碍是肝衰竭患者发病和死亡的重要原因。传统疗法纠正凝血功能的益处往往有限且持续时间短。我们的目的是确定重组人活化凝血因子VII（rFVIIa）在肝衰竭儿童中的使用指征及治疗结果。
对2000年7月至2001年12月期间连续接受rFVIIa治疗的急性或慢性肝衰竭患者进行回顾性研究。通过配对t检验比较治疗前后的凝血酶原时间（PT）。
15例因肝衰竭导致凝血功能障碍的患者接受了rFVIIa治疗。开始rFVIIa治疗时，所有患者均接受新鲜冰冻血浆（平均输注速率为39.7 mL/kg/天）。rFVIIa治疗前的平均PT为32.0±7.0秒。输注后1小时，PT恢复正常，为13.7±2.4秒（P<0.0001），6小时时仍显著降低（19.8±5.3秒；P<0.0001）。在随后3天内维持持续改善。7例有出血并发症的患者中，5例在rFVIIa治疗后临床症状改善。随着新鲜冰冻血浆支持量减少，2例出血患者的液体平衡也得到改善。未发现rFVIIa治疗导致血栓形成事件。
在肝衰竭患者中，rFVIIa治疗可迅速使PT恢复正常并维持止血功能改善，即使凝血功能障碍对新鲜冰冻血浆治疗无效。该治疗可主观减轻临床出血并改善液体平衡，且无并发症。
