Özçay Figen, Karadağ Öncel Eda, Barış Zeren, Canan Oğuz, Moray Gökhan, Haberal Mehmet
Department of Pediatrics, Division of Gastroenterology, Hepatology, and Nutrition, Başkent University School of Medicine, Ankara, Turkey.
Turk J Gastroenterol. 2016 Sep;27(5):450-457. doi: 10.5152/tjg.2016.16431.
BACKGROUND/AIMS: Our aim was to determine the etiologies, outcomes, and prognostic indicators in children with acute liver failure.
Ninety-one patients who were followed for pediatric acute liver failure (PALF) over a 15-year period were included. Patients who survived with supportive therapy were designated as Group 1, while those who died or underwent liver transplantation were designated as Group 2.
There were 37 (40.6%) patients in Group 1 (spontaneous recovery) and 54 (59.4%) patients in Group 2. Thirty-two patients (35.2%) underwent liver transplantation. Infectious and indeterminate causes were the most common etiologies (33% each). Among the infectious causes, hepatitis A (76%) was the most frequent. Hepatic encephalopathy grade 3-4 on admission and during follow-up and high Pediatric Risk of Mortality (PRISM) and Pediatric End-Stage Liver Disease (PELD) scores within the first 24 h were related with a poor prognosis. Group 2 had a more prolonged prothrombin time, higher international normalized ratio, more prolonged activated partial thromboplastin time (aPTT), and higher levels of total and direct bilirubin, ammonia, and lactate (for all, p<0.01).
Infectious and indeterminate cases constituted the most common etiology of PALF, and the etiology was related to the prognosis in our series. Although high PELD and PRISM scores were related to poor prognoses, no sharp thresholds for individual laboratory tests could be elucidated. Liver transplantation was the only curative treatment for patients with poor prognoses and resulted in high survival rates (1-, 5-, and 10-year survival rates of 81.3%, 81.3%, and 75%, respectively) in our study.
背景/目的：我们的目的是确定急性肝衰竭患儿的病因、结局及预后指标。
纳入了15年间接受小儿急性肝衰竭（PALF）随访的91例患者。经支持治疗存活的患者被指定为第1组，而死亡或接受肝移植的患者被指定为第2组。
第1组（自发恢复）有37例（40.6%）患者，第2组有54例（59.4%）患者。32例患者（35.2%）接受了肝移植。感染性和不明原因是最常见的病因（各占33%）。在感染性病因中，甲型肝炎（76%）最为常见。入院时及随访期间的3 - 4级肝性脑病以及最初24小时内较高的小儿死亡风险（PRISM）和小儿终末期肝病（PELD）评分与预后不良相关。第2组的凝血酶原时间延长更明显、国际标准化比值更高、活化部分凝血活酶时间（aPTT）延长更显著，总胆红素、直接胆红素、氨和乳酸水平更高（所有指标，p<0.01）。
感染性和不明原因病例是PALF最常见的病因，在我们的系列研究中病因与预后相关。尽管高PELD和PRISM评分与预后不良相关，但无法明确单个实验室检查的明确阈值。肝移植是预后不良患者的唯一治愈性治疗方法，在我们的研究中其生存率较高（1年、5年和10年生存率分别为81.3%、81.3%和75%）。
