Kathemann Simone, Bechmann Lars P, Sowa Jan-Peter, Manka Paul, Dechêne Alexander, Gerner Patrick, Lainka Elke, Hoyer Peter F, Feldstein Ariel E, Canbay Ali
Department of Pediatrics II, University Hospital, University Duisburg-Essen, Essen, Germany.
Department of Gastroenterology and Hepatology, University Hospital, University Duisburg-Essen, Essen, Germany.
Ann Hepatol. 2015 Sep-Oct;14(5):722-8.
Pediatric acute liver failure (PALF) is a progressive, potentially fatal clinical syndrome occurring in previously healthy children. Our study aimed to determine the current leading causes of PALF in a single center in Germany, identifying possible prognostic markers. Thirty-seven pediatric patients with PALF were included. Medical records were reviewed for demographic, laboratory and clinical data. Laboratory results on admission and at peak value, PELD and MELD score on admission, and intensive care support were assessed. Fifteen patients recovered spontaneously, 14 died without transplantation, and 8 received a liver transplant. Patients who survived were significantly older than patients who died. Specific causes of PALF could be identified as infectious diseases (16%), metabolic diseases (14%), toxic liver injury (11%), immunologic diseases (8%), or vascular diseases (8%). Causes of PALF remained indeterminate in 43%. High ammonia, low albumin, and low ALT levels on admission were associated with worse outcome. Absence of need of ventilation, hemodialysis, and circulatory support predicted spontaneous recovery. In conclusion, infections are the most common known cause of PALF. However, in a large proportion of patients the cause for PALF remains cryptic. Ammonia and albumin levels may be of prognostic value to predict outcomes.
小儿急性肝衰竭（PALF）是一种发生在既往健康儿童中的进行性、潜在致命性临床综合征。我们的研究旨在确定德国一个单一中心目前PALF的主要病因，识别可能的预后标志物。纳入了37例PALF患儿。查阅病历以获取人口统计学、实验室和临床数据。评估入院时及峰值时的实验室结果、入院时的PELD和MELD评分以及重症监护支持情况。15例患者自发恢复，14例未接受移植死亡，8例接受了肝移植。存活患者的年龄显著大于死亡患者。PALF的具体病因可确定为感染性疾病（16%）、代谢性疾病（14%）、中毒性肝损伤（11%）、免疫性疾病（8%）或血管性疾病（8%）。43%的PALF病因仍不明确。入院时高氨、低白蛋白和低ALT水平与较差的预后相关。无需通气、血液透析和循环支持预示着自发恢复。总之，感染是PALF最常见的已知病因。然而，在很大一部分患者中，PALF的病因仍不明。氨和白蛋白水平可能对预测预后有价值。
