Division of Digestive and Liver Diseases, University of Texas Southwestern Medical Center, Dallas, TX 75390-8887, USA.
Best Pract Res Clin Gastroenterol. 2012 Feb;26(1):3-16. doi: 10.1016/j.bpg.2012.01.014.
Acute liver failure is a remarkably rare syndrome, the result of rapid hepatocyte injury occurring over days or a few weeks, and encompassing multiple etiologies, but all with a remarkably similar clinical picture. The clinical features of coagulopathy and encephalopathy characterize this severe and often fatal condition. To date, transplantation has been the only reliable form of rescue for many patients. Recent developments have included a clearer understanding of the different contributing etiologies, how to build a diagnosis and prognosis based on initial laboratory findings, a more aggressive approach to intensive care management and more detailed understanding of the role of transplantation in this setting. This review will provide an overview of standard practices and new research initiatives and findings for this interesting but vexing orphan disease. Particular attention will be paid to practical matters for clinicians to consider in approaching the ALF patient. Few controlled clinical trials have been possible because of the condition's rarity. Critical care of these rare patients is key to their survival and decisions must be made decisively, sometimes with inadequate information. Experience is helpful but experienced clinician managers are even rarer than the disease: few hepatologists or intensivists have in-depth experience with ALF patients.
急性肝衰竭是一种罕见的综合征,其特征为肝细胞在数天或数周内迅速受损,病因多样,但临床表现极为相似。凝血功能障碍和肝性脑病是该严重且常致命疾病的特征。迄今为止,移植是许多患者唯一可靠的抢救手段。最近的进展包括对不同病因的认识更加清晰,如何根据初始实验室发现建立诊断和预后,更积极的重症监护管理方法,以及更详细地了解移植在这种情况下的作用。这篇综述将概述该有趣但棘手的孤儿病的标准治疗方法和新的研究计划和发现。特别关注临床医生在处理 ALF 患者时需要考虑的实际问题。由于该疾病的罕见性,几乎不可能进行对照临床试验。对这些罕见患者的重症监护是其生存的关键,必须果断做出决策,有时信息不足。经验是有帮助的,但经验丰富的临床医生管理者比这种疾病更为罕见:很少有肝病专家或重症监护专家对 ALF 患者有深入的经验。