N-ACETYLCYSTEINE IN TREATMENT OF ACUTE LIVER FAILURE NOT CAUSE BY ACETAMINOPHEN

该子项目是利用NIH/NCRR资助的中心赠款提供的资源的许多研究子项目之一。子项目和研究者（PI）可能从另一个NIH来源获得了主要资金，因此可以在其他CRISP条目中表示。所列机构为中心，不一定是研究者所在机构。本研究的目的是测试一种名为N-乙酰半胱氨酸（NAC）的药物静脉内输注是否能提高非醋氨酚所致急性肝功能衰竭（ALF）儿童的生存率。NAC治疗的最初基础是醋氨酚（Tylenol）过量受试者的获益。根据这一初步观察，在成人中进行的非对照观察性研究表明，NAC可能改善除对乙酰氨基酚外其他原因引起的ALF的结局。NAC可以帮助肝脏从各种损伤中恢复的能力。IV-NAC在欧洲和加拿大用于治疗对乙酰氨基酚过量诱导的ALF和非对乙酰氨基酚诱导的ALF，并且最近在美国被批准用于对乙酰氨基酚过量的这种用途。然而，IV-NAC尚未在美国测试或批准用于非对乙酰氨基酚ALF。这项研究是一项国家计划的一部分，该计划旨在测试IV输注NAC（IV-NAC）在非因乙酰氨基萘而导致ALF的儿童中的价值。将研究在无解毒剂或其他特异性治疗的ALF儿童中静脉注射N-乙酰半胱氨酸7天疗程的安全性和疗效以及总1年生存期（伴或不伴肝移植）。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The purpose of this study is to test whether a drug called N-Acetylcysteine (NAC) given as an intravenous (in the vein) infusion improves survival for children with acute liver failure (ALF) that is not due to acetaminophen.The initial basis for this treatment with NAC is the benefit demonstrated in subjects with acetaminophen (Tylenol) overdose. From this initial observation, uncontrolled observational studies in adults suggest that NAC may improve the outcome of ALF from causes other than acetaminophen. NAC may help the liver's ability to recover from a variety of insults. IV- NAC is used in Europe and Canada for treatment of acetaminophen overdose induced ALF and non-acetaminophen induced ALF and has recently been approved for this use in acetaminophen overdose in the US. However, IV-NAC has not been tested or approved in the U.S. for non-acetaminophen ALF. This study is part of a national program to test the value of an IV infusion of NAC (IV-NAC) in those children with ALF NOT due to acetaminaphen. Safety and efficacy and overall 1 year survival (with or without liver transplantation) of a 7 day course of intravenous N-Acetylcysteine in children with ALF for whom no antidote or other specific treatment is available will be studied.