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Changing Etiologies and Prognostic Factors in Pediatric Acute Liver Failure

机译:在儿科急性肝衰竭中改变病因和预后因素

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After the implementation of universal hepatitis A virus vaccination in Argentina, the outcome of pediatric acute liver failure (PALF) remains unknown. We aimed to identify variables associated with the risk of liver transplantation (LT) or death and to determine the causes and short-term outcomes of PALF in Argentina. We retrospectively included 135 patients with PALF listed for LT between 2007 and 2016. Patients with autoimmune hepatitis (AIH), Wilson's disease (WD), or inborn errors of metabolism (IEM) were classified as PALF-chronic liver disease (CLD), and others were classified as "pure" PALF. A logistic regression model was developed to identify factors independently associated with death or need of LT and risk stratification. The most common etiologies were indeterminate (52%), AIH (23%), WD (6%), and IEM (6%). Overall, transplant-free survival was 35%, whereas 50% of the patients underwent LT and 15% died on the waiting list. The 3-month risk of LT or death was significantly higher among patients with pure PALF compared with PALF-CLD (76.5% versus 42.5%; relative risk, 1.8 [1.3-2.5]; P < 0.001), and 3 risk factors were independently associated with worse outcome: international normalized ratio (INR) >= 3.5 (odds ratio [OR], 3.1; 95% confidence interval [CI], 1.3-7.2]), bilirubin >= 17 mg/dL (OR, 4.4; 95% CI, 1.9-10.3]), and pure PALF (OR, 3.8; 95% CI, 1.6-8.9). Patients were identified by the number of risk factors: Patients with 0, 1, or >= 2 risk factors presented a 3-month risk of worse outcome of 17.6%, 36.6%, and 82%, respectively. In conclusion, although lacking external validation, this simple risk-staging model might help stratify patients with different transplant-free survival rates and may contribute to establishing the optimal timing for LT.
机译:在阿根廷的通用甲型肝炎病毒疫苗接种后,小儿急性肝功能衰竭(PALF)的结果仍然未知。我们旨在鉴定与肝移植(LT)或死亡风险相关的变量,并确定阿根廷氏症的原因和短期结果。我们回顾性地包括在2007年至2016年期间列出的135名PALF患者。患有自身免疫性肝炎(AIH),威尔逊疾病(WD)或原始代谢疾病(IEM)的患者被归类为PALF-慢性肝病(CLD),以及其他人被归类为“纯粹的”帕尔夫。开发了一种逻辑回归模型,以识别与死亡或需求与LT和风险分层无关的因素。最常见的病因是不确定(52%),AIH(23%),WD(6%)和IEM(6%)。总体而言,无移植存活率为35%,而50%的患者在等候名单上死亡,15%死亡。纯Palf患者的3个月或死亡的风险明显高于PALF-CLD(76.5%对42.5%;相对风险,1.8 [1.3-2.5]; P <0.001),3个风险因素独立与更差的结果相关:国际归一化比率(INR)> = 3.5(差距[或],3.1; 95%置信区间[CI],1.3-7.2]),胆红素> = 17mg / dL(或4.4; 95 %CI,1.9-10.3])和纯PALF(或,3.8; 95%CI,1.6-8.9)。患者被危险因素的数量鉴定出来:0,1或> = 2患者的风险因素分别呈现3个月的较差结果,分别为17.6%,36.6%和82%。总之,尽管缺乏外部验证,但这种简单的风险分期模型可能有助于分层自由移植存活率的患者,并且可能有助于建立LT的最佳时间。

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