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儿童急性肝衰竭病因及预后指标分析

Etiology and prognostic indicators of pediatric acute liver failure

    摘要
  • 摘要:
      目的  分析儿童急性肝衰竭（PALF）的病因及预后指标。
      方法  回顾性分析64例PALF患儿的临床资料，根据预后的不同将患儿分为死亡组和存活组，比较2组患儿的年龄、性别、生化指标和儿童终末期肝病模型（PELD）评分。
      结果  本研究PALF患儿的最常见病因为遗传代谢疾病（18例），此后依次为感染因素（13例）、药物毒副作用与中毒（8例）、血液系统疾病（4例）、自身免疫性肝病（2例）、缺血性肝病（1例），另有18例患儿病因不明；死亡组患儿总胆红素（TB）、国际标准化比值（INR）、PELD评分水平高于存活组，γ-谷氨酰转肽酶（GGT）水平低于存活组，差异有统计学意义（P < 0.05或P < 0.01）；受试者工作特征（ROC）曲线分析显示，GGT、TB、INR三者联合的曲线下面积为0.793（95% CI为0.684~0.903），诊断PALF预后的敏感度为0.828，特异度为0.626。
      结论  遗传代谢疾病是PALF的主要病因，PALF患儿出现TB、INR升高和GGT降低时提示预后不良，需尽早完善评估并及时选择合适的治疗方案。

     

    Abstract:
      Objective  To analyze the etiology and the prognostic indicators of pediatric acute liver failure (PALF).
      Methods  A retrospective study was performed on the data of 64 patients with PALF. The patients were then divided into survival group and death group based on their prognosis. Age, gender, biochemical indicators and pediatric of end-stage liver disease (PELD) were compared between the two groups.
      Results  The common causes of PALF were identified as genetic and metabolic diseases (18 cases), followed by infectious diseases (13 cases), drug adverse reations and toxicity(8 cases), hematopathy (4 cases), immunologic liver diseases (2 cases), ischemic liver disease (1 case), and unclear causes (18 cases). The levels of total bilirubin (TB), international standardized ratio (INR) and PELD score in the death group were higher than those in the survival group, and the level of γ-glutamyl transpeptidase (GGT) in the death group was lower than that in the survival group (P < 0.05 or P < 0.01). Receiver operating characteristic (ROC) curve analysis showed that the combined area under the curve of GGT, TB and INR in combination was 0.793 (95%CI, 0.684 to 0.903), and the prognostic sensitivity and specificity of PALF diagnosis were 0.828 and 0.626, respectively.
      Conclusion  Inherited metabolic disorders are the most common known cause of PALF. For patients with PALF, higher levels of TB, INR and lower level of GGT may indicate a poor prognosis.

     

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