儿童自身免疫性肝炎的治疗结果和治疗方法,表现为急性肝衰竭:单个患者数据的元分析
Aly Fawzy1, Harry Sutton2, Shannon M Vandriel2
1Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
概括
患有急性肝衰竭的儿童的自身免疫性肝炎在结合性皮质类固醇和非类固醇免疫调节剂治疗时具有更好的结果. 1型自身免疫性肝炎独立预测本地肝脏的存活率,这表明量身定制的治疗方法至关重要.
科学领域:
- 儿科肝病学 儿科肝病学
- 自免疫性疾病 自免疫性疾病
- 胃肠病学 胃肠病学
背景情况:
- 在儿童中呈现为急性肝衰竭 (ALF) 的自身免疫性肝炎 (AIH) 是一种危急的疾病,通常需要进行肝移植 (LTx).
- 关于儿科AIH-ALF管理和结果的有限的大规模研究存在.
- 本研究通过个体患者数据元分析 (IPD) 解决了这一差距.
研究的目的:
- 分析患有AIH-ALF的儿科患者的管理策略和结果.
- 确定影响本地肝存活 (NLS) 的因素以及对LTx.的需求.
- 为这个脆弱的患者群体提供临床决策的信息.
主要方法:
- 按照PRISMA指南进行了系统审查和IPD元分析.
- 数据来源于PubMed和Excerpta Medica,用于2000年至2020年间发表的研究.
- 包括的患者年龄在21岁以下,患有AIH (1或2型) 呈现为ALF,并提取了临床,生化,干预和结局数据.
主要成果:
- 分析包括335名患者 (61项研究+5项来自该机构),主要是女性 (66.8%),平均年龄为10.
- 原生肝存活率 (NLS) 达到了59.7%,35%接受了LTx,5%在LTx前死亡.
- 联合治疗 (皮质类固醇 + 非类固醇免疫调节剂) 增加了NLS的可能性2.5倍;AIH-1独立地与更高的NLS相关 (OR 3.8).
结论:
- 用皮质类固醇和非类固醇免疫调节剂治疗可能会提高儿科AIH-ALF中NLS的概率.
- 治疗策略应考虑患者特异性因素,特别是AIH的类型.
- 需要进一步的研究来确定预测需要联合免疫抑制的生物标志物,以潜在地避免LTx.
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