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沃伯格效应模仿婴儿血液恶性瘤代谢的先天性错误:一个基于病例的系统审查
Khanittha Permtawee1, Maliwan Tengsujaritkul1, Chane Choed-Amphai2
1Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand.
World journal of clinical pediatrics
|January 5, 2024
概括
在患有血液性恶性瘤的儿童中,B型乳酸,一种罕见的华堡效应并发症,历史上导致致命的结果. 迅速启动化疗显示出更有利的反应,改善缓解率.
科学领域:
- 儿科瘤学 儿科瘤学
- 代谢障碍 代谢障碍 代谢障碍
- 血液学恶性瘤是什么
背景情况:
- B型乳酸和低血糖症在儿科疾病中很常见,通常表明代谢的先天性错误.
- 华堡效应是一种罕见的代谢并发症,也可能发生在患有血液性恶性瘤的儿童中.
- 关于儿童血液性恶性瘤中这种并发症的报道很少.
研究的目的:
- 调查与B型乳酸性酸相关的儿童血液性恶性瘤的临床过程,治疗策略和结果.
- 综合现有的关于这种罕见并发症的文献.
主要方法:
- 在PubMed,Scopus和Cochrane数据库中进行了全面的文献搜索.
- 搜索包括没有时间限制的英语文章,最后一次访问于2023年7月1日.
- 分析包括20个案例报告和案例系列.
主要成果:
- 该研究确定了14例急性淋巴细胞白血病和6例具有华堡效应的非霍奇金淋巴瘤.
- 乳酸发生在55%的新诊断病例和45%的复发病例中,平均乳酸含量为16.9 mmol/L.
- 化疗导致80%的患者出现初始乳酸沉降,在2003年至2023年报告的病例中,完全缓解率为54.5%,而复发病例的死亡率为100%.
结论:
- 在小儿血液性恶性瘤中,B型乳酸性化历来具有很高的死亡率.
- 化疗在治疗这种并发症方面表现出更有利的反应.
- 迅速启动特定治疗对于改善患者的治疗结果至关重要.
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