背景:B型乳酸性酸中毒和低血糖可发生在各种儿科疾病中。在这些代谢紊乱之前有禁食史的幼儿中,应首先考虑先天的代谢错误。然而,Warburg效应,一种罕见的代谢并发症,也可表现为儿童血液系统恶性肿瘤。文献中只有少数关于儿童这种情况的报道。
目的:确定临床病程,治疗策略,儿童恶性血液病伴B型乳酸性酸中毒的结局。
方法:我们对PubMed进行了全面搜索,Scopus,和Cochrane数据库没有任何时间限制,但仅限于英语文章。数据库最后一次访问是在7月1日,2023年。
结果:共20篇出版物被纳入分析,所有这些都是病例报告或病例系列.没有更高质量的证据。在患有血液系统恶性肿瘤和Warburg效应的儿童中,有14例急性淋巴细胞白血病和6例非霍奇金淋巴瘤,包括我们的说明性病例。乳酸性酸中毒发生在55%的新诊断病例和45%的复发病例中。平均年龄为10.3±4.5岁,80%的病例为男性。平均血清乳酸为16.9±12.6mmol/L,43.8%的病例同时伴有低血糖。在接受化疗的患者中,80%的乳酸性酸中毒最初消退,而对比组为60%。新诊断病例死亡率为45.5%,而复发病例代表100%的死亡率。2001年之前报告的所有8名患者均死于疾病相关并发症。然而,2003年至2023年发表的报告中描述的患者完全缓解率为54.5%.
结论:这种并发症在历史上导致了致命的结果;然而,接受化疗的患者表现出更有利的反应.因此,在这种情况下,及时启动特定治疗至关重要。
BACKGROUND: Type B lactic acidosis and hypoglycemia can occur in various pediatric conditions. In young children with a history of fasting preceding these metabolic derangements, inborn errors of metabolism should be primarily considered. However, the Warburg effect, a rare metabolic complication, can also manifest in children with hematologic malignancies. Only a few reports of this condition in children have been published in the literature.
OBJECTIVE: To identify the clinical course, treatment strategies, and outcomes of childhood hematologic malignancies with type B lactic acidosis.
METHODS: We performed a comprehensive search of the PubMed, Scopus, and Cochrane databases without any time restriction but limited to English language articles. The databases were last accessed on July 1st, 2023.
RESULTS: A total of 20 publications were included in the analysis, all of which were case reports or case series. No higher quality evidence was available. Among children with hematologic malignancies and Warburg effect, there were 14 cases of acute lymphoblastic leukemia and 6 cases of non-Hodgkin\'s lymphoma including our illustrative case. Lactic acidosis occurred in 55% of newly diagnosed cases and 45% of relapsed cases. The mean age was 10.3 ± 4.5 years, and 80% of cases were male. The mean serum lactate was 16.9 ± 12.6 mmol/L, and 43.8% of the cases had concomitant hypoglycemia. Lactic acidosis initially subsided in 80% of patients receiving chemotherapy compared to 60% in the contrast group. The mortality rate of newly diagnosed cases was 45.5%, while the relapsed cases represented a 100% mortality rate. All 8 patients reported before 2001 died from disease-related complications. However, patients described in reports published between 2003 and 2023 had a 54.5% rate of complete remission.
CONCLUSIONS: This complication has historically led to fatal outcome; however, patients who received chemotherapy showed a more favorable response. Therefore, it is crucial to promptly initiate specific treatment in this context.