Baldo Francesco, Drago Enrico, Nisticò Daniela, Buratti Silvia, Calvillo Michaela, Micalizzi Concetta, Schiaffino Maria Cristina, Maghnie Mohamad
Institute for Maternal and Child Health IRCCS Burlo Garofolo, 34137 Trieste, Italy.
Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, 16147 Genoa, Italy.
Children (Basel). 2023 Sep 26;10(10):1602. doi: 10.3390/children10101602.
Lactic acidosis is characterized by an excessive production of lactic acid or by its impaired clearance. Thiamine deficiency is an uncommon cause of lactic acidosis, especially in countries where malnutrition is rare. We describe the case of a 5-year-old boy who presented with a central nervous system relapse of acute lymphoblastic leukemia. During the chemotherapy regimen, the patient developed drug-induced pancreatitis with paralytic ileus requiring prolonged glucosaline solution infusion. In the following days, severe lactic acidosis (pH 7.0, lactates 253 mg/dL, HCO3- 8 mmol/L) was detected, associated with hypoglycemia (42 mg/dL) and laboratory signs of acute liver injury. Due to the persistent hypoglycemia, the dextrose infusion was gradually increased. Lactates, however, continued to raise, so continuous venovenous hemodiafiltration was started. While lactates initially decreased, 12 h after CVVHDF suspension, they started to raise again. Assuming that it could have been caused by mitochondrial dysfunction due to vitamin deficiency after prolonged fasting and feeding difficulties, parenteral nutrition and thiamine were administered, resulting in a progressive reduction in lactates, with the normalization of pH during the next few hours. In the presence of acute and progressive lactic acidosis in a long-term hospitalized patient, thiamine deficiency should be carefully considered and managed as early as possible.
乳酸酸中毒的特征是乳酸产生过多或清除受损。硫胺素缺乏是乳酸酸中毒的罕见原因，尤其是在营养不良罕见的国家。我们描述了一名5岁男孩的病例，他出现了急性淋巴细胞白血病的中枢神经系统复发。在化疗期间，患者发生了药物性胰腺炎并伴有麻痹性肠梗阻，需要长时间输注葡萄糖盐水溶液。在接下来的几天里，检测到严重的乳酸酸中毒（pH 7.0，乳酸253 mg/dL，HCO3- 8 mmol/L），伴有低血糖（42 mg/dL）和急性肝损伤的实验室指标。由于持续性低血糖，葡萄糖输注量逐渐增加。然而，乳酸水平持续升高，因此开始进行持续静脉血液透析滤过。虽然乳酸水平最初有所下降，但在连续性静脉-静脉血液透析滤过停止12小时后，又开始升高。假设这可能是由于长期禁食和喂养困难后维生素缺乏导致的线粒体功能障碍引起的，于是给予了肠外营养和硫胺素，乳酸水平逐渐降低，在接下来的几个小时内pH值恢复正常。对于长期住院患者出现急性进行性乳酸酸中毒的情况，应尽早仔细考虑并处理硫胺素缺乏问题。
