Sanz París A, Albero Gamoa R, Acha Pérez F J, Playán Usón J, Casamayor Peris L, Celaya Pérez S
Servicio de Endocrinología y Nutrición, Hospital Miguel Servet, España.
Nutr Hosp. 1994 Mar-Apr;9(2):110-3.
In thiamine deficiency, the Krebs cycle slows large quantities of pyruvate are diverted to lactate production and anaerobic metabolism begins. The most frequent cause of this syndrome is a dietary deficiency associated to a greater or lesser degree with alcoholism. Other less frequent causes are the ingestion of raw fish contaminated with microbial thiaminases, inborn errors of metabolism and total parenteral nutrition. We present the clinical case of a patient with an acute thiamine deficiency after 15 days of total parenteral nutrition, which improved with intravenous administration of thiamine. The incidence of beriberi among patients undergoing total parenteral nutrition is very low because of the almost systematic addition of vitamin complexes. Our patient's clinical picture was sudden, corresponding to the dry form, with typical neurological symptoms and signs, major metabolic acidosis, hyperglycemia and hyponatremia. The clinical response to the administration of thiamine confirmed the diagnosis.
在硫胺素缺乏时,三羧酸循环减缓,大量丙酮酸转向乳酸生成,厌氧代谢开始。该综合征最常见的病因是与酗酒程度不同相关的饮食缺乏。其他较不常见的病因包括摄入被微生物硫胺酶污染的生鱼、先天性代谢缺陷和全胃肠外营养。我们报告了1例在全胃肠外营养15天后出现急性硫胺素缺乏的患者临床病例,该患者经静脉注射硫胺素后病情改善。由于几乎系统性地添加了维生素复合物,接受全胃肠外营养的患者中脚气病的发生率非常低。我们患者的临床表现突发,符合干性脚气病,伴有典型的神经症状和体征、重度代谢性酸中毒、高血糖和低钠血症。给予硫胺素后的临床反应证实了诊断。