Maiorana Arianna, Vergine Gianluca, Coletti Valentina, Luciani Matteo, Rizzo Cristiano, Emma Francesco, Dionisi-Vici Carlo
Division of Metabolism and Research Unit of Metabolic Biochemistry, Department of Pediatric Medicine, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
Division of Nephrology and Dialysis, Department of Nephrology & Urology, Bambino Gesù Children's Hospital and Research Institute, Rome, Italy.
Nutrition. 2014 Jul-Aug;30(7-8):948-52. doi: 10.1016/j.nut.2014.02.019. Epub 2014 Mar 13.
Refeeding syndrome can occur in several contexts of relative malnutrition in which an overaggressive nutritional support is started. The consequences are life threatening with multiorgan impairment, and severe electrolyte imbalances. During refeeding, glucose-involved insulin secretion causes abrupt reverse of lipolysis and a switch from catabolism to anabolism. This creates a sudden cellular demand for electrolytes (phosphate, potassium, and magnesium) necessary for synthesis of adenosine triphosphate, glucose transport, and other synthesis reactions, resulting in decreased serum levels. Laboratory findings and multiorgan impairment similar to refeeding syndrome also are observed in acute thiamine deficiency. The aim of this study was to determine whether thiamine deficiency was responsible for the electrolyte imbalance caused by tubular electrolyte losses.
We describe two patients with leukemia who developed acute thiamine deficiency with an electrolyte pattern suggestive of refeeding syndrome, severe lactic acidosis, and evidence of proximal renal tubular dysfunction.
A single thiamine administration led to rapid resolution of the tubular dysfunction and normalization of acidosis and electrolyte imbalance. This demonstrated that thiamine deficiency was responsible for the electrolyte imbalance, caused by tubular electrolyte losses.
Our study indicates that, despite sharing many laboratory similarities, refeeding syndrome and acute thiamine deficiency should be viewed as separate entities in which the electrolyte abnormalities reported in cases of refeeding syndrome with thiamine deficiency and refractory lactic acidosis may be due to renal tubular losses instead of a shifting from extracellular to intracellular compartments. In oncologic and malnourished patients, individuals at particular risk for developing refeeding syndrome, in the presence of these biochemical abnormalities, acute thiamine deficiency should be suspected and treated because it promptly responds to thiamine administration.
再喂养综合征可发生于多种相对营养不良的情况下,即开始过度积极的营养支持时。其后果危及生命,可导致多器官损害及严重的电解质失衡。在再喂养期间,葡萄糖诱导的胰岛素分泌会使脂肪分解突然逆转,并从分解代谢转变为合成代谢。这会导致细胞突然需要三磷酸腺苷合成、葡萄糖转运及其他合成反应所需的电解质(磷酸盐、钾和镁),从而导致血清水平下降。在急性硫胺素缺乏症中也观察到与再喂养综合征相似的实验室检查结果和多器官损害。本研究的目的是确定硫胺素缺乏是否是由肾小管电解质丢失引起的电解质失衡的原因。
我们描述了两名白血病患者,他们出现了急性硫胺素缺乏,伴有提示再喂养综合征的电解质模式、严重乳酸酸中毒及近端肾小管功能障碍的证据。
单次给予硫胺素可使肾小管功能障碍迅速缓解,酸中毒和电解质失衡恢复正常。这表明硫胺素缺乏是由肾小管电解质丢失引起的电解质失衡的原因。
我们的研究表明,尽管再喂养综合征和急性硫胺素缺乏在许多实验室检查方面有相似之处,但应将它们视为不同的病症,在硫胺素缺乏和难治性乳酸酸中毒的再喂养综合征病例中报告的电解质异常可能是由于肾小管丢失,而非从细胞外转移至细胞内。在肿瘤和营养不良患者中,这些患者是发生再喂养综合征的特别高危人群,在出现这些生化异常时,应怀疑并治疗急性硫胺素缺乏,因为其对硫胺素给药反应迅速。