Elias Isaac M, Sinclair Graham, Blydt-Hansen Tom D
Multi Organ Transplant Program, BC Children's Hospital, Vancouver, British Columbia, Canada.
Department of Pathology and Laboratory Medicine, BC Children's Hospital, Vancouver, British Columbia, Canada.
Pediatr Transplant. 2019 Aug;23(5):e13493. doi: 10.1111/petr.13493. Epub 2019 May 24.
Pediatric kidney transplant surgery is usually well tolerated, despite suboptimal physical conditioning that may result from uremia and nutritional deficiencies that accompany end-stage kidney failure. Nutritional supplementation is used to overcome such deficiencies, especially for children needing dialysis. Thiamine, a water-soluble vitamin also known as vitamin B1, is a critical cofactor in energy metabolism and may be competitively inhibited by the antimetabolite oxythiamine, a uremic toxin that accumulates in kidney failure. We report a case of a thiamine deficiency syndrome leading to overwhelming cardiac dysfunction, metabolic instability, and hemodynamic compromise, after otherwise uneventful kidney transplant surgery. Prior to transplant, this 14-year-old boy was treated with peritoneal dialysis and received thiamine supplementation. Post-transplant, the patient first developed hyperglycemia, then lactic acidosis, and subsequently hemodynamic instability despite escalating treatment with volume resuscitation and inotropic medication. He made a rapid and complete recovery after administration of IV thiamine. This is the first reported case of Shoshin beriberi syndrome in a pediatric kidney transplant recipient. Inadequate dialysis may have been a key factor, with toxin accumulation and thiamine transporter downregulation contributing to his status. Functional thiamine deficiency should be considered as a potential treatable cause of early post-transplant hemodynamic instability.
小儿肾移植手术通常耐受性良好，尽管终末期肾衰竭伴随的尿毒症和营养缺乏可能导致身体状况欠佳。营养补充用于克服此类缺乏，特别是对于需要透析的儿童。硫胺素是一种水溶性维生素，也称为维生素B1，是能量代谢中的关键辅助因子，可能会被抗代谢物氧硫胺素竞争性抑制，氧硫胺素是一种在肾衰竭时积累的尿毒症毒素。我们报告了一例在肾移植手术过程顺利的情况下，硫胺素缺乏综合征导致严重心脏功能障碍、代谢不稳定和血流动力学受损的病例。移植前，这名14岁男孩接受了腹膜透析并补充了硫胺素。移植后，尽管通过容量复苏和使用强心药物进行了强化治疗，患者首先出现高血糖，然后是乳酸酸中毒，随后出现血流动力学不稳定。静脉注射硫胺素后，他迅速完全康复。这是小儿肾移植受者中首例报道的干性脚气病综合征病例。透析不足可能是一个关键因素，毒素积累和硫胺素转运体下调导致了他的病情。功能性硫胺素缺乏应被视为移植后早期血流动力学不稳定的潜在可治疗原因。
