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Acute Shoshin beriberi syndrome immediately post–kidney transplant with rapid recovery after thiamine administration

机译:急性Shoshin Beriberi综合征立即肾脏移植后硫胺素管理后快速恢复

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Abstract 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.
机译:摘要小儿肾移植手术通常是耐受性的,尽管尿毒症和营养缺陷可能导致患有末期肾功能衰竭的尿毒症和营养缺乏。营养补充用于克服这种缺陷,特别是针对需要透析的儿童。硫胺素,一种也称为维生素B1的水溶性维生素是能量代谢的关键辅因子,并且可以通过抗体氨基钛羟胺,一种血糖毒素竞争抑制,这是一种肾脏衰竭。我们报告了硫胺素缺乏综合征,导致心脏功能障碍,代谢不稳定性和血液动力学妥协,否则在否则肾脏移植手术后。在移植之前,这位14岁的男孩被腹膜透析治疗并接受了硫胺素补充剂。移植后,患者首先发育高血糖,然后乳酸中毒,随后血液动力学不稳定尽管用体积复苏和尿液药物的治疗不断升级。他在硫胺素施用后迅速恢复了。这是第一个报告的儿科肾移植受体中的Shoshin Beriberi综合征。透析不足可能是一个关键因素,毒素积累和硫胺素转运仪下调有助于他的地位。功能性硫胺素缺乏应被视为早期移植后期血液动力学不稳定的潜在可治疗原因。

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