肠衰竭儿童胫性脚气1例报告。

Q4 Medicine Critical care explorations Pub Date : 2024-12-06 eCollection Date: 2024-12-01 DOI:10.1097/CCE.0000000000001187
Madeleine Böhrer, Paige Burgess, Tais Da Costa Sao Pedro, Dana Liza Boctor, Samantha Boggs
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引用次数: 0

摘要

背景:如果生理需求超过内源性储存和补充,急性疾病状态下代谢需求增加可导致严重的硫胺素缺乏症。先前存在肠外营养依赖、饮食失调、胃肠疾病或手术等危险因素的患者尤其容易受到伤害。病例总结:一名患有短肠综合征的9岁女孩接受长期PN治疗,包括补充标准硫胺素,在误吸后出现难治性休克。尽管体外膜氧合,深度高乳酸血症、心功能障碍和血管活性需求仍持续存在,仅在经肠外硫胺素治疗后才得以解决。结论:由于硫胺素缺乏症(足气病)引起的血液动力学衰竭可能发生在有营养危险因素的儿童急性疾病中,在难治性高乳酸血症中应予以考虑。及时给予肠外硫胺素治疗可支持显著的血流动力学恢复。对疑似硫胺素缺乏症的治疗不应延迟实验室确认,因为目前的硫胺素参考范围与临床严重程度相关性较差,而且功能检测可能不易获得。
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Shoshin Beriberi in a Child With Intestinal Failure: A Case Report.

Background: Acute illness states with increased metabolic demand can precipitate severe thiamine deficiency if physiologic needs exceed endogenous stores and supplementation. Patients with preexisting risk factors such as parenteral nutrition (PN) dependence, eating disorders, gastrointestinal disorders, or surgeries are especially vulnerable.

Case summary: A 9-year-old girl with short bowel syndrome receiving long-term PN, including standard thiamine supplementation, presented with refractory shock following aspiration. Profound hyperlactatemia, cardiac dysfunction, and vasoactive requirements persisted despite extracorporeal membrane oxygenation and resolved only after treatment with parenteral thiamine.

Conclusions: Hemodynamic collapse due to thiamine deficiency (Shoshin beriberi) may occur during acute illness in children with nutritional risk factors and should be considered in refractory hyperlactatemia. Timely treatment with parenteral thiamine can support dramatic hemodynamic recovery. Treatment of suspected thiamine deficiency should not be delayed for laboratory confirmation as current thiamine reference ranges correlate poorly with clinical severity, and functional testing may not be readily available.

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