Severe perioperative lactic acidosis in a pediatric patient with glycogen storage disease type Ia: a case report.

IF 0.8 Q3 ANESTHESIOLOGY JA Clinical Reports Pub Date : 2023-12-20 DOI:10.1186/s40981-023-00683-z

Tamayo Takahashi, Kana Oue, Eiji Imado, Mitsuru Doi, Yoshitaka Shimizu, Mitsuhiro Yoshida

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Abstract

Background: Glycogen storage disease (GSD) is a group of rare inherited metabolic disorders caused by enzyme deficiencies in glycogen catabolism. GSD type Ia is a congenital deficiency of the enzyme responsible for the final step in glucose production by glycolysis, resulting in impaired carbohydrate metabolism.

Case presentation: A 14-year-old boy with GSD type Ia was scheduled for a maxillary cystectomy under general anesthesia. He was taking oral sugars such as uncooked cornstarch regularly to prevent hypoglycemia. Perioperatively, glucose was administered via the peripheral vein for fasting; however, severe lactic acidosis occurred. He also developed hypercapnia because of intraoperative poor ventilation caused by hepatomegaly.

Conclusions: We experienced a child with GSD type Ia who developed severe lactic acidosis despite continuous glucose infusion. Further studies are required to determine appropriate perioperative management for patients with GSD, including fasting glucose administration.

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糖原贮积症 Ia 型儿科患者围手术期严重乳酸酸中毒：病例报告。

背景：糖原贮积病（GSD）是一组罕见的遗传性代谢性疾病，由糖原分解酶缺乏引起。GSD Ia 型是一种先天性酶缺乏症，这种酶负责糖酵解产生葡萄糖的最后一步，从而导致碳水化合物代谢障碍：一名患有 GSD Ia 型的 14 岁男孩计划在全身麻醉下进行上颌膀胱切除术。他定期口服糖类，如未煮熟的玉米淀粉，以预防低血糖症。围手术期，他通过外周静脉注射葡萄糖以禁食，但发生了严重的乳酸酸中毒。由于肝脏肿大导致术中通气不良，他还出现了高碳酸血症：结论：我们遇到过一名 GSD Ia 型患儿，尽管持续输注葡萄糖，但还是出现了严重的乳酸酸中毒。需要进一步研究来确定 GSD 患者围手术期的适当管理，包括空腹葡萄糖管理。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

JA Clinical Reports ANESTHESIOLOGY-

CiteScore

1.30

自引率

0.00%

发文量

审稿时长

9 weeks

期刊介绍： JA Clinical Reports is a companion journal to the Journal of Anesthesia (JA), the official journal of the Japanese Society of Anesthesiologists (JSA). This journal is an open access, peer-reviewed, online journal related to clinical anesthesia practices such as anesthesia management, pain management and intensive care. Case reports are very important articles from the viewpoint of education and the cultivation of scientific thinking in the field of anesthesia. However, submissions of anesthesia research and clinical reports from Japan are notably decreasing in major anesthesia journals. Therefore, the JSA has decided to launch a new journal, JA Clinical Reports, to encourage JSA members, particularly junior Japanese anesthesiologists, to publish papers in English language.