目的 探讨消化内镜围术期主动血糖监测对Ⅰb型糖原贮积病（glycogen storage disease type Ⅰb, GSD-Ⅰb）低血糖发作的预防作用。 方法 回顾性总结2021年6月—2024年8月在广东省人民医院诊治的GSD-Ⅰb患儿的临床资料，分析消化内镜围术期主动血糖监测对患儿低血糖发作的影响。 结果 14例GSD-Ⅰb患儿中，男女各7例，入组中位年龄10.0岁。34例次住院记录中发生低血糖15次（44%），其中非主动血糖监测下出现症状发作6例次（血糖1.6 mmol/L 1例次，血糖<1.1 mmol/L 5例次），主动血糖监测下无症状性低血糖9例次（血糖1.2~3.9 mmol/L）。诱因包括术前禁食5例次（33%），延迟进食7例次（47%），呕吐2例次（13%），家长疏忽1例次（7%）。2例患儿在同一次住院期间各出现2次低血糖。所有患儿在低血糖发作前均无明显自觉症状。治疗方式包括鼻饲葡萄糖1例次（7%），静脉推注葡萄糖14例次（93%）及静脉滴注葡萄糖4例次（27%）。干预后10 min血糖恢复至3.5~6.9 mmol/L，恢复饮食后血糖正常。 结论 消化内镜围术期主动血糖监测有利于及早发现GSD-Ⅰb患儿的低血糖状态，预防低血糖发作及进行精准诊疗。

Objective To investigate the role of active glucose monitoring in preventing hypoglycemia during the perioperative period of gastrointestinal endoscopy in children with glycogen storage disease type Ⅰb (GSD-Ⅰb). Methods A retrospective analysis was performed for the clinical data of children with GSD-Ⅰb who were diagnosed and treated in Guangdong Provincial People's Hospital from June 2021 to August 2024. The effect of active glucose monitoring on hypoglycemic episodes during the perioperative period of gastrointestinal endoscopy was analyzed. Results A total of 14 children with GSD-Ⅰb were included, among whom there were 7 boys and 7 girls, with a mean age of 10.0 years. Among 34 hospitalizations, there were 15 cases of hypoglycemic episodes (44%), among which 6 symptomatic cases (1 case with blood glucose level of 1.6 mmol/L and 5 cases with blood glucose level of <1.1 mmol/L) occurred without active monitoring, while 9 asymptomatic cases (with blood glucose level of 1.2-3.9 mmol/L) were detected by active monitoring. The predisposing factors for hypoglycemic episodes included preoperative fasting (5 cases, 33%), delayed feeding (7 cases, 47%), vomiting (2 cases, 13%), and parental omission (1 case, 7%). Two children experienced two hypoglycemic episodes during the same period of hospitalization, and no child experienced subjective symptoms prior to hypoglycemic episodes. Treatment methods included nasogastric glucose administration (1 case, 7%), intravenous injection of glucose (14 cases, 93%), and continuous glucose infusion (4 cases, 27%). Blood glucose returned to 3.5-6.9 mmol/L within 10 minutes after intervention and remained normal after dietary resumption. Conclusions Active glucose monitoring during the perioperative period of gastrointestinal endoscopy can help to achieve early detection of hypoglycemic states in children with GSD-Ⅰb, prevent hypoglycemic episodes, and enhance precise diagnosis and treatment.