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Title: [The application of continuous glucose monitoring in the management of hepatic glycogen storage disease].
Author: Du CQ, Wei H, Zhang C, Zhang M, Hu MH, Hou L, Wu W, Liang Y, Luo XP.
Journal: Zhonghua Er Ke Za Zhi; 2021 Jun 02; 59(6):452-458. PubMed ID: 34102817.
Abstract:
Objective: Continuous glucose monitoring (CGM) were performed in children with hepatic glycogen storage disease (GSD) to accurately understand the situation of glucose levels during their treatment, and to provide support for optimizing their nutritional management. Methods: In this retrospective research, 42 patients with hepatic GSD who under went 72 h CGM were collected from Department of Pediatrics, Tongji Hospital of Tongji Medical College, Huazhong University of Science and Technology from October 2019 to January 2020. According to the genetic test results, they were divided into 5 groups: type Ⅰa, type Ⅰb, type Ⅲa, type Ⅵ and type Ⅸa. After long-term follow up and regular treatment, the clinical data (induding course, age, height, weight and biochemical parameters, etc.) on the day of CGM were summarized, and 72 h CGM were performed to assess the occurrence of hypoglycemia and hyperglycemia.χ² test, Fisher exact probability method, t test, analysis of variance or nonparametric test were used for comparison between groups. Results: Forty-two cases of hepatic GSD patients included 25 males and 17 females (20 cases of type Ⅰa, 3 cases of type Ⅰb, 10 cases of type Ⅲa, 3 cases of type Ⅵ and 6 cases of type Ⅸa).The age was 9.5 (6.7, 12.9) years, and the course of disease was 6.8 (5.1, 11.3) years. The average levels of glucose of the patients were all normal. However, the levels of standard deviation of blood glucose (SDBG) and mean amplitude of glycemic excursion (MAGE) were significantly different (F=2.747, 3.029,both P<0.05). Among them, the SDBG of type Ⅰa and Ⅲa were significantly higher than those of type Ⅸa ((1.10±0.36), (0.98±0.30) vs. (0.62±0.26) mmol/L, t=3.010, 2.440, both P<0.05), while the MAGE of type Ⅰ was higher than that of Ⅸa and Ⅲa ((2.3±0.9) mmol/L vs. (1.2±0.6) and (1.7±0.6) mmol/L, t=2.734, 2.302, both P<0.05, respectively). Conclusions: CGMS can accurately assess the fluctuations of blood glucose and effectively detect hidden hypoglycemia and hyperglycemia in hepatic GSD patients. For different types of hepatic GSD, individualized corn starch treatment doses should be given according to the different situation of blood glucose, so as to optimize the patient's treatment and improve their prognosis. 目的： 动态血糖监测（CGM）肝糖原累积病（GSD）患儿生玉米淀粉治疗过程中的血糖变化情况，为优化患儿的营养治疗提供支持。 方法： 回顾性研究。收集2019年10月至2020年1月于华中科技大学同济医学院附属同济医院儿科进行72 h CGM的42例肝GSD患儿，按照基因检测结果分为GSDⅠa、Ⅰb、Ⅲa、Ⅵ和Ⅸa型共5组，总结患儿规律治疗后行CGM当天的临床资料，包括病程、年龄、身高、体重、血生化检测结果等，采用动态血糖监测系统（CGMS）对肝GSD患儿进行72 h CGM，评估不同类型肝GSD患儿全天低血糖和高血糖发生情况。采用χ²检验、Fisher确切概率法、t检验、方差分析或非参数检验法进行组间比较。 结果： 42例肝GSD患儿中男25例、女17例。年龄为9.5（6.7， 12.9）岁，病程为6.8（5.1， 11.3）年。GSDⅠa型组20例、Ⅰb型组3例、Ⅲa型组10例、Ⅵ型组3例和Ⅸa型组6例。CGM结果提示患儿的平均血糖值均在正常范围，但5组间血糖水平标准差（SDBG）和平均血糖波动幅度（MAGE）差异均有统计学意义（F=2.747、3.029，均P<0.05），其中Ⅰa型、Ⅲa型的SDBG明显高于Ⅸa型［（1.10±0.36）、（0.98±0.30）比（0.62±0.26） mmol/L，t=3.010、2.440，均P<0.05］，Ⅰa型的MAGE明显高于Ⅸa型和Ⅲa型［（2.3±0.9）比（1.2±0.6）和（1.7±0.6） mmol/L，t=2.734，2.302，均P<0.05］。 结论： CGMS可正确评估患儿的血糖波动情况，有效发现患儿治疗过程中隐匿的低血糖和高血糖。针对不同类型的肝GSD应根据自身血糖情况给予个体化生玉米淀粉治疗剂量，从而优化患儿的治疗方案改善预后。.

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