The application of continuous glucose monitoring in the management of hepatic glycogen storage disease

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.