Evaluation of continuous glucose monitoring and nutritional status in glycogen storage diseases

Burcu Kumru Akin; Emine Goksoy

doi:10.1515/jpem-2024-0597

Article

Evaluation of continuous glucose monitoring and nutritional status in glycogen storage diseases

Burcu Kumru Akin and Emine Goksoy

Published/Copyright: March 10, 2025

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From the journal Journal of Pediatric Endocrinology and Metabolism Volume 38 Issue 5

Abstract

Objectives

The primary goal in managing glycogen storage disorders (GSD) is to implement dietary therapy through regular glucose monitoring while attempting to prevent complications. Self-monitoring of blood glucose is often insufficient for detecting asymptomatic hypoglycemia in patients. Therefore, Continuous glucose monitoring systems (CGMS) play a crucial role in identifying hypoglycemic episodes and providing detailed glucose profiles throughout the day. In this study, CGMS data, laboratory findings, and daily nutritional intake were examined in patients with GSDIa and GSDIII. The lack of similar studies in GSDIII patients in the literature highlights the need for further research in this field.

Methods

The glucose profiles of 12 patients (7 GSDIa and 5 GSDIII) were analyzed over a 72 h period using CGMS. Nutritional intake, biochemical parameters, and growth parameters were also evaluated.

Results

This study demonstrated that CGMS detected both hypoglycemia (<70 mg/dL) and hyperglycemia (>150 mg/dL) in GSD patients. Growth retardation was also observed in these patients. As complications of the disease, elevated levels of liver enzymes, cholesterol, triglycerides, and creatine kinase were identified, with fatty liver and hepatomegaly detected in all patients. The patients’ nutritional intake is similar to the recommendations in disease-specific treatment guidelines.

Conclusions

The primary dietary treatment goal for GSD patients is to maintain normoglycemia. Patients may experience asymptomatic low glucose and/or asymptomatic hypoglycemic episodes during treatment. CGMS enables a more detailed monitoring of glucose profiles, which not only facilitates the precise adjustment of dietary therapy based on detailed results but also helps prevent complications associated with the disease.

Keywords: glycogen storage disorders; continuous glucose monitoring; diet therapy

Corresponding author: Burcu Kumru Akin, RD, PhD, Division of Nutrition and Diet, Gaziantep Cengiz Gökçek Maternity and Children’s Hospital, Osmangazi Street Cengiz Gökçek Maternity and Children’s Hospital, Sehitkamil, Gaziantep 27060, Türkiye, E-mail: burcu-kumru@hotmail.com

Research ethics: This study was conducted in accordance with the Declaration of Helsinki. The study protocol was approved by the Ethics Committee of Gaziantep University.
Informed consent: Written informed consent was obtained from the patients’ parents or care givers.
Author contributions: Burcu Kumru Akin: Writing – original draft, Writing – review & editing, Supervision, Methodology, Investigation, Formal analysis. Emine Goksoy: Writing – review & editing, Methodology, Investigation, Formal analysis. The authors have accepted responsibility for the entire content of this manuscript and approved its submission.
Use of Large Language Models, AI and Machine Learning Tools: None declared.
Conflict of interest: The authors state no conflict of interest.
Research funding: None declared.
Data availability: Not applicable.

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Received: 2024-12-11

Accepted: 2025-02-14

Published Online: 2025-03-10

Published in Print: 2025-05-26

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Articles in the same Issue

https://doi.org/10.1515/jpem-2024-0597

Keywords for this article

glycogen storage disorders; continuous glucose monitoring; diet therapy