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Continuous glucose monitoring in children with glycogen storage disease type I
E Hershkovitz1, A Rachmel, H Ben-Zaken
1Pediatric Endocrine and Metabolic Unit, Soroka Medical University Center, Beer Sheva, Israel. elih@bgumail.bgu.ac.il
Insights
Continuous glucose monitoring effectively detects asymptomatic hypoglycemia in Glycogen storage disease type I (GSD I). This technology aids in assessing long-term dietary management for GSD I patients.
Area of Science:
- Metabolic disorders
- Pediatric endocrinology
- Medical device technology
Background:
- Glycogen storage disease type I (GSD I) impairs glucose production, leading to severe fasting hypoglycemia.
- Effective management of GSD I requires precise monitoring of glucose levels.
- Continuous subcutaneous glucose monitoring (CGMS) offers a potential tool for enhanced glucose surveillance.
Observation:
- Four children with GSD I were monitored over 72 hours using a CGMS MiniMed system.
- CGMS data demonstrated a strong correlation with traditional glucometer readings.
- The study identified significant, often asymptomatic, hypoglycemic episodes, particularly during nighttime.
Findings:
- CGMS accurately captures glucose fluctuations in GSD I patients.
- Asymptomatic hypoglycemia is a prevalent issue in GSD I, especially overnight.
- The study validates CGMS as a reliable method for assessing glycemic control.
Implications:
- Continuous glucose monitoring can significantly improve the assessment of GSD I management.
- This technology allows for timely dietary adjustments to prevent severe hypoglycemic events.
- Repeated CGMS use may enhance long-term outcomes for individuals with GSD I.
Abstract:
Glycogen storage disease type I (GSD I) is characterized by impaired production of glucose from glycogenolysis and gluconeogenesis resulting in severe fasting hypoglycaemia. The aim of the present study was to examine the efficacy of a continuous subcutaneous glucose monitoring system (CGMS MiniMed), to determine the magnitude and significance of hypoglycaemia in GSD I and to evaluate the efficacy of its dietary treatment. Four children with GSD I were studied over a 72-h period. Results indicated that the values recorded with continuous subcutaneous glucose monitoring were highly correlated with paired blood glucose values measured by glucometer. Significant periods of asymptomatic hypoglycaemia were noted, especially during night-time. The study suggests that repeated continuous subcutaneous glucose monitoring may serve as a useful tool for the assessment of the long-term management of GSD I patients.