A retrospective in-depth analysis of continuous glucose monitoring datasets for patients with hepatic glycogen storage disease: Recommended outcome parameters for glucose management

Fabian Peeks, Irene J Hoogeveen, R Lude Feldbrugge, Rob Burghard, Foekje de Boer, Marieke J Fokkert-Wilts, Melanie M van der Klauw, Maaike H Oosterveer, Terry G J Derks*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Continuous glucose monitoring (CGM) systems have great potential for real-time assessment of glycemic variation in patients with hepatic glycogen storage disease (GSD). However, detailed descriptions and in-depth analysis of CGM data from hepatic GSD patients during interventions are scarce. This is a retrospective in-depth analysis of CGM parameters, acquired in a continuous, real-time fashion describing glucose management in 15 individual GSD patients. CGM subsets are obtained both in-hospital and at home, upon nocturnal dietary intervention (n = 1), starch loads (n = 11) and treatment of GSD Ib patients with empagliflozin (n = 3). Descriptive CGM parameters, and parameters reflecting glycemic variation and glycemic control are considered useful CGM outcome parameters. Furthermore, the combination of first and second order derivatives, cumulative sum and Fourier analysis identified both subtle and sudden changes in glucose management; hence, aiding assessment of dietary and medical interventions. CGM data interpolation for nocturnal intervals reduced confounding by physical activity and diet. Based on these analyses, we conclude that in-depth CGM analysis can be a powerful tool to assess glucose management and optimize treatment in individual hepatic GSD patients.

Original languageEnglish
Pages (from-to)1136-1150
Number of pages15
JournalJournal of Inherited Metabolic Disease
Issue number5
Publication statusPublished - Sept-2021


  • continuous glucose monitoring
  • diabetes mellitus
  • empagliflozin
  • Fourier analysis
  • glycogen storage disease
  • person&#8208
  • centered outcomes

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