A Prospective Study on Continuous Glucose Monitoring in Glycogen Storage Disease Type Ia: Toward Glycemic Targets

Author:

Rossi Alessandro12,Venema Annieke1,Haarsma Petra1,Feldbrugge Lude3,Burghard Rob3,Rodriguez-Buritica David4,Parenti Giancarlo25,Oosterveer Maaike H6,Derks Terry G J1ORCID

Affiliation:

1. Section of Metabolic Diseases, Beatrix Children’s Hospital, University of Groningen, University Medical Centre Groningen , Groningen , The Netherlands

2. Department of Translational Medicine, Section of Pediatrics, University of Naples “Federico II,” Naples , Italy

3. enerGQ BV , Groningen , The Netherlands

4. Division of Medical Genetics, Department of Pediatrics, McGovern Medical School, The University of Texas Health Science Center at Houston , Houston, TX, USA

5. Telethon Institute of Genetics and Medicine , Pozzuoli , Italy

6. Department of Pediatrics, University of Groningen, University Medical Center Groningen , Groningen , The Netherlands

Abstract

Abstract Context Although previous research has shown the benefit of continuous glucose monitoring (CGM) for hepatic glycogen storage diseases (GSDs), current lack of prospectively collected CGM metrics and glycemic targets for CGM-derived outcomes in the hepatic GSD population limits its use. Objective To assess CGM metrics for glycemic variation and glycemic control in adult patients with GSDIa as compared to matched healthy volunteers. Design Prospective CGM data were collected during the ENGLUPRO GSDIa trial (NCT04311307) in which a Dexcom G6 device was used. Ten adult patients with GSDIa and 10 age-, sex- and body mass index–matched healthy volunteers were enrolled. Capillary blood glucose was concurrently measured during 2 standardized 2-hour time intervals. Descriptive [eg, glycemic variability (GV), time below range, time in range (TIR), time above range (TAR)] and advanced (ie, first- and second-order derivatives, Fourier analysis) CGM outcomes were calculated. For each descriptive CGM outcome measure, 95% CIs were computed in patients with GSDIa and healthy volunteers, respectively. Results CGM overestimation was higher under preprandial and level 1 hypoglycemia (ie, capillary glucose values ≥ 3.0 mmol/L and < 3.9 mmol/L) conditions. GV and TAR were higher while TIR was lower in patients with GSDIa compared to healthy volunteers (P < 0.05). Three patients with GSDIa showed descriptive CGM outcomes outside the calculated 95% CI in GSDIa patients. Advanced CGM analysis revealed a distinct pattern (ie, first- and second-order derivatives and glucose curve amplitude) in each of these 3 patients within the patients group. Conclusions This is the first study to prospectively compare CGM outcomes between adult patients with GSDIa and matched healthy volunteers. The generation of a set of CGM metrics will provide guidance in using and interpreting CGM data in GSDIa and will be useful for the definition of glycemic targets for CGM in patients with GSDIa. Future studies should investigate the prognostic value of CGM outcomes and their major determinants in patients with GSDIa.

Funder

Ultragenyx Pharmaceutical Inc.

Associazione Italiana Glicogenosi

Publisher

The Endocrine Society

Subject

Biochemistry (medical),Clinical Biochemistry,Endocrinology,Biochemistry,Endocrinology, Diabetes and Metabolism

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