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Observational Study
. 2024 Sep-Oct;143(1-2):108573.
doi: 10.1016/j.ymgme.2024.108573. Epub 2024 Aug 30.

Continuous glucose monitoring metrics in people with liver glycogen storage disease and idiopathic ketotic hypoglycemia: A single-center, retrospective, observational study

Ruben J Overduin  1 , Annieke Venema  1 , Charlotte M A Lubout  1 , Marieke J Fokkert-Wilts  1 , Foekje De Boer  1 , Andrea B Schreuder  1 , Alessandro Rossi  2 , Terry G J Derks  3
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Free article
Observational Study

Continuous glucose monitoring metrics in people with liver glycogen storage disease and idiopathic ketotic hypoglycemia: A single-center, retrospective, observational study

Ruben J Overduin et al. Mol Genet Metab. 2024 Sep-Oct.
Free article

Abstract

Background: Cohort data on continuous glucose monitoring (CGM) metrics are scarce for liver glycogen storage diseases (GSDs) and idiopathic ketotic hypoglycemia (IKH). The aim of this study was to retrospectively describe CGM metrics for people with liver GSDs and IKH.

Patients and methods: CGM metrics (descriptive, glycemic variation and glycemic control parameters) were calculated for 47 liver GSD and 14 IKH patients, categorized in cohorts by disease subtype, age and treatment status, and compared to published age-matched CGM metrics from healthy individuals. Glycemic control was assessed as time-in-range (TIR; ≥3.9 - ≤7.8 and ≥3.9 - ≤10.0 mmol/L), time-below-range (TBR; <3.0 mmol/L and ≥3.0 - ≤3.9 mmol/L), and time-above-range (TAR; >7.8 and >10.0 mmol/L).

Results: Despite all patients receiving dietary treatment, GSD cohorts displayed significantly different CGM metrics compared to healthy individuals. Decreased TIR together with increased TAR were noted in GSD I, GSD III, and GSD XI (Fanconi-Bickel syndrome) cohorts (all p < 0.05). In addition, all GSD I cohorts showed increased TBR (all p < 0.05). In GSD IV an increased TBR (p < 0.05) and decreased TAR were noted (p < 0.05). In GSD IX only increased TAR was observed (p < 0.05). IKH patient cohorts, both with and without treatment, presented CGM metrics similar to healthy individuals.

Conclusion: Despite dietary treatment, most liver GSD cohorts do not achieve CGM metrics comparable to healthy individuals. International recommendations on the use of CGM and clinical targets for CGM metrics in liver GSD patients are warranted, both for patient care and clinical trials.

Keywords: Continuous glucose monitoring; Glycogen storage disease; Idiopathic ketotic hypoglycemia; Monitoring; Person-centered outcomes.

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Conflict of interest statement

Declaration of competing interest All authors declare that they have no conflicts of interest related to the content of this manuscript. R.J.O.: There are confidentiality agreements with third parties. There is a consultation agreement (with Ultragenyx Pharmaceutical Inc). For all private-public relationships, all contracts are via UMCG Contract Research Desk and all payments are to UMCG. A.V., C.M.A.L.: No conflicts of interest. M.J.F.W., F.D.B., A.B.S.: There are confidentiality agreements with third parties. For all private-public relationships, all contracts are via UMCG Contract Research Desk and all payments are to UMCG. A.R.: There are confidentiality agreements with third parties. In the past 36 months there have been consultation agreements or honoraria for lectures or presentations with Nestlé, Danone and Ultragenyx Pharmaceutical Inc. T.G.J.D.: There are confidentiality agreements with third parties. In the past 36 months, there have been consultation agreements (with Danone, Ultragenyx Pharmaceutical Inc, ModernaTX Inc., and Beam Therapeutics), contracts for financial research support for investigator-initiated research (NCT04311307) and sponsor-initiated research (NCT03517085, NCT03970278, NCT05139316, and NCT05196165), honoraria for lectures or presentations (by MEDTalks, Prelum, and Danone), and participations in a Data Safety Monitoring Board (NCT05095727) and Advisory Boards (Ultragenyx Pharmaceutical Inc, ModernaTX Inc., and Beam Therapeutics). For all private-public relationships, all contracts are via UMCG Contract Research Desk and all payments are to UMCG.

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