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

IF 3.7 2区 生物学 Q2 ENDOCRINOLOGY & METABOLISM Molecular genetics and metabolism Pub Date : 2024-09-01 DOI:10.1016/j.ymgme.2024.108573
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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.

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肝糖原贮积症和特发性酮症性低血糖患者的连续血糖监测指标:一项单中心、回顾性、观察性研究。
背景:肝糖原贮积症(GSD)和特发性酮症性低血糖(IKH)的连续血糖监测(CGM)指标的队列数据很少。本研究旨在回顾性描述肝糖原贮积症和特发性酮症低血糖症患者的 CGM 指标:对 47 名肝脏 GSD 患者和 14 名 IKH 患者的 CGM 指标(描述性、血糖变化和血糖控制参数)进行了计算,并按疾病亚型、年龄和治疗状态进行了分组,与已发表的健康人的年龄匹配 CGM 指标进行了比较。评估血糖控制情况的标准是血糖在量程内的时间(TIR;≥3.9 -≤7.8 和 ≥3.9 -≤10.0 mmol/L)、血糖在量程外的时间(TBR;7.8 和 >10.0 mmol/L):结果:尽管所有患者都接受了饮食治疗,但与健康人相比,GSD 组群的 CGM 指标有显著差异。在 GSD I、GSD III 和 GSD XI(范康尼-比克尔综合征)队列中,TIR 降低,TAR 升高(均为 p):尽管进行了饮食治疗,但大多数肝脏 GSD 队列的 CGM 指标仍无法与健康人相媲美。有必要就肝脏 GSD 患者 CGM 的使用和 CGM 指标的临床目标提出国际建议,这既适用于患者护理,也适用于临床试验。
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来源期刊
Molecular genetics and metabolism
Molecular genetics and metabolism 生物-生化与分子生物学
CiteScore
5.90
自引率
7.90%
发文量
621
审稿时长
34 days
期刊介绍: Molecular Genetics and Metabolism contributes to the understanding of the metabolic and molecular basis of disease. This peer reviewed journal publishes articles describing investigations that use the tools of biochemical genetics and molecular genetics for studies of normal and disease states in humans and animal models.
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