Continuous glucose monitoring (CGM) for effective glucose control in a pregnant woman living with type IIIa glycogenosis. A case report

IF 2.9 Q3 NUTRITION & DIETETICS

Clinical nutrition ESPEN Pub Date : 2024-11-15 DOI:10.1016/j.clnesp.2024.11.010

Jean-Baptiste Bonnet , Martina Fasolo , Lucile Marty , Laëtitia Galibert , Céline Dupy Richard , Ariane Sultan , Vincent Attalin , Antoine Avignon

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引用次数: 0

Abstract

Background & aims

Glycogenosis type III is a rare autosomal recessive disease caused by a mutation in the AGL gene that results in a deficiency of the glycogen debranching enzyme. This deficiency impairs fasting tolerance and leads to hypoglycemia. While the symptomatology tends to improve with age, pregnancy can trigger a recurrence of hypoglycemia due to increased carbohydrate requirements. Since the late 1990s, continuous glucose monitoring (CGM) has been used in insulin-dependent diabetes to aid in the prevention of hypoglycemia. It has also been employed in type I glycogenosis for the same purpose. However, it has never been evaluated for the prevention of hypoglycemia in pregnant women with type III glycogenosis.

Case

report: We present the case of a 32-year-old pregnant woman with type IIIa glycogenosis diagnosed at the age of 2 years old. The resurgence of nocturnal hypoglycemia initially led to the reinstatement of continuous nocturnal nutrition. We established remote glucose monitoring via CGM, facilitating medical and dietary teleconsultations until delivery. These teleconsultations enabled dietary adjustment according to the patient's needs, based on the CGM data. This subsequently led to a reduction in the frequency and duration of nocturnal hypoglycemia, even after discontinuing continuous nutrition. No severe hypoglycemia was observed.

Discussion

Our results suggest that CGM combined with telemonitoring can provide effective support for individuals living with glycogenosis during pregnancy and may eliminate the need for resuming continuous nocturnal enteral nutrition. However, these results need to be confirmed with additional patient cases.

Conclusion

CGM is a safe tool to limit the risk of hypoglycemia and to improve the quality of life of pregnant patients with type III glycogenosis.

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连续血糖监测 (CGM) 可有效控制一名 IIIa 型糖原中毒孕妇的血糖。病例报告。

背景与目的糖原病 III 型是一种罕见的常染色体隐性遗传病，由 AGL 基因突变引起，导致糖原分解酶缺乏。这种缺陷会损害空腹耐受性并导致低血糖。虽然症状会随着年龄的增长而有所改善，但由于对碳水化合物的需求增加，怀孕可能会引发低血糖症复发。自 20 世纪 90 年代末以来，连续血糖监测（CGM）被用于胰岛素依赖型糖尿病，以帮助预防低血糖。I 型糖原中毒症也采用了同样的方法。但是，从未有人评估过它在预防 III 型糖原中毒孕妇低血糖症方面的作用：我们报告了一例 32 岁孕妇的病例，她在 2 岁时被诊断患有 IIIa 型糖原中毒。夜间低血糖的再次出现最初导致了持续夜间营养的恢复。我们通过 CGM 建立了远程血糖监测，为分娩前的医疗和饮食远程会诊提供了便利。通过这些远程会诊，我们可以根据 CGM 数据，按照患者的需求调整饮食。这导致夜间低血糖的发生频率和持续时间减少，即使在停止持续营养后也是如此。没有观察到严重的低血糖：讨论：我们的研究结果表明，CGM 与远程监控相结合可为妊娠期糖原中毒患者提供有效的支持，并可消除恢复持续夜间肠内营养的需要。然而，这些结果还需要更多患者的病例来证实：CGM 是一种安全的工具，可限制低血糖风险并改善 III 型糖原中毒妊娠患者的生活质量。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical nutrition ESPEN NUTRITION & DIETETICS-

CiteScore

4.90

自引率

3.30%

发文量

512

期刊介绍： Clinical Nutrition ESPEN is an electronic-only journal and is an official publication of the European Society for Clinical Nutrition and Metabolism (ESPEN). Nutrition and nutritional care have gained wide clinical and scientific interest during the past decades. The increasing knowledge of metabolic disturbances and nutritional assessment in chronic and acute diseases has stimulated rapid advances in design, development and clinical application of nutritional support. The aims of ESPEN are to encourage the rapid diffusion of knowledge and its application in the field of clinical nutrition and metabolism. Published bimonthly, Clinical Nutrition ESPEN focuses on publishing articles on the relationship between nutrition and disease in the setting of basic science and clinical practice. Clinical Nutrition ESPEN is available to all members of ESPEN and to all subscribers of Clinical Nutrition.