ECE2017 Eposter Presentations: Diabetes, Obesity and Metabolism Clinical case reports - Thyroid/Others (16 abstracts)
Continuous glucose monitoring in glycogen storage disease type Ia – a major improvement for patients
Diana Martins 1 , Diana Oliveira 1 , Carla Baptista 1 , Sandra Paiva 1 , Nuno Vicente 1 , Luís Cardoso 1 , Adriana Lages 1 , Mara Ventura 1 , Nelson Cunha 1 , Sónia Moreira 2 , Hugo Clemente 2 , Helder Esperto 2 & Francisco Carrilho 1
1Department of Endocrinology, Diabetes and Metabolism of Coimbra Hospital and Universitary Centre, Coimbra, Portugal; 2Department of Internal Medicine of Coimbra Hospital and Universitary Centre, Coimbra, Portugal.
Introduction: Glycogen storage disease (GSD) type Ia (von Gierke’s disease) is an inherited metabolic disorder of glycogen metabolism, caused by defects in the glucose-6-phosphatase complex, with associated risk of severe hypoglycemia within 3–4 h after a meal.
The aim of the present study was to evaluate the efficacy of continuous glucose monitoring (CGM) system in determining the magnitude of hypoglycemia in patients with GSD type Ia.
Methods: We retrospectively analyzed data from three patients with GSD type Ia, who underwent CGM with iProTM2 CGM device (Medtronic, Northridge, CA), over a 7.0 days period, under real life conditions.
Results: A total of 5.520 glucose data points were analysed. Concerning to low glycemic excursions (<70 mg/dl), we identified 15 episodes (three of them below 55 mg/dl and reaching 40 mg/dl in one case). Most of the episodes of hypoglycaemia were asymptomatic and occurred 3 or more hours after meals or during the nocturnal period. The individual CGM analysis revealed a mean area under the curve of blood glucose below 70 mg/dl (AUC<70) respectively of 0.1, 0.5 and 0.8. One patient presented with important glycemic variability: MAGE 3.11 (reference range 0.0–2.8).
Conclusions: The analysis of continuous glucose monitoring data revealed significant periods of asymptomatic hypoglycemia and provided better perception into glycemic variations therefore, it may serve as a safe and useful tool for the long-term management of patients with GSD type Ia. We suggest that CGM can be used as an instrument to detect hypoglycemia, especially in the nocturnal period, helping patients with GSD to modify their nutritional regimens if necessary.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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