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Presentation
and Diagnosis
Clinical
Course
Nutrition
Management
Lifelong
Management
Presentation and Diagnosis
Clinical Course
People with GSDIa live with the acute risk of life-threatening hypoglycemic episodes and the ongoing potential for long-term complications.1
Nutrition Management
Management with cornstarch and nutrition can improve glucose homeostasis and metabolic stability.1
Lifelong Management
Frequent blood glucose monitoring, along with lifelong surveillance and management of complications,are essential components of GSDIa care.3
Abbreviation: GSDIa, glycogen storage disease type Ia.
References: 1. Derks TGJ, Rodriguez-Buritica DF, Ahmad A, et al.
Glycogen storage disease type Ia: current management options, burden and unmet need. Nutrients.
2021;13(11):3828. 2. Kishnani PS, Austin SL, Abdenur JE, et al.
Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of
Medical Genetics and Genomics. Genet Med. 2014;16(11):e1. 3.
Bali DS, El-Gharbawy A, Austin S, et al. Glycogen storage disease type I. 2006. In: Adam MP, Feldman J,
Mirzaa GM, et al, eds. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle;
1993-2024. Accessed June 27, 2025. https://www.ncbi.nlm.nih.gov/books/NBK1312/