Abstract
Glycogen Storage Disease Type Ia (GSDIa) is a rare metabolic genetic disorder caused by glucose-6-phosphatase deficiency, characterized by fasting hypoglycemia with hyperlactatemia, hepatomegaly, growth failure, and possible seizures and death. Patients are managed by dietary treatment involving frequent and large amounts of uncooked cornstarch during the day and often night. Older patients are at risk of chronic liver, kidney and bone disease, dyslipidemia, hypertension, and anemia. Evidence about the impact of GSDIa on health-related quality of life (HRQoL) is lacking.
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