Abstract
Glycogen Storage Disease Type Ia (GSDIa) is an inherited disorder caused by deficiency of glucose-6-phosphatase that results in impaired release of glucose from glycogen, putting patients at risk for acute hypoglycemia. Patients also develop hepatomegaly, nephromegaly, and abnormal/delayed growth. Current disease management requires frequent consumption of raw cornstarch on a strict schedule, sometimes throughout the night. This retrospective cohort study investigated comorbidities in adults with GSDIa.
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