Case Report: Glycogen Storage Disease Type IIIa

Abstract

Glycogen storage disease (GSD), is caused by a defect in any of multiple enzymes involved in the synthesis or utilization of glycogen. GSD is autosomal recessive disorder. Different subtypes of the diseases have been defined as related to their location on the metabolic pathway. Hypoglycemia is the cardinal manifestation of the hepatic glycogenoses due to inability to utilize glycogen as a source of glucose in a fasting state. Medical nutrition therapy (MNT) is more effective for better metabolic control in GSD Type I, III and VI than other subtypes. The goal of the medical nutrition therapy is to provide normoglycemia, normal growth and development. We discussed a medical nutrition approach of a child with GSD IIIa.