Nutritional support in glycogen storage diseases: evolution of treatment and unresolved contradictions

Abstract

Hepatic forms of glycogen storage diseases (GSD) are a group of diseases in which abnormal accumulation or cleavage of glycogen leads to potentially life-threatening hypoglycemic conditions and metabolic disorders. The use of specialized diet therapy with the addition of raw corn starch has significantly improved the results of GSD treatment, turning a pathology previously considered fatal into a condition in which people can feel satisfactory with proper care. Despite many years of research, there is no consensus on the optimal dietary treatment of GSD. This article describes the evolution of dietary therapy of hepatic forms of GSD (types 0, I, III, VI, IX and XI), presents historical and modern approaches to the prevention of hypoglycemia and related complications.