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6 mar 2019 · The hepatic glycogen storage diseases (GSDs) are a group of disorders where abnormal storage or release of glycogen leads to potentially life-threatening hypoglycemia and metabolic disturbances.
11 maj 2023 · The patient's diet, evaluated through a 7-day food record, is reported in Table 2. Calorie intake was quite high (3132 Kcal/day) mainly due to an elevated intake of CHO (513 g/day, 61% of Total Energy Intake).
1 mar 2020 · Dietary interventions have markedly improved the outcome for these disorders, from a previously fatal condition to one where people can do well with proper care. This article chronicles the evolution of dietary management and treatment of the hepatic GSDs (types 0, I, III, VI, IX, and XI).
The hepatic glycogen storage diseases (GSDs) are a group of disorders where abnormal storage or release of glycogen leads to potentially life-threatening hypoglycemia and metabolic disturbances.
Glycogen is a stored form of sugar in the body. As a result of the inability to breakdown glycogen, patients with GSD are at risk for low blood sugars (hypoglycemia) during periods of fasting (i.e. between meals). The following is a recommended general nutrition guideline for those with GSDI to help maximize blood sugar and lactic acid control, ...
Nutrition management of GSD type I includes providing supplemental uncooked cornstarch as a source of glucose, avoidance of dietary galactose and fructose, and a moderate restriction of fat. Vitamin and mineral supplementation are needed to prevent deficiencies.
7 wrz 2023 · In some hepatic GSDs that have an intact gluconeogenic pathway, a high-protein diet can be helpful in providing an alternative source of glucose via glucogenic amino acids, minimizing glycogen...
