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1 mar 2020 · 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. Dietary interventions have markedly improved the outcome for these disorders, from a previously fatal con …
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).
11 maj 2023 · The role of dietary lipid manipulations in the nutritional management of GSD III is still debated. A literature overview shows that low-carbohydrate (CHO) / high-fat diets may be beneficial in reducing muscle damage.
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.
7 wrz 2023 · Introduction. Glycogen storage diseases (GSDs) are a group of disorders characterized by a biochemical deficit in glycogenesis (glycogen synthesis), glycogenolysis (breakdown of glycogen to...
Core Messages for GSD Type I. Glycogen storage disease type I is characterized by fasting hypoglycemia and elevated lactic acid, uric acid and triglycerides. Patients with GSD type Ib are also at risk of neutropenia and inflammatory bowel disease.
1 dzień temu · Pompe disease, or glycogen storage disease type II, is a lysosomal storage disorder and a metabolic myopathy caused by deficiency of lysosomal acid alpha-glucosidase (GAA, also referred to as acid maltase). ... The rationale for a high-protein diet is to counteract muscle protein depletion by supplying increased amino acid substrates for ...
