Type 0 glycogen storage disease (GSD 0) is affecting glycogen synthase in the liver by slowing down glycogenesis. In general, GSD 0 develops in the early childhood causing symptoms, such as fatigue, vomiting, and looking pale. The medical doctor recommends frequent meals/snacks every 3-4 hours, keto diet based on fat rich meal and avoid fasting. Please explain why this treatment could be efficient for GSD 0. State your answer in full sentence related to the glucose concentration in blood plasma.
Type 0 Glycogen Storage Disease (GSD 0) is an inherent disorder that is caused by a deficiency of glycogen synthase. glycogen synthase is an enzyme that is essential for the body to glycogen.
Our body requires glucose for energy production but low-sugar or hypoglycemic state, your body starts producing ketones from dietary and stored fat as a source for the production of energy. Glycogen storage disease type 0 (GSD-0 is also accompanied by ketosis as the ketone levels in the blood may increase (ketosis).
Ketones converted to ATP through a process called the Krebs cycle, but ketones don’t increase your blood sugar levels and are not stored as glycogen. Hence hypoglycemia is one of the symptoms of a Type 0 Glycogen Storage Disease (GSD 0), it is an condition that is caused by a very low level of blood sugar (glucose) in the blood stream.
Hence keto- diet is recommended by the doctors for the Type 0 Glycogen Storage Disease (GSD 0). The ketogenic diet contains a high-fat, low-carb nutritional that convert fat for energy requirement and it is effective in treating glycogen storage disease.
Type 0 glycogen storage disease (GSD 0) is affecting glycogen synthase in the liver by slowing...