The next step is usually a carefully monitored fast. Hepatic complications have been serious in some patients. To compensate for the inability of the liver to provide sugar, the total amount of dietary carbohydrate should approximate the hour glucose production rate. It is also a byproduct of purine degradation. The catalytic unit is associated with a calcium binding protein , and three transport proteins T1, T2, T3 that facilitate movement of glucosephosphate G6P , phosphate , and glucose respectively into and out of the enzyme.
Despite hyperlipidemia, atherosclerotic complications are uncommon. This inability to maintain adequate blood glucose levels during fasting results from the combined impairment of both glycogenolysis and gluconeogenesis. It is also a byproduct of purine degradation. Hypoglycemia often occurs within six hours. Cornstarch is an inexpensive way to provide gradually digested glucose. Chronic hypoglycemia produces secondary metabolic adaptations, including chronically low insulin levels and high levels of glucagon and cortisol. Although a liver transplant resulted in the resolution of hypoglycemia it did not however resolve the chronic neutropenia and the risk of infection among patients. Hyperuricemia results from a combination of increased generation and decreased excretion of uric acid , which is generated when increased amounts of G6P are metabolized via the pentose phosphate pathway. In people with GSD I, it may not fall entirely to normal even when normal glucose levels are restored. One tablespoon contains nearly 9 g carbohydrate 36 calories. Fasting hypoglycemia is often the most significant problem in GSD I, and typically the problem that leads to the diagnosis. It may cause clinically significant bleeding, especially epistaxis. Treatment[ edit ] The primary treatment goal is prevention of hypoglycemia and the secondary metabolic derangements by frequent feedings of foods high in glucose or starch which is readily digested to glucose. The lactic acid level rises during fasting as glucose falls. Additional problems reported in adolescents and adults with GSD I have included hyperuricemic gout, pancreatitis, and chronic renal failure. The last step of normal gluconeogenesis, like the last step of glycogenolysis, is the dephosphorylation of G6P by glucosephosphatase to free glucose and PO 4. Genetics[ edit ] Glycogen storage disease type I has an autosomal recessive pattern of inheritance. Normal neuronal and muscle cells do not express glucosephosphatase, so GSD I causes no other neuromuscular effects. Adherence becomes a contentious treatment issue after infancy. This dephosphorylation reaction produces free glucose and free PO 4 anions. The products of these processes are amino acids mainly alanine , free fatty acids , and lactic acid. Infection risk[ edit ] Neutropenia is a manifestation of this disease. Those who survived were stunted in physical growth and delayed in puberty because of chronically low insulin levels. Free fatty acids from triglycerides are converted to ketones , and to acetyl-CoA. The disease is more common in Ashkenazi Jewish populations, people of Mexican, Chinese, and Japanese descent. Triglyceride levels in GSD I can reach several times normal and serve as a clinical index of "metabolic control". A critical blood specimen obtained at the time of hypoglycemia typically reveals a mild metabolic acidosis, high free fatty acids and beta-hydroxybutyrate, very low insulin levels, and high levels of glucagon, cortisol, and growth hormone.
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