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This Concept Map, created with IHMC CmapTools, has information related to: 11.29 Fructose Intolerance.cmap, Sweating consistent with Hereditary Fructose Intolerance, glycogen synthase contributes to accumulation of liver glycogen, Glycolysis when increased consistent with GSD Ib, defective intracellular transport of G-6-P substrate results in accumulation of liver glycogen, Aldolase B Enzyme Deficiency preventing normal cleavage of Fructose-1-phosphate, Glycolysis counteracting liver's efforts to increase blood glucose levels (low in fasting state), Dietary history which revealed Ingestion of solid foods after 6 months, increased levels of G-6-P that can't be dephosphorylated contributing to reduced levels of free hepatic phosphate, seizure suggesting enzyme deficiency, Infant #1: 7 months old presents with Abnormal Sleepiness, accumulation of free Fructose contributing to hyperuricemia, deficiency of myophosphorylase prevents removal of 1,4-glucosyl groups from glycogen, Infant #2: 7 months old presents with Sweating, Infant #2: 7 months old presents with Vomiting, Dihydroxyacetone Phosphate resulting in activation of (but less than normal healthy infant) Glycolysis, hypoglycemia consistent with Serum Glucose: 20 mg/dL, Gluconeogenesis counteracting liver's efforts to increase blood glucose levels (low in fasting state), Deficient in Glucose-6-Phosphatase leading to increased levels of G-6-P that can't be dephosphorylated, reduced levels of free hepatic phosphate inhibits ATP synthase, defective hepatic glucose-6-phosphatase results in accumulation of liver glycogen