A 5 year old child has massive liver enlargement and severe persistent hypoglycemia and ketosis. which of the following disease classes should be suspected?
A. Diabetes B. Glycogen storage disease
C. Lysosomal storage disorder
D. Mucopolysaccharidoses
E. Urea Cycle defect
glycogen storage disease * Impairment of glycogenolysis also causes the characteristic enlargement of the liver due to accumulation of glycogen * The hypoglycemia of GSD I is termed "fasting", or "post-absorptive", meaning that it occurs after completion of digestion of a meal-- usually about 4 hours later. This inability to maintain adequate blood glucose levels during fasting results from the combined impairment of both glycogenolysis and gluconeogenesis.
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glycogen storage disease
* Impairment of glycogenolysis also causes the characteristic enlargement of the liver due to accumulation of glycogen
* The hypoglycemia of GSD I is termed "fasting", or "post-absorptive", meaning that it occurs after completion of digestion of a meal-- usually about 4 hours later. This inability to maintain adequate blood glucose levels during fasting results from the combined impairment of both glycogenolysis and gluconeogenesis.
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