Galactosemia- Decreased Glc? - USMLE Forums
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USMLE Step 1 Classic Clues Classic clues seen in clinical case scenarios in USMLE Step 1 questions.


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Old 03-10-2015
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Default Galactosemia- Decreased Glc?

How galactosemia leads to decreased glc synthesis and hypoglycemia?
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Old 11-16-2015
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Default Gal 1-P uridyltransferase deficiency

When a patient has Gal 1-P uridyltransferase deficiency, the substrate Galactose 1-P will accumulate in the liver leading to liver damage, with further hepatic insufficiency, hyperbilirubinemia decrease in gluconeogenesis and hypoglycemia.
The same won't happen in Galactokinase deficiency (another form of galactosemia) because the galactose won't be phosphorylated to Galactose 1-P, so it can return to the blood stream and won't accumulate on the liver.
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Old 11-22-2015
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Quote:
Originally Posted by Dhiegoncx View Post
When a patient has Gal 1-P uridyltransferase deficiency, the substrate Galactose 1-P will accumulate in the liver leading to liver damage, with further hepatic insufficiency, hyperbilirubinemia decrease in gluconeogenesis and hypoglycemia.
The same won't happen in Galactokinase deficiency (another form of galactosemia) because the galactose won't be phosphorylated to Galactose 1-P, so it can return to the blood stream and won't accumulate on the liver.
a agree with you, for remember. Galactose is asugar body if body cant utilize it it can cause hypoglycemia and as Dhiegocx saied its accumulate in liver because 1st enzyme is fine its converted galactose into galactose1P but 2nd enzyme is not working so its cant enter another pathways or it cant get out from hepatocyte then accumulate...accumulate then lead to liver damage. and as we know out Back up at fasting LIVER gluconeogenesis and beta-oxidation cant run so hypoglycemia.
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Old 12-13-2015
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Also as glactose is phoshorylated ....it depletes phosphate groups used for other biochemical processes
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