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13 Cards in this Set
- Front
- Back
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Type I
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von Gierke's
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Type II
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Pompe's
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Type III
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Cori's
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Type IV
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Andersen's
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Type V
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McArdle's
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Type VI
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Hers'
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von Gierke's
Deficiency and consequence (4) |
G6Phosphatase deficiency
Hypoglycemia (liver can't send out free glc) hepatomegaly lactic acidemia hyperlipidemia |
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Pompe's
Deficiency and consequence (2) |
lysosomal α-glucosidase deficiency- supposed to be cleaving α1,4 bonds. Located in lysosome for unclear reasons.
glycogen granules in lysosome cardiomegaly |
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Cori's
Deficiency and consequence (2) |
debranching enzyme deficiency
hypoglycemia histologically glycogen structure stubby |
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Andersen's
Deficiency and consequence (2) |
branching enzyme deficiency
glycogen looks like starch Not as soluble -> precipitates -> cirrhosis heart and liver -> die by 5 years |
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McArdle's
Deficiency and consequence |
muscle phosphorylase deficiency
excess *muscle* glycogen deposition *exercise-induced* cramps and fatigue (epinephrine pathway doesn't work and ATP runs low) |
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Hers'
Deficiency and consequence |
liver phosphorylase deficiency
very mild hypoglycemia (not as bad as von Gierke's; gluconeogenesis derived free glc can be released) hepatomegaly |
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What are appropriate blood [glc] measurements in different units?
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A normal fasting blood glucose target range for an individual without diabetes is 70-100 mg/dL (3.9-5.6 mmol/L).
The mean normal blood glucose level in humans is about 5.5 mM (5.5 mmol/L or 100 mg/dL, i.e. milligrams/deciliter). |
