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4 Cards in this Set
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Glycogenoses:
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Group of glycogen storage disorders due to a hereditary enzyme defects
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1) Type I:
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glycogenosis with liver and kidney enlargement.
a. Pathogenesis: A glucose-6-phosphatase defect Diagnosis with liver biopsy!. |
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2) Type II
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hereditary, generalized lysosomal glycogenosis with muscle weakness.
a. Pathogenesis: A defect in the acidic a(1,4)-glucosidase in lysosomes of the hepatic, cardiac, and skeletal muscle cells, and in the CNS and lymphocytes, blocks the lysosomal breakdown of glycogen, which accumulates in the lysosomes, creating spider-like glycogen storage cells and cellular damage. Biopsy from muscles! |
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3) Type III
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: glycogenesis with cardiomegaly, hepatomegaly, and muscle weakness.
a. Pathogenesis: An amylo-1,6-glucosidase (debranching enzyme) defect in cardiac and skeletal muscles cells, hepatocytes, and enterocytes. |
