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18 Cards in this Set
- Front
- Back
What is the HMP shunt used for?
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Provides source of NADPH from abundantly available G6P
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Where does the HMP shunt occur?
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Lactating mamillary glands, liver, adrenal cortex, RBC's
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What is the respiratory burst used for?
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Immune response --> rapid release of reactive oxygen intermediates
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How do WBC's protect themselves from the components of the respiratory burst?
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Converts hydrogen peroxide to water
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What is necessary for glutathione to detoxotify free radicals and peroxides?
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NADPH
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What happens to RBC's with decreased NADPH?
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Leads to hemolytic anemia due to poor RBC defense against oxidizing agents
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What are heinz bodies?
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Oxidized hemoglobin precipitated within RBC's
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What are Bite cells?
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Result from phagocytic removal of heinz bodies by splenic macrophages
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What is the pathology of essential fructosuria?
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Involves a defect in fructokinase
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What are the symptoms of essential fructosuria?
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Fructose in blood an d urine
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What is the pathology of fructose intolerance?
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Hereditary deficiency of Aldolase B
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What are the symptoms of fructose intolerance?
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Fructose-1-P accumulates, causing a decrease in available phosphate
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How do you treat fructose intolerance?
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Dec intake of both fructose and sucrose
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What is the pathology of galactokinase deficiency?
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Hereditary deficiency of galactokinase
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What are the symptoms of galactokinase deficiency?
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Galactose appears in blood and urine, infantile cataracts
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What is the pathology of galactosemia?
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Absence of galactose-1-phosphate uridyltransferase
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What are the symptoms of galactosemia?
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Damage cause by accumulation of toxic substances which accumulate in the lens of the eye
Failure to thrive, jaundice, hepatomegaly, infantile cataracts, mental retardation |
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How do you treat galactosemia?
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Exclude galactose and lactose from diet
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