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9 Cards in this Set
- Front
- Back
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Which inherited liver disease only manifests in ADULTS
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Hemochromatosis
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Pathophysiology of Hemochromatosis
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HEPCIDIN -- HFE "sensor" on the liver does not tell the liver to increase hepcidin production when iron HIGH. So iron absorption never shut down. Extra iron deposited in the liver
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2 places where hepcidin works
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Macrophages to stop release from breaking down the RBC, entoeryctes to stop release from absorption
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Inheritance pattern of hemochromatosis
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autosomal recessive
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Gene involved in MOST cases of hemochromatosis
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HFE cystine --> tyrosine at 282
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Triad of hemochromatosis
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Cirrhosis, diabetes, skin pigmentation = "bronzed diabetes"
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Manifestations of hemochromatosis in OTHER organs
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Congestive heart failure, testicular atrophy (hypogonadism)
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Risk of hepatocellular carcinoma with hemochromatosis?
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YES!`
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Labratory findings in hemochromatosis
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Increased: ferritin, iron, trasferrin saturation. Decreased: total iron binding capacity
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