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Metabolic Liver Disease (Iron Metabolism (Dietary ion is Fe3+ and it is…
Metabolic Liver Disease
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Iron Metabolism
Dietary ion is Fe3+ and it is reduced by low gastric PH and vitamin C to Fe2+ which is better absorbed
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Red meats, liver, seafoods, cereals, pulses are iron rich
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Hepcidin is a protein made by hepatocytes that regulates gut iron absorption and haem iron recycling by macrophages
Erythropoeisis requires 5-30mg of iron a day - this is provided by macrophages which recycle haem from old RBCs
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Defects in triggering hepcidin is a rare cause of haemochromatosis, unrelated to HFE mutations
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Wilson's Disease
Definition
This is a rare inherited disorder of biliary copper excretion, with excess deposition in the liver and CNS
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Aetiology
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Caused by a mutation in the ATP7B gene on chromosome 13 which codes for the copper transporting ATPase
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Signs
Mood: depression, libido fluctuation, personality change
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Young adults: tremor, dysarthria, dementia, ataxia, Parkinsonism
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Children: hepatitis, cirrhosis, fulminant liver disease
Investigations
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Liver biopsy: increased hepatic copper, cirrhosis
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Treatment
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Diet: avoid liver, chocolate, nuts, mushrooms, shellfish