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ILOs of the fourth lecture
-What is amyloidosis? -What is the chemical nature of amyloid protein? -What is the physical nature of amyloid protein? -What are the types of amyloidosis? -What is the gross, microscopic picture of amyloidosis? -What are the investigations of amyloidosis?
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Amyloidosis
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AMYLOIDOSIS Oid amyl starch Osis process similar
Protein (amyloid protein) + mucopolysaccharrhides Interstitial tissues +wall of blood vessels Pressure atrophy May lead to organ failure
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Microscopic appearance
CONGO RED + Flourescent M CONGO RED EM
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CONGO RED + POLRIZED LIGHT MICROSCOPY
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Physical nature of amyloid protein
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Chemical nature of amyloid protein
-95% Fibril protein -5% P component Amyloid light chain Amyloid associated protein Transthyretin beta2 microglobulin Beta amyloid protein Others
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Pathogenesis of Alzheimer’s disease
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Primary amyloidosis Kidney, liver, spleen
Multiple myeloma Just increase plasma cells in bone marrow Heart, skin, respiratory Tract, peripheral nerves, tongue Excess kappa and lambda light chains (Bence Jones proteins) excreted in the urine + Defect in enzymatic degradation Kidney, liver, spleen
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Secondary amyloidosis
Kidney, liver, spleen, LNs, adrenal Tuberculosis, Bronchiectasis, Rheumatoid arthritis Renal cell carcinoma Excess amyloid associated protein+ defect enzymatic degredation
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Gross Microscopic Electron microscope non branching fibrils Size
enlarged Surface flat Colour brown Consistency Firm waxy Edge sharp Microscopic Homogenous eosinophilic material in Blood vessel wall + interstitial tissue Special stain Congo red +polarized light apple green Electron microscope non branching fibrils
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Examples of organs affection Clinical presentation
Kidney nephrotic syndrome renal failure Liver Hepatomegaly Spleen sago spleen or diffuse spleen HHeart restrictive cardiomyopathy Others , macroglssia, malabsorption, carpal tunnel syndrome
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Generalized amyloidosis poor prognosis
Diagnosis of Amyloidosis Clinical symptoms and signs Microscopic Congo red stain Rectal and gingival biopsies Generalized amyloidosis poor prognosis
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