ILOs of the fourth lecture

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Presentation transcript:

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?

Amyloidosis

AMYLOIDOSIS Oid amyl starch Osis process similar Protein (amyloid protein) + mucopolysaccharrhides Interstitial tissues +wall of blood vessels Pressure atrophy May lead to organ failure

Microscopic appearance CONGO RED + Flourescent M CONGO RED EM

CONGO RED + POLRIZED LIGHT MICROSCOPY

Physical nature of amyloid protein

Chemical nature of amyloid protein -95% Fibril protein -5% P component Amyloid light chain Amyloid associated protein Transthyretin beta2 microglobulin Beta amyloid protein Others

Pathogenesis of Alzheimer’s disease

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

Secondary amyloidosis Kidney, liver, spleen, LNs, adrenal Tuberculosis, Bronchiectasis, Rheumatoid arthritis Renal cell carcinoma Excess amyloid associated protein+ defect enzymatic degredation

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

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

Generalized amyloidosis poor prognosis Diagnosis of Amyloidosis Clinical symptoms and signs Microscopic Congo red stain Rectal and gingival biopsies Generalized amyloidosis poor prognosis