Term
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Definition
| pink staining amorphous mass made up of cytokeratin with ubiquitin in the cytoplasm of hepatocytes. Highly associated with EtOH injury to hepatocytes. |
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Term
| What can happen to fatty acids once they are delivered to the liver? |
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Definition
| they can be made into 1) ketone bodies via oxidation 2) phospholipids 3) cholesterol esters 4) triglycerides via esterification |
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Term
| Increased accumulation of triglycerides within hepatocytes might be due to three broad categories. Name them. |
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Definition
| Increased delivery of FA (DM, EtoH), decreased oxidation of FA (hypoxia, EtOH, toxins), reduced apoprotein availability (CCl4, malnutrition) |
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Term
| How does EtOH cause a fatty liver? |
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Definition
| increases supply of FFA, increases esterification of FFA to triglycerides and decreases oxidation of FFA |
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Term
| How do toxins cause a fatty liver? |
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Definition
| reduced apoprotein availability and decreased oxidation of FFA |
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Term
| Name two diseases that are characterized by microvesicular fat in the liver. |
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Definition
| Reye's syndrome and fatty liver of pregnancy |
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Term
| How do phagocytic cells end up with large amounts of cholesterol in their cytoplasm? |
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Definition
| They phagocytose cells that have cholesterol in their cell membranes. They are unable to break down the cholesterol so it builds up |
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Term
| Which cells can metabolize cholesterol? |
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Definition
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Term
| What to cells of the kidney when proteins leak across the glomerular basement membrane? What does it look like histologically? |
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Definition
| tubular epithelial cells absorb it faster that they can metabolize/export it. Looks like pink droplets called protein resorption droplets. |
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Term
| What is alpha 1 antitrypsin (A1AT) disease? |
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Definition
| when you make an an abnormally folded form of A1AT that can't be exported out of the hepatocyte ER and so your lung is eaten up by bacterial elastase |
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Term
| What happens to the iron in RBCs when they die? |
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Definition
| RBCs are metabolized by the reticulo-endothelial system. Then iron is first bound to ferritin within the phagocytic cells and then transported via transferrin in the blood to the liver where it is stored inside the hepatocytes bound to ferritin |
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Term
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Definition
| brown intracellular iron-containing pigment that builds up when small amounts of ferritin undergo autophagy (or partial digestion) |
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Term
| How does the body get rid of excess iron? |
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Definition
| It doesn't. There is no physiologic echanism to rid the body of excess iron. Control of total body iron stores lies in the area of absorption from the gut |
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Term
| How is buildup of iron in cells harmful? |
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Definition
| causes lipid peroxidation through iron catalyzed free radical formation. Iron directly interacts with and damages DNA (resulting in death or mutation), and stimulates collagen synthesis |
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Term
| How do you differentiate hemosiderin from other brown pigments? |
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Definition
| you can stain the tissue with Prussian blue stain that will turn hemosiderin bright blue. |
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Term
| What are two types of diseases that can lead to increase iron in cells? |
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Definition
| blood diseases involving ineffective hematopoesis or requiring numerous transfusions, and genetic hemochromatosis |
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Term
| What is the inheritance pattern of hemochromatosis? |
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Definition
|
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Term
| What happens to the excess iron taken up from the GI in a disease like hemochromatosis? |
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Definition
| deposited in parenchymal organs such as the liver, pancreas, and skin |
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Term
| How does the body get rid of copper? |
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Definition
| through the biliary system, so chronic biliary obstruction can lead to excess accumulation of copper within hepatocytes |
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Term
| What is the inheritance pattern of Wilson's Disease? |
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Definition
|
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Term
| What is Wilson's disease? |
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Definition
| a genetic inability to excrete copper from hepatocyte into canaliculi resulting in accumulation of copper in liver and brain (and also cornea, kidneys, bones, and joints) |
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Term
| What is the mechanism by which Wilson's disease causes anemia? |
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Definition
| excess copper builds up in hepatocytes and is reguritated back into the circulatory system where it can cause hemolysis of RBCs |
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Term
| What is the most common exogenous pigment? |
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Definition
|
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Term
| What is anthrocotic pigment made up of? |
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Definition
|
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Term
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Definition
| alveolar macrophages that have phagocytosed carbon particles |
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Term
| How does the carbon you breathe in end up in you lymph nodes? |
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Definition
| carbon particles move into connective tissue then lymphatics then to lymph nodes and stay there forever |
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Term
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Definition
| colored pigments is injected into teh skin, are engulfed by macrophages in the connective tissue and remain in place |
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Term
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Definition
| a yellow-green breakdown product of the heme moiety of hemoglobin and heme-containing enzymes. Causes jaundice |
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Term
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Definition
| the wear and tear pigment that is yellow-brown and is the final product of free radical damage and cross linking of membrane lipids |
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Term
| In what cells is lipofuscin prominent? |
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Definition
| aging myocardial cells and hepatocytes and in the cells of patients with severe malnutrition or cachexia |
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Term
| What is dystrophic calcification? |
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Definition
| condition in which calcium is deposited on necrotic or chronically traumatized tissue. Always local in distribution and in the presence of normal serum calcium levels |
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Term
| Name some common examples of dystrophic calcification. |
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Definition
| happens in areas of fat necrosis, foci of coagulation or caseous necrosis, artherosclerotic vessel, and heart valves (especially aortic) |
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Term
| Why does calcium deposit on damaged tissue? |
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Definition
| terminally damaged mitochondria form calcific densities. Also, the exposed phospholipids of damaged cells may act as a template allowing the orderly binding of calcium and phosphate |
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Term
| What is metastatic calcification? |
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Definition
| condition in which, due to high serum calcium, deposition of calcium salts ocurs in viable or living tissue |
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Term
| Name some principle causes of hypercalcemia. |
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Definition
| hypersecretion of PTH, destruction of bone tissue, poisoning with vitamin D, milk-alkali syndrome, uremia and dialysis |
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Term
| What might cause hypersecretion of PTH with increase bone resorption? |
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Definition
| primary hyperparathyroidism (parathyroid hyperplasia or tumor), secondary to renal failure, and ectopic secretion of PTH by malignant tumor |
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Term
| What might cause destruction of bone tissue(besides PTH)? |
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Definition
| primary tumor of bone marrow, diffuse skeletal metastases, immobilization |
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Term
| Although metastatic calcification may affect any tissue or organ, it tends to affect tissues that... |
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Definition
| may lose acid and therefore generate an internal alkaline milieu. Ex: gastric mucosa, kidneys, lungs, systemic arteries, and pulmonary vein |
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Term
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Definition
| an abnormal proteinaceous deposit in the extracellular space with unique light microscopic features and a characteristic ultrastructure |
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Term
| What is the characteristic ultrastructure of amyloids? What is the characteristic light microscope appearance? |
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Definition
| anti parallel beta pleated sheet with N and C terminals oriented in oposite directions; glassy pink/hyaline with H&E and red with Congo Red |
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Term
| What are the mechanisms that cause amyloids? |
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Definition
| mutation of a gene, protein's nature is to form amyloid after a long time or when in high concentration, abnormal metabolism (proteolysis), or a combo of all of those |
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Term
| Under polarized light, amyloids stained with congo red are what color? |
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Definition
|
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Term
| How many different proteins have been associated with amyloid deposits? |
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Definition
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Term
| AL amyloidosis is also called ________. |
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Definition
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Term
| Amyloid deposits of AL amyloidosis are formed by _____________. |
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Definition
| abnormal immunoglobulin light chains, usually of the lambda type (associated with multiple myeloma) |
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Term
| What is Bence-Jones protein? |
|
Definition
| protein in the urine that is due to excess immunoglobulin in the blood due to multiple myeloma |
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Term
| Amyloid deposition in the heart results in... |
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Definition
| a stiffened less contractile myocardium called a restrictive cardiomyopathy |
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Term
| Amyloid deposits in the kidney results in... |
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Definition
| disruption of the normal filtration membrane allowing serum proteins to leak into urine |
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Term
| AA amyloidosis is sometiems called ___________. |
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Definition
|
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Term
| What causes AA amyloidosis? |
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Definition
| excess serum AA (sAA) protein which is an acute phase reactant secreted by the liver in high concentrations due to chronic inflammatory disorders. THey can be abnormally processed by macrophages and eposited into tissues as amyloid. |
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Term
| Name a few chronic infections that cause AA amyloidosis. |
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Definition
| chronic tuberculosis infection, chronic osteomyelitis, chronic skin infections as in drug users, chronic autoimmune disorders (rheumatoid arthritis, crohn's disease), and certain malignant tumors (medullary carcinoma of the thyroid and renal cell carcinoma) |
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Term
| What do you call the inherited form of AA amyloidosis? |
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Definition
| Familial Mediterranean Fever |
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Term
| In what ethnicities is Familial Mediterranean Fever common? |
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Definition
| Armenian, Turkish, Sephardic Jewish and Arabic |
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Term
| What is the mechanism of Familial Mediterranean Fever? |
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Definition
| recurrent bouts of fever and inflammation of serosal surfaces of unknown origin (but associated with mutations in teh MEFV gene) that result in continual high levels of sAA protein |
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Term
| What is the function of the MEFV gene? |
|
Definition
| down regulator of inflammation within the polymorphonuclear lymphocytes |
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Term
| What is A"beta2"microglobulin amyloidosis also called? |
|
Definition
| dialysis associated amyloidosis |
|
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Term
| What is the mechanism of dialysis associated amyloidosis? |
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Definition
| Pts on chronic dialysis maintain high levels of beta2-microglobulin because dialysis can't remove it. These are abnormally processed or have an intrinsic propensity to form amyloids that deposit in the joints, resulting in a destructive arthropathy |
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Term
| What is the name of the amyloid protein associated with Alzheimer's disease? Where does it deposit? |
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Definition
| beta-amyloid; cerebral plaques and blood vessels |
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Term
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Definition
| adj that refers to a homogeneous glassy pink appearance |
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Term
| What causes hyaline arteriosclerosis? |
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Definition
| when HTN causes slow leakage of plasma proteins through damaged endothelium into the vessel wall where they become lodged |
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Term
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Definition
| noun. the substance itselfthat has a hyaline appearance |
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Term
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Definition
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Term
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Definition
| Congo red staining of amyloid |
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Term
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Definition
| Amyloid depositions in kidney |
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Term
|
Definition
| Hemosiderin in liver cells |
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Term
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Definition
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Term
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Definition
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Term
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Definition
| lipofuscin in cardiac myocytes |
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Term
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Definition
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Term
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Definition
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