Alkaptonuria: homogentisic acid builds up in the cartilage.
Homogentisic acid: an intermediate by product of phynlalanine metabolism which builds up in cartilage when enzymes are not present to convert it to the next metabolite in the pathway.
REMEMBER: Alkaptonuria is the only disease characterized by calcification of the IVD's!
Which condition is characterized by a build up of homogentistic acid in the IVD cartilage?
Ochronosis of the vertebral column.
Patient's urine becomes black after being in oxygen for 40 minutes.
Patient may also present with blue ears.
In regards to alkaptonuria, which enzyme is missing to change phyenlalanine into tyrosine?
Phenylketonuria... so phenylalanine is converted to ketones which builds up in the blood, as phenylpyruvic acid, causing brain damage. These children cannot talk or walk.
Name 1 disease resulting from an accumulation of metabolic by-products.
Gout: results from hyperuricemia, due to the inability for the kidneys to remove uric acid.
Which type of cellular injury results in a loss of structural integrity?
Trauma.
Ex). Direct contact, hypothermia, hyperthermia, mechanical pressure, & microorganisms.
What is an example of both traumatic cell injury and an endogenous toxin?
Ionizing radiation.
Name 3 examples of mechanical pressure.
1. Tumor.
2. Aneurysm.
3. High intensity sound.
Name the 4 types of reversible changes.
1. Hydropic change.
2. Steatosis.
3. Residual bodies.
4. Hyalinization.
Following cellular injury, the cell is unable to produce enough ATP, resulting in cloudy swelling. What type of change is this?
Hydropic change.
Also known as Hydropic Degeneration, or Cloudy Degeneration.
Which reversible (functional) change results in the accumulation of triacylglycerides?
Steatosis (fatty change): results in the cellular contents being moved to the periphery of the cell.
Alcoholism results in a fatty liver.
Can also happen in the kidneys, skeletal muscle, and heart.
Cause by mechanical trauma.
What are the 5 causes of steatosis?
1. Intoxication.
2. Hypoxia (anoxia).
3. Protein deficiency.
4. Obesity.
5. Diabetes.
Which reversible functional change results in lipofuscin granules?
Residual bodies: fragments of bacteria or cellular organelles found in an injured cell.
What are lipofuscin (lipocrone) granules?
Indigestible aging pigments also known as brown atrophy.
(They are traces of damaged cellular membranes).
Which pathology occurs in males & results in damage to the liver & pancreas?
Hemochromatosis: due to too much iron in the body.
True or false: Wilson's Disease is a result of too much iron in the body?
False: Wilson's Disease (Hepatolenticular Degeneration): endogenous pigment accumulation of copper.
Affects the liver first, followed by the brain.
Which reversible change results in an accumulation of glass like proteins?
Hyalinization (hyaline change).
Name the 4 examples of intracellular hyaline changes.
1. Reabsorption droplets: in the proximal tubules of the kidneys, causes proteinuria.
2. Dutcher bodies: intraNUCLEAR inclusions.
3. Russell bodies: intraCYTOPLASMIC inclusions of immunoglobulins in patients with Lymphoplasmacytic Lymphoma.
4. Mallory bodies: intracytoplasmic inclusions that accumulate in the hepatocytes in alcoholics.
True or false: russell bodies are intracytoplasmic inclusions.
Huge production of antibodies (proteins) due to the B-cell lymphoma... remember that B-cells create antibodies.
Results in hyperviscosity syndrome.
Dutcher & Russell bodies are found in patients that have which 2 diseases?
Lymphoplasmacytic lymphoma or multiple myeloma.
Name 2 examples of extracellular hyaline changes.
1. Hyalinization in arterioles.
2. Amyloidosis.
What disease is characterized by inadequate neurological stimulation?
Poliomyelitis: no neuronal stimulation to muscles due to viral damage on the ant. horn of the spinal cord.
What is the most important sign of irreversible cell damage?
Destruction of the nucleus.
Which irreversible change results in a dense nucleus?
Pyknosis: the nucleus shrinks and becomes too dense.
Which irreversible change results in fragmentation of the nucleus?
Karorrhexis: fragmentation of nucleus.
Which irreversible change results in melting of the nucleus?
Karyolysis: melting of the nucleus.
Which type of necrosis preserves a basic outline allowing the tissue to avoid damage?
Coagulative necrosis.
Ex) myocardial infarction.
Which type of necrosis typically occurs in nervous tissue?
Liquefactive necrosis: complete digestion of the dead cells, transforming the tissue into a liquid viscous mass.
Nervous tissue because of little or no collagen fibers.
Which type of necrosis has the appearance of melted cheese?
Casous Necrosis: amorphous granular debris composed of fragmented, coagulated cells, and the granulomatous reaction border.
What are 2 examples of caseous necrosis?
1. TB.
2. Syphillis.
Which necrosis develops only with syphillis?
Gummatous necrosis: if not an answer choice, pick liquefactive.
Which type of necrosis is described by a severe glassy or waxy necrosis of skeletal muscles?
Zenker's necrosis: especially with typhoid fever.
Necrosis of adipose tissue, characterized by formation of small quantities of calcium soaps describes which type of necrosis?
Steatonecrosis (fat necrosis): associated with pancreonecrosis.
Which type of necrosis is associated with immune problems?
Fibroid necrosis: autoimmune... occurs in walls of blood vessels.
Aschoff nodes in heart muscle following rheumatic fever.
Which type of necrosis is characterized by the presence of noxious products?
Gangrenous necrosis.
Anaerobic bacterial metabolism.
Which type of gangrene is very similar to coagulative necrosis?
Dry gangrene: coagulation is sustained.
Which type of necrosis is characterized by phagoctic cells breaking down necrotic debris, producing liquefaction?
Wet gangrene.
Polyarteritis nodosa: wet gangrene of the blood vessel lining, due to auto immune disease.
Which type of calcification may be associated with hypercalcemia?
Metastatic calcification.
Increased secretion of parathyroid hormones.
Destruction of bone.
Paget's Disease: accelerated turnover.
Vitamin D related disorder & Sarcoidosis.
Renal failure.
Which type of necrosis results in deposition of calcium into necrotic tissue?
Dystrophic calcification.
Calcifies Gohn's Focus.
Mestastatic calcification is characterized by increased secretion of parathyroid hormones, destruction of bone, Vit D disorders, & what else?
Renal failure.
True or false? Ischemia means lack of oxygen supply to tissue?
False: BLOOD supply.
Which organs are most susceptible to ischemia?
1. Brain.
2. Heart.
3. Kidneys.
4. Spleen.
5. Lung.
Ionizing radiation is susceptible to tissue with a low mitosis rate. True or false?
False: HIGH mitosis rate.
Which tissues are most susceptible to ionizing radiation?
Bone marrow, hair follicles, reproductive cells, & membranes of the GI-Tract.
Are viruses intra or extraceullar?
Intracellular.
Where does inflammation take place?
In the capillary & the post capillary venule.
Also the only place where there is an exchange between intersitial fluid & blood.
If the skin is cut, the vessels immediately dialate. True or false?
False: vasoCONSTRICTion.
...Quickly followed by dilation to inflame the area (hyperemia).
Exudate is composed of what?
Proteins, WBC's.
Following damage to vessels, there is a disruption of axial blood flow. The RBC's clump together & migrate towards the center, which makes the WBC's move to the periphery. What is this called?
Margination.
When there is damage, the endothelial cells become sticky & adhere the leukocytes to the endotheial cell surface. What is this process called?
Pavementing.
What is the name of the process when leukocytes crawl through a gap between the endothelial cells?
Leukocyte emeigration.
Takes place ONLY @ the post. cap. venules.
What is the mildest form of inflammation?
Serous inflammation: only fluid is allowed to escape to the interstitial tissue.
Ex) Second degree burn.
What is an example of fibrinous inflammation?
Pericarditis: will cause bruits.
Which type of inflammation results in pus formation?
Suppurative (purulent) inflammation: acute type of exudative inflammation where WBC's liquefactive enzymes.
Diapedesis is the active escape of RBC's from the vessels. True or false?
False: PASSIVE escape.
Name the 3 examples of PolyMorphoNuclear cells/ Granulocytes.
What is the name of the natural anti-biotic used with oxygen-independent phagocytosis?
Defensin.
Name 3 substances which the oxygen-dependent mechanisms uses.
1. Superoxide anion.
2. Hypocholrite anion.
3. Hyrogen peroxide.
Name the genetic disease in which phagocytic cells are unable to produce free radicals, resulting in death by 2 years of age.
Chronic Granulomatous Disease of Children: unable to produce oxygenated free radicals. Microbes are unable to be torn down, and the body forms a granuloma to try to contain them. The child is unable to kill off diseases.
What is the name of a genetic disease in which degranulation cannot occur, resulting in inability to digest foreign substances?
Chediak-Highashi Syndrome: dismotility of phagocytes, resulting in inability to prevent degranulation.
Which type of cell initiates inflammation?
Mast cells.
Name the 4 cell derived mediators of inflammation.
1. Histamine.
2. Arachidonic acid.
3. Serotonin: very similar to histamine.
4. Substance P: neuropeptide, increased permeability of vessels.
Name the 4 functions of the vasoactive amine, histamine.
1. Vasodialation.
2. Increased permeability of blood vessels.
3. Bronchospasm.
4. Increased mucous production.
From which pathway are thromboxanes & prostoglandins produced?
Cyclooxygenase.
From which pathway are leukotrienes formed?
Lipoxygenase pathway.
Which substance is responsible for vasodilation & increased permeability?
Prostoglandins.
Which substance is responsible for chemotaxis, vasoconstriction, & increased permeability?
Leukotrienes.
Which substance is responsible for vasoconstriction & platelet aggregation?
Thromboxanes.
Which cell is the major source of histamine in the body?
Mast cells.
What cell is the major source for serotonin?
Platelets: serotonin is very similar to histamine.
Which clotting factor is responsible for activating 4 different cascades as a result of injury?
Clotting factor XII (Hageman Factor).
Which 4 cascades does the Hageman Factor activate?
1. Coagulation cascade: pvrevents blood loss.
2. Fibrinolytic cascade: prevents coagulation from spreading over body.
3. Complement cascade: immunity.
4. Kinin cascade: inflammation.
What is the name of the independent mediator of inflammation?
Antibody: plasma derived, but not associated with any of the cascades.
What is characterized by more than 5,000-9,000 WBC's in 1 cubic mm of blood?
Leukocytosis.
Neutrophillia indicates what 3 things?
Neutrophillia: >65% neutrophils, indicative of bacterial infection, appendicitis, colitis.
Lymphocytosis is indicative of what?
Viral infection.
Eosinophillia is indicative of what?
Allergic reaction or parasites.
Monocytosis is indicative of what?
Chronic baterial infection.
With acute inflammation there is a lot of exudate (swelling). Which 2 cells are present?
1. Neutrophils.
2. Macrophages.
How much exudate is present with chronic inflammation? Which cells are present?
Very little exudate.
Lympocytes & monocytes (macrophages) are present.
Which type of reaction is associated with Chronic Granulomatous Inflammation?
Delayed type 4 hyersensitivity reaction.
TB & leprosy.
Granuloma (formed by macrophages) prevents spreading.
Define regeneration.
Regeneration: healing process in which tissue is replaced from parenchyma.
Function will be regained.
Define repair (Fibroplasia, Fibrosis).
Repair: healing process when the fibroblasts lay down fibrous CT, resulting in a scar.
Restores strength & structural integrity.
Function is lost.
Occurs when regeneration cannot occur.
Labile tissues are characterized by rapid shifting cell, which divide continually to replace cells that are constantly being deleted by normal processes. Name some examples of labile tissues.
What is the term used to describe pinpoint hemorrhages?
Petechiae.
What is the term for a small, but not the smallest hemorrhage?
Purpura.
Bruises are hemorrhages larger than 2cm, which are associated with which syndrome?
Bruises (Ecchymoses): associated with Cushing's Syndrome due to excessive amounts of corticosteroids in the body, resulting in brittle blood vessels.
Define thrombocytopenia.
Thrombocytopenia: lack of platelets (thrombocytes) resulting in the formation of various hemorrhages.
Generally petechiae or purpura.
Clotting factors are produced where? And are under the control of with which vitamin?
Produced in liver.
Under the control of Vitamin K.
True or False: Hemophillia is a genetic disease associated with production of clotting factors.
FALSE: non-production of clotting factors.
What is the name of the disease characterized by an autosomal dominant inheritance, which results in ecchymosis & hematomas commonly affecting the stomach & intestines.
Von Willebrand Disease: one or more parents must have disease in order for child to get it.
Women also have metrorrhagia: leaking of blood from the uterus, not during menses.
"Thrombocytopenia."
Classic hemophellia is due to the non-production of which clotting factor?
Classic Hemophellia (Hemophellia A): non-production of clotting factor VIII.
Genetic disease.
Christmas Disease results from non-production of which clotting factor?
Christmas Disease (Hemophellia B): non-production of clotting factor IX (Christmas Factor).
Genetic disease.
Which type of hematoma develops only in joints?
Hemarthrosis.
Patient has either Hemopheilia A or B.
Males only.
Parents do not have the disease.
Is a blood clot the same things as a thrombus?
No. Blood clots can form outside & inside, but thrombi can only form within a blood vessel.
What are the 4 components of a thrombus?
1. Fibrin.
2. Platelets.
3. WBC's.
4. RBC's.
What are lines of zahn?
Lines of zahn: layers of fibrin with trapped blood cell layers & layers of almost pure platelets on top of each other.
Name the 2 risk factors for thrombosis.
1. Hemodynamic stress.
2. Hypertension.
Atherosclerosis affects smaller arteries. True or false?
False. Atherosclerosis affects larger arteries.
What is the normal ratio for LDL's:HDL's?
4:1.
Homocysteine leads to the destruction of which layer?
Intimal layer of endothelial cells.
What is the most important factor resulting in thrombosis?
Reduction in speed of blood flow: via cardiac failure, increased blood viscosity, polycythemia.
