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Medical Pathology and Genetics
PPT 9: Hemodynamics and BV's
182
Pathology
Graduate
02/21/2012

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Term
Normal fluid homeostasis requires appropriate: what three things?
Definition
-Vessel wall integrity
-Intravascular pressure
-Plasma colloid osmotic (oncotic) pressure
Term
Define edema.
Definition
Increased fluid in interstitial tissue spaces
Term
What are the two types of edema? describe them.
Definition
-Transudate: protein-poor fluid, typically caused by alterations in hemodynamic forces (SG <1.012)

-Exudate: protein-rich fluid, typically caused by inflammation and the associated increased vascular permeability (SG > 1.020)
Term
What are the 5 causes of edema: what type of increased pressure, what causes this? What type of decreased pressure, what causes this? Obstruction of what, caused by what? Retention of what, caused by what? Increased permeability, caused by what?
Definition
-Increased hydrostatic pressure, caused by reduction in venous return
-Decreased plasma colloid osmotic pressure, caused by reduction in plasma proteins
-Lymph obstruction, caused by scarring, tumors, certain infections
-Primary renal sodium retention, caused by renal failure
-Increased vascular permeability, caused by inflammation (plasma proteins get out and cause inflammation)
Term
Why is generalized subcutaneous tissue edema important?
Definition
It may not be initially critical, but the underlying cause of cardiac or renal failure is important
Term
What are two types of edema that are rapidly fatal? Why?
Definition
-Pulmonary: in the lungs you won't be transferring oxygen efficiently and its infection prone
-Brain: there is no room for any expansion
Term
Define hyperemia and congestion. Which process is active, which is passive? Describe the color of tissue associated with each.
Definition
Increased volume of blood in a particular tissue
-hyperemia: active process; caused by arteriolar dilation, such as sites of inflammation or in skeletal muscle during exercise; tissue is redder than normal (erythema)
-Congestion: passive process; caused by impaired venous return out of tissue; tissue is bluer than normal (cyanosis)
Term
What is a hemorrhage?
Definition
rupture of blood vessels with loss of blood
Term
What is petechiae?
Definition
Pin point hemorrhages or spots under the skin or on the mucous membranes. Usually caused by platelet issue, or we don't have enough platelets, or problem with the coagulation system.
-1-2 mm
Term
What is purpura?
Definition
3-5 mm, like petechiae but bigger
Term
What is ecchymoses?
Definition
A bruise. 1-2 cm, subcutaneous bruise; hgb (blue-red), bilirubin (blue-green), hemosiderin (golden-brown)
Term
A hemorrhage can be external or internal. What is another name for an internal hemorrhage?
Definition
hematoma
Term
What are two examples of hemorrhage in a body cavity?
Definition
-hemothorax
-hemopericardium
Term
The clinical significance of a hemorrhage depends on the site and volume of blood loss, what are the consequences of a massive hemorrhage?
Definition
blood loss, hypovolemic shock, exsanguination, death
Term
The clinical significance of a hemorrhage depends on the site and volume of blood loss, what are the consequences of a hematoma?
Definition
compression of tissues
Term
The clinical significance of a hemorrhage depends on the site and volume of blood loss, what are the consequences of a intracerebral hemorrhage?
Definition
stroke, death
Term
The clinical significance of a hemorrhage depends on the site and volume of blood loss, what are the consequences of chronic hemorrhage?
Definition
slow blood loss, iron deficiency anemia
Term
Define hemostasis
Definition
A normal process that maintains the blood in a fluid state and produces a local hemostatic plug at sites of vascular injury
Term
Define thrombosis.
Definition
The pathologic form of hemostasis. The formation of a blood clot (thrombus) in an uninjured or minimally injured vessel
Term
Hemostasis and thrombosis are dependent on what three factors?
Definition
-vascular endothelium
-platelets
-coagulation system
Term
The vascular endothelium has antithrombotic properties and prothombotic properties, name 3 of each.
Definition
-Antithrombotic: anti platelet effects, anticoagulant properties, fibrinolytic properties
-Prothrombotic: adhesion of platelets, synthesis of vWF, synthesis of tissue factor (thromboplastin)
Term
In tissue injury, platelets adhere to the extracellular matrix by binding to _______ and become activated
Definition
vWF
Term
Platelets release ______ and ________/____ for formation of a primary hemostatic plug
Definition
ADP (platelet aggregation)
Thromboxane A2 (platelet activation and vasoconstriction)
Term
Interaction of what two things causes the coagulation cascade? What does this result in the production of?
Definition
-Interaction of tissue factor (on endothelial cells) and platelet phospholipids
-Results in production of thrombin
Term
Thrombin cleaves circulating __________ into insoluble _______, which polymerizes to form a longer-lasting secondary _______ plug
Definition
fibrinogen
fibrin
hemostatic
Term
In a person with Hemophilia A, where is the problem with the coagulation cascade?
Definition
PTT bc it is factor 8 that is effected
Term
When there is an injury, the tissue factor released from endothelial cells and platelets is activated where?
Definition
only at the sites of injury
Term
Anticoagulants are produced at the site of endothelial injury or during activation of the coagulation cascade, give three examples and what they inhibit.
Definition
-Antithrombins inhibit the activity of thrombin
-Protein C and S inactivate cofactors Va and VIIIa
-Tissue factor pathway inhibitor (TFPI) inactivate factor Xa
Term
What is the fibrinolytic cascade?
Definition
Plasminogen activators (e.g. t-PA) convert plasminogen to plasmin, which breaks down fibrin
Term
What is Virchow's triad?
Definition
Three primary influences on thrombus formation:
-endothelial injury (biggest influence)
-stasis of turbulence of blood flow
-Blood hyper coagulability
Term
What are 4 examples of endothelial cell loss or injury that can lead to thrombosis?
Definition
-MI
-ulcerated plaques in atherosclerotic arteries
-trauma
-inflammation (vasculitis)
Term
What are three subtle influences that alter endothelial function that can lead to thrombosis?
Definition
-hypercholesterolemia
-radiation
-products absorbed from cigarette smoke
Term
Turbulence or stasis in blood flow causes what four things that can lead to thrombosis?
Definition
-disruption of flow brings platelets into contact with endothelium
-prevent dilution of activated clotting factors
-retard inflow of clotting factor inhibitors
-promote endothelial cell activation
Term
What is hyper coagulability? How important is it with respect to thrombosis?
Definition
-any alteration of the coagulation cascade that predisposes to thrombosis
-it is important, but less to thrombosis
Term
What is primary (inherited) and secondary (acquired) heritability?
Definition
-Primary: mutations in coagulation factor genes or anticoagulant genes
-Secondary: prolonged bedrest, MI, tissue damage (surgery, fracture, burns), cancer
Term
Factor V Leiden thrombophilia is an inherited disorder of what?
Definition
blood clotting
Term
What is the most common inherited form of thrombophilia?
Definition
Factor V Leiden Thrombophilia
-affects 2-15% of people with european ancestry
Term
People with Factor V Leiden Thrombophilia are highly susceptible to what? Women are at a slightly increased risk for what?
Definition
-DVT (abnormal blood clots most often in legs, often move to lungs--> pulmonary emboli)
-miscarriage (most women have normal pregnancies)
Term
What are some risk factors for Factor V Leiden Thrombophilia?
Definition
-age, obesity, smoking, injury, surgery, BC pills, hormone replacement therapy
-Factor V leiden mutation in combo with another mutation in the F5 gene (or other member of the coagulation cascade.)
Term
Factor V Leiden Thrombophilia is caused by a ________ mutation in coagulation factor V gene that results in single amino acid _________ (Arg506GIn) in the protein
Definition
missense
substitution
Term
in Factor V Leiden Thrombophilia coagulation factor V cannot be _________ normally by activated ________/__
Definition
inactivated
Protein C
Term
Factor V Leiden Thrombophilia is what type of mutation? What is the lifetime risk for DVT based on the number of alleles affected?
Definition
-autosomal dominant gain-of-function
-one allele: 10%
-two alleles: 80%
Term
What is A mural thrombi of the heart and what is it caused by?
Definition
thrombi occurring in heart chambers or in the aortic lumen
overlying myocardial infarct
Term
A valvular thrombi of the heart is caused by?
Definition
endocarditis
Term
An arterial thrombi is caused by?
Definition
atherosclerosis, aneurysms
Term
A venous thrombi is caused by?
Definition
varicose veins
Term
A microvascular thrombi is caused by?
Definition
disseminated intravascular coagulation (DIC)
Term
Name three arteries and three veins that are common sties for thrombus formation.
Definition
-coronary
-cerebral
-femoral

-femoral
-popliteal
-iliac
Term
If a patient survives the initial thrombosis, some combination of the many things can occur. What is propagation?
Definition
thrombus increases in size and obstructs vessel
Term
If a patient survives the initial thrombosis, some combination of the many things can occur. What is embolization?
Definition
thrombus dislodges or fragments, and moves to other locations in vessels.
Term
If a patient survives the initial thrombosis, some combination of the many things can occur. What is dissolution?
Definition
thrombus is removed by fibrinolytic activity
Term
If a patient survives the initial thrombosis, some combination of the many things can occur. What is organization?
Definition
thrombus induces inflammation and fibrosis; may be incorporated in vessel wall
Term
If a patient survives the initial thrombosis, some combination of the many things can occur. What is recanalization?
Definition
re-establish some degree of blood flow
Term
What is an embolus? What percentage of emboli are part of a dislodged thrombus?
Definition
-an detached solid, liquid, or gaseous mass carried by the blood to a site distant from its origin
99%
Term
Emboli can be either arterial or venous. What percentage of arterial emboli arise from the heart? Where doe the majority of venous emboli arise from?
Definition
85%
majority arise from the legs
Term
Pulmonary thromboemboli arise mainly from ______ in the ______
Definition
DVT in the legs
Term
The effect of a pulmonary thromboembolism depends on the size of the embolus, what are 5 possible outcomes?
Definition
-60-80% are small and clinically silent
-sudden death
-R heart failure (for pulmonale)
-infarction
Term
What does the term systemic thromboemboli refer to?
Definition
emboli in the arterial circulation
Term
80% of systemic thromboemboli arise from what?
Definition
intracardiac mural thrombi
Term
Name 4 things that Intracardiac mural thrombi can be possibly caused by
Definition
-L ventricular wall infarcts
-dilated L atria secondary to mitral valve defects
-aortic aneurysm
-atherosclerotic plaque
Term
Name the two major sites for arteriolar embolization
Definition
-lower extremities (75%)
-brain (10%)
Term
Define infarct
Definition
an area of ischemic necrosis within a tissue or organ that is produced by occlusion of either its arterial supply or its venous drainage
Term
Name four common infarctions that are important causes of clinical illness
Definition
-MI
-cerebral infarction
-pulmonary infarction
-bowel infarction
Term
Name four factors that influence the development of an infarct.
Definition
-availability of an alternative blood supply
-rate of development of the occlusion (the longer the period of time, the more chance the body has to develop an alternative source of blood)
-susceptibility of a tissue to hypoxia (heart cells can live for about 30 mins w/o O2, neurons can last about 2 mins
-oxygen content of the blood (cardiac patients who are not efficient at oxygenating blood are already at a disadvantage)
Term
What is a white infarct?
Definition
-anemic
-caused by arterial occlusion
-usually in solid tissues
Term
What is a red infarct?
Definition
-hemorrhagic
-caused by venous occlusion
-loose tissues
-dual or extensive collateral blood supply
-could be bringing blood into an already necrotic area
Term
Shock causes systemic __________, which leads to reduced what two thing?
Definition
hypoperfusion
-CO
-circulating blood vol
Term
Shock is the final common pathway for many potentially lethal clinical events, give three examples.
Definition
heart failure---> cardiogenic shock
blood loss ----> hypovolemic shock
infections----> septic shock
Term
What is the mortality rate of septic shock?
Definition
25-50%
Term
Describe what causes septic shock
Definition
it is an innate immune response to systemic release of endotoxins: bacterial cell walls (Lipopolysaccharides (LPS) have a fatty toxin--endotoxin)
Term
What are some consequences of septic shock?
Definition
hypotension, decreased myocardial contractility, endothelial injury, disseminated intravascular coagulation (DIC), fibrinolysis (plasmin)
Term
Septic shock causes bleeding due to consumption of _______ factors and activation of _________
Definition
coagulation
fibrinolysis
Term
What is the #1 cause of death in ICU's?
Definition
septic shock
Term
Shock of any kind can cause tissue injury bc of prolonged hypoxia, what happens to the brain?
Definition
ischemic encephalopathy
Term
Shock of any kind can cause tissue injury bc of prolonged hypoxia, what happens to the heart?
Definition
subendocardial hemorrhages and necrosis
Term
Shock of any kind can cause tissue injury bc of prolonged hypoxia, what happens to the kidneys?
Definition
acute tubular necrosis or diffuse cortical necrosis
Term
Shock of any kind can cause tissue injury bc of prolonged hypoxia, what happens to the GI tract?
Definition
patchy hemorrhages and necrosis
Term
Shock of any kind can cause tissue injury bc of prolonged hypoxia, what happens to the liver?
Definition
fatty change or central hemorrhagic necrosis
Term
What are the two types of cells that mostly make up normal vessel walls?
Definition
endothelial cells and smooth muscle cells.
Term
Arteries have a thicker ______, veins have a larger __________
Definition
wall
lumen
Term
The large (elastic) arteries such as the aorta, common carotid, and the iliac, are composed of lots of _______ fibers. Use one word to describe them.
Definition
Elastic
pulsatile
Term
Medium (muscular) arteries, such as the coronary and renal arteries, are mostly composed of what type of cell?
Definition
smooth muscle cells
Term
The small arteries/arterioles are made up of strictly what type of cell? What is controlled here?
Definition
smooth muscle cells
blood pressure controlled here
Term
How big are capillaries? Describe the amount of capillaries, the walls of them, and the speed of flow through them. What are capillaries great for?
Definition
-diameter of a RBC
-thin walls
-slow flow
-great for exchanging oxygen and nutrients
Term
Describe the diameter and thickness of the walls of venules/veins. What is a special feature that is unique to veins? Are veins compressible?
Definition
-large diameter
-thin walls
-they have valves
-compressible, penetrable by tumor
Term
What is the function of lymphatics? What happens when lymph passes through lymph nodes? Where does the lymph return the pathogens to?
Definition
-drain excess interstitial fluid
-pass through nodes checking for infection
-returns pathogens (and tumor cells) to circulation
Term
In response of vessel wall cells to injury, signals from the ECs, platelets, and macrophages, as well as complement and coagulation factors activate ________
Definition
SMCs (smooth muscle cells)
Term
_________ disease is responsible for more morbidity and mortality than any other category of human disease
Definition
vascular
Term
What are the two major mechanisms of vascular disease?
Definition
-narrowing, or total obstruction, of vessels
-weakening, leading to dilation or rupture
Term
What is a berry aneurysm? Where do they most typically occur? Are they fatal if ruptured?
Definition
Small dilations in cerebral vessels, typically in the circle of Willis; fatal if ruptured.
Term
What is an arteriovenous fistula? What can they cause?
Definition
Small direct connections between arteries and veins that bypass capillaries. May cause high output cardiac failure
Term
What is fibromuscular dysplasia? What can it result in?
Definition
A focal irregular thickening of the walls of medium to large sized arteries; results in luminal stenosis
Term
What does arteriosclerosis mean?
Definition
It is a generic term meaning hardening of the arteries, loss of elasticity.
Term
What are the three patterns of arteriosclerosis?
Definition
-arteriosclerosis
-monckeberg medial clacific sclerosis
-atherosclerosis
Term
What vessels does arteriosclerosis commonly affect? What happens to these vessel walls? What other diseases is it associated with?
Definition
-small arteries and arterioles
-vessel wall thickening
-often associated with HTM and/or DM
Term
What is monckeberg medial calcific sclerosis? What age group does it usually affect? Is it clinically significant?
Definition
-calcium deposits in the muscle walls
-people older than 50
-usually no stenosis, and not clinically significant
Term
What is atherosclerosis characterized by? It causes ____% of all heart disease.
Definition
-Characterized by intimal lesions called atheromas (atheromatous plaques or atherosclerotic plaques)
-80%
Term
Atherosclerosis is a systemic disease affecting the _______
Definition
arteries
Term
Atherosclerosis is a chronic __________ response of the vascular wall to a variety of _________
Definition
inflammatory
insults
Term
Atherosclerotic lesions require _______ to become clinically significant
Definition
decades
Term
Atherosclerotic plaques may acutely cause symptoms due to what 4 things?
Definition
rupture
thrombosis
hemorrhage
embolization
Term
What are the two types of atherosclerosis
Definition
-generalized: may affect all arteries in the body

-localized: symptoms that predominate in a single organ or region
Term
Name 4 types of localized atherosclerosis and their possible consequences
Definition
-cerebral: stroke
-coronary: MI
-aortic: aortic aneurysm
-Peripheral: gangrene
Term
Atherosclerosis causes about _____% of all deaths in the western world, many of which are the result of ________ heart disease
Definition
50 ischemic
Term
MI is responsible for about ______% of all deaths in the US
Definition
Term
_________atherosclerosis and _______ also cause significant morbidity and mortality
Definition
carotid
stroke
Term
What are 3 risk factors for atherosclerosis that cannot be changed?
Definition
increased age
male gender
heredity
Term
Name some risk factors for atherosclerosis that can be controlled?
Definition
hyperlipidemia, hypertension, cigarette smoking, diabetes, obesity, C-reactive protein, physical inactivity, diet
Term
Multiple risk factors for atherosclerosis have a ________ effect
Definition
multiplicative
-Two risk factors- risk increases four fold
-three- risk increases seven fold
Term
An atherosclerotic plaque is a soft ______ core covered by a firm ______ cap. It protrudes into the lumen, causing ________. It weakens the underlying arterial _______
Definition
lipid
fibrous
stenosis
wall
Term
What are two possible fates of an atherosclerotic plaque?
Definition
-plaque ruptures in vessel, thrombus formation
-vessel wall expands, aneurysm, and rupture
Term
Describe a true aneurysm, saccular type.
Definition
the vessel wall focally bulges outward
Term
What is a true aneurysm, fusiform type?
Definition
circumferential dilation of the vessel
Term
What is a false aneurysm?
Definition
the wall is ruptured, collection of blood (hematoma) is bounded externally by extravascular tissues
Term
What is a dissection?
Definition
blood has entered (dissected) the wall of the vessel and separated the layers
Term
Atherosclerotic aneurysms occur most frequently where?
Definition
in the abdominal aorta
Term
What are 5 clinical consequences of AAA?
Definition
-rupture into the peritoneal cavity; fatal hemorrhage
-obstruction of branch vessel leading to ischemic injury of tissue
-embolism from atherosclerotic plaque thrombus
-compression of adjacent structure
-may present as an abdominal mass that stimulates a tumor (often palpably pulsating)
Term
What are the two primary preventions of atherosclerosis?
Definition
-lessen risk factors
-Statins: inhibit an enzyme in the liver that plays a key role in making bad cholesterol
Term
What are two secondary preventions of atherosclerosis?
Definition
-aspirin (reduces inflammation, may reduce damage to the inside of arteries. Also increases expression of genes involved in making HDL), statins, beta blockers (block the activity of NE and other stress hormones)
-surgery
Term
What are 5 major phenotypic features of familial hypercholesterolemia?
Definition
-age at onset: heterozygate- early to middle adulthood; homozygote- childhood
-hypercholesterolemia
-atherosclerosis
-xanthomas
-arcus corneae
Term
What is the inheritance pattern of familial hypercholesterolemia?
Definition
autosomal semi-dominant. with more severe disease in homozygotes
Term
Familial hypercholesterolemia (FH) is a disorder of ________ and ________ metabolism caused by mutations in the _______ receptor gene
Definition
cholesterol
lipid
LDL
Term
FH accounts for somewhat less than _____% of patients with hypercholesterolemia
Definition
5
Term
What are lipoproteins?
Definition
particles with protein and phospholipid coats that transport cholesterol and other lipids in the blood.
Term
LDLs are made up of ______% cholesterol
Definition
45
Term
HDLs are made up of _____% cholesterol. What is their main function?
Definition
20
they are mainly int he periphery, bringing cholesterol back to the liver
Term
Risk of atherosclerosis is inversely related to what?
Definition
HDL/total cholesterol ratio
Term
What is the LDLR? Where is it expressed? What does it play a key role in?
Definition
a transmembrane glycoprotein predominantly expressed in the liver and adrenal cortex, it plays a key role in cholesterol homeostasis
Term
What protein binds to the LDLR?
Definition
apoprotein B-100, the only protein of LDL
Term
Hepatic LDLSRs clear ~______% of LDL from the circulation by ________. How is the remainder cleared?
Definition
75
endocytosis
remainder cleared by LDLR-independent pathways
Term
Approx 90% of the mutations in the LDLR gene are______/_______/_______, which occur throughout the gene and alter different aspects of LDLR protein function.
Definition
single nucleotide substitutions
Term
Mutations in the LDLR gene cause accumulation of ____/____, what does this then cause?
Definition
plasma LDL
this causes atherosclerosis by increasing clearance of LDL through LDLR-independent pathways, such as endocytosis of oxidized LDL by macrophages and histiocytes
Term
In FH heterozygotes, what is the usually the only finding in the first decade of life?
Definition
hypercholesterolemia, and it manifests at birth
Term
In FH heterozygotes of all ages, the plasma cholesterol is how much higher than in unaffected individuals?
Definition
twice as high
Term
In FH heterozygotes, which two signs begin to show by the end of the second decade?
Definition
arcus corneae
tendon xanthomas
Term
Describe the progression of signs and symptoms in a FH homozygote?
Definition
-first decade of life: arcus corneae and tenon xanthomas. Plasma CHO is twice that of CHO of heterozygotes and w/o aggressive treatment these pts will usually die by age 30
Term
Besides diet changes, what is other management for FH heterozygotes?
Definition
-bile acid sequestrants (divert a fraction of the increased CHO to bile acid synthesis, which increases the production of more hepatic LDLRs)
-statin drugs (inhibit hepatic cholesterol synthesis)
Term
What are some management options other than diet changes for FH homozygotes?
Definition
-LDL apheresis (venous blood is passing through a tube and the tube has a matrix coated with antibodies against apoB100, this will pull the LDL out of the pt's blood before returning it to their body)
-statin drugs may reduce plasma CHO by 70%
Term
Each child born to a FH heterozygous affected parent has _____% chance risk of inheriting the mutant LDLR allele
Definition
50
bc it is autosomal dominant
Term
What is hypertension? What are some diseases that it is a risk factor for?
Definition
atherosclerosis
coronary artery disease
cardiac hypertrophy/ heart failure
CVAs (strokes)
aortic dissection
renal failure
Term
What is essential hypertension: what must your pressure be consistently above? what percentage of the population does this affect? What percentage of cases are idiopathic? Is it compatible with a long life?
Definition
-elevated BP, consistently above 140/90
-25% of total pop
-90-95% are idiopathic
-compatible with long life unless another complication develops such as MI or stroke
Term
What is secondary HTN?
Definition
HTN secondary to renal disease or adrenal glands
Term
What are four things that essential HTN is probably related to?
Definition
-reduced renal sodium excretion
-vascular changes
-genetic factors
-environmental factors
Term
What is an aneurysm?
Definition
a localized abnormal dilation of a BV or the heart
Term
WHat are the two most important causes of aortic aneurysms?
Definition
atherosclerosis
cystic degeneration of the arterial media
Term
What are 4 other possible causes of aneurysm?
Definition
-trauma
-congenital defects (Berry aneurysm)
-infections (mycotic aneurysms)
-syphilis
Term
What is an aortic dissection?
Definition
blood splits apart the laminar planes of the media to form a blood-filled channel within the aortic wall
Term
What is a major consequence of an aortic dissection? What is the major risk factor?
Definition
-may rupture into body spaces, causing massive hemorrhage or cardiac tamponade (hemorrhage into pericardial sac)
-HTN is the major risk factor
Term
Aortic dissections occur mainly in what two groups?
Definition
-men ages 40-60 with prior HTN (90%)
-younger patients with abnormalities of CT int he aorta (Marfan's)
Term
What one symptom do aortic dissection pt's usually present with?
Definition
sudden onset of excruciating pain
Term
What is Marfan Syndrome: what type of genetic disorder? what does it affect? what do pt's look like?
Definition
-autsomal dominant
-affects the skeletal, CV, and eyes
-pt's are tall and thin with long arms and legs and thin fingers
Term
Where is the mutation located in Marfan syndrome?
Definition
loss of function mutation in the fibrillin 1 (FBN1) gene, which is a major component of microfibrils in ECM; scaffold for elastin
Term
What are the CV effects of Marfan Syndrome?
Definition
it weakens the CT around the base of the aorta
Term
What is vasculitis? What are 4 symptoms? What is it usually caused by? What can it lead to?
Definition
-inflammation of the vessel wall
-fever, malaise, myalgias, and arthralgias
-can be caused by direct invasion of vascular walls by infectious pathogens
-more commonly caused by immune-mediated inflammation
-may lead to occlusion, aneurysmal dilation, and may include necrosis
Term
What is the most common vasculitis? Who does it affect? What arteries does it affect? What are 2 symptoms?
Definition
Giant-cell (temporal) arteritis
>50 yo
small and med. sized arteries (esp. cranial: temporal, opthalmic, vertebral)
pain and tenderness
Term
If the ophthalmic artery is involved in temporal arteritis what is a major consequence?
Definition
can lead to sudden and permanent blindness
Term
What is the possible cause of giant-cell (temporal) arteritis?
Definition
possible T-cell mediated autoimmune response to vessel wall antigen
Term
What is Polyarteritis Nodosa (PAN)? Who does it usually affect? What are 3 symptoms? 30% are associated with what antigen? What can it lead to? What is the treatment?
Definition
-segmental necrotizing inflammation of small to medium-sized arteries, esp the kidneys, heart, liver, and GI tract
-young adults
-malaise, weight loss, fever (typically episodic)
-can lead to aneurysms or even rupture
-remission or cure in 90% treated with corticosteroids
Term
Is PAN fatal?
Definition
yea, in most untreated cases from thromboses and rupture/ renal artery is common
Term
What is the leading cause of acquired heart disease in children?
Definition
Kawasaki disease, 80% of patients are under 4 yo
Term
What are some symptoms of kawasaki disease?
Definition
-acute febrile (usually self limiting illness affecting arteries of all sizes)
-presents with erythema of conjunctiva, mouth, palms, and soles, edema of hands and feet, rash, enlargement of cervical lymph nodes
Term
What do 20% of Kawasaki patients develop if untreated?
Definition
coronary arteritis; can result in aneurysms that rupture or thrombus, causing acute MI
Term
In pt's with Kawasake disease, what is the tx for coronary artery disease?
Definition
IV immunoglobulin therapy
Term
Describe the tongue of many kawasaki pt's
Definition
strawberry tongue
Term
What is THrombangitis Obliterans (Buerger Disease)
Definition
superficial nodular phlebitis
Term
What are 4 common signs and symptoms of Buerger disease?
Definition
-cold sensitivity in the hands
-pain in the instep of the foot
-severe pain, even at rest
-chronic ulceration of toes, feet, or fingers, which may be followed by gangrene
Term
What is Raynaud phenomenon
Definition
exaggerated vasoconstriction of digital arteries and arterioles
Term
What is the most common symptom of raynaud phenomenon
Definition
pallor or cyanosis of fingers and toes
Term
Who does raynaud phenomenon usually affect? What is the primary cause?
Definition
prevalence of 3-5% usually in young women
-primary cause is usually benign
-secondary- caused by other vascular insufficiencies (may be first manifestation of those conditions)
Term
Where are varicose veins usually found?
Definition
typically superficial veins of lower extremities, but also anorectal (hemorrhoids), and gastroesophageal (portal HTN)
Term
What are 4 risk factors for varicose veins?
Definition
pregnancy, obesity, familial tendency, positional stasis
Term
What does varicose dilation lead to?
Definition
incompetent valves
stasis
congestion
tortuosity
Term
Vascular tumors can originate from ______/______ or _______
Definition
blood vessels or lymphatics
Term
Vascular tumors can be composed of endothelial cells, give 3 examples
Definition
-hemangioma- benign
-lymphangioma- benign
-angiosarcoma- malignant
Term
Vascular tumors can be composed of vascular support cells,give two examples
Definition
-glomus tumor- benign
-hemangiopericytoma-malignant
Term
What is a hemangioma?
Definition
an increased numbers of normal abnormal vessels
Term
Where are hemangiomas often found?
Definition
head and neck but also liver
Term
What is Kaposi Sarcoma most frequently associated with?
Definition
AIDS
Term
What is KS characterized by?
Definition
red-purple macules and plaques of the skin
Term
What are the 4 forms of KS?
Definition
classic (european)
endemic (african)
transplant-associated (immunosuppression)
AIDS associated
Term
What is an angiosarcoma?
Definition
Malignant proliferation of endothelial cells
Term
WHo do angiosarcomas usually affect? Where do they usually occur? What type are associated with carcinogenic exposures? DO they metastasize? What is the survival rate?
Definition
older males, they are rare
skin, soft tissue, breast, and liver
hepatic angiosarcomas
metastasize readily
5 year survival rates approach 30%
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