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135 Cards in this Set
- Front
- Back
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diff in structure of large arteries vs musclar arteries?
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large arteries: elastin
muscular arteries: musle |
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what are the 3 layers of blodd vssls?
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intima, media, adventia
intiima: single layer of endo cells internal elatic lamina separates intima from media media: sm m cells adventia: connective tissue |
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with age what happens to the aorta?
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loss elastin thus the arteries bcome tortuous and dilated
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fxn of large arteries vs musclar artery?
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large artery: storage and propulsion
muscular artery: regulatio of pressure |
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diamter of capillaries? flow rate?
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dimater= RBC size
slow flow rate endothelial cell lining but no media |
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compare the veins to arteries
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veins: larger diamters, larger lumens, thinner less well organized walls and bc of this are predisposed to irregular dilation, compression and easy penetration
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job of post capiillary venules?
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site of fliuid/cell transfer to tissues in inflammation
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what do endothlail cells contain?
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Weibel Palade bodies: membrane bound storage organelles that contain vWF
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how do we ID endothlial cells?
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vWF contiained inside Weibel Palade bodies
CD31 (PECAM) CD34 |
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what are some impt activators of endothlial cells?
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cytokines, complement, hypoxia
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what are some induced produces of EC"s
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adhesion molecules, MHC
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where do SMCs migrate in injury?
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from media to intima to cause intimal thickening
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how are ECs involved in transport?
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the allow small molcues to cross, large to cross if pressure is high, stop proteins
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how are EC's involved in synthesis?
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collagen prodxn
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how are EC's involved in coagulation regulation?
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anti coagulands, prothrombotics, **vWF
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what happes to SMC after they migrate into intima?
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lose ability to contract and they gain the capacity to divide
decrase in contractile filametns and an incrase in organelles involved in protein syn may lead to narrowing of lumen *intimal thickening can also occur in normal arteries as a result of aging |
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how do EC's regulate blood flow?
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produce endothelin to cause vascoconstriction
produce NO to cause vasodilation |
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what is an AV fistula?
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rare abnormal communication b/n arteries and vv
arise as developemental defects problematic b/c in CV could lead to high output cardiac failure b/c they cause the heart to pump additional volume CNs: irritation CNS: rupture and cause stroke |
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arterioLOsclerosis?
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hardening of ARTERIOLES
thickening of vssl walls with with luminal narrowing that may cause downstream ishcemic injury *most often associated with hypertension and DM |
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Monkeberg medial calcific stenoiss?
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calcific deposits in musclar arteries in persons older than 50
media is inovlved |
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what are three patterns of ateriosclerosis?
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arteriosclerosis=hardening of ateries
Atherosclerosis Monkeberg medial calcfic stenois Arteriolosclerosis |
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what problem is responsbile for 50% of all deaths?
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atherosclerosis
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what problem is responsibe for 25% of all deaths?
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IHD
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what are the earliest lesions of atherosclerosis?
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fatty streaks:lipid filled foam cells
not raised, do not cause disturbance in blood flow begin as fatty dots that coalesce into steaks |
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what are fatty dots?
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intial lesion
foam cells: few intimal macrophages: begining of atherosclerosis |
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all childran older than age 10 have what?
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fatty streaks
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ppl of age 30 and up are most likely to have what atherosclerotic change?
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complicated plaque or fibroatheroma
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at which stage in atherosclerosis is lipid entirely intracellular?
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fatty streak
in this stage the plaque is not raised |
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where do athromas tend to occur?
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at exit pts that leave the aorta, fatty streaks are not seen here
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where do fatty streaks normally form?
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in areas of vaculature that are not susceptible to developing atheromas later in life
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what are the major complications of atherosclerosis?
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MI, cerebral infarction, aortic aneurysm, peripheral vascular disease
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what changes occur in intermediate lesion?
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this leasion is after fatty streak
small extracellular lipid pools but most is still intracellular |
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what is an eccentric lesion?
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atheroma that involves only a partial circumference of the arterial wall and are patchy and variable
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what forms after an intermediate lesion? waht is it composed of?
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atheroma: central core of extracellular lipid
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what forms after an atheroma?
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fibroatheroma
fibrous tissue is laid down calification occurs (dystrophic) *the fibrous tissue provides stability to plaque thus won't form thrombus as easlity |
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what is a complicated plque? what is this pt at risk for?
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the most advanced lesion of atherosclerosis at risk for:
rupture, ulceration, erosion hemorrhage into a plaque thrombus (most feared) that may occlude lumen aneurysmal dialation |
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what are the most heaveily involved vessles in atherosclerosis?
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coronary arteries
popliteal arteries internal carotid vssls of cirle of willis |
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what will you see in the necrotic center of a complicated plaque?
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cell debris
cholesterol crystals that look like needles foam cells calcium |
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how does mortality from IHD in US differ from Japan?
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5x greater in US
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how has the deat rate from IHD changed in US since 1960s
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50% reduction in IHD deaths
70% reduction in stoke due to lifestye changes, diet, cigs |
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how does MI incidence change b/n ages of 40 and 60?
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5x greater risk
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who is more prode to atherosclerosis
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males
uncommon in premeno females |
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what single gene defect can play impt role in atherosclerosis?
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familial hypercholesterolemia
LDL R |
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risk involved in HDL and LDL levels
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LDL=bad delivry of cholesterol to peripheral tissues
HDL=good moves cholesterol from atheromas to liver higher HDL=lower risk |
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what can incrse HDL? waht can decrase HDL?
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increse: exercise
decraes: obesity, smoking |
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how does dietary intake contribue to hyperlipidemia?
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omega 3 lipids in fish=good
trans fats= bad statsin: good, lower circulating cholesterol by inhibiting HMG CoReducatase |
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how impt is BP in atherosclerosis
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if BP exceeds 169/95 5x greater risk than 140/90
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increasing incidence of IHD in women is due to what?
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smoking
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incrased risk of atherosclerosis with DM?
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2x increased risk
higher LDL levels prone to strokes 100x risk of foot gangrene |
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ingestion of what can reduce the incidence of CV disease in pts with hyperhomocystinemia?
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folate and B6
therefore give to pts with incrased homocystine |
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what are the big 4 risk factors for atherosclerosis
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hypertension
DM smoking hyperlipidemia |
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what is the first step in pathogenesis of atherosclerosis
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endothelial injury
LDL accumulation in intima LDL become oxidized |
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waht are the 3 cell types involved in atherosclerosis after endothelial injur
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monocytes bind to damaged endo to become foam cells
platelets adhere to damaged intima and release factors taht cause migration of SMCs SMCs proliferate in intima and produce matrix, lipids accumulate in SMcells |
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what damages teh endothelium in atherosclerosis
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hemodynamic trauma
elevated cholesterol |
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how do the monocytes attach to the damaged EC to migrate into intima?
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they bind to VCAM on endo cells and migrate into imtima
in intima they turn into macrophages and engulf oxidized LDLs the lesion then incresaes in size b/c of cytokines released for recruitemen |
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the major lipids in atheromatous plques are what?
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cholesteral and cholesterl esters
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how is Familal hypercholesterolemia involved in lipids in atherosclerosis
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defect in LDL R leading to inadquate hepatic uptake of LDL and incrsed circulaign LDL
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how does hypercholestolemia direcly impair EC fxn
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through increased prodxn of O2 free radicals that deactivate NO
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how do macrophages cause chemical change in lipids?
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macrophages and EC's make free radicals that change lipid to make it oxidize
the oxidized LDL is then ingested by macrophages through the scavenger R (not the LDL R) forming foam cels |
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what growth factors are involved in the proliferation of SMC?
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PDGF
FGF TGF alph |
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what is the oligoclonality theroy of atherogenesis?
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plques equi to bengin neoplastic growth
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what are the most common atherosclerosis aneurysms?
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abdominal aortic aneurysm
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what is an AAA and where do they occur?
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pulsatile abdominal mass
below renal arteries above aota bifurcation thromus present in lumen |
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in who do AAA occur?
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after 50
men genetic susceptibility |
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what is thought to be the pathogenesis concerning AAA
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MMPs throught incrased proteolysis of ECM..degrade all compoents of ECM inlcuding collagen, elastin
there is a decreese in TIMP in AAA pts |
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how do AAA's present? what are the consequences associated with them?
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present as a pulsatile abdominal mass that simulates a tumor
may obstruct local renal structurs, embolism from atheroma to legs, rupture |
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risk of rupture of AAA is related to what?
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size of AAA
bigger = higher chance of rupture |
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what is a leutic aneurysm?
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thoracic aorta aneurysm characteristic of tertiary syphillis that can include the aorta annulus
less likely to rupture can encroach on mediastinal structures resp difficulties difficuly in swallowing |
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sypphiic endarteritis obliterans involves what?
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aneurysm of VASA VASORUM of the arotic wall
narrowing of lumina of vasa causes ischemia and destrxn of media, aorta now loses elasticity and dilates forming aneurysm (**loss of elastin fibers and replae with fibrin) fibrous scars lead to "tree barking" |
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tree barking is seen in ?
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syphillic endarteritis obliterans
caused by the loss of elastin fibers and replaced with fibrin..the scarring looks like tree bark |
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waht is destroyed in sphyilic endarteritis obliterans?
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narrowing of the vasa vasorum causes ishcmea to media..destrxn of MEDIA
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are aortic dissections associated with aortic dilatation/
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NO not always but they are called aneurysm anywas
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what is an aortic dissection/
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dissection of blood bn and along the laminar planes of the media with a formation of blood filled channel w/n aortic wall which often ruptures outward cuasing massive hemoorhage
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in who do aortic dissections occur?
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90% men b/n 40 and 60 with hypertension
or in Marfan younger pts |
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waht will you think if aortic dissection occurs in young pts? older?
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young=marfan
old=hypertension |
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where do aortic dissections occur?
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extends into but not through the media of asc aorta w/n 10cm of aortic valve
spreads along the laminar planes of the aorta* bn middle and outer 1/3 of media |
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what is the major risk factor in dissectoin?
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hypertension
trigger for intimal tear is unkonwn but once it is torn incrased BP accelerates teh problem **antihypertensive therapy |
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when is an aortic dissection most dangerous?
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from the aortic valve to the arch
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what does severe pain, radiating through to back and progresses downwards indicate?
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aortc dissection
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what type of dissection is more common?
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proximal involving the asc aorta
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into what 3 body cavities can rupture ofa dissection enter?
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pericardium
pleura peritoenum |
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how is the prognosis of arotic dissection?
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improved b/c of surgical repair success after operaion is 65-75%
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is a berry aneurysm a congenital disorder?
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no it is a congenital defect present at birth...congential predispostion
the berry aneurysm is not present at birth |
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what are the 3 categorires of mutations?
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genome: loss or gain of whole chromosomes giving rising to monosomy or trisomy
chromosome: rearrangemnt of genetic material Gene: partial or complete deletion of a gene or affect single base |
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result of nondisjxn in MI? MII?
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MI: 2 disomic, 2 nullsomic
MII: 1 disomic, 1 nullisomic, 2 normal |
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what is pleitropy?
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single mutant gene may lead to many end effects ex: sickle cell anemia
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charactristics of AD inheritance
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50:50 chance of transmitting phenotype to each offsrping
males and femals eqally effected male to male transmission vertical transmission |
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which diseae results from complete or partial monosomy of the X and is characterized by hypogonadism in phenotypic females?
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turner
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what percent of Turners are mosaics?
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30%: they have a 45X population along with one or more karytypically normal or abnromal cell types such as 45X/46XX
*all surviviing Turners might be mosaics |
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classic characteristics of Turners
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webbing of neck
**amenorrhea infertitlity short coarctation of aorta**killer failure to develop normal secondary sexual characteristics |
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waht is the single most impt cause of amenorrhea?
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Turners
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what is an impt gene involved in Turner phenotype?
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SHOX short stature homeobox gene on the X chromosome-one of the genes that remains active in both X chormosoms and its has a homolgoe on short arm of Y
haplo of this gene = short stature SHOX is located in PAR region |
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what is a malformation?
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structural defect b/c of intrinsically abnromal developmental process like a cleft lip
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what is a disruption?
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structral defect resulting from breakdown of or interference with an orgininally nl developemental procese
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structral defect resulting from breakdown of or interference with an orgininally nl developemental procese is what?
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disruption
**previously normal |
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what is a deformation?
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abnormaility in form of position of a body part by a non-disruptive mxnl force
uterine constriction |
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what is the most common underlying factor responsible for deformation?
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uterine constrain
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abnormal deveopmental process
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malformation
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waht is dysplasia? ex?
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abnormal org of cells into tissues
achondroplasia |
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waht is a sequence
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pattern of cascade anomalies
structral defect or mechanical factor leading to multiple 2ndary effects ex: potter sequence, pierre robin |
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potter seq also known as ?
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oligohydraminos: decreaed amniotic fluid due to leakage, renal agenesis (fetal urine is a major constituent of amniotic fluid)
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describe potter seq?
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renal agenesis of fetus or urethral obstruction leads to reduced urinary output
nl the fetal urine contributes to amniotic fluid oligohydraminos (or by chronic leakage) result is sqashed facial features, pulmonary hyoplasia, dislocation of hips and talipes |
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oligohydramnios causes waht?
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fetal compression: flattened facies and postitional abnormalities of hands and feet, dislocated hips
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what is a syndrome?
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multiple anomalies with SINGLE BASE cause hat occur independentaly rather than sequentaily
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when is the embyo extremely susceptible to teratogenesis?
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3-9 weeks
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what percent of congenital abnormalities are caused by teratogens?
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2%
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how does thalidomide cause congential malformations?
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high freq of limb abnormalities by blocking the proliferation of mesenchymal cells in the limb bud
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what kind of problems can anti-epileptic drugs cause?
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learning difficulties
behavioural problems mom's take antiepileptic drugs broad nasal root, blunt nasal tip, thin upper lip |
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features of fetal alcohol syndrome?
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short palpebral fissures
epianthal folds low nasal bridge 2% of live births in US: strong coorelation with socio-economic status *common in Native Ams |
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timing of CNS development
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begin in 6th week
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how much alcohol is safe for mom?
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none...one session of binge drinking is as likely to cause FAS
later in preg decrased risk |
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what type of defecits occur with retinoic acid embryopathy?
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eye
lung CV genitourinary changes in HOX gene expression..nature of malformation determined by dosage and timing |
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why retinoic acid embryopathy?
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mom taking retinoic acid for acne
HOX genes have retinoid acid response elements |
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most common CV abnormality?
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ventricular septal defect
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most common CNS abnormality?
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spina bifida
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most common urogenital abnormality?
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bilateral renal agenesis
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waht perent of 1trimester miscarriage have structral abnormalities? 2nd trimester?
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ist: 80-85%
2nd: 25% 15% of all recognzed conceptuses are structurally abnormal |
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are genetic causes or environmental cuases more common in congenital abnormalities?
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genetic: 30-40
5% environment 50% unknown |
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gene involved in Tetraology of Fallot?
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JAG1: single gene defect
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Tetrology of Fallot?
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aorta shifted right
right vent outflow obstruction opening b/n ventricles r ventricular hypertrophy |
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neural tube defects can be reduced to 75% by?
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supplementation of folic acid
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waht is a holoprosencephaly?
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failure of cleavage of prosencephalon
get single ventricle includes Smith-Lemi-Opitz syndrome (lack of cholesterol to combine with SHH) |
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mutations in SHH are freq cause of what
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holoprosencephaly
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cleft lip and palate can be due to?
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cig smoking + TGF alpha, MTHFR gene
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what single gene shows 12-16% genetic predisposition for cleft lip/palate?
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IRF6
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what is Pierre Robin Seq and what can it reuslt from?
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alters 1st arch structures esp manible
can result from oligohydramnoiis |
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laterality defects are due to wha?
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defects in cilia fxn
they sweep high concs of SHH and FGF8 towards the left resulting in asymmetric cascade of gene expression *Lefty1 prevents diffusion to the right side |
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is dizygotic twinning genetic or monozygotic?
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dizygotic: separate placenta and chorion or fusing placentas and fused chorions
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what is reason for the major diff in gene number bn mammal and other animals?
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number of TFs
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what are teh 2 HOX genes that the Drosophila had no homologs for?
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A13 and D13: for limb devleopment thus they are well characterized with human syndroms
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mutations in HOXD13? A13?
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D13: synpolydactly
A13: hand-foot-genital (affect distal ends of limb development) |
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waht are HOX genes involved in?
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patterning of limbs, vertebrae and craniofacial structures
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whare are PAX genes invlved in?
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code for DNA-biding proteins taht code for TF's
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what are the PAX genes?
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paried box genes: code for paired structures like eyes, kidneys, thyroid
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muations in PAX3 cause what?
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Waardenburg sydnrome: mos common form of inherited congenital deafness
Hypopigmenation all AD disorders, No ethnic predispostion |
