Vascular Disorder - Pathology

Front 1

What is the rate limiting step in cholesterol synthesis?

Back 1

HMG CoA is reduced to mevalonic acid by HMG CoA reductase (with NADPH)

Front 2

Chylomicrons carry what apolipoproteins and what are the functions of them?

Back 2

Apo E: bind to receptors in liver.
Apo C-II: activate lipoprotein lipase in capillary walls of most tissues. Lipoprotein lipase degrades triacylglycerol.
Apo B-48: specific for chylomicrons

Front 3

What are the functions of HDL?

Back 3

Source of apolipoproteins (apo-C II and apo-E) for chylomicrons, VLDL, LDL. Removes cholesterol from plaques.

Front 4

Patient presents with tendon xanthomas and has suffered a stroke recently. Her LDL levels are above 190 mg/dL. Diagnosis?

Back 4

Familial hypercholesterolemia which is a type II hyperlipoproteinemia.

Front 5

What are the causes of type II hyperlipoproteinemia? Pathogenesis?

Back 5

Acquired: hypothyroidism, nephrotic syndrome
Familial hypercholesterolemia: autosomal dominant disorder
Pathogenesis: increased LDL due to decrease LDL receptors

Front 6

What are the causes of type III hyperlipoproteinemia? Pathogenesis?

Back 6

Familial dysbetalipoproteinemia.
Deficiency of apolipoprotein E, increase in serum cholesterol and triglyceride.

Front 7

What are the causes of type IV hyperlipoproteinemia?

Back 7

Acquired: EtOH, oral contraceptives, diabetes mellitus
Familial hypertriglyceridemia: autosomal dominant.
Path: increased synthesis or decrease metabolism of VLDL.

Front 8

What side effects do niacin have?

Back 8

Intense cutaneous flush and pruritus. Use aspirin to reduce this prostaglandin mediated reaction.
Niacin can also cause hyperuricemia.

Front 9

What are the clinical findings in apolipoprotein B (abetalipoproteinemia) deficiency?

Back 9

Decreased serum cholesterol and TG's. Malabsorption due to chylomicron accumulation in villi. Ataxia, hemolytic anemia with acanthocytes.

Front 10

Most common sites for atherosclerosis:

Back 10

Abdominal aorta, coronary artery, popliteal artery, internal carotid artery.

Front 11

Complications of atherosclerosis?

Back 11

Vessel weakness (aneurysm)
Vessel thrombosis (stroke, MI)
Hypertension (activate RAS)
Peripheral vascular disease (gangrene)
Cerebral atrophy

Front 12

In short, what is the reaction to injury in atherosclerosis?

Back 12

Endothelial cell injury. Macrophages/platelets adhere and release cytokines which cause SMC proliferation and migration to intima. Cholesterol deposits and macrophages engulf to form foam cells. SMC produce ECM. Fibrous covers plaque.

Front 13

What is arteriosclerosis? What is hyaline arteriolosclerosis and name two associated conditions?

Back 13

Hardening of arterioles.
Protein is deposited in the vessel wall and occludes the lumen.
Diabetes mellitus: nonenzymatic glycosylation of proteins
Hypertension: intraluminal pressure pushes plasma proteins into vessel wall.

Front 14

What is an aneurysm?

Back 14

Weakening, outpouching of a vessel wall. Dilation and rupture normally ensues.

Front 15

Explain the difference between a true aneurysm, false aneurysm, and a dissection.

Back 15

True: bounded by arterial wall components or the attenuated wall of the heart (atherosclerosis, syphilis, congenital, MI's)
False: breach in the vascular wall leading to an extravascular hematoma
Dissection: blood enters wall, hematoma dissecting between layers

Front 16

Patient presents with severe back pain, hypotensive, and has a pulsatile mass in his central abdomen. Whats the diagnosis?

Back 16

Ruptured abdominal aortic aneurysm.

Front 17

Why do aneurysms tend to form in the abdominal aorta below the renal arteries?

Back 17

No vasovasorum.

Front 18

Where is blood released when an aneurysm of a cerebral artery is ruptured?

Back 18

Subarachnoid space.

Front 19

What is the pathogenesis of a berry aneurysm?

Back 19

There is a defect at the junction of communicating branches with main cerebral vessels. The vessels lack internal elastic lamina and smooth muscle.

Front 20

What is the most common cause of aortic arch aneurysm?

Back 20

Tertiary syphilis (Treponema pallidum).

Front 21

How does syphilis cause aortic arch aneurysm?

Back 21

Treponema pallidum infects the vasa vasorum causing vasculitis (endarteritis obliterans). Plasma cells infiltrate vessel wall. Vessel ischemia leads to dilation of aorta (aortic valve regurgitation).

Front 22

Describe Marfan syndrome.

Back 22

Autosomal dominant disorder resulting in the production of weak elastic tissue due to a defect in synthesizing fibrillin. Common findings: dilation of ascending aorta (aortic regurgitation and dissection), mitral valve prolaps, eunuchoid proportions and arachnodactyly.

Front 23

What is the pathogenesis of aortic dissection? What are risk factors?

Back 23

Cystic medial degeneration: elastic tissue fragmentation. Risk factors: hypertension, pregnancy, coarctation, Ehlers danlos, Marfans.

Front 24

What is the most common cause of death in aortic dissection?

Back 24

Cardiac tamponade.

Front 25

Patient has a pansystolic murmur increasing on expiration heard on right sternal border, no upper extremity pulse and severe retrosternal pain. What artery is being compressed causing the pulselesness?

Back 25

Aortic dissection.
No pulse: dissection closes lumen of subclavian artery.

Front 26

What is phlebothromnbosis? What most commonly causes this conditions?

Back 26

Thrombosis of a vein without inflammation. Most common cause is stasis of blood flow. Hypercoagulable states (ATIII deficiency) is another cause.

Front 27

How will a patient with a DVT present?

Back 27

Swelling, pain on dorsiflexion of foot and compression of calf (Homan sign), pitting edema distal to the thrombosis, statis dermatitis (orange discoloration around the ankles), varicosities.

Front 28

Trousseau sign can be seen as a result of a paraneoplastic syndrome. Why?

Back 28

Carcinomas (especially of the pancreatic head) cause the paraneoplastic syndrome of hypercoagulability. Venous thromboses that appear and disappear in the venous system are migratory thrombophlebitis (Trousseau sign).

Front 29

What is thrombophlebitis?

Back 29

Pain and tenderness along the course of a superficial vein.

Front 30

Patient presents with puffiness and blue face, arms, and shoulders. He also has headache and blurry vision (retinal hemorrhage). What is the diagnosis?

Back 30

Superior vena cava syndrome. Normally a small cell carcinoma of the lung.

Front 31

Patient has a positive Adson test. What is the diagnosis?

Back 31

Adson: pulse disappears when the arm is outstretched and the patient looks to the side of the outstretched arm. This along with numbness or paresthesias is common in thoracic outlet syndrome. Compression of the neurovascular compartment in the neck (weight lifters).

Front 32

What does this kid have?

Back 32

Sturge-Weber syndrome. Vascular malformation on the trigeminal nerve distribution. Also has an AV malformation on the ipsilateral side of the brain.

Front 33

What is a spider telangiectasia?

Back 33

Arteriovenous fistula, disappears when compressed. Associated with hyperestrinism (pregnancy, cirrhosis). In cirrhosis, you can't break down estrogen.

Front 34

What is a capillary hemangioma?

Back 34

Facial lesion in newborns that regress with age.

Front 35

What is the organism associated with Kaposi sarcoma?

Back 35

Human herpesvirus type 8.

Front 36

What is the benign capillary proliferation involving skin and visceral organs in AIDS patients? (Looks like Kaposi's but it not!)

Back 36

Bacillary angiomatosis. Caused by bartonella henselae (also causes cat-scratch disease). Treat it with a sulfa drug.

Front 37

(VAT) Vinyl chloride (plastics), arsenic (pesticides), and thorium dioxide can cause what vascular tumor in the liver?

Back 37

Liver angiosarcoma.

Front 38

What is the most common benign tumor of the liver and spleen?

Back 38

Cavernous hemangioma, which arise from the endothelial cells that line the blood vessels.

Front 39

What is Von Hippel-Lindau syndrome? How will the patient present.

Back 39

von Hippel-Lindau syndrome (VHL) is an autosomal dominant, inherited, neurocutaneous dysplasia complex. VHL is characterized by cavernous hemangiomas in cerebellum (ataxia) and retina (blindness). Increased incidence of pheochromocytoma and bilateral renal cell carcinomas.

Front 40

What is a glomus tumor?

Back 40

Glomus bodies are AV anastomosis that are involved in thermoregulation. A glomus tumor involves the SMC of glomus bodies and are commonly found under the fingernails and are very painful.

Front 41

A large lesion is found on the neck of a child with Turner's. What is it?

Back 41

Cystic hygroma. aka Lymphangioma.

Front 42

95% of the time, small vessel vasculitis will be what type of hypersensitivity? What skin lesions are present?

Back 42

Type III - immune complex depositing causing fibrinoid necrosis.
Skin lesion - palpable purpura.

Front 43

What is the most common cause of a myocardial infarction in children?

Back 43

Kawasaki's disease (children < 4 years of age). Medium-sized vessel vasculitis. Presents with vessel thrombosis and infarction or aneurysm. Clinical features: desquamating rash, lymphadenopathy, swelling of hands/feet, oral erythema.

Front 44

Name the medium-sized vessel vasculitis that is associated with HBsAg. It normally occurs in middle-aged men. Arteries at risk of infarction include renal, coronary, and mesenteric.

Back 44

Polyarteritis nodosa.

Front 45

Male, 52, presents with a headache, jaw claudiaction, tenderness of the scalp and unilateral blindness. Labs show an increase in ESR. What is the diagnosis?

Back 45

Giant cell (temporal) arteritis. Granulomatous large vessel vasculitis involving superficial temporal and ophthalmic arteries (blindness); thrombi contain microabscesses.

Front 46

Male, 28, presents with foot claudication, ulcerations on same foot, and his small toes appears to be falling off. History reveals he smokes a pack a day. What is the diagnosis?

Back 46

Buerger's disease (thromboangiitis obliterans). Medium-sized vessel vasculitis with digital vessel thrombosis.

Front 47

Female, 21, had fingers which turned white-blue-red in response to cold. What is the diagnosis?

Back 47

Raynaud's disease.

Front 48

Young boy presents with hematuria, polyarthritis, and palpable purpura of buttocks and lower extremeties.

Back 48

Small vessel vasculiti (most common in children): Henoch-Schonlein purpura. IgA-anti-Iga immunocomplexes. Glomerulonephritis (RBC casts) and GI bleeding will also be present.

Front 49

25 y/o male presents with a saddle nose deformity, sinus infection, and nodular masses in the lung. Labs show c-ANCA (anti-neutrophil cytoplasmic antibody) positive.

Back 49

Wegener's granulomatosis: necrotizing medium/small sized vessel vasculitis involving upper respiratory tract, lung, and renal vessels. Necrotizing granulomas in upper respiratory tract (saddle nose).

Front 50

Cyclophosphamide is used to treat Wegener's granulomatosis. What can cyclophsphamide cause? How do you prevent this?

Back 50

Hemorrhagic cystitis. MESNA.

Front 51

Vascular, red pedunculated mass that ulcerates and bleeds easily. What is it?

Back 51

Pyogenic granuloma. Post-traumatic or associated with pregnancy (estrogen related).

Front 52

Patient presents with several skin lesions that have been present since birth. You determine that the lesions are talengiectasia. Dx?

Back 52

Hereditary hemorrhagic telangiectasia. aka Osler-Weber-Rendu disease. Autosomal dominant.

Front 53

Young Asian women presents with pulseless extremities and blurry vision. What vasculitis could this be?

Back 53

Takayasu's arteritis. Granulomatous large vessel vasculitis involving aortic arch vessels.

Front 54

Young boy presents to your office with palpable purprua and hematuria. His mother asks why the penicillin you gave him last week made his condition worse. What caused the new symptoms?

Back 54

Microscopic polyangiitis. Small vessel vasculitis involving skin, lung brain, GI tract, and postcapillary venules and glomerular capillaries. Often precipitated by drugs, infections, or immune disorders. Labs: p-ANCA.

Front 55

What are some clinical findings in Churg-Strauss syndrome?

Back 55

Small vessel vasculitis in children/adults involving lung, heart, skin. Allergic rhinitis, asthma. Labs: p-ANCA (labs) and eosinpholia.

Front 56

Patient in Arizona presents with a rattle snake bite. He is given an antivenom but rapidly develops a fever and urticaria. What happened?

Back 56

Serum sickness due to envenomation with horse-antivenom. Serum sickness causes small vessel vasculitis involving immunocomplex deposition in skin vessels.

Front 57

Upon returning from a camping trip in Colorado, a boyscout presents with a fever and a rash on his palms that has began to spread to his trunk. What could be the diagnosis?

Back 57

Rocky Mountain spotted fever. Ricketsia rickettsiae. Organisms invade endothelial cells causing a small vessel vasculitis

Front 58

How does sodium cause hypertension?

Back 58

Excess sodium increases plasma volume. Excess sodium produces vasoconstriction of TPR arterioles: sodium enters arteriole SMCs and opens Ca channel causing vasoconstriction.

Front 59

Name the elastic blood vessels.

Back 59

Aorta, braciocephalic, subclavian, common carotid, ileac.

Front 60

Name the adrenal causes of secondary hypertension.

Back 60

Cushings (increased mineralcorticoids). Pheochromocytoma (increaesd catecholamines). Neuroblastoma (increased catecholamines). 11-hdroxylase deficiency (increased mineralocorticoids). Conn syndrome (increased aldosterone).

Front 61

What is the most common cause of hypertension in young women?

Back 61

Oral contraceptives. They increase synthesis of angiotensinogen.

Front 62

Does Graves disease cause hyper or hypotension? How?

Back 62

Hypertension. Graves disease increases cardiac contraction via hyperthyroidism.

Front 63

What is the most common complication in hypertension? What is the most common cause of death?

Back 63

Left ventricular hypertrophy.
Acute myocardial infarction.

Front 64

What is the primary mechanism of essential hypertension in black Americans and the elderly?

Back 64

Reduced renal sodium excretion.

Front 65

What is the most common cause of secondary hypertension?

Back 65

Renovascular hypertension: activation of RAS. The cause in elderly men is atherosclerotic plaques. The cause in young women is fibromuscular hyperplasia in the renal artery.

Front 66

What are some inherited causes of DVT's?

Back 66

Factor V Leiden, prothrombin mutant (20210A transition), deficiency in ATIII, protein C, or protein S. Homocysteinemia can be acquired or inherited.

Front 67

What is Virchow's triad?

Back 67

Endothelial injury, stasis/turbulence, and hypercoagulability.

Front 68

What INR values do you want to achieve with a patient on long term warfarin treatments?

Back 68

INR 2.0 - 3.0

Front 69

What test are used to diagnose or rule out a DVT?

Back 69

D-dimer can be used to rule out a DVT in patients with a low pre-test probability.
Ultrasonography to test the compressability of the vessel lumen.
Venography.

Front 70

Compare a venous thrombus to an arterial thrombus.

Back 70

Venous - due to stasis/hypercoagulability, dark red, and treated with heparin/warfarin.

Arterial - due to endothelial injury, gray/white with lines of Zahn (alternating fibrin and platelets), and treated with aspirin.

Front 71

Antiphospholipid syndrome (antibodies against phospholipids) can induce a hypercoagulable state. What would a PTT test show?

Back 71

The PTT is paradoxically prolonged. This is an in vitro artifact caused by the interaction of the antibodies with the reagent used in the test.

Front 72

Explain how a women with no sexual history can test positive for syphilis.

Back 72

She has SLE. Anti-cardiolipin antibodies can cause a false-positive serologic test for syphilis because the antigen in the standard tests is embedded in cardiolipin.

Front 73

Adult polycystic kidney disease is associated with: a) AV fistulas, b) atherosclerotic aneurysm, c) berry aneurysm, d) dissecting aneurysm

Back 73

C) berry aneurysm

Front 74

"Flea-bitten" kidney is seen in what condition.

Back 74

Malignant hypertension

Front 75

What is the significance of where a DVT is located in the lower extremity?

Back 75

DVT's distal to the knee in the calf area are normally asymptomatic and do not cause PE. DVT's proximal to the knee normally form from propagation of distal DVT's. Half of patients with symptomatic proximal DVTs have a silent PE and 10% have symptomatic PE.

Front 76

What are some ECG findings due to a pulmonary embolism?

Back 76

Most commonly: sinus tachycardia and nonspecific anterior T wave inversion. Pathoneumonic and less common: S1, Q3 (pathological Q in lead III), T3 (inverted). Right axis deviation. RBBB.