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66 Cards in this Set

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  • Back
What is Arteriosclerosis adn name three types?
General term for vascular disease, characterized by rigidity (sclerosis), and often thickening, of blood vessels.
1) Monckeberg arteriosclerosis
2) Arteriolosclerosis
3) Atherosclerosis
What is Monckeberg arteriosclerosis?
1) Involves the media of medium-sized muscular arteries, most typically teh radial and ulnar arteries, and usually affects persons older than 50 years of age.
2) Characterized by ring-like calcifications in teh media of the arteries
3) Does not obstruct arterial flow because the intima is not involved
4) Results in stiff, calcific "pipestem" arteries.
5) May coexist with atherosclerosis but is distinct from and unrelated to it.
What is Arteriolosclerosis?
1) Characterized by hyaline thickening or proliferative changes of small arteries adn arterioles, especially in the kidneys.
2) Usually associated wiht hypertension or DM
List the two variants of arteriolosclerosis.
Hyaline arteriolosclerosis
Hyperplastic arteriolosclerosis
What is Hyaline arteriolosclerosis?
1) Characterized by hyaline thickening of arteriolar walls
2) In the kidneys, is called benign nephrosclerosis and is associated with hypertension
What is hyperplastic arteriolosclerosis?
1) Marked by concentric, laminated, "onionskin" thickening of the arteriolar walls.
2) May be accompanies by necrotizing arteriolitis, intramural deposition of fibrinoid material in arterioles with vascular necrosis and inflammation.
3) In the kidneys, is called malignant nephrosclerosis and is associated with malignant hypertension.
What is atherosclerosis?
1) The most frequent caused of significant morbidity caused by vascular disease.
2) Seen worldwide, bu the highest incidence occurs in Finland, G, and other northern European countries, the US, adn Canada. Incidence is more than 10-fold greater in Finland than in Japan.
What are the characteristics of atherosclerosis?
Fibrous plaques, or atheromas, within the intima of arteries, most frequently teh proximal portions of the coronary arteries, the larger branches of the carotid arteries, the circle of Willis, the large vessels of the lower extremities, and the renal mesenteric arteries.
Describe the plaque of atherosclerosis.
1) Central core of cholesterol adn cholesterol esters
2) Lipid-laden macrophages, or foam cells
3) Calcium
4) Necrotic debris
Describe the core of atherosclerosis.
1) Covered by a subendothelial fibrous cap, made up of smooth muscle cells, foam cells, fibrin adn other coagulation proteins, as well as extracellular matrix material, such as collagen, elastin, glycosaminoglycans, and proteoglycans
How are plaques complicated?
1) Ulceration, hemorrhage into the plaque, or calcification of the plaque
2) Thrombus formation at teh site of the plaque, producing obstructive disease
3) Embolization of an overlying thrombus or of plaque material itself
How do atheromas develop?
From the fatty streak, a lesion that is characterized by focal accumulations if the intima of lipid-laden foam cells that may appear as early as the first year of life and that is present in the aorta of most older children.
What are the consequences of atherosclerosis?
1) Most significant consequence is ischemic heart disease and MI, the most common cause of death in the US.
2) Other significant complications iinclude stroke from cerebral ischemia and infarction, ischemic bowel disease, peripheral vascular occlusive disease with findings varying from claudication to ischemic necrosis and gangrene, and renal arterial ischemia with secondary hypertension
3) Weakening of the vessel wall may lead to aneurysm formation.
What are the risk factors of atherosclerosis?
1)Incidence increases with age
2) Gender plays a role; more common in men in all age groups, athough the incidence increases in postmenopausal women.
3) Considerable evidence links hypercholesterolemia with atherosclerosis
4) Hypertension is a major risk factor for, and is associated with premature atherosclerosis.
5) DM is associated with premature atherosclerosis. Atherosclerotic peripheral vascular occlusive disease, often leading to gangrene of the lower extremities, is common in diabetic patients.
6) Cigarette smoking is well established RF.
7) Obesity, physical inativity, type A personalities, hyperuricemia, hyperhomocysteinemia, methylene tetrahydrofolate reductase mutations, lipoprotein A, infection with C pneumoniae and oral contraceptives are less firmly established
What is the current concept of atherosclerosis?
1) Primary event as injury to or dystunction of arterial endothelium, which may be produced by hypercholesterolmia, mechanical injury, hypertension, immune mechanisms, toxins, or viruses or other infectious agents.
2) Hyperlipidemia may initiate endothelial injury, promote foam cell formation, act as a chemotactic factor for monocytes, inhibit macrophate motility, or injure smoothe muscle cells.
What are the steps in the current concept of atherosclerosis?
1) Entry of monocytes and lipid into subendothelium, sometimes wiht platelet adhesion and aggregation at teh injury site.
2) Release of mitogenic factors from platelets and perhaps also from monocytes
3) Induction by these growth factors of proliferatoin adn migration of smooth moscle cells into the intima, with the production of CT matrix proteins
4) Monocytes and smooth muscle cells engulf lipid and contribute to the deposition of lipid into lesions. Monocyte conversion to lipid-laden foam cells is mediated by specific monocyte receptors, the Beta-VLDL receptor, and the scavenger receptor, which recognizes modified LDL.
What are the mitogenic factors that are released in atherosclerosis?
1) PDGF
2) Fibroblast GF (possibly)
3) Epidermal GF (possibly)
4) Transforming GF-alpha (possibly)
What are aneurysms?
1) Localized abnormal dilations of either arteries or veins.
2) Can erode adjacent structures or rupture.
3) May be of several different types.
What is an atherosclerotic aneurysm?
Most frequently occurs in the descending, especially the andominal, aorta.
What is an aneurysm due to cystic medial necrosis?
Most frequent aneurysms of the aortic root.
What is a berry aneurysm?
1) Small, saccular lesions most often seen in teh smaller arteries of the brain, especially in the circle of Willis
2) Are NOT preesnt at birth but develop at sites of congenital medial weakness at biurcations of cerebral arteries.
3) are UNRELATED to atherosclerosis.
4) Are often an association of adult polycystic kidney disease
5) Are the most frequent cause of subarachnoid hemorrhage.
What is a syphilitic (leutic) aneurysm?
1) Manifestation of tertiary syphilis, which has become rare with better treatment and control of the disease.
2) Caused by syphilitic aortitis, which is characterized by obliterative endarteritis of the vasa vasorum and necrosis of the media. Grossly, these changes result in a "tree-bark" appearance.
3) In contrast to atherosclerotic aneurysm, characteristically involves the ascending aorta. Dilation of the ascending aorta may widen the aortic commissures, leading to aortic valve insufficiency.
What is a dissecting aneurysm (dissecting hematoma)?
1) Longitudinal intraluminal tear, usually in the wall of the ascending aorta, forming a second arterial lumen within the media.
2) Clinically dominated by severe, tearing chest pain, often radiating through to the back. An x-ray may reveal widening of the aortic shadow.
3) May be clinically confused with acute MI, but electrocardiogram, serum troponin I, and serum myocardial enzymes are norma.
4) Characteristically results in aortic rupture, most often into the pericardial sac, causing hemopericardium and fatal cardiac tamponade.
5) Typically associated with hypertension or with cystic medial necrosis, which is characterized by degenerative changes in teh media with descruction of elastic muscular tissue.
6) has NO relation to atherosclerosis
What is an arteriovenous fistula (aneurysm)?
1) An abnormal communication between an artery and a vein.
2) Can be secondary to trauma or other pathologic processes that mechanically penetrate the walls of both vessels.
3) May result in ischemic changes from the diversion of blood, balloning and aneurysm formation from venous pressure, and high-output cardiac failure from hypervolemia.
What is a venous thrombosis (phlebothrombosis)?
1) arises most often in teh deep veins of teh lower extremities.
2) Predisposed by venous circulatory stasis or partially obstructed venous return such as occurs with cardiac failure, pregnancy, prolonged bed rest, or varicose veins.
3) May give rise to embolism with resultant pulmonary infarction. Pulmonary infarcts are charactistically hemorrhagic, subpleural, and wedge-shaped.
4) Often associated with inflammation and is then termed thrombophlebitis.
What are varicose veins?
1) Abnormally dilated and tortuous veins, most often superficial veins of the lower extremities.
2) Predisposed by increased venous pressure such as occurs with pregnancy, obesity, or thrombophlebitis, and in persons whose occupation require prolonged standing.
What are benign vascular tumors?
Usually not true neoplasms but are better characterized as malformations or hamartomas and include:
1) Spider telangiectasias
2) Hereditary hemorrhagic telangiectasia (Osler-Webber-Rendu syndrome)
3) Hemangioma
4) Glomangioma
5) Cystic hygroma
What is spider telangiectasia?
1) Dilated small vessel surrounded by radiating fine channels
2) Associated with hyperestrinism, as seen in chronic liver disease or pregnancy.
What is hereditary hemorrhagic telangiectasia?
Autosomal dominant condition characterized by localized dilation and convolution of venules and capillaries of the skin and mucous membranes, often complicated by epistaxis or gastrointestinal bleeding.
What is hemangioma?
1) Malformation of a larger vessel composed of masses of channels filled with blood.
2) Most common tumor of infancy and is responsible for port-wine stain birthmarks
What is a capillary hemangioma?
Consists of a tangle of closely packed capillary-like channels that may occur in teh skin, subcutaneous tissues, lips, lier, spleen, or kidneys.
What is a cavernous hemangioma?
1) Consists of large cavernous vascular spaces in the skin and mucosal surfaces and in internal organs such as the liver, pancreas, spleen, and brain.
2) Can occur in von Hippel-Lindau disease, an autosomal dominant disorder that is also marked by hemangioblastomas of the cerebellum, brain stem, and retina, as well as by adenomas and cysts of the liver, kidneys, pancreas, and other organs, and by an increased incidence of renal cell carcinoma.
What is a glomangioma (glomus tumor)?
Small purplish, painful subungual node in a finger or toe.
What is a cystic hygroma?
Cavernous lymphangioma that occurs most often in the neck or axilla
List four malignant vascular tumors.
Hemangioendothelioma
Hemangiopericytoma
Hemangiosarcoma
Kaposi sarcoma
What is a hemangioendothelioma?
Intermediate in behavior between a benign and malignant tumor
What is a hemangiopericytoma?
Arises from pericytes and varies in behavior from benign to malignant.
What is a hemangiosarcoma?
1) Rare malignant vascular tumor occuring in teh skin, musculoskeletal system, breast, or liver.
2) Associated with toxic exposures to arsenic or the radioactive diagnostic agent thorium dioxide (Thorotrast).
3) Polyvinal chloride is associated with hemangiosarcoma of the liver.
What is Kaposi sarcoma?
1) Classic KS most often affects older men of Ashkenazic Jewish or Mediterranean origin.
2) Endemic (or African) KS tends to affect young African men adn children, accounting for up to 10% of all cancers in Africa.
3) Epidemic DS occurs as a component of AIDS, especially in the homosexual male risk group and may, be related to coinfection with a virus that has been termed KS herpesvirus.
4) KS is also a feature of immunosuppression associated with causes other than AIDS.
What are vasculitis syndromes (Vasculitides)?
Inflammatory and often necrotizing vascular lesions that occur in almost any organ.
2) Usually mediated by immune mechanisms, most often immune complex depositions. Frequent antigens in immune complexes include DNA, hepatitis B surface antigen, and hepatitis C RNA.
What is polyarteritis nodosa?
1) Characterized by necrotizing immune complex inflammation of small- and medium-sized arteries
2) Marked by destruction of arterial media and internal elastic lamella, resulting in aneurysmal nodules.
3) Associated with hepatitis B viral infection in 30% of patients.
4) Often manifest clinically by fever, weight loss, malaise, abdominal pain, headache, myalgia, and hypertension.
5) Often accompanied by serum antibodies to neutrophilic myeloperoxidase. These antibodies are referred to as perinuclear antineutrophil cytoplasmic antibodies (P-ANCAs)
Where is polyarteritis nodosa found?
1) Kidneys, with immune complex vasculitis in the arterioles and glomeruli; renal lesions and hypertension cause most deaths form PN.
2) Coronary arteries, resulting in ischemic heart dz.
3) Musculoskeletal system, resulting in myalgia, arthralgia, or arthritis.
4) GI tract, manifesting as nausea, vomiting, or abdominal pain.
5) CNS or PNS, the eye or skin
What is Churg-Strauss syndrome (allergic granulomatous angiitis)?
1) Necrotizing vasculitis considered by some to be a variant of PN.
2) Characterized by prominent involvement of the pulmonary vasculature, marked peripheral eosinophilia, and clinical manifestations of asthma.
What is Hyperesnsitivity (leukocytoclastic) vasculitis?
Group of immune complex-mediated vasculitides characterized by acute inflammation of small blood vessels; multiple lesions tend to be of the same age. These are in contrast to the findings in PN.
2) Manifest by palpable purpura when the skin is involved byt can involve any site, including the glomeruli or the gastrointestinal tract.
3) May be precipitated by exogenous antigens such as drugs, foods, or infectious organisms; may also occur as a complication of systemic illnesses such as CT disorders or malignancies.
4) henoch-Schonlein purpura and serum sickness are examples.
What is Henoch-Schonlein purpura?
1) Most common in young children
2) Can sometimes be poststreptococcal in origin
3) Characterized by hemorrhagic urticaria of extensor surfaces of the arms, legs, and buttocks, with fever, arthralgias, and gastrointestinal and renal involvement.
4) Associated with antecedent upper respiratory infections, suggesting that infectious agents may be the inciting antigens; other antigens may include drugs or foods.
What is serum sickness?
1) Seen in the experimental model in which rabbits, after serial injections of bovine serum albumin, develop generalized deposition of antigen-antibody complexes in the heart, joints, and kidney
2) Now rare in humans, bu tin the past was caused by therapeutic administration of various antitoxins
What is wegener granulomatosis?
disease of unknown etiology characterized by necrotizing granulomatous vasculitis of the small to medium sized vessels of the respiratory tract, kidneys, and other organs.
2) Dominated clinicaly by respiratory tract signs anad symptoms, especially of the paranasal sinuses and lungs, and necrotizing glomerulonephritis
3) Manifest by fibrinoid necrosis of small arteries and veins, early in filtration by neutrophils, subsequent mononuclear cell infiltration, and fibrosis. Granuloma formation with giant cells is prominent
4) Associated in most cases with circulating antineutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (C-ANCAs)
What is giant cell arteritides?
1) Seen in medium- to large-sized arteries and are characterized by granuloma formation with giant cells as well as by infiltrates of mononuclear cells, neutrophils, and eosinophils.
2) Includes temporal arteritis and Takayasu arteritis (pulseless dz)
What is temporal arteritis?
1) Most frequetly occurring form of vasculitis
2) Systemic vasculitis occurring most often in elderly persons.
3) Usually affects branches of the carotid artery, particularly the temporal artery.
How is temporal arteritis manifest?
1) Malaise and fatigue
2) Headache or claudication of the jaw
3) Tenderness, absent pulse, and palpable nodules along the course of the involved artery.
4) Visual impairment, especially with involvement of the opthalmic artery
5) Polymyalgia rheumatica, a complex of symptoms including proximal muscle pain, periarticular pain, and morning stiffness
6) Markedly elevated erythrocyte sedimentation rate
What is takayasu arteritis (pulseless dz)?
1) Characterized by inflammation and stenosis of medium- and large-sized arteries with frequent involvement of teh aortic arch adn its branches, producing aortic arch syndrome.
2) Absent pulses in carotid, radial, or ulnar arteries
3) Nonspecific findings such as fever, night sweats, malaise, myalgia, arthritis and arthralgia, eye problems, and painful skin nodules.
What is Mucocutaneous lymph node syndrome (Kawasaki disease)?
1) Acute, self-limited illness of infants and young children characterized by acute necrotizing vasculitis of small- adn medium-sized vessels
2) manifest clinically by fever; hemorrhagic edema of conjunctivae, lips, and oral mucosa; and cervical lymphadenopathy
3) Can be a cause of coronary artery vasculitis with aneurysm formation.
What is thromboangiitis obliterans (Buerger dz)?
1) Acute inflammation involving small- to medium-sized arteries of the extremities, extending to adjacent veins and nerves.
2) Results in painful ischemic dz, often leading to gangreen.
3) Exacerbated by heavy cigarette smoking.
4) Occurs with greater frequency in Jewish populations and is most common in young men.
What is Lymphomatoid granulomatosis?
1) Rare granulomatous vasculitis characterized by infiltration by atypical lymphocytoid adn plasmacytoid cells.
2) May progress from a chronic inflammatory condition to a fully developed lymphoproliferative neoplasm, most often a T cell non-Hodgkin lymphoma.
What is Raynaud disease?
1) Manifest by recurrent vasospasm of small arteries adn arterioles, with resultant pallor or cyanosis, most often in the fingers and toes.
2) Most often precipitated by chilling
3) Most commonly occurs in young, healthy women.
What is Raynaud phenomenon?
Clinically similar to Raynaud dz, but is always secondary to an underlying disorder, most characteristically systemic lupus erythematosus or progressive systemic sclerosis (scleroderma)
What is essential hypertension?
1) HTN of unknown etiology, accounting for the majority of cases.
2) Represents an interaction of predisposing determinants with a number of exogenous factors
What are the genetic factors of essential HTN?
1) Family history of hypertensive disease is seen in three of four patients with the disorder.
2) It is more common and usually more severe in persons of African lineage
What are the environmental factors of essential HTN?
1) Evidence linking levels of dietary sodium intake with hypertension prevalence in populatoin groups is impressive, although not everyone wiht excesive salt intake develops hypertension
2) Stress, probaby mediated by neurogenic vasoconstriction, is a factor in the development of hypertension
3) Other factors include obesity, cigarette smoking, and physical inactivity.
What are the results of essential hypertension?
1) If untreated, can lead eventually to retinal changes, left ventricular hypertrophy and cardiac failure, and benign nephrosclerosis.
2) Can predispose to ischemic heart disease or stroke.
What is secondary hypertension?
Secondary to known causes:
1) Renal disease
2) Endocrine disorders
3) Coarctation of the aorta
4) Toxemia of pregnancy
5) CNS disorders, esp. brain tumors
6) Drugs and chemicals, notably amphetamines adn steroids.
What are the causes of renal HTN?
1) Disorders of the renal parenchyma
2) Unilateral renal artery stenosis; can be caused by atherosclerosis or unilateral fibromuscular dysplasia; marked by atrophy of the affected kidney adn may be corrected surgically.
What is the mechanism of renal HTN?
1) Juxtaglomerular cells respond to decreased vascular tone by secreting renin, which facilitates the conversion fo angiotensinogen to angiotensin I, which is further converted to angiotensin II.
2) Ang II promotes hypertension by acting both as a vasoconstrictor and as an activator of aldosterone secretion
3) Aldosterone promotes sodium and water retention.
What is primary aldosteronism, or Conn syndrome?
1) Usually associated with an adrenocortical adenoma or bilateral adrenal hyperplasia
2) In addition to hypertension, is marked by increased serum sodium and reduced serum potassium
What endocrine disorders cause secondary HTN?
1) Primary aldosteronism
2) Acromegaly
3) Cushing syndrome of pituitary or adrenocortical origin
4) Pheochromocytoma
5) Hyperthyroidism
6) Diabetes Mellitus
What is malignant hypertension?
1) CAN BE a complication of either essential or secondary HTN
2) Follows an accelerated clinical course.
3) Characterized by a marked increase in diastolic blood pressure, focal retinal hemorrhages and papilledema, LV hypertrophy, and LV failure
4) Produces the renal changes of malignant nephrosclerosis: arterioles or glomerular capillaries rupture, resulting in "flea-bitten" kidney, multiple pinpoint petechial hemorrhages on the kidney surface; large, swollen kidneys; necrotizing arteriolitis and glomerulitis with fibrinoid necrosis; and hyperplastic arteriolosclerosis, affecting both glomeruli and arterioles.
5) Most often results in early death from CHF, cerebrovascular accident, or renal failure
6) Occurs in less than 5% of patients with elevated blood pressure, most often in young African-American males