path blood vessels - formatted

Question 1

1. 72.APRIL02 You are asked for advice on screening for cerebral berry aneurysms. Which of the following conditions has THE LEAST risk of aneurysm of the circle of Willis?
2. Neurofibromatosis type 1
3. Marfan Syndrome
4. Coarctation of the aorta
5. Adult/ [Autosomal dominant] Polycystic Kidney Disease
6. Systemic Lupus Erythematosis

Answer 1

5. Systemic Lupus Erythematosis - F -

Known associations include: 
ADPCKD, 
Ehlers-Danlos type 4, 
NF1, 
Marfans, 
FMD of extracranial arteries, 
coarctation of aorta, 
hypertension (in 54% of patients).  
Most are sporadic.

Question 2

2. 68.APRIL02 A 53-year-old female has increasing congestive cardiac failure and normal vessels at coronary angiography. As a child she suffered rheumatic fever. The cardiac valves most likely to be affected are:
3. Aortic alone
4. Mitral and aortic
5. Mitral and pulmonic
6. Aortic and pulmonic
7. Tricuspid and mitral

Answer 2

2. Mitral and aortic - T - Rheumatic heart disease is overwhelmingly the most frequent cause of mitral stenosis (99% of cases). In patients with rheumatic heart disease, the mitral valve alone is involved in 65 to 70% of the cases, mitral and aortic in about 25%. Similar but generally less severe fibrous thickenings and stenoses can occur in the tricuspid valve

Question 3

3. 66.APRIL02 A 12-year-old male dies after a motor vehicle accident. At autopsy, mutiple yellow elongated lesions are seen on the intima of his otherwise normal abdominal aorta. Which of the following statements about this entity IS LEAST correct?
4. Coronary lesions are less common than aortic lesions
5. They are composed of lipid-filled foam cells and T lymphocytes
6. More common in Western children than in Africa
7. Can be seen in children less than 1 year of age
8. Not all progress to fibrous atheromatous plaques

Answer 3

3. More common in Western children than in Africa - F - Fatty streaks occur in all children older than 10y regardless of geography, race, sex or environment.
4. 66.APRIL02 + APRIL 2004 A 12-year-old male dies after a motor vehicle accident. At autopsy, multiple yellow elongated lesions are seen on the intima of his otherwise normal abdominal aorta. Which of the following statements about this entity IS LEAST correct? (JS)
5. Coronary lesions are less common than aortic lesions - T - Coronary fatty streaks are less common than aortic, beginning to form in adolescence, and they occur at the same anatomic sites that are later prone to develop plaques
6. They are composed of lipid-filled foam cells and T lymphocytes - T - composed of lipid-filled foam cells with T lymphocytes and extracellular lipid present in smaller amounts than in plaques
7. More common in Western children than in Africa - F - Fatty streaks occur in all children older than 10y regardless of geography, race, sex or environment.
8. Can be seen in children less than 1 year of age - T
9. Not all progress to fibrous atheromatous plaques T - although fatty streaks may be precursors of plaques, not all fatty streaks are destined to become fibrous plaques or more advanced lesions)

• Fatty streaks are not significantly raised and thus do not cause any disturbance in blood flow.
• They may be precursors, however, of the more ominous atheromatous plaques.
• The streaks begin as multiple yellow; flat spots ( fatty dots) less than 1 mm in diameter that coalesce into elongated streaks; 1 cm long or longer.
• Fatty streaks are composed of lipid-filled foam cells with T lymphocytes and extracellular lipid present in smaller amounts than in plaques.
• Fatty streaks appear in the aortas of some children younger than 1 year of age and all children older than 10 years, regardless of geography, race, sex, or environment.
• Coronary fatty streaks are less common than aortic but begin to form in adolescence, and they occur at the same anatomic sites that are later prone to develop plaques.
• They subsequently decrease in number as atherosclerotic plaques become more prevalent.
• The relationship of fatty streaks to atherosclerotic plaques is complex. o Fatty streaks are related to the known risk factors of atherosclerosis in adults (especially serum lipoprotein cholesterol concentrations and smoking), and some experimental evidence supports the concept of the evolution of fatty streaks into plaques.
• Fatty streaks, however, often occur in areas of the vasculature that are not particularly susceptible to developing atheromas later in life.
• Moreover, they frequently affect individuals in geographic locales and populations in which atherosclerotic plaque is uncommon.
• Thus, although fatty streaks may be precursors of plaques, not all fatty streaks are destined to become fibrous plaques or more advanced lesions.

Question 4

Atherosclerosis

1) define
2) modiafiable factors
3) non-modifiable factors
4) describe fatty streaks
5) list the step of atherosclerosis

Answer 4

DEFINE
- Greek root words for “Gruel” and “hardening”.
- LIpid deposition on arterial walls leading to thickening, hardening, and occlusion
- Extra: ARTERIOsclerosis means hardening of the arteries (generic term). ARTERIOLOsclerosis is hardening of the small arteries and arterioles.

MODIFIABLE RISK FACTORS
- Hyperlipidemia
- Hypertension
- Cigarette Smoking
- Diabetes
- Inflammation

NON-MODIAFIABLE RISK FACTORS
- Genetic abnormalities (familial hypercholesterolemia)
- Family history
- Increasing age
- Male gender. But note, after menopause, the incidence of atherosclerosis in women actually exceeds that of men.

FATTY STREAKS
- Composed of lipid-filled foamy macrophages.
- Although they can evolve into plaques, not all are destined to become advanced lesions.
- Can be seen in infacts (aorta), and in other places in virtually all adolescents
- The observation that coronary fatty streaks begin to form in adolescence, at the same anatomic sites that later tend to develop plaques, suggests a temporal evolution

AHTEROSCLEROSIS PATHOGENESIS
- Endothelial dysfunction via hyperlipidemia, hyperglycemia, hypertension etc.
- Resulting platelet adhesion and recruitment of circulating monocytes and T cells, with cytokine and FGF release
- this leads to SM migration and proliferation and macrophage activation.
- Macrophages and SM cells ingest the LDLs, becoming foam cells.
- Foamy cell-> cause SM proliferation
- enlarging atheroma develop
- thin fibrous capsule
- eventually ruptures then clots with platelets and thrombin
- repeated rupture narrows lumen

Question 5

4. 62.APRIL02 Which of the following IS NOT correct regarding polyarteritis nodosa?
5. There is a transmural necrotising vasculitis of small to medium size arteries
6. There is associated glomerulonephritis
7. There is sparing of the pulmonary circulation
8. It is associated with Hepatitis B antigen positivity in over 20% of cases
9. Remission or cure is achieved in 90% with steroid and cycle

Answer 5

2. There is associated glomerulonephritis - F - Renal involvement is one of the prominent manifestations, BUT because there is no small vessel involvement, glomerulonephritis is not present.
3. 62.APRIL02 Which of the following IS NOT a correct regarding polyarteritis nodosa? (JS)
4. There is a transmural necrotising vasculitis of small to medium size arteries - T - Systemic vasculitis manifested by transmural necrotizing inflammation of small or medium-sized muscular arteries, typically renal and visceral, sparing pulmonary circulation. The common manifestations are malaise, fever,weight loss, hypertension, abdominal pain, malaena, muscle aches, peripheral neuritis. Little association with ANCA. Angiography shows aneurysms or occlusions of the main visceral arteries in 50% of cases. (Robbins)
5. There is associated glomerulonephritis - F - Renal involvement is one of the prominent manifestations, BUT because there is no small vessel involvement, glomerulonephritis is not present.
6. There is sparing of the pulmonary circulation - T
7. It is associated with Hepatitis B antigen positivity in over 20% of cases - T - 30% patients have hepatitis B antigen in serum
8. Remission or cure is achieved in 90% with steroid and cyclophosphamide - T - Disease is fatal if untreated but therapy is very effective.

Question 6

63.APRIL02 Which of the following would be an UNEXPECTED finding following coronary angioplasty?

1. Luminal expansion
2. Plaque rupture
3. Thickened intact intima
4. Medial dissection
5. Proliferative restenosis in 30-50% of patients at 6 months

Answer 6

3. Thickened intact intima - F - The split encompasses the intima and media
4. 63.APRIL02 Which of the following would be an UNEXPECTED finding following coronary angioplasty? (JS)
5. Luminal expansion - T - The key elements of luminal expansion in angioplasty are plaque rupture, medial dissection and stretching of the media of the dissected segment.
6. Plaque rupture - T
7. Thickened intact intima - F - The split encompasses the intima and media
8. Medial dissection - T
9. Proliferative restenosis in 30-50% of patients at 6 months - T -

The long-term success of angioplasty is limited by the development of proliferative restenosis that occurs in approximately 30 to 50% of patients within the first 4 to 6 months after angioplasty
• The process of balloon dilation of an atherosclerotic vessel characteristically causes plaque fracture, often with accompanying localized hemorrhagic dissection of the adjacent arterial wall.
• The plaque splits at its weakest point, which is not necessarily the area most severely obstructed.
• The key elements of luminal expansion in angioplasty are plaque rupture, medial dissection, and stretching of the media of the dissected segment, leading to local flow abnormalities and generation of new, potentially thrombogenic blood-contacting surfaces.
• Thus, an atherosclerotic plaque after angioplasty is unstable, having many features of the disrupted plaque associated with the acute coronary syndromes and other complications of atherosclerosis
• Uncommonly, abrupt reclosure follows the angioplasty. o This reclosure usually occurs as a result of compression of the lumen by an extensive circumferential or longitudinal dissection or by thrombosis.o Nevertheless, most patients improve symptomatically after angioplasty, thereby avoiding the need for aortocoronary bypass graft surgery at that time.
• The long-term success of angioplasty is limited by the development of proliferative restenosis that occurs in approximately 30 to 50% of patients within the first 4 to 6 months after angioplasty

Question 7

6. 64.APRIL02 A 33-year-old woman has sudden onset of blindness in her left eye. The multislice CT angiogram shows marked diffuse narrowing of her brachiocephalic, subclavian and common carotid arteries. There is also involvement both of her external and internal carotid arteries. This distribution is most suggestive of:
7. Takayasu arteritis
8. Atherosclerosis associated with familial
   hypercholesterolaemia
9. Giant cell arteritis
10. Polyarteritis nodosa
11. Fibromuscular hyperplasia

Answer 7

1. Takayasu arteritis - T - clinical syndrome characterized principally by ocular disturbances and marked weakening of the pulses in the upper extremities (pulseless disease), related to fibrous thickening of the aortic arch with narrowing or virtual obliteration of the origins or more distal portions of the great vessels arising in the arch. It has been reported in most areas of the world, including the United States. The illness is seen predominantly in women younger than 40 years old6.
2. APRIL02 A 33-year-old woman has sudden onset of blindness in her left eye. The multislice CT angiogram shows marked diffuse narrowing of her brachiocephalic, subclavian and common carotid arteries. There is also involvement both of her external and internal carotid arteries. This distribution is most suggestive of: (JS)
3. Takayasu arteritis - T - clinical syndrome characterized principally by ocular disturbances and marked weakening of the pulses in the upper extremities (pulseless disease), related to fibrous thickening of the aortic arch with narrowing or virtual obliteration of the origins or more distal portions of the great vessels arising in the arch. It has been reported in most areas of the world, including the United States. The illness is seen predominantly in women younger than 40 years old
4. Atherosclerosis associated with familial hypercholesterolaemia - F - causes premature atherosclerosis in the same distribution as non-familial cases (ie abdominal aorta, ostia of major branches, coronary arteries, popliteal, descending thoracic, ICA and circle of Willis)
5. Giant cell arteritis - F - most common of the vasculitides, is an acute and chronic, often granulomatous, inflammation of medium-sized and small arteries. It affects principally the arteries in the head– especially the temporal arteries– but also the vertebral and ophthalmic arteries. The latter may lead to blindness. In other expressions of this disorder, lesions have been found in arteries throughout the body, and in some cases the aortic arch has been involved to produce so-called giant cell aortitis. The disease is most common in older individuals and rare before the age of 50
6. Polyarteritis nodosa - F - necrotising inflammation of medium sized or small arteries without GN or vasculitis within arterioles, capillaries or venules.
7. Fibromuscular hyperplasia - F - proliferation of the muscular and fibrous elements in middle and large sized arteries of unknown aetiology. Affects renal arteries, ICA or vertebral, iliac, visceral.

Question 8

7. 10.02.11 Which of the following conditions IS NOT a cause (or association) of dissection ?
8. Hypertension
9. Arteriosclerosis
10. Pregnancy
11. Marfan’s syndrome
12. Cystic medial degeneration

Answer 8

A: Arteriosclerosis (NOT A CAUSE).

FROM ROBBINS
- Dissection is unusual in the presence of substantial atherosclerosis or other cause of medial scarring such as syphilis, presumably because the medial fibrosis inhibits propagation of the dissection hematoma

• Hypertension is the major risk factor of dissection. Men aged 40-60 years with hypertension account for >90% of aortic dissection cases.
• Rarely, pregnancy is associated with aortic dissection (10-20 cases per million births). Typically occurs during/after 3rd trimester.
• The 2 group of patients accounting for aortic dissections include younger adults with systemic/localized connective tissue disorders affecting the aorta (Marfan sydrome).
• The most frequent preexisting histological detectable lesion is cystic medial degeneration (which can be seen in a variety of settings, including Marfan Syndrome and Scurvy.

Question 9

8. 10.03.18 Patient A has dissection involving ascending aorta, Patient B has dissection involving ascending aorta, arch and descending aorta
9. Both have type A
10. Both have type B
11. Patient A has type A and patient B has type B
12. Patient A has DeBakey III and patient B has type B
13. Patient A has DeBakey I and patient B has DeBakey III

Answer 9

1. Both have type A

Question 10

9. 10.02.21 Kawasaki disease – which of the following is FALSE?
10. Mostly occur in children younger than 2 years of age
11. Is self-limited disorder with less than 1% death rate
12. > 20% develop CVS sequale
13. characterised by recurrent attacks

Answer 10

4. characterised by recurrent attacks - F - Kawasaki syndrome is an arteritis involving large, medium and small arteries (often coronaries), associated with the mucocutaneous lymph node syndrome (fever, conjunctival and oral erythema and erosion, oedema of hands and feet, rash and cervical lymph nodes), usually in young children and infants. It is self limited.
5. 10.02.21 Kawasaki disease – which of the following is FALSE? Rob p350 (JS)
6. Mostly occur in children younger than 2 years of age - T - occurs in young children and infants with 80% younger than 4 years of age
7. Is self-limited disorder with less than 1% death rate - T - acute fatalities occur in 1% of patients owing to coronary arteritis with superimposed thrombosis or ruptured coronary artery aneurysm
8. > 20% develop CVS sequale - T - approximately 20% develop CVS sequelae including vasculitis of coronary arteries, ectasia, aneurysm formation with rupture, thrombosis, MI or sudden death
9. characterised by recurrent attacks - F - Kawasaki syndrome is an arteritis involving large, medium and small arteries (often coronaries), associated with the mucocutaneous lymph node syndrome (fever, conjunctival and oral erythema and erosion, oedema of hands and feet, rash and cervical lymph nodes), usually in young children and infants. It is self limited.

KAWASAKI SYNDROME (MUCOCUTANEOUS LYMPH NODE SYNDROME)
• arteritis involving large, medium-sized, and small arteries (often the coronary arteries)• associated with the mucocutaneous lymph node syndrome, usually in young children and infants (80% younger than 4 years old).
• The acute illness is manifested by fever, conjunctival and oral erythema and erosion, edema of the hands and feet, erythema of the palms and soles, a skin rash often with desquamation, and enlargement of cervical lymph nodes
• It is usually self-limited
• Epidemic in Japan, the disease has also been reported in Hawaii and increasingly in the United States
• Approximately 20% of patients develop cardiovascular sequelae, with a range of severity from asymptomatic vasculitis of the coronary arteries, coronary artery ectasia, or aneurysm formation to giant coronary artery aneurysms (7 to 8 mm) with rupture or thrombosis, myocardial infarction, or sudden death.
• Kawasaki syndrome is the leading cause of acquired heart disease in children in the United States
• Acute fatalities occur in approximately 1% of patients owing to coronary arteritis with superimposed thrombosis or ruptured coronary artery aneurysm.
• Pathologic changes outside the cardiovascular system are rarely significant.
• Although the vasculitis resembles that of polyarteritis nodosa, with necrosis and pronounced inflammation affecting the entire thickness of the vessel wall, fibrinoid necrosis is usually less prominent in Kawasaki syndrome

Question 11

10. 10.02.31 What is the COMMONEST LESION/ SYSTEM involved in Wegener’s granulomatosis?
11. Lungs
12. Nasal cavity
13. Kidneys
14. Skin
15. Paranasal sinus

Answer 11

A: Lungs - T - persistent pneumonitis with bilateral nodular and cavitary infiltrates (95%)

OTHER (From robbins)
- chronic sinusitis (90%)
- Mucosal ulceration of the nasopharynx (75%)
- Renal disease(80%).
- Other features include skin rashes, muscle pain, articular involvement, mono or polyneuritis, fever

Question 12

11. 10.02.73 Features of cystic lymphangioma do NOT include ?
12. axillary location
13. child 18 months old
14. large cystic spaces
15. benign lesion
16. encapsulated

Answer 12

encapsulated - F - The margins of the tumor are not discrete, and these lesions are not encapsulated. Their removal can therefore be difficult.

11. 10.02.73 Features of cystic lymphangioma do NOT include ? Rob p357
12. axillary location - T - Almost invariably occur in children in the neck or axilla (rarely retroperitoneal)
13. child 18 months old - T - Lymphangiomas are congenital malformations with 50-65% present at birth and 80-90% evident by 2 years
14. large cystic spaces - T - made up of massively dilated cystic spaces lined by endothelial cells and separated by scant connective tissue stroma
    benign lesion - T
    encapsulated - F - The margins of the tumor are not discrete, and these lesions are not encapsulated. Their removal can therefore be difficult.

Question 13

12. Sep03.03 Which finding makes it less likely to be Wegener’s granulomatosis?
13. lack of sinus involvement.
14. Negative for cANCA
15. Lack of renal involvement

Answer 13

A: Negative for cANCA

FACTS (robbins):
- cANCA is positive in 90% of patients with active generalised disease and has a specificity of 95%
- 90% show chronic sinusitis
- 80% show evidence of renal disease

Question 14

13. Sep03.47 Aortic dissection.
14. Can occur without an intimal flap
15. Longitudinal
16. 30-50cm long
17. aortic valve prevents involvement coronary arteries

Answer 14

A: Can occur without an intimal flap

FACTS (ROBBINS):
- There is usually (but not always) an intimal tear that extends into but not through the media of the ascending aorta (Robbins). According to Primer, intimal flap is detectable in 85-90%.
- tears are usually TRANSVERSE or OBLIQUE
- tears are usually 1-5 cm in length with sharp jagged areas.
- length of dissection is variable.
- coronary involvement in aortic dissection is 8%

Question 15

1. PATH2004 Which of the following statements with regards to fatty plaques is LEAST CORRECT?
2. frequently affect individuals in geographic locales and populations in which atherosclerotic plaque is uncommon.
3. ?% of kids that have them
4. ?site
5. bld vess and heart pg 503 blue box know well

Answer 15

1. frequently affect individuals in geographic locales and populations in which atherosclerotic plaque is uncommon. (T)
2. PATH2004 Which of the following statements with regards to fatty plaques is LEAST CORRECT? (–)
3. frequently affect individuals in geographic locales and populations in which atherosclerotic plaque is uncommon. (T)
4. ?% of kids that have them (some younger than 1, all older than 10 yoa)
5. ?site (aorta more common than coronary arteries)
6. bld vess and heart pg 503 blue box know well
   • Fatty streaks are not significantly raised and thus do not cause any disturbance in blood flow.
   • They may be precursors, however, of the more ominous atheromatous plaques.
   • The streaks begin as multiple yellow; flat spots ( fatty dots) less than 1 mm in diameter that coalesce into elongated streaks; 1 cm long or longer.
   • Fatty streaks are composed of lipid-filled foam cells with T lymphocytes and extracellular lipid present in smaller amounts than in plaques.
   • Fatty streaks appear in the aortas of some children younger than 1 year of age and all children older than 10 years, regardless of geography, race, sex, or environment.
   • Coronary fatty streaks are less common than aortic but begin to form in adolescence, and they occur at the same anatomic sites that are later prone to develop plaques.
   • They subsequently decrease in number as atherosclerotic plaques become more prevalent.
   • The relationship of fatty streaks to atherosclerotic plaques is complex.
   o Fatty streaks are related to the known risk factors of atherosclerosis in adults (especially serum lipoprotein cholesterol concentrations and smoking), and some experimental evidence supports the concept of the evolution of fatty streaks into plaques.
   • Fatty streaks, however, often occur in areas of the vasculature that are not particularly susceptible to developing atheromas later in life.
   • Moreover, they frequently affect individuals in geographic locales and populations in which atherosclerotic plaque is uncommon.
   Thus, although fatty streaks may be precursors of plaques, not all fatty streaks are destined to become fibrous plaques or more advanced lesions.

Question 16

2. PATH2004 Regarding polyarteritis nodosa. Which of the following is/are false:
3. The kidneys are most commonly involved in autopsy
4. Aneurysms are seen at angiography in 50%
5. 70% are seropositive for Hepatitis B surface antigen
6. Churg Strauss syndrome frequently involves pulmonary and splenic vessels
7. Serum anti-neutrophil antibody titres correlate with disease activity

Answer 16

A: 70% are seropositive for Hepatitis B surface antigen (FALSE)

FACTS (ROBBINS)
- 30% have hepatitis B antigenaemia, and HBsAg-HBsAb immune complexes can be demonstrated in the lesions.
- The kidneys are most commonly involved in autopsy (involvement in descending order - kidneys, heart, liver, and GIT)
- Aneurysms are seen at angiography in 50% - T - multiple small intrarenal microaneurysms typically at branchpoints in 50-60% - hallmark feature. Lesions usually involve only part of the vessel circumference; the inflammatory process weakens the arterial wall and can lead to aneurysms or even rupture.
- Churg Strauss syndrome frequently involves pulmonary and splenic vessels - T - aka allergic granulomatosis and angiitis; vascular lesions can resemble PAN and microscopic polyangiitis, but in the lung, heart, spleen, peripheral nerves and skin there are also intra- and extravascular granulomas. PAN spares the pulmonary circulation.
- Serum anti-neutrophil antibody titres correlate with disease activity **note, Robbins table 11-3 shows Serum ANCA positivity as +/-, even though in the text body it say no association.

Question 17

3. PATH2004 Which of the following conditions IS TRUE with regards to hypertension?
4. 5-10% of dissections have no obvious intimal tear - this was the true answer

Answer 17

3. PATH2004 Which of the following conditions IS TRUE with regards to hypertension?
4. 5-10% of dissections have no obvious intimal tear - this was the true answer

Question 18

4. PATH2004 Leiomyoma is least common
5. Heart/Myocardium
6. Blood vessels/vascular smooth muscle
7. Oesophagus
8. Stomach
9. Uterus

Answer 18

1. Heart/Myocardium - T - extremely rare (if at all existent!); there are a case reports of benign metastasizing leiomyomas to heart (most common site is lung); occurring years after fibroid removal.
   Common benign cardiac tumors are myxoma (30% of all primary cardiac tumors), papillary fibroelastoma (8%), rhabdomyoma, fibroma, hemangioma, lipomatous hypertrophy, AV nodal tumors, other (5% or less each). In children, common tumors are rhabdomyoma, fibroma and teratoma.
2. PATH2004 Leiomyoma is least common: (GC)
3. Heart/Myocardium - T - extremely rare (if at all existent!); there are a case reports of benign metastasizing leiomyomas to heart (most common site is lung); occurring years after fibroid removal.
   Common benign cardiac tumors are myxoma (30% of all primary cardiac tumors), papillary fibroelastoma (8%), rhabdomyoma, fibroma, hemangioma, lipomatous hypertrophy, AV nodal tumors, other (5% or less each). In children, common tumors are rhabdomyoma, fibroma and teratoma.
4. Blood vessels/vascular smooth muscle - F - aka angioleiomyoma, relatively common, painful tumours - usually females 30-60yo., often in soft tissues of lower limbs; tumours in males more common in UL and H&N.
5. Oesophagus - F - most common benign submucosal tumour of oesophagus; 50% of all benign oesoph tumours. Frequently lower and mid 1/3.
6. Stomach - F - second most common benign gastric tumour (after polyps); most common of calcified benign tumours; submucosal in 60% (accounts for 90% of submucosal tumours, ulcerated in 50%).
7. Uterus - F - most common gynae neoplasm; commonest cause for uterine enlargement after pregnancy. Incidence 20-25% of whites, 50% of blacks. Intramural, subserosal, submucosal.
   [Path outlines; Dahnert]

Question 19

5. PATH2004 Wegener’s granulomatosis is characterised by all of the following except
6. generalised vasculitis
7. GN
8. Granulomatosis of the upper and lower respiratory tract
9. ANA +ve
10. ANCA +ve

Answer 19

8. ANA +ve - F
9. PATH2004 Wegener’s granulomatosis is characterised by all of the following except (JS)
10. generalised vasculitis - T - A necrotising vasculitis characterised by (1) acute necrotising granulomas of the upper and lower respiratory tracts, (2) focal necrotizing or granulomatous vasculitis affecting small to medium sized vessels and (3) renal disease in the form of focal or necrotising, often crescentic glomerulitis.
11. GN - T
12. Granulomatosis of the upper and lower respiratory tract - T
13. ANA +ve - F
14. ANCA +ve - T - c-ANCA is present in 90% of patients with active disease

Question 20

6. PATH2004 Aortic dissection
7. Associated with Marfan’s
8. Stanford
9. Bicuspid valve
10. Intimal flap visible in 90%
11. DeBakey type 2 is the most common

Answer 20

21. DeBakey type 2 is the most common - F - DeBakey Type 1 involves ascending and descending aorta (50%), Type 2 involves ascending aorta only (10%), Type 3 involves descending aorta (40%)
22. PATH2004 Aortic dissection (JS)
23. Associated with Marfan’s T - Causes include hypertension, Marfans, Ehlers-Danlos, Aortic coarctation and bicuspid or unicuspid valve
24. Stanford classification system - T - Stanford classification - Type A involves ascending aorta, Type B involves descending aorta
25. Bicuspid valve T - Causes include hypertension, Marfans, Ehlers-Danlos, Aortic coarctation and bicuspid or unicuspid valve
26. Intimal flap visible in 90% - T - Intimal flap is usually present with haematoma spreading between the middle and outer 1/3 of the media.
27. DeBakey type 2 is the most common - F - DeBakey Type 1 involves ascending and descending aorta (50%), Type 2 involves ascending aorta only (10%), Type 3 involves descending aorta (40%)

Question 21

7. Concerning risk factors for atherosclerosis, which of the following is LEAST CORRECT:
8. Most striking association is with elevated serum levels of LDL
9. After 45, hypertension is a greater risk factor than hypercholesterolaemia
10. Smoking increases incidence of sudden death among victims of heart attacks
11. There is a two fold increase in the incidence of MI in diabetics vs non-diabetics
12. Systolic hypertension is more important than diastolic hypertension

Answer 21

A: LEAST CORRECT
Either “After 45, hypertension is a greater risk factor than hypercholesterolemia” OR “Systolic hypertension is more important than diastolic hypertension”

Robbins doesn’t specifically mention either of those facts. Reading around the topic, systolic BP has been shown to be more important than diastolic BP. However, 45 seems plucked out of nowhere, and Robbins graphs depicting hypertension and risk refers to IHD (not atherosclerosis formation).

FACTS (ROBBINS)
–> Hyperlipidemia (hypercholesterolemia) is a major risk factor for atherosclerosis; even in the absence of other risk factors, hypercholesterolemia is sufficient to initial lesion development.
–> Prolonged smoking of one pack of cigarettes or more daily DOUBLES the death rate from ischemic heart disease.
–> Other factors being equal, the incidence of Myocardial infarction is twice as high in patient with diabetes than in those without.
–> Hypertension is another major risk factor for atherosclerosis; both systolic and diastolic levels are important.

Question 22

8. PATH2004 Regarding fatty streaks, which of the following is LEAST CORRECT:
9. Extracellular lipid is present in smaller amounts than in atheromatous plaques
10. Streaks decrease in number as atheromatous plaques begin to predominate
11. 10% of the aortic intimal surface is involved by the third decade of life
12. Development is independent of racial origin and geography
13. Distribution of plaques differs from the distribution of fatty streaks.

Answer 22

3. 10% of the aortic intimal surface is involved by the third decade of life - F - The area of fatty streak develops by 10y and comprises 4-5% of the intimal surface

SCS: answer 5 is also incorrect. See prior recalls in CVS formatted section. Robbins directly mentions the distribution/locations are the same.

8. PATH2004 Regarding fatty streaks, which of the following is LEAST CORRECT: (JS)
9. Extracellular lipid is present in smaller amounts than in atheromatous plaques - T - composed of lipd-filled foam cells with T lymphocytes and extracellular lipid, present in smaller amounts than in plaques
10. Streaks decrease in number as atheromatous plaques begin to predominate - T
11. 10% of the aortic intimal surface is involved by the third decade of life - F - The area of fatty streak develops by 10y and comprises 4-5% of the intimal surface
12. Development is independent of racial origin and geography - T - Occur in all children over the age of 10y regardless of geography, race, sex or environment.
13. Distribution of plaques differs from the distribution of fatty streaks. - T - According to Robbins, fatty streaks often occur in areas of vasculature that are not particularly susceptible to developing atheromas later in life.

Question 23

9. PATH2004 Regarding aortic dissection. Which of the following is the MOST CORRECT:
10. 5-10% do not have an obvious intimal tear
11. Hypertension is an antecedent in 50% of cases
12. Haemorrhage typically occurs between the inner and middle thirds of the media
13. Elastic fragmentation of the media is rare at autopsy in patients free of dissection
14. Intimal tear is found in the proximal descending thoracic aorta in 90% of cases

Answer 23

9. 5-10% do not have an obvious intimal tear T - There is usually (but not always) an intimal tear that extends into but not through the media of the ascending aorta (Robbins). According to Primer, intimal flap is detectable in 85-90%.
10. PATH2004 Regarding aortic dissection. Which of the following is the MOST CORRECT: (JS)
11. 5-10% do not have an obvious intimal tear T - There is usually (but not always) an intimal tear that extends into but not through the media of the ascending aorta (Robbins). According to Primer, intimal flap is detectable in 85-90%.
12. Hypertension is an antecedent in 50% of cases F – more than 90% occur in patients with hypertension
13. Haemorrhage typically occurs between the inner and middle thirds of the media F – haematoma spreads between the middle and outer 1/3 of the media
14. Elastic fragmentation of the media is rare at autopsy in patients free of dissection - F - cystic medial degeneration (fragmentation and separation of the elastic and fibromuscular elements of the tunica media) is frequently found incidentally at autopsy in patients free of dissection (Robbins)
15. Intimal tear is found in the proximal descending thoracic aorta in 90% of cases F - typically involves the ascending aorta

Question 24

10. PATH2004 Regarding aortic coarctation, which of the following IS NOT a well recognised association:
11. PDA
12. Ventriculoseptal defect
13. Dural AVM
14. Berry aneurysms of the circle of Willis
15. Bicuspid valve

Answer 24

3. Dural AVM
4. PATH2004 Regarding aortic coarctation, which of the following IS NOT a well recognised association: (TW)
5. PDA – 33%
6. Ventriculoseptal defect – 15%
7. Dural AVM
8. Berry aneurysms of the circle of Willis - associated
9. Bicuspid aortic valve – assoc with bicuspid aortic valve in 25-50%

*LW:
Robbins associations:
Bicuspid valve 50%
Congenital AS
ASD
VSD
MR
Berry aneurysms.
PDA (infantile presentation)

(Dahnert 6th ed.) Coarctation – 50% associated with
o bicuspid arotic valve (in 25-50%)
o intracardiac malformations – PDA, VSD, AS, AR, ASD, TGV, ostium primum defect, truncus arteriosus, double-outlet RV.
o Non-cardiac malformations. Turner syndrome.
o Cerebral berry aneurysms
o Mycotic aneurysm distal to CoA.