Pathology of Pulmonary Hypertension & Embolism Flashcards

1
Q

What gene mutation is associated with primary pulmonary hypertension?

A

Bone morphogenic protein receptor 2 (BMPR2)

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2
Q

A 30yo female presents to the ER with shortness of breath, JVD, and heptaosplenomegaly. The patient has no prior medical history. What is the likely diagnosis?

A

Idiopathic pulmonary hypertension - most common in women 20-40yo

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3
Q

A woman experiences a complicated labor and delivers a stillborn fetus. During labor the woman experiences respiratory distress and expires. What is the likely COD?

A

Amniotic fluid embolism

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4
Q

What is Homans sign?

A

Indicative of a DVT - pain in the calf with dorsiflexion of the foot with the knee extended

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5
Q

A 25yo man with 7-year pack history presents to the ER with hemoptysis and hematuria. Kidney biopsy reveals linear deposits of IgG and crescent-shaped cell proliferation. What is the diagnosis?

A

Goodpasture Syndrome

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6
Q

True/False. Atherosclerotic lesions in pulmonary arteries are always pathologic.

A

True - atheromatous deposits in the pulmonary artery are a cause of secondary pulmonary hypertension

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7
Q

What is the function of BMPR2?

A

This gene inhibits proliferation and favors apoptosis. When the gene is mutated in primary pulmonary hypertension, vascular proliferation and concentric laminar intimal fibrosis occurs

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8
Q

This type of thromboembolism lodges at the bifurcation of the R/L bronchi.

A

Saddle embolus

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9
Q

This vascular disorder is characterized by autoantibodies against collagen IV that destroy the basement membrane.

A

Goodpasture Syndrome

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10
Q

How can Wegener’s granulomatosis be differentiated from other vasculitis based on clinical presentation?

A

URI symptoms (sinusitis, otitis media, nasal ulceration)

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11
Q

How can idiopathic pulmonary hemosiderosis be differentiated from Goodpasture syndrome?

A

Absence of vasculitis, renal damage, and anti-basement membrane autoantibodies, presentation in young children

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12
Q

A patient presents to the ED with respiratory distress and petechial hemorrhages. PMH is significant for a R femur fracture 3 days ago. What is the diagnosis?

A

Fat embolism - arises 1-3 days after trauma

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13
Q

What histologic findings would present on examination of a pulmonary vessel in a patient with pulmonary hypertension.

A

Medial hypertrophy and intimal thickening

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14
Q

How do symptoms of arterial and venous thromboemboli differ?

A

Arterial - pain, pulselessness, parathesia, pallor, paralysis

Venous - pain, edema, erythema, warmth

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15
Q

What antibodies are present with Wegener’s granulomatosis?

A

c-ANCA (PR3-ANCA)

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16
Q

What are the four possible outcomes of a thrombus?

A

Propagation, embolization, dissolution, or recanalization

17
Q

What physical exam findings may present in a patient with pulmonary hypertension?

A

JVD, parasternal lift, hepatomegaly, peripheral edema, ascites

18
Q

What is a common cause of death in patients with Goodpasture syndrome?

A

Uremia