Lipoprotein Disorders Flashcards

1
Q

How are diet derived TGs transported in the blood?

A

Chylomicrons

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2
Q

How are diet derived TGs transported in the blood?

A

Chylomicrons

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3
Q

What is the least dense lipoprotein?

A

Chylomicrons

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4
Q

Enterocytes lining villi reabsorb monoglycerides and FAs which are converted into TGs in the cytosol. TGs are then packed into a chylomicron and require an apolipoprotein for asseblym/secretion. What apolipoprotein is required?

A

apoB48

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5
Q

Where do chylomicrons enter the blood stream?

A

Thoracic duct

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6
Q

How do nascent chylomicrons become mature chylomicrons?

A

Obtain apoE/apoC2 from HDL

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7
Q

What enzyme hydrolyzes TGs in chylomicrons?

A

Lipoprotein lipase

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8
Q

What is the function of apoC2 and apoE with regards to chylomicrons?

A

apoC2: activates LPL
apoE: removes chylomicrons remnants from the circulation

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9
Q

Where is VLDL synthesized?

A

Liver

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10
Q

What apolipoprotein is necessary for TG to be packaged into VLDL and secreted into the blood?

A

apoB100

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11
Q

VLDL is hydrolyzed by LPL. What are the lipoprotein products formed by this reaction?

A

IDL, and LDL

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12
Q

What is cholesterol ester transport protein (CETP)?

A

Transfers cholesterol from HDL to VLDL in exchange for TG- leads to removal of HDL from circulation

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13
Q

What is the optimal serum TG level?

A

Less than 150 mg/dL

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14
Q

What is a high serum TG level?

A

Greater than 200 mg/dL

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15
Q

TG/5 = ?

A

VLDL

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16
Q

Enterocytes lining the villi reabsorb monoglycerides and FAs which are then converted into TG in the cytosol. TGs are then packed into a chylomicron and require an apolipoprotein for asseblym/secretion. What apolipoprotein is required?

A

apoB48

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17
Q

Nascent chylomicrons enter the intestinal lymphatics, where do chylomicrons enter the blood stream?

A

Thoracic duct

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18
Q

How do nascent chylomicrons become mature chylomicrons?

A

Obtain apoE/apoC2 from HDL

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19
Q

What enzyme hydrolyzes TGs in chylomicrons?

A

Lipoprotein lipase

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20
Q

What is the function of apoC2 and apoE with regards to chylomicrons?

A

apoC2: activates LPL
apoE: removes chylomicrons remnants from the circulation

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21
Q

Where is VLDL synthesized?

A

Liver

22
Q

What apolipoprotein is necessary for TG to be packaged into VLDL and secreted into the blood?

A

apoB100

23
Q

What effect does VLDL levels have on HDL levels?

A

Increased VLDL causes decreased HDL

24
Q

What are the functions of HDL?

A

Source of apolipoproteins for other lipoproteins (apoE,C2)

Removes CH from fatty streaks and atherosclerotic plaques

25
Q

What pathology is due to a deficiency of apoE?

A

Type 3 hyperlipoproteinemia (broad beta disease)- build up of chylomicron remnants

26
Q

What would a 16 hour standing test show for a patient with familial hyperchylomicronemia?

A

Turbid supranate

27
Q

Detail the pathogenesis of genetic type 2a hyperlipoproteinemia (familial hypercholesterolemia).

A

AD inheritance of deficient LDL receptors

28
Q

Increased serum TGs is due to an increase in chylomicrons and/or VLDL. How can you differentiate which lipoprotein component is elevated?

A

Standing chylomicron test- test tube is left in a refrigerator overnight to give TG a change to settle based on density

29
Q

What physical exam findings are characteristic of familial hypercholesterolemia?

A

Achilles tendon xanthoma

Xanthelasma

30
Q

What is LDL derived from?

A

VLDL

31
Q

LDL=?

A

CH-HDL-TG/5

32
Q

Why is fasting required to accurately calculate LDL?

A

Increased chylomicrons falsely lower calculated LDL

33
Q

Small dense LDL particles are associated with increased risk of what disease(s)?

A

Atherosclerosis, CAD

34
Q

What is the function of cholesterol?

A

Synthesis of: vitamin D, adrenal cortex hormones, bile

35
Q

What is the optimal serum LDL level?

A

Less than 100

36
Q

What increases HDL?

A

Niacin (nicotinic acid), exercise

37
Q

Detail the pathogenesis of type 1 hyperlipoproteinemia (familial hyperchylomicronemia)

A

AR inheritance of deficient LPL or apoC2

38
Q

What are the clinical findings of familial hyperchylomicronemia ?

A

Pancreatitis

39
Q

Detail the pathogenesis of genetic type 2a hyperlipoproteinemia (familial hypercholesterolemia).

A

AD inheritance of deficient LDL receptors.

40
Q

What does a lipid profile show for a patient with familial hypercholesterolemia?

A

Elevated CH and LDL, decreased HDL

41
Q

What physical exam findings are characteristic of familial hypercholesterolemia?

A

Achilles tendon xanthoma

Xanthelasma

42
Q

Detail the pathogenesis of type 3 hyperlipoproteinemia (broad beta disease).

A

AR inheritance of deficient apoE

43
Q

Patients who are homozygotes for type 2 hyperlipoproteinemia are at an increased risk for having an MI before what age?

A

20

44
Q

What physical exam finding is characteristic of broad beta disease?

A

Palmar xanthomas in flexor creases

45
Q

Detail the pathogenesis of genetic type 4 hyperlipoproteinemia (familial hypertriglyceridemia).

A

AD inheritance resulting in overproduction of VLDL

46
Q

What physical exam finding is characteristic of type 4 hyperlipoproteinemia?

A

Eruptive xanthomas

47
Q

What hyperlipoproteinemias can present with acute pancreatitis?

A

Type 4- familial hypertriglyceridemia

Type 1- familial chylomicronemia

48
Q

What are acquired causes of type 2 hyperlipoproteinemia?

A

Hypothyroidism- decreased LDL receptor synthesis

Nephrotic syndrome- increased VLDL synthesis

49
Q

What are acquired causes of type 4 hyperlipoproteinemia?

A

Excess EtOH- increased VLDL production
OCPs- increased VLDL production
DM- decreased LPL due to decreased insulin

50
Q

A standing chylomicron test distinguishes which lipoprotein component is increased. Detail possible outcomes.

A

Turbid supernate- increased chylomicrons (person did not fast, type 1 hyperlipoproteinemia)
Infranate- increased VLDL (type 4 hyperlipoproteinemia)
Turbid Supranate + infranate- increased chylomicrons and VLDL (type 5 hyperliproteinemia)