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Type I hyperlipoproteinemia (familial hyperchylomicronemia) is a defect in?
- defect in LPL
- Deficiency in apo C-II (can't clear chylomicrons well)
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Type I hyperlipoproteinemia (familial hyperchylomicronemia) has what symptoms?
- increase chylomicrons
- turbid retinal vessels
- PINK MILKSHAKE PLASMA!!!!!
- LOW LDL and HDL
- treat with low fat high carb diet
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Type IIa (familial hypercholesterolemia) is a defect of what?
4 CLASSES of LDL receptor
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Type IIa (familial hypercholesterolemia) symptoms?
- reduced LDL clearance results in high LDL
- Coronary Heart Disease (CAD)
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Type III (familial dys-beta lipoproteinemia) is a defect of what?
- apo E2 homozygotes
- E2 is non functional apo protein, so being a homozygote you can't clear VLDL or IDL
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Type III (familial dys-beta lipoproteinemia) symptoms?
- XANTHOMAS.
- Hypertriglyceridemia
- Hypercholesterolemia
- CAD/Plaques
KNOW KNOW KNOW
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Your patient has Xanthomas! What inherited hyperlipoproteinemia do you think of immediately?
Type III!
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Type IV (familial hypertriglyceridemia) has what characteristics?
- elevated VLDL....Think Type IV = VLDL elevated
- associated with glucose = hyperinsulinemia
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Your patient has hyperinsulinemia. What inherited hyperlipoproteinemia does he have?
Type IV (familial hypertriglyceridemia)
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Type IV hyperlipoproteinemia ...?
- Elevated VLDL production.
- Insulin resistance
- obestiy (fat people!)
- diabetes
- EtOH consumption
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Type V (familial) hyperlipoproteinemia associated with what?
- elevated VLDL...again think V = VLDL issues
- elevated chylomicrons
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Type V (familial) hyperlipoproteinemia symptoms
- hypertriglyceride
- hypercholesterol
- decreased LDL/HDL
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Which hyperlipoproteinemia is good for your health? Which lipoprotein is it an increase in?
- Familial hyperalpha-lipoproteinemia
- Increased HDL
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Type IIb (familial combined hyperlipidemia) what is unique about it?
- Most common!
- Increased LDLDelayed clearance of VLDL
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What clinically do you see with people with Type V (familial) hyperlipoproteinemia
- hypertriglycerides
- hypercholesterol
- decreased LDL HDL
- Elevated VLDL, Chylomicrons
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What do you see clinically with patients with type IIb (familial combined hyperlipidemia)?
- increased LDLdelayed clearance of VLDLHigh risk of CAD
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Your young patient has xanthelasmas on her eyelids. What is in high amounts and what inherited hyperlipoproteinemia does she probably have?
- Probably has Type IIa (hypercholesterolemia)
- She has reduced LDL clearance, so she has a lot of cholesterol and get's xanthelasmas
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Your patient has Arcus senilis (peripheral corneal opacity). What inherited lipoproteinemia does she have?
- Type IIa familial hypercholesterolemia
- reduced LDL clearance (which is high in cholesterol)
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You patient presents with a tendon xanthoma of his achilles tendon. What inherited hyperlipoproteinemia does he have?
- Type IIa (familial hypercholesterolemia)
- due to defects in 4 classes of LDL receptors...reduced LDL clearance...which are high in cholesterol.
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Your nice patient is embarassed because she has eruptive xanthomas on her elbow and everytime one breaks open it's distgusting. What inherited lipoproteinemia does she have?
- Type I (hyperchylomicronemia)....
- Eruptive xanthomas are characteristic of hypertriacyglyceridaemia!
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How do you treat type I hyperlipidemia?
low fat, Gemfibrozil
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How do you treat type IIa hyperlipidemia?
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How do you treat type IIb hyperlipidemia?
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How do you treat type II hyperlipidemia?
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How do you treat type IV hyperlipidemia?
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How do you treat type V hyperlipidemia
- Niacin
- Gemfibrozil
- Low Fat Diet
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What do your statins do? List all three.
Statins inhibit HMG-CoA Reductase (the ultimate rate limiting step of cholesterol synthesis)
MLS = Mevastatin, Lovastatin, Simvastatin
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What do you use Gemfibrozil for?
PTs with hypertriglyceridemia (so high chylo or VLDL)
So for Type I, III, IV, V
Know these!
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What are Resins used for?
- hypercholesterolemia (lowers cholesterol)
- binds BILE ACIDS in intestines
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What does Niacin do? What hyperlipoproteinemia is it used for?
- Niacin inhibits release of FREE FA's from adipose
- decreases VLDL/LDL
- Treats Type IIB
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Niacin is great treatment for what?
Type IIb
(high LDL, reduced VLDL clearance)
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Your patient has palmar xanthomas what's wrong?
probably Type III, homozygous for apo E2 (defective E2)
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Type IIb
- increased LDL/ delayed clearance of VLDL
- so hypertriacylglycerides, hypercholesterolemia
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