Type I hyperlipoproteinemia (familial hyperchylomicronemia) is a defect in?
- defect in LPL
- Deficiency in apo C-II (can't clear chylomicrons well)
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
Type IIa (familial hypercholesterolemia) is a defect of what?
4 CLASSES of LDL receptor
Type IIa (familial hypercholesterolemia) symptoms?
- reduced LDL clearance results in high LDL
- Coronary Heart Disease (CAD)
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
Type III (familial dys-beta lipoproteinemia) symptoms?
KNOW KNOW KNOW
Your patient has Xanthomas! What inherited hyperlipoproteinemia do you think of immediately?
Type IV (familial hypertriglyceridemia) has what characteristics?
- elevated VLDL....Think Type IV = VLDL elevated
- associated with glucose = hyperinsulinemia
Your patient has hyperinsulinemia. What inherited hyperlipoproteinemia does he have?
Type IV (familial hypertriglyceridemia)
Type IV hyperlipoproteinemia ...?
- Elevated VLDL production.
- Insulin resistance
- obestiy (fat people!)
- EtOH consumption
Type V (familial) hyperlipoproteinemia associated with what?
- elevated VLDL...again think V = VLDL issues
- elevated chylomicrons
Type V (familial) hyperlipoproteinemia symptoms
- decreased LDL/HDL
Which hyperlipoproteinemia is good for your health? Which lipoprotein is it an increase in?
- Familial hyperalpha-lipoproteinemia
- Increased HDL
Type IIb (familial combined hyperlipidemia) what is unique about it?
- Most common!
- Increased LDLDelayed clearance of VLDL
What clinically do you see with people with Type V (familial) hyperlipoproteinemia
- decreased LDL HDL
- Elevated VLDL, Chylomicrons
What do you see clinically with patients with type IIb (familial combined hyperlipidemia)?
- increased LDLdelayed clearance of VLDLHigh risk of CAD
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
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)
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.
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!
How do you treat type I hyperlipidemia?
low fat, Gemfibrozil
How do you treat type IIa hyperlipidemia?
How do you treat type IIb hyperlipidemia?
How do you treat type II hyperlipidemia?
How do you treat type IV hyperlipidemia?
How do you treat type V hyperlipidemia
- Low Fat Diet
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
What do you use Gemfibrozil for?
PTs with hypertriglyceridemia (so high chylo or VLDL)
So for Type I, III, IV, V
What are Resins used for?
- hypercholesterolemia (lowers cholesterol)
- binds BILE ACIDS in intestines
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
Niacin is great treatment for what?
(high LDL, reduced VLDL clearance)
Your patient has palmar xanthomas what's wrong?
probably Type III, homozygous for apo E2 (defective E2)
- increased LDL/ delayed clearance of VLDL
- so hypertriacylglycerides, hypercholesterolemia