Hyperlipoproteinemia

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Hypercholesterolemia Patient Information
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Overview
Classification Familial hyperchylomicronemia Familial hypercholesterolemia Familial combined hyperlipidemia Dysbetalipoproteinemia Primary hypertriglyceridemia Mixed hyperlipoproteinemia
Differential Diagnosis
Screening
ACC/AHA Guideline Recommendations
Summary
Treatment
Major recommendations for statin therapy
Therapeutic response to statin therapy
Blood cholesterol LDL and non-HDL treatment goals
Treatment in heart failure and hemodialysis
Primary prevention
Secondary prevention
Intensity of statin therapy in primary and secondary prevention
Safety Recommendations
Guideline on Lifestyle Management

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synopsis

Hyperlipoproteinemia	Synonyms	Problems	Labs description	Treatment
Type I	Buerger-Gruetz syndrome, primary hyperlipoproteinaemia, or familial hyperchylomicronemia	Decreased lipoprotein lipase (LPL) or altered ApoC2	Elevated chylomicrons	Diet control
Type IIa	Polygenic hypercholesterolaemia or familial hypercholesterolemia	LDL receptor deficiency	Elevated LDL only	Bile acid sequestrants, statins, niacin
Type IIb	Combined hyperlipidemia	Decreased LDL receptor and increased ApoB	Elevated LDL, VLDL and triglycerides	Statins, niacin, gemfibrozil
Type III	Familial Dysbetalipoproteinemia	Defect in ApoE synthesis	Increased IDL	Drug of choice: Gemfibrozil
Type IV	Endogenous Hyperlipemia	Increased VLDL production and decreased elimination	Increased VLDL	Drug of choice: Niacin
Type V	Familial Hypertriglyceridemia	Increased VLDL production and decreased LPL	Increased VLDL and chylomicrons	Niacin, gemfibrozil

Differential Diagnosis

	Diseases	Mode of Inheritance	Laboratory Findings						Other Findings	Management	Complications	Prognosis
			Lipid Profile					Genetic mutations
			Total Cholesterol	LDL	HDL	Triglycerides	Plasma Appearance
Primary Hyperlipoprotenemia	Type I	Autosomal Recessive	Normal or ↑	Normal	Normal	↑↑↑↑	Creamy	LPL gene mutation	Chylomicrons Elevated	Treatment for hyperlipoproteinemia type 1 is intended to control blood triglyceride levels with a very low-fat diet	-Recurrent Pancreatitis -Rarely life threatening	Good
	Type IIA
	Type IIB
	Type III
	Type IV
	Type V
Secondary Hyperlipoprotenemia	Diabetes Mellitus
	Alcohol Abuse
	Estrogen Therapy
	Glucocorticoid therapy
	Renal Disease
	Obesity
	High-fat diet
	Poor physical activity
	Paraproteinemic disorders
	Hypothyroidism

Classification

Hyperlipoproteinemia

Type I: Familial hyperchylomicronemia

Type II

Type III: Dysbetalipoproteinemia

Type IV: Primary hypertriglyceridemia

Type V: Mixed hyperlipoproteinemia

Type A: Familial hypercholesterolemia

Type B: Familial combined hyperlipidemia