Hyperlipidemia

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List of terms related to Hyperlipidemia

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

Overview

Hyperlipidemia, hyperlipoproteinemia or dyslipidemia is the presence of raised or abnormal levels of lipids and/or lipoproteins in the blood. Lipids (fatty molecules) are transported in a protein capsule, and the density of the lipids and type of protein determines the fate of the particle and its influence on metabolism.

Lipid and lipoprotein abnormalities are extremely common in the general population, and are regarded as a highly modifiable risk factor for cardiovascular disease due to the influence of cholesterol, one of the most clinically relevant lipid substances, on atherosclerosis. In addition, some forms may predispose to acute pancreatitis.

Classification

Hyperlipidemias are classified according to the Fredrickson classification which is based on the pattern of lipoproteins on electrophoresis or ultracentrifugation. It was later adopted by the World Health Organization (WHO). It does not directly account for HDL, and it does not distinguish among the different genes that may be partially responsible for some of these conditions. It remains a popular system of classification, but is considered dated by many.

Fredrickson classification of Hyperlipidemias

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 and 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

Hyperlipoproteinemia type I

This very rare form (also known as Buerger-Gruetz syndrome, primary hyperlipoproteinaemia, or familial hyperchylomicronemia) is due to a deficiency of lipoprotein lipase (LPL) or altered apolipoprotein C2, resulting in elevated chylomicrons, the particles that transfer fatty acids from the digestive tract to the liver. Lipoprotein lipase is also responsible for the initial breakdown of endogenously made triacylglycerides in the form of very low density lipoprotein (VLDL). As such, one would expect a defect in LPL to also result in elevated VLDL. Its prevalence is 0.1% of the population.

Hyperlipoproteinemia type II

Hyperlipoproteinemia type II, by far the most common form, is further classified into type IIa and type IIb, depending mainly on whether there is elevation in the triglyceride level in addition to LDL cholesterol.

Type IIa

Main article: Familial hypercholesterolemia

This may be sporadic (due to dietary factors), polygenic, or truly familial as a result of a mutation either in the LDL receptor gene on chromosome 19 (0.2% of the population) or the ApoB gene (0.2%). The familial form is characterized by tendon xanthoma, xanthelasma and premature cardiovascular disease.

Type IIb

The high VLDL levels are due to overproduction of substrates, including triglycerides, acetyl CoA, and an increase in B-100 synthesis. They may also be caused by the decreased clearance of LDL. Prevalence in the population is 10%.

• Familial combined hyperlipoproteinemia (FCH)
• Secondary combined hyperlipoproteinemia (usually in the context of metabolic syndrome, for which it is a diagnostic criterion)

Treatment

While dietary modification is the initial approach, many patients require treatment with statins (HMG-CoA reductase inhibitors) to reduce cardiovascular risk. If the triglyceride level is markedly raised, fibrates may be preferable due to their beneficial effects. Combination treatment of statins and fibrates, while highly effective, causes a markedly increased risk of myopathy and rhabdomyolysis and is therefore only done under close supervision. Other agents commonly added to statins are ezetimibe, niacin and bile acid sequestrants. There is some evidence for benefit of plant sterol-containing products and ω₃-fatty acids

Hyperlipoproteinemia type III

This form is due to high chylomicrons and IDL (intermediate density lipoprotein). Also known as broad beta disease or dysbetalipoproteinemia, the most common cause for this form is the presence of ApoE E2/E2 genotype. It is due to cholesterol-rich VLDL (β-VLDL). Prevalence is 0.02% of the population.

Hyperlipoproteinemia type IV

This form is due to high triglycerides. It is also known as hypertriglyceridemia (or pure hypertriglyceridemia). According to the NCEP-ATPIII definition of high triglycerides (>200 mg/dl), prevalence is about 16% of adult population.

Hyperlipoproteinemia type V

This type is very similar to type I, but with high VLDL in addition to chylomicrons.

It is also associated with glucose intolerance and hyperuricemia

Unclassified forms

Non-classified forms are extremely rare:

• Hypo-alpha lipoproteinemia
• Hypo-beta lipoproteinemia (prevalence 0.01-0.1%)

External links

• The Fredrickson papers (with photos from early lipoprotein research)
• Template:GPnotebook

Hyperlipoproteinemia	OMIM	GPnotebook	WebMD	Others
Type I	Online Mendelian Inheritance in Man (OMIM) 238600	Template:GPnotebook	.	MeritCare
Type IIa	Online Mendelian Inheritance in Man (OMIM) 144400	Template:GPnotebook	.	Merck
Type IIb		Template:GPnotebook	.
Type III	.	Template:GPnotebook	WebMD	Yahoo
Type IV	Online Mendelian Inheritance in Man (OMIM) 144600	Template:GPnotebook	WebMD	Yahoo
Type V	Online Mendelian Inheritance in Man (OMIM) 144600	Template:GPnotebook	.	.

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