Hyperlipidemia


Hyperlipidemia

DiseasesDB = 6255
MeshID = D006949

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. [Frederickson DS, Lee RS. A system for phenotyping hyperlipidemia. "Circulation" 1965;31:321-7. PMID 14262568.] 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.

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

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. The incidence of this disease is about 1 in 500 for heterozygotes, and 1 in 1,000,000 for homozygotes.

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 [Thompson GR. Management of dyslipidaemia. "Heart" 2004;90:949-55. PMID 15253984.]

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 (type 4 = familial)=

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. [Third Report of the National Cholesterol Education Program (NCEP) Expert Panel on Detection, Evaluation and Treatment of High Blood Cholesterol in Adults (Adult Treatment Panel III) Final Report. "Circulation" 2002; 106; page 3240]


=Hyperlipoproteinemia type V (type 5 = endogenous)=

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%)

References

External links

* [http://profiles.nlm.nih.gov/FF/Views/Exhibit/visuals/scientist.html The Fredrickson papers (with photos from early lipoprotein research)]
*


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Look at other dictionaries:

  • hyperlipidemia — [hī΄pər li΄pi dē′mē ə] n. the presence of excessive lipids in the blood hyperlipidemic adj. * * * hy·per·li·pi·de·mi·a (hī pər lĭp ĭ dēʹmē ə, lī pĭ ) n. An excess of fats or lipids in the blood. Also called hyperlipemia. * * * …   Universalium

  • hyperlipidemia — [hī΄pər li΄pi dē′mē ə] n. the presence of excessive lipids in the blood hyperlipidemic adj …   English World dictionary

  • hyperlipidemia — lipemia lip*e mi*a (l[i^]p*[=e] m[ e]*[.a]), n. [Gr. li pos fat + ai^ma blood.] (Med.) The presence of an abnormally high concentration of lipid in the blood. Called also {hyperlipemia}, {hyperlipidemia}, {hyperlipoidemia}, {lipidemia},… …   The Collaborative International Dictionary of English

  • hyperlipidemia — noun Etymology: New Latin Date: 1961 the presence of excess fat or lipids in the blood …   New Collegiate Dictionary

  • hyperlipidemia — noun An excess quantity of lipid in the blood; a symptom of several medical conditions …   Wiktionary

  • hyperlipidemia — SYN: lipemia. mixed h. SYN: mixed hyperlipoproteinemia familial, type 5 h.. mixed hyperlipoproteinemia familial, type 5 h. elevations of VLDL and chylomicrons found in plasma. SYN: mixed h.. * * * hy·per·lip·id·emia or chiefly Brit… …   Medical dictionary

  • hyperlipidemia — n. abnormally high level of fat in the blood (Medicine) …   English contemporary dictionary

  • hyperlipidemia — hy·per·lip·i·de·mia …   English syllables

  • hyperlipidemia —    Elevated blood fats, either from heredity, from having so many calories in the diet that they are ending up as liver synthesized storage fats, from an excessively anabolic metabolism...and from a constellation of less common disease causes …   Herbal-medical glossary

  • hyperlipidemia — noun presence of excess lipids in the blood • Syn: ↑lipemia, ↑lipaemia, ↑lipidemia, ↑lipidaemia, ↑lipoidemia, ↑lipoidaemia, ↑hyperlipemia, ↑hyperlipaemia, ↑hyperlipidaemia …   Useful english dictionary


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