Hyperlipidemia Classification and external resources
ICD-10	E78.
ICD-9	272.0-272.4
DiseasesDB	6255
MeSH	D006949

Hyperlipidemia, hyperlipoproteinemia or dyslipidemia is the presence of raised or abnormal levels of lipids and/or lipoproteins in the blood. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings E00-E35 - Endocrine diseases (E00-E07 Thyroid gland / Thyroid hormone ( Congenital iodine-deficiency syndrome ( The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Lipids are broadly defined as any fat- Soluble ( lipophilic) naturally-occurring Molecule, such as fats oils waxes cholesterol sterols fat-soluble A lipoprotein is a biochemical assembly that contains both Proteins and Lipids The lipids or their derivatives may be covalently or non-covalently bound Blood is a specialized Bodily fluid that delivers necessary substances to the body's cells €”such as nutrients and oxygen—and transports Waste products 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. Proteins are large Organic compounds made of Amino acids arranged in a linear chain and joined together by Peptide bonds between the Carboxyl Metabolism is the set of Chemical reactions that occur in living Organisms in order to maintain Life.

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. Cardiovascular disease or cardiovascular diseases refers to the class of diseases that involve the Heart or Blood vessels ( arteries and Cholesterol is a Lipid found in the Cell membranes and transported in the Blood plasma of all Animals It is an essential component of mammalian Atherosclerosis is a Disease affecting arterial Blood vessels It is a chronic inflammatory response in the walls of arteries in large part due to the accumulation In addition, some forms may predispose to acute pancreatitis. Acute Pancreatitis is a sudden Inflammation of the Pancreas. Depending on its severity it can have severe complications and high mortality despite

Contents 1 Classification 1.1 Hyperlipoproteinemia type I 1.2 Hyperlipoproteinemia type II 1.2.1 Type IIa 1.2.2 Type IIb 1.2.3 Treatment 1.3 Hyperlipoproteinemia type III 1.4 Hyperlipoproteinemia type IV 1.5 Hyperlipoproteinemia type V 2 Unclassified forms 3 References 4 External links

Classification

Hyperlipidemias are classified according to the Fredrickson classification which is based on the pattern of lipoproteins on electrophoresis or ultracentrifugation. Donald Sharp "Don" Fredrickson (August 8 1924 - June 7 2002 was an American medical researcher, principally of the lipid and Cholesterol Electrophoresis is the most well-known electrokinetic phenomenon. The ultracentrifuge is a Centrifuge optimized for spinning a rotor at very high speeds capable of generating acceleration as high as 1000000 g (9800 km/s² ^[1] 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. High-density lipoproteins ( HDL) is one of the 5 major groups of Lipoproteins ( Chylomicrons, VLDL, IDL, LDL, HDL History See also History of genetics The existence of genes was first suggested by Gregor Mendel (1822-1884 who in the 1860s studied inheritance 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, Fibrate
Type III	Familial Dysbetalipoproteinemia	Defect in ApoE synthesis	Increased IDL	Drug of choice: Fibrate
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, Fibrate

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 an Enzyme that Hydrolyzes Lipids in Lipoproteins like those found in Chylomicrons and very low-density Chylomicrons are large lipoprotein particles that transport dietary lipids from the intestines to other locations in the body Familial hypercholesterolemia (abbreviated FH, also spelled familial hypercholesterolaemia) is a Genetic disorder characterized by high cholesterol The LDL Receptor is a mosaic protein that mediates the Endocytosis of cholesterol-rich LDL. Low-density lipoprotein ( LDL) is a type of Lipoprotein that transports Cholesterol and Triglycerides from the Liver to peripheral The statins (or HMG-CoA reductase inhibitors) form a class of hypolipidemic drugs used to lower Cholesterol levels in people with or at risk of Cardiovascular Niacin, also known as vitamin B3, is a water-soluble vitamin which prevents the deficiency disease Pellagra. In Medicine, combined hyperlipidemia (or - aemia) is a commonly occurring form of Hypercholesterolemia (elevated Cholesterol levels characterised The LDL Receptor is a mosaic protein that mediates the Endocytosis of cholesterol-rich LDL. Low-density lipoprotein ( LDL) is a type of Lipoprotein that transports Cholesterol and Triglycerides from the Liver to peripheral The statins (or HMG-CoA reductase inhibitors) form a class of hypolipidemic drugs used to lower Cholesterol levels in people with or at risk of Cardiovascular Niacin, also known as vitamin B3, is a water-soluble vitamin which prevents the deficiency disease Pellagra. In Pharmacology, the fibrates are a class of amphipathic Carboxylic acids They are used for a range of metabolic disorders mainly Hypercholesterolemia In Pharmacology, the fibrates are a class of amphipathic Carboxylic acids They are used for a range of metabolic disorders mainly Hypercholesterolemia Niacin, also known as vitamin B3, is a water-soluble vitamin which prevents the deficiency disease Pellagra. Chylomicrons are large lipoprotein particles that transport dietary lipids from the intestines to other locations in the body Niacin, also known as vitamin B3, is a water-soluble vitamin which prevents the deficiency disease Pellagra. In Pharmacology, the fibrates are a class of amphipathic Carboxylic acids They are used for a range of metabolic disorders mainly Hypercholesterolemia Lipoprotein lipase ( is an Enzyme that Hydrolyzes Lipids in Lipoproteins like those found in Chylomicrons and very low-density Chylomicrons are large lipoprotein particles that transport dietary lipids from the intestines to other locations in the body The liver is a vital organ in the human body and is present in Vertebrates and some other animals 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. Familial hypercholesterolemia (abbreviated FH, also spelled familial hypercholesterolaemia) is a Genetic disorder characterized by high cholesterol The LDL Receptor is a mosaic protein that mediates the Endocytosis of cholesterol-rich LDL. Chromosome 19 is one of the 23 pairs of Chromosomes in Humans People normally have two copies of this chromosome 2% of the population) or the ApoB gene (0. 2%). The familial form is characterized by tendon xanthoma, xanthelasma and premature cardiovascular disease. A xanthoma (or xanthomata or xanthomatosis) (from Greek xanthos, ξανθος, "yellow" is a deposition of yellowish Cholesterol Xanthelasma (or xanthelasma palpebrarum) is a sharply demarcated yellowish collection of Cholesterol underneath the skin usually on or around the Eyelids

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. Metabolic syndrome is a combination of medical disorders that increase the risk of developing Cardiovascular disease and diabetes. The statins (or HMG-CoA reductase inhibitors) form a class of hypolipidemic drugs used to lower Cholesterol levels in people with or at risk of Cardiovascular If the triglyceride level is markedly raised, fibrates may be preferable due to their beneficial effects. In Pharmacology, the fibrates are a class of amphipathic Carboxylic acids They are used for a range of metabolic disorders mainly Hypercholesterolemia 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. In Medicine, a myopathy is a neuromuscular Disease in which the Muscle fibers do not function for any one of many reasons resulting in Muscular weakness Rhabdomyolysis is the rapid breakdown ( Lysis) of Skeletal muscle tissue ( rhabdomyo) due to injury to muscle tissue Other agents commonly added to statins are ezetimibe, niacin and bile acid sequestrants. Ezetimibe (ɛˈzÉ›tÉ™mɪb is an anti-hyperlipidemic medication which is used to lower cholesterol levels Niacin, also known as vitamin B3, is a water-soluble vitamin which prevents the deficiency disease Pellagra. The bile acid sequestrants are a group of medications used for binding certain components of Bile in the gastrointestinal tract. There is some evidence for benefit of plant sterol-containing products and ω₃-fatty acids^[2]

Hyperlipoproteinemia type III

This form is due to high chylomicrons and IDL (intermediate density lipoprotein). n −3 fatty acids (popularly referred to as ω−3 fatty acids or omega-3 fatty acids) are a family of unsaturated Fatty acids that Chylomicrons are large lipoprotein particles that transport dietary lipids from the intestines to other locations in the body 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. (more properly known as, TAG or triacylglyceride) is Glyceride in which the Glycerol is Esterified with three Fatty acids It is the It is also known as hypertriglyceridemia (or pure hypertriglyceridemia). In Medicine, hypertriglyceridemia (or "Hypertriglyceridaemia" denotes high ( hyper-) blood levels ( -emia) of Triglycerides the most According to the NCEP-ATPIII definition of high triglycerides (>200 mg/dl), prevalence is about 16% of adult population. ^[3]

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%)

References

External links

• The Fredrickson papers (with photos from early lipoprotein research)
• 745209914 at GPnotebook

Hyperlipoproteinemia	OMIM	GPnotebook	WebMD	Others
Type I	Online 'Mendelian Inheritance in Man' (OMIM) 238600	-1389035478 at GPnotebook	. GPnotebook is a British medical database for General practitioners (GPs The Mendelian Inheritance in Man project is a Database that catalogues all the known Diseases with a genetic component, and—when possible—links them GPnotebook is a British medical database for General practitioners (GPs	MeritCare
Type IIa	Online 'Mendelian Inheritance in Man' (OMIM) 144400	-1664090094 at GPnotebook	. The Mendelian Inheritance in Man project is a Database that catalogues all the known Diseases with a genetic component, and—when possible—links them GPnotebook is a British medical database for General practitioners (GPs	Merck
Type IIb		-1375338454 at GPnotebook	. GPnotebook is a British medical database for General practitioners (GPs
Type III	.	630849560 at GPnotebook	WebMD	Yahoo
Type IV	Online 'Mendelian Inheritance in Man' (OMIM) 144600	-1362100182 at GPnotebook	WebMD	Yahoo
Type V	Online 'Mendelian Inheritance in Man' (OMIM) 144600	-1355481046 at GPnotebook	. GPnotebook is a British medical database for General practitioners (GPs The Mendelian Inheritance in Man project is a Database that catalogues all the known Diseases with a genetic component, and—when possible—links them GPnotebook is a British medical database for General practitioners (GPs The Mendelian Inheritance in Man project is a Database that catalogues all the known Diseases with a genetic component, and—when possible—links them GPnotebook is a British medical database for General practitioners (GPs	.

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