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Hyperlipoproteinemia type IV

Hyperlipoproteinemia is the presence of elevated levels of lipoprotein 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. more...

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Although the terms hyperlipoproteinemia and hypercholesterolemia are often used interchangeably, the former is more specific. The term "hyperchylomicronemia" is used for an excess of chylomicrons.

Hyperlipoproteinemias are classified according to the Fredrickson/WHO classification (Fredrickson et al 1967), which is based on the pattern of lipoproteins on electrophoresis or ultracentrifugation.

Hyperlipoproteinemia type I

This very rare form (also known as "Buerger-Gruetz syndrome", "Primary hyperlipoproteinaemia", or "familial hyperchylomicronemia"), is due to high chylomicrons, the particles that transfer fatty acids from the digestive tract to the liver.

Hyperlipoproteinemia type II

Hyperlipoproteinemia Type II is hyperlipidemia (hypercholesterolemia) in the Fredrickson classification, which is determined by lipoprotein electrophoresis.

Hyperlipoproteinemia type II is further classified into:

  • Type IIa (elevated LDL only)
    • Polygenic hypercholesterolaemia
    • Familial hypercholesterolemia (FH)
  • Type IIb - combined hyperlipidemia (elevated LDL and VLDL, leading to high triglycerides levels)
    • Familial combined hyperlipoproteinemia
    • Secondary combined hyperlipoproteinemia

Hyperlipoproteinemia type III

This form is due to high chylomicrons and IDL (intermediate density lipoprotein).

Hyperlipoproteinemia type IV

This form is due to high triglycerides. It is also known as "hyperglyceridemia" (or "pure hyperglyceridemia".

Hyperlipoproteinemia type V

This type is very similar to Type I, but with high VLDL.

Unclassified forms

Non-classified forms are extremely rare:

  • Hypo-alpha lipoproteinemia
  • Hypo-beta lipoproteinemia

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Fish oils in hypertriglyceridemia - Fish Oils Revisited
From Nutrition Research Newsletter, 4/1/90

FISH OILS IN HYPERTRIGLYCERIDEMIA Earlier studies have documented dramatic decreases in triglyceride levels in hypertriglyceridemic subjects receiving fish oils containing n-3 fatty acids. In a study conducted at the Lipid Disorders Clinic at the Oregon Health Sciences University, researchers studied the effects of three different supplemental doses of n-3 fatty acids given to 11 subjects (five with type IV, five with type IIb, and one with type III hyperlipoproteinemia) for three six-week periods. The study was preceded by a three-week baseline period, and supplemented periods were separated by four-week washout periods. The doses of fish oils used were 15, 25, or 40 ml daily, equivalent to 4.5,7.5, and 12g of n-3 fatty acids. A single 24-hour dietary recall showed that total dietary intake of cholesterol (including that from the fish oil) ranged from 188 to 259 mg/day. The ratio of polyunsaturated to saturated fatty acids (P:S) of the fish-oil supplement was 1.1. Plasma very-low-density lipoprotein (VLDL) cholesterol and total triglycerides decreased progressively as the amount of fish oil increased in the patients with Type IV and Type IIb hyperlipidemia. Low-density lipoprotein (LDL) increased concurrently, but the increase was greater in patients with Type IV hyperlipidemia. The combined results for all 10 patients showed a 14% decrease in total cholesterol concentrations, produced by a 54% decline in VLDL, a 9% increase in LDL concentrations, and a 6% decrease in HDL concentrations. Triglyceride concentrations decreased by 53%. Bleeding times increased only at the highest dose. Patients gained weight appropriate for the increased calories from added n-3 fatty acids. The one patient with Type III hyperlipoproteinemia "responded dramatically" to the n-3 supplements: his VLDL cholesterol concentrations decreased from 6.55 mmo1/L at baseline to 2.45, 1.30, and 0.70 mmo1/L with increasing doses, and were associated with a normalization of his ratio of VLDL cholesterol to total plasma triglyceride. Based on the results of this study, the authors conclude that the lowest dose (15 ml) is potentially the most effective in terms of lipid changes per gram of n-3 fatty acids, and that the effects of the fish-oil supplement result entirely from a decrease in VLDL concentrations. As they observe, "it is somewhat paradoxical that an agent that reduces the synthesis of a precursor (VLDL) can simultaneously increase the concentrations of the product (LDL)." William S Harris et al, Fish Oils in Hypertriglyceridemia: A Dose-Response Study, American J Clinical Nutrition 51(3):399406 (Mar 1990) [Reprints: William S Harris, Lipid and Arteriosclerosis Prevention Clinic, 3800 Cambridge Street, University of Kansas Medical Center, Kansas City KS 66103]*

COPYRIGHT 1990 Frost & Sullivan
COPYRIGHT 2004 Gale Group

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