Lipoprotein disorders classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Lipoprotein Disorders Microchapters

Patient Information

Overview

Causes

Classification

Hyperlipoproteinemia
Hypolipoproteinemia

Treatment

Overview

Lipoprotein disorders can be classified according to the Fredrickson classification which is based on the pattern of lipoproteins on electrophoresis or ultracentrifugation.[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. It remains a popular system of classification, but is considered outdated by many.

Classification

There are several ways in which lipoprotein abnormalities are classified. Lipoprotein disorders can be classified according to:

  • The pattern of change in the lipoprotein levels, described as hyperlipidemia (increase in lipid levels) and hypolipidemia (decrease in lipid levels): However, this classification is problematic because the lipids and lipoproteins levels in some situation can be elevated in some types of lipoproteins and lipids and decreased in others.
  • Phenotype, or the specific type of lipid that is increased, as classified by Fredrickson: This classification is problematic because it does not include abnormalities in the level of HDL.
  • Etiology, as primary (genetic) or secondary to another condition: This classification can be problematic, because most conditions involve the intersection of genetics and lifestyle issues. However, there are a few well defined genetic conditions that are usually easy to identify.
  • Levels of measured lipids (cholesterol and triglycerides), described as hypercholesterolemia and hypocholesterolemia or hypertriglyceridimia and hypotriglyceridemia: This distinction is not specific because it does not reflect the specific lipoprotein(s) that are abnormally high or low.

Fredrickson Classification of Hyperlipoproteinemia/Hyperlipidemia

 
 
 
 
 
 
 
 
 
 
 
 
 
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
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Type A
 
Type B
 
Type C
 
 
 
 


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, 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

Unclassified forms

Non-classified forms are extremely rare:

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

Classification According to Etiology

 
 
 
 
 
 
 
 
 
 
Lipoprotein/Lipid disorders
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary
(Genetic)
 
 
 
 
 
 
 
 
 
 
 
 
 
Secondary
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
LDL
 
Chylomicron Remnants
 
Lipoproteins Rich in Triglyceride
(Chylomicrons, VLDL, IDL)
 
HDL
 
Multiple lipoproteins
 
Alcohol
Diabetes
Drugs
Liver disease
Obesity
Renal disease
Smoking
Thyroid
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
High LDL:
-Familial hypercholesterolemia
-Familial defective apo B 100
-Autosomal dominant hypercholesterolemia (PCSK9)
-Autosomal recessive hypercholesterolemia
-Familial sistosterolemia
-Familial lipoprotein a lipoproteinemia

Low LDL:
-Abetalipoproteinemia
-Hypobetalipoproteinemia
-PCSK 9 deficiency
 
-Deficiency in hepatic lipase
-Type III dysbetalipoproteinemia
 
-Deficiency in lipoprotein lipase
-Deficiency in Apo C-II
-Deficiency in Apo A-V
-Familial combined hyperlipidemia
-Familial hypertriglyceridemia
- Chylomicron retention disease
 
High LDL:
-Cholesteryl ester transferase protein deficiency

Low HDL:
-Deficiency in Apo A-I
-Deficiency in lecithin cholesterol acyltransferase (LCAT)
-Familial hypoalphalipoproteinemia
-Nieman-Pick disease
-Tangier disease
 
- Familial combined hypolipidemia (ANGPTL3)

Classification According to Laboratory Results

 
 
 
 
 
 
 
 
 
 
 
 
 
 
Lipid Laboratory Tests
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Total cholesterol
 
 
 
 
 
LDL-C
 
 
 
 
 
HDL-C
 
 
 
 
 
Triglycerides
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
High total cholesterol
 
Low total cholesterol
 
High LDL
 
Low LDL
 
High HDL
 
Low HDL
 
High triglyceride
 
Low triglyceride

External links

References

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


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