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| [[Dyslipidemia resident survival guide|'''Resident'''<br>'''Survival'''<br>'''Guide''']]
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{{Lipoprotein disorders}}
{{Lipoprotein disorders}}
{{CMG}}; {{AE}} {{Rim}}
{{CMG}}; {{AE}} {{Rim}}, {{HP}}, {{TarekNafee}}, {{PTD}}, {{AKI}}, {{USAMA}}, {{SCh}}, {{VD}}
 
{{SK}} Dyslipidemia, dyslipoproteinemia, hyperlipidemia


{{SK}}
==Overview==
==Overview==
Lipoproteins, which are aggregates of proteins and lipids, allow the circulation of hydrophobic lipids in the body. Disorders of lipids and lipoproteins metabolism have important health consequences, mainly on the cardiovascular system. Lipoprotein disorders can be described as abnormalities in the level of the lipids, which include cholesterol and triglycerides, or as abnormalities in the levels of lipoproteins that include LDL, HDL, VLDL and chylomicrons.
[[Lipoproteins]] are aggregates of proteins and lipids that facilitate the circulation of [[hydrophobic]] lipids in the body. Disorders of [[Lipid|lipids]] and [[Lipoprotein|lipoproteins]] metabolism have important health consequences, primarily on the cardiovascular system; however, may also affect the cerebrovascular system as well as the gastrointestinal system. Lipoprotein disorders can be described as abnormalities in the level of the lipids, which include cholesterol and triglycerides, or as abnormalities in the levels of lipoproteins that include [[Low density lipoprotein|LDL]], [[High density lipoprotein|HDL,]] [[Very low density lipoprotein|VLDL]] and [[chylomicrons]].
 
Lipoprotein disorders have been initially classified in 1967 into different phenotypes by Fredrickson according to the type of lipoproteins that accumulate. However; Fredrickson's classification of hyperlipoproteinemias took into consideration the elevation in chylomicrons, LDL, VLDL but did not include abnormalities in HDL levels.  Other classifications have been suggested, one of which is the National Cholesterol Education Program (NCEP) classification of lipoprotein disorders.  NCEP classifies lipid disorders according to laboratory cut off points for the levels of total cholesterol, LDL-C and HDL.
 
Lipoprotein disorders can be classified according to different criteria.  First of all, lipoprotein disorders can be classified as primary disorders resulting from genetic mutations and secondary to other diseases.  Another way of classifying lipoprotein disorders is as hypolipidemia (or hypolipoproteinemia) and hyperlipidemia (hyperlipoproteinemia) where the lipoprotein levels are decreased and increased respectively.  However, the latter classification is not precise and creates some ambiguity, because some people can be labeled as having hyperlipidemia but have simultaneously high level of some lipoproteins and low levels of other lipoproteins depending on the underlying pathophysiology.  Hence, a better term to describe the constellation of abnormal lipid profiles is "disorders of lipoproteins", or dyslipoproteinemia or dyslipidemia.
 
==Definitions==
In order to understand the different lipoprotein disorders, it is important to correctly define the keywords used to define them.
 
* Hyperlipidemia: Hyperlipidemia is a commonly used term that describes high level of lipids, whether cholesterol or triglycerides.  Despite being commonly used to describe abnormalities in lipid metabolism in our daily practice, this term is not very specific.  For instance, people who have metabolic syndrome have high LDL and low HDL and yet they are described to have hyperlipidemia when their HDL level is decreased.
 
* Hyperlipoproteinemia: Hyperlipoproteinemia is the elevation of lipoproteins, the aggregates of lipids and proteins. Hyperlipoproteinemia is the term used by Fredrickson in his classification of lipoprotein disorders.  Hyperlipoproteinemia can be used interchangeably with hyperlipidemia in some instances, however, they are not synonyms.  In fact, some lipoprotein disorders can have elevated or decreased level of lipoproteins while the toltal cholesterol level is within normal ranges.
 
* Dyslipoproteinemia: Dyslipoproteinemia describes abnormalities of lipoproteins.  Dyslipoproteinemia reflects the lipoprotein disorders better than hyperlipoproteinemia since is does not limited these disorders only to cases where some types of lipoproteins are elevated.
 
* Dyslipidemia: Dyslipidemia is the most general term that can describe lipoprotein and lipid disorders since the definition includes variation in the levels of lipoproteins, total cholesterol and triglycerides.
 
==Lipid Profile==
The lipid profile measured in daily clinical practice includes the following variables:
* Total cholesterol
* Triglycerides
* HDL cholesterol
* LDL cholesterol, which is calculated by the formula: LDL = Total cholesterol - HDL - (Triglycerides/5)
 


Formula:  Total cholesterol= HDL + LDL + Triglycerides/5
Lipoprotein disorders (also referred to as ''Lipid disorders, or Dyslipidemias, or Dyslipoproteinemias'') were first classified in 1967 into different phenotypes by Fredrickson according to the type of [[lipoproteins]] that are affected. This approach is considered outdated for a number of reasons. Firstly, Friedrickson's classification failed to classify disorders of [[Hypolipoproteinemia|low lipids]]. Secondly, Fredrickson's classification of [[hyperlipoproteinemia]]<nowiki/>s took into consideration the elevation in [[chylomicrons]], [[LDL]], [[Very low density lipoprotein|VLDL]] but did not include abnormalities in [[HDL Cholesterol|HDL]] levels. Other classifications have been suggested, one of which is the National Cholesterol Education Program (NCEP) classification of lipoprotein disorders. NCEP classifies lipid disorders according to laboratory cut off points for the levels of total cholesterol, [[LDL-C]] and [[High-density lipoprotein|HDL]].
According to the previous formula the measured level of total cholesterol reflects changes in LDL, triglycerides and HDL.


Lipoproteins are composed of a protein part, the apolipoprotein, and the lipid part which includes cholesterol, triglycerides and fatty acids. The lipoproteins differ among each other in terms of density and size as a result of difference in the percentage of each components. While VLDL, chylomicrons and IDL are rich in triglycerides, LDL is rich in cholesterol. Hence, abnormalities in measured lipid levels can be simplified as follows:
Lipoprotein disorders must be initially classified broadly into [[Hypolipoproteinemia|hypolipidemias]] and [[Hyperlipoproteinemia|hyperlipidemias]] corresponding to low or high [[lipid]] levels, respectively. Each of these broad categories may be further classified into primary (genetic) causes or secondary environmental causes (e.g. [[substance abuse]], medication use, lifestyle habits, or underlying diseases, etc.). Secondary causes of lipid disorders are more common and thus must be ruled out before exploring the primary causes of dyslipidemia.
* Hypercholesterolemia, defined as high level of measured cholesterol, is reflected by high LDL.
* Hypertriglyceridemia, defined as high level of measured triglycerides, is reflected by high VLDL and/or IDL and/or chylomicrons.
* Combined hyperlipidemia, defined as high levels of measured cholesterol and triglycerides, is due to elevation in both LDL and VLDL and/or IDL and/or chylomicrons.


==Classification==
Primary dyslipidemias are generally consistent in the way they affect the [[lipoproteins]]. [[Hyperlipoproteinemia|Hyperlipoproteinemias]] generally cause elevations in the affected lipids/lipoproteins and [[hypolipoproteinemia]]<nowiki/>s generally cause reductions in the affected lipids/lipoproteins. Secondary dyslipidemias, on the other hand, may cause elevations in some lipoproteins and reductions in others. An example of this is the [[lipid profile]] in patients with [[diabetes mellitus]], which commonly reveals a dyslipidemic triad consisting of elevated [[Low density lipoprotein|LDL]] and [[triglycerides]] with a concurrent reduction in [[High density lipoprotein|HDL]].<ref name="pmid20524075">{{cite journal| author=Musunuru K| title=Atherogenic dyslipidemia: cardiovascular risk and dietary intervention. | journal=Lipids | year= 2010 | volume= 45 | issue= 10 | pages= 907-14 | pmid=20524075 | doi=10.1007/s11745-010-3408-1 | pmc=2950930 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20524075  }} </ref><ref name="pmid16259526">{{cite journal| author=Nesto RW| title=Beyond low-density lipoprotein: addressing the atherogenic lipid triad in type 2 diabetes mellitus and the metabolic syndrome. | journal=Am J Cardiovasc Drugs | year= 2005 | volume= 5 | issue= 6 | pages= 379-87 | pmid=16259526 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16259526  }} </ref> In this case, [[diabetes mellitus]] and [[metabolic syndrome]] would be widely considered a hyperlipidemic disease due to the array of cardiovascular and cerebrovascular sequelae that arise consequent to the chronic hyperlipidemia associated with the disease.<ref name="pmid16259526">{{cite journal| author=Nesto RW| title=Beyond low-density lipoprotein: addressing the atherogenic lipid triad in type 2 diabetes mellitus and the metabolic syndrome. | journal=Am J Cardiovasc Drugs | year= 2005 | volume= 5 | issue= 6 | pages= 379-87 | pmid=16259526 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16259526  }} </ref> This is to say that in cases of mixed (elevated and decreased) findings on the [[lipid profile]], clinicians must evaluate the secondary causes of the dysipidemia and manage the affected lipoproteins accordingly.
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.
==Causes==
'''For a complete list of causes of hyperlipoproteinemia click [[hyperlipoproteinemia causes|here]].'''<br>
'''For a complete list of causes of hypolipoproteinemia click [[hypolipoproteinemia causes|here]].'''


* 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.
The table below summarizes is a list of primary lipoprotein disorders:


===Fredrickson Classification of Hyperlipoproteinemia===
{| class="wikitable sortable" style="font-size:90%"
{{familytree/start |summary=Hyperlipoproteinemia}}
!Type of lipoprotein disorder
{{familytree | | | | | | | | | | | | | | A01 | | | | | | | | | | | | | A01= '''Hyperlipoproteinemia'''}}
! '''Genetic Lipoprotein Disorder'''
{{familytree | | | | | |,|-|-|-|-|v|-|-|-|+|-|-|-|v|-|-|-|.| | | | }}
! '''Gene Involved'''
{{familytree | | | | | D01 | | | D02 | | D03 | | D04 | | D05 | | D01= '''Type I:'''<br> Familial hyperchylomicronemia| D02= '''Type II'''| D03= '''Type III:'''<br>[[Dysbetalipoproteinemia]]| D04= '''Type IV:'''<br>[[Primary hypertriglyceridemia]]<br>| D05= '''Type V:''' <br>[[Mixed hyperlipoproteinemia]]}}
! '''Main Lipoprotein Involved'''
{{familytree | | | | | |!| | |,|-|^|-|.| | |}}
|-
{{familytree | | | | | |!| | E01 | | E02 | | E01= '''Type A:'''<br> Familial hypercholesterolemia| E02= '''Type B:'''<br> Familial combined hyperlipidemia}}
| rowspan="9" |[[Hyperlipoproteinemia|Hyperlipoproteinemias]]
{{familytree | |,|-|-|-|+|-|-|-|.| | | | | | |}}
| Autosomal dominant hypercholesterolemia || [[PCSK9]] || [[LDL]]
{{familytree | F01 | | F02 | | F03 | | | | | F01= Type A| F02= Type B| F03= Type C}}
|-
{{familytree/end}}
| Autosomal recessive hypercholesterolemia || ARH || [[LDL]]
|-
| Familial sitosterolemia || [[ABCG5]] - [[ABCG8]]|| [[LDL]]
|-
| Familial lipoprotein(a) hyperlipoproteinemia || [[APOA2|Apo(a)]]|| [[LDL]]
|-
| Familial defective apo B100|| [[Apo B]] ||[[LDL]]
|-
| Hepatic lipase deficiency|| [[Hepatic lipase|HL]] ||
|-
| [[Lipoprotein lipase deficiency]] || [[LPL]]|| [[Chylomicron]]
|-
| Apo C-II deficiency|| [[Apo C-II]] || [[Chylomicron]]
|-
| Apo A-V deficiency || [[Apo A-V]] deficiency || [[Chylomicron]]
|-
| rowspan="5" |[[Hypolipoproteinemia|Hypolipoproteinemias]]
| [[Familial hypoalphalipoproteinemia]]|| ||
|-
| GPIHBP1 deficiency|| [[GPIHBP1]]|| [[Chylomicron]]
|-
| [[Apo A-I]] deficiency|| [[Apo A-I]] || [[HDL]]
|-
| [[CETP]] deficiency || [[CETP]]|| [[HDL]]
|-
| [[Niemann-Pick disease]]|| SMPD1, NPC1|| [[HDL]]
|}


===Classification According to Etiology===
== Classification ==
{{familytree/start |summary=Lipoprotein Disorders}}
{{familytree/start |summary=Lipid disorders}}
{{familytree | | | | | | | | | | | | | | | | A01 | | | | | | || A01= '''Lipoprotein Disorders'''}}
{{familytree | | | | | | | | | | | | | | | | A01 | | | | | | | A01= '''Lipid disorders'''}}
{{familytree | | | | | | | | | |,|-|-|-|-|-|-|^|-|-|-|-|-|-|.| }}
{{familytree | | | | | | | |,|-|-|-|-|-|-|-|-|^|-|-|-|-|-|-|-|-|.| | | | | | }}
{{familytree | | | | | | | | | B01 | | | | | | | | | | | | B02 | B01= '''Primary'''<br>(Genetic)| B02= '''Secondary'''}}
{{familytree | | | | | | | B01 | | | | | | | | | | | | | | | | B02 | B01= '''[[Hypolipoproteinemia]]'''| B02= '''[[Hyperlipoproteinemia]]'''}}
{{familytree | | | |,|-|-|-|-|-|+|-|-|-|v|-|-|-|-|-|.| | | |!| | | }}
{{familytree | | | | |,|-|-|^|-|-|-|.| | | | | | | | | | |,|-|-|^|-|-|-|.| }}
{{familytree | | | C01 | | | | C02 | | C03 | | | | C04 | | C05 | C01= '''LDL'''| C02= '''Chylomicron Remnants'''| C03= '''Lipoproteins Rich in Triglyceride'''<br> '''(Chylomicrons, VLDL, IDL)'''| C04= '''HDL'''| C05= [[Alcohol]] <br> [[Diabetes]] <br> [[Drug]]s <br> [[Liver disease]] <br> [[Obesity]] <br> [[Renal disease]] <br> [[Smoking]] <br> [[Thyroid]]}}
{{familytree | | | | C01 | | | | | C02 | | | | | | | | | C03 | | | | | C04 | |C01=Primary|C02=Secondary|C03=Primary|C04=Secondary}}
{{familytree | |,|-|^|-|.| | | |!| | | |!| | | |,|-|^|-|.| | | }}
{{familytree | |,|-|-|^|-|-|.| | | |!| | | | | | | | | | |!| | | | | | |!| | | | | }}
{{familytree | D01 | | D02 | | D03 | | D04 | | D05 | | D06 | D01= '''High LDL:''' <br> - Familial hypercholesterolemia <br> - Familial defective apo B 100 <br> - Autosomal dominant hypercholesterolemia (PCSK9) <br> - Autosomal recessive hypercholesterolemia <br> - Familial sistosterolemia <br> - Familial lipoprotein a lipoproteinemia| D02= '''Low LDL:''' <br> - Abetalipoproteinemia <br> - Hypobetalipoproteinemia| D03= - Deficiency in hepatic lipase<br> - Type III dysbetalipoproteinemia| D04= - Deficiency in lipoprotein lipase<br> - Deficiency in Apo C-II <br> - Deficiency in Apo A-V <br> - Familial combined hyperlipidemia<br> - Familial hypertriglyceridemia| D05= '''High LDL''':<br> - Cholesteryl ester transferase protein deficiency| D06= '''Low HDL:''' <br> - Deficiency in Apo A-I<br> - Tangier disease<br> - Deficiency in lecithin cholesterol acyltransferase (LCAT) <br> - Nieman-Pick disease}}
{{familytree | D01 | | | | D02 | | D03 | | | | | | | | | |!| | | | | | D04 | D01=Low LDL| D02=Low HDL | D03=[[Anemia]],<br> [[Chronic inflammation]],<br> [[Chronic liver disease]],<br> [[Hyperthyroidism]], <br>[[Infection]],<br> [[Malabsorption]],<br> [[Malignancy]],<br> Criticial illness,<br>'''[[Hypolipoproteinemia causes|More Causes...]]'''| D04= [[Alcohol]],<br> [[Diabetes]],<br> [[Drug]]s, <br> [[Liver disease]],<br>[[Obesity]],<br> [[Renal disease]],<br>[[Smoking]],<br>[[Hypothyroidism]]<br>'''[[Hyperlipoproteinemia causes|More Causes...]]''' }}
{{familytree | |!| | | | | |!| | | | | | |,|-|-|-|v|-|-|-|+|-|-|-|v|-|-|-|.| | | }}
{{familytree | E01 | | | | E02 | | | | | E03 | | E04 | | E05 | | E06 | | E07 | |E01=[[Abetalipoproteinemia]]<br>[[Hypobetalipoproteinemia]]<br> [[PCSK9 deficiency]]<br> [[Chylomicron retention disease]]<br>Familial combined hypolipidemia|E02=[[LCAT]] deficiency,<br> Apolipoprotein 1 deficiency,<br> Familial hypoalphalipoproteinemia,<br>[[Tangier disease]], FISH eye disease, Familial combined hypolipidemia|E03='''Type I:'''<br> [[Familial hyperchylomicronemia]]|E04='''Type II'''| E05='''Type III:'''<br>[[Dysbetalipoproteinemia]]| E06='''Type IV:'''<br>[[Primary hypertriglyceridemia]]<br>| E07= '''Type V:''' <br>[[Mixed hyperlipoproteinemia]]| }}
{{familytree | | | | | | | | | | | | | | | |,|-|-|^|-|-|.| }}
{{familytree | | | | | | | | | | | | | | | F01 | | | | F02 | F01='''Type A:'''<br> [[Familial hypercholesterolemia]]| F02= '''Type B:'''<br> [[Familial combined hyperlipidemia]]}}
{{familytree/end}}
{{familytree/end}}
 
==Treatment==
==Classification According to Laboratory Results==
*To view detailed treatment of dyslipidemia [[Dyslipidemia resident survival guide|click here]].
{{familytree/start |summary= Lipid Laboratory Tests}}
{{familytree | | | | | | | | | | | | | | | A01 | | | |  A01= '''Lipid Laboratory Tests'''}}
{{familytree | | | |,|-|-|-|-|-|-|-|v|-|-|-|^|-|-|-|v|-|-|-|-|-|-|-|.| }}
{{familytree | | | B01 | | | | | | B02 | | | | | | B03 | | | | | | B04 | | | B01= '''Total cholesterol'''| B02= '''LDL-C'''| B03= '''HDL-C'''| B04='''Triglycerides'''}}
{{familytree | |,|-|^|-|.| | | |,|-|^|-|.| | | |,|-|^|-|.| | | |,|-|^|-|.| | }}
{{familytree | C01 | | C02 | | C03 | | C04 | | C05 | | C06 | | C07 | | C08 | C01= High total<br> cholesterol| C02=Low total <br> cholesterol| C03= High LDL| C04= Low LDL| C05= High HDL| C06= Low HDL| C07= High triglycerdies| C08= Low triglycerides}}
{{familytree/end}}
 
==Types==
===Increases===
* [[Hyperlipidemia]]: [[lipids]]
::* [[Hypercholesterolemia]]: [[cholesterol]]
::* [[Hypertriglyceridemia]]: [[triglycerides]]
 
* [[Hyperlipoproteinemia]]: [[lipoproteins]] (usually [[LDL]] unless otherwise specified)
* [[Hyperchylomicronemia]]: [[chylomicrons]]
 
* Combined hyperlipidemia: both [[LDL]] and [[triglycerides]]
 
*[[Familial hypercholesterolemia]] is a specific form of [[hypercholesterolemia]] due to a defect on [[chromosome 19]] (19p13.1-13.3).
 
===Decreases===
* [[Hypolipoproteinemia]]: [[lipoproteins]]
* [[Hypocholesterolemia]]: [[cholesterol]]
* [[Abetalipoproteinemia]]: beta [[lipoproteins]]
* [[Tangier disease]]: [[high density lipoprotein]]
 
==Related Chapters==
*[[Hyperlipidemia]]
*[[Hypercholesterolemia]]
*[[Hypertriglyceridemia]]
*[[Hyperlipoproteinemia]]
 
==References==
==References==
{{Reflist|2}}
{{reflist|2}}
 
{{Lipopedia}}
{{Lipopedia}}
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Disease]]
[[Category:Lipopedia]]
[[Category:Lipids]]
[[Category:Up-To-Date]]
{{WH}}
{{WS}}

Latest revision as of 18:42, 17 December 2020



Resident
Survival
Guide

Lipoprotein Disorders Microchapters

Patient Information

Overview

Causes

Classification

Hyperlipoproteinemia
Hypolipoproteinemia

Treatment

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rim Halaby, M.D. [2], Hardik Patel, M.D., Tarek Nafee, M.D. [3], Prince Tano Djan, BSc, MBChB [4], Aravind Kuchkuntla, M.B.B.S[5], Usama Talib, BSc, MD [6], Shivani Chaparala M.B.B.S [7], Vishal Devarkonda, M.B.B.S[8]

Synonyms and keywords:

Overview

Lipoproteins are aggregates of proteins and lipids that facilitate the circulation of hydrophobic lipids in the body. Disorders of lipids and lipoproteins metabolism have important health consequences, primarily on the cardiovascular system; however, may also affect the cerebrovascular system as well as the gastrointestinal system. Lipoprotein disorders can be described as abnormalities in the level of the lipids, which include cholesterol and triglycerides, or as abnormalities in the levels of lipoproteins that include LDL, HDL, VLDL and chylomicrons.

Lipoprotein disorders (also referred to as Lipid disorders, or Dyslipidemias, or Dyslipoproteinemias) were first classified in 1967 into different phenotypes by Fredrickson according to the type of lipoproteins that are affected. This approach is considered outdated for a number of reasons. Firstly, Friedrickson's classification failed to classify disorders of low lipids. Secondly, Fredrickson's classification of hyperlipoproteinemias took into consideration the elevation in chylomicrons, LDL, VLDL but did not include abnormalities in HDL levels. Other classifications have been suggested, one of which is the National Cholesterol Education Program (NCEP) classification of lipoprotein disorders. NCEP classifies lipid disorders according to laboratory cut off points for the levels of total cholesterol, LDL-C and HDL.

Lipoprotein disorders must be initially classified broadly into hypolipidemias and hyperlipidemias corresponding to low or high lipid levels, respectively. Each of these broad categories may be further classified into primary (genetic) causes or secondary environmental causes (e.g. substance abuse, medication use, lifestyle habits, or underlying diseases, etc.). Secondary causes of lipid disorders are more common and thus must be ruled out before exploring the primary causes of dyslipidemia.

Primary dyslipidemias are generally consistent in the way they affect the lipoproteins. Hyperlipoproteinemias generally cause elevations in the affected lipids/lipoproteins and hypolipoproteinemias generally cause reductions in the affected lipids/lipoproteins. Secondary dyslipidemias, on the other hand, may cause elevations in some lipoproteins and reductions in others. An example of this is the lipid profile in patients with diabetes mellitus, which commonly reveals a dyslipidemic triad consisting of elevated LDL and triglycerides with a concurrent reduction in HDL.[1][2] In this case, diabetes mellitus and metabolic syndrome would be widely considered a hyperlipidemic disease due to the array of cardiovascular and cerebrovascular sequelae that arise consequent to the chronic hyperlipidemia associated with the disease.[2] This is to say that in cases of mixed (elevated and decreased) findings on the lipid profile, clinicians must evaluate the secondary causes of the dysipidemia and manage the affected lipoproteins accordingly.


Causes

For a complete list of causes of hyperlipoproteinemia click here.
For a complete list of causes of hypolipoproteinemia click here.

The table below summarizes is a list of primary lipoprotein disorders:

Type of lipoprotein disorder Genetic Lipoprotein Disorder Gene Involved Main Lipoprotein Involved
Hyperlipoproteinemias Autosomal dominant hypercholesterolemia PCSK9 LDL
Autosomal recessive hypercholesterolemia ARH LDL
Familial sitosterolemia ABCG5 - ABCG8 LDL
Familial lipoprotein(a) hyperlipoproteinemia Apo(a) LDL
Familial defective apo B100 Apo B LDL
Hepatic lipase deficiency HL
Lipoprotein lipase deficiency LPL Chylomicron
Apo C-II deficiency Apo C-II Chylomicron
Apo A-V deficiency Apo A-V deficiency Chylomicron
Hypolipoproteinemias Familial hypoalphalipoproteinemia
GPIHBP1 deficiency GPIHBP1 Chylomicron
Apo A-I deficiency Apo A-I HDL
CETP deficiency CETP HDL
Niemann-Pick disease SMPD1, NPC1 HDL

Classification

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Lipid disorders
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hypolipoproteinemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hyperlipoproteinemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary
 
 
 
 
Secondary
 
 
 
 
 
 
 
 
Primary
 
 
 
 
Secondary
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Low LDL
 
 
 
Low HDL
 
Anemia,
Chronic inflammation,
Chronic liver disease,
Hyperthyroidism,
Infection,
Malabsorption,
Malignancy,
Criticial illness,
More Causes...
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Alcohol,
Diabetes,
Drugs,
Liver disease,
Obesity,
Renal disease,
Smoking,
Hypothyroidism
More Causes...
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Abetalipoproteinemia
Hypobetalipoproteinemia
PCSK9 deficiency
Chylomicron retention disease
Familial combined hypolipidemia
 
 
 
LCAT deficiency,
Apolipoprotein 1 deficiency,
Familial hypoalphalipoproteinemia,
Tangier disease, FISH eye disease, Familial combined hypolipidemia
 
 
 
 
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

Treatment

  • To view detailed treatment of dyslipidemia click here.

References

  1. Musunuru K (2010). "Atherogenic dyslipidemia: cardiovascular risk and dietary intervention". Lipids. 45 (10): 907–14. doi:10.1007/s11745-010-3408-1. PMC 2950930. PMID 20524075.
  2. 2.0 2.1 Nesto RW (2005). "Beyond low-density lipoprotein: addressing the atherogenic lipid triad in type 2 diabetes mellitus and the metabolic syndrome". Am J Cardiovasc Drugs. 5 (6): 379–87. PMID 16259526.