Hyperlipoproteinemia: Difference between revisions
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| rowspan="13" |'''Secondary Causes of<br> Hyperlipoprotenemia''' | |||
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! rowspan="1" |Type I | |||
! rowspan="1" |Type IIA | |||
! rowspan="1" |Type IIB | |||
! rowspan="1" |Type III | |||
! rowspan="1" |Type IV | |||
! rowspan="1" |Type V | |||
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Revision as of 19:57, 17 November 2016
Hyperlipoproteinemia Microchapters |
ACC/AHA Guideline Recommendations |
Intensity of statin therapy in primary and secondary prevention |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Tarek Nafee, M.D. [2]
Overview
Classification
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 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||
Synopsis
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 |
Differential Diagnosis
Diseases | Mode of Inheritance | Laboratory Findings | Other Findings | Management | Complications | Prognosis | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Lipid Profile | Other Laboratory Findings | |||||||||||||
Total Cholesterol | LDL | HDL | Triglycerides | Plasma Appearance | Chylomicrons | VLDL | Genetic mutations | |||||||
Primary Hyperlipoprotenemia | Type I | Autosomal Recessive
& Autosomal Dominant(Rare) |
Normal or ↑ | ↓ | ↓↓↓ | ↑↑↑↑ | Milky | ↑↑↑↑ | ↓ | -LPL gene mutation | -Fat tolerance markedly abnormal
-Carbohydrate inducibility may be abnormal |
Treatment for hyperlipoproteinemia type 1 is intended to control blood triglyceride levels with a very low-fat diet | -Recurrent Pancreatitis
-Rarely life threatening |
Good |
Type IIA | ||||||||||||||
Type IIB | ||||||||||||||
Type III | ||||||||||||||
Type IV | Autosomal Recessive
& Autosomal Dominant |
Normal or ↑ | Prebeta-HDL ↑
& HDL-C ↓ |
↑↑ | Clear or Cloudy | Normal | ↑ | -LPL genes (Gly188Glu,Asp9Asn, Asn291Ser,Ser447Ter)
-APOA5 -LMF1 -GPIHBP1 |
Hyperglycemia, Pancytopneia and pseudo-Niemann
pick cells |
-Weight reduction
-Niacin or Fibrates -Gene therapy |
-Ischemic Heart Disea
-Recurrent Pancreatitis -NIDDM -NAFLD |
|||
Type V | Variable | ↑ to ↑↑ | ↓ | ↓↓↓ | ↑↑↑↑ | Creamy supernatant and turbid infranatant | ↑ | ↑↑↑ | ❑ Apo E, Apo A5 mutations ❑ LPL gene mutation in 10% of western population patients |
❑ Restriction of dietary fat eliminates Chylomicrons and reverts to type IV HLP ❑ When triglyceride levels are >1000mg/dl given the rarity of type I it is almost always type V HLP |
❑ Weight reduction ❑ Niacin or Fibrates or Strong statins ❑ Low fat diet |
❑ Recurrent Pancreatitis | ❑ Good |
Secondary Causes of Hyperlipoprotenemia |
Type I | Type IIA | Type IIB | Type III | Type IV | Type V | |
---|---|---|---|---|---|---|---|
Diabetes Mellitus | |||||||
Alcohol Abuse | |||||||
Estrogen Therapy | |||||||
Glucocorticoid therapy | |||||||
Renal Disease | |||||||
Obesity | |||||||
High-fat diet | |||||||
Poor physical activity | |||||||
Paraproteinemic disorders | |||||||
Hypothyroidism |
Diagnostic Algorithm
Shown below is a diagnostic algorithm to diagnose hyperlipidemia.[1]
Hyperlipidemia | |||||||||||||||||||||||||||||||||||||||||
Triglycerides > 75th Percentile | NO | Type IIa | |||||||||||||||||||||||||||||||||||||||
YES | |||||||||||||||||||||||||||||||||||||||||
Types I, IIb, IV, V | |||||||||||||||||||||||||||||||||||||||||
Total Cholesterol/Apo B ratio ≥ 6.2 | NO | Types IIb, IV | |||||||||||||||||||||||||||||||||||||||
YES | |||||||||||||||||||||||||||||||||||||||||
Types I, III, V | |||||||||||||||||||||||||||||||||||||||||
Triglycerides/Apo B ratio < 10.0 | NO | Types I, V | |||||||||||||||||||||||||||||||||||||||
YES | |||||||||||||||||||||||||||||||||||||||||
Type III | |||||||||||||||||||||||||||||||||||||||||
References
- ↑ Sniderman A, Tremblay A, Bergeron J, Gagné C, Couture P (2007). "Diagnosis of type III hyperlipoproteinemia from plasma total cholesterol, triglyceride, and apolipoprotein B". Journal of Clinical Lipidology. 1 (4): 256–63. doi:10.1016/j.jacl.2007.07.006. PMID 21291689. Retrieved 2012-10-24. Unknown parameter
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