Sandbox:Hyperlipoproteinemia type5: Difference between revisions

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Latest revision as of 18:31, 28 November 2016

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Person 1, Person 2, Your Name

Differentiating Any Disease from other Diseases

Natural History, Complications and Prognosis

A reduced HDL-C level is associate with ASCVD

Diagnosis

Diagnosis of HLP type 5 is usually by clinical presentation.

History (including age of onset of symptomes in the patient and family members).
Physical findings such as eruptive xanthomas and tendon xanthomas.
Laboratory evaluation such as lipid levels and apolipoprotein assays.

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
The evaluation and treatment decisions of type 5 HLP should be based on patient-centered and individual circumstances. Lifestyle therapies, such as appropriate nutrition and physical activity, are important elements of ASCVD risk reduction, with or without lipid-altering drug therapy. For patients in whom lipid-altering drug therapy is indicated, statin treatment is the primary pharmacologic modality for reducing ASCVD risk.

Clinical algorithm for screening and management of elevated TG^[1]

Medical Therapy

When the triglyceride concentration is very high (≥500 mg/dL, and especially if ≥1000 mg/dL), the primary goal of therapy is to reduce the triglyceride level to <500 mg/dL for the intent of reducing the risk of pancreatitis.

Triglyceride concentration	First line of therapy
≥1000mg/dl	Triglyceride lowering agents such as ❑ Fibric acids ❑ High-dose [2 to 4 g/d] long-chain omega-3 fatty acids ❑ Nicotinic acid
500-999mg/dl	❑ Triglyceride lowering agents, ❑ Statins
200-499mg/dl	Statin will generally be first-line drug therapy.If maximum tolerated statin therapy does not lower non-HDL-C below goal levels in patients with triglycerides 200 to 499 mg/dL, adding an agent that primarily lowers triglycerides may help to achieve atherogenic cholesterol goals.

When triglycerides are between 200 and 499mg/dL, the primary targets of lipid therapy are non–HDL-C and LDL-C for the purpose of reducing ASCVD risk.


Lipid treatment goals to reduce ASCVD risk^[1]
Risk of ASCVD	Indication for drug therapy	Goal of drug therapy
Low ASCVD risk	Non HDL- C* level ≥190mg/dl LDL-C level ≥160mg/dl	Non HDL- C level <130mg/dl LDL-C level <100mg/dl
Moderate ASCVD risk	Non HDL- C* level ≥160mg/dl LDL-C level ≥130mg/dl	Non HDL- C level <130mg/dl LDL-C level <100mg/dl
High ASCVD risk	Non HDL- C level ≥130mg/dl LDL-C level ≥100mg/dl	Non HDL- C level <130mg/dl LDL-C level <100mg/dl
Very high ASCVD risk	Non HDL- C level ≥100mg/dl LDL-C level ≥70mg/dl	Non HDL- C level <100mg/dl LDL-C level <70mg/dl

Non–HDL-C comprises the cholesterol carried by all atherogenic particles, including LDL, intermediatedensity lipoproteins, very low-density lipoproteins (VLDL) and VLDL remnants, chylomicron remnants, and lipoprotein.^[2]

Secondary Prevention

Lifestyle interventions are a key to efforts to reduce triglycerides that includes^[1]
- weight loss if overweight or obese
- physical activity (≥ 150 minutes per week of moderate or higher intensity activity)
Restriction of alcohol
Restriction of sugar/refined carbohydrate intakes
For patients with very high TG level (≥500 mg/dL),chylomicronemia will generally be present. For such patients, a low-fat diet (,15% of energy) may be helpful to reduce entry of new chylomicron particles into the circulation.^[1]
For patients with triglycerides <500 mg/dL, partial replacement of dietary carbohydrate (especially sugars and other refined carbohydrates) with a combination of unsaturated fats and proteins may help to reduce the triglyceride and non-HDL-C concentrations.

Case Studies

Case #1

References

Template:WH Template:WS

↑ ^1.0 ^1.1 ^1.2 ^1.3 Jacobson TA, Ito MK, Maki KC, Orringer CE, Bays HE, Jones PH et al. (2015) National lipid association recommendations for patient-centered management of dyslipidemia: part 1--full report. J Clin Lipidol 9 (2):129-69. DOI:10.1016/j.jacl.2015.02.003 PMID: 25911072
↑ Bays HE, Jones PH, Orringer CE, Brown WV, Jacobson TA (2016) National Lipid Association Annual Summary of Clinical Lipidology 2016. J Clin Lipidol 10 (1 Suppl):S1-43. DOI:10.1016/j.jacl.2015.08.002 PMID: 26891998

[pmid25911072-1] 1.0 ^1.1 ^1.2 ^1.3 Jacobson TA, Ito MK, Maki KC, Orringer CE, Bays HE, Jones PH et al. (2015) National lipid association recommendations for patient-centered management of dyslipidemia: part 1--full report. J Clin Lipidol 9 (2):129-69. DOI:10.1016/j.jacl.2015.02.003 PMID: 25911072

[pmid26891998-2] Bays HE, Jones PH, Orringer CE, Brown WV, Jacobson TA (2016) National Lipid Association Annual Summary of Clinical Lipidology 2016. J Clin Lipidol 10 (1 Suppl):S1-43. DOI:10.1016/j.jacl.2015.08.002 PMID: 26891998

[1]

[2]

@@ Line 26: / Line 26: @@
 ==Diagnosis==
+Diagnosis of HLP type 5 is usually by clinical presentation.
+*History (including age of onset of symptomes in the patient and family members).
+*Physical findings such as eruptive xanthomas and tendon xanthomas.
+*Laboratory evaluation such as lipid levels and apolipoprotein assays.
 [[Type page name here history and symptoms|History and Symptoms]] | [[Type page name here physical examination|Physical Examination]] | [[Type page name here laboratory findings|Laboratory Findings]] | [[Type page name here electrocardiogram|Electrocardiogram]] | [[Type page name here chest x ray|Chest X Ray]] | [[Type page name here CT|CT]] | [[Type page name here MRI|MRI]] | [[Type page name here echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Type page name here other imaging findings|Other Imaging Findings]] | [[Type page name here other diagnostic studies|Other Diagnostic Studies]]
 ==Treatment==
-[[Type page name here medical therapy|Medical Therapy]] | [[Type page name here surgery|Surgery]] | [[Type page name here primary prevention|Primary Prevention]] | [[Type page name here secondary prevention|Secondary Prevention]] | [[Type page name here cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Type page name here future or investigational therapies|Future or Investigational Therapies]]
+[[Type page name here medical therapy|Medical Therapy]] | [[Type page name here surgery|Surgery]] | [[Type page name here primary prevention|Primary Prevention]] | [[Type page name here secondary prevention|Secondary Prevention]] | [[Type page name here cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Type page name here future or investigational therapies|Future or Investigational Therapies]]<br>
-This summary should not be interpreted as rules or directives with regard to the most appropriate care of any single patient with dyslipidemia, because no set of recommendations or guidelines can have 100% applicability to an individual patient. Thus, evaluation and treatment decisions should be based on patient-centered, individual circumstances. As such, this document should be used in conjunction with, and not a replacement for the preferences of patients with dyslipidemia and the judgment of their treating clinicians.<ref name="pmid26891998">Bays HE, Jones PH, Orringer CE, Brown WV, Jacobson TA (2016) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=26891998 National Lipid Association Annual Summary of Clinical Lipidology 2016.] ''J Clin Lipidol'' 10 (1 Suppl):S1-43. [http://dx.doi.org/10.1016/j.jacl.2015.08.002 DOI:10.1016/j.jacl.2015.08.002] PMID: [https://pubmed.gov/26891998 26891998]</ref>
+The evaluation and treatment decisions of type 5 HLP should be based on patient-centered and individual circumstances. Lifestyle therapies, such as appropriate nutrition and physical activity, are important elements of ASCVD risk reduction, with or without lipid-altering drug therapy. For patients in whom lipid-altering drug therapy is indicated, statin treatment is the primary pharmacologic modality for reducing ASCVD risk.<br>
-Lifestyle therapies, such as appropriate nutrition and physical activity, are important elements of ASCVD risk reduction, with or without lipid-altering drug therapy.
-For patients in whom lipid-altering drug therapy is indicated, statin treatment is the primary pharmacologic modality for reducing ASCVD risk.
+==== Clinical algorithm for screening and management of elevated TG<ref name="pmid25911072">Jacobson TA, Ito MK, Maki KC, Orringer CE, Bays HE, Jones PH et al. (2015) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=25911072 National lipid association recommendations for patient-centered management of dyslipidemia: part 1--full report.] ''J Clin Lipidol'' 9 (2):129-69. [http://dx.doi.org/10.1016/j.jacl.2015.02.003 DOI:10.1016/j.jacl.2015.02.003] PMID: [https://pubmed.gov/25911072 25911072]</ref> ====
+[[Image:Clinical algorithm for screening and management of elevated TG.jpg|1200px|description]]
 ===Medical Therapy===
-When the triglyceride concentration is very high (≥500 mg/dL, and especially if ≥1000 mg/dL), the primary goal of therapy is to reduce the triglyceride level to , ≥500 mg/dL for the intent of reducing the risk of pancreatitis.
+When the triglyceride concentration is very high (≥500 mg/dL, and especially if ≥1000 mg/dL), the primary goal of therapy is to reduce the triglyceride level to <500 mg/dL for the intent of reducing the risk of pancreatitis.
-When triglycerides are between 200 and 499mg/dL, the primary targets of lipid therapy are non–HDL-C and LDL-C for the purpose of reducing [[ASCVD]] risk.
+{| border="2" cellpadding="4" cellspacing="0" style="margin: 1em 1em 1em 0; background: #f9f9f9; border: 1px #aaa solid; border-collapse: collapse;" width="75%"
+! '''Triglyceride concentration''' !! '''First line of therapy'''
-'''Lipid treatment goals''' to reduce ASCVD risk<ref name="pmid26891998">Bays HE, Jones PH, Orringer CE, Brown WV, Jacobson TA (2016) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=26891998 National Lipid Association Annual Summary of Clinical Lipidology 2016.] ''J Clin Lipidol'' 10 (1 Suppl):S1-43. [http://dx.doi.org/10.1016/j.jacl.2015.08.002 DOI:10.1016/j.jacl.2015.08.002] PMID: [https://pubmed.gov/26891998 26891998]</ref>
+|-
+| ≥1000mg/dl
+| Triglyceride lowering agents such as <br>❑ Fibric acids<br> ❑ High-dose [2 to 4 g/d] long-chain omega-3 fatty acids<br> ❑ Nicotinic acid
+|-
+| 500-999mg/dl
+| ❑ Triglyceride lowering agents,<br> ❑ Statins
+|-
+| 200-499mg/dl
+| Statin will generally be first-line drug therapy.If maximum tolerated statin therapy does not lower non-HDL-C below goal levels in patients with triglycerides 200 to 499 mg/dL, adding an agent that primarily lowers triglycerides may help to achieve atherogenic cholesterol goals.
+|}<br clear="left" />
+When triglycerides are between 200 and 499mg/dL, the primary targets of lipid therapy are non–HDL-C and LDL-C for the purpose of reducing [[ASCVD]] risk.<br>
 {| class="wikitable" Left|style="margin: 1em auto 1em auto"
+|+
+! colspan="3" |'''Lipid treatment goals''' to reduce ASCVD risk<ref name="pmid25911072">Jacobson TA, Ito MK, Maki KC, Orringer CE, Bays HE, Jones PH et al. (2015) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=25911072 National lipid association recommendations for patient-centered management of dyslipidemia: part 1--full report.] ''J Clin Lipidol'' 9 (2):129-69. [http://dx.doi.org/10.1016/j.jacl.2015.02.003 DOI:10.1016/j.jacl.2015.02.003] PMID: [https://pubmed.gov/25911072 25911072]</ref>
 |+
 ! '''Risk of ASCVD''' || '''Indication for drug therapy''' || '''Goal of drug therapy'''
+|-
+|'''Low''' ASCVD risk  || Non HDL- C* level ≥190mg/dl <br> LDL-C level ≥160mg/dl || Non HDL- C level <130mg/dl <br> LDL-C level <100mg/dl
 |-
 | '''Moderate''' ASCVD risk  || Non HDL- C* level ≥160mg/dl <br> LDL-C level ≥130mg/dl || Non HDL- C level <130mg/dl <br> LDL-C level <100mg/dl
@@ Line 49: / Line 68: @@
 |}
 Non–HDL-C comprises the cholesterol carried by all atherogenic particles, including LDL, intermediatedensity lipoproteins, very low-density lipoproteins (VLDL) and VLDL remnants, chylomicron remnants, and lipoprotein.<ref name="pmid26891998">Bays HE, Jones PH, Orringer CE, Brown WV, Jacobson TA (2016) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=26891998 National Lipid Association Annual Summary of Clinical Lipidology 2016.] ''J Clin Lipidol'' 10 (1 Suppl):S1-43. [http://dx.doi.org/10.1016/j.jacl.2015.08.002 DOI:10.1016/j.jacl.2015.08.002] PMID: [https://pubmed.gov/26891998 26891998]</ref>
+=== Secondary Prevention ===
+*Lifestyle interventions are a key to efforts to reduce triglycerides that includes<ref name="pmid25911072">Jacobson TA, Ito MK, Maki KC, Orringer CE, Bays HE, Jones PH et al. (2015) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=25911072 National lipid association recommendations for patient-centered management of dyslipidemia: part 1--full report.] ''J Clin Lipidol'' 9 (2):129-69. [http://dx.doi.org/10.1016/j.jacl.2015.02.003 DOI:10.1016/j.jacl.2015.02.003] PMID: [https://pubmed.gov/25911072 25911072]</ref>
+**weight loss if overweight or obese
+**physical activity (≥ 150 minutes per week of moderate or higher intensity activity)
+*Restriction of alcohol
+*Restriction of sugar/refined carbohydrate intakes
+*For patients with very high TG level (≥500 mg/dL),chylomicronemia will generally be present. For such patients, a low-fat diet (,15% of energy) may be helpful to reduce entry of new chylomicron particles into the circulation.<ref name="pmid25911072">Jacobson TA, Ito MK, Maki KC, Orringer CE, Bays HE, Jones PH et al. (2015) [https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=25911072 National lipid association recommendations for patient-centered management of dyslipidemia: part 1--full report.] ''J Clin Lipidol'' 9 (2):129-69. [http://dx.doi.org/10.1016/j.jacl.2015.02.003 DOI:10.1016/j.jacl.2015.02.003] PMID: [https://pubmed.gov/25911072 25911072]</ref>
+*For patients with triglycerides <500 mg/dL, partial replacement of dietary carbohydrate (especially sugars and other refined carbohydrates) with a combination of unsaturated fats and proteins may help to reduce the triglyceride and non-HDL-C concentrations.
 ==Case Studies==