Sandbox:Hyperlipoproteinemia type5

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

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Any Disease from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

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.

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

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]

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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


  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
  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

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