Hyperlipoproteinemia Type IV
Hyperlipoproteinemia type IV, also known as familial hypertriglyceridemia, is a disorder in which an individual has a higher-than-normal trig...

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Hyperlipoproteinemia Type IV

Hyperlipoproteinemia type IV, also known as familial hypertriglyceridemia, is a disorder in which an individual has a higher-than-normal triglyceride level due to a genetic defect. As a result, the affected individual may experience hardening of the arteries and be at risk for various heart conditions, including coronary artery disease.

Triglycerides

Triglycerides are a form of fat that is found within the tissues and in the bloodstream. High levels of triglycerides in the body can cause hardened arteries (atherosclerosis). This can affect the amount of blood and oxygen that is circulated throughout the body.

What Causes Hyperlipoproteinemia Type IV?

The disorder is caused by a genetic defect that is passed down through families. The defect is autosomal dominant. This means that if at least one of your parents has the defective gene, it is likely that you will develop the condition as well.

People who have the condition generally also share other traits. These include obesity, diabetes, pancreatitis, and high blood glucose or insulin levels. These related conditions can also cause a rise in your level of triglycerides. This combination may put you at a higher risk for a severe case of hypertriglyceridemia.

Who Is at Risk for Hyperlipoproteinemia Type IV?

If you have a family history of heart disease before the age of 50, you may also be at higher risk for hyperlipoproteinemia type IV. Likewise, people with high levels of very low-density lipoprotein (“bad” cholesterol) are considered to be at risk. The National Institutes of Health estimate that the disorder occurs in one out of every 500 people in the United States (NIH).

Symptoms to Look For

If you have this disorder, you may not experience any symptoms. Since the disorder is genetic, the largest indicators can be found in your family history. Having parents or family members who have had the disorder or related heart conditions is a strong indicator that you may be at risk. Some people who have this disorder also develop coronary artery disease at a younger age than usual.

How Will I Be Diagnosed?

Your doctor may notice that your triglyceride levels are higher than they should be during a routine blood cholesterol test. If you have a family history of high triglyceride levels, or if other people in your family have had heart disease before the age of 50, it may indicate to your doctor that you have the disorder. Your doctor may recommend a series of blood tests called a coronary risk profile to check the amount of very low-density lipoproteins and triglycerides in your blood.

How Is Hyperlipoproteinemia Type IV Treated?

The goal of treatment is to control factors that may raise your triglyceride levels. This includes managing conditions such as diabetes and obesity. Certain medications can also make your levels rise, such as hormonal birth control. You and your doctor can create a treatment plan that will be suitable for your unique needs.

Dietary changes are necessary to treat this disorder. Your doctor will suggest that you avoid drinking alcohol. Additionally, he or she may recommend that you avoid consuming any extra calories. Pay attention to what you are eating and try to avoid foods that are high in saturated fats and carbohydrates.

Medications

If you still have high triglyceride levels after making these changes, your doctor may prescribe medications to help lower them, including:

  • fenofibrate
  • nicotinic acid
  • gemfibrozil

What Can I Expect in the Long-Term?

People who are diagnosed with this disorder have an increased risk of developing other conditions. For example, you may have a higher risk of developing pancreatitis or coronary artery disease. If you focus on managing your diabetes and losing weight, you may be able to see more positive results.

Complications

If you do not get treatment for the disorder, or if you do not respond to treatment, it can put you at risk for serious complications, including:

  • stroke
  • heart attack
  • pancreatitis
  • kidney failure
  • heart disease
Written by: Elly Dock
Edited by: Heather Ross
Medically Reviewed by: Brenda B. Spriggs, MD, MPH, FACP
Published: Jun 25, 2012
Last Updated: Apr 7, 2014
Published By: Healthline Networks, Inc.
Sources:
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