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Familial hypertriglyceridemia

Definition

Familial hypertriglyceridemia is a common disorder passed down through families in which the level of triglycerides (a type of fat) in a person's blood are higher than normal.

The condition is not associated with a significant increase in cholesterol levels.

Causes

Familial hypertriglyceridemia is caused by a genetic defect, which is passed on in an autosomal dominant fashion. This means that if you get a bad copy of the gene from just one of your parents, you will have the condition.

Some people with this condition also have high levels of very low density lipoprotein (VLDL). The reason for the rise in triglycerides and VLDL is not understood.

Familial hypertriglyceridemia does not usually become noticeable until puberty or early adulthood. Obesity, hyperglycemia (high blood glucose levels), and high levels of insulin are frequently associated with this condition and make cause even higher triglyceride levels.

Familial hypertriglyceridemia occurs in about 1 in 500 individuals in the United States. Risk factors are a family history of hypertriglyceridemia or a family history of heart disease before the age of 50.

Symptoms

You may not notice any symptoms. People with the condition may have coronary artery disease at an early age.

Exams and Tests

People with a family history of this condition should have blood tests to check very low density lipoprotein (VLDL) and triglyceride levels. Blood tests usually show a mild to moderate increase in triglycerides.

A coronary risk profile may also be done.

Treatment

This version of the Encyclopedia has no treatment information. Please discuss any and all treatment options for your condition with your healthcare professional.

Outlook (Prognosis)

Persons with this condition have an increased risk of coronary artery disease and pancreatitis.

Losing weight and keeping diabetes under control helps improve the outcome.

Possible Complications

  • Pancreatitis
  • Coronary artery disease

Prevention

Screening family members for high triglycerides may detect the disease early.


Review Date: 1/23/2008
Reviewed By: Glenn Gandelman, MD, MPH, Assistant Clinical Professor of Medicine, New York Medical College, Valhalla, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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