Familial lipoprotein lipase deficiency

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Coronary artery disease

Overview

Symptoms

Treatment

Prevention

Familial lipoprotein lipase deficiency

Alternative Names:

Type I hyperlipoproteinemia; Familial chylomicronemia

Treatment:

Treatment is intended to control the symptoms and blood triglyceride levels with a very low-fat diet. Fat intake usually must be less than 20 grams per day to keep the symptoms from coming back.

Twenty grams of fat is equivalent to one of the following:

2, 8-ounce glasses of whole milk
4 teaspoons of margarine
4-ounce serving of meat

The average American diet has an average fat content of up to 45% of total calories. Fat-soluble vitamins A, D, E, and K and mineral supplements are recommended. Dietary counseling may be helpful for patients trying to stick to a strict diet and maintain adequate calorie and nutrient intake. Pancreatitis responds to conventional treatments for that disorder.

Expectations (prognosis):

By following a very low-fat diet, a person may live into adulthood.

Complications:

Pancreatitis and recurrent episodes of abdominal pain may develop. Numerous xanthomas typically occur in the skin, but are not usually painful unless at the site of recurrent rubbing. Surprisingly, there is no increased risk of atherosclerosis or heart attacks.

Calling your health care provider:

Call your health care provider for screening if lipoprotein lipase deficiency has been diagnosed in a family member, or if you have extremely elevated triglyceride levels. Genetic counseling is recommended for anyone with a family history of this disease.

Review Date: 4/20/2005
Reviewed By: Neal Sondheimer, M.D., PhD., Division of Genetics and Metabolism, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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