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Familial lipoprotein lipase deficiency   

Overview | Symptoms | Treatment | Prevention

Alternative names:

Type I hyperlipoproteinemia; Familial chylomicronemia

Treatment

The purpose of treatment is to control the symptoms and blood triglyceride levels by a very low fat diet. Fat intake usually must be less than 20 grams per day for the symptoms not to recur. 20 grams of fat is equivalent to 1 of the following: 2 - 8 ounce glasses of whole milk, 4 teaspoons of margarine, or a 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 supplementation is recommended. Dietary counseling is helpful for patients to adhere to a restrictive diet and maintain adequate calorie and nutrient intake. Pancreatitis responds to conventional treatments for that disorder.

Prognosis

By following a diet in which fat is restricted people can 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 contact/rubbing. Suprisingly, there is no increased risk of atherosclerosis or heart attacks.

Call Your Health Care Provider If:

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

Ency. home > Disease > F > Familial lipoprotein lipase deficiency

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