Familial lipoprotein lipase deficiency

Alternative names:

type I hyperlipoproteinemia

Treatment:

The purpose of treatment is to control the symptoms and blood triglyceride levels by a fat-free diet. Fat intake 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.

Expectations (prognosis):

Symptoms can be controlled by following a diet in which fat is restricted.

Complications:

Pancreatitis may develop.

Calling your health care provider:

Call for an appointment with your health care provider (for screening) if lipoprotein lipase deficiency has been diagnosed in a member of your family.