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):

Persons with this condition who follow a very low-fat diet may live into adulthood.

Complications:

Pancreatitis and recurrent episodes of abdominal pain may develop.

Xanthomas are not usually painful unless they are rubbed a lot.

Calling your health care provider:

Call your health care provider for screening if someone in your family has lipoprotein lipase deficiency. Genetic counseling is recommended for anyone with a family history of this disease.