Familial lipoprotein lipase deficiency
Alternate Names : Type I hyperlipoproteinemia, Familial chylomicronemia
Treatment
Treatment aims to control the symptoms and blood triglyceride levels with a very low-fat diet. You should eat no more than 20 grams of fat per day to prevent the symptoms from coming back. Twenty grams of fat is equal 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 a 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 still get enough calories and nutrients. Pancreatitis associated with lipoprotein lipase deficiency responds to treatments for that disorder.
Prognosis (Expectations)
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.
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