Type I hyperlipoproteinemia; Familial chylomicronemia
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:
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 for people who eat a very low-fat diet. Dietary counseling may be helpful for patients who are trying to stick to a strict diet and still get enough calories and nutrients.
Pancreatitis that is related to lipoprotein lipase deficiency responds to treatments for that disorder.
Persons with this condition who follow a very low-fat diet may live into adulthood.
Pancreatitis and recurrent episodes of abdominal pain may develop.
Xanthomas are not usually painful unless they are rubbed a lot.
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.
Gennest J, Libby P. Lipoprotein disorders and cardiovascular disease. In: Bonow RO, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 47.
Semenkovich CF. Disorders of lipid metabolism. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007: chap 217.
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