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  • Title: The familial chylomicronemia syndrome.
    Author: Santamarina-Fojo S.
    Journal: Endocrinol Metab Clin North Am; 1998 Sep; 27(3):551-67, viii. PubMed ID: 9785052.
    Abstract:
    The chylomicronemia syndrome is a disorder characterized by severe hypertriglyceridemia and fasting chylomicronemia. Genetic causes of the syndrome are rare and include deficiency of lipoprotein lipase (LPL), apolipoprotein C-II, and familial inhibitor of LPL. Patients with familial forms of hypertriglyceridemia in combination with secondary acquired disorders account for most individuals presenting with chylomicronemia. The clinical manifestations--lipid and other biochemical abnormalities--as well as treatment options for chylomicronemic patients are discussed.
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