hyperchylomicronemia

hyperchylomicronemia /-kī′lōmī′krōnē″mē·ə/ [Gk, hyper + chylos, juice, mikros, small, haima, blood] , type I hyperlipoproteinemia, a rare congenital deficiency of an enzyme essential to fat metabolism. Fat accumulates in the blood as chylomicron. The condition affects children and young adults, in whom xanthomas (fatty deposits) in the skin, hepatomegaly, and abdominal pain develop. Pancreatitis is the most significant complication. Strict limitation of dietary fat may allow the person to prevent discomfort and complications. Also called familial lipoprotein lipase deficiency. See also chylomicron.