type I familial hyperlipoproteinemia

type I fa·mil·i·al hy·per·lip·o·pro·tein·e·mi·a

(tīp fă-mil'ē-ăl hī'pĕr-lip'ō-prō-tēn-ē'mē-ă)
Increased hematologic lipoprotein levels characterized by the presence of large amounts of chylomicrons and triglycerides in the plasma when the patient has a normal diet, and their disappearance on a fat-free diet. It is accompanied by bouts of abdominal pain, hepatosplenomegaly, pancreatitis, and eruptive xanthomas; autosomal recessive inheritance.
Synonym(s): familial fat-induced hyperlipemia, familial hyperchylomicronemia, familial hypertriglyceridemia (1) .


Max, German physician, 1885–.
Bürger-Grütz disease - obsolete term for idiopathic hyperlipemia.
Bürger-Grütz syndrome - an inherited disorder of lipoprotein metabolism. Synonym(s): type I familial hyperlipoproteinemia