disorder of lipid metabolism inherited as an autosomal recessive trait characterized by the near absence of apolipoprotein B and apoB containing lipoproteins in plasma; microsomal triglyceride transfer protein is deficient or absent in enterocytes; clinical and laboratory findings include acanthocytosis, hypocholesterolemia, peripheral neuropathy, posterior column degeneration, ataxia, and steatorrhea; intellectual abilities may also be impaired.
presence of abnormally low levels of lipoproteins in the serum.
Also applicable to/known as
Abetalipoproteinemia
Bassen-Kornzweig syndrome
High-density lipoid deficiency
Hypoalphalipoproteinemia
Hypobetalipoproteinemia (familial)
ICD-9-CM Volume 2 Index Entries That Refer To 272.5