noun Pathology.
- a rare inherited disorder of fat metabolism due to an inability to synthesize certain apolipoproteins necessary for the transport of triglycerides, leading to diarrhea, steatorrhea, and failure to thrive.
n.
- An inherited disorder characterized by the absence of low-density lipoproteins in the plasma, the presence of acanthocytes in the blood, retinal pigmentary degeneration, malabsorption of fats, and neuromuscular abnormalities.Bassen-Kornzweig syndrome