Bassen-Kornzweig syndrome
Alternate Names : Abetalipoproteinemia, Acanthocytosis, Apolipoprotein B deficiency
Definition
Bassen-Kornzweig syndrome is a rare, inherited disease in which a person is unable to fully absorb dietary fats through the intestines.
Overview, Causes, & Risk Factors
Bassen-Kornzweig syndrome is an autosomal recessive condition that more often affects males, but can also affect girls. It is caused by a defect in the microsomal triglyceride transfer protein (MTP) gene. The defect makes the body unable to create lipoproteins (molecules of fat combined with protein). Persons with this condition are unable to properly digest fat and essential vitamins.
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