Bassen-Komzweig syndrome

Abetalipoproteinemia is a rare lEM involving lipid dysfunction. Also called Bassen-Komzweig syndrome, it is characterized by extremely low cholesterol due to deficient or absent beta lipoproteins, which are an important component of the cholesterol molecular complex. Symptoms include growth retardation, neurological dysfunction, retinal pigment degeneration, and upper intestinal malabsorption. 

Deficiency of low-density lipoproteins (a, P-lipoproteinemia, Bassen-Komzweig syndrome)... 

As long as incomplete knowledge of the pathogenesis of the disease prevents the use of a more specific term, the name Bassen-Komzweig syndrome or a-jS-lipoproteinemia should be used. 

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