Remnant removal disease occurs as a result of a combination of a defect in the apo E gene, leading to a defect in apo E structure, and an abnormality leading to VLDL overproduction (for example, familial combined hyperlipidaemia or occasionally diabetes mellitus) (Fig. 6.14). The disorder is also known as broad-beta disease because of the electrophoretic appearance of the remnant accumulation, dysbetalipoproteinaemia, and type III hyperlipoproteinaemia.
Very rarely, a total absence of apo E will result in remnant removal disease.
