Lipid Malabsorption

The three major forms of lipid malabsorption are abetalipoproteinemia, familial hypobetalipoproteinemia (565), and diabetes. Several other related syndromes result in lipid accumulations in enterocytes producing histologic patterns indistinguishable from abetalipoproteinemia (Table 6.41) (566). The pathogenesis of several lipid malabsorption syndromes is shown in Figure 6.203.

Abetalipoproteinemia

Abetalipoproteinemia is a recessive genetic disease characterized by the virtual absence of apolipoprotein (apo) B and apo B–containing lipoproteins in plasma. Affected patients are usually individuals of Jewish or Mediterranean descent. Approximately one third of cases result from consanguineous marriages, and family studies suggest an autosomal recessive mode of inheritance (567). The sex ratio is 1:1.

TABLE 6.41 Diseases Associated with Fatty Deposits in Enterocytes

Abetalipoproteinemia
Familial hypobetalipoproteinemia
Diabetes
Gluten-sensitive enteropathy
Cow’s milk–sensitive enteropathy (and related entities)
Anderson disease (chylomicron retention disease)
Fasting states
Impaired chylomicron metabolism
Juvenile nutritional megaloblastic anemia
Tropical sprue
Following the ingestion of a fatty meal

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