Clinicopathological Significance of Lipoprotein Phenotypes

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Phenotype	Familial Syndrome	May Occur Secondary to	Remarks
I	Abdominal pain Eruptive xanthomas Lipemia retinalis Early vascular disease absent	Insulin-dependent diabetes Lupus erythematosus Dysglobulinemias Pancreatitis	Lipoprotein lipase is deficient.
II	Early, severe vascular disease Prominent xanthomas	High-cholesterol diet Nephrotic syndrome Porphyria Hypothyroidism Dysglobulinemias Obstructive liver diseases	Familial trait is autosomal dominant; homozygotes are especially severely affected.
III	Accelerated vascular disease, onset in adulthood Xanthomas, palmar yellowing Abnormal glucose tolerance Hyperuricemia	Hypothyroidism Dysglobulinemias Uncontrolled diabetes	Diet, lipid-lowering drugs are very effective.
IV	Accelerated vascular disease, onset in adulthood Abnormal glucose tolerance Hyperuricemia	Obesity High alcohol intake Oral contraceptives Diabetes Nephrotic syndrome Glycogen storage disease	Weight loss lowers VLDL. High-fat diet may convert to type V.
V	Abdominal pain Pancreatitis Eruptive xanthomas Abnormal glucose tolerance Vascular disease not associated	High alcohol intake Diabetes Nephrotic syndrome Pancreatitis Hypercalcemia	Weight loss does not lower VLDL.

From Sacher, RA, and McPherson, RA: Widmann's Clinical Interpretation of Laboratory Tests, ed 11. FA Davis, Philadelphia, 2000, p. 477, with permission.

[Table Outline]

• I
• II
• III
• IV
• V