Familial Type IV and Type V Hypertriglyceridemias

These two disorders may have overlapping pheno-types. In type IV or familial endogenous hypertrigly-ceridemia, triacylglycerol levels are increased and HDL is usually decreased. This disorder appears to be autosomal dominant and relatively frequent in populations consuming high-fat diets. The precise molecular defect has not been defined; however, the increase in triacylglycerol is associated with overproduction of triacylglycerol by the liver and often with consequent reduced clearance. Diet should be the first step in therapy, followed if necessary by pharmacotherapy using fibrates or nicotinic acid. Premature CHD has been seen in some but not all cases presenting with this phenotype.

Type V hyperlipidemia is a much more rare disorder. Usually the first signs of this abnormality are abdominal pain or pancreatitis. VLDL levels are high and chylomicrons are present in the fasting state. This abnormality has not been linked to any specific molecular defect. Besides the primary genetic defect, other secondary causes of type V hyperlipidemia are poorly controlled diabetes melli-tus, nephrotic syndrome, hypothyroidism, glycogen storage disease, and pregnancy. Recent data indicate increased susceptibility to atherosclerosis.

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