Contributor: Gordon K. Klintworth
Hyperlipidemia type V (carbohydrate-inducible hyperlipemia, familial hyperprebetalipoproteinemia, hyperlipoproteinemia type IV, OMIM #238400) is a heterogenous disorder characterized by an increased plasma VLDL on a regular diet. Triglycerides, VLDL accumulate. The plasma triglycerides and VLDL remain persistently elevated, but the cholesterol and phospholipids are usually normal. The basic defect remains unknown, but many conditions cause this type of hyperlipoproteinemia (uremia, hypopituitarism, contraceptive steroids, glycogen storage disease type I, [triglyceridemia - familial, hyperlipoproteinemia - familial type III, hyperlipoproteinemia - familial type V, and hyperlipidemia - familial combined). Some cases have an autosomal dominant mode of inheritance. The systemic manifestations include precocious atherosclerosis, occlusovascular disease, diabetes mellitus, abnormal glucose tolerance and xanthomas [xanthoma]. The ophthalmological signs include arcus lipoides and lipemia retinalis.