144600
ICD+
| ||||||
| HYPERLIPOPROTEINEMIA, TYPE IV | ||||||
| Alternative titles; symbols | ||||||
| CARBOHYDRATE-INDUCIBLE HYPERLIPEMIA | ||||||
| Clinical Synopsis | ||||||
| TEXT | ||||||
| On a regular diet the patient demonstrates increased plasma VLDL. Plasma triglycerides are persistently increased, while plasma cholesterol and phospholipids are usually within normal limits. Precocious atherosclerosis, abnormal glucose tolerance, and atheroeruptive xanthoma may occur. The disorder is undoubtedly heterogeneous and the phenotype strongly influenced by environmental factors, particularly carbohydrate and ethanol consumption. Other conditions causing hyperlipoproteinemia IV are uremia, hypopituitarism, contraceptive steroids, and glycogen storage disease I (232200). Most individuals with familial hypertriglyceridemia (145750) have a hyperlipoproteinemia IV phenotype. Individuals with familial hyperlipoproteinemia type 3 or type 5 or familial combined hyperlipidemia may also have this phenotype. In an old American family living in New England, Schreibman et al. (1969) described hyperprebetalipoproteinemia behaving as an autosomal dominant with reduced penetrance. Although triglyceride levels as high as 2000 mg per 100 ml were observed in some children of this family, precocious atherosclerosis was not observed. The absence of obesity and glucose intolerance may account for the favorable prognosis. Goldman et al. (1972) emphasized the association of rheumatic manifestations. | ||||||
| REFERENCES | ||||||
| ||||||
| ||||||