ABSTRACT. We characterized the lipid-lipoprotein abnormalities encountered in a series of 45 nonnephrotic uremic children with various degrees of chronic renal insufficiency. A mild hypertriglyceridemia associated with decreased serum high-density lipoprotein cholesterol levels was confirmed. The correlation between high-density lipoprotein cholesterol and creatinine clearance showed a power behavior with a marked decrease in high-density lipoprotein cholesterol below a creatinine clearance value of 40 ml/min/1.73 m2. A number of uremic children accumulate an abnormal population of very low-density lipoprotein particles in their plasma. On agarose gel electrophoresis these particles migrate as a slow moving pre-j3 band and are clearly distinguished from the regular fast moving pre-8 very low-density lipoprotein band. This electrophoretic phenomenon has been called double pre-j3 lipoproteinemia. The prevalence of double pre-j3 lipoproteinemia increased significantly with the degree of impairment of renal function, reaching highest figures in patients on hemodialysis. Accordingly, the very low-density lipoprotein cholesterol/triglyceride ratio also was significantly increased. The double pre-j3 lipoproteinemia phenomenon was not detected in any of the control, nonuremic subjects. The clinical importance of double pre-j3 lipoproteinemia in uremic plasma is related to its possible atherogenic role. (Pediatr Res 21: 462-465,1987) Abbreviations DPBL, double pre-j3 lipoproteinemia VLDL, very low-density lipoproteins HDL-CH, high-density lipoprotein cholesterol AGE, agarose gel electrophoresis