LIPOPROTEIN ABNORMALITIES IN PATIENTS WITH EARLY PRIMARY BILIARY-CIRRHOSIS

In 12 females with early forms of primary biliary cirrhosis (PBC) serum lipoproteins, the i.v. fat tolerance test (IVFTT) and the lecithin:cholesterol acyl transferase (LCAT) rate were determined and compared to healthy controls. The cholestatic LDL [low density lipoprotein] (LP-X) test was negative in all cases. PBC patients had lower very low density lipoprotein TG [triglycerides] concentrations than controls and had levels of (high-density lipoprotein) HDL-TG, -cholesterol and -phospholipids that were about 50% higher than in controls. In PBC the HDL2-cholestrol concentration was double, but the HDL3-cholesterol concentration was 60% of control values (P < 0.001 for both). The LCAT rate and the IVFTT value did not differ between the groups. A typical finding on agarose gel electrophoresis was the appearance of a slow-moving .alpha.-band. Several interpretations of these results are possible. In PBC the hepatic lipase activity may be impaired leading to a shift of the HDL2/HDL3 relation. The transport of HDL2 to the liver lipase site may also be affected and HDL3 production reduced due to malabsorption in the intestine.