Correctable plasma lipoprotein abnormalities in infants with choledochal cysts

Plasma lipoproteins from two female patients—patient A, 4 wk and patient B, 19 months—were examined prior to and at 1 and 5 wk after surgical correction of biliary obstruction due to choledochal cyst. The findings were correlated with standard indices of hepatic function, namely SGPT, GGTP, 5' nucleotidase, serum bile salts, and total and conjugated bilirubin. Prior to surgery in both patients plasma cholesterol, phospholipid, and triglyceride were elevated; cholesterol esters were low; high-density lipoprotein (HDL) cholesterol and apolipoprotein A–I, the major protein constituent of HDL, were subnormal; most of the plasma lipids were contained in the low–density lipoprotein density region; lipoprotein–X was present. Patient B had had a relatively brief obstruction and suffered little secondary hepatic injury. One week after surgery, plasma lipid concentrations returned to normal; apoliprotein A–I increased in the HDL density region and a concomitant rise in cholesterol esters to near normal, 65%, was observed; plasma lipids were contained predominantly in HDL; hepatic function improved markedly. Patient A had had intrauterine obstruction and suffered major hepatic injury with cirrhosis. One week after surgery, plasma lipid concentrations, cholesterol esters, low–density lipoprotein lipid predominance, and hepatic function remained essentially unchanged. Five weeks after surgery, the lipoprotein levels and composition and hepatic function were near normal. In conclusion, children with biliary obstruction have lipoprotein abnormalities similar to those seen in adult patients. These alterations are rapidly reversible with surgical relief and may be used as prognostic indicators of outcome.