Correctable Plasma Lipoprotein Abnormalities in Infants with Choledochal Cysts

Abstract

ABSTRACT. 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, S'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.

Bile composition in Alagille Syndrome and PFIC patients having Partial External Biliary Diversion

“…Sixty to eighty percent of hepatic lobules lack biliary drainage while remaining capable of producing bile, more in the periphery than the core [ 25 ]. AGS patients have serum values characteristic of biliary obstruction with markedly elevated serum cholesterol, bile salts, and lipoprotein-X values, presumably due to reflux of bile constituents from canaliculi and ductules with inadequate bile drainage [ 26 - 28 ]. Our findings suggest that patients with AGS make normal bile similar to that reported for normal children [ 29 ].…”

Clinical and Biochemical Findings in Progressive Familial Intrahepatic Cholestasis

“…Patients with PFIC also have relatively normal serum cholesterol levels, in contrast to those in most other forms of cholestatic liver disease. Adults (38) and children with extrahepatic biliary obstruction (34,39,40) have been shown to have increased levels of lipoprotein X and low levels of apolipopro-tein A-I [and, presumably, low high-density lipoprotein (HDL)] in addition to elevated total cholesterol levels. Although no extensive study has been made of lipid profiles in children with intrahepatic cholestasis, the markedly elevated cholesterol levels noted in patients with arteriohepatic dysplasia would suggest a similar pattern of lipid abnormalities.…”