Influence of Partial External Biliary Diversion on the Lipid Profile in Children With Progressive Familial Intrahepatic Cholestasis.

Abstract

The concentration of bile acids is highly increased in progressive familial intrahepatic cholestasis (PFIC). Bile acids are the end products of cholesterol metabolism, and aid in the absorption of fat-soluble vitamins and dietary fat. The aim of our study was to investigate lipid metabolism in patients with PFIC with focus on the effect of partial external biliary diversion (PEBD). In 26 patients with PFIC, who underwent PEBD surgery at the median age of 2.2 years (range: 0.4-16.6), we analyzed the concentrations of lipids and apolipoproteins both before and 6 months after PEBD. Patients were split into 2 groups according to the outcome of surgery (either "good" or "poor"), and were analyzed separately. A "good" result following surgery was defined as complete relief from pruritus, and normalization of total bilirubin (<1.0 mg/dL) and bile acid concentration in serum (<12 μmol/L). We found abnormal lipid concentrations at baseline in all 26 patients: cholesterol was increased (>190 mg/dL) in 13 patients, phospholipids were increased (>250 mg/dL) in 5 patients, and triglyceride concentration was increased (>150 mg/dL) in 13 patients. After PEBD, the concentrations of plasma cholesterol, triglycerides, and phospholipids decreased significantly, whereas, ApoA-I and high-density lipoprotein cholesterol concentrations increased and the concentrations of apolipoprotein B, low-density lipoprotein cholesterol, and very low-density lipoprotein cholesterol significantly decreased. PEBD had neither an effect on ApoE concentration nor on lecithin-cholesterol acyl transferase activity. In the group with a "poor" outcome report following PEBD, total serum cholesterol concentration decreased significantly, and no effect on the concentrations of triglycerides and phospholipids were observed. Patients with PFIC present with a high risk of lipid disturbances. PEBD has a beneficial effect on lipid profile in the majority of cases.