Influence of Partial External Biliary Diversion on the Lipid Profile in Children with PFIC.

Background and aim: 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 PFIC patients with focus on the effect of partial external biliary diversion (PEBD). Methods: 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 two 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 ( 190 mg/dl) in 13 patients, phospholipids were increased (>250 mg/dl) in five 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 HDL-C concentrations increased and the concentrations of ApoB, LDL-C and VLDL-C significantly decreased. PEBD had neither an effect on ApoE concentration nor on LCAT 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. Conclusion: Patients with PFIC present with a high risk of lipid disturbances. PEBD has a beneficial effect on lipid profile in the majority of cases. (C) 2016 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,

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