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Omega (ω)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nu-trition-associated liver disease, and several mechanisms were proposed for this observation. An 8-week-old male infant with cholestasis and acholic stool was diagnosed non-syndromic intrahepatic interlobular bile duct paucity by open-wedge liver biopsy. Initially he was treated with usual supportive medical therapy, including ursodeoxycholic acid. However, the clinical status and laboratory tests did not improve. Omega (ω)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including amino-transferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effec-tiveness of ω-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease.

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