• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권2호
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• pp.121-124
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• 2014

Omega (${\omega}$)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nutrition-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 (${\omega}$)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including aminotransferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effectiveness of ${\omega}$-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제5권2호
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• pp.192-198
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• 2002

저자들은 지속적인 황달을 주소로 연세의대 세브란스병원에 입원하였던 10개월 된 남아에서 특징적인 얼굴모양, 심혈관계 이상, 간 생검 조직검사상 담즙 정체, 거대세포 형성 및 소염관 담관의 수가 감소되어 있었고, 퇴원 후 간경변으로 이행되어 간 이식수술을 받았던 Alagille증후군 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.