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      간관 폐쇄증이 동반된 알라질 증후군 (Alagille Syndrome) 1예 = A Case of Alagille Syndrome with Atresia of the Hepatic Duct

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      https://www.riss.kr/link?id=A101490188

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      다국어 초록 (Multilingual Abstract)

      A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operation were performed. Because the liver biopsy pathology revealed a paucity of intrahepatic bile ducts, the patient was diagnosed with the Alagille syndrome. We report the case of an infant diagnosed with the Alagille syndrome with atresia of the hepatic duct.
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      A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operat...

      A two-month-old baby had acholic stool, neonatal hyperbilirubinemia and congenital heart disease. Atresia of the hepatic duct was confirmed by open cholangiography, which showed a non-opacified intrahepatic bile duct. Liver biopsy and the Kasai operation were performed. Because the liver biopsy pathology revealed a paucity of intrahepatic bile ducts, the patient was diagnosed with the Alagille syndrome. We report the case of an infant diagnosed with the Alagille syndrome with atresia of the hepatic duct.

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      참고문헌 (Reference)

      1 Kohsaka T, "The significance of human jagged 1 mutations detected in severe cases of extrahepatic biliary atresia" 36 : 904-912, 2002

      2 Alagille D, "Syndromic paucity of interlobular bile duct (Alagilles syndrome or arteriohepatic dysplasia): review of 80 cases" 110 : 195-200, 1987

      3 Libbrecht L, "Peripheral bile duct paucity and cholestasis in the liver of a patient with Alagille Syndrome: futher evidence supporting a lack of postnatal bile duct branching and elongation" 29 : 820-826, 2005

      4 Lykavieris P, "Outcome of liver disease in children with Alagille syndrome: a study of 163 patients" 49 : 431-435, 2001

      5 Sookpotarom P, "Non-correctable biliary atresia with large extrahepatic cyst: a report of two cases" 17 : 295-297, 2007

      6 Suchy FJ, "Neonatal Cholestasis" 25 : 388-396, 2004

      7 Nio M, "Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry" 38 : 997-1000, 2003

      8 de Carvalho E, "Extrahepatic biliary atresia: current concepts and future directions" 83 : 105-120, 2007

      9 Quiros-Tejeira RE, "Does liver transplantation affect growth pattern in Alagille Syndrome?" 6 : 582-587, 2000

      10 Davenport M, "Biliary atresia" 14 : 42-48, 2005

      1 Kohsaka T, "The significance of human jagged 1 mutations detected in severe cases of extrahepatic biliary atresia" 36 : 904-912, 2002

      2 Alagille D, "Syndromic paucity of interlobular bile duct (Alagilles syndrome or arteriohepatic dysplasia): review of 80 cases" 110 : 195-200, 1987

      3 Libbrecht L, "Peripheral bile duct paucity and cholestasis in the liver of a patient with Alagille Syndrome: futher evidence supporting a lack of postnatal bile duct branching and elongation" 29 : 820-826, 2005

      4 Lykavieris P, "Outcome of liver disease in children with Alagille syndrome: a study of 163 patients" 49 : 431-435, 2001

      5 Sookpotarom P, "Non-correctable biliary atresia with large extrahepatic cyst: a report of two cases" 17 : 295-297, 2007

      6 Suchy FJ, "Neonatal Cholestasis" 25 : 388-396, 2004

      7 Nio M, "Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry" 38 : 997-1000, 2003

      8 de Carvalho E, "Extrahepatic biliary atresia: current concepts and future directions" 83 : 105-120, 2007

      9 Quiros-Tejeira RE, "Does liver transplantation affect growth pattern in Alagille Syndrome?" 6 : 582-587, 2000

      10 Davenport M, "Biliary atresia" 14 : 42-48, 2005

      11 Piccoli DA, "Alagille syndrome and the Jagged1 gene" 21 : 525-534, 2001

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      학술지 이력

      학술지 이력
      연월일 이력구분 이력상세 등재구분
      2023 평가예정 해외DB학술지평가 신청대상 (해외등재 학술지 평가)
      2020-01-01 평가 등재학술지 유지 (해외등재 학술지 평가) KCI등재
      2013-01-01 평가 등재학술지 유지 (등재유지) KCI등재
      2012-04-03 학술지명변경 한글명 : 대한소아소화기영양학회지 -> Pediatric Gastroenterology, Hepatology & Nutrition
      외국어명 : Korean J Pediatr Gastroenterol Nutr -> Pediatric Gastroenterology, Hepatology & Nutrition
      KCI등재
      2010-01-01 평가 등재학술지 유지 (등재유지) KCI등재
      2007-07-06 학회명변경 영문명 : The Korean Society Of Pediatric Gastroenterology And Nutrition -> The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition KCI등재
      2007-01-01 평가 등재학술지 선정 (등재후보2차) KCI등재
      2006-06-27 학술지명변경 외국어명 : 미등록 -> Korean J Pediatr Gastroenterol Nutr KCI등재후보
      2006-01-01 평가 등재후보 1차 PASS (등재후보1차) KCI등재후보
      2005-05-30 학술지등록 한글명 : 대한소아소화기영양학회지
      외국어명 : 미등록
      KCI등재후보
      2005-01-01 평가 등재후보 1차 FAIL (등재후보1차) KCI등재후보
      2003-07-01 평가 등재후보학술지 선정 (신규평가) KCI등재후보
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      학술지 인용정보

      학술지 인용정보
      기준연도 WOS-KCI 통합IF(2년) KCIF(2년) KCIF(3년)
      2016 0.07 0.07 0.09
      KCIF(4년) KCIF(5년) 중심성지수(3년) 즉시성지수
      0.09 0.1 0.367 0.03
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