Journal of the Korean Pediatric Society 1999;42(6):858-862.
Published online June 15, 1999.
A Case of Nonsyndromic Paucity of Interlobular Bile Ducts in Down Syndrome
Chun Hyuk Chang1, Jun Ho Kim1, Sun Ju Le1, Dong Seok Lee1, Doo Kwun Kim1, Sung Min Choi1, Woo Taek Kim1, Tae Jung Jang2
1Department of Pediatrics, Dongguk University Medical College, Kyongju, Korea
2Department of Pathology, Dongguk University, Medical College, Kyongju, Korea
다운 증후군에서 비증후군적 간내 담도 형성부전증 1례
장준혁1, 김준호1, 이선주1, 이동석1, 김두권1, 최성민1, 김우택1, 장태정2
1동국대학교 의과대학 소아과학교실
2동국대학교 의과대학 병리학교실
Chun Hyuk Chang, Email: 1
The nonsyndromic paucity of interlobular bile ducts, which belongs to intrahepatic biliary atresia, is characterized by conjugated hyperbilirubinemia, suggesting cholestasis in newborn infants it has little relationship with extrahepatic congenital abnormalities. Pathologic findings through percutaneous liver biopsy show portal changes(duct paucity and fibrosis) and lobular changes(cholestasis, giant cell transformation, extramedullary hematopoiesis and perisinusoidal fibrosis). The overall incidence of intrahepatic biliary atresia may be as rare as 1 in 50,000 to 75,000 live births. Puri et al first described intrahepatic biliary atresia in Down syndrome in 1975 and Kahn et al revealed 17 cases of nonsyndromic paucity of interlobular bile ducts, including 2 cases of Down syndrome in 1986. The only treatment available in patients, with intrahepatic biliary atresia, is symptomatic because it is not necessary to operate. The prognosis of these patients remains quite varied; approximately half of patients will later develop cirrhosis with portal hypertension and will die from liver failure in the first year of life. We report this case with the review of the associated literatures.
Key Words: Down syndrome, Neonatal cholestasis, Nonsyndromic paucity of interlobular bile ducts

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