Pediatric Gastroenterology, Hepatology & Nutrition

The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition (대한소아소화기영양학회)
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A Case of Idiopathic Congenital Neonatal Cholestasis in a Patient with Down Syndrome

  • Huh, Tae-Eon (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Do, Hyun Jeong (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Park, Ji Sook (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Yeom, Jung Sook (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Park, Eun Sil (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Seo, Ji Hyun (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Lim, Jae Young (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Park, Chan-Hoo (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Woo, Hyang Ok (Department of Pediatrics, Gyeongsang National University Hospital) ;
  • Youn, Hee-Shang (Department of Pediatrics, Gyeongsang National University Hospital)
  • Received : 2011.11.28
  • Accepted : 2012.06.14
  • Published : 2012.06.30
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Abstract

Down syndrome is a rare cause of neonatal cholestasis. Neonatal cholestasis in a patient with Down syndrome is usually associated with severe liver diseases, such as neonatal hemochromatosis, myeloproliferative disorder and intrahepatic bile duct paucity. We experienced a case of idiopathic neonatal cholestasis in a patient with Down syndrome, which resolved spontaneously.

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References

  1. Ruchelli ED, Uri A, Dimmick JE, Bove KE, Huff DS, Duncan LM, et al. Severe perinatal liver disease and Down syndrome: an apparent relationship. Hum Pathol 1991;22:1274-80.
  2. Chang CH, Kim JH, Lee SJ, Lee, DS Lee, Kim DK, Choi SM, et al. A case of Nonsyndromic paucity of interlobular bile ducts in Down syndrome. J Korean Pediatr Soc 1999;42:858-62.
  3. Crosti N, Bajer J, Gentile M, Resta G, Serra A. Catalase and glutathone peroxidase activity in cells with trisomy Clin Genet 1989;36:107-16
  4. Bacon BR, Tavill AS, Brittenham GM, Park CH, Recknagel RO. Hepatic lipid peroxidation in vivo in rats with chronic iron overload. J Clin Invest 1983;71:429-39.
  5. Jacquemin E, Lykavieris P, Chaoui N, Hadchouel M, Bernard O. Transient neonatal cholestasis: Origin and outcome. J Pediatr 1998;133:563-7.
  6. Suchy FJ, Bucuvalas JC, Novak DA. Determinants of bile formation during development: ontogeny of hepatic bile acid metabolism and transport. Semin Liver Dis 1987;7:77-84.
  7. Lemasters JJ, Stemkowski CJ, Ji S, Thurman RG. Cell surface changes and enzyme release during hypoxia and reoxygenation in the isolated perfused rat liver. J Cell Biol 1983;97:778-86.
  8. Choe BH. Early exclusive diagnosis of biliary atresia among infants with cholestasis. Korean J Pediatr Gastroenterol Nutr 2011;14:122-9.
  9. Ko JS, JK Seo. The etiologies of neonatal cholestasis. Korean J Pediatr 2007;50:835-40.