본문 바로가기
KERD
논문KCI 등재

영아기 담즙정체성 황달 질환 중 담도폐쇄증의 조기 배제 진단

Early Exclusive Diagnosis of Biliary Atresia among Infants with Cholestasis

1

조회

0

다운로드

2

인용

0

참고문헌

키워드

주제분류소아과학

초록

The persistence of jaundice beyond the first 2 weeks of life require further investigation and this can be determined if the conjugated bilirubin levels are greater than 1.5 mg/dL or greater than 20% of the total bilirubin level. There is a diverse differential diagnosis for the cause of neonatal cholestasis due to hepatobiliary disease including biliary atresia, which eventually leads to liver cirrhosis if uncorrected before 60∼80 days of life. Long-established initial studies include abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy, but better diagnostic methods are needed. Promising new options are described including MRCP (magnetic resonance cholangiography), ERCP (endoscopic retrograde cholangiography), and PCC (percutaneous cholecysto-cholangiography). Though no single test can differentiate biliary atresia from other neonatal cholestasis with confidence, a combination of diagnostic methods is usually consistently beneficial. By excluding biliary atresia as early as possible, the risk of unnecessary explolaparotomy with intraoperative cholangiography is decreased. Further evaluation would be required for the diagnosis of neonatal cholestasis after excluding biliary atresia. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 122∼129)

Abstract

The persistence of jaundice beyond the first 2 weeks of life require further investigation and this can be determined if the conjugated bilirubin levels are greater than 1.5 mg/dL or greater than 20% of the total bilirubin level. There is a diverse differential diagnosis for the cause of neonatal cholestasis due to hepatobiliary disease including biliary atresia, which eventually leads to liver cirrhosis if uncorrected before 60∼80 days of life. Long-established initial studies include abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy, but better diagnostic methods are needed. Promising new options are described including MRCP (magnetic resonance cholangiography), ERCP (endoscopic retrograde cholangiography), and PCC (percutaneous cholecysto-cholangiography). Though no single test can differentiate biliary atresia from other neonatal cholestasis with confidence, a combination of diagnostic methods is usually consistently beneficial. By excluding biliary atresia as early as possible, the risk of unnecessary explolaparotomy with intraoperative cholangiography is decreased. Further evaluation would be required for the diagnosis of neonatal cholestasis after excluding biliary atresia. (Korean J Pediatr Gastroenterol Nutr 2011; 14: 122∼129)

참고문헌 (40)

  1. Choe BH, "Transplantation for cholestatic liver disease in the pediatric patient, in Transplantation of the Liver. 2nd ed", pp.303~321 (2005), Elsevier Saunders
  2. Danks DM, "Prognosis of babies with neonatal hepatitis", Arch Dis Child, 52, pp.368~372 (1977)
  3. Mowat AP, "Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants", Arch Dis Child, 51, pp.763~770 (1976)
  4. Tazawa Y, "Clinical heterogeneity of neonatal intrahepatic cholestasis caused by citrin deficiency: case reports from 16 patients", Mol Genet Metab, 83, pp.213~219 (2004)
  5. Jae Sung Ko, "Neonatal Intrahepatic Cholestasis Caused by Citrin Deficiency in Korean Infants", Journal of Korean Medical Science, 22(6), pp.952~956 (2007), 대한의학회
  6. Alagille D, "Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases", J Pediatr, 110, pp.195~200 (1984)
  7. Deprettere A, "Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood", J Pediatr Gastroenterol Nutr, 6, pp.868~871 (1987)
  8. Oda T, "Mutations in the human Jagged1 gene are responsible for Alagille syndrome", Nat Genet, 16, pp.235~242 (1997)
  9. Bates MD, "Biliary atresia: pathogenesis and treatment", Semin Liver Dis, 18, pp.281~293 (1998)
  10. Mieli-Vergani G, "Late referral for biliary atresia--missed opportunities for effective surgery", Lancet, 1, pp.421~423 (1989)
  11. Grosfeld JL, "The efficacy of hepatoportoenterostomy in biliary atresia", Surgery, 106, pp.692~700 (1989)
  12. Volpert D, "Outcome of early hepatic portoenterostomy for biliary atresia", J Pediatr Gastroenterol Nutr, 32, pp.265~269 (2001)
  13. Schoen BT, "The Kasai portoenterostomy: when is it too late", J Pediatr Surg, 36, pp.97~99 (2001)
  14. Carceller A, "Past and future of biliary atresia", J Pediatr Surg, 35, pp.717~720 (2000)
  15. Dolgin SE, "Answered and unanswered controversies in the surgical management of extra hepatic biliary atresia", Pediatr Transplant, 8, pp.628~631 (2004)
  16. Maller ES, "Liver function tests in Clinical pediatric gastroenterology. 1st ed.", pp.575~578 (1998), Churchill Livingstone
  17. Park WH, "A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis", J Pediatr Surg, 32, pp.1555~1559 (1997)
  18. Park WH, "The ultrasonographic ‘triangular cord' coupled with gallbladder images in the diagnostic prediction of biliary atresia from infantile intrahepatic cholestasis", J Pediatr Surg, 34, pp.1706~1710 (1999)
  19. Park WH, "Technical innovation for noninvasive and early diagnosis of biliary atresia: the ultrasonographic “triangular cord” sign", J Hepatobiliary Pancreat Surg, 8, pp.337~341 (2001)
  20. Lee HJ, "Objective criteria of triangular cord sign in biliary atresia on US scans", Radiology, 229, pp.395~400 (2003)
  21. Kanegawa K, "Sonographic diagnosis of biliary atresia in pediatric patients using the “triangular cord” sign versus gallbladder length and contraction", AJR Am J Roentgenol, 181, pp.1387~1390 (2003)
  22. Tan Kendrick AP, "Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length", Pediatr Radiol, 30, pp.69~73 (2000)
  23. 김정미, "신생아 담즙정체성 간 질환에서 담도 폐쇄증 감별을 위한 DISIDA 스캔의 진단적 한계성과 유용성", Korean Journal of Pediatrics, 49(7), pp.737~744 (2006), 대한소아과학회
  24. Norton KI, "MR cholangiography in the evaluation of neonatal cholestasis: initial results", Radiology, 222, pp.687~391 (2002)
  25. Ryeom HK, "Biliary atresia: feasibility of mangafodipir trisodium-enhanced MR cholangiography for evaluation", Radiology, 235, pp.250~258 (2005)
  26. Ohnuma N, "The role of ERCP in biliary atresia", Gastrointest Endosc, 45, pp.365~370 (1997)
  27. Petersen C, "Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia", J Hepatol, 51, pp.1055~1060 (2009)
  28. Shanmugam NP, "Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 days", J Pediatr Gastroenterol Nutr, 49, pp.435~441 (2009)
  29. Hadzic N, "Selective rather than routine approach to endosopic retrograde cholangio-pancreatography in diagnosis of biliary atresia", J Hepatol, 52, pp.777~ (2010)
  30. 신경민, "영아에서 담도폐쇄증의 배제를 위한 초음파 유도하의 경피경담낭담관조영술", 대한영상의학회지, 55(2), pp.177~182 (2006), 대한영상의학회
  31. Nwomeh BC, "Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography", Pediatr Surg Int, 23, pp.845~849 (2007)
  32. Lee SY, "The efficacy of ultrasound-guided percutaneous cholecysto-cholangiography for the early exclusion and type determination of biliary atresia", Radiology (2011)
  33. Yamamoto H, "Laparoscopic cholecystcholangiography in a patient with biliary atresia", Surg Laparosc Endosc, 4, pp.370~372 (1994)
  34. Hay SA, "Neonatal jaundice: the role of laparoscopy", J Pediatr Surg, 35, pp.1706~1709 (2000)
  35. Balistrieri WF, "Pediatric hepatology. A half-century of progress", Clin Liver Dis, 4, pp.192~210 (2000)
  36. Finegold MJ, "Common diagnostic problems in pediatric liver pathology", Clin Liver Dis, 6, pp.421~454 (2002)
  37. Kahn E, "Paucity of interlobular bile ducts. Arteriohepatic dysplasia and nonsyndromic duct paucity", Perspect Pediatr Pathol, 14, pp.168~215 (1991)
  38. ByungHoChoe, "The Pattern of Differentially Expressed Genes in Biliary Atresia", Journal of Korean Medical Science, 18(3), pp.392~396 (2003), 대한의학회
  39. 최보화, "신생아 담즙정체성 간질환에서 간조직 유전자의 발현 양상", Pediatric Gastroenterology, Hepatology & Nutrition, 8(2), pp.177~193 (2005), 대한소아소화기영양학회
  40. Wang H, "Serum markers may distinguish biliary atresia from other forms of neonatal cholestasis", J Pediatr Gastroenterol Nutr, 50, pp.411~416 (2010)

관련 논문AI추천

이 간행물의 다른 논문 (9건)