Biliary atresia: Clinical aspects

Seminars in Pediatric Surgery

Volumn 21, Issue 3, 2012, Pages 175-184

  Davenport, Mark ^a  

^a KING S COLLEGE HOSPITAL   (United Kingdom)

Author keywords

BASM; Biliary atresia; Clinical features; Cystic biliary atresia; Outcome

Indexed keywords

ASPARTATE AMINOTRANSFERASE; BILE ACID; BILIRUBIN; BILIRUBIN GLUCURONIDE; CYTOMEGALOVIRUS ANTIBODY; GENTAMICIN; MEROPENEM; OCTREOTIDE; PIPERACILLIN PLUS TAZOBACTAM; PREDNISOLONE; PROPRANOLOL; TERLIPRESSIN; URSODEOXYCHOLIC ACID;

ALAGILLE SYNDROME; ALPHA 1 ANTITRYPSIN DEFICIENCY; ARTICLE; ASCITES; ASPARTATE AMINOTRANSFERASE BLOOD LEVEL; BILE ACID BLOOD LEVEL; BILE DUCT ATRESIA; BILE DUCT MALFORMATION; BILE DUCT OBSTRUCTION; BILIRUBIN BLOOD LEVEL; CHOLANGITIS; CHOLECYSTOSTOMY; CHOLESTASIS; CHRONIC LIVER DISEASE; CLINICAL CLASSIFICATION; CLINICAL FEATURE; CONTROLLED CLINICAL TRIAL (TOPIC); CYTOMEGALOVIRUS; DEVELOPMENTAL DISORDER; ECHOGRAPHY; ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY; ESOPHAGUS ATRESIA; ESOPHAGUS VARICES; ESOPHAGUS VARICES BLEEDING; ETIOLOGY; EUROPE; GENERAL PRACTITIONER; GESTATIONAL AGE; HEPATOJEJUNOSTOMY; HISTOPATHOLOGY; HUMAN; IMMUNOHISTOCHEMISTRY; INCIDENCE; JAUNDICE; LAPAROTOMY; LIVER BIOPSY; LIVER CIRRHOSIS; LIVER FIBROSIS; LIVER TRANSPLANTATION; LOW DRUG DOSE; MALABSORPTION; MATERNAL DIABETES MELLITUS; MORBIDITY; PEDIATRIC SURGERY; PORTAL HYPERTENSION; PORTAL VEIN; PORTAL VEIN BLOOD PRESSURE; PORTOENTEROSTOMY; POSTOPERATIVE COMPLICATION; PRIORITY JOURNAL; PROGNOSIS; RANDOMIZED CONTROLLED TRIAL (TOPIC); SACRUM AGENESIS; SEROLOGY; SEX DIFFERENCE; SURGICAL TECHNIQUE; TANDEM MASS SPECTROMETRY; VITAMIN DEFICIENCY;

ADRENAL CORTEX HORMONES; ANTI-INFLAMMATORY AGENTS; BILIARY ATRESIA; CHEMOTHERAPY, ADJUVANT; CHOLAGOGUES AND CHOLERETICS; HUMANS; LIVER TRANSPLANTATION; PORTOENTEROSTOMY, HEPATIC; POSTOPERATIVE COMPLICATIONS; PROGNOSIS; SPLEEN; SYNDROME; URSODEOXYCHOLIC ACID;

EID: 84863832928     PISSN: 10558586     EISSN: 15329453     Source Type: Journal    
DOI: 10.1053/j.sempedsurg.2012.05.010     Document Type: Article

References (64)

1. Hadzić N., Davenport M., Tizzard S., et al. Long-term survival following Kasai portoenterostomy: Is chronic liver disease inevitable?. J Pediatr Gastroenterol Nutr 2003, 37:430-433.
2. Hadžić N., Quaglia A., Portmann B., et al. Hepatocellular carcinoma in biliary atresia: King's College Hospital experience. J Pediatr 2011, 159:617-622.
3. Hartley J.L., Davenport M., Kelly D.A. Biliary atresia. Lancet 2009, 374:1704-1713.
4. Davenport M., Savage M., Mowat A.P., et al. The biliary atresia splenic malformation syndrome. Surgery 1993, 113:662-668.
5. Davenport M., Tizzard S.A., Underhill J., et al. The biliary atresia splenic malformation syndrome: A 28-year single-center retrospective study. J Pediatr 2006, 149:393-400.
6. Caponcelli E., Knisely A.S., Davenport M. Cystic biliary atresia: An etiologic and prognostic subgroup. J Pediatr Surg 2008, 43:1619-1624.
7. Chardot C., Carton M., Spire-Bendelac N., et al. Epidemiology of biliary atresia in France: A national study 1986-96. J Hepatol 1999, 31:1006-1013.
8. Hsiao C.H., Chang M.H., Chen H.L., et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 2008, 47:1233-1240.
9. Livesey E., Cortina Borja M., Sharif K., et al. Epidemiology of biliary atresia in England and Wales (1999-2006). Arch Dis Child Fetal Neonatal Ed 2009, 94:F451-F455.
10. Sokol R.J., Mack C., Narkewicz M.R., et al. Pathogenesis and outcome of biliary atresia: Current concepts. J Pediatr Gastroenterol Nutr 2003, 37:4-21.
11. Muller F., Oury J.F., Dumez Y., et al. Microvillar enzyme assays in amniotic fluid and fetal tissues at different stages of development. Prenat Diagn 1988, 8:189-198.
12. Mushtaq I., Logan S., Morris M., et al. Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry. BMJ 1999, 319:471-477.
13. Harpavat S., Finegold M.J., Karpen S.J. Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics 2011, 128:e1428-e1433.
14. Chu A.S., Russo P.A., Wells R.G. Cholangiocyte cilia are abnormal in syndromic and non-syndromic biliary atresia. Mod Pathol 2012, 25:751-757.
15. Davit-Spraul A., Baussan C., Hermeziu B., et al. CFC1 gene involvement in biliary atresia with polysplenia syndrome. J Pediatr Gastroenterol Nutr 2008, 46:111-112.
16. Park W.H., Choi S.O., Lee H.J., et al. 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 1997, 32:1555-1559.
17. Humphrey T.M., Stringer M.D. Biliary atresia: US diagnosis. Radiology 2007, 244:845-851.
18. Mittal V., Saxena A.K., Sodhi K.S., et al. Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. Am J Roentgenol 2011, 196:W438-W445.
19. Hinds R., Davenport M., Mieli-Vergani G., et al. Antenatal presentation of biliary atresia. J Pediatr 2004, 144:43-46.
20. Shen O., Rabinowitz R., Yagel S., et al. Absent gallbladder on fetal ultrasound: Prenatal findings and postnatal outcome. Ultrasound Obstet Gynecol 2011, 37:673-677.
21. Makin E., Quaglia A., Kvist N., et al. Congenital biliary atresia: Liver injury begins at birth. J Pediatr Surg 2009, 44:630-633.
22. Shalaby A., Makin E., Davenport M. Portal venous pressure in biliary atresia. J Pediatr Surg 2012, 47:363-366.
23. Russo P., Magee J.C., Boitnott J., et al. (Biliary Atresia Research Consortium). Design and validation of the biliary atresia research consortium histologic assessment system for cholestasis in infancy. Clin Gastroenterol Hepatol 2011, 9:357-362.
24. Shanmugam N.P., Harrison P.M., Devlin J., et al. Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 days. J Pediatr Gastroenterol Nutr 2009, 49:435-441.
25. Nose S., Hasegawa T., Soh H., et al. Laparoscopic cholecystocholangiography as an effective alternative exploratory laparotomy for the differentiation of biliary atresia. Surg Today 2005, 35:925-928.
26. Tseng J.J., Lai M.S., Lin M.C., et al. Stool color card screening for biliary atresia. Pediatrics 2011, 128:e1209-e1215.
27. Wong K.K., Chung P.H., Chan K.L., et al. Should open Kasai portoenterostomy be performed for biliary atresia in the era of laparoscopy?. Pediatr Surg Int 2008, 24:931-933.
28. Ure B.M., Kuebler J.F., Schukfeh N., et al. Survival with the native liver after laparoscopic versus conventional Kasai portoenterostomy in infants with biliary atresia: A prospective trial. Ann Surg 2011, 253:826-830.
29. Zhao R., Li H., Shen C., et al. Hepatic portocholecystostomy (HPC) is ineffective in the treatment of biliary atresia with patent distal extrahepatic bile ducts. J Invest Surg 2011, 24:53-58.
30. Davenport M., Kerkar N., Mieli-Vergani G., et al. Biliary atresia: The King's College Hospital experience (1974-1995). J Pediatr Surg 1997, 32:479-485.
31. Superina R., Magee J.C., Brandt M.L., et al. The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg 2011, 254:577-585.
32. Meyers R.L., Book L.S., O'Gorman M., et al. High dose steroids, ursodeoxycholic acid and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia. J Pediatr Surg 2004, 38:406-411.
33. Kobayashi H., Yamataka A., Koga H., et al. Optimum prednisolone usage in patients with biliary atresia postportoenterostomy. J Pediatr Surg 2005, 40:327-330.
34. Davenport M., Stringer M.D., Tizzard S.A., et al. Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia. Hepatology 2007, 46:1821-1827.
35. Petersen C., Harder D., Melter M., et al. Postoperative high-dose steroids do not improve mid-term survival with native liver in biliary atresia. Am J Gastroenterol 2008, 103:712-719.
36. Sarkhy A., Schreiber R.A., Milner R.A., et al. Does adjuvant steroid therapy post-Kasai portoenterostomy improve outcome of biliary atresia?. Systematic review and meta-analysis. Can J Gastroenterol 2011, 25:440-444.
37. A-Kader H.H., Santangelo J.D., Setchell K.D.R., et al. The effects of ursodeoxycholic acid therapy in biliary atresia: A double blind, randomized, placebo controlled trial. Pediatr Res 1993, 33:97A.
38. Willot S., Uhlen S., Michaud L., et al. Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia. Pediatrics 2008, 122:e1236-e1241.
39. Ecoffey C., Rothman E., Bernard O., et al. Bacterial cholangitis after surgery for biliary atresia. J Pediatr 1987, 111:824-829.
40. Kang N., Davenport M., Driver M., et al. Hepatic histology and the development of esophageal varices in biliary atresia. J Pediatr Surg 1993, 28:63-66.
41. Stringer M.D., Howard E.R., Mowat A.P. Endoscopic sclerotherapy in the management of esophageal varices in 61 children with biliary atresia. J Pediatr Surg 1989, 24:438-442.
42. Duché M., Ducot B., Tournay E., et al. Prognostic value of endoscopy in children with biliary atresia at risk for early development of varices and bleeding. Gastroenterology 2010, 139:1952-1960.
43. Eroglu Y., Emerick K.M., Whitingon P.F., et al. Octreotide therapy for control of acute gastrointestinal bleeding in children. J Pediatr Gastroenterol Nutr 2004, 38:41-47.
44. Sökücü S., Süoglu O.D., Elkabes B., et al. Long-term outcome after sclerotherapy with or without a beta-blocker for variceal bleeding in children. Pediatr Int 2003, 45:388-394.
45. Serinet M.O., Wildhaber B.E., Broué P., et al. Impact of age at Kasai operation on its results in late childhood and adolescence: A rational basis for biliary atresia screening. Pediatrics 2009, 123:1280-1286.
46. Davenport M., Caponcelli E., Livesey E., et al. Surgical outcome in biliary atresia: Etiology affects the influence of age at surgery. Ann Surg 2008, 247:694-698.
47. McClement J.W., Howard E.R., Mowat A.P. Results of surgical treatment for extrahepatic biliary atresia in United Kingdom 1980-2. Survey conducted on behalf of the British Paediatric Association Gastroenterology Group and the British Association of Paediatric Surgeons. BMJ 1985, 290:345-347.
48. McKiernan P.J., Baker A.J., Kelly D.A. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000, 355:25-29.
49. Lampela H., Ritvanen A., Kosola S., et al. National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes. Scand J Gastroenterol 2012, 47:99-107.
50. Kvist N., Davenport M. Thirty-four years' experience with biliary atresia in Denmark: A single center study. Eur J Pediatr Surg 2011, 21:224-228.
51. Serinet M.O., Broué P., Jacquemin E., et al. Management of patients with biliary atresia in France: Results of a decentralized policy 1986-2002. Hepatology 2006, 44:75-84.
52. Schreiber R.A., Barker C.C., Roberts E.A., et al. Biliary atresia: The Canadian experience. J Pediatr 2007, 151:659-665.
53. Davenport M., Howard E.R. Macroscopic appearance at portoenterostomy-A prognostic variable in biliary atresia. J Pediatr Surg 1996, 31:1387-1390.
54. Howard E.R., Driver M., McClement J., et al. Results of surgery in 88 consecutive cases of extrahepatic biliary atresia. J R Soc Med 1982, 75:408-413.
55. Tan C.E., Davenport M., Driver M., Howard E.R. Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival?. A review of 205 cases. J Pediatr Surg 1994, 29:1459-1464.
56. Davenport M., Gonde C., Redkar R., et al. Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia. J Pediatr Surg 2001, 36:1017-1025.
57. Ohi R. Biliary atresia: Long-term results of hepatic portoenterostomy. Surgery of Liver Disease in Childhood 1991, 60-71. Butterworth-Heinemann, London. E.R. Howard (Ed.).
58. Davenport M., Ong E., Sharif K., et al. Biliary atresia in England and Wales: Results of centralization and new benchmark. J Pediatr Surg 2011, 46:1689-1694.
59. Shneider B.L., Brown M.B., Haber B., et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 2006, 148:467-474.
60. Wildhaber B.E., Majno P., Mayr J., et al. Biliary atresia: Swiss national study, 1994-2004. J Pediatr Gastroenterol Nutr 2008, 46:299-307.
61. de Vries W., de Langen Z.J., Groen H., et al. Biliary atresia in The Netherlands: Outcome of patients diagnosed between 1987 and 2008. J Pediatr 2012, 160:638-644.
62. Nio M., Ohi R., Miyano T., et al. Five- and 10-year survival rates after surgery for biliary atresia: A report from the Japanese biliary atresia registry. J Pediatr Surg 2003, 38:997-1000.
63. Huang H.P., Chang M.H., Chen Y.T., et al. Persistent elevation of hepatocyte growth factor activator inhibitors in cholangiopathies affects liver fibrosis and differentiation. Hepatology 2012, 55:161-172.
64. Sharma S., Kumar L., Mohanty S., et al. Bone marrow mononuclear stem cell infusion improves biochemical parameters and scintigraphy in infants with biliary atresia. Pediatr Surg Int 2011, 27:81-89.