Biliary atresia: how medical complications and therapies impact outcome

References

• Haber BA, Russo P. Biliary atresia. Gastroenterol. Clin. North Am.32(3), 891–911 (2003).
   PubMed Web of Science ®Google Scholar
• Wildhaber BE, Majno P, Mayr J et al. Biliary atresia: Swiss national study, 1994–2004. J. Pediatr. Gastroenterol. Nutr.46(3), 299–307 (2008).
   PubMed Web of Science ®Google Scholar
• Shneider BL, Brown MB, Haber B et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J. Pediatr.148(4), 467–474 (2006).
   PubMed Web of Science ®Google Scholar
• Schwarz K, Shepherd R, Magee J et al. Clinical and demographic features of three major biliary atresia phenotypes in the BARC. Presented at: American Association for the Study of Liver Diseases, 59th Annual Meeting. San Francisco, CA, USA, 31 October–4 November 2008.
   Google Scholar
• Serinet MO, Broue P, Jacquemin E et al. Management of patients with biliary atresia in France: results of a decentralized policy 1986–2002. Hepatology44(1), 75–84 (2006).
   PubMed Web of Science ®Google Scholar
• Davenport M, De Ville de Goyet J, Stringer MD et al. Seamless management of biliary atresia in England and Wales (1999–2002). Lancet363(9418), 1354–1357 (2004).
   PubMed Web of Science ®Google Scholar
• Nio M, Ohi R, Miyano T, Saeki M, Shiraki K, Tanaka K. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J. Pediatr. Surg.38(7), 997–1000 (2003).
   PubMed Web of Science ®Google Scholar
• Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology46(2), 566–581 (2007).
   PubMed Web of Science ®Google Scholar
• Chardot C, Serinet MO. Prognosis of biliary atresia: what can be further improved? J. Pediatr.148(4), 432–435 (2006).
   PubMed Web of Science ®Google Scholar
• Altman RP, Lilly JR, Greenfeld J, Weinberg A, van Leeuwen K, Flanigan L. A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers. Ann. Surg.226(3), 348–353; discussion 353–345 (1997).
   PubMed Web of Science ®Google Scholar
• McKiernan PJ, Baker AJ, Lloyd C, Mieli-Vergani G, Kelly DA. British paediatric surveillance unit study of biliary atresia: outcome at 13 years. J. Pediatr. Gastroenterol. Nutr.48(1), 78–81 (2009).
   PubMed Web of Science ®Google Scholar
• Ryckman F, Fisher R, Pedersen S et al. Improved survival in biliary atresia patients in the present era of liver transplantation. J. Pediatr. Surg.28(3), 382–385; discussion 386 (1993).
   PubMed Web of Science ®Google Scholar
• Balistreri WF, Grand R, Hoofnagle JH et al. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology23(6), 1682–1692 (1996).
   PubMed Web of Science ®Google Scholar
• Kasai M, Suzuki H, Ohashi E, Ohi R, Chiba T, Okamoto A. Technique and results of operative management of biliary atresia. World J. Surg.2(5), 571–579 (1978).
   PubMedGoogle Scholar
• Karrer FM, Lilly JR, Stewart BA, Hall RJ. Biliary atresia registry, 1976 to 1989. J. Pediatr. Surg.25(10), 1076–1080; discussion 1081 (1990).
   PubMed Web of Science ®Google Scholar
• Schreiber RA, Barker CC, Roberts EA et al. Biliary atresia: the Canadian experience. J. Pediatr.151(6), 659–665, 665, e651 (2007).
   PubMed Web of Science ®Google Scholar
• Davenport M, Caponcelli E, Livesey E, Hadzic N, Howard E. Surgical outcome in biliary atresia: etiology affects the influence of age at surgery. Ann. Surg.247(4), 694–698 (2008).
   PubMed Web of Science ®Google Scholar
• Hsiao CH, Chang MH, Chen HL et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology47(4), 1233–1240 (2008).
   PubMed Web of Science ®Google Scholar
• Mushtaq I, Logan S, Morris M et al. Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry. BMJ319(7208), 471–477 (1999).
   PubMed Web of Science ®Google Scholar
• Kasai M, Suzuki S. A new operation for non-correctable biliary atresia – portoenterostomy. Shujyutsu13, 733–739 (1959).
   Google Scholar
• Davenport M, Ure BM, Petersen C, Kobayashi H. Surgery for biliary atresia – is there a European consensus? Eur J. Pediatr. Surg.17(3), 180–183 (2007).
   PubMed Web of Science ®Google Scholar
• Matsuo S, Suita S, Kubota M, Shono K, Kamimura T, Kinugasa Y. Hazards of hepatic portocholecystostomy in biliary atresia. Eur J. Pediatr. Surg.11(1), 19–23 (2001).
   PubMed Web of Science ®Google Scholar
• Martinez-Ferro M, Esteves E, Laje P. Laparoscopic treatment of biliary atresia and choledochal cyst. Semin. Pediatr. Surg.14(4), 206–215 (2005).
   PubMedGoogle Scholar
• Meehan JJ, Elliott S, Sandler A. The robotic approach to complex hepatobiliary anomalies in children: preliminary report. J. Pediatr. Surg.42(12), 2110–2114 (2007).
   PubMed Web of Science ®Google Scholar
• Tsao K, Rosenthal P, Dhawan K et al. Comparison of drainage techniques for biliary atresia. J. Pediatr. Surg.38(7), 1005–1007 (2003).
   PubMed Web of Science ®Google Scholar
• Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Auvert B. Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996. Hepatology30(3), 606–611 (1999).
   PubMed Web of Science ®Google Scholar
• Davenport M, Tizzard SA, Underhill J, Mieli-Vergani G, Portmann B, Hadzic N. The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study. J. Pediatr.149(3), 393–400 (2006).
   PubMed Web of Science ®Google Scholar
• Davenport M, Savage M, Mowat AP, Howard ER. Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup. Surgery113(6), 662–668 (1993).
   PubMed Web of Science ®Google Scholar
• De Matos V, Erlichman J, Russo PA, Haber BA. Does “cystic” biliary atresia represent a distinct clinical and etiological subgroup? A series of three cases. Pediatr. Dev. Pathol.8(6), 725–731 (2005).
   PubMed Web of Science ®Google Scholar
• Muise AM, Turner D, Wine E, Kim P, Marcon M, Ling SC. Biliary atresia with choledochal cyst: implications for classification. Clin. Gastroenterol. Hepatol.4(11), 1411–1414 (2006).
   PubMed Web of Science ®Google Scholar
• Nio M, Hayashi Y, Sano N, Ishii T, Sasaki H, Ohi R. Long-term efficacy of partial splenic embolization in children. J. Pediatr. Surg.38(12), 1760–1762 (2003).
   PubMed Web of Science ®Google Scholar
• Wu ET, Chen HL, Ni YH et al. Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome. Pediatr. Surg. Int.17(5–6), 390–395 (2001).
   PubMed Web of Science ®Google Scholar
• Lai HS, Chen WJ, Chen CC, Hung WT, Chang MH. Long-term prognosis and factors affecting biliary atresia from experience over a 25 year period. Chang Gung Med. J.29(3), 234–239 (2006).
   PubMedGoogle Scholar
• Luo Y, Zheng S. Current concept about postoperative cholangitis in biliary atresia. World J. Pediatr.4(1), 14–19 (2008).
   PubMed Web of Science ®Google Scholar
• Bu LN, Chen HL, Chang CJ et al. Prophylactic oral antibiotics in prevention of recurrent cholangitis after the Kasai portoenterostomy. J. Pediatr. Surg.38(4), 590–593 (2003).
   PubMed Web of Science ®Google Scholar
• Ernest van Heurn LW, Saing H, Tam PK. Cholangitis after hepatic portoenterostomy for biliary atresia: a multivariate analysis of risk factors. J. Pediatr.142(5), 566–571 (2003).
   PubMed Web of Science ®Google Scholar
• Oh M, Hobeldin M, Chen T, Thomas DW, Atkinson JB. The Kasai procedure in the treatment of biliary atresia. J. Pediatr. Surg.30(7), 1077–1080; discussion 1080–1071 (1995).
   PubMed Web of Science ®Google Scholar
• Sasaki H, Nio M, Hayashi Y, Ishii T, Sano N, Ohi R. Problems during and after pregnancy in female patients with biliary atresia. J. Pediatr. Surg.42(8), 1329–1332 (2007).
   PubMed Web of Science ®Google Scholar
• Subramaniam R, Doig CM, Bowen J, Bruce J. Initial response to portoenterostomy determines long-term outcome in patients with biliary atresia. J. Pediatr. Surg.35(4), 593–597 (2000).
   PubMed Web of Science ®Google Scholar
• Vazquez-Estevez J, Stewart B, Shikes RH, Hall RJ, Lilly JR. Biliary atresia: early determination of prognosis. J. Pediatr. Surg.24(1), 48–50; discussion 50–41 (1989).
   PubMed Web of Science ®Google Scholar
• Inoue Y, Kato Y, Tamura T et al. Prognostic implications of bile lakes after surgery for biliary atresia. J. Pediatr. Surg.43(12), 2165–2168 (2008).
   PubMed Web of Science ®Google Scholar
• Jiang CB, Lee HC, Yeung CY et al. A scoring system to predict the need for liver transplantation for biliary atresia after Kasai portoenterostomy. Eur. J. Pediatr.162(9), 603–606 (2003).
   PubMed Web of Science ®Google Scholar
• Yanchar NL, Shapiro AM, Sigalet DL. Is early response to portoenterostomy predictive of long-term outcome for patients with biliary atresia? J. Pediatr. Surg.31(6), 774–778 (1996).
   PubMed Web of Science ®Google Scholar
• Biecker E, Classen L, Sauerbruch T, Schepke M. Does therapy of oesophageal varices influences the progression of varices? Eur. J. Gastroenterol. Hepatol. (2009).
   PubMedGoogle Scholar
• Gonzalez R, Zamora J, Gomez-Camarero J, Molinero LM, Banares R, Albillos A. Meta-analysis: combination endoscopic and drug therapy to prevent variceal rebleeding in cirrhosis. Ann. Intern. Med.149(2), 109–122 (2008).
   PubMed Web of Science ®Google Scholar
• Duche M, Habes D, Roulleau P, Haas V, Jacquemin E, Bernard O. Prophylactic endoscopic sclerotherapy of large esophagogastric varices in infants with biliary atresia. Gastrointest. Endosc.67(4), 732–737 (2008).
   PubMed Web of Science ®Google Scholar
• Miga D, Sokol RJ, Mackenzie T, Narkewicz MR, Smith D, Karrer FM. Survival after first esophageal variceal hemorrhage in patients with biliary atresia. J. Pediatr.139(2), 291–296 (2001).
   PubMed Web of Science ®Google Scholar
• Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology, 41(2), 366–371 (2005).
   PubMed Web of Science ®Google Scholar
• Utterson EC, Shepherd RW, Sokol RJ et al. Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation. J. Pediatr.147(2), 180–185 (2005).
   PubMed Web of Science ®Google Scholar
• DeRusso PA, Ye W, Shepherd R et al. Growth failure and outcomes in infants with biliary atresia: a report from the Biliary Atresia Research Consortium. Hepatology46(5), 1632–1638 (2007).
   PubMed Web of Science ®Google Scholar
• Greer R, Lehnert M, Lewindon P, Cleghorn GJ, Shepherd RW. Body composition and components of energy expenditure in children with end-stage liver disease. J. Pediatr. Gastroenterol. Nutr.36(3), 358–363 (2003).
   PubMed Web of Science ®Google Scholar
• Pierro A, Koletzko B, Carnielli V et al. Resting energy expenditure is increased in infants and children with extrahepatic biliary atresia. J. Pediatr. Surg.24(6), 534–538 (1989).
   PubMed Web of Science ®Google Scholar
• Baker A, Stevenson R, Dhawan A, Goncalves I, Socha P, Sokal E. Guidelines for nutritional care for infants with cholestatic liver disease before liver transplantation. Pediatr. Transplant.11(8), 825–834 (2007).
   PubMed Web of Science ®Google Scholar
• Feranchak AP, Sokol R. Medical and nutritional management of cholestasis in infants and children. In: Liver Disease in Children. Suchy, FJ, Sokol, RJ, Balistreri, WF (Eds). Cambridge University Press, NY, USA, 190–231 (2007).
   Google Scholar
• Novy MA, Schwarz KB. Nutritional considerations and management of the child with liver disease. Nutrition13(3), 177–184 (1997).
   PubMed Web of Science ®Google Scholar
• Meyers RL, Book LS, O’Gorman MA 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.38(3), 406–411 (2003).
   PubMed Web of Science ®Google Scholar
• Dillon PW, Owings E, Cilley R, Field D, Curnow A, Georgeson K. Immunosuppression as adjuvant therapy for biliary atresia. J. Pediatr. Surg.36(1), 80–85 (2001).
   PubMed Web of Science ®Google Scholar
• Muraji T, Nio M, Ohhama Y et al. Postoperative corticosteroid therapy for bile drainage in biliary atresia – a nationwide survey. J. Pediatr. Surg.39(12), 1803–1805 (2004).
   PubMed Web of Science ®Google Scholar
• Escobar MA, Jay CL, Brooks RM et al. Effect of corticosteroid therapy on outcomes in biliary atresia after Kasai portoenterostomy. J. Pediatr. Surg.41(1), 99–103; discussion 199–103 (2006).
   PubMed Web of Science ®Google Scholar
• 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.103(3), 712–719 (2008).
   PubMed Web of Science ®Google Scholar
• Davenport M, Stringer MD, Tizzard SA, McClean P, Mieli-Vergani G, Hadzic N. Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia. Hepatology46(6), 1821–1827 (2007).
   PubMed Web of Science ®Google Scholar
• Hagey LR, Crombie DL, Espinosa E, Carey MC, Igimi H, Hofmann AF. Ursodeoxycholic acid in the Ursidae: biliary bile acids of bears, pandas, and related carnivores. J. Lipid Res.34(11), 1911–1917 (1993).
   PubMed Web of Science ®Google Scholar
• Bachrach WH, Hofmann AF. Ursodeoxycholic acid in the treatment of cholesterol cholelithiasis. Part I. Dig. Dis. Sci.27(8), 737–761 (1982).
   PubMed Web of Science ®Google Scholar
• Flamm S, Kaplan M. Ursodeoxycholic acid in the treatment of primary biliary cirrhosis. In: Rose DB. UpToDate. Bonis P (Ed.). UpToDate, MA, USA (2008).
   Google Scholar
• Kotb MA. Review of historical cohort: ursodeoxycholic acid in extrahepatic biliary atresia. J. Pediatr. Surg.43(7), 1321–1327 (2008).
   PubMed Web of Science ®Google Scholar
• Willot S, Uhlen S, Michaud L et al. Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia. Pediatrics122(6), e1236–e1241 (2008).
   PubMed Web of Science ®Google Scholar
• Stringer MD, Davison SM, Rajwal SR, McClean P. Kasai portoenterostomy: 12-year experience with a novel adjuvant therapy regimen. J. Pediatr. Surg.42(8), 1324–1328 (2007).
   PubMed Web of Science ®Google Scholar
• Okamoto T, Yokoi A, Okamoto S et al. Pretransplant risk factors and optimal timing for living-related liver transplantation in biliary atresia: experience of one Japanese children’s hospital and transplantation center. J. Pediatr. Surg.43(3), 489–494 (2008).
   PubMed Web of Science ®Google Scholar
• Barshes NR, Lee TC, Balkrishnan R, Karpen SJ, Carter BA, Goss JA. Orthotopic liver transplantation for biliary atresia: the USA experience. Liver Transpl.11(10), 1193–1200 (2005).
   PubMed Web of Science ®Google Scholar
• Fouquet V, Alves A, Branchereau S et al. Long-term outcome of pediatric liver transplantation for biliary atresia: a 10-year follow-up in a single center. Liver Transpl.11(2), 152–160 (2005).
   PubMed Web of Science ®Google Scholar
• Chen CL, Concejero A, Wang CC et al. Living donor liver transplantation for biliary atresia: a single-center experience with first 100 cases. Am. J. Transplant.6(11), 2672–2679 (2006).
   PubMed Web of Science ®Google Scholar
• Otte JB. History of pediatric liver transplantation. Where are we coming from? Where do we stand? Pediatr. Transplant6(5), 378–387 (2002).
   PubMed Web of Science ®Google Scholar
• Howard ER, MacLean G, Nio M, Donaldson N, Singer J, Ohi R. Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England. J. Pediatr. Surg.36(6), 892–897 (2001).
   PubMed Web of Science ®Google Scholar
• Kuroda T, Saeki M, Nakano M, Morikawa N. Biliary atresia, the next generation: a review of liver function, social activity, and sexual development in the late postoperative period. J. Pediatr. Surg.37(12), 1709–1712 (2002).
   PubMed Web of Science ®Google Scholar
• Kuroda T, Saeki M, Morikawa N, Fuchimoto Y. Biliary atresia and pregnancy: puberty may be an important point for predicting the outcome. J. Pediatr. Surg.40(12), 1852–1855 (2005).
   PubMed Web of Science ®Google Scholar
• Kuroda T, Saeki M, Morikawa N, Watanabe K. Management of adult biliary atresia patients: should hard work and pregnancy be discouraged? J. Pediatr. Surg.42(12), 2106–2109 (2007).
   PubMed Web of Science ®Google Scholar
• Hadzic N, Davenport M, Tizzard S, Singer J, Howard ER, Mieli-Vergani G. Long-term survival following Kasai portoenterostomy: is chronic liver disease inevitable? J. Pediatr. Gastroenterol. Nutr.37(4), 430–433 (2003).
   PubMed Web of Science ®Google Scholar
• Nio M, Ohi R, Hayashi Y, Endo N, Ibrahim M, Iwami D. Current status of 21 patients who have survived more than 20 years since undergoing surgery for biliary atresia. J. Pediatr. Surg.31(3), 381–384 (1996).
   PubMed Web of Science ®Google Scholar
• Nio M, Ohi R, Shimaoka S, Iwami D, Sano N. The outcome of surgery for biliary atresia and the current status of long-term survivors. Tohoku J. Exp. Med.181(1), 235–244 (1997).
   PubMed Web of Science ®Google Scholar
• Davenport M. Biliary atresia: outcome and management. Indian J. Pediatr.73(9), 825–828 (2006).
   PubMedGoogle Scholar
• Valayer J. Conventional treatment of biliary atresia: long-term results. J. Pediatr. Surg.31(11), 1546–1551 (1996)
   PubMed Web of Science ®Google Scholar