Zusammenfassung
Die meisten Kinder mit einer Gallengangatresie müssen im Verlauf lebertransplantiert werden, nur ca. 20% überleben langfristig mit der eigenen Leber. Verlässliche prognostische Faktoren fehlen bisher, um den individuellen Krankheitsverlauf vorhersagen zu können. Ziel dieser retrospektiven Auswertung der 85 Patienten mit Gallengangatresie unserer Klinik (1993 bis 2003) war der Nachweis möglicher prädiktiver Faktoren, die mit einem transplantatfreien Überleben assoziiert sind. Die 5-Jahres-Überlebensrate mit eigener Leber betrug 40% (34/85), 26 Kinder (31%) waren ikterusfrei. Sowohl das Alter bei der Kasai-Operation (p=0,46) als auch der Grad der Leberfibrose (p=0,95) und die präoperativen Laborwerte korrelierten nicht mit dem Überleben mit eigener Leber (Cox-Regressionsanalyse). Die Höhe des Bilirubins 3, 6 und 12 Monate postoperativ und die Höhe von Aspartat-Aminotransferase und γGT nach 6 Monaten waren mit einem Überleben mit eigener Leber assoziiert. Zusammenfassend unterstützen unsere Daten die Forderung, bei Kindern mit einer Gallengangatresie keine primäre Lebertransplantation durchzuführen, da es keine verlässlichen präoperativen Faktoren gibt, die den weiteren Krankheitsverlauf voraussagen.
Abstract
Most children with bililary atresia require liver transplantation, and only about 20% survive in the long term with their native livers. Prognostic factors that determine disease progression are still lacking. This retrospective survey of 85 BA patients from 1993 to 2003 was aimed to evaluate prognostic factors using the log rank test. After 5 years 40% of the patients are alive with their native livers (35/85), 26 of them with normal bilirubin (31%). Age at Kasai operation (P=0.46), degree of liver fibrosis (P=0.95), and all laboratory test results before Kasai failed to correlate with outcome. Normal levels of bilirubin 3, 6, and 12 months after Kasai and of aspartate aminotransferase with γGT after 6 months are associated with survival with native liver. In conclusion our data demonstrate that a lack of predictive factors must prevent primary liver transplantation in BA patients.
Literatur
Altman RP, Lilly JR, Greenfeld J et al (1997) A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers. Ann Surg 226:348–353
Baerg J, Zuppan C, Klooster M (2004) Biliary atresia – a fifteen-year review of clinical and pathologic factors associated with liver transplantation. J Pediatr Surg 39:800–803
Chardot C, Carton M, Spire-Bendelac N et al (1999) Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996. Hepatology 30:606–611
Davenport M, Puricelli V, Farrant P et al (2004) The outcome of the older (> or =100 days) infant with biliary atresia. J Pediatr Surg 39:575–581
Davenport M, Ure BM, Petersen C, Kobayashi H (2007) Surgerey for biliary atresia – is there a European consensus? Eur J Pediatr Surg 17:180–183
Dhawan A, Trivedi P, Cheeseman P et al (2001) Serum hyaluronic acid as an early prognostic marker in biliary atresia. J Ped Surg 36:443–446
Fouquet V, Alves A, Branchereau S et al (2005) Long-term outcome of pediatric liver transplantation for biliary atresia: A 10-year follow-up in a single center. Liver Transpl 11:152–160
Hadzic N, Davenport M, Tizzard S et al (2003) Long-term survival following Kasai portoenterostomy: is chronic liver disease inevitable? J Pediatr Gastroenterol Nutr 37:430–433
Hung PY, Chen C, Chen WJ et al (2006) Long-term prognosis of patients with biliary atresia: a 25 year summary. J Pediatr Gastroenterol Nutr 42:190–195
Ishak KG (1995) Histological grading and staging of chronic hepatitis C. J Hepatol 22:696–699
Karrer FM, Price MR, Bensard DD et al (1996) Long-term results with the Kasai operation for biliary atresia. Arch Surg 131:493–496
Kasai M, Suzuki S (1959) A new operation for“non correctable” biliary atresia: portoenterostomy. Shijitsu 13:457–481
Kobayashi H, Narumi S, Tamatani T (1999) Serum IFN-inducible protein-10: a new clinical prognostic predictor of hepatocyte death in biliary atresia. J Pediatr Surg 34:308–311
Lykavieris P, Chardot C, Sokhn M et al (2005) Outcome in adulthood of biliary atresia: A study of 63 patients who survived for over 20 years with their native liver. Hepatology 41:366–371
McKiernan PJ, Baker AJ, Kelly DA (2000) The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 355:25–29
Nio M, Ohi R, Miyano T et al (2003) Japanese Biliary Atresia Registry. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg 38:997–1000
Ohi R (2001) Surgery for biliary atresia. Liver 21:175–182
Perlmutter DH, Shepherd RW (2002) Extrahepatic biliary atresia: a diesease or a phenotype? Hepatology 35:1297–1304
Petersen C (2006) Pathogenesis and treatment opportunities for biliary atresia. Clin Liver Dis 10:73–88
Rauschenfels S, Krassmann M, Al-Masri AN et al (2009) Incidence of hepatotropic viruses in biliary atresia. Eur J Pediatr 168:469–476
Schrem H, Till N, Becker T et al (2008) Langzeit-Outcome nach Lebertransplatation. Der Chirurg 79:121–129
Serinet MO, Broué P, Jaquemin E et al (2006) Management of patients with biliary atresia in France: results of a decentralized policy 1986–2002. Hepatology 44:75–86
Shneider BL, Brown MB, Haber B (2006) A multicenter study of the outcome of biliary atresia in the United States 1999 to 2000. J Pediatr 148:467–474
Shteyer E, Ramm GA, Xu C et al (2006) Outcome after portoenterostomy in biliary atresia: pivotal role of degree of liver fibrosis and intensity of stellate cell activation. J Pediatr Gastroenterol Nutr 42:93–99
Sokol RJ, Mack C, Narkewicz MR et al (2003) Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 37:4–21
Vejchapipat P, Theamboonlers A, Chaokhonchai R et al (2004) Serum hepatocyte growth factor and clinical outcome in biliary atresia. J Pediatr Surg 39:1045–1049
Wildhaber BE, Coran AG, Drongowski RA et al (2003) The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients. J Pediatr Surg 38:1480–1485
Interessenkonflikt
Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Leonhardt, J., Kuebler, J., Turowski, C. et al. Prognostische Marker bei Kindern mit Gallengangatresie. Chirurg 80, 628–633 (2009). https://doi.org/10.1007/s00104-009-1708-x
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00104-009-1708-x