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Prognostische Marker bei Kindern mit Gallengangatresie

Predictive parameters in children with biliary atresia

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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.

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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

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