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Gallengangsatresie

Wo stehen wir heute, und wie geht es weiter?

Biliary atresia

Where are we now and what is next?

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Zusammenfassung

Die interdisziplinäre Vernetzung und die Zentralisation v. a. seltener Krankheiten sind ebenso wie die Transition Merkmale moderner Kindermedizin. Diese Entwicklung kann am Beispiel der Gallengangsatresie („biliary atresia“, BA) exemplarisch dargestellt werden. Denn bereits die frühzeitige Diagnose und Therapie sind Voraussetzungen für einen günstigen klinischen Verlauf, der in diesem Fall ein möglichst langes Überleben mit der eigenen Leber bedeutet. Allerdings repräsentiert die BA nach wie vor die häufigste Indikation zur Lebertransplantation (LTx) im Kindesalter. Aber auch nach einer erfolgreichen Transplantation bleibt jeder BA-Patient ein „Leber-Patient“, der mit fortschreitendem Alter seine Transition in die Erwachsenenmedizin erlebt. Um die klinischen Dimensionen der BA richtig einordnen zu können, muss sich der behandelnde Pädiater vergegenwärtigen, dass immer noch nur sehr geringes Wissen über die BA vorliegt. Mit anderen Worten: Es werden die Symptome einer im Wesen unbekannten Erkrankung behandelt. Aus diesem Grund haben sich in den letzten Jahrzehnten weltweit zahlreiche Arbeitsgruppen zur klinischen und zur translationalen Grundlagenforschung gebildet. Im vorliegenden Beitrag wird daher der aktuelle Stand zusammengefasst, und es werden Perspektiven für die kommenden Jahre aufgezeigt.

Abstract

Interdisciplinary networking and centralization particularly for rare diseases as well as transition are characteristics of modern pediatric medicine. This development can be presented using the example of biliary atresia (BA) because the early diagnosis and treatment are prerequisites for a favorable outcome, which in this case is long-term survival with a native liver; however, BA is still the most common indication for liver transplantation during childhood. Even after successful liver transplantation, every patient with BA remains a so-called liver patient and with advancing age young patients face transition into adult care. To be able to understand the clinical dimension of BA, the treating pediatrician needs to realize that even now little is known about BA. In other words: the symptoms of an essentially unknown disease must be treated. For this reason, during the last decades many working groups were formed worldwide, focusing on clinical and translational basic research. Therefore, this article presents a summary of the current situation and perspectives for treatment of BA in the coming years.

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  1. Klinik für Kinderchirurgie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    C. Petersen & O. Madadi-Sanjani

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Correspondence to C. Petersen.

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C. Petersen und O. Madadi-Sanjani geben an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden von den Autoren keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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Petersen, C., Madadi-Sanjani, O. Gallengangsatresie. Monatsschr Kinderheilkd 167, 1010–1017 (2019). https://doi.org/10.1007/s00112-019-00768-z

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