What's New in Biliary Atresia?

Abstract

The main problem with biliary atresia (BA) is three-fold. Early diagnosis and timely therapy is mandatory in this rare and life-threatening disease. In as much as the aetiology of BA is unknown, all therapeutic attempts are still symptomatically orientated and finally, at the end-stage of the disease, the majority of the patients need organ replacement. Due to promising interdisciplinary cooperation and improved outcomes after liver transplantation, the overall survival rate of patients with BA increased remarkably during the last decades. Additionally, every effort was made in clinical and basic research to obtain a better understanding of the disease and its clinical course. Nevertheless, the nature of biliary atresia still remains unclear and therapeutic options are unsatisfactory. Numerous papers about BA have appeared, reflecting clinical and scientific activity. Considering recent publications and prospective activities, in the following we will summarise what is new in biliary atresia.

Résumé

La problématiqe de l'atrésie des voies biliaires est triple. Il s'agit d'établir un diagnostic précoce pour permettre une prise en charge thérapeutique optimale en raison du risque vital. L'étiologie de l'atrésie des voies biliaires étant inconnue, le traitement reste essentiellement symptomatique et la plupart des patients seront candidats à la transplantation. Grâce à une meilleure concertation pluridisciplinaire et aux progrès de la transplantation hépatique, le taux de survie des patient atteints d'atrésie des voies biliaires s'est nettement amélioré ces dernières annŽes. Par ailleurs, des avancées de la recherche clinique et fondamentale ont permis une meilleure connaissance de la maladie et de son évolution. Néanmoins, la nature de l'atrésie biliaire demeure peu claire et les options thérapeutiques ne sont pas satisfaisantes. En nous appuyant sur de nombreuses publications, témoignant de l'activité clinique et scientifique dans ce domaine, nous présentons une mise au point des nouveautés de l'atrésie des voies biliaires.

Resumen

El principal problema con la atresia biliar (AB) es triple: 1. el diagnóstico precoz y el tratamiento a tiempo son imprescindibles en esta enfermedad rara y amenazadora. 2. Al ser la etiología de la AB desconocida todos los intentos de tratamiento son todavía orientados sintomáticamente y finalmente 3. En fases finales de la enfermedad la mayoría de los pacientes requieren trasplante. Debido a la prometedora cooperación interdisciplinaria y al progresivamente mejor resultado tras el trasplante hepático la sobrevivencia global de los pacientes con AB ha aumentado considerablemente en la últimas décadas. Adicionalmente se siguen haciendo esfuerzos de investigación clínica y básica para mejor comprender la enfermedad y su curso clínico. Sin embargo, la naturaleza de la atresia biliar todavía es poco clara y las opciones terapeúticas no son satisfactorias. Numerosos artículos sobre la AB reflejan esta actividad clínica y científica. Considerando las publicaciones recientes y las actividades prospectivas resumimos el estado actual de esta enfermedad.

Zusammenfassung

Die Gallengangsatresie (BA) ist eine seltene Erkrankung, die oft zu spät diagnostiziert und nicht immer rechtzeitig behandelt wird. Zumal die Ätiologie der BA bis heute nicht bekannt ist, erfolgen alle therapeutischen Ansätze symptomorientiert. Trotz technisch erfolgreicher Portoenterostomie benötigen die meisten der betroffenen Patienten früher oder später eine Lebertransplantation (LTx). Dank vielversprechender interdisziplinärer Kooperation und deutlicher Verbesserung der Ergebnisse nach LTx ist die Lebenserwartung von Kindern mit BA erheblich angestiegen. Außerdem wurden große Fortschritte in der klinischen und auch der Grundlagenforschung gemacht. Das eigentliche Wesen der BA bleibt aber bis heute unklar und die Behandlungsoptionen sind weiterhin unbefriedigend. Jährlich erscheinen zahlreiche Veröffentlichungen zur BA und spiegeln die klinischen und wissenschaftlichen Aktivitäten wider. Unter Berücksichtigung dieser Publikationen sowie zukünftiger Aktivitäten werden wir im Folgenden zusammenfassen, was es Neues zur Gallengangatresie gibt.

Key words

Biliary atresia

Mots-clés

Atrésie de voies biliaires

Palabras clave

Atresia biliar

Schlüsselwörter

Gallengangsatresie

References

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