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胆道闭锁有哪些新进展?

What's new in biliary atresia?

作者信息

Petersen C, Ure B M

机构信息

Department of Paediatric Surgery, Medical School Hannover, Hannover, Germany.

出版信息

Eur J Pediatr Surg. 2003 Feb;13(1):1-6. doi: 10.1055/s-2003-38294.

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.

摘要

胆道闭锁(BA)的主要问题有三个方面。对于这种罕见且危及生命的疾病,早期诊断和及时治疗至关重要。由于BA的病因不明,所有治疗尝试仍以对症治疗为导向,最终,在疾病末期,大多数患者需要器官置换。由于跨学科合作前景良好且肝移植后预后改善,在过去几十年中,BA患者的总体生存率显著提高。此外,临床和基础研究都在尽一切努力更好地了解该疾病及其临床病程。然而,胆道闭锁的本质仍不清楚,治疗选择也不尽人意。关于BA的众多论文不断涌现,反映了临床和科研活动。考虑到近期的出版物和前瞻性研究,接下来我们将总结胆道闭锁领域的新进展。

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