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胆道闭锁:异中有同。

Biliary atresia: unity in diversity.

作者信息

Petersen Claus

机构信息

Department of Pediatric Surgery, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hanover, Germany.

出版信息

Pediatr Surg Int. 2017 Dec;33(12):1255-1261. doi: 10.1007/s00383-017-4156-6. Epub 2017 Oct 5.

DOI:10.1007/s00383-017-4156-6
PMID:28983682
Abstract

Biliary atresia (BA) is a rare disease of unknown origin and unsatisfying outcome. Single, multicenter and national evaluations of epidemiological and outcome data on BA have been periodically published over the course of decades. However, the diversity of the registered parameters and outcome measures impede comparability and cumulative analysis of these very worthwhile studies. Taking into account the fact that BA is a good example of translational research and transition of patients from pediatric surgery and hepatology to transplant surgery and hepatology in general, the interdisciplinary community should make every effort to develop a common platform upon which further activities are conducted. Extending this topic to BA-related diseases might increase the acceptance of research studies and enhance the effectiveness of any recommendations outlined therein. The use of the Internet-based communication platform and registry on http://www.bard-online.com represents the first step in this direction, and the database should be viewed as a helpful tool that guides further activities.

摘要

胆道闭锁(BA)是一种病因不明且预后不佳的罕见疾病。数十年来,关于BA的流行病学和预后数据的单中心、多中心及全国性评估报告定期发表。然而,所记录参数和预后指标的多样性阻碍了这些极有价值研究的可比性和累积分析。鉴于BA是转化研究以及患者通常从小儿外科和肝病学过渡到移植外科和肝病学的一个典型例子,跨学科群体应全力打造一个开展进一步活动的共同平台。将这一话题扩展至与BA相关的疾病可能会提高研究的接受度,并增强其中所概述的任何建议的有效性。使用基于互联网的交流平台及http://www.bard-online.com上的注册系统是朝着这个方向迈出的第一步,该数据库应被视为指导进一步活动的有用工具。

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本文引用的文献

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Outcomes of laparoscopic Kasai portoenterostomy for biliary atresia: A systematic review.腹腔镜下Kasai肝门空肠吻合术治疗胆道闭锁的疗效:一项系统评价。
J Pediatr Surg. 2017 Feb;52(2):264-267. doi: 10.1016/j.jpedsurg.2016.11.022. Epub 2016 Nov 14.
2
Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis.Kasai 门腔分流术后辅助类固醇治疗与胆道闭锁患者的临床结局:一项更新的荟萃分析。
World J Pediatr. 2017 Feb;13(1):20-26. doi: 10.1007/s12519-016-0052-8. Epub 2016 Oct 15.
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Long-term follow-up for anicteric survival with native liver after redo Kasai: a first report.
Future Sci OA. 2020 Mar 17;6(5):FSO466. doi: 10.2144/fsoa-2019-0153.
再次行肝门空肠吻合术后无黄疸生存并保留自体肝脏的长期随访:首例报告
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4
Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care.胆道闭锁:肝移植的指征、时机及移植前护理的优化
Liver Transpl. 2017 Jan;23(1):96-109. doi: 10.1002/lt.24640.
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Biliary atresia and other cholestatic childhood diseases: Advances and future challenges.先天性胆道闭锁和其他胆汁淤积性儿童疾病:进展与未来挑战。
J Hepatol. 2016 Sep;65(3):631-42. doi: 10.1016/j.jhep.2016.04.032. Epub 2016 May 6.
6
Registries for Biliary Atresia and Related Disorders.胆道闭锁及相关疾病登记处。
Eur J Pediatr Surg. 2015 Dec;25(6):469-73. doi: 10.1055/s-0035-1569152. Epub 2015 Dec 7.
7
Newborn Screening for Biliary Atresia.胆道闭锁的新生儿筛查
Pediatrics. 2015 Dec;136(6):e1663-9. doi: 10.1542/peds.2015-3570.
8
Home-Based Screening for Biliary Atresia Using Infant Stool Color Cards in Canada: Quebec Feasibility Study.加拿大使用婴儿粪便颜色卡进行的基于家庭的胆道闭锁筛查:魁北克可行性研究
J Pediatr Gastroenterol Nutr. 2016 Apr;62(4):536-41. doi: 10.1097/MPG.0000000000001042.
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Gastroenterology. 2016 Jan;150(1):229-241.e5. doi: 10.1053/j.gastro.2015.09.008. Epub 2015 Sep 25.
10
Optimizing the US Diagnosis of Biliary Atresia with a Modified Triangular Cord Thickness and Gallbladder Classification.优化美国改良三角索带厚度和胆囊分类法对胆道闭锁的诊断。
Radiology. 2015 Oct;277(1):181-91. doi: 10.1148/radiol.2015142309. Epub 2015 May 6.