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胆道闭锁及肝外胆管先天性疾病

Biliary atresia and congenital disorders of the extrahepatic bile ducts.

作者信息

Islek Ali, Tumgor Gokhan

机构信息

Department of Pediatric Gastroenterology, Cukurova University School of Medicine, Adana 01320, Turkey.

出版信息

World J Gastrointest Pharmacol Ther. 2022 Jul 5;13(4):33-46. doi: 10.4292/wjgpt.v13.i4.33.

DOI:10.4292/wjgpt.v13.i4.33
PMID:36051179
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9297290/
Abstract

Biliary atresia (BA) and choledochal cysts are diseases of the intrahepatic and extrahepatic biliary tree. While their exact etiopathogeneses are not known, they should be treated promptly due to the potential for irreversible parenchymal liver disease. A diagnosis of BA may be easy or complicated, but should not be delayed. BA is always treated surgically, and performing the surgery before the age of 2 mo greatly increases its effectiveness and extends the time until the need for liver transplantation arises. While the more common types of choledochal cysts require surgical treatment, some can be treated with endoscopic retrograde cholangiopancreatography. Choledochal cysts may cause recurrent cholangitis and the potential for malignancy should not be ignored.

摘要

胆道闭锁(BA)和胆总管囊肿是肝内和肝外胆管树的疾病。虽然它们的确切病因尚不清楚,但由于存在发生不可逆实质性肝病的可能性,应及时进行治疗。BA的诊断可能简单或复杂,但不应延迟。BA总是通过手术治疗,在2个月龄前进行手术可大大提高其疗效,并延长至需要进行肝移植的时间。虽然较常见类型的胆总管囊肿需要手术治疗,但有些可以通过内镜逆行胰胆管造影术进行治疗。胆总管囊肿可能导致复发性胆管炎,其恶变的可能性不容忽视。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e51b/9297290/676a9add7b92/WJGPT-13-33-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e51b/9297290/6d3aa69b031f/WJGPT-13-33-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e51b/9297290/2c563fa077ac/WJGPT-13-33-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e51b/9297290/676a9add7b92/WJGPT-13-33-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e51b/9297290/6d3aa69b031f/WJGPT-13-33-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e51b/9297290/2c563fa077ac/WJGPT-13-33-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e51b/9297290/676a9add7b92/WJGPT-13-33-g003.jpg

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引用本文的文献

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Int J Surg Case Rep. 2025 Jun 19;133:111534. doi: 10.1016/j.ijscr.2025.111534.
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Outcomes of Liver Transplantation in Patients With Congenital Heart Disease and Biliary Atresia. A Multicenter Analysis.先天性心脏病合并胆道闭锁患者肝移植的结局。一项多中心分析。
Pediatr Transplant. 2025 Aug;29(5):e70110. doi: 10.1111/petr.70110.
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Engineered Exosomes Carrying Super-Repressor IκB Reduced Biliary Atresia-Induced Liver Fibrosis in Minipig and Mouse Models.

本文引用的文献

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Pediatr Surg Int. 2022 Feb;38(2):193-199. doi: 10.1007/s00383-021-05047-1. Epub 2021 Dec 2.
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Impact of the Kasai Procedure and the Length of Native Liver Survival Time on Outcomes of Liver Transplantation for Biliary Atresia.Kasai 手术和肝内生存时间对胆道闭锁肝移植结局的影响。
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