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部分内胆管分流术：治疗进行性家族性肝内胆汁淤积症 1 型顽固性瘙痒的一种方法。

Partial internal biliary diversion: a solution for intractable pruritus in progressive familial intrahepatic cholestasis type 1.

机构信息

Kanchi Kamakoti CHILDS Trust Hospital & The CHILDS Trust Medical Research Foundation, Nungambakkam, Chennai, India.

出版信息

Saudi J Gastroenterol. 2011 May-Jun;17(3):212-4. doi: 10.4103/1319-3767.80387.

DOI:10.4103/1319-3767.80387
PMID:21546727
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3122094/
Abstract

Biliary diversion offers a potential option for intractable pruritus in children with chronic cholestatic disorders. Progressive familial intrahepatic cholestasis (PFIC) is an inherited disorder of impaired bile acid transport and excretion, which presents with jaundice and pruritus in the first few months of life and progresses to cirrhosis by infancy or adolescence. We report a child with PFIC type 1 who underwent internal biliary diversion for intractable pruritus and was relieved of his symptoms.

摘要

胆道分流术为慢性胆汁淤积性疾病所致难治性瘙痒的患儿提供了一种潜在的选择。进行性家族性肝内胆汁淤积症（PFIC）是一种胆汁酸转运和排泄受损的遗传性疾病，患儿在生命最初几个月即出现黄疸和瘙痒，并在婴儿期或青春期进展为肝硬化。我们报告了一例 PFIC 1 型患儿，其因难治性瘙痒行内胆管分流术，症状得到缓解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab33/3122094/c4d26861d6b5/SJG-17-212-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab33/3122094/16477b68dcf5/SJG-17-212-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab33/3122094/0bc2a5a1126a/SJG-17-212-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab33/3122094/c4d26861d6b5/SJG-17-212-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab33/3122094/16477b68dcf5/SJG-17-212-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab33/3122094/0bc2a5a1126a/SJG-17-212-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab33/3122094/c4d26861d6b5/SJG-17-212-g003.jpg

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本文引用的文献

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Partial internal biliary diversion through a cholecystojejunocolonic anastomosis--a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report.通过胆囊空肠结肠吻合术进行部分肝内胆汁转流——一种治疗进行性家族性肝内胆汁淤积症患者的新手术方法：初步报告
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Outcome of partial internal biliary diversion for intractable pruritus in children with cholestatic liver disease.部分性肝内胆汁转流术治疗儿童胆汁淤积性肝病顽固性瘙痒的疗效
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