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发育医学电子杂志  2023, Vol. 11 Issue (5): 384-388    DOI: 10.3969/j.issn.2095-5340.2023.05.010
  结构畸形   综述 |
法尼醇X 受体影响胆道闭锁肝纤维化机制的研究进展
于成皓 任红霞
(1. 山西医科大学 儿科医学系,山西 太原 030000;2. 山西医科大学附属儿童医院 新生儿外科,山西 太原 030000)
Research progress on the mechanism of farnesoid X receptor affecting liver fibrosis in biliary atresia
Yu Chenghao, Ren Hongxia
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摘要  胆道闭锁是一种罕见的导致新生儿及婴儿胆汁淤积的先天性胆道系统疾病,以肝内外胆管进行性炎症以及肝纤维化为特征,导致胆汁在胆道内大量淤积,并最终引起肝硬化。如不及时治疗,胆道闭锁患儿往往于出生后2 年内死于终末期肝[1-2]。胆道闭锁早期诊断困难的特性与其高病死率一直是困扰小儿外科医师的难题。转录因子法尼醇X 受体(farnesoid X receptor,FXR)是一种表达在人体肝脏,可以调节机体合成和利用胆汁酸的核受体[3]。转化生长因子(transforming growth factor,TGF)-β1 介导的Smad 信号通路是一种在发育胚胎与成熟机体中均起着重要作用的细胞传导通路,参与包括细胞生长、分化与凋亡在内的各种细胞功能。诸多研究表明,FXR 与其介导的TGF-β1/Smad 信号通路在调节胆道闭锁肝纤维化过程中发挥重要作用[4-5]。本文对FXR 直接激活或通过TGF-β1/Smad 信号通路间接激活调节胆道闭锁中肝纤维化进程进行分析与阐述,为揭示FXR 与胆道闭锁肝纤维化的关系提供理论依据。
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关键词:  胆道闭锁  肝纤维化  法尼醇衍生物X受体    
收稿日期:  2022-08-30                出版日期:  2023-09-30      发布日期:  2023-09-27      期的出版日期:  2023-09-30
基金资助: 山西省卫生健康委员会科研课题(2022074);山西省儿童医院院内课题(201928)
通讯作者:  任红霞    E-mail:  renhongxia100@sina.com
引用本文:    
于成皓 任红霞. 法尼醇X 受体影响胆道闭锁肝纤维化机制的研究进展[J]. 发育医学电子杂志, 2023, 11(5): 384-388.
Yu Chenghao, Ren Hongxia. Research progress on the mechanism of farnesoid X receptor affecting liver fibrosis in biliary atresia. Journal of Developmental Medicine(Electronic Version), 2023, 11(5): 384-388.
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http://www.fyyxzz.com/CN/10.3969/j.issn.2095-5340.2023.05.010  或          http://www.fyyxzz.com/CN/Y2023/V11/I5/384
[1] 何峰 刘卓. T 淋巴细胞在胆道闭锁炎症和纤维化发生过程中调控机制的研究进展[J]. 发育医学电子杂志, 2024, 12(1): 75-80.
[2] 吉泽  刘晓书  任红霞. 胆道闭锁无创整合诊断的研究进展[J]. 发育医学电子杂志, 2023, 11(4): 301-307.
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