| 进行性家族性肝内胆汁淤积 |
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| 引用本文： | 陈朱波,姚定康. 进行性家族性肝内胆汁淤积[J]. 国际消化病杂志, 2006, 26(4): 279-281 |
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| 作者姓名： | 陈朱波 姚定康 |
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| 作者单位： | 第二军医大学附属长海医院,200433;第二军医大学附属长海医院,200433 |
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| 摘 要： | 进行性家族性肝内胆汁淤积(PFIC)是一组常染色体隐性遗传肝细胞源性儿童胆汁淤积症。根据病因可以分为3型：1型源于ATP8B1基因的突变,2型源于ABCB11基因的突变,3型源于MDR3基因的突变。临床以有胆汁淤积以及严重的皮肤瘙痒为特征。治疗方法包括药物治疗、外科部分胆道外分流术和肝移植三种方法。
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| 关 键 词： | 进行性家族性肝内胆汁淤积 基因突变 肝移植 |
| 收稿时间： | 2005-05-17 |
| 修稿时间： | 2005-05-17 |
Progressive familial intrahepatic cholestasis |
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| CHEN Zhu-bo,YAO Ding-king. Progressive familial intrahepatic cholestasis[J]. International Journal of Digestive Disease, 2006, 26(4): 279-281 |
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| Authors: | CHEN Zhu-bo YAO Ding-king |
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| Abstract: | Progressive familial intrahepatic choleatasia(PFIC)is an autosomal recessive inherited children liver cholestasis characterized by severe jaundice and pruritus.It can be categorizated three types by their causes,and type 1 is caused by muta- tion of ATP8B1 gene,type 2 is caused by mutation of ABCBll gene,whereas type 3 is caused by mutation of MDR3 gene. Therapy to this desease includes medical treatment,partial external biliary diversion and liver transplantation. |
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| Keywords: | Progressive familial intrahepatic cholestasis(PFIC) Gene mutation Liver transplantation |
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