Abstract

摘要:

<正>常染色体隐性遗传性多囊肾 (autosomal recessive polycystic kidney disease,ARPKD) 是一种多发于儿童肾脏和胆道系统的严重单基因遗传病[1],以肝门静脉系统发育不全为特征,包括胆管板重塑缺陷、胆管增生和先天性肝纤维化 (congenital hepatic fibrosis,CHF) [2-4]。ARPKD发病率为1∶20 000～40 000,属罕见病[5],合并CHF的发病率更低。本文将一家系 (3姐弟)

Details

Title
常染色体隐性遗传性多囊肾合并先天性肝纤维化一家系3例报告及文献复习
Author
曹丽丽; 董漪; 徐志强; 陈大为; 王福川; 甘雨; 王丽旻; 闫建国; 王璞; 李爱芹; 张敏
Pages
166-168
Section
Case reports
Publication year
2019
Publication date
2019
Publisher
Journal of Clinical Hepatology
ISSN
10015256
e-ISSN
20973497
Source type
Scholarly Journal
Language of publication
Chinese
DOI
https://doi.org/10.3969/j.issn.1001-5256.2019.01.033
ProQuest document ID
3240456067
Copyright
© 2019. This work is published under https://creativecommons.org/licenses/by-nc-nd/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.