Chinese Journal of Practical Pediatrics

CJPP

Previous Articles     Next Articles

Problems of liver and spleen related to inborn error of immunity

  

  1. Department of Rheumatology and Immunology，Children’s Hospital of Chongqing Medical University；Ministry of Education Key Laboratory of Child Development and Disorders；National Clinical Research Center for Child Health and Disorders（Chongqing）；China International Science and Technology Cooperation Base of Child Development and Critical Disorders；Chongqing Key Laboratory of Child Infection and Immunity，Chongqing  400014，China
  • Online:2021-07-06 Published:2021-08-31

出生免疫错误相关的肝脾问题

  

  1. 重庆医科大学附属儿童医院风湿免疫科  儿童发育疾病研究教育部重点实验室  国家儿童健康与疾病临床医学研究中心（重庆）  儿童发育重大疾病国家国际科技合作基地  儿童感染免疫重庆市重点实验室， 重庆  400014

Abstract: Currently inborn error of immunity（IEI） has been used to replace primary immunodeficiency disease（PID）. IEI is a genetic disease with abnormal immune function caused by single gene mutation. Liver and spleen are important immune organs in the human body，which are closely related to the infection，lymphatic proliferation and autoimmunity that complicate IEI. This paper makes a summary of the problems of liver and spleen related to IEI.

Key words: inborn error of immunity, primary immunodeficiency disease, lymphatic proliferation, live, spleen

摘要： 目前已用出生免疫错误（IEI）来替代原发性免疫缺陷病这一概念。IEI是一类由单基因突变引起免疫功能异常的遗传性疾病，肝脏和脾脏作为人类重要的免疫器官，与IEI疾病过程中伴发感染、淋巴增殖及自身免疫现象等有重要关联。文章就与IEI相关的肝脾问题进行概述。

关键词: 出生免疫错误, 原发性免疫缺陷病, 淋巴增殖, 肝脏, 脾脏