天然免疫缺陷病的研究进展

Progress of innate hninunity defect disease

天然免疫缺陷是原发性免疫缺陷病的一种。根据最新文献报道，天然免疫缺陷病主要包括七种疾病，这些疾病发病的根本原因均为基因突变。其中三种疾病的病因与NF-κB信号通路受阻有关，而有两种疾病与一般原发性免疫缺陷病发病机制不同，患者被相关的病毒感染后才会诱导发病。天然免疫缺陷在原发性免疫缺陷病中占有较重要的位置，但是由于对此病的认识不足，临床上很多天然免疫缺陷的疾病无法确诊。

Primary immunodeficiency diseases can be divided into eight diseases, including defects in innate immunity. According to new literature, defects in innate immunity involves seven diseases which am all resulted from gene mutation. Three diseases have a correlation with NF-κB signaling pathway. Different from common primary immunodeficiency diseases, two diseases have a correlation with virus infection. Defects in innate immunity play an important role in primary immunodeficiency diseases. But it can＇ t make a definite diagnosis because of incompletely understanding.