Allergy Asthma Proc. 2024 Sep 1;45(5):299-304. doi: 10.2500/aap.2024.45.240049.
The immune system is divided into two major branches: innate and adaptive. The innate immune system is the body's first line of defense and rapidly responds in a nonspecific manner to various microorganisms, foreign materials, or injuries. Phagocytes, which include macrophages, monocytes, and neutrophils, are innate immune cells that can surround and kill microorganisms, ingest foreign material, and remove dead cells. They also indirectly boost both innate and adaptive immune responses through various activation signals. Phagocytic defects characteristically lead to fungal and bacterial infections of the respiratory tract, lymph nodes, skin, and other organ systems, and they are commonly associated with inflammatory bowel disease. This primer will review high-yield innate defects of phagocytic cells, including defects of respiratory (oxidative) burst, defects of neutrophil migration, cyclic and severe congenital neutropenias and associated disorders, and other phagocyte defect disorders.
先天免疫和适应性免疫。先天免疫系统是身体的第一道防线,能够以非特异性方式快速应对各种微生物、异物或损伤。吞噬细胞包括巨噬细胞、单核细胞和中性粒细胞,是先天免疫细胞,能够包围并杀死微生物、吞噬异物和清除死亡细胞。它们还通过各种激活信号间接增强先天和适应性免疫反应。吞噬细胞缺陷特征性地导致呼吸道、淋巴结、皮肤和其他器官系统的真菌感染和细菌感染,并且通常与炎症性肠病有关。本指南将回顾吞噬细胞的高收益先天缺陷,包括呼吸(氧化)爆发缺陷、中性粒细胞迁移缺陷、周期性和严重先天性中性粒细胞减少症及相关疾病,以及其他吞噬细胞缺陷疾病。