Recent advance in primary immune deficiency disorders.
10.3345/kjp.2009.52.6.649
- Author:
Hyoung Jin KANG
1
;
Hee Young SHIN
;
Hyo Seop AHN
Author Information
1. Department of Pediatrics, Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea. hyshin@snu.ac.kr
- Publication Type:Review
- Keywords:
Primary immune deficiency;
Innate immunity;
Adaptive immunity
- MeSH:
Adaptive Immunity;
Complement System Proteins;
Immune System;
Immunity, Innate;
Killer Cells, Natural;
Leukocytes;
Lymphocytes;
Phagocytes
- From:Korean Journal of Pediatrics
2009;52(6):649-654
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The immune system is comprised of cells and molecules whose collective and coordinated response to the introduction of foreign substance is referred to as the immune response. Defense against microbes is mediated by the early reaction (innate immunity) and the late response (adaptive immunity). Innate immunity consists of the epithelial barrier, phagocytes, complement and natural killer cells. Adaptive immunity, a more complex defense reaction, consists of activation of later-developed lymphocytes that, when stimulated by exposure to infectious agents, increase in magnitude and defensive capabilities with each successive exposure. In this review we discuss recent advances in important primary immune deficiency disorders of innate immunity (chronic granulomatous disease, leukocyte adhesion deficiency) and adaptive immunity (severe combined immune deficiency, Wiskott-Aldrich syndrome).