All issues > Volume 52(6); 2009

Review Article

Korean J Pediatr. 2009;52(6):649-654. Published online June 15, 2009.

Recent advance in primary immune deficiency disorders

Hyoung Jin HJ Kan¹, Hee Young HY Shin¹, Hyo Seop HS Ahn¹

¹Department of Pediatrics, Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea

Correspondence Hee Young HY Shin ,Email: hyshin@snu.ac.kr

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).

Keywords :Primary immune deficiency, Innate immunity, Adaptive immunity