All issues > Volume 28(3); 1985

Original Article

J Korean Pediatr Soc. 1985;28(3):263-282. Published online March 31, 1985.

Primary Immunode Ficiency Disorders in Infancy and Childhood.

Hoan Jong Lee, Jung han Choi, Jeong Kee Seo, Hyo Sup Ahn, Kwang Wook Ko

¹Department of Pediatrics, College of Medicine,Seoul National University,Seoul,Korea

Abstract

The immunodeficiency disorders are a diverse group of illness which results from defectsin one or more of the four major mechanisms of immune system-antibody-mediated(B cell) immunity, cell-mediated(T cell) immunity, phagocytosis, and complements. Though immunodeficiency disorders are uncommon, the possibility should be considered in any individuals with recurrent or persistent infection. During the period between January, 1973 and October, 1984, we experienced 13 cases of primary immunodeficiency disorders-congenital agammaglobulinemia(2 cases), selective IgG deficiency(1 case), hyper-IgE syndrome(1 case), Di George syndrome(1 case), severe combined immunodeficiency disease(2 cases), chronic granulomatous disease(2 cases), ataxia-telangie-ctasia (1 case), and chronic neutropenia(3 cases). We presented the patients briefly and reviewed the literature, with special reference to early detection and diagnosis of primary immunodeficiency disorders.

Keywords :Primary immunodeficiency disorders