A Case of Congenital Agammaglobulinemia.
- Author:
Sang Yong KIM
1
;
Jong Hyun KIM
;
Jin Han KANG
;
Joon Sung LEE
;
Sang In SHIM
Author Information
1. Department of Pediatrics, Catholic University Medical College, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
Congenital agammaglobulinemia;
B lymphocyte maturation
- MeSH:
Agammaglobulinemia*;
Anti-Bacterial Agents;
Arthritis;
Child;
Cough;
Fever;
Hip Joint;
Humans;
Immunity, Cellular;
Immunoglobulins;
Lymphocytes;
Male;
Osteomyelitis;
Otitis Media, Suppurative;
Pneumonia;
Skin
- From:Journal of the Korean Pediatric Society
1995;38(1):99-103
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Congenital agammaglobulinemia was described first among all human immunodeficiencies, and characterized by defect of B lymphocyte maturation and severe panhypogammaglobulinemia. These patients frequently acquire infections with high-grade extracellular pyogenic pyogenic organisms since infancy. We experienced a case of congenital agammaglobulinemia in a 10 years old male. He had history of recurrent pneumonia and purulent otitis media since late infancy. Also he had recieved operations for pyogenic arthritis of hip joint & osteomyelitis at 4 years of age. He had been suffered from productive cough, intermittent fever and skin infections for 3 months before admission. His immune status revealed panhypogammaglobulinemia and deficiency in mature B lymphocyte, but cell-mediated immunity was intact. He was treated with antibiotics and intravenous immunoglobulin with good results.
