J Korean Pediatr Soc.
1995 Jan;38(1):99-103.
A Case of Congenital Agammaglobulinemia
- Affiliations
-
- 1Department of Pediatrics, Catholic University Medical College, Seoul, Korea.
- 2Department of Clinical Pathology, Catholic University Medical College, Seoul, Korea.
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.
