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A Case of Congenital Agammaglobulinemia

Journal of the Korean Pediatric Society 1995;38(1):99-103.
Published online January 15, 1995.
A Case of Congenital Agammaglobulinemia
Sang Yong Kim1, Jong Hyun Kim1, Jin Han Kang1, Joon Sung Lee1, Sang In Shim2
1Department of Pediatrics, Catholic University Medical College, Seoul, Korea
2Department of Clinical Pathology, Catholic University Medical College, Seoul, Korea
선천성 무감마글로불린혈증 1례
김상용1, 김종현1, 강진한1, 이준성1, 심상인2
1가톨릭대학교 의과대학 소아과학교실
2가톨릭대학교 의과대학 임상병리학교실
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.
Key Words: Congenital agammaglobulinemia, B lymphocyte maturation


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