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 |
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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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