A Case Of Common Variable Immunodeficiency with Fatal Measles |
Su Jin Cho, So Hyun Kim, Soo Jung Lee, Kyung Ha Yoo, Kyung Hyo Kim |
Department of Pediatrics, College of Medicine, Ewha Womans University, Seoul, Korea |
홍역으로 사망한 공통 가변성 면역 부전 1례 |
조수진, 김소현, 이수정, 유경하, 김경효 |
이화여자대학교 의과대학 소아과학교실 |
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Abstract |
Common variable immunodeficiency(CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, and a variety of immunological abnormalities. We report a 10-year-old boy diagnosed with CVID and a fatal case of measles. He was admitted for fever, cough, conjunctival injection with purulent discharge and maculopapular rash with some scattered pinpoint sized petechial lesions on the whole body for a duration of 5 days. Laboratory findings showed hypogammaglobulinemia [IgG 617 mg/dL(639-1,349 mg/dL), IgA
8.5 mg/dL(70-312 mg/dL), IgM 34.1 mg/dL(56-352 mg/dL)] and anti-measles antibody and anti HIV antibody were negative. He was treated with intravenous immunoglobulin 700 mg/kg on admission. Radiologic findings progressed from hyperaeration to fluffy margined interstitial and alveolar infiltrations on both lung fields. In spite of supportive and ventilator therapy, his pneumonia progressed to acute respiratory distress syndrome and he expired. |
Key Words:
Common variable immunodeficiency, Measles |
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