면역글로불린 A 및 G2, G4 결핍에 동반된 만성 난치성 설사 1례
A Case of Chronic Intractable Diarrhea with IgA, IgG2 and IgG4 Deficiency
안성연, 김영미, 남상욱, 박재홍, 이창훈,
소속 상세정보
안성연 ( Ahn Sung-Ryon )
부산대학교 의과대학 소아과학교실
김영미 ( Kim Young-Mi )
부산대학교 의과대학 소아과학교실
남상욱 ( Nam Sang-Ook )
부산대학교 의과대학 소아과학교실
박재홍 ( Park Jae-Hong )
부산대학교 의과대학 소아과학교실
이창훈 ( Lee Chang-Hun )
부산대학교 의과대학 병리학교실
KMID : 0816120010040020243
Abstract
저자들은 생후 2개월부터 지속되는 만성 설사를 주소로 내원한 환아에서 면역글로불린 정량 검사상 IgA의 결핍과 함께 IgG2 및 IgG4 결핍이 동반되어 있고 영아기에 빈번한 설사와 감염을 동반한 증례를 경험하였기에 보고하는 바이다.
In most cases, acute diarrhea in childhood heals spontaneously, but it may become the form of chronic diarrhea in immunodeficient children and then cause weight loss, dehydration, malabsorption and malnutrition. The immunodeficient diseases associated with chronic diarrhea include severe combined immunodeficiency syndrome, common variable immunodeficiency, acquired immunodeficiency syndrome, agammaglobulinemia or selective IgA deficiency. IgA deficiency is the most common primary immunodeficiency. Because many IgA deficient individuals seem to have compensated for their deficiency with increased IgM production and various nonimmunologic factors, the incidence of gastrointestinal involvement is not prominent. Some of those with IgA deficiency and recurrent infections have been found to also have IgG subclass deficiency. IgA deficiency with IgG2 and IgG4 subclass deficiency have high susceptability to infection and chronic diarrhea. IgG subclass deficiency, when present, is more likely to be found in association with a partial IgA deficiency rather than complete IgA deficiency. We report a 3-month-old male with intractable diarrhea accompanied by IgA, IgG2, and IgG4 deficiency.
키워드
Chronic diarrhea;IgA; IgG2 and IgG4 deficiency
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