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Clinical Study on Chronic Granulomatous Disease (CGD) -Occurrence in Siblings-

Journal of the Korean Pediatric Society 1994;37(6):794-803.
Published online June 15, 1994.
Clinical Study on Chronic Granulomatous Disease (CGD) -Occurrence in Siblings-
Yong Hoon Jun, Sung Won Yang, Dong Wook Kim, Hoan Jong Lee, Joong Gon Kim
Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
만성 육아종 질환의 임상적 고찰 -형제에서의 발생-
전용훈, 양성원, 김동욱, 이환종, 김중곤
서울대학교 의과대학 소아과학교실
Abstract
To analyse the age of onset, etiologic microorgnisms, clinical manifestations, managements and genetic variation of chronic granulomatous disease, the authors reviewed four patients who were diagnosed as CGD at Department of Pediatrics, Seoul National Univesity Children's Hospital. They were siblings in relationship-two of them were brothers, the others wete brother-sister. The results were as follows; 1) Initial manifestations developed within 1 year old, and lymphadenopathy associated with BCG vaccination was the most common. 2) In culture study of micro-organism, catalase positive microorganisms such as Staphylococcus aureus, Serratia marcescens, Coagulase (-) Staphylococcus, Enterococcus, Proterus vulgaris, Klebsiella pneumoniae, E. coli and fungus such as Candida albicans were isolated. In 2 cases, culture study revealed no gorwth. 3) Fever, lymphadenopathy, hepatomegaly, suppurative dermatitis and pneumonia were the most common manifestations. In most of cases, antituberculous medications were administered under the impression of tuberculosis without response. 4) Immunologic screening tests including B-cell system, T-cell system, and complement system were within normal limit except NBT test. 5) In spite of severe infections, NBT scores of all the cases were less than those of controls. Liver biopsies of 3 cases showed granuloma formation with characteristic yellow brown pigment-laden macrophages.
Key Words: Chronic granulomatous disease, Siblings


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