頁籤選單縮合
題 名 | Chronic Granulomatous Disease: A Case Report=慢性肉芽腫疾病:一病例報告 |
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作 者 | 馬瑞杉; 陳伯彥; 傅令嫻; 遲景上; 黃雅芳; 林清淵; 謝奇璋; | 書刊名 | 微免與感染雜誌 |
卷 期 | 33:2 2000.06[民89.06] |
頁 次 | 頁118-122 |
分類號 | 415.96 |
關鍵詞 | 慢性肉芽腫疾病; Chronic granulomatous disease; Flow cytometry; Phagocyte respiratory burst; |
語 文 | 英文(English) |
英文摘要 | Chronic granulomatous disease (CGD) is a rare inherited disorder caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex of phagocytic leukocytes. The leukocytes of the CGD patients cannot produce adequate amount of superoxide and other oxygen metabolites which are toxic to microorganisms. As a result, the phagocytes fail to kill the ingested microorganisms, especially those with catalase activity. Typically, CGD patients suffer from recurrent pyogenic infections starting from the first year of life. We report a young boy who had experienced recurrent perianal abscess, osteomyelitis and bacterial enterocolitis. Flow cytometric analysis revealed defects in the neutrophil respiratory burst pathway and defined the carrier state of his mother and younger sister. He received antimicrobial prophylaxis at our out-patient clinics and remained well at present. We try to make clinical physician keep in mind the diagnosis of CGD by presenting this typical case. In the meantime, we review the recent literature regarding the advances in diagnosis and management of CGD. |
本系統中英文摘要資訊取自各篇刊載內容。