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目的总结分析感染洋葱伯克霍尔德菌的X连锁慢性肉芽肿病患儿的临床特点。方法回顾分析2010年1-2月于北京儿童医院住院治疗的2例感染洋葱伯克霍尔德菌的X连锁慢性肉芽肿病患儿的临床资料。结果 2例男性患儿,分别为0.5岁和1.7岁,均经CYBB基因突变分析明确诊断为X连锁慢性肉芽肿病。1例经反复血培养、1例经反复尿培养诊断为洋葱伯克霍尔德菌感染。药敏试验结果提示均为敏感菌株。结论洋葱伯克霍尔德菌是人类机会性病原,常表现为慢性肉芽肿病。常规抗生素治疗可根除感染。预防应用复方磺胺药物对此类患儿具有保护意义。

Burkholderia cepacia infections in children with X-linked chronic granulomatous disease

Objective To summarize and analyze clinical features of Burkholderia cepacia infections in two children with X-linked chronic granulomatous disease. M ethods Clinical data of 2 patients with Burkholderia cepacia infections with X-linked chronic granulomatous disease of Beijing children’s hospital from Jan.2010 to Feb.2010. Results Two male children who were 0.5 and 1.7 years old , respectively , were admitted to our hospital , diagnosed with X-linked chronic granulomatous disease through CYBB mutation analysis. Burkholderia cepacia infections were ascertained by recurrent blood and urine cultures. Drugs sensitivity analysis showed that they were sensitive to relevant antibiotics. Conclusion Burkholderia cepacia is opportunistic pathogen for human. Community-acquired infection implies underlying disease , and chronic granulomatous disease is the most common. Routine antibiotic treatment can eradicate its infection. Prophylactic treatment with trimethoprim-sulfamethoxazole can be beneficial to this fragile group of people.