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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。
Objective To summarize the clinical features of children with Burkholderia cepacia infection of X-linked chronic granulomatosis. Methods The clinical data of 2 children with Burkholderia cepacia infection who were hospitalized in Beijing Children’s Hospital from January to February in 2010 were retrospectively analyzed. Results Two male patients aged 0.5 and 1.7 years, respectively, were diagnosed as X-linked chronic granulomatosis by CYBB mutation analysis. One case was repeatedly cultured in blood and one case was diagnosed as Burkholderia cepacia by repeated urine culture. Susceptibility test results suggest that are sensitive strains. Conclusion Burkholderia cepacia is a human opportunistic pathogen, often presenting with chronic granulomatous disease. Routine antibiotic treatment eradicates infection. Prevention of compound sulfonamides have protective significance for such children.