目的探讨慢性肉芽肿病患儿肺部感染的临床特点。方法回顾性分析5例以肺部感染为首发症状的慢性肉芽肿病患儿的临床资料,总结其症状、体征、实验室检查结果、影像学表现及治疗转归情况。结果 5例患儿均因发热就诊,病程4~60d,体温37.8~40.1℃,伴轻微咳嗽,无明显感染中毒症状,无发绀、呼吸急促、喘息等症状,3例肺部体征无明显异常,2例有少许中湿性啰音;外周血中性粒细胞计数明显增多;肺部CT表现为多发结节、不规则、球形或类球形高密度影;下呼吸道分泌物细菌、真菌涂片及培养、真菌D-葡聚糖试验均阴性;抗生素治疗无效,抗真菌药物治疗1~2d发热好转,约1个月肺部病灶明显吸收。结论以肺部感染为首发症状的慢性肉芽肿病患儿可有不同程度发热,呼吸道症状及体征较轻,但影像学表现重,肺部CT可见多发结节、不规则、球形或类球形高密度影;真菌病原常见,抗真菌治疗有效。 Objective To investigate the clinical features of pulmonary infection in children with chronic granulomatous disease. Methods The clinical data of 5 children with chronic granulomatous disease with pulmonary infection as the first symptom were retrospectively analyzed. The symptoms, signs, laboratory findings, imaging findings and treatment outcomes were summarized. Results All the 5 cases were treated with fever, the course of disease was 4 ~ 60d, the temperature was 37.8 ~ 40.1 ℃, with mild cough, no obvious symptoms of poisoning, no cyanosis, shortness of breath, wheezing and other symptoms. 2 cases had a slight wet rales; peripheral blood neutrophil count was significantly increased; pulmonary CT showed multiple nodules, irregular, spherical or spherical high-density shadow; lower respiratory secretions bacteria, fungal smear and culture , Fungi D-glucan test were negative; antibiotic treatment is invalid, antifungal therapy 1 ~ 2d fever improved, about 1 month lung lesions were significantly absorbed. Conclusions Children with chronic granulomatous disease with pulmonary infection as the first symptom may have different degrees of fever, mild respiratory symptoms and signs, but have severe imaging findings. Pulmonary CT shows multiple nodules, irregular, spherical or spheroidal Density shadow; fungal pathogen common antifungal therapy effective.