| 包玉玲,郦琳琳,田曼,等.慢性肉芽肿病5例临床分析[J].安徽医药,2018,22(9):1790-1792. |
| 慢性肉芽肿病5例临床分析 |
| Clinical analysis of 5 cases of chronic granulomatous disease |
| 投稿时间：2017-07-08 |
| DOI： |
| 中文关键词: 慢性肉芽肿病 儿童 |
| 英文关键词: Chronic granulomatous disease Child |
| 基金项目: |
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| 摘要点击次数: 2008 |
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| 中文摘要: |
| 目的 探讨慢性肉芽肿病(CGD)的临床表现、诊断及治疗。方法 对于2009年1月至2016年10月在南京医科大学附属儿童医院诊断的5例CGD患儿临床表现、实验室检查、治疗及预后进行总结分析。 结果 起病年龄16 d至3.3岁。CGD患儿起病年龄早,可表现为反复下呼吸道感染、淋巴结炎及消化系统疾病、皮肤感染等,易出现分枝杆菌、真菌、金黄色葡萄球菌、洋葱伯克霍尔德菌等病原感染。4例直接经基因确诊,1例中性粒细胞呼吸暴发试验阳性,后续基因检查再次证实。CGD患儿需长期应用抗生素和抗真菌药物预防细菌和真菌感染。 结论 对于自幼反复出现严重肺部、淋巴结、肝脾、骨骼和皮肤细菌、真菌感染,有肉芽肿形成,接种卡介苗后出现感染或怀疑结核而抗结核治疗效果欠佳者,均应考虑到CGD,基因序列测定可以从分子水平明确诊断。CGD的常规治疗包括感染的预防、治疗及并发症治疗。 |
| 英文摘要: |
| Objective To understand the clinical manifestations,diagnosis and treatment of chronic granulomatous disease (CGD). Methods The clinical manifestations,laboratory tests,treatment and prognosis of 5 children with CGD in Childrens Hospital of Nanjing Medical University from January 2009 to October 2016 were analyzed. Results The onset age range from 16 days to 3.3 years.Children with CGD have an early onset ages.The major clinical presentations were characterized by recurrent lower respiratory tract infections,lymphadenitis and digestive system diseases,skin infections,etc.,and prone to be infected by mycobacteria,fungi,staphylococcus aureus,and burkholderia cepacia.4 cases were directly confirmed by gene,1 case of neutrophil respiratory burst test was positive,follow-up gene examination was reconfirmed.The children with CGD need long-term use of antibiotics and antifungal agents to prevent bacterial and fungal infections. Conclusions CGD should be considered in children who have repeated serious bacteria or fungal infections of lung,lymph nodes,liver and spleen,bones,skin,granuloma formation,have BCG infection after BCG vaccination,patients who are suspected to have tuberculosis infection but the response to treatment is not good.Gene sequences can be clearly diagnosed at the molecular level.Routine treatment of CGD includes prevention and treatment of infections and treatment of complications. |
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