儿童中性粒细胞功能异常的临床特点研究  

Clinical characteristics of neutrophil dysfunction in children

作　　者：夏敏[1] 郑月 许无恨[1] 肖咏梅[2] 张泓[1] XIA Min;ZHENG Yue;XU Wu-hen;无(Department of Clinical Laboratory,Shanghai Children’s Hospital,Shanghai Jiao Tong University,Shanghai 200040,China;Department of Gastroenterology,Shanghai Children’s Hospital,Shanghai Jiao Tong University,Shanghai 200040,China)

机构地区：[1]上海市儿童医院/上海交通大学附属儿童医院检验科,上海200040 [2]上海市儿童医院/上海交通大学附属儿童医院消化科,上海200040

出　　处：《中国实验诊断学》2021年第6期840-843,共4页Chinese Journal of Laboratory Diagnosis

基　　金：上海市重点临床专科建设项目(shslczdzk06902)。

摘　　要：目的通过对41例中性粒细胞功能测定异常的患儿进行回顾性分析,明确其发病和临床特点、以指导疾病诊断和预后判断。方法 2014年10月-2019年12月,上海市儿童医院应用流式细胞法进行中性粒细胞功能检测,以DHR123标记中性粒细胞设门,PBS为对照,经PMA刺激后,其百分比小于90%或刺激指数小于100定义为异常,每次测试均以正常儿童为对照。结果中性粒细胞功能异常者共41例,其中男28例,女13例,年龄23天-13岁,中位年龄3岁,住院35例,门诊6例;住院科室依次为呼吸科13例、肾脏免疫科6例、消化科8例、PICU 4例、新生儿科3例、血液科1例;门诊共6例:其中免疫门诊4例,儿内科门诊2例,临床表现和特点多为反复肺炎、呼吸道感染,脓毒血症或败血症,以及各组织脓肿、关节炎、中耳炎等,确诊和疑似慢性肉芽肿的仅为7例;所有基因检测仅6例,4例阳性。结论中性粒细胞功能异常患儿分布于各科室,临床几乎均出现反复、严重感染、预后差等特点,与慢性肉芽肿病高度相关,易漏诊,需基因检测进一步明确诊断。Objective To retrospectively analyze 41 pediatric patients with abnormal neutrophil function measurement to clarify their onset and clinical characteristics to guide the diagnosis and prognosis of the disease.Methods From October 2014 to December 2019,Shanghai Children’s Hospital applied neutrophil function testing using flow cytometry.DHR123 was used to label neutrophils,and PBS was used as a control.After PMA stimulation,the percentage and stimulation The index decrease was defined as abnormal,and each test was compared with normal children.Results There were 41 neutrophil dysfunction patients,including 28 males and 13 females,aged 23 d-13 years,with a median age of 3 years,35 hospitalized patients,and 6 outpatient clinics;13 cases of respiratory department,6 cases of kidney immunology department,8 cases of digestive department,4 cases of PICU,3 cases of neonatal department,1 cases of hematology department;a total of 6 cases of outpatient clinics:4 cases of immune clinics,2 cases of pediatric clinics,clinical manifestations and characteristics are mostly recurrent pneumonia,Respiratory infections,sepsis or sepsis,as well as abscesses,arthritis,otitis media,and other tissues,only 7 cases were diagnosed and suspected of chronic granulomatous.All genes tested in only 6 cases,4 cases were positive.Conclusion Children with neutrophil dysfunction are distributed in various specialties,with clinical features such as recurrence,severe infection,and poor prognosis,which are highly related to chronic granulomatous disease and are easy to miss diagnosis.Genetic testing is needed to further confirm the diagnosis.

关 键 词：中性粒细胞功能 流式法 慢性肉芽肿病 基因检测 

分 类 号：R725.5[医药卫生—儿科]