摘要
目的了解新生儿慢性肉芽肿病的临床特点以提高对此病的认识,做到对此疾病的早诊断、早治疗而进一步提高患儿的长期预后。方法回顾性分析新生儿重症监护室确诊为新生儿慢性肉芽肿病4例患儿的相关临床资料,并进行文献复习。结果4例患儿均为男性,平均发病年龄为(17±2)d,患儿均足月顺产或剖宫产娩出,出生体重>3.5 kg,产前无特殊病史,羊水、胎盘、脐带均正常。生后均给予接种卡介苗。局部卡瘢未见有化脓。否认既往反复感染病史。首发症状均为持续高热、咳嗽,呼吸困难,颜面部散在脓疱疹,治疗过程中患儿呼吸困难呈进行性加重。影像学检查提示肺内大小不等结节影,病变广泛而严重。实验室检查示:患儿血常规中白细胞计数及中性粒细胞比例明显升高,C反应蛋白、降钙素原升高。抗生素(美罗培南联合万古霉素)治疗5~7 d后患儿体温,咳嗽有所好转,但血常规及皮肤破溃均未见明显好转,复查肺部CT仍提示两肺机遇性感染存在,较前无明显改变。结合患儿实验室检查及临床表现考虑患儿可能存在免疫性疾病。立即完善呼吸爆发试验示:中性粒细胞占全部有核细胞50%左右,未见明显活化表现。同时送检患儿基因检查,结合患儿基因检测结果确诊为新生儿慢性肉芽肿病。结论新生儿持续发热、咳嗽、呼吸困难,病情进行性加重,同时结合影像学检查提示金葡菌感染或机遇性感染。根据患儿临床表现、实验室检查、影像学检查,考虑新生儿慢性肉芽肿病的可能,须及时完善呼吸爆发实验及基因检测。
Objective To understand the clinical characteristics of neonatal chronic granulomatosis,thus to improve the understanding,early diagnosis and treatment of this disease.Methods Clinical data of 4 cases of neonatal chronic granulomatosis diagnosed in neonatal intensive care unit(NICU)of our hospital(Zhengzhou Children's Hospital)from January 2016 to January 2017 were retrospectively analyzed,and literature review was also conducted.Results All the 4 cases were male,the average age of onset was 17±2 d.All the infantswere spontaneously delivered or with cesarean section.The birth weight was more than 3.5 kg.No special prenatal history was reported,and the amniotic fluid,placenta and umbilical cord were normal.All were inoculated with BCG after birth,andwith no infection.Previous history of recurrent infections was denied.The first symptoms were persistent high fever,cough,dyspnea,facial scattered pustular rash,dyspnea was progressive aggravation in the treatment process.The imaging examination indicated that the pulmonary nodules were of different sizes and the lesions were extensive and severe.Laboratory examination showed that the white blood cell(WBC)count and neutrophilic granulocyte proportion in the blood routine examination were significantly increased,so were thec-reactive protein and procalcitonin.After 5 to 7 days treatment ofantibiotics(meropenem combined with vancomycin),the patients'temperature and cough were improved,but no significant improvement was observed in blood routine test orskin ulceration.Pulmonary CT reexamination still indicated the presence of opportunistic infection atboth lungs,which was not significantly changed.The possibility of immunological diseases was considered in combination with laboratory examination and clinical manifestations.The immediate improvement of respiratory burst test showed that neutrophils accounted for about 50%of all nucleated cells,and no significant living cultural expression was observed.At the same time,the children were sent for genetic examination,which w
作者
周炎娟
康文清
张耀东
刘大鹏
ZHOU Yan-juan;KANG Wen-qing;ZHANG Yao-dong;LIU Da-peng(Newborn Intensive Care Unit of Children's Hospital Affiliated to Zhengzhou University,Henan Children's Hospital,Zhengzhou Children's Hospital,Zhengzhou 450018,Henan,China)
出处
《广东医学》
CAS
2020年第10期1041-1046,共6页
Guangdong Medical Journal
关键词
新生儿
慢性肉芽肿
呼吸爆发实验
基因
neonatal
chronic granuloma
respiratory outbreak test
gene
