摘要
目的观察原发性免疫缺陷病(primary immunodeficiency disease,PID)合并重症肺孢子菌肺炎(pneumocystis pneumonia,PCP)的临床特点、治疗及预后。方法回顾性分析河南省儿童医院内科重症监护室(PICU)诊断PID合并重症PCP的临床特点、治疗及预后。结果5例PID合并重症PCP患儿,其中高IgM血症3例,联合免疫缺陷病2例。5例患儿均存在发热、咳嗽、呼吸困难和低氧血症表现;确诊PCP前均应用过广谱抗生素;4例曾应用糖皮质激素。5例患儿IgG、IgA均明显降低;3例IgM正常,1例升高,1例降低。4例患儿T淋巴细胞、CD8^(+)、CD4^(+)、NK和B淋巴细胞计数处于正常范围或增高;1例患儿T淋巴细胞、CD8^(+)、CD4^(+)、NK细胞计数均明显降低,B淋巴细胞计数升高。肺部听诊2例未闻及干湿性啰音,3例存在少量湿啰音。肺泡灌洗液宏基因组测序(mNGS)检出肺孢子菌序列数中位数960523(31364~1214369),患儿均合并有其他病原体感染。5例患儿中3例接受甲氧苄啶磺胺甲基异(TMP-SMZ)联合卡泊芬净治治疗,1例单用TMP-SMZ治疗,1例结果回示前转院。经过治疗3例高IgM血症好转出院,余2例严重免疫缺陷患儿死亡。结论PID合并PCP起病至确诊时间较长,多混合其它病原体感染,往往需要机械通气,且机械通气时间大于1周。临床上不能把WBC、CRP、PCT、LDH、G试验、六胺银染色等作为判断有无PID合并PCP的诊断依据。对于常规检测方法不能明确病原时,mNGS可提高PC检出率,协助诊断PCP。是否接受规范抗PC的治疗、免疫缺陷的类型及合并其他病原菌的感染等可能与PID合并PCP的预后相关。
Objective To observe the clinical characteristics,treatment and prognosis of primary immunodeficiency disease(PID)complicated with severe pneumocystis pneumonia(PCP).Methods The clinical characteristics,treatment and prognosis of PID complicated with severe PCP diagnosed by PICU in Henan Children's Hospital were analyzed retrospectively.Results There were 5 cases of PID complicated with severe PCP,including 3 cases of hyper immunoglobulin M sydrome and 2 cases of combined immunodeficiency.All the 5 children presented with fever,cough,dyspnea and hypoxemia.Broad-spectrum antibiotics were used before PCP diagnosis.Glucocorticoid was used in 4 cases.IgG and IgA were significantly decreased in 5 cases.IgM was normal in 3 cases,elevated in 1 case and decreased in 1 case.The number of total T,CD8^(+)T,CD4^(+)T,NK and B cell remain normal or increased in 4 cases.In 1 case,the count of total T,CD8^(+)T,CD4^(+)T,NK cell were decreased significantly,while B cell count was increased.In lung auscultation,2 cases had no rales and 3 cases had a small amount of wet rales.The median sequence number of pneumocystis was 960523(31364-1214369)by metagenomic sequencing(mNGS)of alveolar lavage fluid,and all the children were co-infected with other pathogens.Among the 5 children,3 patients were received TMP-SMZ combined with capofungine,1 patient was received TMP-SMZ alone,and 1 patient was transferred to other hospital before the result returned.After treatment,3 patients with hyper immunoglobulin M syndrome were discharged after improvement,and the 2 children with severe immunodeficiency died.Conclusion PID combined with PCP takes a long time from onset to diagnosis,is mixed with other pathogens,and often requires mechanical ventilation,and the mechanical ventilation time is longer than 1 week.WBC,CRP,PCT,LDH,G test,silver hexacamine staining,etc.can not be used as the diagnostic basis to judge whether PID combined with PCP.When the pathogen cannot be identified by conventional detection methods,mNGS can improve the detection rate of
作者
崔利丹
梅世月
成怡冰
金志鹏
贾鑫磊
CUI Li-dan;MEI Shi-yue;CHENG Yi-bing;JIN Zhi-peng;JIA Xin-lei(Pediatric Intensive Care Unit,Affiliated Childrens Hospital of Zhengzhou University,Henan Children's Hospital,Zhengzhou Children's Hospital,Zhengzhou 450018,China;Pediatric Intensive Care Unit,National Center for Children's Health,Beijing Childrens Hospital,Capital Medical University,Beijing 100045,China)
出处
《医药论坛杂志》
2023年第21期10-15,共6页
Journal of Medical Forum
关键词
肺孢子菌肺炎
原发性免疫缺陷
儿童
Pneumocystis pneumonia
Primary immune deficiency
Children
