摘要
目的 了解肺受累的朗格罕细胞组织细胞增生症（Langerhans cell histiocytosis,LCH）儿童的临床表现及影像学特点.方法 回顾分析首都医科大学附属北京儿童医院呼吸二科近4年来因肺部症状或肺部影像学异常而就诊,并通过活检确诊为肺受累的LCH的14例患儿临床表现和影像学特征.结果 14例中男10例,女4例,男女比2.5∶1;中位发病年龄为1.3岁.所有均伴有多系统受累,最常见的是伴皮肤受累（10例,71％）,其次为肝受累（8例,57％）及骨受累（7例,50％）.最常见的表现为咳嗽伴发热（7例,50％）,呼吸道表现无特异性,3例患儿无呼吸道症状,但肺CT提示广泛肺间质改变.最常见的肺CT表现为结节合并囊泡（6例,43％）,其他表现为仅囊泡无结节（5例,36％）、仅结节无囊泡（1例）、既无结节也无囊泡（2例,14％）.结节可多发可散发,多为小结节,可大小不等,可含有囊腔.囊泡大小不等,形态不均,壁厚不均,可融合,可多发也可散发,可呈蜂窝样.7％患儿合并气胸.结论 肺受累的LCH常伴有皮肤、肝脏等多系统受累表现,易被误诊,仔细查找皮疹对于LCH的诊断有重要作用.高分辨率肺CT的特征性表现[肺间质改变、结节和（或）合并囊泡]有助于诊断.
Objective To improve the recognition of the clinical presentation and radiologic manifestation of children with Langerhans cell histiocytosis （LCH） with pulmonary involvement.Method A retrospective analysis was conducted on children who presented with respiratory symptoms or abnormal lung radiologic findings,and finally diagnosed with LCH in Ward 2 of Divison of Respiratory Diseases,Beijing Children＇s Hospital during the last 4 years.Result Fourteen children （10 boys and 4 girls） were included in this study.Male to female ratio was 2.5 ∶ 1.The median age was 1.3 years.Pulmonary involvements were coexisted with other involved organs in all the patients,such as skin （10 cases,71％）,liver （8 cases,57％）,and bone involvement （7 cases,50％）.The most common symptoms were cough and fever （7cases,50％）.Respiratory symptoms were nonspecific,and 3 children had no respiratory symptom but abnormal findings on lung high-resolution CT （HRCT）.The most common HRCT finding was the coexistence of nodules and cysts （6 cases,43％）.Other findings include cysts only （5 cases,36％）,nodules only （1 case）,and with neither nodule nor cyst （2 cases,14％）.Pneumothorax was found in 7％ of children.Conclusion Pulmonary involvement in children with LCH is easily misdiagnosed,and often coexisted with other involved tissues/organs such as skin and liver.Rash,which is easily missed in physical examination is very important for the diagnosis of LCH.The characteristic findings of lung HRCT （nodules and/or cysts） are helpful for diagnosis.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2014年第12期902-905,共4页
Chinese Journal of Pediatrics
