CAJ | 학술논문

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.
목적 료해폐수루적랑격한세포조직세포증생증(Langerhans cell histiocytosis,LCH)인동적림상표현급영상학특점.방법 회고분석수도의과대학부속북경인동의원호흡이과근4년래인폐부증상혹폐부영상학이상이취진,병통과활검학진위폐수루적LCH적14례환인림상표현화영상학특정.결과 14례중남10례,녀4례,남녀비2.5∶1;중위발병년령위1.3세.소유균반유다계통수루,최상견적시반피부수루(10례,71％),기차위간수루(8례,57％)급골수루(7례,50％).최상견적표현위해수반발열(7례,50％),호흡도표현무특이성,3례환인무호흡도증상,단폐CT제시엄범폐간질개변.최상견적폐CT표현위결절합병낭포(6례,43％),기타표현위부낭포무결절(5례,36％)、부결절무낭포(1례)、기무결절야무낭포(2례,14％).결절가다발가산발,다위소결절,가대소불등,가함유낭강.낭포대소불등,형태불균,벽후불균,가융합,가다발야가산발,가정봉와양.7％환인합병기흉.결론 폐수루적LCH상반유피부、간장등다계통수루표현,역피오진,자세사조피진대우LCH적진단유중요작용.고분변솔폐CT적특정성표현[폐간질개변、결절화(혹)합병낭포]유조우진단.
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.