title: [Clinical characteristics of children with Langerhans cell histiocytosis with pulmonary involvement].
abstract: OBJECTIVE
  To improve the recognition of the clinical presentation and radiologic manifestation of children with Langerhans cell histiocytosis (LCH) with pulmonary involvement.
  
  
  METHODS
  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.
  
  
  RESULTS
  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 (7 cases, 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Ward 2 of Division of Respiratory Diseases, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
authors.forename: Xiaolei
authors.initials: X
authors.lastname: Tang
authors.affiliation: Ward 2 of Division of Respiratory Diseases, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Wang
authors.affiliation: Ward 2 of Division of Respiratory Diseases, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
authors.forename: Jinrong
authors.initials: J
authors.lastname: Liu
authors.affiliation: Ward 2 of Division of Respiratory Diseases, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
authors.forename: Haiming
authors.initials: H
authors.lastname: Yang
authors.affiliation: Ward 2 of Division of Respiratory Diseases, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China.
authors.forename: Shunying
authors.initials: S
authors.lastname: Zhao
authors.affiliation: Ward 2 of Division of Respiratory Diseases, Beijing Children's Hospital Affiliated to Capital Medical University, Beijing 100045, China. Email: lihuimindoc@163.com.
authors.forename: Huimin
authors.initials: H
authors.lastname: Li
mesh_terms: D002648:Child; D003371:Cough / Q000209:etiology; D003560:Cysts; D003951:Diagnostic Errors; D005076:Exanthema / Q000209:etiology; D005260:Female; D005334:Fever / Q000209:etiology; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000000981:diagnostic imaging*; D006801:Humans; D007223:Infant; D008099:Liver; D008171:Lung Diseases / Q000000981:diagnostic imaging*; D008297:Male; D012189:Retrospective Studies; D012867:Skin; D014057:Tomography, X-Ray Computed