Oikonomou Anastasia, Prassopoulos Panos, Hytiroglou Prodromos, Kirvassilis Fotios V, Tsanakas John N
Department of Radiology, University Hospital of Alexandroupolis, Democritus University of Thrace, Dragana, Alexandroupolis, Greece.
J Thorac Imaging. 2007 May;22(2):176-9. doi: 10.1097/01.rti.0000213565.40667.8f.
A 4-year old boy was admitted to hospital with progressive respiratory failure. A chest roentgenogram revealed hyperinflated lungs and a diffuse reticular pattern. There was no smoking history in the family environment. A thin slice computed tomography of the lungs exhibited extended and diffuse cystic lung disease with no zonal predominance. An open lung biopsy showed pulmonary Langerhans cell histiocytosis. No other organs were involved. Despite treatment initiation the boy succumbed to his pulmonary insufficiency 3 weeks later. Isolated pulmonary Langerhans cell histiocytosis in children may have an unfavorable prognosis and in contrast with adults, it may present on computed tomography with lower lobe predominance and without sparing the lung bases or the anterior parts of middle lobe and lingula.
一名4岁男孩因进行性呼吸衰竭入院。胸部X线片显示肺过度充气和弥漫性网状影。家庭环境中无吸烟史。肺部薄层计算机断层扫描显示广泛弥漫性囊性肺病，无区域优势。开放性肺活检显示为肺朗格汉斯细胞组织细胞增多症。无其他器官受累。尽管开始了治疗，但男孩在3周后死于肺功能不全。儿童孤立性肺朗格汉斯细胞组织细胞增多症可能预后不良，与成人不同，其在计算机断层扫描上可能以下叶为主，不累及肺底部或中叶和舌叶前部。
