Okten A, Mocan H, Erduran E, Aslan Y, Gumele H R, Ozoran Y
Department of Pediatrics, Karadeniz Technical University Faculty of Medicine, Trabzon, Turkey.
Turk J Pediatr. 1996 Jan-Mar;38(1):125-30.
Pulmonary involvement of Langerhans cell histiocytosis (LCH) is an uncommon but important cause of pulmonary fibrosis and honeycombing in young adults. Rarely, pulmonary LCH may be complicated by spontaneous pneumothorax. It may be isolated or associated with multiple organ involvement. We describe here a case of LCH with diabetes insipidus, skin lesions and pulmonary involvement in a 15-year-old boy. The case was complicated by four episodes of spontaneous pneumothorax with typical radiologic findings of pulmonary LCH. His presentation, radiologic findings, treatment and clinical course during three-years of follow-up are discussed.
朗格汉斯细胞组织细胞增多症（LCH）的肺部受累是年轻成年人肺纤维化和蜂窝肺的一个不常见但重要的原因。罕见情况下，肺部LCH可能并发自发性气胸。它可能是孤立性的，也可能与多器官受累相关。我们在此描述一名15岁男孩，患有LCH，伴有尿崩症、皮肤病变和肺部受累。该病例并发了4次自发性气胸，具有肺部LCH的典型影像学表现。讨论了他的临床表现、影像学检查结果、治疗方法以及三年随访期间的临床病程。
