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| 題 名 | Pulmonary Langerhans Cell Granulomatosis (Histiocytosis X) Associated with Bilateral Spontaneous Pneumothorax--A Case Report=肺組織細胞症X合併雙側自發性氣胸--一病例報告 |
|---|---|
| 作 者 | 王玠仁; 林長怡; 彭明仁; 陳培然; 許清寅; 劉洪彰; 曾岐元; | 書刊名 | 胸腔醫學 |
| 卷 期 | 14:2 1999.06[民88.06] |
| 頁 次 | 頁75-81 |
| 分類號 | 415.434 |
| 關鍵詞 | 組織氣胸症X; 蘭格漢氏細胞肉芽增生症; 氣胸; Histiocytosis X; Pulmonary; PLCG; Langerhans cell grandomatosis; LCH; Penumothorax; S-100; Birbeck granule; |
| 語 文 | 英文(English) |
| 中文摘要 | 肺組織細胞症X是一種少見,與抽菸有關的間質性肺部疾患,它通常 見於年輕成人,並可合併其他器官系統的侵犯。我們在此報告一18歲男性病患: 他有3年的抽煙史,以氣促,咳嗽為初始症狀。其胸部X光攝影呈現雙側性氣胸, 瀰漫性網狀結節病灶及多發性囊狀空腔。而經開胸生檢術後發現,病灶在蘇木素 及伊紅染色下可見到許多蘭革漢氏細胞,其S-100染色反應亦為陽性。在電子顯 微鏡下,我們也見到典型的細胞內“Birbook"顆粒,診斷因而確立。此患並無皮 病灶及尿崩症狀,其它器官系統亦無明顯侵犯。肺功能檢查則呈重度跼限性通氣 障礙及肺泡瀰漫容積低下。雖經戒菸嘗試,類固醇投予及肋膜癒著術治療,此病 人之氣胸在發後二個月仍未痊癒。 |
| 英文摘要 | Pulmonary Langerhans cell granulomatosis (PLCG) is an uncommon, smoking-related interstitial lung disease that usually attacks young adult, with or without other systems involvement. We reported a case of an 18 years old male, with a 3 years cigarette smoking history, who presented with the complaints of shortness of breath, nonproductive cough, associated with radiologic findings of bilateral pneumothorax, reticulonodular infiltration, and multiple thin wall cysts. Open lung biopsy was performed and revealed the presence of pulmonary Langerhans' cell granulomatosis (PLCG) that was ascertained by the findings of typical Langerhans' cell on H&E stain , positive S-100 stain and intracytoplasmic "Birbeck granule" on electron microscopy. There were no skin lesions, diabetes insipidus symptoms present. Pulmonary function test revealed a severe restrictive defect and impairment of diffusiog capacity without an obstructive defect. Despite smoking cessation, systemic steroid therapy, and surgical and chemical pleurodesis, his lungs remained un- expanded after 2 months of hospitalization. |
本系統中英文摘要資訊取自各篇刊載內容。