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題名 | Pulmonary Lymphangioleiomyomatosis--A Case Report=肺部淋巴血管肌肉增生症--病例報告 |
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作者 | 辛國輝; 林明杰; 賴信良; 蕭光明; | 書刊名 | 胸腔醫學 |
卷期 | 17:1 2002.03[民91.03] |
頁次 | 頁61-67 |
分類號 | 415.46 |
關鍵詞 | 淋巴血管肌肉增生症; 乳糜胸; Pulmonary lymphangioleiomyomatosis; Chylothorax; |
語文 | 英文(English) |
中文摘要 | 淋巴血管肌肉增生症(LAM)是一個罕見的疾病,原因不明,幾乎全發生於生殖年齡的婦女。此病一向鮮少有中國人的報導,在此我們報告一位年輕女性,呈現左側卵巢囊豻、乳糜腹水、左側腸骨淋巴結腫大、右側乳糜胸及瀰漫性細網狀間質性肺病變。高解析度電腦斷層攝影及左腸骨淋巴切片檢查,確認淋巴血管肌肉增生症的診斷。以全靜脈營養治療、中鏈三酸甘油脂飲食治療、胸管引流術及肋膜黏連術治療,再加上每日賀爾蒙治療(Tamoxifen 20mg & Medroxyprogesterone 15mg),其肺部情況逐漸改善,胸管終於成功移除,胸部影像及肺功能也獲改善。因此,生殖年齡之國人婦女若合併有氣胸及乳糜胸時,LAM也應列入鑑別診斷。 |
英文摘要 | Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of unknown cause that occurs mainly in women of reproductive age. It has seldom been reported among the Chinese population. Herein, we report a young female presenting with a left ovarian cyst, chylous ascites, a left iliac lymphadenopathy, right chylothorax, and diffuse reticular interstitial pulmonary lesions. A high resolution computerized tomography (HRCT) and left iliac lymph node biopsy confirmed the diagnosis of LAM. She then was treated with total parenteral hyperalimentation, diet therapy with medium chain triglycerides (MCT), chest tube drainage with chemical pleurodesis, and hormone therapy with Tamoxifen 20mg and Medroxyprogesterone 15mg daily. Her lung condition gradually improved. The chest tube was successfully removed, and both the radiographic findings and pulmonary function had improved, too. It is therefore concluded that or Chinese women of child-bearing age, LAM should be included in the differential diagnosis of pneumochylothoras. |
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