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題名 | Pulmonary Lymphangioleiomyomatosis in a 29-Year-Old Woman with Bilateral Spontaneous Pneumothorax: Case Report=肺部淋巴血管平滑肌增生症以兩側自發性氣胸來表現:病例報告 |
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作者姓名(中文) | 吳自成; 賴永發; 趙東瀛; 謝敏璋; 陳維仁; | 書刊名 | 長庚醫學 |
卷期 | 23:3 2000.03[民89.03] |
頁次 | 頁164-168 |
分類號 | 415.434 |
關鍵詞 | 淋巴血管平滑肌增生症; 兩側自發性氣胸; Lymphangioleiomyomatosis; Bilateral spontaneous pneumothorax; |
語文 | 英文(English) |
英文摘要 | Pulmonary lymphangioleiomyomatosis is a rare, progressive and fatal interstitial lung disease that affects women of child-bearing age. It is characterized by the nonneoplastic proliferation of atypical smooth muscle cells around lymphatics, venules, and bronchioles. Patients with pulmonary lymphangioleiomyomatosis most commonly present with exertional dyspnea and a cough. Pneumothorax is almost always found on the initial chest radiograph. We report a case of a 29-year-old woman who presented with abrupt right chest pain and progressive dyspnea. Physical examination in the emergency department was significantly diminished breath sounds over the right lung, and a chest X-ray revealed a right-sided pneumothorax. She required a chest tube for complete lung re-expansion. On the sixth day of admission, left-sided pneumothorax occurred and another chest tube was inserted. She underwent high-resolution computed tomography of the thorax with the findings of numerous cysts evenly distributed throughout the parenchyma of both lungs. Bilateral thoracotomy with lung biopsies and pleurodesis were performed and the surgical findings confirmed the presence of multiple, small, pink-colored cystic lesions on the surfaces of both lungs. Pathological examination showed the characteristic findings of pulmonary lymphangioleiomyomatosis. |
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