書誌事項
- タイトル別名
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- A case of Recklinghausen's disease associated with multiple emphysematous bullae.
抄録
A 46 year-old male was admitted because of recurrent right spontaneous pneumothorax after an interval of 8 years. He had multiple cutaneous neurofibromas and cafe-au-lait spots, and a diagnosis of Recklinghausen's disease was made. Chest X-ray film showed collapse of the right lung. Contrast thoracography revealed some thumb sized cysts in the lung apex, and cobblestone appearance on the lung surface. At thoracoscopy numerous cysts were seen all over the lung. Open lung biopsy and resection of large cysts was performed. Microscopically the cysts were bullae and there were emphysematous changes arround them, therefore the pathological diagnosis was emphysematous bullae. Six months after operation, chest CT revealed multiple peripheral lung cysts bilaterally, but respiratory function tests showed no abnormality.<br>Although Recklinghausen's disease associated with multiple lung cysts is rare in Japan, some cases were reported in international journals. Many of them had interstitial pneumonitis and it has been considered that alveoli were destroyed by fibrosis and accumulated to form bullae. However in our case, emphysematous changes with neither interstitial pneumonitis nor airway obstruction were seen. Therefore we consider that these findings are very interesting and may have some relation to Recklinghausen's disease.
収録刊行物
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- 日本胸部疾患学会雑誌
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日本胸部疾患学会雑誌 23 (7), 819-823, 1985
社団法人 日本呼吸器学会