A case report of pulmonary lymphangioleiomyomatosis that was thought of a potential recipient for lungtransplantation.

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A 52-year-old woman was admitted to our hospital on March 5, 1992 suffering from progressive dyspnea. She had experienced pneumothorax eight times since the age of 40. Chest radiographs demonstrated bilateral diffuse reticulonodular shadows, and a chest CT scan revealed multiple bilateral cystic lesions. Respirator function tests indicated both severe obstruction and restriction. Lymphangioleiomyomatosis was confirmed by open lung biopsy.<BR>Following hospitalization, pneumothorax occurred twice and she was treated with chest tube drainage and pleurodesis. Her pulmonary function continued to decline. Becase she was in a terminal stage in spite of oxygen therapy, and because the estrogen and progestogen receptors were negative in the biopsy lung tissue, hormone therapy was considered to be having no effect.<BR>In this case, we concluded that lung transplantation would be the only effective method of treatment. Lymphangioleiomyomatosis is a rare disease, but due to the lack of response to conventional treatment, lung transplantation should be considered as a possible option.
特定非営利活動法人日本呼吸器外科学会の論文