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KMID : 0364019930260020160
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Korean Journal of Thoracic and Cardiovascular Surgery
1993 Volume.26 No. 2 p.160 ~ p.162
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Pulmonary Lymphangiomyomatosis -1Case Report-
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정수상
박병률/이종수/양석숭
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Abstract
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Pulmonary lymphangiomyomatosis is rare but extremely interesting condition caused by progressive widespread smooth muscle proliferation in the perilymphatic regions throughout the lungs.
The patient was a 25-year-old female. She had angiofibromas in the face, and angiomyolipoma in the left kidney which was removed 5 years earlier. Three years ago she started having severe dyspnea with bilateral pneumothoraces. Treatment was
initiated
with bilateral closed thoracotomies followed by open thoracotomy through median sternotomy and lung biopsy, which revealed the diagnosis of pulmonary lymphangiomyomatosis. Recurrence of pneumothorax was treated by repeated chemical pleurodesis
with
tetracycline. She has been in good condition during medroxyprogesterone administration for 3 years. (Korean J Thoracic Cardiovas Surg 1994; 27:160-3)
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KEYWORD
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