• Tuberculosis and Respiratory Diseases
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• v.44 no.5
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• pp.1184-1193
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• 1997

Lymphangioleiomyomatosis(LAM) is rare and essentially limited to women in the reproductive ages. A 39-year-old female was admitted due to progressive exertional dyspnea and intermittent productive cough. Chest PA showed marked hyperinflation of the lung associated with a diffuse reticulo-nodular pattern. High resolution CT scan of the thorax demonstrated that diffusely scattered thin-walled cysts were distributed throughout the bilateral lung fields. Abdominal CT scan showed variable sized multiple angiomyolipoma of both kidney. By open lung biopsy, she was diagnosed as pulmonary LAM associated with Tuberous sclerosis and renal Angiomyolipoma. We present the case and discuss the connection between pulmonary LAM and Tuberous sclerosis.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.542-547
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• 1992

Tuberous sclerosis is an autosomal dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceum. Associated lesions include retinal phacomata, shagreen patches, subungal fibromata, and benign visceral tumors such as pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis occurs exclusively in women, usually during the child-bearing years, and is characterized by proliferation of smooth muscle along the lymphatic vessels of the lung, thorax, abdomen. Proliferation of smooth muscle results in interstitial and obstructive lung disease, recurrent pneumothorax, and chylous pleural effusions. We saw two cases of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in women of reproductive age. We report the cases with a brief review of the literatures.

• Tuberculosis and Respiratory Diseases
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• v.42 no.2
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• pp.244-249
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• 1995

We experienced one case of pulmonary lymphangioleiomyomatosis in 26-year-old female patient. She had taken antituberculous medication under the impression of miliary tuberculosis on simple chest X-ray at peripartum period. On outpatient follow-up she complained of progressive exertional dyspnea in spite of medication. Through careful history taking and physical examination, high resolutional CT, and open lung biopsy she was diagnosed as pulmonary lymphangioleiomyomatosis combined with incomplete type of tuberous sclerosis. So, we presented the case with the brief review the literatures.

• Tuberculosis and Respiratory Diseases
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• v.42 no.3
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• pp.375-380
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• 1995

The lymphangioleiomyomatosis(LAM) is a rare disorder, which afflicts mainly young woman of childbearing age, characterized by proliferation of immature smooth muscle cell in the lymphatics. We experienced a case of LAM in 26-years-old pregnant woman, confirmed pathologically by inguinal lymph node biopsy. She has suffered from exertonal dyspnea and dry coughing. The symptoms and chest X-ray were aggravated with pregnancy, but improved after delivery with two times of pregnancy. The chest X-ray showed diffuse reticulonodular infiltration and chest HRCT showed diffuse scattered tiny thin-walled cyst of lung parenchyma. We noted chylous ascites of which triglyceride level is 396 mg/dl. After delivery, the symptoms were getting better. We treated with medroxyprogesterone and planned close observation and follow-up.