• Journal of Chest Surgery
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• v.37 no.12
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• pp.1029-1031
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• 2004

We had expierienced pulmonary lymphangioleiomyomatosis(LAM) with bilateral chylothorax and chylous ascites. A twenty-one-year-old lass with chief complaint of abdominal pain was admitted through the emergency room. She received emergent pelvicoscopic surgery for the rupture of the right corpus luteum. We aspirated 1000ml of the uncoagulated blood. The bleeding point was cautherized electrically. LAM was diagnosed with tissue from the retroperitoneum. Chylous ascites and bilateral chylothorax were occurred despite of various treatments. On thoracotomy, bullous changed lung and lymphatic leakage from visceral and parietal pleura were observed. She died of respiratory insufficiency and general weakness after 6 months from admission.

• Tuberculosis and Respiratory Diseases
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• v.50 no.2
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• pp.245-251
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• 2001

Recently we have experienced one case of pulmonary lymphangioleiomyomatosis(LAM). A 49 year-old woman visited the outpatient department complaining of longstanding dyspnea, which was aggravated by exercise. Although the chest PA film showed nothing more than a slight increase in interstitial marking, a lung HRCT revealed multiple cystic lesions of a similar size that were scattered through out the whole field in both lungs. An abdominal CT detected an angiomyolipoma located in the midbody of the left kidney. Video-assisted thoracic surgery(VATS) was performed for the pathologic diagnosis. On gross examination of the biopsy lung, a pulmonary LAM was confirmed by a finding of smooth muscle proliferation in the interstitum of the lung. After the final diagnosis, oral medroxyprogesterone was prescribed and she is presently in a stable condition.

• Tuberculosis and Respiratory Diseases
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• v.42 no.4
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• pp.624-628
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• 1995

Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. We experienced a 32-year-old female who was admitted because of worsening exertional dyspnea after hormonal therapy with Clomifen for five months and intermittent hemoptysis. Chest PA showed diffuse ground glass appearance with some reticular infiltrations. High resolution CT scan showed multiple small thin walled cysts distributed homogeneously throughout the entire lung. Pulmonary function test showed characteristic obstructive pattern despite the restrictive interstitial pattern of chest radiography. Thoracoscopic lung biopsy specimen showed abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and pulmonary vessels typical of pulmonary lymphangioleiomyomatosis. Hormonal therapy with medroxyprogesterone was initiated.

• Tuberculosis and Respiratory Diseases
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• v.47 no.4
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• pp.533-537
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• 1999

Pulmonary lymphangiomatosis is a very rare pulmonary lesion with an aggressive potential that occurs mainly in newborns, infants and young children of both sexes. It is characterized by pulmonary abnormalities of lymphatic system, showing an increased number of complex anastomosing lymphatic channels in the pleura, in the subpleural interlobular septa, and along the bronchovascular lymphatic route and uniformly fatal. We report a case of lymphangiomatosis behaving like lymphangioleiomyomatosis in a 26-year-old woman.

• Tuberculosis and Respiratory Diseases
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• v.40 no.1
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• pp.79-83
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• 1993

A 38-year-old female was admitted due to progressive exertional dyspnea and intermittent blood tinged sputum. Chest PA showed diffuse reticular infiltration accompanied by hyperinflation. $PaO_2$ was normal in resting state but profoundly decreased during exercise. Spirometry showed severe obstructive pattern but DLCO was markedly decreased. Lung volumes measured by helium equilibrium method was increased. On HRCT, numerous and relatively uniform sized cysts were evenly distributed throughout the bilateral lung fields. Open lung biopsy was performed and pulmonary lymphangioleiomyomatosis(LAM) was diagnosed based on the findings of abnormal proliferation of smooth muscle cells in the walls of lymphatic vessels, bronchioles, and small pulmonary veins. Immunohistochemical staining was negative for estrogen receptor but positive for progesterone receptor. Medroxyprogesterone therapy was initiated.

• Tuberculosis and Respiratory Diseases
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• v.71 no.3
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• pp.163-171
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• 2011

Recently published articles on interstitial lung disease (ILD) have focused on the accurate diagnosis of idiopathic pulmonary fibrosis (IPF), serum biomarkers, acute exacerbation of IPF, the prognostic factors of ILD and the trial of new treatment. In particular, reports on the serum biomarkers such as CC-chemokine ligand 18, surfactant protein, circulating fibrocytes, and acute exacerbation of IPF are sufficient to be mentioned here. Pirfenidone therapy is the most important trial for the treatment of IPF. Other newer treatment trials such as interferon-gamma, sildenafil and imatinib have been reported to be unsuccessful. On the other hand, the sirolimus trial for lymphangioleiomyomatosis is promising. Combined pulmonary fibrosis and emphysema and IgG4-related disease are established to be the new disease entities of ILD.

• Tuberculosis and Respiratory Diseases
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• v.39 no.5
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• pp.433-437
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• 1992

Tuberous sclerosis is a syndrome characterized by the facial skin lesion, epilepsy, and mental retardation. Pulmonary involvement is uncommon, but when the lung is involved by tuberous sclerosis it shows characteristic reticulonodular infiltration and cystic changes. We experienced a 34-year-old female patient of known tuberous sclerosis admitted due to pneumothorax. Chest PA showed diffuse fine reticulonodular infiltration and chest CT revealed diffuse cystic changes of the lung. Pleurodesis with tetracycline was done to prevent recurrence, but chylothorax occurred four months after the treatment. Pleurosis was tried again and anti estrogen treatment began.

• Journal of Chest Surgery
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• v.33 no.1
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• pp.88-95
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• 2000

Pulmonary lymphangioleiomyomatosis is a chronic destruct8ive disease of the lung affecting women of childbearing ages which eventually leads to respiratory failure. Lung transplantation is the only conclusive therapeutic measure because this disease responds poorly to other therapies, To date only a few reports in the literature describes the clinical experience of the bilateral sequential lung transplantation of this rare condition. We performed a bilateral sequential lung transplantation on a 32-year-old woman suffering from lymphangioleiomyo-matosisw. The heart-lung block was harvested from a 51-year-old donor. We transplanted the left lung first through the clam-shell incision. As the hemodynamics deteriorated suddenly during the dissection of the right lung the right lung was transplanted under the cardio-pulmonary bypass. Although the patient's lung function was initially satisfactory the patient died of sepsis and subsequent cardiogenic shock at the postoperative 18th day. Autopsy findings showed infection of Candida albicans on the pericardium and the left lung which had been initiated possibly from the left bronchial anastomosis site,. Through detailed review of the clinical course we concluded that lung transplantation could have been performed safely on this disease provided that early diagnosis and proper management or the oppor-tunistic infection have been carried out.

• Tuberculosis and Respiratory Diseases
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• v.74 no.3
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• pp.97-103
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• 2013

A lung cyst is an air-filled lucent structure surrounded by a thin wall. The presence of multiple intrapulmonary cysts is defined as cystic lung disease. Although cystic lung disease is rare, incidental detection has increased significantly in recent years by screening using computed tomography. There are many conditions that can mimic lung cysts and cause cystic lung disease. Clinical, radiographic, and histologic findings are all necessary for a proper diagnosis, and multidisciplinary approaches are frequently required. The aim of this report is to review the causes and characteristics of cystic lung disease to better understand and improve treatment.

• Tuberculosis and Respiratory Diseases
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• v.49 no.2
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• pp.246-252
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• 2000

Lymphangioleiomyomatosis(LAM) is a rare disease of unknown etiology that occurs mainly in woman in her reproductive age. We recently experienced two cases of bilateral diffuse cystic lesion of the lung on chest X-ray and HRCT. The first case, a 26-year-old female, who had been diagnosed with tuberous sclerosis by the presence of clinical manifestation such as mental retardation, bilateral renal angiomyolipoma, adenoma sebaceum and generalized seizure, was admitted due to a recently developed hemoptysis. Chest PA showed diffuse ground-glass opacity with radiolucent cystic lesions of various sizes on both lung fields. HRCT showed innumerable small cystic lesions with suspicious diffuse ground-glass opacity on both lung fields The second case, a 30-year-old fema1e was admitted due to dyspnea and spewing of blood-tinged sputum for 2 weeks, shortly after delivery. Chest PA showed diffuse reticular and ground-glass opacities on both lung field. HRCT showed multiple well-defined and relatively uniform size air cysts with a uniform wall thickness on entire both lung fields, with small amount of right pleural effusion. By thoracoscopic lung biopsy she was diagnosed with pulmonary lymphangioleiomyomatosis. We report these cases with a brief review of the literatures.