• Journal of Chest Surgery
• /
• 제37권12호
• /
• pp.1029-1031
• /
• 2004

림프관평활근종증은 드문 질환이며, 임상경과상 악성으로 분류할 수 있다. 복통을 주소로 입원한 21세 여자 환자가 골반 내시경을 통해 난소 황체 출혈을 지혈하였고, 그 후 유미성 복수로 개복하여 후 복막에 위치한 낭성 종양을 절제하여 림프관평활근종증으로 조직 진단 받았다. 홀몬 치료 중 유미흉이 발생하여 흉부외과에서 개흉하여 림프액 누출부위를 봉합결찰하였고, 10% 포타딘 관주로 유미흉은 호전되었으나 유미성 복수가 재발하였으며 진단 6개월에 전신 쇠약과 호흡 부전으로 사망하였다.

• Tuberculosis and Respiratory Diseases
• /
• 제50권2호
• /
• pp.245-251
• /
• 2001

저자들은 신장의 혈관근지방종을 동반한 폐임파관평활근종증 1예를 경험하였기에 폐임파관평활근종증에 대한 문헌고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
• /
• 제42권4호
• /
• pp.624-628
• /
• 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
• /
• 제47권4호
• /
• pp.533-537
• /
• 1999

저자들은 수년동안 반복적으로 유미흉이 재발하였던 31세 여자환자에서 환자의 연령, 임상 양상 등을 고려하여 볼 때 LAM이 의심되었으나, 흉부전산화단층 촬영상 흉막비후와 흉막하 소엽간중격의 비대 소견을 보이고, 낭성병변은 보이지 않으며, 조직검사상 폐실질의 림프혈관들의 확장성 변화를 동반한 증식을 관찰하였으나 평활근의 증식은 동반되지 않았으며 면역조직화학 염색 검사상 HMB-45와 에스트로젠수용체검사에서 음성을 보여 폐림프관종증으로 진단된 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
• /
• 제40권1호
• /
• pp.79-83
• /
• 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
• /
• 제71권3호
• /
• pp.163-171
• /
• 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
• /
• 제39권5호
• /
• pp.433-437
• /
• 1992

저자들은 결절성 경화증으로 추적 관찰중인 환자에서 기흉과 유미흉이 발생하고 그 고해상도 전산화 단층촬영의 소견이 알려진 병리적 소견에 부합되는 1예를 경험하였기에 이를 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제33권1호
• /
• pp.88-95
• /
• 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
• /
• 제74권3호
• /
• pp.97-103
• /
• 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
• /
• 제49권2호
• /
• pp.246-252
• /
• 2000

저자들은 양측성 신혈관근지방종, 경련, 지능저하 및 피지선종을 동반한 결절성 경화증으로 진단된 26세 여자 환자에서 객혈을 주증상으로 하여 흉부 X-선 및 고해상도 흉부전산화단층 촬영상 폐림프관평활근종증이 의심되었던 1예와 분만후 호흡곤란과 객혈을 주소로 내원한 30세 여자 환자에서 흉부 X-선 촬영, 고해상도 흉부전산화단층 촬영 및 폐생검을 통해 확진한 폐임파관 평활근종증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.