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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case is reported of pulmonary lymphangiomyomatosis in 44-year-old woman presenting with progressive exertional dyspnoea, unproductive cough and haemoptysis. The patient showed no symptoms or signs of tuberous sclerosis, a condition often found in association with pulmonary lymphangiomyomatosis. A pulmonary biopsy specimen showed disruption of alveolar septa and fibrosis. Subpleural lymph vessels and pulmonary venules showed cuffs of spindle-shaped cells. The leiomyomatous nature of these cells was evident from their appearance and staining reactions with light microscopy, and the presence of smooth muscle antigens as demonstrated by immunofluorescence technique. Since the lesion is not restricted to lymphatics, but can also be present in blood vessels, the condition may more appropriately be designated as pulmonary angiomyomatosis.
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PMID:Pulmonary lymphangiomyomatosis. Demonstration of smooth muscle antigens by immunofluorescence technique. 32 67

A 14-year-old girl was admitted because of cough, chest pain and hemosputum. Chest roentgenogram on admission showed a pneumothorax and a cavitary lesion with niveau formation in the right lung and cystic lesions in the bilateral lung fields. After bed rest and intravenous administration of antibiotics for two weeks, the right lung inflated well and the niveau formation disappeared, and the patient was discharged. One week later, she was readmitted with sudden-onset severe dyspnea, caused by bilateral pneumothoraces. Emergency tube thoracostomy and wedge resection of the bullous lesion was performed. Macroscopically, multiple small cystic changes were seen on the surface of the right lung. Histological examination revealed nodular proliferations of smooth muscle cells in the interstitium and vessel walls in the lung, which contained slit-like lymphatic channels. The diagnosis of pulmonary lymphangiomyomatosis was made. In this case, we could not measure receptors for estrogen and progesterone. Recently, hormonal therapy and oophorectomy have been reported as being useful. Tamoxifen (Norvadex) was therefore initiated, and the patient has remained well with slight dyspnea on exertion. There has been no recurrence of pneumothorax. Lymphangiomyomatosis is a rare disease of unknown etiology which occurs exclusively in women, mostly in those of reproductive age. We report a 14-year-old female patient with lymphangiomyomatosis associated with repeated pneumothorax, who had been under treatment for epilepsy. We believe this case to be of importance because of the long discussed relation between pulmonary lymphangiomyomatosis and tuberous sclerosis.
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PMID:[A 14-year-old with pulmonary hamartomatous lymphangiomyomatosis associated with bilateral pneumothoraces]. 143 26

Lymphangiomyomatosis was first reported more than forty years ago. Although its incidence is rare, its occurrence is being increasingly recognized and has been the subject of a growing number of cases reports in recent years. This study adds 2 more cases to the file. Both cases involved young women with the characteristic symptoms of dyspnea, cough, abdominal discomfort and swelling, chest pain, and hemoptysis, with abundant formation of refractory chylous, serous ascites. Although the progression of the disease differed in each case, pulmonary function was affected in a similar way by the presence of obstructive and restrictive defects and a decrease in diffusing capacity. Underlying abnormalities were dilated lymphatics, thickened lymphatic walls, and muscular proliferation of leiomyomatous origin, leading to bronchial restriction. The authors point to the subtlety required in arriving at a differentiated diagnosis of lymphangiomyomatosis lymphangioleiomyomatosis and the difference between the two conditions. They also make particular recommendations in respect to the importance of preliminary hormone receptor tests and to the wisdom to be exercised in ligating a main lymphatic duct in the chest to control the formation of ascites. Pneumothorax, a frequent manifestation of lymphangiomyomatosis, is found to be the result of chronic air trapping due to a combination of narrowing of conducting airways and disruption of normal lung parenchyma.
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PMID:Lymphangiomyomatosis--newer concepts in pathogenesis and management--case reports. 224

A 32-year-old primigravida presented with cough and dyspnea at 16 weeks' gestation. Chest roentgenogram revealed a large pleural effusion and diffuse interstitial infiltrates. Moderate arterial hypoxemia and a significant reduction in vital capacity were present. Thoracentesis revealed sterile chyle with no evidence of malignancy. Spontaneous delivery of a healthy infant occurred at 38 weeks, but no change was seen in either the pulmonary infiltrates or chylothorax. Open lung biopsy confirmed the clinical impression of pulmonary lymphangiomyomatosis, and a pleurodesis was performed. Progesterone and estrogen receptor assays on the lung biopsy material revealed only minimal binding. Following two years of therapy with tamoxifen citrate and megestrol acetate, the chylothorax has not recurred, and there has been no other appreciable change in pulmonary function.
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PMID:Pulmonary lymphangiomyomatosis complicating pregnancy. A case report. 362 22

The symptoms of progressive dyspnea, hemoptysis, spontaneous pneumothorax, chylous effusions, and cough in conjunction with ventilatory obstruction and abnormal gas exchange in a young woman should prompt the diagnosis of pulmonary lymphangiomyomatosis. Cytosol steroid-receptor assays and postmortem studies were conducted in an extensive investigation of a case of this disease. A biopsy specimen of the lung disclosed evidence of nuclear translocation of [3H]progesterone and the presence of a cytosolic receptor for progesterone, an indication that this disease could be treated effectively with progestin.
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PMID:Pulmonary lymphangiomyomatosis: with particular reference to steroid-receptor assay studies and pathologic correlation. 669 30

The diagnosis of lymphangioleiomyomatosis was established in a 35-year-old woman with hemoptysis, mild cough, and dyspnea based on histologic review of results of a transbronchial biopsy correlated with high-resolution computed tomographic scan findings. A chest x-ray film revealed diffuse interstitial lung disease, and a high-resolution computed tomographic scan showed diffuse cystic changes throughout both lungs. The transbronchial biopsy specimen revealed cystic changes and a patchy, sometimes nodular proliferation of smooth muscle that focally expanded the interstitium suggestive of lymphangioleiomyomatosis. The smooth-muscle nature of the cells was confirmed by positive immunohistochemical stains for actin and desmin; positive staining for HMB-45 was also observed. Although the diagnosis of lymphangioleiomyomatosis usually requires an open lung biopsy, this case shows that rarely, in the appropriate clinical setting, the diagnosis may be rendered based on results of a transbronchial biopsy in conjunction with findings from ancillary immunohistochemical studies and high-resolution computed tomography.
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PMID:Pulmonary lymphangioleiomyomatosis. Diagnosis based on results of transbronchial biopsy and immunohistochemical studies and correlation with high-resolution computed tomography findings. 806 Feb 40

A 50-year-old woman was admitted with the complaint of cough and dyspnea on exertion for the previous two months. A radiograph of the chest showed a right-sided hydropneumothorax, which was proven to be a chylous effusion by lipoprotein electrophoresis and was very refractory to tetracycline-pleurodesis. The chylopneumothorax was cured by ligation of the thoracic duct and surgical pleurodesis. After an open lung biopsy, lymphangioleiomyomatosis (LAM) was diagnosed histopathologically with smooth muscle nodules scattered throughout the lungs, obstructing the small airways, venules and lymphatics. An immunohistochemistry study using the avidin biotin complex method with monoclonal antibodies for actin and desmin showed the small nodules to be of muscle origin. During the past two years, the patient has remained stable both in respiratory status and roentgenographically without hormonal manipulation or oophorectomy. We present this case to illustrate the heterogeneous nature of this condition. While our patient's initial presentation was acute and associated with chylothorax, her postoperative course has shown no progression despite withholding of hormonal therapy.
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PMID:Early chylopneumothorax in a patient with pulmonary lymphangioleiomyomatosis. 810 84

A 55-year-old white woman with pulmonary lymphangioleiomyomatosis (LAM) presented to the emergency department with odynophagia and subplatysmal emphysema after a paroxysm of coughing. Lateral neck films showed subcutaneous emphysema and a retropharyngeal air stripe. Chest radiographs showed neither pneumothorax nor pneumomediastinum. Patients with LAM frequently develop pulmonary barotrauma and pneumothoracies. This patient, however, had undergone prior bilateral talc pleuradesis as treatment for recurrent pneumothoracies and, thus, could not manifest this complication of barotrauma. This case illustrates the uncommon occurrence of superior dissection of air after pulmonary barotrauma.
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PMID:Pulmonary barotrauma as the cause of pneumoretropharynx in pulmonary lymphangioleiomyomatosis. 863 7

A 49-year-old woman was admitted to our hospital because of coughing and dyspnea. A chest roentogenogram showed emphysematous changes and a diffuse reticular shadow. A high-resolution CT scan of the chest showed many small cysts throughout the lungs. Lymphangiomyomatosis was diagnosed after examination of a specimen obtained by transbronchial biopsy. Abdominal distention due to chylous ascites developed during the hospital stay despite anti-estrogen therapy. Because the ascites was resistant to conservative therapy, we decided to begin peritoneo-venous shunting with a Denver Shunt system. After the operation, the abdominal distention was controlled for 1 year and 11 months, at which time the patient died of respiratory and heart failure with pneumonia. At autopsy, the shunt was patent and functional although about 900 ml of serous ascites fluid was present. An adenocarcinoma was found in the upper lobe of the right lung, but it may not have been related to the lymphangiomyomatosis. Peritoneovenous shunting with a Denver Shunt can be used to treat chylous ascites due to lymphangiomyomatosis when conservative therapy is insufficient.
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PMID:[Lymphangiomyomatosis with chylous ascites treatment successfully by peritoneo-venous shunting]. 875 14

Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious, chronic disease whose etiology is unknown and which affects young women almost exclusively. It produces typical clinical and radiological characteristics. Diagnosis is confirmed on finding histological evidence of irregular proliferation of the smooth muscle cells of the lungs. Treatment is not usually effective and there is rapid worsening of the pulmonary function leading to serious respiratory failure which often results in the patients death. We present two cases of LAM in women, one of whom was 33 and the other 38. In the first case the patient had all the pulmonary findings described in the literature, while the second also had bilateral renal angiomyolipomas. We discuss the histological, radiological and clinical characteristics and evolution of both cases, as well as the treatment given. We also review the literature. It is concluded that LAM should be suspected in young women who have dyspnea, cough and/or hemoptysis and an interstitial radiological pattern, especially when associated with a pleural effusion or pneumothorax.
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PMID:[Pulmonary lymphangioleiomyomatosis. Description and course of 2 cases]. 1132 35


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