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Query: UMLS:C0019079 (hemoptysis)
 6,129 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

The authors report on 5 cases of diffuse pulmonary lymphangiomyomatosis. This disease occurs in women during the period of genital activity and usually presents with pulmonary signs (9/10) : dyspnoea, pneumothorax, pleural effusion, and hemoptysis. It is also found in association with renal angiomyolipomas and uterine fibromyolipomas. X-ray films of the lungs show : an interstitial syndrome, gas or fluid pleural effusions, and thoracic distention. None of these signs is constant, but the association of an interstitial syndrome with thoracic distention in a young woman appears to be specific for this affection.
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PMID:[Radiological aspects of diffuse pulmonary lymphangiomyomatosis (author's transl)]. 72 60

Anatomic and clinical observations of 28 cases, including 23 previously unpublished, of pulmonary lymphangiomyomatosis are recorded and discussed. This brings the total reported to 57. All patients were women in the reproductive age group with the major complaint of breathlessness. This was usually progressive, and death from pulmonary insufficiency resulted within 10 years. Functional changes were obstructive or restrictive, or both. Pneumothorax, chylous effusions and hemoptysis were frequent complications. Radiographically the lesions initially appear as fine, linear and nodular, predominantly basal densities, and progress to a pattern of bullous change, or honeycombing, involving all portions of the lungs not sparing the region of the costophrenic sinuses as is typical of eosinophilic granuloma. There may be associated pleural effusions. A progressively increasing lung volume is characteristic. The lesions consist of an irregular, nodular or laminar "irrational" proliferation of smooth muscle within all portions of the lung, with loss of parenchyma leading to honeycombing. Proliferated muscle can obstruct bronchioles (with air trapping and formation of bullae often complicated by pneumothorax), venules (with pulmonary hemorrhage and hemosiderosis accompanied clinically by hemoptysis) and lymphatics (with chylothorax or chyloperitoneum). Both thoracic and abdominal lymph nodes and the thoracic duct can also be involved in the myoproliferative process with formation of subsidiary minute channels and obstruction. Renal or perirenal angiomyolipomas can also occur, as exemplified by 2 patients in the present series. Identical pulmonary lesions occasionally occur in tuberous sclerosis. Especially since these patients usually have no neurologic disturbances and are almost women, the possibility of a relationship between tuberous sclerosis and lymphangiomyomatosis must be considered. One feature of note in pulmonary lesions of tuberous sclerosis is the presence of adenomatoid proliferations of epithelium. Such changes were also observed in 2 patients of the present series, and it is remarkable that both of these women had "retarded"children. At present the question of whether by lymphangiomyomatosis is a forme fruste of tuberous sclerosis must be considered as unresolved. It may yield to further investigation, possibility including chromosomal studies.
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PMID:Pulmonary lymphangiomyomatosis. A review. 114 65

Lymphangioleiomyomatosis is a rare devastating disease affecting women mostly of child-bearing age. It presents with spontaneous pneumothorax, chylous effusions, hemoptysis and progressive breathlessness. Most patients die from respiratory failure within 10 years. There are no controlled studies on the efficacy of various treatment regimens. We report our experience with progesterone therapy in three patients. Two failed to respond, one died about 11 years after presentation and another after 5 1/2 years. The third patient has survived 11 years after onset of disease.
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PMID:Lymphangioleiomyomatosis--treatment with progesterone. 177 7

Pulmonary lymphangiomyomatosis (PLM) is an idiopathic disease of females in fertile age. It results in respiratory failure characterized by obliteration of the small airways, emphysema, formation of bullae, hemoptysis, pneumothorax, pulmonary fibrosis, severe hypoxemia and reduced carbon monoxide diffusion capacity. The major lymphatic ducts are also involved, resulting in chylous pleural effusion and ascites. Pulmonary abnormalities improve objectively and subjectively after surgical therapy, which consists in bilateral oophorectomy. Its evolution results in death in no more than 10 years. We report the anesthetic approach to 2 patients with PLM. Patient 1 was a female who had already been diagnosed of PLM and had received medical and surgical therapy, requiring reoperation for the resection of cystic intestinal masses and abdominal eventration. Patient 2 had been scheduled for bilateral oophorectomy after a diagnosis of PLM. We discuss the clinical condition of both patients, the course of the disease, the previous treatments and the anesthetic technique in each case.
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PMID:[Anesthetic approach in 2 patients with pulmonary lymphangiomyomatosis]. 187 32

Lymphangioleiomyomatosis (LAM) is an uncommon disease affecting women of reproductive capacity. It is characterized by non-neoplastic proliferation of smooth muscle in the lungs and, occasionally, in lymph nodes and in the thoracic duct. The patients present with dyspnea, chylous pleural effusion, pnx, and hemoptysis. The authors evaluated 4 patients with biopsy-proven LAM, by means of CT. In all cases, CT revealed small well-defined cystic air spaces with smooth and thin walls, scattered in the lungs. CT was very useful because it was more sensitive and specific than conventional radiography. The CT appearance of LAM distinctly differed from that of other diseases than can produce cystic air spaces in the lungs, such as UIP, eosinophilic granuloma, centri-acinar emphysema, and bronchiectasis. Our experience confirms the clinical usefulness of CT in diagnosing LAM.
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PMID:[Pulmonary lymphangioleiomyomatosis. Radiologic aspects and the diagnostic role of computed tomography]. 223 87

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

Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. The patients present with progressive shortness of breath, pneumothorax, chylous effusion, and hemoptysis. Four patients with biopsy-proved lymphangioleiomyomatosis of the lung were evaluated using high-resolution CT. In all patients, the scan showed well-defined cystic air spaces, surrounded by uniformly thin walls, distributed diffusely throughout both lungs. The cystic air spaces ranged in size from a few millimeters to 5 cm. Pathologically, these cysts were predominantly bounded by normal-looking parenchymal components, with occasional patchy involvement by a smooth-muscle proliferative process. The CT appearance of lymphangioleiomyomatosis differs quite distinctly from that of other diseases that can cause cystic air spaces, such as fibrosing alveolitis, neurofibromatosis, and bronchiectasis, and less distinctly from pulmonary emphysema and eosinophilic granuloma. Our experience in these few cases suggests that the high-resolution CT findings in lymphangioleiomyomatosis are characteristic of the disease.
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PMID:Pulmonary lymphangioleiomyomatosis: high-resolution CT findings in four cases. 270 53

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

Lymphangioleiomyomatosis (LAM) is a rare disease, which only affects women of childbearing age. A case of LAM in a 36-year-old woman is presented. Patho-anatomical findings are smooth muscle hyperplasia leading to obstruction of lymphatics (possibly resulting in chylothorax), blood vessels (possibly resulting in haemoptysis) and bronchi (possibly resulting in emphysema and pneumothorax). High resolution computed tomography (HRCT) of the chest can show the typical multiple small cysts and bullous emphysema distributed diffusely throughout both lungs together with interstitial fibrosis. Treatment of the disease aims at an inhibition of the presumed growth-promoting effect of oestrogen on the smooth muscle cells. However, progression is often seen in spite of treatment.
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PMID:[Lymphangioleiomyomatosis]. 784 79


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