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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Anatomic and clinical observations of 28 cases, including 23 previously unpublished, of pulmonary lymphangiomyomatosis are recorded and discussed.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 (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

A case of pulmonary lymphangiomyomatosis in a 28-year-old woman with dyspnea, hemoptysis, chylothorax and irregular menstruation as presenting symptoms was reported. Chest CT scan showed the presence of pleural effusion on right side and a honeycomb pattern throughout the lung parenchyma bilaterally. The diagnosis was confirmed histologically by transbronchial lung biopsy. Medroxyprogesterone was administered; improvement in arterial blood gas analysis and reduction of the amount of pleural effusion were noted after one month. The etiology, clinical manifestations, pathological characteristics and treatment of this disease were discussed.
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PMID:[Pulmonary lymphangiomyomatosis: report of a case]. 826 59

A review is presented of the clinical and morphological manifestations of lymphangioleiomyomatosis (LAM), a systemic disorder of unknown etiology that affects women. The clinical features include dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, and chylous ascites. It is characterized by: 1) proliferation of abnormal smooth muscle cells (LAM cells) in pulmonary interstitium and along the axial lymphatics of the thorax and abdomen; 2) thin-walled pulmonary cysts, and 3) a high incidence of angiomyolipomas. The pulmonary cystic lesions have a characteristic appearance on high resolution computed tomography. The most specific method for diagnosing LAM is lung biopsy to demonstrate the presence of LAM cells, either by their characteristic histological appearance or by specific immunostaining with HMB-45 antibody. LAM cells differ in several important respects from the types of smooth muscle cells normally present in lung. Their reactivity with HMB-45 antibody is localized in stage I and stage II melanosomes. LAM cells show additional evidence of incomplete melanogenesis, and the significance of these observations remains to be determined. Two types of LAM cells are recognized: 1) small, spindle-shaped cells that are centrally located in the LAM nodules and are highly immunoreactive for matrix metalloproteinase-2 (MMP-2), its activating enzyme (MT-1-MMP), and proliferating cell nuclear antigen (PCNA), and 2) large, epithelioid cells that are distributed along the periphery of the nodules and show a high degree of immunoreactivity with HMB-45 antibody and with antibodies against estrogen and progesterone receptors. Types of treatment used for LAM include oophorectomy, administration of Lupron or progesterone and in very severe cases, pulmonary transplantation (following the onset of respiratory insufficiency, not relieved by O(2)).
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PMID:Lymphangioleiomyomatosis (LAM): a review of clinical and morphological features. 1103 60

Lymphangioleiomyomatosis is a rare and complicated disorder that affects the young, almost exclusively women. It may be associated with the tuberous sclerosis complex, which includes renal angiolipoma, chylothorax and lymph node myomatosis. Its clinical pulmonary manifestations vary from simple cough to the development of recurrent pneumothoraces, hemoptysis, and even complicated pleural effusions. Progressive dyspnea develops as the disease evolves. Eventually most patients require lung transplantation. This wide array of symptoms and signs makes the differential diagnosis extensive, and the clinician must be familiar with this disorder to arrive promptly to the correct diagnosis. We report a case of a 36-year-old woman with a long history of recurrent pleuritic chest pain with associated dyspnea before being diagnosed with lymphangioleiomyomatosis. A review of the literature pertinent to this case is provided.
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PMID:Lymphangioleiomyomatosis: an unusual cause of dyspnea in young women. 1201 58

The need and outcome of surgical intervention in patients with pulmonary tuberculosis were assessed retrospectively. Between 1993 and 2003, 72 major surgical procedures were performed in 57 patients with pulmonary tuberculosis. There were 44 males and 13 females with a mean age of 34 years. Indications for surgery were: trapped lung in 18 (31.6%), multidrug-resistant tuberculosis in 10 (17.5%), aspergilloma in 10 (17.5%), destroyed lung in 5 (8.8%), massive hemoptysis in 4 (7%), bronchopleural fistula in 3 (5.3%), persistent cavity in 2 (3.5%), and undiagnosed nodule in 5 (8.8%) patients. The most common procedure was lobectomy (31.9%). Other procedures included decortication, wedge resection, pneumonectomy, segmentectomy, and myoplasty. There were 28 complications in 18 patients, including prolonged air leak in 12 (21.1%), residual space in 7 (12.3%), empyema in 5 (8.8%), hematoma in 2 (3.5%), chylothorax and bronchopleural fistula in 1 (1.8%) each. There was no operative death, but one patient died from sepsis late in the follow-up period (mortality, 1.8%). As morbidity and mortality rates are acceptable, surgical intervention can be considered safe and effective in patients with pulmonary tuberculosis.
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PMID:Role and outcome of surgery for pulmonary tuberculosis. 1700 80

Lymphangioleiomyomatosis (LAM) is extreme rare diffuse lung disease of unknown cause seen almost exclusively in women of child-bearing age and rarely postmenopausal which indicates the involvement of hormones-estrogens. It results from proliferation of the cells having a smooth muscle cell phenotype (LAM cells) in the lung, and very often in the kidney and axial lymphatics and lymph nodes or any combination. It may occur sporadically or in association with the neurocutaneous syndrome--tuberous sclerosis. LAM cell proliferation may obstruct bronchioles, lymph vessels and venules that lead to airflow obstruction, formation of lung cysts, bullas and pneumothoraces, chylothorax, chylous ascites, hemosiderosis and hemoptysis. Approxymately 400 cases of LAM have been reported so far, most of them in USA. The average survival is about 8.5 years. There is no specific therapy. There are attempts with progesterone, lung transplantation, Doxycicline besides the symptomatic therapy. A new drug Rapamycin is tested. We are reporting 43-year old woman admitted in the Clinic for pulmonary diseases and TB "Podhrastovi" because of progressive dyspnea and suspect lung diffuse fibrosis, after the surgical treatment of spontaneous pneumothorax. The diagnosis of lung lymphangiomyomatosis was established by chest X ray, computerized chest tomography (CT), pathohistological findings of open lung biopsy. The treatment with progesterone is underway with other symptomatic therapy.
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PMID:[Lung lymphangioleiomyomatosis (LAM)]. 1882 52

Pulmonary lymphangiomyomatosis (LAM) is a rare disease which occurs mainly in women of child-bearing age. The clinical characteristics of LAM include recurrent spontaneous pneumothorax, dyspnea on exertion, hemoptysis, chylothorax, and so on. A 41-year old woman was referred to our department for treatment of recurrent spontaneous pneumothorax. Chest computed tomography (CT) showed right pneumothorax and multiple small bullae located bilateral lung. These CT findings were most suggestive of LAM. To obtain a definitive diagnosis and treat pneumothorax, we performed a thoracoscopic lung biopsy, bullectomy, plication of bullae, and also performed mechanical and chemical pleurodesis. Histopathological examination of surgical specimen using immunohistochemical staining of HMB-45 and alpha-SMA revealed the proliferation of LAM cells, confirming a diagnosis of LAM. Here, we report a case that was diagnosed as LAM by thoracoscopic lung biopsy and treated by thoracoscopic surgery and pleurodesis.
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PMID:[Pulmonary lymphangiomyomatosis diagnosed by video-assisted thoracic surgery; report of a case]. 1904 13

Lymphangiomas are regarded as malformations arising from sequestration of lymphatic tissue that fail to communicate with the lymphatic system. Lymphangiomatosis is defined as a pathological condition where either multiple lymphangiomas are present or multiple organ systems are involved. We report a 30-year-old male who presented with cough, hemoptysis and severe dyspnea as the result of progressive chylothorax and chylopericardium. Despite three months of treatment with total parenteral fat-free nutrition, octreotide, repeated pleurocentesis, and pleuroperitoneal shunting, a life-threatening deterioration of his disease developed. Radiologic findings included multiple bilateral cystic lesions in the mediastinum, hili, pleura and pericardium, along the lymphatic vessels. Although general experience with surgical treatment of lymphangiomatosis is limited, our patient nevertheless made an almost complete functional recovery after pulmonary lymphangiectomy. Thus, preoperative risk assessment based primarily on radiologic findings may be misleading. To our knowledge this is the first report in the literature to indicate that surgery should be an option in patients with multiorgan lymphangiomatosis.
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PMID:Successful surgical treatment of thoracic multiorgan lymphangiomatosis. 1992 Nov 32

Paragonimiasis is a parasitic infection endemic in Asia but uncommon in the United States. It presents most frequently with cough and hemoptysis. Pleural effusions are common and can manifest occasionally as a pseudochylothorax, but a true chylothorax has, to our knowledge, never been reported. We report a case of a chylothorax from infection with Paragonimus westermani. The case is also unique because it occurred in a native Californian who had not traveled to Asia and was infected by eating raw crabs at a local sushi restaurant. The freshwater crabs had been imported from Asia.
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PMID:Chylothorax caused by Paragonimus westermani in a native Californian. 2197 85


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