Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pulmonary lymphangioleiomyomatosis is a very rare disease that affects women of childbearing age. It presents a challenge for the anesthesiologist during mechanical ventilation because of the restrictive pattern caused by the disease and because of such complications as recurrent pneumothorax and hemoptysis. We report the fulminant course of lymphangioleiomyomatosis in a 38-year-old woman with a history of recurrent spontaneous pneumothorax whose condition was unsuspected. The literature is reviewed.
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PMID:[Fulminant pulmonary lymphangioleiomyomatosis: a case report and review of the literature]. 1564 5

Lymphangioleiomyomatosis, a rare disease of unknown etiology that is seen almost exclusively in women of childbearing age, generally presents with features of pulmonary involvement. It may be associated with tuberous sclerosis. Its clinical pulmonary manifestations vary from simple cough to the development of recurrent pneumothorax, hemoptysis, and even complicated pleural effusions. Progressive dyspnea develops as the disease evolves. Most patients eventually require lung transplant. 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. Here we report a case of a 35-year-old woman with a history of pleuritic effusion with associated dyspnea before being diagnosed with lymphangioleiomyomatosis. A review of the literature pertinent to this case is also provided.
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PMID:Lymphangioleiomyomatosis: a case report and review of the literature. 1565 45

A young female patient of 30 years of age previously asymptomatic presented with complaints of exertional dyspnoea and hemoptysis with a normal chest roentgenogram. She was eventually diagnosed to have lymphangioleiomyomatosis, a rare pulmonary disorder. In this patient it occurred without any history of current pregnancy or estrogen consumption, as a sporadic entity.
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PMID:A rare cause of exertional dyspnoea and hemoptysis in a 30-year-old female. 1570 18

Lymphangioleiomyomatosis and Langerhans cell Histiocytosis are two different diseases that can involve the lungs, with unknown etiology and origin. Both are rare, present a similar radiological pattern (multiple nodules and cysts in the lungs) and may have a similar clinical presentation, with progressive dyspnea, hemoptysis, pneumothorax and a variable course usually culminating in respiratory failure. Due to these similarities a differential diagnosis may be difficult in some instances. This review underlines differences in epidemiology, pathogenesis and prognosis that could assist clinicians in making a differential diagnosis of these rare and severe pulmonary diseases.
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PMID:An integrated clinical approach to diffuse cystic lung diseases. 1645 15

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology affecting woman, primarily in their reproductive years. It is characterized by non-neoplasic proliferation of atypical smooth muscle cells that may lead to airway, lymphatic and blood vessel obstruction over time.A nine-year followed-up case of LAM is described,which presents with some unusual details as worsening haemoptysis in the setting of pregnancy and absence of cough or pneumothorax along the course of the disease. Neither haemoptysis nor gradual pulmonary function deterioration seemed to be influenced by hormone therapy. A brief re-view of the disease is then presented.
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PMID:[Lymphangioleiomyomatosis: a case report]. 1651 17

LAM, a rare lung disease typically affecting women of reproductive age, is characterized by abnormal proliferation of smooth--muscle cells and progressive loss of pulmonary function due to destruction of lung parenchyma. Two cases of bilateral successive recurrent spontaneous pneumothorax and haemoptysis are presented. Repeated conventional and video-assisted surgery was required in both cases, for drainage of the recurrent pneumothorax and resection of subpleural bulla, with good immediate postoperative evolution. Immunohistochemical studies of resected specimens revealed LAM cells in the lung parenchyma with receptors for oestrogen and progesterone. HMB45 monoclonal antibodies in the LAM cells were identified in one case. The follow-up of the patients revealed no signs of recurrence at 84 and 18 months respectively, although pulmonary transplantation should be considered in case of further deterioration of respiratory function.
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PMID:[Lymphangioleiomyomatosis (LAM)--an uncommon cause of bilateral spontaneous pneumothorax]. 1759 57

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

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown cause that traditionally affects young women of reproductive age. It is characterized by a proliferation of atypical smooth muscle cells, preferentially along the bronchovascular structures, that causes progressive respiratory failure. LAM is almost universally fatal without a lung transplant, although new clinical trials are ongoing. Because of its rareness and nonspecific presenting symptoms, patients often receive a missed or delayed diagnosis. We present the case of a 51-year-old postmenopausal woman who had hemoptysis ultimately determined to be due to LAM. As is common for patients with LAM, the initial chest radiograph was unremarkable, whereas subsequent computed tomography (CT) demonstrated the distinctive pulmonary parenchymal cysts. Biopsy of an HMB-45-positive, para-aortic lymphangiomyoma provided further confirmation of the diagnosis. LAM may be more common than previously recognized, and it is imperative for primary care providers to be able to recognize this disease so they can make prompt referrals to appropriate specialty centers.
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PMID:Lymphangioleiomyomatosis: a case report. 1936 21

Tuberous sclerosis complex (TSC) is an autosomal-dominant disorder characterized by seizures, mental retardation, and various hamartomatous lesions, including renal angiomyolipoma (AML) and pulmonary lymphangioleiomyomatosis. A 22-year-old woman with TSC presented with multiple renal AMLs exceeding 4 cm in diameter. She underwent two transcatheter embolization procedures with a mixture of ethanol and iodized oil. She complained of dyspnea and minor hemoptysis 4 and 6 hours after the procedure. Findings on chest radiographs and computed tomographic images were indicative of pulmonary edema. The patient was treated with conservative therapy. The possible causes of the pulmonary edema are discussed in the text.
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PMID:Pulmonary edema as a complication of transcatheter embolization of renal angiomyolipoma in a patient with pulmonary lymphangioleiomyomatosis due to tuberous sclerosis complex. 1946 8


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