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

Lymphangioleiomyomatosis (LAM) is a rare lung disease affecting premenopausal women characterized by an abnormal proliferation of smooth muscle cells that leads to the obstruction of airways, lymph and blood vessels. We present a case of a 46-year-old woman who was admitted to our department with dyspnoea and dry cough. The patient had a history of spontaneous pneumothorax 2 years prior to admission. Physical examination revealed dull percussion note on the lower right side of the chest. The chest X-ray film showed diffuse interstitial parenchymal infiltration and flattened costodiaphragmatic angle on the right side. The high resolution computerized tomography (HRCT) scan showed the numerous air filled cysts, about 25 mm in diameter with thin regular walls and liquid in the right pleural cavity. The effusion in a pleural cavity was chylous. Airway obstruction (FEV1/FVC 57% of predicted), markedly elevated residual volume (140%), and decreased DLCO were observed in functional pulmonary tests, and she underwent diagnostic videothoracoscopy. Pulmonary biopsy specimens confirmed diagnosis of LAM. The patient has been under careful observation, no treatment was instituted. The patient remains clinically stabile. During the last six months of observation she has normal sex hormone levels, therefore there exists a possibility of postmenopausal remission of symptoms.
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PMID:[Pulmonary lymphangioleiomyomatosis as a very rare interstitial lung disease]. 1150 51

A 66-year-old woman had a history of partial gastrectomy and resection of the residual stomach because of early gastric cancer and its recurrence. She had been suffering from dyspnea on effort, hemosputum, and cough since the age of 52 years. Chronic pulmonary emphysema and bronchial asthma were diagnosed when she was 59. On January 31, 1996, she was admitted to UOEH hospital with a complaint of increased dyspnea. In spite of treatment with oxygen, steroid, and inhaled anti-cholinergic agent, her condition deteriorated. Subsequently, she had DIC, respiratory failure and reticulolinear opacities were seen on chest radiographs, and she died 2 weeks after admission. At autopsy, the lung specimen revealed numerous cystic spaces surrounded by a proliferation of smooth muscle cells. Immunohistological examination showed these cells to be positive for HMB-45. Signet cells were seen in the lymphatics and vessels, and hemosiderin-laden macrophages were found in the alveolar spaces. This was a rare case of lymphangioleiomyomatosis with carcinomatous lymphangiosis.
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PMID:[A case of lymphangioleiomyomatosis with carcinomatous lymphangiosis]. 1168 Oct 22

This is a case history of a 24-year-old female patient in whom two rare pulmonary diseases occurred: Langerhans cell histiocytosis and four years later lymphangioleiomyomatosis were diagnosed. Both diseases were verified by the examination of the lung tissue removed by the surgery for pneumothorax. The patient's symptoms were characterised by coughing, dyspnoe, repeated pneumothorax. The authors summarised the characteristics and treatment of these diseases. This case history is worth of reporting for its unique rareness.
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PMID:[Rare association of rare diseases: Langerhans-cell histiocytosis and lymphangioleiomyomatosis in the lung]. 1168 Dec 32

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

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of an unknown cause affecting women of reproductive age and characterised by smooth muscle proliferation along lung lymphatic channels. Pneumothorax develops in up to 80% of patients with LAM and may be the presenting manifestation of the disease. Pneumothorax also precedes or complicates the clinical course of 25% of patients with Langerhans'-cell histiocytosis (LCH, histiocytosis X) pathologically characterised by involved tissue infiltration with large numbers of unusual Langerhans' cells, often organised as granulomas. A 41-year-old female patient was treated twice by simple tube drainage due to left pneumothorax in 1996. She was then diagnosed with chronic obstructive lung disease demonstrating with dyspnea, cough and wheeze. Abnormalities found in the high-resolution computed tomography (HRCT) scanning were characteristic of LAM with thin-walled parenchymal cysts distributed homogeneously in both lungs and with thickening of interlobular septa. A 38-year-old man was hospitalised due to chronic lung failure in the course of LCH characterised by small, poorly limited nodular lesions and thin-walled cysts revealed in HRCT scans. For two years of observation, he has suffered five episodes of right pneumothorax. When diagnosing pneumothorax in the middle-age patients, the possible cause of the disease is LAM or LCH. The use of HRCT scanning may enable good determination of the nature and distribution of parenchymal abnormalities found in the diseases.
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PMID:Difficulties in the diagnosis of rare immunological diseases manifesting with cystic lung disease and spontaneous pneumothorax: Case reports. 1498 35

Lymphangioleiomyomatosis (LAM) is a progressive and usually fatal interstitial lung disease characterized by an abnormal smooth-muscle proliferation in the lung and axial lymphatics. It affects almost exclusively young women of childbearing age. The presenting features most commonly include dyspnea, symptoms of pneumothorax and cough. Less commonly patients can present with chest pain, pleural or pericardial effusion and lymphedema. Our patient, a 41-year-old woman, complained mainly of fatigue that had lasted for 2 months and finally became febrile and dispneic, especially when lying down. Pulmonary diagnostic procedures revealed several multicystic destruction of lung parenchyma. There was also respiratory insufficiency with O2 saturation of 87% and lung diffusion capacity reduced to 48%. The retroperitoneum was filled with neoplastic mass as shown on an abdominal CT scan. Pathohistologic analysis of retroperitoneal mass together with the radiologic finding of the lungs correlated with the diagnosis of LAM. The patient was prescribed corticosteroid therapy, which led to rapid clinical improvement. After making a definite diagnosis, the patient was recommended further treatment with medroxyprogesterone. This case shows that LAM, although rare, can present a diagnostic problem to clinicians and should always be considered as one of the diagnostic possibilities in young women with nonspecific pulmonary symptoms.
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PMID:[Lymphangioleiomyomatosis]. 1550 89

Pulmonary lymphangioleiomyomatosis is a rare, progressive and fatal interstitial lung disease that affects women of child-bearing age. It is characterized by the nonneoplastic proliferation of atypical smooth muscle cells around lymphatics, venules, and bronchioles. Patients with pulmonary lymphangioleiomyomatosis most commonly present with exertional dyspnea and a cough. Pneumothorax is almost always found on the initial chest radiograph. We report a case of a 29-year-old woman who presented with abrupt right chest pain and progressive dyspnea. Physical examination in the emergency department was significantly diminished breath sounds over the right lung, and a chest X-ray revealed a right-sided pneumothorax. She required a chest tube for complete lung re-expansion. On the sixth day of admission, left-sided pneumothorax occurred and another chest tube was inserted. She underwent high-resolution computed tomography of the thorax with the findings of numerous cysts evenly distributed throughout the parenchyma of both lungs. Bilateral thoracotomy with lung biopsies and pleurodesis were performed and the surgical findings confirmed the presence of multiple, small, pink-colored cystic lesions on the surfaces of both lungs. Pathological examination showed the characteristic findings of pulmonary lymphangioleiomyomatosis.
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PMID:Pulmonary lymphangioleiomyomatosis in a 29-year-old woman with bilateral spontaneous pneumothorax: case report. 1564 Dec 20

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

The case of lymphangioleiomyomatosis (LAM) was presented. Woman (55 years) receiving for several years estrogens with the symptoms of disease (dyspnea, nonproductive cough, chylous pleural fluid) appeared after menopause. Suggestive clinical symptoms and chest HRCT scan confirmed clinical diagnosis of LAM. Two years after discontinuation of estrogen medication her clinical condition and radiological picture of lungs improved.
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PMID:[Lymphangioleiomyomatosis in a menopausal woman on estrogen replacement therapy]. 1575 74

A 30 year-old housewife presented with cough and shortness of breath which progressed during her ensuing pregnancy, culminating in a still-birth at 9 months of gestation and requiring her hospitalisation in the immediate postpartum period. HRCT scan of the thorax showed thin-walled cysts and open lung biopsy confirmed the clinical impression of lymphangioleiomyomatosis. She was put on oral medroxyprogesterone acetate. After disease flare-up in the postpartum period her symptoms have stabilised.
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PMID:Pregnancy unmasking lymphangioleiomyomatosis. 1590 61


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