Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 61-year-old woman was admitted to the Oita Medical University Hospital because of a nonproductive cough and exertional dyspnea. Interstitial changes had been seen on her chest radiograph 5 years previously, but no respiratory symptoms were identified at that time. On admission, chest radiography revealed linear and ground-glass opacities in the middle and lower lung fields. Computed tomography provided evidence of bronchiectasis and micro-honeycombing of the lungs, while lymphocyte and neutrophil counts in the bronchoalveolar lavage fluid were increased. Transbronchial lung biopsy demonstrated alveolitis and Masson's bodies. The patient was not a pigeon breeder, but she could have been exposed to pigeons at her workplace. Indeed, she had specific antibodies against pigeon serum and droppings, and her peripheral lymphocytes showed proliferation in response to pigeon serum. A positive provocation test involving inhalation of pigeon serum confirmed that she had chronic hypersensitivity pneumonitis caused by allergy to pigeons. This is a rare case of chronic hypersensitivity pneumonitis associated with wild pigeons, that progressed to pulmonary fibrosis. Antigen provocation testing proved to be of great value.
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PMID:[A case of chronic hypersensitivity pneumonitis due to wild pigeons]. 1143 19

We report the case of a 65-year-old man with no history of respiratory disease who came to the emergency room complaining of fever, cough, yellowish sputum and increasing dyspnea of one week's duration. Severe respiratory insufficiency was evident upon examination and a chest film showed diffuse pulmonary infiltrates. Microbiological tests were negative. Lung biopsy gave evidence consistent with diffuse alveolar damage in organizing phase. Evolution of symptoms and blood gases was good after corticosteroid treatment was begun, although pulmonary fibrosis was still evident in a follow-up radiograph.
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PMID:[Acute interstitial pneumonia: favorable outcome with steroid therapy]. 1148 Oct 59

Allergic bronchopulmonary aspergillosis (ABPA) is an underdiagnosed pulmonary disorder in asthmatic patients and patients with cystic fibrosis. Its clinical and diagnostic manifestations arise from an allergic response to multiple antigens expressed by fungi, most commonly Aspergillus fumigatus, colonizing the bronchial mucus. The clinical course is one of recurrent exacerbations characterized by chest infiltrates evident on chest x-ray films and associated with cough, wheeze, and sputum production that usually respond to oral corticosteroid treatment. Specific immunologic and radiologic markers of disease include elevation of the total serum IgE levels, presence of aspergillus IgE antibodies, and the occurrence of central bronchiectasis. Long-term treatment with corticosteroids is often required for effective management. The adverse effects of chronic corticosteroid use have led to attempts at treatment with antifungal agents such as itraconazole. Itraconazole has been reported anecdotally to be effective, and evidence for its effectiveness in randomized trials is still accruing. Consideration should be given to its use as a corticosteroid-sparing agent or for treatment of patients in whom corticosteroid response is poor. The natural history and prognosis of ABPA are not well characterized but may be complicated by progression to bronchiectasis and pulmonary fibrosis. If ABPA is diagnosed and treated before the development of bronchiectasis and fibrosis, these complications may be prevented.
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PMID:Diagnosis and treatment of allergic bronchopulmonary aspergillosis. 1156 Mar 5

Inhalation of dusts is an important cause of interstitial lung disease in the tropical countries such as India. While dusts of organic origin, such as the cotton dust causing byssinosis, generally cause bronchial or bronchiolar involvement and hypersensitivity pneumonitis, inorganic metallic dusts cause progressive pulmonary fibrosis. Silicosis, coal workers' pneumoconiosis, and asbestosis are the three most commonly recognized forms of pneumoconiotic pulmonary fibrosis. Pulmonary tuberculosis is an important complication seen in up to 50% of patients of silicosis in some reports from India. The presentation is generally chronic, although acute and accelerated forms of silicosis are known when the exposures are heavy. Breathlessness, dry cough, and general constitutional symptoms are commonly seen. Patients with silicotuberculosis or other forms of infection may also have significant expectoration, hemoptysis, fever, and rapid progression. Respiratory failure and chronic cor pulmonale occur in the later stages. The diagnosis is easily established if the occupational history is available. Dense nodular opacities on chest roentgenograms, which may be large in patients with massive pulmonary fibrosis, are characteristic. Emphysematous changes generally appear in advanced stages or in patients who smoke. Bronchoalveolar lavage and/or lung biopsy may occasionally be required to establish or exclude other causes of interstitial lung disease. Treatment is largely palliative, although a variety of drugs including corticosteroids and procedures such as whole lung lavage have been tried. None of these methods has yet been found successful in the treatment. Preventive safety steps, including removal of the patient from the site of exposure, are the only effective strategies to control disease progression.
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PMID:Dust-induced interstitial lung disease in the tropics. 1158 75

Classical radiation pneumonitis has been described after single dose whole lung irradiation in experimental animals where above a threshold dose of irradiation, there is a sigmoid dose response curve with increasing morbidity and mortality. After clinical fractionated irradiation, however, acute radiation pneumonitis consisting of cough shortness of breath and patchy radiological changes, occurs in <10% of patients, has dyspnoea out of proportion to the volume of lung irradiated and usually resolves completely without long-term effects. There is increasing evidence that this represents a bilateral lymphocytic alveolitis or hypersensitivity pneumonitis and has been termed sporadic pneumonitis. Late radiation toxicity results in pulmonary fibrosis. This is a consequence of repair, which is initiated by tissue injury within the radiation portal. It follows release of chemotactic factors for fibroblasts including transforming growth factor-beta, fibronectin and platelet derived growth factor. Radiation fibrosis is the clinically more significant syndrome for patients. It may result in progressive dyspnoea and mortality in patients. The most predictable change in laboratory lung function tests is a decrease in transfer factor due to damage at the capillary-alveolar level. It also results in decreased lung compliance, which will affect the total lung capacity and the forced vital capacity. The forced expiratory volume in 1 s is less affected, although this seems to depend on the volume of lung irradiated. There is also a decrease in perfusion in the irradiated lung. Radiation fibrosis seems to depend, amongst other factors, on the volume of lung, which is irradiated above a threshold of 20-30 Gy. The morbidity of radiation fibrosis may therefore be minimized by the use of dose volume histogram to minimize the volume of normal lung irradiated in patients at high risk, e.g., patients with who present with poor lung function. The importance of the baseline perfusion in the irradiated areas continues to be studied.
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PMID:Lung toxicity following chest irradiation in patients with lung cancer. 1180 81

Three patients, men aged 65 and 49 and a woman of 38 years, presented with drug-induced interstitial pneumonitis due to nilutamide, bleomycin and nitrofurantoin usage respectively. The signs and symptoms of drug-induced interstitial pneumonitis occur from within several hours up to months or years after the start of treatment, and sometimes even after the cessation of treatment, and consist of a dry cough, dyspnoea, fever and crackles upon auscultation. The results of further investigations are atypical and consist of abnormalities on the X-ray or CT of the thorax and lymphocytosis in the bronchoalveolar lavage fluid. The mechanisms of damage usually include direct pulmonary toxicity and indirect inflammatory or immunological processes. In most patients, the pulmonary abnormalities disappear after cessation of the drug treatment. Sometimes the disease progresses to pulmonary fibrosis and respiratory failure despite treatment with corticosteroids.
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PMID:[Respiratory side-effects of drugs]. 1184 61

Treatment options for patients with pulmonary fibrosis associated with rheumatoid disease are limited. We report a case of a 71-year-old man with a 3-year history of seropositive rheumatoid arthritis (RA) referred to the pulmonary clinic because of progressive pulmonary symptoms associated with radiographic fibrosis that was progressive in spite of corticosteroid treatment. In an attempt to control his articular symptoms and alter the course of his pulmonary fibrosis, treatment with IV infusion of the tumor necrosis factor (TNF)-alpha inhibitor infliximab was initiated. Following 1 year of therapy with this agent, the patient reported sustained improvement in dyspnea, cough, and exercise tolerance, in addition to improvement in joint symptoms. Stabilization of pulmonary function was indicated by repeat pulmonary function test findings. This report suggests that inhibition of TNF-alpha may be of significant benefit to patients with fibrosing lung conditions in the setting of RA.
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PMID:Clinical response of rheumatoid arthritis-associated pulmonary fibrosis to tumor necrosis factor-alpha inhibition. 1285 58

Congenital tracheobronchomegaly or Mounier-Kuhn Syndrome is a rare disorder of controversial etiology. It is characterized by an abnormal enlargement of the trachea and primary bronchi, because of atrophy or absence of their elastic fibers and smooth muscles. Such alterations lead to the collapse of the respiratory tract during forced exhalation, making expectoration by coughing of little use. Subjects with this disorder are, therefore, predisposed to the development of phlogistic bronchopulmonary pathologies such as bronchitis, emphysema, bronchiectasis and pulmonary fibrosis. The present work reports the case of a 65-year-old man suffering from asymptomatic congenital tracheobronchomegaly which was unknown until preoperative testing was performed (standard chest x-ray, tracheobronchoscopy) following a diagnosis of squamous cell carcinoma of the larynx. The patient underwent total laryngectomy and bilateral neck dissection without any intra- and postoperative complication. This is the first case reported in the literature of an association between laryngeal carcinoma and Mounier-Kuhn Syndrome, although a cause-effect relationship between the two pathologies cannot be advanced at this time. This paper also reports how the patient was managed in terms of anesthesiology and surgical technique, both conditioned by the marked tendency for anteroposterior tracheal wall collapse and its high reactivity to mechanical insults.
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PMID:[Laryngeal carcinoma associated with congenital tracheobronchomegaly (Mounier-Kuhn syndrome): a case report]. 1223 10

There exists a rarely observed association between pulmonary histiocytosis X and bronchopulmonary cancer. However, the frequency of bronchopulmonary cancer in these patients is higher than in the general population. A 28-year-old patient who currently smokes ten packs of cigarettes a year came to our department of pneumology with complains of cough and hemoptysis. An x-ray of the thorax revealed bilateral cysts and a shadow in the upper part of the right pulmonary field. In addition, a chest tomography showed multiple cysts dispersed throughout the two pulmonary fields and an irregular mass with a diameter of four centimetres in the upper right lobe. Bronchopulmonary adenocarcinoma was diagnosed during a cytologic exam of the bronchial washing. We decided to perform a thoracotomy on the patient, since there was no far metastasis. An upper lobectomy and wedge resection of the upper segment of the lower right lobe, which had been invaded by the tumour, were performed. Histology confirmed the diagnosis of adenocarcinoma. A pulmonary biopsy was carried out on the tumour-free site and showed the presence of histiocytosis X. There is a hypothesis that a neoplasm developed on the pulmonary fibrosis could be an epiphenomenon of bronchopulmonary cancer in patients who smoke and have pulmonary histiocytosis X. It is interesting to note that histiocytosis X and bronchopulmonary cancer were diagnosed at the same time, since the bronchopulmonary cancer may have occurred within a few years following the diagnosis of histiocytosis X, even if she was a smoker. Hemoptysis, which is found in 5% of patients with histiocytosis X, may suggest cancer. This young patient, a smoker, who complained of hemoptysis, is a particularly rare case of the association between pulmonary histiocytosis X and bronchopulmonary cancer whose pathogenesis is not clear cut. It is thus important to note that smoking can have major consequences, even in young people.
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PMID:[Histiocytosis X and bronchopulmonary adenocarcinoma: a rare coexistence]. 1252 89

We report the case of a 58-year-old woman who was considered to have died of cyclophosphamide (CPA)-induced late-onset lung disease. She underwent a right-sided mastectomy due to breast cancer at the age of 50 followed by daily administration of 50 mg CPA and 20 mg tamoxifen for 2 years. A refractory cough and dyspnea began at the age of 56. Chest radiographs revealed diffuse infiltrates and pleural thickening. Her vital capacity was markedly reduced. Corticosteroid therapy was ineffective. The disease rapidly progressed with occasional episodes of pneumothoraces to her death. Postmortem examination revealed pulmonary fibrosis with marked elastosis.
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PMID:Cyclophosphamide-induced late-onset lung disease. 1258 25


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