Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0037116 (silicosis)
 1,822 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Experimental exposure to quartz dust for 100 days (= 700 h) induces in SPF rats histologic changes of the lungs, which have such striking similarities with human cases of pulmonary alveolar proteinosis that they may represent an animal model of human disease. Conventional stock rats, the standard model of experimental silicosis research, react upon the same dosage of quartz dust by the formation or an increase in size of perivascular lymphatic sheaths, in which epitheloid cell granulomas can arise. In SPF rats such granulomas can only be developed in the sparse pre-existent lymphatic tissue, mostly in hilar lymph nodes. The reaction of SPF rats opens an interesting aspect on the significance of the phenomenon of lymphatic "drainage," as comparable reactions are only known in conventional rats following extreme dosage and in human pathology as so-called acute silicoproteinosis.
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PMID:Atypical quartz dust-induced pneumoconiosis in SPF rats. Aspects of the role of the lymphatic system in the pathogenesis of silicosis. 13 73

Three pulmonary disease conditions result from the accumulation of phospholipids in the lung. These conditions are the human lung disease known as pulmonary alveolar proteinosis, the lipoproteinosis that arises in the lungs of rats during acute silicosis, and the phospholipidoses induced by numerous cationic amphiphilic therapeutic agents. In this paper, the status of phospholipid metabolism in the lungs during the process of each of these lung conditions has been reviewed and possible mechanisms for their establishment are discussed. Pulmonary alveolar proteinosis is characterized by the accumulation of tubular myelin-like multilamellated structures in the alveoli and distal airways of patients. These structures appear to be formed by a process of spontaneous assembly involving surfactant protein A and surfactant phospholipids. Structures similar to tubular myelin-like multilamellated structures can be seen in the alveoli of rats during acute silicosis and, as with the human condition, both surfactant protein A and surfactant phospholipids accumulate in the alveoli. Excessive accumulation of surfactant protein A and surfactant phospholipids in the alveoli could arise from their overproduction and hypersecretion by a subpopulation of Type II cells that are activated by silica, and possibly other agents. Phospholipidoses caused by cationic amphiphilic therapeutic agents arise as a result of their inhibition of phospholipid catabolism. Inhibition of phospholipases results in the accumulation of phospholipids in the cytoplasm of alveolar macrophages and other cells. While inhibition of phospholipases by these agents undoubtedly occurs, there are many anomalous features, such as the accumulation of extracellular phospholipids and surfactant protein A, that cannot be accounted for by this simplistic hypothesis.
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PMID:Alveolar proteinosis and phospholipidoses of the lungs. 166 56

Histopathological analysis of massive fibrosis (MF) in lungs obtained from 79 patients with chronic pneumoconiosis revealed evidence of pulmonary alveolar proteinosis (AP) in a surprisingly high proportion of the cases (73%), suggesting that AP may be a usual constituent of MF in chronic pneumoconiosis. AP is not a specific morphological counterpart responsible for acute silicosis. In order to explain the occurrence of AP in MF, several hypothetical mechanisms are proposed and discussed.
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PMID:Pulmonary alveolar proteinosis as a component of massive fibrosis in cases of chronic pneumoconiosis. An autopsy study of 79 cases. 161 Jul 67

Pulmonary alveolar proteinosis is a rare disease with varied radiographic and clinical manifestations. Thirteen patients are presented and the role of pulmonary lavage in management is illustrated. Six patients had associated diseases such as nocardiosis, leukemia, and silicosis. The broad spectrum of relationships found in alveolar proteinosis suggests that it represents one mechanism by which the lung responds to a variety of insults.
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PMID:Pulmonary alveolar proteinosis: relationship to silicosis and pulmonary infection. 736 Sep 77

We describe a case of a patient with idiopathic pulmonary alveolar proteinosis (PAP), who had an elevated serum level of antigranulocyte-macrophage colony stimulating factor (anti-GM-CSF) antibody accompanied by T-cell receptor gene rearrangements in BAL fluid cells. Histopathological examination of the lung excluded lymphoma but revealed PAP and silicosis. There was no detectable serum anti-GM-CSF antibody in 50 outpatients with advanced silicosis who did not have PAP, suggesting that anti-GM-CSF antibody is directly linked to PAP but not to silicosis. We speculate that monoclonal expansion of a T-cell population may play a role in the production of anti-GM-CSF antibody and the development of PAP.
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PMID:A case of idiopathic pulmonary alveolar proteinosis accompanied by T-cell receptor gene rearrangement in bronchoalveolar lavage fluid cells. 1518 85

Abstract Pulmonary alveolar proteinosis (PAP) is a rare disease, with several aetiologies. This study reports the first Finnish case of PAP with possible induction by silica dust. A 58-year-old male patient had a documented history of heavy exposure to silica dust over a long period, although he himself considered the exposure to be low. The patient's cumulative exposure to silica dust was approximately 10 mg m(-3) years according to the workplace measurements. The patient developed classical symptoms and signs of PAP that closely mimicked those of acute silicosis, but he did not have any signs of classic silicosis. We conclude that significant chronic exposure to silica favours the diagnosis of PAP rather than acute silicosis in this case. PAP should be taken into account when patients exposed to silica dust complain of respiratory symptoms. A patient's assessment of his/her exposure to silica may not always be reliable.
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PMID:Pulmonary alveolar proteinosis induced by silica dust? 1724 96

Miliary mottling on chest radiography is seen in miliary tuberculosis, certain fungal infections, sarcoidosis, coal miner's pneumoconiosis, silicosis, hemosiderosis, fibrosing alveolitis, acute extrinsic allergic alveolitis, pulmonary eosinophilic syndrome, pulmonary alveolar proteinosis, and rarely in hematogenous metastases from the primary cancers of the thyroid, kidney, trophoblasts, and some sarcomas. Although very infrequent, miliary mottling can be seen in primary lung cancers. Herein, we report the case of a 28-year-old female with chest X-ray showing miliary mottling. Thoracic computed tomography (CT) features were suggestive of tuberculoma with miliary tuberculosis. CT-guided fine needle aspiration cytology confirmed the diagnosis as lower-lobe, left lung non-small cell carcinoma (adenocarcinoma). It is rare for the non-small cell carcinoma of the lung to present as miliary mottling. The rarity of our case lies in the fact that a young, non-smoking female with miliary mottling was diagnosed with non-small cell carcinoma of the lung.
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PMID:A rare case of non-small cell carcinoma of lung presenting as miliary mottling. 2364 61

The 1930 International Labour Office Conference on silicosis in Johannesburg identified silicosis by setting a medicolegal framework to its nosology: as with other occupational illnesses, its medical content was fixed under economic pressure. This article follows a reading of all the proceedings of this conference (debates and reports of experts) to examine their potential impact on the etiology and nosology of other diseases, specifically sarcoidosis and pulmonary alveolar proteinosis (PAP), "idiopathic" diseases in which inorganic particles may be involved. We propose renewed study of the role of inorganic particles in these diseases. To do this, we propose to mobilize detection means such as mineralogical analysis and electron microscopy and in depth interviewing that are currently seldom used in France, in order to establish diagnosis and the potential occupational and environmental origin of these diseases.
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PMID:From the definition of silicosis at the 1930 Johannesburg conference to the blurred boundaries between pneumoconioses, sarcoidosis, and pulmonary alveolar proteinosis (PAP). 2650 52

Pulmonary alveolar proteinosis (PAP) is a rare pathology characterized by accumulation of phospholipoproteinaceous material within the alveoli. The evolution of PAP is variable and treatment modalities are limited. Pharmacological therapeutic targets are being actively developed, but whole-lung lavage (WLL), first described in the 1960s, remains the cornerstone of therapy. The preferential treatment for PAP in our center is sequential WLL, where each lung is separately and sequentially perfused with warmed saline. However, some patients do not tolerate single lung ventilation (SLV), as there is a greater risk of severe hypoxemia with this method. Extracorporeal membrane oxygenation (ECMO), referring to an extracorporeal circuit that directly oxygenates and removes carbon dioxide from the blood, may be considered in highly selected patients with severe respiratory failure who otherwise would not be able to undergo WLL. In this context, veno-venous ECMO is most often utilized. We describe a case of a 44-year-old male diagnosed with silicosis five years earlier who presented with severe hypoxemic respiratory failure not amenable to WLL under general anesthesia with SLV, which was successfully managed with ECMO-assisted WLL.
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PMID:Whole-lung lavage for severe pulmonary alveolar proteinosis assisted by veno-venous extracorporeal membrane oxygenation: a case report. 3074 96