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 case of tobacco-associated pulmonary fibrosis, with the results of histological, ultrastructural, and spectrometric analysis is reported. Abnormalities of the alveolar macrophages, which are particularly affected by tobacco inhalation were found. The size of the macrophages was increased and many large, polymorphous inclusions, including fat vacuoles and granular deposits, which were either homogeneous or electron lucent vacuoles, were seen in the cytoplasm. A few laminar structures were observed. All of these lesions are frequently found in cigarette smokers. Still more interesting was the discovery of numerous fiber-, needle-, or laminar-like inclusions that varied in size from 0.2 to more than 2 mu. The digestions of the inclusions with potassium hydroxide confirmed the presence of various metals, such as sodium, magnesium, potassium, iron, sulfur, and especially, aluminum, and silicon; these last two elements correspond to the presence of kaolinite in the tissue, as has been previously described, and can be considered as evidence of the use of tobacco.
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PMID:Inorganic cytoplasmic inclusions in alveolar macrophages. The role of cigarette smoking. 20 46

Most cell types have receptors for transforming growth factor-beta (TGF-beta) and respond similarly to TGF-beta 1 and TGF-beta 2. We have demonstrated the presence of a single class of high-affinity receptors (approximately 10,000 sites/cell) for TGF-beta 1 (Kd = 23 pM) and TGF-beta 2 (Kd = 41 pM) on early-passage rat lung fibroblasts (RLF). Incubation with unlabeled TGF-beta 1 and TGF-beta 2 resulted in concentration-dependent inhibition of binding of 15 pM [125I]TGF-beta 1 (ED50, 20 and 28 pM, respectively) and [125I]TGF-beta 2 (ED50, 36 and 56 pM, respectively). TGF-beta receptors affinity-cross-linked with 100 pM [125I]TGF-beta 1 or [125I]TGF-beta 2 were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis and exhibited labeled protein bands of 68, 88, and 286 kD. Densitometric analysis of the resulting autoradiograms showed that the different molecular weight TGF-beta binding proteins exhibited separate affinities for the two forms of TGF-beta. Both TGF-beta 1 and TGF-beta 2 altered the morphology and cytoskeleton of RLF in a similar manner, but TGF-beta 1 was more potent than TGF-beta 2 in the inhibition of RLF growth and colony formation, with 50% inhibition by 0.12 pM TGF-beta 1 and 4.4 pM TGF-beta 2. Different affinities for the TGF-beta s may indicate selectivity among the receptor subtypes with regard to the biologic responsiveness of RLF to TGF-beta s. We believe this to be the first demonstration of biologically responsive TGF-beta receptors with different affinities for TGF-beta 1 and TGF-beta 2 on cells derived from normal, nonimmortal RLF. In establishing the basic mechanisms of pulmonary fibrosis, it will be essential to understand the biology and biochemistry of the receptors that may control cell division and production of extracellular matrix components by fibroblasts.
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PMID:Receptors for transforming growth factor-beta (TGF-beta) on rat lung fibroblasts have higher affinity for TGF-beta 1 than for TGF-beta 2. 185 Jun 5

Based on the highly suspicious radiological findings of exposure to asbestos (case 1) or on a positive occupational history (cases 2 and 3), the authors looked for the presence of fibers in blocks of lung tissue removed in autopsy or surgical biopsies of three cases of bronchogenic carcinoma. The blocks were submitted to sodium hypochloride digestion followed by fiber identification in phase contrast light microscopy. The authors were able to demonstrate the presence of fibres in the three cases. The likelyhood of those carcinomas being caused by exposure to asbestos is very high, as two out of the three cases showed pulmonary fibrosis (cases 1 and 2) and the other case showed typical parietal pleural plaques at thoracotomy.
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PMID:[Asbestos and bronchogenic carcinoma: a search for fibers in the pulmonary tissue of 3 patients with bronchogenic carcinoma]. 269 31

Bleomycin is a chemotherapeutic agent sometimes associated with pulmonary fibrosis and skin lesions in patients undergoing treatment. We examined the mechanisms of increased collagen deposition on bleomycin-induced fibrosis by incubating human lung and skin fibroblast cultures with [14C]proline; the synthesis of [14C]hydroxyproline relative to DNA or cell protein was taken as an index of procollagen formation. Procollagen synthesis by lung cells in the presence of 0.1 and 1.0 microgram/ml bleomycin was significantly increased and similar results were obtained with skin fibroblasts. The relative synthesis of genetically distinct types of collagen was measured by isolating the newly synthesized type I and type III procollagens by DEAE-cellulose chromatography. The proportion of type III procollagen of total newly synthesized procollagen in control lung fibroblast cultures was 17.4 +/0 0.6% (mean +/- S.E.) while the corresponding value in cells incubated in 1 microgram/ml bleomycin was 12.5 +/- 0.6% (n = 6, P < 0.01). Similar results were obtained when the ratios of newly synthesized type I and type III collagens were estimated by interrupted polyacrylamide disc gel electrophoresis in sodium dodecyl sulfate after a limited proteolytic digestion with pepsin. The results indicate that the increased procollagen synthesis induced by bleomycin in fibroblast cultures is predominantly directed towards the synthesis of type I procollagen.
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PMID:Bleomycin-induced synthesis of type I procollagen by human lung and skin fibroblasts in culture. 615 25

Bronchial complications, including stricture, stenosis, and/or anastomotic dehiscence, are a major cause of morbidity following single lung transplantation. This report describes a 19-year-old man with a diagnosis of end-stage pulmonary fibrosis secondary to prior chemotherapy for non-Hodgkins lymphoma who underwent single lung transplantation. The immunosuppressive regimen included cyclosporine, azathioprine, and methylprednisolone sodium succinate (Solu-Medrol) intravenously for six doses during the first 3 days postoperatively followed by oral prednisone. Sixteen weeks following transplantation, the patient complained of dyspnea. Spirometry revealed a decrease in FEF25-75 and the flow-volume curve demonstrated a bioconcave appearance. The flow-volume loop showed a relatively high initial flow phase occurring over the first 2 to 3 s followed by a low-flow phase. The expiratory phase also showed the same characteristics. Bronchoscopy revealed 75 percent stenosis of the bronchial lumen to the transplanted lung. A transbronchial biopsy specimen obtained at that time was consistent with acute rejection. The patient was treated with a methylprednisolone bolus. A repeated bronchoscopy showed the persistence of stenosis distal to the anastomosis. The patient underwent several bronchoplastic balloon dilatations without complete resolution of the stenosis and a stainless steel mesh stent was placed. Repeated spirometry showed marked improvement of the FEF25-75 and normalization of the flow-volume loop. We conclude that the flow-volume loop curve is a noninvasive procedure that may help monitor the patency of the bronchial anastomoses following single lung transplantation.
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PMID:Use of the flow-volume loop in the diagnosis of bronchial stenosis after single lung transplantation. 751 Jun 2

Study was designed to explore the effect of sodium tanshionone IIA sulfonate on bleomycin-induced pulmonary fibrosis in rat. The amount of lipid peroxides (LPO), hydroxyproline in rat lung tissue homogenate was determined at 3, 7, 14, 28 day after administration respectively. The results suggest that the amount of LPO, hydroxyproline of rat lung homogenate were significantly decreased in sodium tanshionone IIA sulfonate group. In addition, histological changes and width of alveolar septa and percentage area of pulmonary fibrosis scars was measured. The results show that sodium tanshionone IIA sulfonate could ameliorate bleomycin induced pulmonary fibrosis in rat. The mechanism that sodium tanshionone IIA sulfonate could prevent and treat bleomycin-induced pulmonary fibrosis in rat was discussed.
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PMID:[Effects of tanshionone to bleomycin induced pulmonary fibrosis of rats on histological changes and production of lipid peroxides and hydroxyproline]. 753 36

Glycosaminoglycans are constituents of proteoglycans, which are integral components of lung connective tissue. Glycosaminoglycans not only provide structural support to organs, but also influence extracellular matrix assembly, cell adhesion, and cell proliferation. Changes in the metabolism of glycosaminoglycans have been noted in several pulmonary diseases, for example, pulmonary fibrosis and emphysema. We studied quantitative and qualitative changes of glycosaminoglycans in the lungs of rats exposed to a range of ozone levels (0, 0.12, 0.5, 1.0 parts per million) for 20 months. Glycosaminoglycans were isolated from dry-defatted lung tissues through successive digestions by pronase, papain, and 2 M sodium hydroxide. The glycosaminoglycans then were fractionated into individual components using high-performance liquid chromatography. The concentration of total glycosaminoglycans in the tissues varied from 1.5 to 4.2 micrograms of uronate/mg of dry-defatted tissue. Although wide variations in total glycosaminoglycan concentrations exist among individual animals within each exposure group, regression analyses of data indicate a monotonic and statistically significant decrease of total glycosaminoglycans after ozone exposure (p = 0.02). Among individual glycosaminoglycans, hyaluronan, chondroitin 4-sulfate, and chondroitin 6-sulfate levels decreased significantly (p < 0.001, p < 0.05, and p < 0.01, respectively) in animals exposed to ozone when compared with control animals. Heparan sulfate concentration exhibited a significant (p < 0.05) trend toward increase with increasing doses of ozone, but the difference in heparan sulfate concentration between ozone-exposed animals and control animals was not significant. Gel filtration studies of glycosaminoglycans in pooled samples indicated that the molecular size of hyaluronan in animals exposed to ozone was lower than it was in control animals. We noted differences in heparan sulfate's chemical properties and affinity to antithrombin III in ozone-exposed animals and control animals. Although these studies do not provide the mechanism responsible for the observed changes in the lung glycosaminoglycans in ozone-exposed animals, the observations indicate that inhalation of ozone for 20 months affects normal cellular metabolism of proteoglycans, which may contribute to the functional impairment of the lung.
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PMID:Consequences of prolonged inhalation of ozone on F344/N rats: collaborative studies. Part III: Effects on complex carbohydrates of lung connective tissue. 781 21

Paraquat intoxication is a common medical problem in this country. The mortality is high, particularly in the cases of high dose ingestion. Mediastinal emphysema observed in paraquat intoxication always means mortality, however, we experienced a survivor. A 29 y/o female ingested about 10-15 c.c. of 24% (2.4-3.6 gm) paraquat to attempt suicide. Nausea and protracted vomiting occurred shortly after. During hospitalization, mediastinal emphysema developed on the 7th day and subsided 10 days later. Serum paraquat level determination revealed 185 ng/ml in 20 hours after ingestion and 34 ng/ml on the 6th day. She received general supportive treatments with the augmentation of sodium thiosulfate. Hypokalemia had been observed for 11 days and was intractable until hypomagnesemia was identified and corrected. So far, she had returned back to her work for more than 1 year, although the chest x-ray revealed slight pulmonary fibrosis. The high ingested dose, oral ulcers, high serum level and mediastinal emphysema of this patient all implicate a poor prognosis of paraquat intoxication; however, these contradict the observed survival of our patient. In conclusion, mediastinal emphysema observed in paraquat intoxication is not related to the serum level of paraquat. It does not absolutely lead to death and may simply come from the esophageal rupture after vigorous vomiting in paraquat intoxication.
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PMID:Survival of paraquat intoxication complicated with mediastinal emphysema: a case report. 783 61

High resolution computed tomographic (HRCT) scans were obtained in 215 patients with rheumatoid arthritis to assess pulmonary fibrosis (PF). We classified the HRCT appearances as five-point scale (0-4) based on the degrees of PF. The results were as follows: 1. We found 117 cases (54.4%) of PF on HRCT. 2. Patients with PF (grade 1-4) showed significantly increased leucocyte cell counts and significantly worsened pulmonary function test than patients without PF (grade 0). 3. Patients with advanced articular involvement had significantly higher prevalence of PF than others without them. 4. Patients who were previously or currently receiving gold sodium thiomalate (GST) injection or administration of methotrexate had higher prevalence of PF than others. However, patients who were receiving long term GST therapy (1 year long or sigma 1000 mg) had slightly lower prevalence of PF than others. This finding suggests that dose-dependent lung injury is not related to GST therapy. 5. Patients with advanced PF (grade 3, 4) had high prevalence of male sex, smoker, extraarticular manifestation.
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PMID:[Clinical study of rheumatoid interstitial lung disease evaluated by high resolution CT]. 849 80

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by widespread localization of calcispherites in the alveolar spaces. The patients are symptomless for a long time. Nevertheless, this disease slowly develops into pulmonary fibrosis and cardiac failure. The chest X-rays and high-resolution computed tomography strongly point towards a diagnosis of PAM. As for therapeutic approaches, repeated broncho-alveolar lavages (BAL) have been performed with improvement of symptoms but without recovery, and a new oral drug treatment is still under way. We report 2 familial cases of PAM. Both patients underwent chest X-ray examination showing diffuse bilateral micronodular opacities of calcific density. After 5 years, in May 1993, one of them developed exertional dyspnoea, cyanosis, dry cough and was admitted to our Division. Cardiokinetic and diuretic drugs as well as oxygen were administered with satisfactory results. Then repeated BAL were performed. The chest X-ray after 6 months of sodium etidronate (300 mg t.i.d.) administration was unchanged.
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PMID:Pulmonary microlithiasis. Report of two cases. 909 54


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