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 45 yr old male developed pulmonary fibrosis after 29 yrs of employment as a dental technician. He subsequently developed adenocarcinoma of the lung. Diffuse interstitial fibrosis was seen using light microscopy. Neutron activation analysis of non-neoplastic lung tissue demonstrated high levels of chromium and cobalt suggesting the possibility of a chromium-cobalt alloy pneumoconiosis.
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PMID:A dental technician with pulmonary fibrosis: a case of chromium-cobalt alloy pneumoconiosis? 209 Apr 88

To understand better the pathogenesis of peripherally occurring well-differentiated adenocarcinoma of the lung in relation to alveolar epithelial hyperplasia (AEH), immunohistochemical, morphometric, and electron microscopic studies were done on surgical pathologically examined cases of lung cancer. Cases with interstitial pulmonary fibrosis were excluded so that the effects of diffuse scarring of the lung on the development of neoplasia were eliminated. Of 70 specimens with various types of pulmonary carcinomas, 15 were found to have coexistent typical or atypical AEH lesions. No area of transition from AEH to neoplasm was found. The immunohistochemistry studies showed significant differences in the reactions of carcinoembryonic and blood group antigens between typical and atypical AEH lesions, but no significant differences could be obtained between atypical AEH lesions and adenocarcinoma. However, the morphometry of the mean nuclear areas revealed a highly significant difference between atypical AEH lesions and adenocarcinoma. Electron microscopy showed many Clara granules in atypical AEH cells. The results did not prove that AEH lesions are precancerous, but the presence of Clara granules in atypical AEH cells raises speculation on the histogenetic relationship between AEH and pulmonary adenocarcinoma.
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PMID:Alveolar epithelial hyperplasia and adenocarcinoma of the lung. 232 96

We have described a patient who had a clinical picture of CREST syndrome and pulmonary interstitial fibrosis, and in whom adenocarcinoma of the lung developed over a four-year period. Despite absence in the literature of the association of lung carcinoma in patients with CREST syndrome, our case is an example of pulmonary fibrosis complicated by lung cancer without any evidence of other risk factors. We believe this to be the first report of such an association. Clinicians, therefore, must be aware that pulmonary interstitial fibrosis in patients with the CREST syndrome may represent a risk for lung cancer.
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PMID:CREST syndrome: a variant of progressive systemic sclerosis, associated with interstitial pulmonary fibrosis and malignancy. 342 Apr 53

This chapter has briefly reviewed the development and progression of peripheral-type adenocarcinoma of the lung, focusing particularly on bronchioloalveolar carcinoma consisting of the nonmucus-producing cell type with or without sclerosis. Histoloical examination reveals that scar cancers are rare except in cases of diffuse pulmonary fibrosis and that many nonmucus-producing bronchioloalveolar carcinomas appear to develop from atypical adenomatous hyperplasia, which can be called adenoma or very well-differentiated adenocarcinoma, and to progress stepwise. Stepwise progression in malignancy can be disclosed not only by cytological and histological examination but also by proliferative activity of the tumor, such as mitotic activity, the percentage of DNA-synthesizing cells and the frequency of proliferating cell nuclear antigen-positive cells, the mean nuclear DNA content of tumor cells and occurrence of aneuploid cell lines, and abnormalities of oncogenes (c-Ki-ras, myc family, and c-erbB2), such as point mutation, rearrangement, amplification, and tumor suppressor genes (point mutation and deletion) such as p53.
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PMID:The development and progression of adenocarcinoma of the lung. 770 84

To study the role of transforming growth factor-beta 1 (TGF-beta 1) in the pathogenesis of pulmonary fibrosis we have examined lung biopsies from nine patients with systemic sclerosis and interstitial lung disease, eight with 'lone' cryptogenic fibrosing alveolitis, two with cystic fibrosis, two with extrinsic allergic alveolitis, two with Langerhans' cell histiocytosis, one with lymphangioleiomyomatosis, one with giant cell interstitial pneumonia, and one adenocarcinoma of the lung. In cryptogenic fibrosing alveolitis, both 'lone' and associated with systemic sclerosis alveolar macrophages, bronchial epithelium and hyperplastic type II pneumonocytes expressed intracellular TGF-beta 1. Extracellular TGF-beta 1 was found in the fibrous tissue immediately beneath the bronchial and hyperplastic alveolar epithelium. In normal lung, however, the alveolar epithelium and alveolar interstitium were negative for both forms of TGF-beta 1. There was strong expression of TGF-beta 1 in hyperplastic mesothelium and its underlying connective tissue and in Langerhans' cells in the two cases of histiocytosis. In the organizing pneumonia in cystic fibrosis, the intraalveolar buds of granulation tissue reacted strongly for the extracellular form of TGF-beta 1 and the overlying hyperplastic epithelium expressed the intracellular form. In lymphangioleiomyomatosis, the aberrant smooth muscle cells strongly expressed intracellular TGF-beta 1 and the extracellular form was expressed in the adjacent connective tissue. In giant cell interstitial pneumonia, the numerous alveolar macrophage including the multinucleate forms, expressed intracellular TGF-beta 1, as did the hyperplastic alveolar epithelium.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Immunohistochemical localization of transforming growth factor-beta 1 in the lungs of patients with systemic sclerosis, cryptogenic fibrosing alveolitis and other lung disorders. 818 7

We performed a cross-sectional study of the demography, clinical and laboratory features of patients with systemic sclerosis patients followed up in our centre from 1984 to 2007. There were 23 cases with the majority of them (96%) being female. They have a mean age of 50.3 years and a mean disease duration of 6.02 (SD 5.82) years. Our patients comprised of multi-ethnic groups with predominantly Chinese (52%), Sarawak natives (35%) and Malays (13%). They have a mean lag time to diagnosis of 24.8 (SD 34.8) months. All the patients have sclerodermatous skin changes with 16(70%) having diffuse scleroderma and 7(30%) having limited scleroderma. The common clinical manifestations found in our patients were Raynaud's phenomenon (91%), sclerodactyly (65%), digital ulcers (52%) and pulmonary fibrosis (52%). There was low incidence of pulmonary hypertension (13%) and renal involvement (4%). The majority of our patients (67%) have positive ANA with 33% positive Scl-70. The majority received calcium channel blockers (87%), aspirin (48%) and low-dose prednisolone (48%). One patient developed adenocarcinoma of the lung on follow-up. This study demonstrated the rarity of systemic sclerosis in our centre with considerable lag time to diagnosis in our patients. Diffuse cutaneous systemic scleroderma is more common in our centre with rare pulmonary hypertension and renal involvement.
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PMID:Systemic sclerosis in Sarawak: a profile of patients treated in the Sarawak General Hospital. 1937 65

Rare heterozygous mutations in the gene encoding surfactant protein A2 (SP-A2, SFTPA2) are associated with adult-onset pulmonary fibrosis and adenocarcinoma of the lung. We have previously shown that two recombinant SP-A2 mutant proteins (G231V and F198S) remain within the endoplasmic reticulum (ER) of A549 cells and are not secreted into the culture medium. The pathogenic mechanism of the mutant proteins is unknown. Here we analyze all common and rare variants of the surfactant protein A2, SP-A2, in both A549 cells and in primary type II alveolar epithelial cells. We show that, in contrast with all other SP-A2 variants, the mutant proteins are not secreted into the medium with wild-type SP-A isoforms, form fewer intracellular dimer and trimer oligomers, are partially insoluble in 0.5% Nonidet P-40 lysates of transfected A549 cells, and demonstrate greater protein instability in chymotrypsin proteolytic digestions. Both the G231V and F198S mutant SP-A2 proteins are destroyed via the ER-association degradation pathway. Expression of the mutant proteins increases the transcription of a BiP-reporter construct, expression of BiP protein, and production of an ER stress-induced XBP-1 spliced product. Human bronchoalveolar wash samples from individuals who are heterozygous for the G231V mutation have similar levels of total SP-A as normal family members, which suggests that the mechanism of disease does not involve an overt lack of secreted SP-A but instead involves an increase in ER stress of resident type II alveolar epithelial cells.
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PMID:Surfactant protein A2 mutations associated with pulmonary fibrosis lead to protein instability and endoplasmic reticulum stress. 2046 29

This a case of a 77 years old male heavy smoker, known to have Combined Pulmonary Fibrosis and Emphysema complicated by a primary invasive adenocarcinoma of the lung with bone metastasis, who presented with a two weeks history of right inguino-scrotal pain and swelling. Imaging studies revealed a right paratesticular formation that appeared to involve the epididymis and the scrotal wall. A biopsy of the mass showed morphological and Immunophenotypic features in favor of metastasis of an adenocarcinoma of the lung. Based on our literature review, there are only few published cases about scrotal wall metastasis of a lung primary.
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PMID:Scrotal wall metastasis from a primary lung adenocarcinoma. 2623 9

Erlotinib is one of the most widely used tyrosine kinase inhibitor targeting human epidermal growth factor receptor. Since its introduction, it has revolutionized the treatment of advanced non-small cell lung cancer. Skin rashes and diarrhea are the most often reported side effects of erlotinib however it is also associated with interstitial pneumonitis or interstitial lung disease, which often turns out to be fatal complication of using this medicine. Though reported scarcely in the western world, the association of interstitial lung disease with epidermal growth factor receptor has attracted a lot of attention in the recent times. Various researches working with murine models of bleomycin-induced pulmonary fibrosis have found a pro and con role of the receptor in development of the interstitial lung disease. We present the case of a patient diagnosed with stage IV adenocarcinoma of the lung with metastasis to brain. He was found to be positive for the human epidermal growth factor mutation and was hence started on erlotinib. Within a few weeks of starting the medicine the patient was admitted with diarrhea. During the course of this admission he developed acute shortness of breath diagnosed as interstitial pneumonitis. The purpose of this case report is to review the literature associated with erlotinib induced interstitial pneumonitis and make the practicing oncologists aware of this rare yet fatal complication of erlotinib. Here we will also review literature, pertaining to the role of epidermal growth factor receptor in development of interstitial lung disease.
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PMID:Erlotinib Induced Fatal Interstitial Lung Disease in a Patient with Metastatic Non-Small Cell Lung Cancer: Case Report and Review of Literature. 2774 84