Genetics of fibrosing lung diseases

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Genetics of fibrosing lung diseases.

Genetic studies in familial lung fibrosis have demonstrated an association with surfactant protein C genes: two mutations have been found resulting in protein misfolding and causing type-II epithelial cell injury. Remarkably, different histological patterns were observed in the affected subjects, suggesting the influence of modifier genes and/or environmental factors. Surfactant protein C gene ...

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Fibrosing interstitial lung diseases involve different pathogenic pathways with similar outcomes.

Fibrosing interstitial lung diseases (ILDs) are a large group of diseases triggered by external or internal stimuli that can have similar outcomes, i.e. lung fibrosis. Some ILDs are primarily fibro-proliferative disorders in which alveolar loss and epithelial/fibroblastic proliferation and dysplasia lead to lung fibrosis and architectural derangement, while other ILDs are considered inflammator...

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Cryptogenic fibrosing alveolitis and lung cancer.

Lung cancer was found in 20 (9.8%) of 205 patients with cryptogenic fibrosing alveolitis (CFA) or 12.9% of the 155 patients in this series followed to death. An excess relative risk of lung cancer of 14.1 was found in patients with CFA compared to the general population of comparable age and sex, allowing for the lengths of follow-up of the CFA patients. The relative risk for male smokers was (...

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ژورنال

عنوان ژورنال: European Respiratory Journal

سال: 2005

ISSN: 0903-1936,1399-3003

DOI: 10.1183/09031936.05.00133404