Extensive pulmonary fibrosis is a rare occurrence in pulmonary alveolar proteinosis. We report 2 cases that have interesting implications. A female patient was diagnosed with autoimmune pulmonary alveolar proteinosis that evolved over 7 years into diffuse fibrosis. In a male patient with diffuse fibrosis we incidentally detected electron microscopic features of alveolar surfactant accumulation...

cystic fibrosis is an autosomal recessive disease caused by a wide spectrum of mutations in the gene encoding for the cystic fibrosis transmembrane conductance regulator protein. these mutations that correlate with different phenotypes, vary in their frequency and distribution in different populations. in this study missense mutation r117h that associated with the different clinical symptoms wa...

Transplant 1999;18:884e90. 12. Dahele M, Ung Y, Meharchand J, et al. Integrating regional and community lung cancer services to improve patient care. Curr Oncol 2007;14:234e7. 13. Murray SA, Boyd K, Sheikh A. Palliative care in chronic illness. BMJ 2005;330:611e12. 14. Hofer M. Advanced chronic lung disease: need for an active interdisciplinary approach. Swiss Med Wkly 2007;137:593e601. 15. Rag...

The ventricular weights in 43 patients with interstitial pulmonary fibrosis were retrospectively compared with those in 172 patients with emphysema and chronic bronchitis. The mean right ventricular weight of patients with interstitial pulmonary fibrosis and those with emphysema and chronic bronchitis was 85.5 g (SD 23.2) and 88.8 g (34.3), respectively. Thirty five patients (81%) with intersti...

Although several animal models have been developed to study human pulmonary fibrosis, lack of a perfect model has raised the need for various animal models of pulmonary fibrosis. In this study, we evaluated the pulmonary effect of polyhexamethyleneguanidine phosphate instillation into the lungs of mice to determine the potential of these mice as a murine model of pulmonary fibrosis. Intratrache...

Disease conditions associated with pulmonary fibrosis are progressive and have a poor long-term prognosis with irreversible changes in airway architecture leading to marked morbidity and mortalities. Using murine models we demonstrate a role for interleukin (IL)-25 in the generation of pulmonary fibrosis. Mechanistically, we identify IL-13 release from type 2 innate lymphoid cells (ILC2) as suf...

Based on current evidence, transforming growth factor (TGF)-beta plays a central pathogenic role in the development of pulmonary fibrosis. There is growing evidence that angiotensin II can serve as a stimulus for TGF-beta-mediated lung fibrosis. However, the role of angiotensin II in the pathobiology of pulmonary fibrosis in vivo remains unclear and the therapeutic potential for targeting angio...

Pulmonary fibrosis is a common feature of numerous lung disorders, including interstitial lung diseases, asthma, and chronic obstructive pulmonary disease. Despite the prevalence of pulmonary fibrosis, the molecular mechanisms governing inflammatory and fibroproliferative aspects of the disorder are not clear. Adenosine is a purine-signaling nucleoside that is generated in excess during cellula...

The clinical and histopathological findings are presented in two cases of diffuse interstitial pulmonary fibrosis in which carcinoma of the lung developed. In one case with rheumatoid arthritis and diffuse interstitial pulmonary fibrosis there was ;malignant pulmonary adenomatosis', an association which does not appear to have been reported before. In the second case idiopathic diffuse intersti...

Idiopathic pulmonary fibrosis is a progressive and lethal form of interstitial lung disease that lacks effective therapies at present. Glycyrrhizic acid (GA), a natural compound extracted from a traditional Chinese herbal medicine Glycyrrhiza glabra, was recently reported to benefit lung injury and liver fibrosis in animal models, yet whether GA has a therapeutic effect on pulmonary fibrosis is...