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Idiopathic Pulmonary Fibrosis (IPF) as the name suggests is a progressive disorder with no known aetiology. It is characterised by the thickening of the alveoli due to scarring resulting in cough. It is known to primarily occur in older adults over 60 years of age. The findings of IPF have a known association of Usual Interstitial Pneumonia (UIP) (Raghu et al., 2011; Kawano-Dourado & Kairalla, 2013; Wells, 2013). It has been deemed that the prognosis is generally poor when UIP has been confirmed (King et al., 2001b). The median survival rate of IPF is 50%, typically around two years after diagnosis (Raghu et al., 2011; King et al., 2001b). Idiopathic Pulmonary Fibrosis (IPF) as the name suggests is a progressive disorder with no known aetiology. It is characterised by the thickening of the alveoli due to scarring resulting in cough. It is known to primarily occur in older adults over 60 years of age. The findings of IPF have a known association of Usual Interstitial Pneumonia (UIP) (Raghu et al., 2011; Kawano-Dourado & Kairalla, 2013; Wells, 2013). It has been deemed that the prognosis is generally poor when UIP has been confirmed (King et al., 2001b). The median survival rate of IPF is 50%, typically around two years after diagnosis (Raghu et al., 2011; King et al., 2001b).

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http://jcm.asm.org/cgi/doi/10.1128/JCM.41.6.2633-2640.2003. Taniguchi, H., Ebina, M., Kondoh, Y., Ogura, T., Azuma, A., Suga, M., Taguchi, Y., Takahashi, H., Nakata, K., Sato, A., Takeuchi, M., Raghu, G., Kudoh, S., Nukiwa, T. & Pirfenidone Clinical Study Group in Japan (2010). Pirfenidone in idiopathic pulmonary fibrosis. The European respiratory journal. [Online]. 35 (4). pp. 821–9. Available from: http://www.ncbi.nlm.nih.gov/pubmed/19996196. Taskar, V.S. & Coultas, D.B. (2006). Is idiopathic pulmonary fibrosis an environmental disease? Proceedings of the American Thoracic Society. [Online]. 3 (4). pp. 293–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/16738192. Thomas, A.Q., Lane, K., Phillips, J., Prince, M., Markin, C., Speer, M., Schwartz, D.A., Gaddipati, R., Marney, A., Johnson, J., Roberts, R., Haines, J., Stahlman, M. & Loyd, J.E. (2002). Heterozygosity for a surfactant protein C gene mutation associated with usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one kindred. American journal of respiratory and critical care medicine. [Online]. 165 (9). pp. 1322–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/11991887. Tian, X., Yao, W., Guo, Z., Gu, L. & Zhu, Y. (2006). Low dose pirfenidone suppresses transforming growth factor beta-1 and tissue inhibitor of metalloproteinase-1, and protects rats from lung fibrosis induced by bleomycina. Chinese medical sciences journal = Chung-kuo i hsueh k’o hsueh tsa chih. [Online]. 21 (3). pp. 145–51. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17086734. Tilg, H. & Moschen, A.R. (2006). Adipocytokines: mediators linking adipose tissue, inflammation and immunity. Nature reviews. Immunology. 6 (10). pp. 772–783. Tobin, R.W., Pope, C.E., Pellegrini, C.A., Emond, M.J., Sillery, J. & Raghu, G. (1998). Increased Prevalence of Gastroesophageal Reflux in Patients with Idiopathic Pulmonary Fibrosis. American Journal of Respiratory and Critical Care Medicine. 158 (6). pp. 1804–1808. Toll, D.B., Janssen, K.J.M., Vergouwe, Y. & Moons, K.G.M. (2008). Validation, updating and impact of clinical prediction rules: A review. Journal of Clinical Epidemiology. 61 © 2017-2018 All Rights Reserved, No part of this document should be modified/used without prior consent Tutors India - Your trusted mentor since 2001 www.tutorindia.com I UK # +44-1143520021, info@tutorsindia.com Page 164 of 172

(11). pp. 1085–1094. Tomioka, H., Kuwata, Y., Imanaka, K., Hashimoto, K., Ohnishi, H., Tada, K., Sakamoto, H. & Iwasaki, H. (2005). A pilot study of aerosolized N-acetylcysteine for idiopathic pulmonary fibrosis. Respirology (Carlton, Vic.). [Online]. 10 (4). pp. 449–55. Available from: http://www.ncbi.nlm.nih.gov/pubmed/16135167. Tomioka, H., Sakurai, T., Hashimoto, K. & Iwasaki, H. (2007). Acute exacerbation of idiopathic pulmonary fibrosis: Role of Chlamydophila pneumoniae infection. Respirology. 12 (5). pp. 700–706. Toubas, D., Prévost, A., Deschamps, F. & Pinon, J.M. (1995). [Extrinsic allergic alveolitis of occupational origin]. Presse medicale (Paris, France : 1983). 24 (30). pp. 1391–1396. Travis, W.D., Costabel, U., Hansell, D.M., King, T.E., Lynch, D.A., Nicholson, A.G., Ryerson, C.J., Ryu, J.H., Selman, M., Wells, A.U., Behr, J., Bouros, D., Brown, K.K., Colby, T. V, Collard, H.R., Cordeiro, C.R., Cottin, V., Crestani, B., Drent, M., Dudden, R.F., Egan, J., Flaherty, K., Hogaboam, C., Inoue, Y., Johkoh, T., Kim, D.S., Kitaichi, M., Loyd, J., Martinez, F.J., Myers, J., Protzko, S., Raghu, G., Richeldi, L., Sverzellati, N., Swigris, J., Valeyre, D. & ATS/ERS Committee on Idiopathic Interstitial Pneumonias (2013). An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. American journal of respiratory and critical care medicine. [Online]. 188 (6). pp. 733–48. Available from: http://www.ncbi.nlm.nih.gov/pubmed/24032382. Tsakiri, K.D., Cronkhite, J.T., Kuan, P.J., Xing, C., Raghu, G., Weissler, J.C., Rosenblatt, R.L., Shay, J.W. & Garcia, C.K. (2007). Adult-onset pulmonary fibrosis caused by mutations in telomerase. Proceedings of the National Academy of Sciences. [Online]. 104 (18). pp. 7552–7557. Available from: http://www.pnas.org/cgi/doi/10.1073/pnas.0701009104. Tsukamoto, K., Hayakawa, H., Sato, A., Chida, K., Nakamura, H. & Miura, K. (2000). Involvement of Epstein-Barr virus latent membrane protein 1 in disease progression in patients with idiopathic pulmonary fibrosis. Thorax. [Online]. 55 (11). pp. 958–61. © 2017-2018 All Rights Reserved, No part of this document should be modified/used without prior consent Tutors India - Your trusted mentor since 2001 www.tutorindia.com I UK # +44-1143520021, info@tutorsindia.com Page 165 of 172

http://jcm.asm.org/cgi/doi/10.1128/JCM.41.6.2633-2640.2003.<br />

Taniguchi, H., Ebina, M., Kondoh, Y., Ogura, T., Azuma, A., Suga, M., Taguchi, Y.,<br />

Takahashi, H., Nakata, K., Sato, A., Takeuchi, M., Raghu, G., Kudoh, S., Nukiwa, T. &<br />

Pirfenidone Clinical Study Group in Japan (2010). Pirfenidone in idiopathic pulmonary<br />

fibrosis. The European respiratory journal. [Online]. 35 (4). pp. 821–9. Available from:<br />

http://www.ncbi.nlm.nih.gov/pubmed/19996196.<br />

Taskar, V.S. & Coultas, D.B. (2006). Is idiopathic pulmonary fibrosis an environmental<br />

disease? Proceedings of the American Thoracic Society. [Online]. 3 (4). pp. 293–8.<br />

Available from: http://www.ncbi.nlm.nih.gov/pubmed/16738192.<br />

Thomas, A.Q., Lane, K., Phillips, J., Prince, M., Markin, C., Speer, M., Schwartz, D.A.,<br />

Gaddipati, R., Marney, A., Johnson, J., Roberts, R., Haines, J., Stahlman, M. & Loyd,<br />

J.E. (2002). Heterozygosity for a surfactant protein C gene mutation associated with<br />

usual interstitial pneumonitis and cellular nonspecific interstitial pneumonitis in one<br />

kindred. American journal of respiratory and critical care medicine. [Online]. 165 (9).<br />

pp. 1322–8. Available from: http://www.ncbi.nlm.nih.gov/pubmed/11991887.<br />

Tian, X., Yao, W., Guo, Z., Gu, L. & Zhu, Y. (2006). Low dose pirfenidone suppresses<br />

transforming growth factor beta-1 and tissue inhibitor of metalloproteinase-1, and<br />

protects rats from lung fibrosis induced by bleomycina. Chinese medical sciences<br />

journal = Chung-kuo i hsueh k’o hsueh tsa chih. [Online]. 21 (3). pp. 145–51. Available<br />

from: http://www.ncbi.nlm.nih.gov/pubmed/17086734.<br />

Tilg, H. & Moschen, A.R. (2006). Adipocytokines: mediators linking adipose tissue,<br />

inflammation and immunity. Nature reviews. Immunology. 6 (10). pp. 772–783.<br />

Tobin, R.W., Pope, C.E., Pellegrini, C.A., Emond, M.J., Sillery, J. & Raghu, G. (1998).<br />

Increased Prevalence of Gastroesophageal Reflux in Patients with Idiopathic Pulmonary<br />

Fibrosis. American Journal of Respiratory and Critical Care Medicine. 158 (6). pp.<br />

1804–1808.<br />

Toll, D.B., Janssen, K.J.M., Vergouwe, Y. & Moons, K.G.M. (2008). Validation, updating<br />

and impact of clinical prediction rules: A review. Journal of Clinical Epidemiology. 61<br />

© 2017-2018 All Rights Reserved, No part of this document should be modified/used without prior consent<br />

<strong>Tutors</strong> <strong>India</strong> - Your trusted mentor since 2001<br />

www.tutorindia.com I UK # +44-1143520021, info@tutorsindia.com<br />

Page 164 of 172

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