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Hermansky F, Pudlak P. Albinism associated with hemorrhagic diathesis and unusual pigment reticular cells in the bone marrow: report of two cases with histochemical studies. Blood 14: 162-169, 1959."},{"key":"2","doi-asserted-by":"crossref","unstructured":"2. Mahavadi P, Korfei M, Henneke I, et al. Epithelial stress and apoptosis underlie Hermansky-Pudlak syndrome-associated interstitial pneumonia. Am J Respir Crit Care Med 182: 207-219, 2010.","DOI":"10.1164\/rccm.200909-1414OC"},{"key":"3","doi-asserted-by":"crossref","unstructured":"3. Young LR, Gulleman PM, Bridges JP, et al. The alveolar epithelium determines susceptibility to lung fibrosis in Hermansky-Pudlak syndrome. Am J Respir Crit Care Med 186: 1014-1024, 2012.","DOI":"10.1164\/rccm.201207-1206OC"},{"key":"4","unstructured":"4. Wei ML. Hermansky-Pudlak syndrome: a disease of protein trafficking and organelle function. Pigment Cell Res 19: 19-42, 2006."},{"key":"5","unstructured":"5. Nakatani Y, Nakamura N, Sano J, et al. Interstitial pneumonia in Hermansky-Pudlak syndrome: significance of florid foamy swelling\/degeneration (giant lamellar body degeneration) of type-2 pneumocytes. Virchows Arch 437: 304-313, 2000."},{"key":"6","doi-asserted-by":"crossref","unstructured":"6. Gahl WA, Brantly M, Troendle J, et al. Effect of pirfenidone on the pulmonary fibrosis of Hermansky-Pudlak syndrome. Mol Genet Metab 76: 234-242, 2002.","DOI":"10.1016\/S1096-7192(02)00044-6"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7. O'Brien K, Troendle J, Gochuico BR, et al. Pirfenidone for the treatment of Hermansky-Pudlak syndrome fibrosis. Mol Genet Metab 103: 128-134, 2011.","DOI":"10.1016\/j.ymgme.2011.02.003"},{"key":"8","doi-asserted-by":"crossref","unstructured":"8. Ito S, Suzuki T, Inagaki K, et al. High frequency of Hermansky-Pudlak syndrome type 1 (HPS1) among Japanese albinism patients and functional analysis of HPS1 mutant protein. J Invest Dermatol 125: 715-720, 2005.","DOI":"10.1111\/j.0022-202X.2005.23884.x"},{"key":"9","unstructured":"9. Witkop CJ, Nuenz-Babcock M, Rao GH, et al. Albinism and Hermansky-Pudlak syndrome in Puerto Rico. Bol Assoc Med P R 82: 333-339, 1990."},{"key":"10","unstructured":"10. Avila NA, Brantly M, Premkumar A, et al. Hermansky-Pudlak syndrome: radiography and CT of the chest compared with pulmonary function tests and genetic studies. Am J Roentgenol 179: 887-892, 2002."},{"key":"11","doi-asserted-by":"crossref","unstructured":"11. Brantly M, Avila NA, Shotelersuk V, et al. Pulmonary function and high-resolution CT findings in patients with an inherited form of pulmonary fibrosis, Hermansky-Pudlak syndrome, due to mutations in HPS-1. Chest 117: 129-136, 2000.","DOI":"10.1378\/chest.117.1.129"},{"key":"12","doi-asserted-by":"crossref","unstructured":"12. Yokoyama A, Kohno N, Hamada H, et al. Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 158: 1680-1684, 1998.","DOI":"10.1164\/ajrccm.158.5.9803115"},{"key":"13","doi-asserted-by":"crossref","unstructured":"13. Korfei M, Ruppert C, Mahavadi P, et al. Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis. Am J Respir Cri Care Med 178: 838-846, 2008.","DOI":"10.1164\/rccm.200802-313OC"},{"key":"14","doi-asserted-by":"crossref","unstructured":"14. Young LR, Pasula R, Gulleman PM, et al. Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosis. Am J Respir Crit Care Med 37: 67-74, 2007.","DOI":"10.1165\/rcmb.2006-0469OC"},{"key":"15","unstructured":"15. Hongou M, Ikura K, Taira H, et al. A case of Hermansky-Pudlak syndrome associated with interstitial pneumonia. Kokyu (Respiration Research) 12: 238-243, 1993 (in Japanese, Abstract in English)."},{"key":"16","unstructured":"16. Yano T, Tomita Y, Honda Y, et al. A case of diffuse interstitial pneumonia with Hermansky-Pudlak syndrome - review of previous reports in Japan-. Naika (Journal of the Japanese Society of Internal Medicine) 58: 867-870, 1986 (in Japanese, Abstract in English)."},{"key":"17","unstructured":"17. Okano A, Sato A, Chida K, et al. Pulmonary interstitial pneumonia in association with Hermansky-Pudlak syndrome. Nihon Kyobu Shikkan Gakkai Zasshi (The Japanese Journal of Thoracic Diseases) 28: 1596-1602, 1991 (in Japanese, Abstract in English)."},{"key":"18","doi-asserted-by":"crossref","unstructured":"18. Iyer SN, Gurujeyalakshmi G, Giri SN. Effects of pirfenidone on transforming growth factor-β gene expression at transcriptional level in bleomycin hamster model of lung fibrosis. J Pharmacol Exp Ther 291: 367-373, 1999.","DOI":"10.1016\/S0022-3565(24)35110-9"},{"key":"19","doi-asserted-by":"crossref","unstructured":"19. Gurujeyalakshmi G, Hollinger MA, Giri SN. Pirfenidone inhibits PDGF isoforms in bleomycin hamster model of lung fibrosis at translational level. Am J Physiol 276: L311-L318, 1999.","DOI":"10.1152\/ajplung.1999.276.2.L311"},{"key":"20","doi-asserted-by":"crossref","unstructured":"20. Oku H, Nakazato H, Horikawa T, et al. Pirfenidone suppresses tumor necrosis factor-α, enhances interleukin-10 and protects mice from endotoxic shock. Eur J Pharmacol 446: 167-176, 2002.","DOI":"10.1016\/S0014-2999(02)01757-0"},{"key":"21","doi-asserted-by":"crossref","unstructured":"21. Misra HP, Rabideau C. Pirfenidone inhibits NADPH-dependent microsomal lipid peroxidation and scavenges hydroxyl radicals. Mol Cell Biochem 204: 119-126, 2000.","DOI":"10.1023\/A:1007023532508"},{"key":"22","doi-asserted-by":"crossref","unstructured":"22. Rouhani FN, Brantly ML, Markello TC, et al. Alveolar macrophage dysregulation in Hermansky-Pudlak syndrome type 1. 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