TY - JOUR T1 - Study of tetrahydrobiopterin in idiopathic pulmonary fibrosis and COPD JF - European Respiratory Journal JO - Eur Respir J VL - 38 IS - Suppl 55 SP - p793 AU - Patricia Almudéver Folch AU - Alfredo De Diego Damia AU - Julio Cortijo Gimeno AU - Montserrat Leόn Fábregas AU - M.J. Fandόs AU - Xavier Milara Y1 - 2011/09/01 UR - http://erj.ersjournals.com/content/38/Suppl_55/p793.abstract N2 - Introduction: Tetrahydrobiopterin (BH4) is an essential cofactor for the activity of nitric oxide synthase enzyme (eNOS). Deficiency of BH4 induced by oxidative stress could produce eNOS uncoupling and contribute to pulmonary damage.Objective: To study the role of endogenous BH4 in patients with stable idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD).Materials and methods: Twenty eight patients (15 IPF, 13 COPD; 61 (13) years) and 9 healthy controls were studied. Lung function tests (spirometry, pletismography and lung diffusion capacity), HRCT (High Resolution Computed Tomography) lung scan and 6-min. walk test were performed in all patients. Blood neutrophils, plasma BH4 (reverse-phase high-performance liquid chromatographic (RP-HPLC) with fluorescence detection), hemoglobin, fibrinogen, and CRP were also measured. Differences in BH4 levels between groups and its relationship with clinical, functional or biological parameters of disease severity were analized.Results: BH4 was significantly reduced in IPF 1.32 (0.16)), and COPD (1.44 (0.27)) patients versus controls (2.42 (0.29)). There were no differences in BH4 levels between either IPF and COPD or bronchitis and emphysema phenotypes. BH4 levels were not related with parameters of lung function, radiological extension, inflammatory markers or smoking severity. In patients with COPD, BH4 levels were related with the number of previous exacerbations.Conclusions: Plasma BH4 levels are reduced in IPF and COPD, which may be of potential value as a future biomarker of oxidative stress related diseases.Supported by grant SVN 2010 and CIBERES (CB06/06/0027) ER -