TY - JOUR T1 - Evaluation of Oxidative Stress Markers and their Correlation with Respiratory Muscle Strength in Congenital Generalized Lipodystrophy JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2019.PA2352 VL - 54 IS - suppl 63 SP - PA2352 AU - Julliane Tamara Araújo de Melo Campos AU - Aquiles Sales Craveiro Sarmento AU - Jorge Luiz Dantas De Medeiros AU - Bruno Carneiro Bezerra AU - Thaiza Teixeira Xavier Nobre AU - Lucien Peroni Gualdi AU - Lucymara Fassarella Agnez Lima AU - Josivan Gomes Lima Y1 - 2019/09/28 UR - http://erj.ersjournals.com/content/54/suppl_63/PA2352.abstract N2 - Introduction: Congenital Generalized Lipodystrophy (CGL) is a disease characterized by metabolic, cardiovascular and respiratory disturbances. Although oxidative stress (OS) have been related to decreased Respiratory Muscle Strength (RMS), their contribution in RMS of CGL subjects needs to be investigated.Objective: To evaluate the levels of metabolic parameters and OS markers, correlating with Maximal Respiratory Pressures in CGL subjects.Methods: Eleven CGL subjects participated in this cross-sectional study. Peripheral blood was collected for biochemical dosages of glucose, triglycerides, total cholesterol, HDL, oxidized glutathione:total glutathione (GSSG:GSH) ratio and malondialdehyde (MDA). MIP and MEP were measured by manovacuometry according to the ATS/ERS guidelines. Data normality was assessed by Shapiro Wilk test. Correlations were performed according to data distribution. Significance level was set at 5%.Results: The majority of CGL subjects were genetically diagnosed with type 2 (BSCL2 mutation) and 67% were females. Mean age was 31.45±8.0 years. GSSG:GSH ratio correlation between MIP and MEP were r= -0.70 (p=0.0358) and r= 0.61 (p=0.076), respectively. MDA correlation between MIP and MEP were r= -0.34 (p=0.3) and r= 0.38 (p=0.3), respectively. No correlation was found between metabolic parameters and MIP and MEP.Conclusions: A strong significant correlation was found between MIP and GSSG:GSH ratio. These data suggest that CGL is an oxidative stress-related metabolic disease and the assessment of OS markers, in addition to metabolic parameters, may be essential to reduce pulmonary comorbidities associated with CGL subjects.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA2352.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -