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Evaluation of Oxidative Stress Markers and their Correlation with Respiratory Muscle Strength in Congenital Generalized Lipodystrophy

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.