ABSTRACT
The most common form of muscular dystrophy is known as Myotonic dystrophy Type 1 (DM1) in adults. It was aimed to investigate the relationship between antioxidant imbalance and diaphragm thickness with pulmonary function test results in peripheral blood of Myotonic Dystrophy Type 1 patients. In the prospective study, 33 DM1 and 32 healthy control groups were taken after the ethics committee decision (2018-10529). Antioxidant defence system enzymes superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPX), glutathione reductase (GR), glutathione S-transferase (GST) and thiobarbituric acid reactive species (TBARS) levels were studied in blood samples. Also, muscular strength (MRC score), creatine kinase (CK) and diaphragm thicknesses were measured, and pulmonary function tests were performed. Among the studied parameters, TBARS levels and GPX, GR and GST activities in erythrocytes of DM1 patients showed a significant decrease in the range of 29-45% compared to the control group. MRC score, diaphragm thickness and inspiratory function test results at the end of inspiration and expiration were found lower though CK levels were higher in DM1 group. In the patient group, a positive correlation was found between antioxidant parameters (TBARS, CAT and GST) with diaphragm thicknesses and pulmonary function test though GPX showed a negative correlation with them. It was emphasized that the data obtained shows the harmful/pathogenic role of oxidative stress caused by free radicals in DM1, and also provide useful data for the treatment and processes of this disease.
