Renal, Hepatic and Immune Function Indicies in Patients with Duchenne Muscular Dystrophy

Abstract

The purpose of the study is to examine in depth and analyze renal, hepatic and immune function indices in patients with Duchenne muscular dystrophy. We analyzed the follow up clinical and laboratory data of Duchenne muscular dystrophy in 32 patients. The patients underwent a standardized examination, involving studying the medical case history, general clinical data, determining Sheldon's somatotype and the constitutional type, the detailed neurological status examination, testing a personality type, laboratory and instrumental examinations. Through the laboratory examination we determined the general blood test indicators, total serum protein levels, total cholesterol, the ALAT, ASAT, CPK levels, creatinine and urea blood levels, glomerular filtration rate (GFR), the immunogram indices (dynamic data (B-lymphocytes (CD19/CD45), %; T-lymphocytes (CD3/CD 45), %; T-helpers (CD3/CD45/CD4), %; T-suppressors (CD3/CD45/ CD8), %; CD4:CD8 ratio; natural killer cells ratio, myositis profile (Mi-2, IgG antibodies (idiopathic myositis marker); Ku, IgG antibodies (sclerodermia and myositis combination marker); the PM-Scl complex, IgG antibodies (sclerodermia marker); histidyl tRNA synthetase (Jo-1), IgG antibodies; threonyl-tRNA synthetase (PL-7), IgG antibodies; alanyl-tRNA synthetase (PL-12), IgG antibodies; RING-type E3 ubiquitin-ligase (Ro-52), IgG antibodies and the genetic markers of the disease. The instrumental examination included the ultrasound of the abdominal organs, muscles, as well as echo-cardiography, electroneuromyography. According to the analysis of the immunograms, the T-helpers level was found to be below the reference value in 14 patients (45,2%). Herewith, the B-lymphocytes count was observed to deviate from the norm in only 4 patients (8%). However, there was established the presence of an indirect moderate correlation between the B-lymphocytes count and Creatine phosphokinase level in patients with Duchenne muscular dystrophy; the correlation coefficient was equal to - 0.42 (р<0,05). Having analyzed the renal function indices, we revealed that the creatinine blood level was lower than the lower limit of the reference values in almost a third of patients (21%), but the glomerular filtration rate (GFR) by the SKD-EPI formula was found to be insignificantly reduced in 16% of patients, and it was within 66-87 ml/min/1.73 m2. Regardless, no correlation between the Creatine phosphokinase level, on one side, and creatinine blood level and the glomerular filtration rate, on the other side, was found. However, there was established the presence of an indirect weak correlation between the Creatine phosphokinase level and serum urea level in patients with Duchenne muscular dystrophy (the correlation coefficient was equal to - 0.28, p<0.05). Having analyzed the hepatic function indices, we revealed that such indicators of liver enzymatic activity as alanine aminotransferase and aspartate aminotransferase were significantly over the limit in almost all the patients (94%) with Duchenne muscular dystrophy. Concurrently, we revealed the presence of a direct strong correlation between the Creatine phosphokinase level and the alanine aminotransferase level, which was equal to +0,86 (р<0,05) and the presence of a direct moderate correlation between the aspartate aminotransferase and the Creatine phosphokinase level which was equal to + 0.56 (р<0,05). We also found that the level of alkaline phosphatase was also significantly higher than the normal one in 69% of our patients. The patients with Duchenne muscular dystrophy had various multidirectional disorders of the immune status, impaired renal function (in particular, a decrease in serum creatinine concentration and reduced glomerular filtration rate), as well as the divergence of liver enzyme parameters (in particular, a significant increase in transaminase levels).