MONITORING OF RENAL, HEPATIC AND IMMUNE FUNCTION INDICES IN PATIENTS WITH NEUROMUSCULAR DISORDERS: AMYOTROPHIC LATERAL SCLEROSIS AND DUCHENNE MUSCULAR DYSTROPHY.

The purpose of the study is to examine in depth and analyze renal, hepatic and immune function indices in patients who suffered from neuromuscular disorders: аmyotrophic lateral sclerosis and Duchenne muscular dystrophy (one-year prospective follow-up). We analyzed the follow up clinical and laboratory data of 55 patients, aged 56±10, with amyotrophic lateral sclerosis and 53 patients, aged 18±5, with Duchenne muscular dystrophy at presentation and a year after the treatment. The patients underwent a standardized examination, involving studying the medical case history, general clinical data, the detailed neurological status examination, 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 serum urea levels, glomerular filtration rate (GFR), the lactic dehydrogenase level, the alkaline phosphatase level, 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. The instrumental examination included the ultrasound of the abdominal organs, muscles, as well as echo-cardiography, electroneuromyography. Having analyzed the renal, hepatic and immunological indices in patients with such neuromuscular disorders as аmyotrophic lateral sclerosis and Duchenne muscular dystrophy, we found that both the patients with аmyotrophic lateral sclerosis and those with Duchenne muscular dystrophy suffered from renal dysfunction. Thus, most patients with аmyotrophic lateral sclerosis (69%) were observed to have the below normal serum creatinine level and 5,5% of the patients had the above normal serum creatinine level. A similar situation was observed in patients with Duchenne muscular dystrophy: 58.5% of the patients were detected to have the below normal serum creatinine level and 3.8% of the patients had the above normal serum creatinine level. At the same time, 40% of 25 patients with Duchenne muscular dystrophy, who we followed up, had low serum creatinine at presentation, while a year later, the%age of the patients increased to 44%. The serum urea level was within normal limits in most patients (89,1%) with аmyotrophic lateral sclerosis and in all the patients with Duchenne muscular dystrophy. The glomerular filtration rate was observed to be within the reference value in most patients with аmyotrophic lateral sclerosis and in all the patients with Duchenne muscular dystrophy (at presentation and a year after the treatment), however, it was observed a mild renal filtration dysfunction in 21,8% of patients with аmyotrophic lateral sclerosis; it was found a moderate renal filtration dysfunction in 5,5% of patients with аmyotrophic lateral sclerosis. At that we detected a weak positive correlation between the glomerular filtration rate and the lifespan of patients with аmyotrophic lateral sclerosis (r=0.28, p<0.01). Concurrently, we found no correlation between the serum creatinine and myoparesis severity in patients with аmyotrophic lateral sclerosis that somewhat contradicts Inês Martins' findings obtained in 2020. The serum aspartate aminotransferase level was found to be above normal in 40% of patients with amyotrophic lateral sclerosis (at the same time the alanine aminotransferase level was observed to be above normal only in 18% of the patients). The lactic dehydrogenase level was below normal in most patients. We found the significant changes when evaluating the immune status in patients with amyotrophic lateral sclerosis: almost all the patients (94.5%) had decreased levels of B-lymphocytes; 41.8% of patients had a reduced level of a subpopulation of T-lymphocytes - T-suppressors. The conducted comprehensive longitudinal study has shown the presence of significant predictive renal function impairments in patients with amyotrophic lateral sclerosis and Duchenne muscular dystrophy. The patients with amyotrophic lateral sclerosis were detected to have significantly deviated B-cell and T-cell components of the immune status and enzyme systems, including lactate dehydrogenase and aspartate aminotransferase levels; their nature and prognostic value need further investigation.