Efficacy and safety of hydrokinesitherapy in patients with dystrophinopathy.

Duchenne muscular dystrophy (DMD) is one of the most common forms of hereditary muscular dystrophies in childhood and is characterized by steady progression and early disability. It is known that physical therapy can slow down the rate of progression of the disease. According to global recommendations, pool exercises, along with stretching, are preferable for children with DMD, as these types of activities have a balanced effect on skeletal muscles and allow simultaneous breathing exercises. The present study aimed to evaluate the effectiveness of regular pool exercises in patients with Duchenne muscular dystrophy who are capable of independent movement during 4 months of training. 28 patients with genetically confirmed Duchenne muscular dystrophy, who were aged 6.9 ± 0.2 years, were examined. A 6-min distance walking test and timed tests, namely, rising from the floor, 10-meter running, and stair climbing and descending, muscle strength of the upper and lower extremities were assessed on the baseline and during dynamic observation at 2 and 4 months. Hydrorehabilitation course lasted 4 months and was divided into two stages: preparatory and training (depend on individual functional heart reserve (IFHR)). Set of exercises included pool dynamic aerobic exercises. Quantitative muscle MRI of the pelvic girdle and thigh was performed six times: before training (further BT) and after training (further AT) during all course. According to the results of the study, a statistically significant improvement was identified in a 6-min walking test, with 462.7 ± 6.2 m on the baseline and 492.0 ± 6.4 m after 4 months (p < 0.001). The results from the timed functional tests were as follows: rising from the floor test, 4.5 ± 0.3 s on the baseline and 3.8 ± 0.2 s after 4 months (p < 0.001); 10 meter distance running test, 4.9 ± 0.1 s on the baseline and 4.3 ± 0.1 s after 4 months (p < 0.001); 4-stair climbing test, 3.7 ± 0.2 s on the baseline and 3.2 ± 0.2 s after 4 months (p < 0.001); and 4-stair descent test, 3.9 ± 0.1 s on the baseline and 3.2 ± 0.1 s after 4 months (p < 0.001). Skeletal muscle quantitative MRI was performed in the pelvis and the thighs in order to assess the impact of the procedures on the muscle structure. Muscle water T2, a biomarker of disease activity, did not show any change during the training period, suggesting the absence of deleterious effects and negative impact on disease activity. Thus, a set of dynamic aerobic exercises in water can be regarded as effective and safe for patients with DMD.

[Kisspeptins: role in the aging of the reproductive system and the development of comorbid pathology.]

The literature review is devoted to the role of kisspeptins in aging. There are data about the involvement of kisspeptins in the development of menopause and ovarian aging, as well as metabolic syndrome. In addition, the role of kisspeptins in the development of age-related diseases such as diabetes mellitus, coronary heart disease, and Alzheimer's disease is described. Involvement of kisspeptins and kisspeptin receptors in the development of malignant neoplasms are postulated. Evidence of the antimetastatic properties of the kisspeptin protein, as well as the possibility of using it as a tumor marker, is presented.

[Proton magnetic resonance spectroscopy in children with atypical autism comorbid with psychomotor disinhibition syndrome]. / Protonnaya magnitno-rezonansnaya spektroskopiya u detei s atipichnym autizmom, sochetayushchimsya s sindromom psikhomotornoi rastormozhennosti.

OBJECTIVE: To study the relationship of N-acetylaspartate (NAA) metabolism and choline (Cl) metabolism in different parts of the brain based on magnetic resonance spectrography (1H-MRS) with clinical manifestations of autism spectrum disorders (ASD). MATERIAL AND METHODS: Twenty-two children (16 boys, 6 girls), aged 2-10 years, were studied. Russian-language adapted versions of the Autism Treatment Evaluation Checklist (ATEC) and the Nisonger Child Behavior Rating Form (NCBRF) were administered. The ratio of metabolites NAA/creatine (Cr), Cho/Cr, Cho/NAA in the prefrontal cortex, postcentral gyrus and temporal lobes was studied using 1H-MRS. RESULTS: The following correlations were found: 1) between the NAA/Cr value and the Sensory/Cognitive Awareness scale in the prefrontal cortex on the left (ρ=0.479) and on the right (ρ=0.483); the Health/Physical Behavior scale in the precentral gyrus on the left (ρ=0.572) and on the right (ρ=0.463); the Sociability scale in the temporal lobe on the left (ρ=0.481) and on the right (ρ=0.796); the Speech/Language/Communication scale in the right temporal lobe (ρ=-0.552); 2) between the Cho/Cr value and the Adaptive Social scale in the postcentral gyrus on the left (ρ=-0.466) and on the right (ρ=-0.518); the Compliant/Calm scale in the prefrontal cortex on the right (ρ=0.624) and on the left (ρ=-0.541); 3) between the Cho/NAA ratio and the Speech/Language/Communication scale in the right pre-central (ρ=-0.471) and post-central gyrus (ρ=-0.507); the Self-Isolated/Ritualistic¼ scale in the left (ρ=-0.486) and right temporal lobe (ρ=-0.596). CONCLUSIONS: Thus, the predominant localization of disorders of N-acetylaspartate metabolism in communication disorders (bilaterally in the temporal lobes), cognitive, behavioral and somatic manifestations (bilaterally in the prefrontal regions) was established. Increased CI metabolism has identified deficits in interaction skills in both postcentral gyrus, and reveals bilateral differences in the effect on behavioral control in the prefrontal cortex. The results confirm the previously established numerous patterns between abnormal activation of the prefrontal cortex and neuronal dysfunction in ASD. But unlike other studies, it was possible to trace these relationships within a narrower phenotype of disorders - atypical autism comorbid with psychomotor disinhibition.

[Palivizumab: four seasons in Russia].

In 2010, the Russian Federation (RF) registered palivizumab--innovative drug, based on monoclonal antibodies for passive immunization of seasonal respiratory syncytial virus (RSV) infection in children of disease severe progress risk group, which include primarily premature infants, children with bronchopulmonary dysplasia and hemodynamically significant congenital heart disease. Currently, palivizumab is included in the list of recommended medicines and medical care standards of different countries, including Russia. In the review the results of Russian research on the progress of RSV infection, its epidemiology and immunization experience gained over the 2010-2014 period are summarized in relation to the foreign data. During the four epidemic seasons palivizumab immunization covered more than 3,200 children of severe RSV infection risk group with a progressive annual increase in the number of patients who received the drug. Geography of palivizumab immunization is also greatly expanded in our country during this time. If during the first two seasons measures of immunization were taken mainly in Moscow and St. Petersburg, at the present time, thirty one territorial entities of the Russian Federation have the experience in the drug application. Analysis of the results of RSV infection immunization (made in several regions) confirms the high clinical efficacy and palivizumab safety already demonstrated in international studies. In addition, the analysis presents the potential to improve the efficiency of the integrated RSV infection immunization programs, realizing in the establishment of high-risk child group register, adequate counseling for parents, as well as the development of the routing of patients and coordination of interaction between different health institutions during the immunization.

[Estimation of the validity of pediatric severity rating scales in multidisciplinary children's intensive care units].

The aim of this study was to estimate the validity of pediatric severity rating scales at general pediatric intensive care units. This was a prospective, observational multicenter study that was performed from November 1, 2007, to January 31, 2008. The end points were the estimation of the severity of disease, by using the PRISM, PIM II, RELOD scores, and 28-day survival. The investigation covered 226 patients whose mean age was 4.1 +/- 0.3 years. Twelve (5.3%) patients died during the study. The Medcalc computer program was employed for statistical analysis. The area under the receiver operating curve (ROC) was 0.623 +/- 0.116 for PRISM (p = 0.289); 0641 +/- 0.116 for PELOD (p = 0.224); 0.833 +/- 0.096 for PIM II (p < 0.001). The calibration of scores according to the intervals of mortality risk (Hosmer-Lemeshov test) showed that the observed and predicted mortality rates were similar for PIM II scores (chi2 = 8.23; p = 0.084) and were different for PRISM scores (chi2 = 203.5; p < 0.001) and PELOD ones (chi2 = 26.16; p < 0.001). Thus, the PIM II scores showed the best validity. PRISM and PELOD can be used to estimate the severity of disease in individual subgroups of high mortality-risk patients.

[Cerebral hemodymanics as a criterion for the severity of neonatal posthypoxia and efficiency of its treatment].

Twenty-five full-term neonates with severe posthypoxic syndrome, including 9 (36%) with prior acute moderate asphyxia, 8 (32%) with acute asphyxia, and 8 (32%) with associated acute asphyxia, were examined. The severity of posthypoxia and the efficiency of its treatment were evaluated, by studying cerebral and systemic hemodynamics. In children with moderate and severe asphyxia, artificial ventilation contributed to normalization of acid-base balance and infusion of dopamine in a dose of up to 5 microg/kg x min could achieve adequate cardiac output. In children with associated severe asphyxia, infusion of dopamine in a dose of 5.5-10 microg/kg x min, which failed to improve cerebral perfusion pressure, was required to maintain the optimal cardiac output. This situation requires additional cerebral protective therapy aimed at lowering intracranial pressure.