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The aim: Among the adverse psychological changes associated with medical work, a significant place belongs to the emotional burnout syndrome, which significantly affects a doctor’s social functioning. The purpose of the study was to determine the behavioural activity types and to study the muscular sensitivity threshold of family doctors with the formed emotional burnout syndrome. Materials and methods: We examined 83 female family doctors with diagnosed emotional burnout syndrome. The treatment was carried out with the help of the Taiji-Quan eastern wellness system. Results: The analysis of indicators of the behavioural activity revealed before treatment in 72,5% of women in the intervention group and in 69,8% of women in the control group (p>0,05) signs of the high probability of type A behaviour. After a three-month course of treatment, the percentage of manifestations of type A behaviour in women in the intervention group decreased from 72,5% to 40,0% (p 0,05). When examining the features of muscular tone at the beginning of treatment, it was found that 69,9% of women in the intervention group and 65,1% of the control group had a high degree of muscle blocks (p>0,05). After treatment, the percentage of women with a high degree of muscle blocks decreased to 37,5% (p 0,05) in the control group. Conclusions: The taiji-Quan treatment made it possible to significantly reduce type A behaviour and to decrease the amount of “muscle blocks”.

Background. Wilson’s disease (WD) is a rare inherited disorder caused by mutations in the ATP7B gene resulting in copper accumulation in different organs. However, data on ATP7B mutation spectrum in Russia and worldwide are insufficient and contradictory. The objective of the present study was estimation of the frequency of ATP7B gene mutations in the Russian population of WD patients. Materials and methods. 75 WDpatients were examined by next-generation sequencing (NGS). A targeted panel NimbleGen SeqCap EZ Choice: 151012_HG38_CysFib_EZ_HX3 (ROCHE)was designed for analysis of ATP7B gene and possible modifier genes. Retrospective assessment of a diagnostic WD score (Leipzig, 2001) was also performed. Results. 31 mutations in ATP7B gene were detected. Two most frequent mutations were c.3207C > A (51,85% of alleles) and c.3190 G > A (8,64% of alleles). Single rare mutations were detected in 29% of cases. In 96% cases mutations of both copies of the ATP7B were revealed. We also observed 3 novel potentially pathogenic variants which were not previously described (c.1870-8A > G, c.3655A > T (p.Ile1219Phe), c.3036dupC (p.Lys1013fs). For 25% of patients at the time of the manifestation the diagnosis WD could not be established using the earlier proposed diagnostic score. There was a remarkable delay in diagnosis for the majority of patients. Only 33% of patients WD was diagnosed in three months after the first symptoms, 29%patients - in 3–12 months, 30% – in 1–10 years, in 8% – more than 10 years. Generally, clinical appearance of WD may be rather variable at manifestation and genetic profiling at this step is the only way to confirm the presence of WD.

The results of investigation of the inflammatory processes dynamics in operative wounds in 238 patients after surgical intervention, performed for noncomplicated hernias of anterior abdominal wall of various localization, as well as the impact of polarized light on correction of the vegetative nervous system disorders, for prophylaxis of infiltrative-purulent complications were analyzed.

Rates of Down syndrome (DS) show considerable international variation, but a systematic assessment of this variation is lacking. The goal of this study was to develop and test a method to assess the validity of DS rates in surveillance programs, as an indicator of quality of ascertainment. The proposed method compares the observed number of cases with DS (livebirths plus elective pregnancy terminations, adjusted for spontaneous fetal losses that would have occurred if the pregnancy had been allowed to continue) in each single year of maternal age, with the expected number of cases based on the best-published data on rates by year of maternal age. To test this method we used data from birth years 2000 to 2005 from 32 surveillance programs of the International Clearinghouse for Birth Defects Surveillance and Research. We computed the adjusted observed versus expected ratio (aOE) of DS birth prevalence among women 25-44 years old. The aOE ratio was close to unity in 13 programs (the 95% confidence interval included 1), above 1 in 2 programs and below 1 in 18 programs (P

A polymorphic CAG repeat was identified in the human alpha 1A voltage-dependent calcium channel subunit. To test the hypothesis that expansion of this CAG repeat could be the cause of an inherited progressive ataxia, we genotyped a large number of unrelated controls and ataxia patients. Eight unrelated patients with late onset ataxia had alleles with larger repeat numbers (21-27) compared to the number of repeats (4-16) in 475 non-ataxia individuals. Analysis of the repeat length in families of the affected individuals revealed that the expansion segregated with the phenotype in every patient. We identified six isoforms of the human alpha 1A calcium channel subunit. The CAG repeat is within the open reading frame and is predicted to encode glutamine in three of the isoforms. We conclude that a small polyglutamine expansion in the human alpha 1A calcium channel is most likely the cause of a newly classified autosomal dominant spinocerebellar ataxia, SCA6.

110 patients (52 men, 58 women) in a state of acute intoxication were examined in emergency department of psychoneurologic hospital. Neuroleptics of different groups were used to commit a suicide (91.3% of the cases), to obtain a toxicomanic effect (6.3%) and accidentally (1.8%). The patients suffered from borderline mental disorders (39.2%), schizophrenia (40.9%), manic-depressive psychosis in depressive phase (10.9%), chronic alcoholism (4.5%) and organic damages of CNS (4.5%). The patients with borderline states used various drugs and had more light disorders of consciousness (deafness). Meanwhile, the patients with endogenic mental disorders used strong neuroleptics, as a rule, that resulted in coma. In residual period there were different syndromes from asthenic to psychoorganic ones. They were more severe after poisoning with aminazinum, haloperidol, leponex.

Background Postoperative morbidity after reversal of Hartmann's procedure remains high. Aim to evaluate efficacy of laparoscopic-assisted approach. Patients and methods 36 patients (19 men, aged 55.71 +/- 1.5 years) underwent laparoscopic-assisted reversal procedures in May 2008 - June 2010. The comparable control group consisted of 35 patients (16 men, aged 51.5 +/- 13.9 years). Results operation time was 179.5 +/- 65.1 min, 266.9 +/- 71.8 min in controls. Blood loss was 64.7 +/- 33.7 ml, 181.8 +/- 120.4 ml in controls. No conversions occurred in the main group. In three patients of the main group preventive ileostomy was performed. There were 11 diverting stomas in the control group. Postoperative hospital stays were 9.1 +/- 2.7 days (12.9 +/- 3.4 days in controls). There were 2 (5.9%) postoperative complications in the main group: one wound infection and one parastomal fistula. No mortality occurred. In the control group 3 (9.1%) complications (wound infection and haematoma) were detected. Conclusion laparoscopic-assisted reversal of Hartmann's procedure promotes faster rehabilitation, its results are not worse than after open approach.

531 patients with non-complicated and non-metastatic colon cancer were operated on during 1995-2008 years. Surgery was radical in all cases. Various types of surgical treatment of the colon cancer, postoperative complications and lethality were analyzed. Open methods reported a 1,6% complications rate and lethality rate was 0,4%. Whereas, laparoscopically operated patients showed the complication rate of 3,6% and lethality was 1,2%. Overall 5-year survival was 91,7% for T1-3N0M0; 75,6% for T4N0M0 and 55,3% for T1-4N1-2M0 stages.

Investigation of the vegetative nervous system (VNS) state according to ratio of the sympathetic and parasympathetic parts activity in patients with acute appendicitis, strangulated hernia, perforative ulcer was conducted. There were revealed significant VNS tone disorders before and after operation with signs of sympathicotony or parasimpathicotony, which needed to be corrected in complex of treatment of the patients.

Seven hundreds and fifty three patients with colon cancer were operated in the Unit of Colon Surgery of the State Scientific Centre of Coloproctology within the period from 1998 to 2003. 352 (46.7%) of them were subjected to systemic chemotherapy. The patients subjected to the surgical and antitumor drug therapy were divided into 3 groups according to the main disease stage: 103 (29.3%) patients with the disease stage T4N0M0 (including those with the tumor local spread), 116 (32.9%) patients with the disease stage T(3-4)N+M0 and 133 (37.8%) patients with the disease stage T(3-4)NxM1. In the patients with the colon serous membrane diffusion and locally spread tumors (total of 103 patients) 3- and 5-year survival was stated in 81.0 +/- 2.8% and 76.5 +/- 3.7% respectively. The control group included 85 patients subjected to the surgical treatment alone. In the latter group 5-year survival was recorded in 68.0 +/- 2.9% of the patients. In the group of the patients with affection of the regional lymph nodes subjected to antitumor drug therapy 3- and 5-year survival was recorded in 64.1 +/- 3.5% and 57.5 +/- 4.2% respectively. In 52 patients not subjected to the systemic chemotherapy the analogous index was equal to 44.5 +/- 3.4%. The systemic chemotherapy was applied to 111 (78.2%) patients with metastases to the liver. In 50 of them cytoreductive operations were performed and in 61 patients palliative resection of the colon was carried out. The average period without clinical manifestations of the disease in the patients subjected to the colon palliative resection and systemic chemotherapy was equal to 8.5 +/- 3.5 months. In the patients with metastases to the liver subjected to the cytoreductive operations the average lifespan after the operations amounted to 24.5 months. The average lifespan of the patients with canceromatosis subjected to the systemic chemotherapy and cytoreductive operations amounted to 13.5 months. When the chemotherapy in such patients was effective the average lifespan was 16 months, while in case of the therapy failure it was 8.5 months.

One hundred and thirty six women with genital tuberculosis received combined antibacterial treatment (ABT) with adjuvant physiotherapy. The patients were divided into three groups: patients of group 1 had active genital tuberculosis treated with ABT plus electrophoresis of 3% sodium thiosulphate, group 2 had chronic tuberculous process treated with ABT plus ultrasound hydrocortisone therapy, group 3 received ABT alone. The findings demonstrate that noticeable improvement and reduction of treatment duration were observed in groups 1 and 2.

146 patients with multiple sclerosis are treated by copaxone in the Moscow MS Centre. The relaps exacerbation rate decreased by 72%, the relapse rate reduced by 25.6% during the copaxone treatment. EDSS score did not increase both in group with and without relapses. The condition of 2/3 patients was stable. To achieve the best effect of treatment the program of patient support was successfully used in the Moscow MS Centre.

The efficacy of cytoreductive approach for colon cancer with carcinomatosis was assessed. 34 patients (the main group) underwent colon resections, peritonectomy, omentectomy and intraperitoneal chemotherapy (mitomycin and 5-fluorouracil). 22 patients (control group) underwent palliative colon resections only. The spread of peritoneal dissemination was assessed in all patients, basing on Peritoneal Cancer Index (PCI). It was found, that the rate of postoperative morbidity is higher in the main group, but it was not accompanied by enhanced postoperative mortality. It was also detected, that cytoreductive approach permits to prolong the survival (17.0 +/- 3.5 months in main group vs. 7.5 +/- 2.1 months in controls). The main prognostic factor in these patients was the PCI. The most favorable prognosis is identified if PCI5 (few discrete implants are present); the survival in this group was 23.0 +/- 4.5 months and disease-free interval was 14.0 +/- 2.5 months. When PCI was higher then 5, the survival was 14.5 +/- 2.5 months and disease-free interval was 7.0 +/- 1.3 months.

Results of the 2-year open postregistration study of Copaxone (glatiramer acetate), conducted in 3 Moscow leading medical centres are given. 32 MS patients with remitting-relapsing MS were investigated in accordance with international requirements (neurological scales, MRI of the brain, multimodal evoked potential). Significant reduction of annual relapse rate for 70.3%, stabilization of neurological status and benign safety profile were demonstrated during Copaxone treatment. Practical recommendations for Copaxone use are given.

The paper presents the specific features of diagnosis and clinical course of genital tuberculosis under the present conditions. It shows it expedient to make a differential diagnosis of genital tuberculosis in 2 steps: 1) the use of the routine examination and 2) the study of specific antituberculous immunity. Genital tuberculosis risk groups to be obligatorily examined at a specialized tuberculosis control facility are identified. The specific features of the clinical course of genital tuberculosis, such as aptness to exudative processes, a combination with extragenital tuberculosis and hormonally depended gynecological diseases are noted. Indications for and efficiency of endosurgical interventions in genital tuberculosis are shown.

One of the largest environmental polluters in Chapaevsk (Samara Region, Russia) is the Middle Volga chemical plant. From 1967 to 1987, it produced hexachlorocyclohexane (lindane) and its derivatives. Currently, it produces crop protection chemicals (liquid chlorine acids, methyl chloroform, vinyl chloride, and some other chemicals). Dioxins were detected in air (0.116 pg/m3), in soil (8.9-298 ng/kg), in the town's drinking water (28.4-74.1 pg/liter), and in the cow's milk (the content of 2,3,7,8-TCDD was 17.32 pg TEQ/g fat). The mean content of dioxins in seven pooled samples of human milk (40 individual trials) was 42.26 pg TEQ/g fat, in four female workers' blood samples -412.4 pg TEQ/g fat, in six residents blood samples (those who lived 1-3 km from the chemical plant) -75.2 pg TEQ/g fat, in four residents' blood samples (5-8 km from the plant) -24.5 pg TEQ/g fat. To assess cancer risk and reproductive health status, official medical statistical information was used. In general, the male cancer mortality observed rate in Chapaevsk is higher than expected. The SMR is higher for lung cancer 3.1(C.I. 2.6-3.8), urinary organs 2.6(C.I. 1.7-3.6). Chapaevsk women have a higher risk overall due to breast cancer 2.1(C.I. 1.6-2.7) and cervix cancer 1.8(C.I. 1.0-3.1). The incidence rates were higher for lung cancer in males and for female breast cancer in all age groups compared to Russia and Samara Region in 1998. Significant disruptions in reproductive function were detected. The mean frequency of spontaneous abortions in the last seven years was statistically higher 24.4% in Chapaevsk (compared to other of the towns region). The average rate of premature labor was 45.7 per 1000 women in Chapaevsk that is significantly higher than in most Samara Region towns. The frequency of newborns with low birth weight was 7.4%. In Russia and in most of the Samara Region towns, this rate is lower (6.2-5.1%) but not statistically different. For the determination of congenital morphogenetic conditions (CMGC), 369 children born between 1990 and 1995 were examined. The average number of CMGC per child was significantly higher, 4.5 for boys and 4.4 for girls. The first results indicated serious disruptions associated with high dioxin levels in human milk and blood in Chapaevsk. We suggest that Chapaevsk is an incredibly interesting site for further environmental-epidemiological research to assess the impact of dioxins on human health.

Reciprocal probing has been used to identify a cDNA clone (xh8H11) representing a gene preferentially expressed in striated muscle. The gene maps close to DXS7101 31.9 cM from the short arm telomere of the X-chromosome at Xp22.1. On searching expressed and genomic databases, 21 expressed sequence tags were found that allowed the assignment of a human extended consensus sequence of 887 bp, suggesting a completely expressed gene symbolized as SMPX. By using the human consensus sequence, the orthologous mouse Smpx and rat SMPX genes could be aligned and confirmed by complete sequencing of additional SMPX-related clones obtained by library screening. An open reading frame was identified encoding a peptide of 88-86 and 85 amino acids in human and rodents, respectively. The predicted peptide had no significant homologies to known structural elements. The human consensus cDNA sequence was used to define the genomic structure of the human SMPX that had been missed by a previous large scale sequencing approach. The gene consists of five exons (or =172, 57, 84, 148,or =422 bp) and four introns (3639, 10410, 6052, 31134 bp) comprising together 52.1 kb and is preferentially and abundantly expressed in heart and skeletal muscle. Thus, a novel human gene encoding a small muscular protein that maps to Xp22.1 (SMPX) has been identified and structurally characterized as a basis for further functional analysis.

The analysis of original and literature evidence on nimodipin administration in senile patients with vascular dementia and Alzheimer's disease allows the conclusion on positive effects of the drug on cognitive, motor, behavioral, neuropsychological and postural defects. Further efforts should be directed to the study of long-term regimens and elucidation of nimodipin ability to inhibit progression of dementia.

Comparison of the official and research data on congenital developmental defects frequency (CDDF) in children of Novomoskovsk showed their disagreement. The average CDDF in these children is not more than the Middle European level. Physical development of children is insufficient for their biological age in 25% of cases. Many children suffer from respiratory diseases because of ambient air pollution. The majority of children (88-93%) are referred to 2-4 th health groups according to WHO classification.

Combined treatment with the use of intensive methods of preoperative radiotherapy was carried out in 41 patients with malignant tumors of the colon. Study of the specific features of the accomplished surgical interventions and the postoperative management of patients showed that short courses of irradiation on a betatron with energy of 25 MeV, given in 5 daily exposures to a total basic dose of 20 Gy or a single exposure to a basic dose of 7.5 Gy had no unfavourable effect on the performance of the operation and the patients' condition in the postoperative period. The immediate results of the operation were found to be good in all patients.

The study discusses the experience of treatment of 207 patients with locally-advanced cancer of the sigmoid colon who made up 13.5% of the total number of cases of cancer of this site seen in the clinic in 1972-1990. Radical surgery was performed in 170 (82.1%) patients. Postoperative lethality rate was 5.8%, five-year survival rate--70.5 +/- 5.6%. The use of adjuvant preoperative radiotherapy assured a higher resection rate and had no adverse effect on immediate results.

The study discusses the experience gained with application of a complex of methods for early diagnosis of perifocal inflammation in 50 patients with cancer of the colon. Endoscopic, roentgenologic and radionuclide (using 67Ga citrate) methods were mainly employed. Perifocal inflammation was established in 38 (76%) patients. The diagnosis was further confirmed by morphologic examination of resected material. Application of the methods assured early diagnosis of asymptomatic perifocal inflammation associated with tumor.

Carcinoma of the right half of the colon (CRHC) is diagnosed in 21-35% of patients with colorectal carcinomas. The Research Institute of Proctology of the RSFSR Ministry of Health has an experience with treatment of 338 patients with CRHC. Most of them (84.9%) were admitted to the clinic with advanced forms of the disease because of poor clinical manifestations at early stages of the disease and resulting in late consultations with the doctor. Surgical treatment was performed in 332 patients. The operation of choice for locally removable tumors of the right half of the colon still remains right-side hemicolectomy. For locally spread CRHC combined operations are thought to be expedient. Immediate results of the surgical treatment of CRHC by the correct curative methods are perfectly favorable, the amount of postoperative complications was 14.5%, lethality--6.6%.

Leucine aminopeptidase of myelin was discovered to contain a protein subunit possessing, in contrast to the main enzymatic mass, antigenic properties and contained by different structures of the central nervous system. The protein was shown to be accumulated by neurons and astrocytes of embryonic tissue culture. The protein was absent from biological fluids of healthy persons and in schizophrenia but it was recordable in the blood and cerebrospinal fluid of patients with different forms of neurological pathology. In patients with neuronal degenerative diseases, the protein level in biological fluids in the terminal stages was significantly lower than in compensated patients.

Surgical treatment of chronic colonic stasis is still a complicated and difficult for solution problem. The undertaken study characterizes one of the causes of chronic constipation in adults--the hypoganglionic variant of Hirschsprung's disease whose clinical course is marked by a prolonged period of compensation while the complex diagnosis is extremely difficult. The severe disorders of the motor-evacuation function of the large intestine make surgery necessary; operation leads to recovery of 96% of patients.

Among 100 patients subjected to a complex examination 44 were found to have Hirschsprung's disease. Operation was fulfilled in 37 patients. Resection of dilated portions of the colon can not be considered to be a radical intervention. With the morphological features of Hirschsprung's disease in adults taken into account, Duhamel's operation is recommended as a readical operation. In patients with a pronounced decompensation of the colon accompanied by ileus the treatment should consist of many steps. In some cases the conservative treatment might be performed under conditions of dispensary observation of the patient.