[THE ANALYSIS OF LIFE SPAN AND MORTALITY OF PATIENTS WITH SPINOCEREBELLAR ATAXIA TYPE I].

The article presents results of investigation of certain unclear aspects of mortality of patients with spinocerebellar ataxia type I including patients with the same number of CAG-repetitions. The analysis of mortality of patients observed from 1993 to nowadays was implemented. Sampling included 112 patients during that period 53 patients died. The comparative analysis was implemented concerning received data and results of analysis of mortality of patients died prior to 1980. According received data, average value of CAG-repetitions of normal allele was equal to 30.2, and ofpathologic allele--48.7. The average life span made up to 52.8 years, average age of disease onset--38 years and natural duration of disease--14.8 years. The analysis of life span of patients with equal length of repetitions demonstrated that range of life span of patients makes up to from 8 to 23 years. It is established that life of patients becomes shorter because of accidents, cancer and concomitant diseases of cardiovascular system. The presence of such concomitant disease as tuberculosis of lungs results in no shortening of life of patients. The comparative analysis of mortality during the period over 34 years demonstrated that age of disease onset turned out to be more conservative and stable indicator of morbidity. Despite of lacking of effective methods of treatment of disease, the natural duration of disease increased statistically reliable up to 1.8 times during period of observation. The analysis of life span ofpatients with spinocerebellar ataxia type I demonstrated that their life span except length of CAG-expansion depends on a number of factors accelerating and retarding development of disease. At that, life span of patients with the same number of CAG-repetitions can significantly differ The malignant neoplasms, diseases of cardiovascular system and external causes are to be referred to factors accelerating and retarding development of main disease. The addition oftuberculosis in our case resulted in no alteration of natural course of disease. The other factors exist prolonging life of patients, including factors of social economic and medical character They require additional specification and thorough investigation with the purpose of developing methods ofpreventive correction of neuro-degeneration processes.

REGIONAL TRENDS IN THE WORKING-AGE POPULATION MORTALITY RATE IN THE REPUBLIC OF SAKHA (YAKUTIA) IN 1990-2012.

Regions of the Russian Federation differ in climatic-geographic, medical-demographic and social-economic situations. One of the regions with distinct peculiarities is the Republic of Sakha (Yakutia). Ranking first by the territory (3,103.2 thousand sq x km), Yakutia is on the 81th place by the population density among regions of the Russian Federation (0.3 people per 1 km2).Yakutia is one of the most isolated and inaccessible regions of the world: 90% of the territory lacks all-the-year-round transportation. Regions of the republic, as well, differ significantly in the climatic conditions and the levels of social-economic development, which influences the population health indicators, including mortality. This survey aimed to study the trends of mortality in the working-age population in different groups of regions. To do this, basing on the statistical data, we compared the levels, trends and structure of mortality in 1990-2012. It was established that the different groups of regions show a significant variation in the working-age population mortality, depending on the social-economic conditions. Since 2000, the Arctic group of regions has demonstrated higher mortality in working-age men and women, especially of cardiovascular and digestive system diseases, and external causes. Lying beyond the Arctic Circle, these regions have severe conditions and a relatively low level of social-economic development. As for the rural regions, despite the relatively favourabe situation, they also show a high level of mortality of external causes. The industrial regions are characterized by higher social-economic development, better transport infrastructure, a satisfactory material base of medical institutions. They also have sufficient resources of health institutions, including the staff and modern equipment for treatment and diagnostics, as well as, which is critical, the full range of medical specialists. Thus, these regions demonstrate lower population mortality; however, there is still mortality of infectious diseases, neoplasms, and respiratory diseases.