New view on education modernization

This article presents a model for the modernization of education. The transition to new technologies requires a change in pedagogical thinking and the creation of a new educational culture.В данной статье представлена модель модернизации образования. Переход на новые технологии требует изменения педагогического мышления и создания новой образовательной культуры

Rare cases of mucopolysaccharidosis type I in children with Hurler and Hurler-Scheie syndromes

Two rare cases of mucopolysaccharidosis (MPS) I in children are presented. In the first case, the ophthalmologist suspected a Hurler syndrome in a child aged 1 year with corneal opacities in both eyes, taking into account the unusual appearance of the child, which gradually formed over the year after birth, retarded psychic and speech development and the presence of hypoxic ischemic central nervous system injury (CNS) with hypertensive syndrome, hepatomegalia, and mitral valve prolapse. The diagnosis was confirmed by the results of a biochemical blood test, which showed a decrease in the activity of the enzyme of alpha-L-iduronidase. Genetic testing was recommended for the determination of the proband genotype. The second case was a boy S., aged 3 years and 7 months, with Hurler - Scheie syndrome. Numerous comorbidities (CNS, hypertensive syndrome, hip dysplasia, kyphosis, ambilateral inguinal hernia, etc.) combined with gradual formation of grotesque facial features by the age of eighteen months suggested that genetic testing should be taken during the second year of life. As a result, Q 70X mutation was identified in the heterozygous state and replacement therapy with Aldurazyme was prescribed. Clinical polymorphism and different severity of symptoms combined with rare occurrence was the reason why difficulties were experienced in the early identification of MPS I. Early diagnosis is very important so that the children could be timely referred to experts of the interdisciplinary center with an experience in the specific treatment, which is the most effective in the early stages of the disease. For citation: Babushkin A.E., Khusnutdinova E.G., Ryskulova E.K., Mukhametshina R.M. Rare cases of mucopolysaccharidosis type I in children with Hurler and Hurler - Scheie syndromes. Russian ophthalmological journal. 2018; 11 (1): 53-8. doi: 10.21516/2072-0076-2018-11-1-53-58 (In Russian)

Visual impairment and eye care among Alaska native people

Purpose: To estimate the prevalence of visual impairment, eye disease and eye care in the Alaska Native (AN) population, by demographic and socioeconomic factors. Methods: Population-based cross-sectional study of 3,793 AN adults aged 18&ndash;94 years enrolled in the Education and Research Towards Health (EARTH) Study from March 2004&ndash;March 2006. Data on self-reported visual impairment, cataract, glaucoma, diabetic eye disease and previous dilated eye examinations were collected using audio computer-assisted self-administered questionnaires. Results: The unadjusted prevalence of self-reported visual impairment was 8.7% (95% confidence interval (CI): 7.9&ndash;9.7), cataract 5.9% (95% CI: 5.2&ndash;6.7), glaucoma 2.5% (95% CI: 2.0&ndash;3.0) and diabetic eye disease 1.3% (95% CI: 0.9&ndash;1.7). In all cases, age-sex adjusted prevalence estimates for the AN population were greater than available estimates for the general U.S. population. Prevalence of visual impairment and each eye disease increased with age (P &lt; 0.01). Additional factors associated with visual impairment were education and annual household income. Overall, 70.0% (95% CI: 68.5&ndash;71.6) of participants reported a dilated eye examination within the previous two years. Dilated eye examination within the previous two years was associated with increasing age (P &lt; 0.001). However, men and participants with lower formal education were less likely to report recent dilated eye examination. Among those with diabetes, only 67.7% (95% CI: 60.8&ndash;74.1) reported a dilated eye examination within the recommended previous one year. Conclusions: Self-reported visual impairment, cataract, glaucoma and diabetic eye disease are prevalent in the AN population. These data may be useful in healthcare planning and education programs.<br /