Клинический случай синдрома Блоха — Сульцбергера

Purpose.To present a clinical case of Bloch Sulzberger syndrome. Material and methods. The examinations were performed to diagnose the disease: а visual examination of the skin, cytological analysis of the gallbladder fluid, general and biochemical blood tests, genetic research. Results.During a visual examination of the skin, a differential diagnosis was made with infectious dermatitis, toxic-allergic dermatitis, epidermolysis bullosa and linear IgA-dependent dermatosis in children. Crucial in the diagnosis belonged to a genetic study, after which a deletion of exons 410 of the IKBKG gene was detected, which confirmed Bloch Sulzberger syndrome. Conclusion.Newborns with vesicle-bullous rashes entering the neonatal pathology department and observed by neonatologists require a thorough examination, a mandatory consultation of a dermatologist inorder to determine further management tactics.Цель.Представить клинический случай синдрома БлохаСульцбергера. Материал и методы.Для диагностики заболевания были проведены осмотр кожи, цитологический анализ пузырной жидкости, общий и биохимический анализы крови, генетическое исследование. Результаты.При осмотре кожи был проведен дифференциальный диагноз с дерматитами, буллезным эпидермолизом и линеарным IgA-зависимым дерматозом у детей. Решающее значение в постановке диагноза принадлежало генетическому исследованию, после проведения которого была выявлена делеция экзонов 410 гена IKBKG, что подтвердило синдром БлохаСульцбергера. Заключение.Новорожденные с везикуло-буллезными высыпаниями, поступающие в отделение патологии новорожденных и наблюдаемые неонатологами, требуют тщательного обследования, обязательной консультации дерматолога с целью определения дальнейшей тактики ведения

Actual problems of environmental factors risk assessment on human health and ways to improve it

The article provides an analysis of global trends and new areas of health risk assessment and analysis methodology caused by exposure to chemicals, environmental pollutants, and the contemporary issues of national assessment methodology. Most details are considered: risk assessment evidence base, modern methods and problems of carcinogenic risk assessment, hygienic regulation based on risk assessment, the economic aspects of the methodology. Particular attention is paid to reasons of recent years perceived gaps in the Russian methodological basis of the best foreign samples. The urgent measures to improve the national risk assessment methodology are proposed, the main of which are: legislative consolidation of the basic concepts of risk assessment, a further update of the methodology and the practice of hygienic regulation on the basis of risk assessment, improving the valuation of damage to human health, the tightening of the requirements to the developed regulatory guidance documents on risk assessment, as well as to the training and retraining of personnel in the risk assessment