ADVANCED THERAPY IN PATIENTS WITH PRIMARY AND POSTPOLYCYTHEMIC MYELOFIBROSIS

Ph-negative myeloproliferative neoplasm are the group of hematologic disorders which includes primary myelofibrosis, polycythemia vera, essential trombocytemia and several rare diseases. After the discovery of V617 Fgain-of-function mutation the new period of diagnostics, treatment and evaluating of MPN prognosis began. At the current moment several molecules inhibiting JAK2 function are developed. Advanced therapy in patients with primary and post-polycythemic myelofibrosis included molecules inhibiting JAK2 function resulted in rapid and durable improvements in splenomegaly and disease-related symptoms in the phase 3 trials COMFORT-I and COMFORT-II. The effectiveness of the advanced therapy included molecule inhibiting JAK2 was evaluated in three patients with primary myelofibrosis and post-polycitemic myelofibrosis. All represented clinical cases demonstrated positive dynamics of the disease manifested in spleen size reduction, improvement of the symptoms and in one case in reduction of blood transfusions. None of three patients met serious adverse events leading to dose reduction or discontinuation of the molecule inhibiting JAK2. Target agents therapy demonstrated high treatment rates in patients with primary and post-polycythemic myelofibrosis. Thus, it is clearly necessary to perform molecular diagnosis, screening tests at early stages of the chronic myeloproliferative disease for the selection of patients in need for specific treatment

Особенности состояния мягких тканей на вершине деформации у больных кифосколиозом на фоне нейрофиброматоза 1-го типа

Objective. Evaluation of skin sensitivity and analysis of morphological changes in paravertebral muscles and back skin in kyphoscoliotic deformity projection in patients with type 1 neurofibromatosis (NF-1).Materials and methods. Ten NF-1 patients who underwent surgery to treat kyphoscoliosis were examined. Using an electrical esthesiometer thermal pain sensitivity before the surgery was studied in dermatomes corresponding to the apex of the deformity. Skin and muscle biopsy samples were collected intraoperatively in the projection of the apex of the deformity curve and were subsequently analyzed by light and scanning electron microscopy.Results. Patients with kyphoscoliosis with underlying NF-1 were characterized by abnormal thermal pain sensitivity, pathological structural changes in skin and muscles accompanied by disrupted innervation and blood supply.Discussion. The observed changes may be responsible for lowered postoperative reparative potential of tissues and they must be considered in prevention and prognosis of treatment and rehabilitation efficacy.Цель работы. Оценка кожной чувствительности и анализ морфологических изменений паравертебральных мышц и кожи спины в проекции вершины кифосколиотической деформации у пациентов с нейрофиброматозом 1-го типа (НФ-1).Материалы и методы. Обследованы 10 больных НФ-1, которым проводилось хирургическое лечение по поводу кифосколиоза. Температурно-болевую чувствительность до операции исследовали с использованием электрического эстезиометра в дерматомах, соответствующих вершине деформации. Биоптаты кожи и мышцы забирали интраоперационно в проекции вершины дуги деформации позвоночника с последующим гистологическим анализом с помощью световой и сканирующей электронной микроскопии.Результаты. У больных кифосколиозом на фоне НФ-1 были выявлены нарушения температурно-болевой чувствительности, патологические структурные изменения кожи и мышц, нарушения их полноценной иннервации и кровоснабжения.Обсуждение. Обнаруженные изменения могут обусловливать снижение репаративного постоперационного потенциала тканей, что необходимо учитывать в профилактике и прогнозе эффективности лечения и реабилитации

Russian-Speaking Population in Far-Abroad Countries

Attracting compatriots living abroad is strategically vital in the context of continuing depopulation in Russia. However, a multilayered definition of the category of a compatriot creates blurred boundaries. As a result, it is somewhat problematic to assess the number of Russian compatriots living abroad objectively. The paper presents the results of a study of the socio-demographic structure of the Russian-speaking population in far-abroad countries. The statistical data of Rosstat, the UN, Eurostat, OECD, and national statistical services of foreign countries were analyzed to assess the number of Russian-speaking populations and determine the main emigration channels and geography of resettlement. Within the study, the authors have conducted an expert survey of the Ministry of Foreign Affairs of the Russian Federation, the Embassies of the Russian Federation, and representative offices of Rossotrudnichestvo in foreign countries. The survey results indicate the heterogeneous structure of Russian-speaking communities by reasons of emigration, socio-economic status, degree of integration into the host society, gender, and ethnic composition, and geography of resettlement. However, state policy analysis towards compatriots shows that it targets people who already demonstrate an interest in Russia, participate in Russian-speaking organizations, and get involved in cultural, religious, and sports events. There is a need for cooperation and interaction with Russian-speaking people. Moreover, given that young people adapt and get integrated more efficiently, it is essential to prevent the loss of cultural capital of Russian-speaking youth living abroad. It is necessary to develop and implement a more differentiated approach towards interaction with the Russian-speaking population