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Toxic epidermal necrolysis (TEN) is a severe drug allergy. The objective was to analyze the clinical experience of 17 children of different age with toxic epidermal necrolysis. The authors found that medications against infection diseases were the most common reason for the development of the disease. In some cases we observed the delayed diagnosis, therapy without consideration of causative agents, and wrong doses of corticosteroids. The majority of cases require combined use of corticosteroids and intravenous immunoglobulins, however bacterial complications are possible in some cases.

The article presents an overview of the development of hematology service in the Republic of Tatarstan. The well-known scientist Nikolay Konstantinovich Goryaev (1875-1943), who worked in Kazan for a long time, began to develop this direction and after passing an internship in Germany proposed an improved device for calculating the blood elements known throughout the world. Adherents of Professor Goryaev continued research in the field of hematology, a blood transfusion station was organized. Professor S.I. Sherman proposed new methods of diagnosis and treatment of B12 deficiency anemia. Professor Sh.I. Ratner studied the changes in the blood picture in diseases of the abdominal cavity. The first 15 specialized hematological beds were opened in 1968 in the hospital named “Old Clinic”. The physician who treated such patients was Rakhil Sholomovna Dashevskaya, PhD. At present, hematology service is provided by three hospitals in Kazan, hematological and therapeutical beds in Naberezhnye Chelny and Nizhnekamsk, outpatient hematology service in Zelenodolsk. In recent years, the introduction of stem cell therapy has begun, and modern combined methods of chemotherapy have been introduced.

To develop and validate a Russian version of The Central Sensitization Inventory (CSI-R).The study included 3 stages: 1) direct and reverse translation, linguistic validation of the questionnaire; 2) assessment of internal consistency, reliability and sensitivity (The Russian version of CSI-R is valid, reliable and can be used in clinical practice as a diagnostic tool for revealing central sensitization. The study of the sensitivity of the questionnaire in patients during drug therapy proved its effectiveness in assessing the dynamics of the disease and the effect of therapy.Создание и психометрическая валидизация русскоязычного варианта Опросника центральной сенситизации (CSI-R).Исследование включало 3 этапа: 1) прямой и обратный переводы, лингвистическая валидизация опросника; 2) оценка внутренней согласованности, надежности и чувствительности (Русскоязычная версия опросника (CSI-R) является валидной, надежной и может применяться в клинической практике как диагностический инструмент для выявления центральной сенситизации. Исследование чувствительности опросника у пациентов на фоне лекарственной терапии доказало его эффективность для оценки динамики заболевания и эффекта терапии.

Idiopathic generalized dystonia is a group of neurodegenerative diseases primarily charecterized by dystonic hyperkinetic disorder (irregular slow involuntary movements of different body parts accompanied by specific muscle tone changes and pathological poses). A 54-year-old female was admitted with various complaints. The leading syndrome was extrapyramidal due to which the differential diagnosis included Parkinson’s disease, multiple system atrophy, corticobasal degeneration, etc. The diagnosis of idiopathic generalized dystonia, in the form of lower extremity hyperkinesia with dystonic equinus of the left foot and hallux extension and cheirospasm, was made by a method of exclusion. The clinical case described in the article illustrates variety of clinical manifestations of the disease imitating clinical presentation of various neurological and psychiatric disorders, complexity of diagnostic search and importance of timely diagnosis of this disease.

SUMMARY: The aim of the study was the assessment of treatment quality of patients with diabetes mellitus type II in the family policlinic № 4 of Samarkand city carried out by team of medical staff. Material and Methods. This investigation was carried out in the areas of family policlinic № 4 of Samarkand city. The amount of general population registered to this policlinic was 53556 people. The number of patients on dispenser observation because of different diseases was 31886 people. In order to carry out investigation we select patients with diabetes mellitus, the number of them was 656 patients. For the assessment of quality of treatment of patients with diabetes mellitus we elaborated standards and indicators according to the card of health care system. Results. In the investigated policlinic № 4 they practically do not have clinical protocols, guidelines and standards on diagnostic and treatment of diabetes mellitus based on evidence, also they have lack of information materials and hand-outs on effective treatment of diabetes mellitus, from 656 patients with diabetes mellitus who were on dispenser observation only 395 patients (60.2 %) got appropriate treatment based on evidence, from 25 general practitioners who worked in the city family policlinic № 4, 15 of them (60 %) know about methods of primary, secondary and tertiary prevention of diabetes mellitus. Conclusions. As a result of the performed assessment we determined a low treatment quality of patients with the II type of diabetes mellitus within family policlinic № 4 of Samarkand city.

Резюме. Дана стаття присвячена оцінці поширеності та якості лікування хворих із серцевою недостатністю в умовах первинної ланки медико-санітарної допомоги за останні 5 років.Були відібрані і аналізувати амбулаторні карти 125 хворих із серцевою недостатністю різної етіології, які перебувають на обліку в сільському лікарському пункті (СВП). З них 77 жінок (61,6%) і 48 чоловіків (38,4%). В процесі оцінки якості медичної допомоги хворим із серцевою недостатністю були виявлені відсутність стандартів і протоколів лікування серцевої недостатності, засновані на доказовій медицині.Також спостерігається дефіцит лікарів загальної практики, які володіють теоретичними знаннями і практичними навичками лікування серцевої недостатності на сучасному рівні. Велика частина хворих, які страждають серцевою недостатністю не отримують адекватного немедикаментозного і медикаментозного лікування, заснованого на доказовій медицині.

The degeneration of dopaminergic (DA-ergic) neurons of the nigrostriatal system results in the development of Parkinson’s disease (PD). It is assumed that the increase of DA release from survived DA-ergic neurons might be one of the compensatory processes serving to maintain the DA concentration in the extracellular space at a normal level and thereby providing a long-lasting period of asymptomatic development of the disease. The goal of this study was to estimate DA release in the substantia nigra (SN) containing somas of DA-ergic neurons and in the striatum containing their axonal terminals under the different extents of the degradation of the nigrostriatal system corresponding to either presymptomatic or symptomatic stage of parkinsonism in mice. In the ex vivo studies of the brain slices under perfusion, it has been shown that the values of both spontaneous and K+-stimulated release of DA in the SN at both stages of parkinsonism were the same as those in the controls suggesting the increase of DA release from the survived somas of DA-ergic neurons. In the striatum at presymptomatic and symptomatic stages, no compensatory modification of spontaneous DA release was observed whereas K+-stimulated release of DA from the survived axonal terminals increased significantly. However, the total amount of DA released at symptomatic stage was below the control level that was in contrast to that at presymptomatic stage. This observation is probably among the reasons of the appearance of the initial symptoms of parkinsonism.

International audience; Progressive degeneration of nigrostriatal dopaminergic (DA-ergic) neurons is a key component in the pathogenesis of Parkinson's disease, which develops for a long time at the preclinical stage with no motor dysfunctions due to the initiation of compensatory processes. The goal of this study was to evaluate the changes in surviving nigrostriatal DA-ergic neurons with focus on tyrosine hydroxylase (TH) in MPTP-treated mice at the presymptomatic and early symptomatic stages of parkinsonism. According to our data, a partial degeneration of DA-ergic neurons at the presymptomatic stage was accompanied by: (i) no change in TH mRNA content in the substantia nigra (SN) suggesting a compensatory increase of TH gene expression in individual neurons; (ii) a decrease of TH protein content in the nigrostriatal system and no change in individual neurons, suggesting a slowdown of TH translation. When comparing DA-ergic neurons at the early symptomatic stage and presymptomatic stage, it becomes evident: (i) a decrease of TH mRNA content in the SN and hence gene expression in individual neurons; (ii) a decrease of TH content in the striatum and its increase in the SN and individual neurons suggesting an acceleration of TH translation. TH activity, an index of the rate of DA synthesis, was unchanged in the SN and decreased in the striatum to the same degree at both stages of parkinsonism. In the meantime, TH activity in individual neurons appeared to be compensatory increased, but to a higher degree at the symptomatic stage than at the presymptomatic one. These data first show that DA depletion, which provokes motor dysfunction, is not a result of the decrease of TH activity and the rate of DA synthesis but is rather related to either a decrease of DA release or an increase of DA uptake in striatal DA-ergic axons.Copyright © 2014 Elsevier B.V. All rights reserved.

Activities of monoamine oxidases A and B were examined on the models of presymptomatic and early symptomatic stages of Parkinson's disease developed in mice treated with MPTP, a specific neurotoxin affecting dopaminergic neurons. Activity of monoamine oxidases A, the key enzyme of dopamine degradation, is increased in neuronal somas during the symptomatic stage, and it is augmented in the axons during both stages. Neuronal activity of monoamine oxidases A is higher during the symptomatic stage than that during the presymptomatic stage, which can explain depletion of intercellular dopamine and appearance of motor disturbances. Activity of monoamine oxidase B in the striatum is reduced during the presymptomatic stage, but returns to the control level during the symptomatic stage. Variation in monoamine oxidase activity seems to reflect the compensatory mechanisms triggered in degrading nigrostriatal dopaminergic system.

The degeneration of dopaminergic (DAergic) neurons of the nigrostriatal system results in the development of Parkinson's disease (PD). It is assumed that the increase of DA release from survived DA� ergic neurons might be one of the compensatory processes serving to maintain the DA concentration in the extracellular space at a normal level and thereby providing a longlasting period of asymptomatic develop� ment of the disease. The goal of this study was to estimate DA release in the substantia nigra (SN) containing somas of DAergic neurons and in the striatum containi ng their axonal terminals under the different extents of the degradation of the nigrostriatal system corresponding to either presymptomatic or symptomatic stage of parkinsonism in mice. In the ex vivo studies of the brain slices under perfusion, it has been shown that the values of both spontaneous and K + �stimulated release of DA in the SN at both stages of parkinsonism were the same as those in the controls suggesting the increase of DA release from the survived somas of DAergic neurons. In the striatum at presymptomatic and symptomatic stages, no compensatory modification of spon� taneous DA release was observed whereas K + �stimulated release of DA from the survived axonal terminals increased significantly. However, the total amount of DA released at symptomatic stage was below the control level that was in contrast to that at presymptomatic stage. This observation is probably among the reasons of the appearance of the initial symptoms of parkinsonism.