Schwartz–Jampel syndrome: comprehensive diagnostics and orthopedic treatment

Schwartz–Jampel syndrome (chondrodystrophic myotonia) is a monogenic genetic disorder, demonstrating unique coincidence of the features of skeletal dysplasia and neuromuscular disease. We present four clinical cases of Schwartz–Jampel syndrome. Principles of diagnostic and results of surgical treatment of the lower limbs deformities are discussed. Importance of this study comprises unique description of rare cases. Surgical treatment for orthopaedic conditions is important and can lead to functional improvement, prevent progression of deformities and enhance daily activity

Production of the Calcium Fluoride From Solid and Iiquid Wastes of Aluminum Production Process

Для регенерации фтора разработана технология получения фторида кальция из насыщенных растворов газоочистки. Исследованы и определены оптимальные условия процесса синтеза фторида кальция. Производство CaF2, полученного из растворов газоочистки, позволило снизить себестоимость 1 т вторичного криолита и закупку свежего фторида кальция на 80 %. Результат по регенерации фтора из вторичных жидких отходов положил начало научному поиску по извлечению его из твердых отходов. Проведены лабораторные исследования по извлечению фтора из твердых отходов способом выщелачивания с последующим получением вторичных фторидов, пригодных для использования в процессе электролиза алюминия. Безотходная технология позволит снизить образование отходов алюминиевого производства, получить доход от использования вторичного сырья в процессе электролиза и реализации углеродного концентрата в другие отрасли промышленностиA technology for the regeneration of fluorine to produce calcium fluoride from a saturated solution of gas purification had developed. Investigated the optimal conditions for the synthesis of calcium fluoride gas cleaning solutions. Production of calcium fluoride will reduce the cost of secondary cryolite, and the purchase of a primary CaF2 to 80 %. The laboratory study on extraction of fluoride from solid waste leaching process followed by obtaining secondary fluorides. Waste technology will reduce the waste of aluminum production, to receive income from the use of recycled materials and implementation of carbon concentrate in other industrie

Синдром Шварца–Джампела: опыт диагностики и ортопедического лечения

Schwartz–Jampel syndrome (chondrodystrophic myotonia) is a monogenic genetic disorder, demonstrating unique coincidence of the features of skeletal dysplasia and neuromuscular disease. We present four clinical cases of Schwartz–Jampel syndrome. Principles of diagnostic and results of surgical treatment of the lower limbs deformities are discussed. Importance of this study comprises unique description of rare cases. Surgical treatment for orthopaedic conditions is important and can lead to functional improvement, prevent progression of deformities and enhance daily activity.Синдром Шварца–Джампела (хондродистрофическая миотония) – моногенное наследственное заболевание, характеризующееся уникальным сочетанием признаков скелетной дисплазии и нейромышечного заболевания. Представлены 4 клинических случая синдрома Шварца–Джампела. Рассмотрены принципы диагностики и опыт хирургического лечения деформаций нижних конечностей. Ввиду того, что в современной научной отечественной литературе имеются единичные описания клинических примеров данного синдрома, настоящие наблюдения имеют большую значимость. Хирургическое лечение деформаций опорно-двигательного аппарата патогенетически обоснованно, так как позволяет добиться функциональных улучшений и опороспособности, предотвратить прогрессирование деформаций и контрактур, увеличивая двигательную активность пациентов

REPRODUCIBILITY OF THE BASIC X-RAY PARAMETERS OF LOWER EXTREMITY DEFORMATIONS IN CHILDREN WITH SKELETAL DYSPLASIA

Introduction: axial deformities in the lower extremities of children lead to an uneven distribution of the load in different compartments of the knee joint, which can contribute to the development of osteoarthritis. Regardless of the cause of their occurrence, the main goal of treatment is the restoration of the mechanical axis. Various calculation methods are used to determine the amount of deformity, its apex and the degree of required correction. The aim: to evaluate the reproducibility of the basic X-ray parameters characterizing the deformities of the lower extremities in the frontal plane in children with skeletal dysplasia based on the calculation of inter-rater reliability.Materials and methods: the article presents calculations of the main angulometric parameters of the knee joint (deviation of the mechanical axis — MAD, distal mechanical angle of the femur — mLDFA, proximal angle of the tibia — MPTA) in 18 patients with skeletal dysplasia (30 lower extremities) that had axial deformities of the lower extremities in the frontal plane at the level of the knee joint. The control group included the results of similar calculations of the angulometric parameters in 19 children (30 lower extremities) with valgus and varus deformities of the knee joint without skeletal dysplasia (idiopathic axial deformations, posttraumatic deformities, malformations of the lower limbs). The estimation of inter-expert reliability was made in the SPSSv. 23.Results: the analysis demonstrated that calculation of reference parameters in children without primary lesion of the growth plate has a high degree of inter-rater reliability: an intra-class correlation coefficient with a 95% confidence interval (ICC [95% CI]) when calculating the reference angles of mMPTA and mLDFA was 0.981% [0.971–0.991] and 0.993% [0.989–0.997] respectively, for MAD 0.996% [0.993–0.998]. When calculating the same parameters in children with skeletal dysplasia, the results differed. Thus, the intra-class correlation coefficient for the deviation of the mechanical axis was 0.861% [0.763–0.926] (which corresponds to the normal reproducibility of this parameter), for mMPTA — 0.586% [0.295–0.781], for mLDFA — 0.796% [0.653–0.892]. This indicates a low reproducibility and may lead to errors in the planning of correction of axial deformities of the lower limbs in children with skeletal dysplasia.Conclusion: when calculating the severity of axial deformities in the lower extremities in children with skeletal dysplasia, the most reproducible parameter is the determination of the deviation of the mechanical axis of the lower extremities as compared to the studied angulometric methods

Coherence of EEG frequency components during manual movements executed by the subdominant hand in women

The academic community is paying more and more attention to the question of the individual characteristics of the brain processes which ensure the manual motor programming of movements performed not only by the leading, but also by the subdominant hand. Researchers do not exclude the existence of the particular parameters of the human brain correlating with manual motor activities. This study involved 136 women at the age of 19–21 years. The testees were divided into two groups according to high and low values of the EEG modal α-frequency determined individually and in a motionless state. We evaluated the coherence status of the EEG frequency components in the motionless state and during movements performed by fingers of the subdominant (left) hand in response to rhythmic sound signals. The testing stages involved the sequential execution of motor tasks including clamping and unclamping performed by the fingers of the subdominant hand (such as grasping movements) without effort. The testees also performed fingering (a manual response to each stimulus) at in different times and not by all the fingers of the hand simultaneously, but separately, one by one, in a given sequence). Clamping and unclamping was executed by the fingers subject to power loading the (additional load on the fingers being 10H). Execution of manual movements by means of the subdominant hand in response to the sensory signals was accompanied by an increase in coherence in the EEG frequency components, especially in the frontal, temporal, and parietal cortexes of the central areas. Women with a low individual α-rate of such a regularity had significantly increased scores at the high (α3-, β-) frequencies of the EEG spectrum. At the same time, women in both groups mainly showed a decrease in the coherence coefficients of θ-, α1- and α3-activity in the frontal cortex leads in terms of the execution of the sequential finger movements and movements under power loading. The analysis of differences proved that women with a low IαF had relatively higher coherence of the EEG frequency components in the frontal lobes. However, relatively higher frequency components of EEG coherence in the central, posterior temporal and parietal leads were set in women with high IαF. Changes in cortical activity were established during different movements performed by fingers of the subdominant hand, namely, grasping movements of common occurrence, successive movements and movements under power loading. They differ from each other in their low specificity. Thus, the factor of motor performance of tests by means of the subdominant hand had obviously more decisive importance in the nature of the organization of brain activity than for the actual specifics of manual movements

Извлечение из глиноземсодержащих сметок алюминиевого производства оксидов кремния и железа

Текст статьи не публикуется в открытом доступе в соответствии с политикой журнала.The method of recovery of contaminating components from sweepings of the aluminum production for their further return into the electrolyzer is proposed. To concentrate the material, the following processing flowsheet is proposed: milling-classification-reverse flotation-thickening. For the most complete removal of silicon and iron oxides during the flotation of sweepings, the Flotigam 7266 flotation reagent produced by Clariant (Germany), which is a mixture of primary fatty alkyl amines, is used. To remove carbon particles, the combination of pine oil in a mixture with kerosene is used. Flotation is performed using a FML 0.3 flotation machine. The initial material, chamber product, and tails are analyzed for the content of carbon and aluminum, iron, and silicon oxides using X-ray spectral (XSA), X-ray phase (XPA), and chemical analyses. It is established that processing the total material mass does not make it possible to acquire a product with an acceptable content of silicon and iron oxides. Based on the XPA of various fractions of the initial material, it is proposed to process the material fractions containing the minimal amount of contaminating substances (carbon and silicon and iron oxides). Two fractions are selected for processing using the flotation method by the XRS results of various material fractions:-0.071 mm and +5.0 mm. When processing the first of them, the chamber product of the acceptable quality is acquired. A product with a high content of alumina and fluorinated components at low carbon and iron oxide concentrations but a considerable amount of silicon oxide is acquired from a coarse electrolyte-containing fraction (+5.0 mm). The further use of this product is possible to fabricate aluminum-silicon alloys