Variant of rare hermansky — pudlak syndrome associated with granulomatous colitis: diagnostics, clinical course and treatment

Aim: To study the relationship between the genotype and the phenotype in the patients with Hermansky — Pudlak syndrome (HPS) associated with granulomatous colitis; to monitor clinical course of the disease for adequate treatment, cancer surveillance and genetic counseling. Materials and Methods: The diagnosis of HPS is established by physical examination, chest X-ray, computed tomography, endoscopic examination with biopsy, and laboratory tests, including histology, baseline laboratory blood, urine and feces tests, determination of ASCA-C and ANCA antibodies using an ELISA. Molecular genetic testing for HPS gene mutations, R702W, G908R, L1007fs and P268S mutations in NOD2 gene, and TaqI variant of the VDR gene were carried out. Results: We report 2 cases of HPS from unrelated families. Both were complicated by inflammatory bowel disease with pathologic features of Crohn’s disease refractory to antibiotics and corticosteroids. One patient (family 1) with Ashkenazi Jewish ancestry had pathogenic variant of the HPS-4 gene in exon 8, mutation P268S of NOD2 genes and “Tt” genotype of TaqI variant of the VDR gene. Another patient (family 2) carried two mutations P268S and G908R of NOD2 gene, and had a large paraovarian cyst diagnosed. No consistent success with the standard medical therapy, used for treating granulomatous colitis, associated with HPS, in presented cases was achieved. Patients needed surgical interventions at a young age and a long-term surveillance of the probable development of tumors and other complications. Azathioprine at 2 mg/kg/day and mesalazine 3 g/day were used with some positive effect for prevention of Crohn’s disease postoperative recurrence. Conclusion: The occurrence of perianal lesions, the histopathological findings and the results of the molecular genetic analysis confirmed the mutations P268S and G908R of NOD2 gene in these cases suggest that HPS was truly associated with Crohn’s disease variant with early onset and severe course. The search for the molecular causes of the disease in some individuals may help in the development of new therapeutic and surgical approaches, as well in the improvement of understanding of premalignant inflammatory conditions in a large bowel

Effect of Temperature on Solid-state Hydride Metal Synthesis According to Thermodynamic Modeling

Thermodynamic modeling of the reduction of copper dichloride in the media of various gaseous hydrides (ammonia, monosilane, methane) in the temperature range 273-1000 K was carried out. Calculations show that in narrower temperature ranges corresponding to the reactions of solid-state hydride synthesis (SHS) of metal sub- stances metal formation is usually supported by theoretical propositions. As a result of thermodynamic modeling, a principal result was obtained on the suppression of  competing processes of nitriding, siliconizing and carbonization  of metal under SHS conditions, which is important for metallurgical production. This additionally substantiates the correctness of previous experimental studies of SHS metals with modified surface and improved properties. By mod- eling, it was found that the reduction of solid copper dichloride to metal in ammonia or methane occurs stepwise (se- quentially, according to the Baykov rule) through the intermediate stages of the formation of a compound of low- valent copper – copper (I)chloride

THE INFLUENCE OF THERMOELASTOPLASTS ON THERMAL STABILITY OF POLYETHYLENE’S COMPOSITIONS

The article considers theimpacton the stability of compositions consisting thermoelastoplast against thermo-oxidative degradation

СВОЙСТВА ЛАКОВЫХ ПОКРЫТИЙ НА ОСНОВЕ МОДИФИЦИРОВАННЫХ ТЕРПЕНОМАЛЕИНОВЫХ СМОЛ

The development of electrical products with increased technical and economic indicators requires further improvement of electrical insulating materials. Due to the wide introduction of automatic winding methods, it is of great importance to develop varnish coatings that ensure the reliability of insulation under significant mechanical stresses. This study was devoted to the development of the operational properties of electrical insulating varnishes by developing technologies for high-efficiency secondary products of terpenomaleinic resins (TMS resins) and thermosetting compositions based on them. In this work, physical and chemical properties of TMS resins modified with heavy metal salts such as acid number (AN, mg of KOH/g), softening temperature (Ts, °C), viscosity (v20) and destruction temperature (Td, °C) were studied. The effect of the obtained TMS resins on physical and mechanical properties of lacquer coatings based on them such as film thickness (μm), hardness of the varnish coating (conventional units) and the corrosion resistance was studied. The effect of TMS resins modified with salts of heavy metals (Co, Mn and Zn acetates) on physical and mechanical properties of lacquer coatings based on them was studied as well. Создание электротехнических изделий с повышенными технико-экономическими показателями требует дальнейшего совершенствования электроизоляционных материалов. В связи с широким внедрением автоматических методов намотки большое значение имеет разработка лаковых покрытий, обеспечивающих надежность изоляции при значительных механических воздействиях. Данное исследование посвящено повышению эксплуатационных свойств электроизоляционных лаков путем разработки технологий высокоэффективных вторичных продуктов терпеномалеиновых смол (смол ТМС) и термоотверждаемых композиций на их основе. В процессе выполнения работы изучены физико-химические свойства смол ТМС, модифицированных солями тяжелых металлов (ацетатами Со, Mn и Zn), такие как кислотное число (КЧ, мг КОН/г), температура размягчения (Тр, °С), вязкость (ν20) и температура деструкции (Т срД, °С). Исследовано влияние полученных смол ТМС на физико-механические свойства лаковых покрытий на их основе: толщина пленки (мкм), твердость лакового покрытия (усл. ед.) и антикор- розионная стойкость.

Получение и антисептические свойства состава на основе таллового пека

The tall pitch, modified by polyethylenepolyamine, has been prepared. Antimicrobic and anticorrosion properties of the product obtained, have been studied. The possibility of its use in impregnating compositions for protection of cable yarn and underground power cable, has been shownПриведены результаты получения и изучения свойств таллового пека, модифицированного полиэтиленполи-амином. Изучены антимикробные и антикоррозионные свойства полученного продукта. Показана возможность его использования в пропиточных составах, применяемых для защиты кабельной пряжи, силовых кабелей подземной прокладки

Модельные составы для точного литья

The review is devoted to model compositions for exact molding of the metal articles with complicated geometrical configuration obtained from petrochemical, polymer and rosin products.Обзор посвящен модельным составам для точного литья металлических изделий сложной геометрической конфигурации, полученных с использованием нефтехимических, полимерных и канифольных продуктов

ПОЛУЧЕНИЕ, СВОЙСТВА И ПРИМЕНЕНИЕ ТЕРПЕНОИДНОМАЛЕИНОВЫХ АДДУКТОВ

The review is devoted to terpenoid-maleic adducts and their derivatives. Terpenoid-maleic adducts find wide application in various industries. In this review, the ways of adducts’ preparation, their properties, afterproducts on their basis and application in various compositions are discussed. Levopimaric and abietic rosin acids, turpentine components, terpene hydrocarbons, solid turpentine polymers and rosin oils formed in production of rosin and its glycerol ester, have been used as raw materials for production of adducts.Обзор посвящен терпеноидномалеиновым аддуктам и их производным. Терпеноидномалеиновые аддукты находят широкое применение в различных отраслях промышленности. Приводятся способы получения аддуктов, их свойства, вторичные продукты на их основе и применение в различных композиционных составах. В качестве сырья для получения аддуктов были использованы левопимаровая, абиетиновая кислоты канифоли, компоненты терпентина, терпеновые углеводороды и твердые полимеры скипидара, а также канифольные масла, которые образуются в процессе получения канифоли и ее глицеринового эфира

Anticonvulsant and antidepressant activity of new complexes of sncl4 with salicyloylhydrazones of benzaldehyde and 4-brombenzaldehyde by oral administration

Работа посвящена изучению антидепрессивной и противосудорожной активности новых комплексных соединений на основе хлорида олова (IV) с салицилоилгидразонами бензальдегида и 4-бромбензальдегида при их пероральном введении. Полученные данные показывают, что оба соединения обладают высокой антидепрессивной активностью на протяжении 24 ч по методу Порсолта. Через час после введения оба комплекса полностью устраняют иммобильность животных по сравнению с контролем. Новые комплексные соединения SnCl4 с алицилоилгидразонами бензальдегида и 4-бромбензальдегида обладают пролонгированным противосудорожным и высоким антидепрессивным действием, при этом введение брома в 4-е положение бензальдегида усиливает как продолжительность эффекта, так и сам эффект. Данные соединения перспективны для дальнейших фармакологических исследований.This work is devoted to the study of antidepressant and anticonvulsant activity of new complex compounds based on tin chloride (IV) with salicyloylhydrazones benzaldehyde and 4-brombenzaldehyde by oral administration. The data show that both compounds have high antidepressant activity over 48 hours using Porsolt method. One hour after administration of both complexes, completely eliminating the immobility of animals compared with controls. Antidepressant activity of complex II is more pronounced (7% of control values) by administering to 4-position of benzaldehyde bromine atom. Data on the anticonvulsant effect of the new compounds by antagonism with pentylenetetrazole indicate about their prolonged action within 48 hours, the maximum effect was recorded 3 hours after their oral administration. For complex I data DCTC (clonic-tonic ceizures) and DTE (clonic-tonic extension) were 152 and 158%, for complex II — 170 and 190% correspondingly. Thus, the new complex compounds of SnCl4 with salicyloylhydrazones benzaldehyde and 4-brombenzaldehyde has anticonvulsant and high antidepressant effects. The introduction of bromine in benzaldehyde position 4 strengthens both duration of effect and the effect itself. These compounds are promising for further pharmacological researching

A review of hyperfibrinolysis in cats and dogs

The fibrinolytic system is activated concurrently with coagulation; it regulates haemostasis and prevents thrombosis by restricting clot formation to the area of vascular injury and dismantling the clot as healing occurs. Dysregulation of the fibrinolytic system, which results in hyperfibrinolysis, may manifest as clinically important haemorrhage. Hyperfibrinolysis occurs in cats and dogs secondary to a variety of congenital and acquired disorders. Acquired disorders associated with hyperfibrinolysis, such as trauma, cavitary effusions, liver disease and Angiostrongylus vasorum infection, are commonly encountered in primary care practice. In addition, delayed haemorrhage reported in greyhounds following trauma and routine surgical procedures has been attributed to a hyperfibrinolytic disorder, although this has yet to be characterised. The diagnosis of hyperfibrinolysis is challenging and, until recently, has relied on techniques that are not readily available outside referral hospitals. With the recent development of point‐of‐care viscoelastic techniques, assessment of fibrinolysis is now possible in referral practice. This will provide the opportunity to target haemorrhage due to hyperfibrinolysis with antifibrinolytic drugs and thereby reduce associated morbidity and mortality. The fibrinolytic system and the conditions associated with increased fibrinolytic activity in cats and dogs are the focus of this review article. In addition, laboratory and point‐of‐care techniques for assessing hyperfibrinolysis and antifibrinolytic treatment for patients with haemorrhage are reviewed