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Objective: Griscelli syndrome type 2 is rare authosomal ressesive disorder caused by a defect in the RAB27A gene, which affects a melanosome-anchoring complex in melanocytes, affecting release of cytolytic granules from T and NK cells. Children with GS type 2 develop an uncontrolled T-lymphocyte and macrophage activation syndrome known as hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH). We describe a 3 years old girl patient classic features of Griscelli syndrome type 2 Case report: A 3-year-old girl was admitted to hematology with complaints of LAP, hepatosplenomegaly and pancytopenia (WBC- 3080/µl, Hb-7.6 g/dl, Neutr.-350/µl, PLT - 165000/µl). The patient's condition was below the percentile, her skin was bronze, her hair was silver-grey. HLH criteria were met (triglycerides 458 mg/mL, ferritin 3445 ng/mL, fibrinogen 180 mg/dl). Morphology of the bone marrow was hypocellular, signs of dyserythropoiesis (stage I) and megakaryocytes were reduced Methodology: According to the clinical and laboratory data(hepatosplenomegaly, increased ferritin, hypertriglyceridemia, pancytopenia, hyperthermia resistant to antimicrobial therapy, silver-gray hair, pigment balls of hair seen light microscope) and the death of another undiagnosed child in the family, suggested likely primary HLH and GS. As a result of genetic analysis (homozygous mutation c.514_ 518delCAAGC(p.GLN172Asnfs*,rs767481076)1 in the RAB27A gene), the diagnosis of GS type 2 was confirmed. Results: The patient was treated according to the HLH 2004 protocol. CSA levels were measured once a week. IVIG support was given based on IgG levels. HSCT was planned from patient's healthy HLA-matched sibling, but HSCT was delayed because the brother was infant. After 45 weeks of maintenance therapy, etoposide was discontinued, dose of dexamethasone was reduced to 5 mg/kg, but CSA was continued at the same dose. Control studies are carried out once a week. As far as possible HSCT is planning Conclusion: The prognosis of patients with Griscelli syndrome is poor. It is usually rapidly fatal within 1-4 years without aggressive treatment and bone marrow transplantation at onset of an accelerated phase. HSCT is more successful when implemented early course of the disease. Palliative care includes treatment and prophylaxis care infections and immunosupressor therapy in accelerated phases. Some patients have died after transplantation, but others have had lasting remissions

Kavramların incelenmesiyle ilgili son araştırmalara bakarsak, onların çoğunun tek taraflı yürütüldüğünü görebiliriz. Belirli bir kavram bazen onun diğer kavramlarla bağlantıları dikkate alınmadan, başka dillerdeki benzer kavramlarla karşılaştırılmadan araştırmaya dahil edilir. Aslında, bu yolla bir kavramın bir dilin kavram sahası içindeki yerini ve önemini belirlemek mümkündür. Ancak herhangi bir kavramın, özellikle de kişilerle bağlı kavramların, bir insanın düşüncesinde, günlük yaşamında ve ahlaki değerlerindeki yerini belirlemek için, onu farklı kavramlarla karşılaştırmak gerekir. Çalışmamızın en önemli yönü bu tür karşılaştırmaların geniş çapta kullanılmasıdır. Çalışmada aile, yani anne-baba, anne-kız, anne-oğul ilişkileri çerçevesinde “anne” kavramı incelenir. Öte yandan “anne” kavramından doğan “analık” ve “kaynana” gibi kavramlar da karşılaştırmalı bir yöntemle incelenerek “anne” kavramının genel resmine katkıda bulunmaktadır. Çalışma sadece Azerbaycan Türkçesinin sınırları içinde değil, aynı zamanda Azerbaycan Türklerinin ve Rusların anneye karşı tutumları arasındaki benzerlikleri ve farklılıkları belirlemeye olanak sağlayan Rus dilinin kavram sahası ve paremiyolojisi ile karşılaştırmalı şekilde yapılmıştır.

Hamid Araslı, one of the leading literary critics of the 20th century, made great contributions to Azerbaijani literature with his research. As a result of his research, the poets who lived and created between Nizami and Fuzuli's creativity in literature were examined and he conducted a study on this subject. It has revealed a lot of unknown information about the poets. Hamid Araslı, in his work "The Life of the Poet", described the city of Ganja in the 12th century and the life and works of Nizami Ganjavi. In the work, extensive information is given about the atmosphere in Ganja at that time, the Ozan neighborhood and the Ahi brotherhood organization that emerged in Ganja, as well as the writing history of the genius poet's "Hamsa". In the work, along with Nizami, Abul-ula Ganjavi, his grandfather Zeki Muayyadin, his father Yusif, his father's friend and teacher the poet Imadeddin, his brother Givami Ganjavi, Mehsati Ganjavi, Hatibi, whose works he read with love, Ganja emir Garasungur, scholar Ebu Zekeriyya Yahya, Hakani, Muhammed Felaki, Valuable figures such as Mujireddin Beylagani are mentioned. [ABSTRACT FROM AUTHOR]

The article examines the history of the city of Ganja, which occupies an important place in the history of Azerbaijan. Based on the works of the scientist Hamid Arasly and historical sources, the social, scientific and literary environment of Ganja in the 11th -12th centuries was comparatively examined. The considered work of H. Arasly, dedicated to the life and work of the great thinker Nizami Ganjavi, is written on the basis of historical facts, as well as the artistic thought of the author. At the same time, the historical facts of the events mentioned by H. Arasli in his work on the history of Ganja are also given with reference to sources. In the book, H. Arasly writes about Ganja - the scientific, literary, social environment of the city, etc. - correspond to historical facts, introducing us to the real Ganja of the Nizami period. And this acquaintance once again reflects the indisputable confirmation that such a genius as Nizami lived and worked in a city like Ganja, where revival and development were observed in all areas. The article also highlights the importance of Ganja's scientific environment in the early 20th century, touching on the scientists from whom Hamid Arasli received his scientific education in Ganja in his youth, the science he adopted from them, and the impact of all this on H. Arasli's life. Ganja, historically the birthplace of great figures, was chosen due to its importance in the history of Azerbaijan, regardless of period, and the enthusiasm and warmth of its literary and cultural environment. And for this reason, in addition to its important place in history, it has also become the main subject of countless works in literature. [ABSTRACT FROM AUTHOR]

The article deals with the trends of population ageing in the Republic of Azerbaijan. The author considers the stages of change in population size and age structure in the 20th and the 21st centuries as a result of historical evolution of birth rate and death rate. Based on the analysis of fertility trends and life expectancy, it is shown that the decline in fertility has so far been the most important factor in the population ageing, whereas the decline in the mortality in older ages is only beginning to contribute to this process. It is emphasized that anomalies in the sex ratio at birth lead to accelerated ageing of the population. The gradual ageing and reduction of the share of the working-age population, as well as the growth of the dependency ratio and the exhaustion of the potential of the first demographic dividend, are also analysed in the paper.

The article examines the prospects for the application of active learning methods in modern education. The survey shows that the prospects for the application of active learning methods in modern education are conditioned by the influence of different factors. These factors can be divided into several groups: comprehensive organization of teaching, building adequate communication, the use of new learning technologies, organization of personality-oriented education and teamwork. The survey showed that there are real and potential barriers for teachers to use innovative teaching methods, but there is no serious paradox. There is no doubt that there is an intensive interest in the prospects and future of the application of active learning methods in modern education and their application in education. When choosing a teaching method, teachers' ability to use this method and local conditions should be considered.

Purpose – to analyze concept as a unit of mental code, mental formation, fragment of person’s life experience, quantum science, category of thought, cognitive structure attributed to lingua cultural context, as a part of culture in person’s mind. Methodology. The basis of the research is the method of description, general scientific methods of analysis and synthesis, cognitive analysis, experimental method, contextual analysis, structural and semantic analysis, reception of transformation, comparative analysis. Results. Actuality of the article is determined by the approaches of cognitive linguistics and linguaculturology to “concept”. Linguists’ and culturologists’ opinions about the essence of concept are presented in the article. It is also stated that behind the concept formation and its associative relationships stands historical-cultural development of certain nation, and Russian researchers ideas about typology, structure and characteristics of concept are presented. Another field explored in the article is concept sphere. Main aspects of concept sphere having direct link to each nation’s cultural and material heritage are also mentioned in the article.

The contemporary state of the plant drugs market requires a close attention of researches and herbal drugs producers. Constant change of correlation between the use of synthetic drugs and natural origin drugs, and the constant change of herbal drugs range determines the necessity of updating of our knowledge about what herbal medicinal products and of what producers are more required. Regular control of the herbal medicinal products market is possible to implement with different methods. They are the analysis of accounts and records about the pharmacy goods consumption, questioning of pharmacies customers, doctors of medical facilities etc. The analysis of the data obtained will allow enhancing the range of herbal drugs, implemented for the treatment of diseases of different organs and systems of patients at any age, and which have chronic form and at the rehabilitation treatment stage. Competent instruction of doctors and pharmacy customers by the pharmacist about the possibilities of herbal drugs implementation would allow significant improvement of medicinal support for the population.

The article deals about how study the control measures against ascariasis, which is widespread among birds in the western zone of the republic. The best effective drugs against helminthiasis of birds are fenbendazole, nilverm, mebednazol, fenesal, filixan, hyperazine. These drugs should be given with feed or as a solution in water, in the dosage and order according to their instructions. The research work was carried out in the "Chinarli" peasant farm located in Shamkir region, in the laboratory of the "Epizootology, microbiology and parasitology" department of the "Veterinary Medicine" faculty. Mattresses and feathers of birds should be neutralized by biothermal or thermal methods, buildings and equipment should be disinfected regularly, a disinfecting place should be made at the entrance of birdhouses, and the place of birds kept in camp conditions should be changed every year. If helminths adapt to one of these drugs, deworming should be replaced by another drug. [ABSTRACT FROM AUTHOR]

Increasing the productive capacity of dairy cows increases the incidence of non-communicable animal diseases, including such metabolic diseases as ketosis, osteodystrophy, postpartum hypocalcemia, etc. Under conditions of excessive physiological stress, violation of the optimal mode of feeding and keeping animals, technological stresses often lead to disruption of neurohumoral regulation and the appearance of diseases similar to postpartum hypocalcemia. The wide distribution of postpartum hypocalcemia, its insufficient knowledge in conditions of intensive animal husbandry determined the scientific direction of our work.The main goal of our work was to study the effect of increasing calcium in the ration of cows during the drying period of milk in highyielding cows in large specialized farms on the frequency of postpartum hypocalcemia. [ABSTRACT FROM AUTHOR]

The influence of γ-quanta on the current flow in TlInSe2 single crystals and the process of filling and emptying localized levels in crystals has been experimentally studied. Investigations show that the current -voltage characteristics (CVC) of TlInSe2 single crystals obey Lampert theory and are determined by currents limited by the space charge. It is shown that the defects occurring from γ-quanta in TlInSe2 single crystals result from radiation processes. [ABSTRACT FROM AUTHOR]