Background: Cystinosis is a rare autosomal recessive lysosomal storage disease with high morbidity and mortality. It is caused by mutations in the CTNS gene that encodes the cystine transporter, cystinosin, which leads to lysosomal cystine accumulation. It is the major cause of inherited Fanconi syndrome, and should be suspected in young children with failure to thrive and signs of renal proximal tubular damage. The diagnosis can be missed in infants, because not all signs of renal Fanconi syndrome are present during the first months of life. Elevated white blood cell cystine content is the cornerstone of the diagnosis. Since chitotriosidase (CHIT1 or chitinase-1) is mainly produced by activated macrophages both in normal and inflammatory conditions which suggest that cystinosis should be included within the differential diagnosis of disorders associated with increased plasma chitotriosidase activity. This study is aimed to estimate serum chitotriosidase level, as a screening marker and therapeutic monitor for cystinosis disease in Iraqi children with cystinosis.
Subjects and Methods: The present study is a case-control study that included samples of 30 children with nephropathic cystinosis, compared to 25 healthy control children from those attending at The Genetic Rare Diseases Center / AL-Emamain AL-Kadhimain Teaching Hospital, Baghdad-Iraq.
Results: Our results reported that cystinotic children had a marked elevation of serum chitotriosidase activity, compared to age-matched healthy children, besides a significant associated with leukocyte-cystine content for cystinotic patients.
CHT1 as a Novel Biomarker
Conclusion: Estimation of serum chitotriosidase activity might aid in monitoring the therapeutic benefits of cysteamine therapy, as well as the prognosis of the disease when WBC cystine assessment is not available.
Key Words: Cystinosis, Cysteamine, Chitotriosidase.
The research is conducted on target of investigating the role of growth strategy via diversification in value maximization of a firm in terms of controversies literatures had witnessed. Using a descriptive approach for analyzing and verifying the harmony of variables of research and their conceptualized logic , it could be reached to many conclusions agreed in their essence upon that the related diversification has the major role in value maximization of a firm and the wealth of its owners .
The relationship between the vision of the scenario and the director is represented in the second search for new mechanisms and possibilities that possess the direct effect and synonyms of the vision of the scenario, so he works on investing them in achieving his ideas and visions with more effectiveness and flexibility to achieve creative potentials, without resorting to the installations and expressive structures themselves that have exhausted their meaning, and lost their luster due to Its prevalence and familiarity on the one hand, and its activation on the other hand to achieve modernity at the level of aromas, after the director takes a method or approach that has privacy and exclusivity, and based on the foregoing, getting
... Show MoreThe theatre is one of the main pillars of the human aesthetic thinking as it contains logical explanations for the public human life aspects outside time and environment. When we find that the directive philosophical thinking moving away from the stylistic constants and the virtual laws in taboos that control the accomplishment in terms of the traditional shapes, the employment and the theatrical reception. Some directors moved in post-modernism concepts in a perceptive that liberates thought from its natural context in thinking into an innovative perceptive. The aesthetic images are the basic premise in the prohibition of the philosophical thinking interpretation in order to move the directive thinking and this movement depends on the v
... Show MoreThe discus throwing event is one of the complex events in athletics, and it is characterized by a performance method that depends on the principle of mechanical moments and requires high explosive capabilities of the thrower in addition to some physical specifications,which depends effectively and effectively on the biomechanical aspects in generating large moments during rotation. The importance of the research is highlighted by the interest in athletics, especially the effectiveness of the discus throw and the continuation of its development process, the importance of kinetic analysis in revealing the most important weaknesses and strengths of shooters, and the importance of explosive power And the moments generated in the rotation of the
... Show MoreThe study aims at explaining the extent to which the principles of educational management reform contribute to Ibn Ashour in achieving educational management reform and the extent to which the pillars of the Kingdom's Vision 2030 in the field of Education in achieving the educational management reform. The study also aims to provide a future vision of what the educational administrative reform and its results should be in the Kingdom during the next ten years. To achieve the goals of the study, the researcher followed two approaches: on the theoretical side, he relied on applying the content analysis method. As for the applied side, the researcher adopted the Delphi method by two questionnaires to ask (36) participants from the experts a
... Show MoreAbstract
This study aims at investigating the effectiveness of the cooperative inquiry strategy in developing the achievement and development of mathematical communication skills of elementary school students. To achieve this aim, the experimental method was utilized. The study sample consisted of an experimental group (n = 34) and a control group (n = 31), which were randomly selected from primary schools. A Teacher's Guide for teaching a unit entitled "Statistics and Graphic Representations" for the sixth elementary graders in the first semester were devised. A (30) multiple-choice item achievement test distributed at the levels of (recall, comprehension, and application), as well as an (18) item mathematical c
... Show Moreالمقدمة
تتعامل الجهات الضريبية في مختلف دول العالم بأساليب عديدة لجباية الضرائب من المكلفين بغض النظر عن فئات وأصناف هؤلاء المكلفين،وفي العراق تم اعتماد العديد من الأساليب لجباية الضرائب على امتداد المدد الزمنية المتعاقبة،وكان لأسلوب التقدير الذاتي وهو أحد تلك الأساليب مجالاً للتطبيق خلال مدة زمنية معينة،حيث جرى تطبيق هذا الأسلوب على وحدات اقتصادية معينة، وبالرغم من المساوئ التي قد ترافق تطبيق
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Key findings from the study include:
1. The Category "Photos from the Gulf Cup matches" predominated, accounting for 291 instances or 42.794% of the total.
2. Photojournalists exhibited a keen interest in capturing images of attending fans and documenting celebrations surrounding th