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.
Multimedia is one of the most important elements of modern educational media and must be used in educational websites in order to disseminate knowledge on a large scale and should be used to provide scientific information to all, as the current research tried to explore the possibilities of employing them in the design of educational websites and highlight their role in promoting the scientific aspects of the user. This study included four axes, the first of which was devoted to the introduction which includes the problem of research, its importance, objectives and its objective, temporal and spatial limitations, which were limited to the study of the main pages of Arabic educational websites published in 2019. The second axis contained th
... Show MoreThe research studies the main variables for the concept of sharing in knowledge in one of the most important control agencies in Iraq, which is (The Federal Board of Supreme Audit). Also, the quality of the controlling businesses by the Board in light of the major challenges that facing the fight against signs of cheating and administrative and financial corruption for offices submitted to controlled and auditing, with the increasing and intensification of these appearances. In order to enable the Board to cope with this situation, has to be thinking hard about how to achieve excellence, progress and development to face these situations, through the application of sharing in knowledge for the financial controller, and then achiev
... Show MoreThroughout this paper we study the properties of the composition operator
C
p1 o
p2 o…o
pn induced by the composition of finite numbers of special
automorphisms of U,
pi (z) i
i
p z
1 p z
Such that pi U, i 1, 2, …, n, and discuss the relation between the product of
finite numbers of automorphic composition operators on Hardy space H2 and some
classes of operators.
Brainstorming is considered as one of the manners that develop learners' mental abilities. Besides, it can help learners get a lot of ideas and thoughts. And by following applied steps to answer the problem concerned, the researcher carried out this practical study aimed at:Developing the ideas of design of third year students/Institute of Fine Arts/Evening Studies- Baghdad/First Rusafa by employing Brainstorming mechanism to develop the ideas of design of institute students in designing the technical advertisement and to achieve the authenticity of the goal of the research, Department of Plastic Arts/Institute of Fine Arts/Evening Studies/Baghdad-First Rusafa was chosen as a case study for the research. It embraced (20) students who rep
... Show MoreThe semiconductor ZnO is one of II – VI compound group, it is prepare as thin films by using chemical spray pyrolysis technique; the films are deposited onto glass substrate at 450 °C by using aqueous zinc chloride as a spray solution of molar concentration 0.1 M/L. Sample of the prepared film is irradiating by Gamma ray using CS 137, other sample is annealed at 550°C. The structure of the irradiated and annealed films are analyzed with X-ray diffraction, the results show that the films are polycrystalline in nature with preferred (002) orientation. The general morphology of ZnO films are imaged by using the Atomic Force Microscope (AFM), it constructed from nanostructure with dimensions in order of 77 nm.
The optical properties o
Experienced economic environmentRadical changes at the end of the last century and the beginning of the present century, resulting in new concepts and expectations in all aspects of economic, political, social and even behavioral.Each of these concepts is the result of rapid developments in the intangible space. Competition is no longer limited to the mere possession of tangible material resources, but because of its link to knowledge and technology content and to the comprehensive quality standards and efficient and effective policies of States. With the increasing pace of growth and interdependence among the global economies, this resulted in the birth of a new economic system led by technological development and financial liberalizati
... Show MoreCitizenship education is the effective educational tool in building a citizen who is able to participate in building his country. This can only come through the educational efforts that teachers make in the classroom. Such a step requires teachers to have knowledge of citizenship education, its principles, and the foundations for its development. Thus, the study aims to examine the degree of social studies teachers' knowledge of the dimensions of citizenship education at the basic education schools in the Sultanate of Oman. It also aims to examine its relationship with a set of variables. The researchers used the quantitative descriptive approach to achieve the objectives of the study. It further adopted a test tool, which consisted in i
... Show MoreThe present study aims at identifying the effect of organizational pressure with its aspects (management, work team, nature and conditions of work, external environment) on job performance in all its dimensions (commitment and effort made, capabilities and the rapidity of performance, motivation and job satisfaction, work environment) for the university teacher at the Faculty of Economics, Commercial and Management Sciences at the University of Djelfa, Algeria.
In this research, the descriptive analytical approach is used. Data was collected through the distribution of a questionnaire to a sample that included 130 permanent teachers before being analyzed using the (SPSS) statisti
... Show MoreThe research aimed to identify the effectiveness of instructional design according to whole brain theory of Herman in the achievement of chemistry at the fifth scientific students at a secondary school of the General Directorate for Educational in Diyala / Baladruz in Iraq. The research sample Consisted of (57 student, (29) students as experimental group studied according to instructional design strategies for whole brain theory of Herrmann and (28) a student as a control group studied by the usual way for two semesters, a prepared achievement test as article and objective type of multiple choice, the coefficient stability of alpha-Cronbach equation reached (0.86). The research Results showed the presence of a statistically significant d
... Show MoreAbstract
Should be the goal of Government controls, including environmental controls and safety laws to protect citizens from the harmful effects of negative secreted by human's additions and changes in the environment. and perhaps the protection aspects of the protection of citizens from the adverse effects of communications towers, including those produced by towers of health effects. The people the right to choose the nature of the physical environment, which should not be imposed on them by others. Communications towers are one of the main problems that have been
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