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 division partitioning technique has been used to analyze the four electron systems into six-pairs electronic wave functions for ( for the Beryllium atom in its excited state (1s2 2s 3s ) and like ions ( B+1 ,C+2 ) using Hartree-Fock wave functions . The aim of this work is to study atomic scattering form factor f(s) for and nuclear magnetic shielding constant. The results are obtained numerically by using the computer software (Mathcad).
Began the process of re-engineering processes in the private sector as a way to assist organizations in re-thinking how to run the business in order to improve production processes and reduce operational cost, to get to compete on a global level. That was a major restructuring by further evolution in the use of technology to support innovative operations.
Entered the technology in all areas of life and different regulations, This led to use as a change in all aspects The companies achieved success and progress today through the use of resources so as to ensure the wishes of the customers and their needs, and the requirements of the market primarily, Which is reflected on the basis of building strate
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his project try to explain the using ability of spatial techniques for land cover change detection on regional level with the time parameter and did select for explain these abilities study case (Hewaizah marsh ) . this area apply to many big changes with the time. These changes made action on characters and behaviors of this area as well as all activities in it . This Project concerting to recognize the Using importance of remote sensing and GIS Methodology in data collecting for the changes of land use and the methodology for the analyses and getting the results for the next using as a base data for development and drawing the plans as well as in regional planning .This project focus on practical
... Show MoreThe first flow injection spectrophotometric method is characterized by its speed and sensitivity which have been developed for the determination of promethazine-HCl in pure and pharmaceutical preparation. It is based on the in situ detection of colored cationic radicals formed via oxidation of the drug with sodium persulphate to pinkish-red species and the same species was determined by using homemade Ayah 3SX3-3D solar flow injection photometer. Optimum conditions were obtained by using the high intensive green light emitted diode as a source. Linear dynamic range for the absorbance versus promethazine-HCl concentration was 0-7 mmol.L-1, with the correlation coefficient (r) was 0.9904 while the percentage linearity (r2%) was 98.09%. the L.
... Show MoreThe research aims to apply one of the techniques of management accounting, which is the Quality Function Deployment(QFD) on the Pepsi product in Baghdad Soft Drinks Company and to determine the technical requirements objectively that have been applied in practice in Baghdad Soft Drinks Company / a private shareholding company, as it focuses on meeting the quality requirements and achieving positive quality to provide a product It meets the requirements of current and future customers, hence the importance of research that indicates that the Quality Function Deployment(QFD) is a useful tool to develop the requirements of new products, being a design process driven by customers through their voices, and thus contribute to achieve a competi
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Musa son of Yaqoob son of Izra, the Spanish poet, linguist, and philosopher, was born in Granada. He has many poems representing types of poetry. Also, he had important books, such as "the Poetry of Israel" and "Contemplative Research". He is knowledgeable in both Hebrew and Arabic, because most of his books were written in Arabic and the then translated to Hebrew. It seemed that he was influenced by Arab poets, specifically Abo Firas, and borrowed many issues, such as comparison, repetition, exaggeration, etc. and included them in his poetry.
All know that Arabic literature clearly influenced Hebrew literature in the Spanish era. Ara
... Show MoreBackground: The value of lateral cephalometric radiographs to evaluate the pharyngeal airway is limited because it provided 2-dimensional (2D) images of complex 3-dimensional (3D) anatomic structures. Three dimensional analyses of the airway volumes are required to understand oral and pharyngeal adaptations in mouth breathing and nasal breathing subjects. The aim of this study was to measure the pharyngeal airway volume and the size of the face, then compare between pharyngeal airway volume in mouth breathing and nasal breathing subjects and find the gender difference in each group, also to study the relation between pharyngeal airway volume and the size of the face. Material and Methods: Fifty patients including 28 males and 22 females wit
... Show MoreA new method is characterized by simplicity, accuracy and speed for determination of Oxonuim ion in ionisable inorganic acid such as hydrochloric (0.1 - 10) ,Sulphuric ( 0.1 - 6 ),nitric ( 0.1 - 10 ), perchloric ( 0.1 - 7 ), acetic (0.1 - 100 ) and phosphoric ( 0.1 - 30 ) ( mMol.L-1 )acids. By continuous flow injection analysis. The proposed method was based on generation of bromine from the Bro-3-Br-- H3O+. Bromine reacts with fluorescein to quenches the fluorescence . A sample volume no.1 (31μl) and no.2 (35μl) were used with flow rate of 0.95 mL.min-1 using H2O line no.1as carrier stream and 1.3 mL.min-1 using fluorescein sodium salt line no.2. Linear regression of the concentration ( mMol.L-1 ) Vs quenched fluorescence gives a correla
... Show MoreThis research is qualitative in nature. It aims to investigate descriptively, analytically, and comparatively the modern AK model represented by the Sudan Open University Series, and the European framework, the common reference for Teaching Foreign Languages, to uncover what was achieved in them in terms of communication and language use. Accordingly, an integrated, multi-media approach has been adopted to enable the production and reception activities, and the spread of Arabic in vast areas of the world. Such a spread helps Arabic language to be in a hegemonic position with the other living languages. The study is based on getting benefit from human experiences and joint work in the field of teaching Arabic to non-Arabic speakers to mee
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