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.
Most of the Internet of Things (IoT), cell phones, and Radio Frequency Identification (RFID) applications need high speed in the execution and processing of data. this is done by reducing, system energy consumption, latency, throughput, and processing time. Thus, it will affect against security of such devices and may be attacked by malicious programs. Lightweight cryptographic algorithms are one of the most ideal methods Securing these IoT applications. Cryptography obfuscates and removes the ability to capture all key information patterns ensures that all data transfers occur Safe, accurate, verified, legal and undeniable. Fortunately, various lightweight encryption algorithms could be used to increase defense against various at
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... Show MoreBegan 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 MoreA theoretical calculation of the binding and excitation energies have been used at low – lying energies based on shell model and quantum theory. In this model, we evaluated the energies under assume Ni 28 56 30 as inert core with two nucleon extra, nucleons in the 2P3/2 , 1f 5/2 and 2P1/2 configuration. Modified Surface Delta Interaction (MSDI) and Reid's Potential (RP) theory for two body matrix elements are evaluated by using a Matlab program to calculate the energies of experimental and Reid single particle energies. Our results of the theoretical calculation have been compared with the experimental results, which show no good agreement with the experiment but have a good agreement wit
... Show MoreSatire is genre of the literary arts that has always been the source of human interest. Because it is difficult to accept direct criticism, Satire appears as a literary tool in which vices, follies, abuses and shortcomings are held up to ridicule, with the intent of shaming individuals, corporations, government, or society itself into improvement. A satirical critic usually employs irony to attain this goal. Although satire is usually meant to be humorous, its greater purpose is often profitable social criticism, using wit to draw at
... Show MoreThe objective of the research is to identify the effect of an instructional design according to the active learning modelsالباحثين in the achievement of the students of the fifth grade, the instructional design was constructed according to the active learning models for the design of education. The research experience was applied for a full academic year (the first & the second term of 2017-2018). The sample consisted of 58 students, 28 students for the experimental group and 30 students for the control group. The experimental design was adopted with partial and post-test, the final achievement test consisted of (50) objectives and essays items on two terms, the validity of the test was verified by the adoption of the Kudoric
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