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 study aimed to identify the treatment of the press image of the Great Return Marches in the French international news agency AFP by knowing the most important issues, their direction and the degree of interest in them. The study belongs to the descriptive research, and used the survey method, within the context of the content analysis method, and the researcher relied on the content analysis form tool and the interview tool to collect data. The study population is represented in the photos published by the French News Agency about the Great Return Marches during the period (end of March / 2018 until the end of November / 2019. The researcher chose an intentional sample using the Complete Census method. The study material represented
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This study aims to examine the relationship between emotional intelligence and academic adaptation among a sample of gifted students in intermediate and high schools in Jeddah, Saudi Arabia. The study also seeks to examine the differences between group means in emotional intelligence and academic adaptation due to demographic variables (gender and school level). In addition, the study aims to examine the role of emotional intelligence in predicting the level of academic adaptation. The researcher performed the descriptive, correlational, predictive, and comparative approaches to collect the data from a sample comprised of (309) gifted students using the emotional intelligence scale developed by Bar-on (2000), whi
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This study showed that the lens in baloot muluki fish Chondrostoma regium is transparent, spherical shape, and solid in textures, while in the tree frog Hyla arborea savignyi, freshwater turtles Clemmys caspia caspia, white–eared bulbul Pycnonotus leucotis and brown rat Rattus norvegicus are transparent, soft and biconvex, it is very soft in white–eared bulbul. There are many significant differences have been recorded between the average weight lens and the total concentration of the protein in the lens all studied animals. Electrical migration process for lens proteins showed that there is one bundle of crystalline –? and one bundle also crystalline–? in all studied species, either crystalline–? may represent one bundle character
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Financing is one of the important pillars for activating and activating the agricultural sector, through which we can see an agricultural project on the ground. However, supplying the agricultural sector with financial resources requires a credit policy that is capable of making the right financing decision, because the financial resources are limited. The credit policy, and the financing decision, must be the best use not only to provide the necessary money, but to work to provide everything that would develop and activate the agricultural sector.
The transformation of the Agricultural Cooperative Bank of Iraq from specialized banking to the overall would lead to a decrease in the volume
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