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.
This research involves the synthesis of some sulphanyl benzimidazole derivatives (Ia-c), which were prepared from reaction of 2-mercaptobenzimidazole substituted benzyl halide, and structures were identified by spectral methods[FTIR, 1H-NMR and 13C-NMR].These compounds were investigated as corrosion inhibitors for carbon steel in 1M H2SO4 solution using weight loss, potentiostatic polarization methods; obtained results showed that the sulphanyl benzimidazole derivatives retard both cathodic and anodic reactions in acidic media, by virtue of adsorption on the carbon steel surface. This adsorption obeyed Langmuir’s adsorption isotherm. The inhibition efficiency of (Ia-c) ranging between (65-92) %. By using different Ib derivative conc
... Show MoreA paraffin wax and copper foam matrix were used as a thermal energy storage material in the double passes air solar chimney (SC) collector to get ventilation effect through daytime and after sunset. Air SC collector was installed in the south wall of an insulated test room and tested with different working angles (30o, 45o and 60o). Different SC types were used; single pass, double passes flat plate collector and double pass thermal energy storage box collector (TESB). A computational model based on the finite volume method for transient tw dimensional domains was carried out to describe the heat transfer and storage in the thermal energy storage material of collector. Also, equivalent specific heat metho
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... Show MoreThe present study investigated the use of pretreated fish bone (PTFB) as a new surface, natural waste and low-cost adsorbent for the adsorption of Methyl green (MG, as model toxic basic dye) from aqueous solutions. The functional groups and surface morphology of the untreated fish bone (FB) and pretreated fish bone were characterized using Fourier transform infrared (FTIR), scanning electron microscopy (SEM) and Energy dispersive X-ray spectroscopy (EDS), respectively. The effect of operating parameters including contact time, pH, adsorbent dose, temperature, and inorganic salt was evaluated. Langmuir, Freundlich and Temkin adsorption isotherm models were studied and the results showe
The current study aims to demonstrate the role of strategic renewal in enhancing organizational immunity through strategic renewal mechanisms represented by (Reanimating, Rejuvenation, Venturing, and restructuring). The main idea of this study relates to the extent to which organizations’ immunity is achieved through strategic Renewal mechanisms, by measuring these dimensions with Organizational immunity represented by organizational learning, organizational memory, and organizational DNA. The study population was the headquarters of the Iraqi Ministry of Education, and the sample included (330) individuals from the upper, middle, and lower leadership levels. Several statistical methods were used to deal with the results of the questionna
... Show MoreThe research aims to explain the role of cloud computing technology in facilitating the application of international financial reporting standards in Iraqi banks, by preparing a checklist that included a set of paragraphs to measure the level of application of IFRSs. In those banks and then the need to use cloud computing technology, and the researchers reached a set of conclusions, including, that cloud computing technology has a role in facilitating the application of international financial reporting standards in the banks, the research sample, because of the ease it provides in preparing and saving accounting information and the possibility of accessing it in any time and from any place where the Internet service is available. T
... Show MoreThe current research aims to find out the effect of strategic sensitivity in enhancing organizational immunity at the leadership levels in the Iraqi Ministry of Education, as the strategic sensitivity variable includes two dimensions (strategic foresight and strategic Insight), and the organizational immunity variable addresses three dimensions (organizational learning, organizational memory and organizational DNA). The main purpose of this research was related to the extent to which the Ministry’s immunity was achieved through the role played by strategic sensitivity. A sample of (349) individuals was selected . The questionnaire was relied upon to collect data, and the number of questionnaires suitable for analysis was (330). Re
... Show MoreThe techniques of contemporary Iraqi painting and their reflection on the productions of students of art education is an important subject in the field of painting at the theoretical and practical levels in academic study, whether theoretical or practical. Al-Iraqi is one of the arts with historical roots and a distinguished position among other artistic genres. Painting has received a sufficient level of development through the use of various contemporary techniques to advance it for the better.
The methodological framework included the problem of research and the need for it, and then the importance of research came in shedding light on the techniques of contemporary Iraqi painting, and the impact of these techniques on the producti