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 influence of sensing element length of no-core fiber strain sensor has been studied and experimentally demonstrated, four different lengths of 125 μm diameter no-core fiber is fused between two standard single-mode fibers and bi-directionally strained, the highest obtained sensitivity was around 16.37 pm με -1 which was exhibited in the shortest no-core fiber segment, to the best of our knowledge this is the first study of the influence of no-core fiber strain sensors length on sensor sensitivity. The proposed sensor can be used in many opto-mechanical applications such as, structural health monitoring, aerospace vehicles and airplane components monitoring.
This article explores the role of talk shows in setting the public's agenda regarding political mobilization in Iraq. The researcher designed a questionnaire that contains both closed and open-ended questions to allow respondents to express their opinions more freely.
The researcher chose a phased purposive sample consisting of 294 respondents. The data was then subjected to analysis and verification using the Statistical Package for the Social Sciences (SPSS).
Fast economical developments in the US. in second half of nineteenth century contained many social results, such as increasing of stealing and killing crimes and expand of Labor strikes, which resulted in violence actions, and presented acceptable reason for emergence specialist security agencies, first of them "Pinkerton Agency". which played important role in all of that, besides that .. its role in American Civil War such as discovering an attempt to kill elected republican president Abraham Lincoln. This research studied all that aspects, focused on period of 1850 - 1899 of agency's history.
Abu Al-Ala Al-Ma’arri is a linguist and writer who showed his prowess in poetry, so he had poetry collections, including “The ulna fell,” which is one of the books that attracted the attention of scholars such as (Tabrizi, Al-Khwarizmi, Al-Bateliusi and Al-Zamakhshari) .
To explain his language in it, the investigators’ hands finally revealed an explanation of this book, which was the work of Abu Al-Ala Al-Ma’arri himself. Those who studied the Divan (The Zind) and its explanations or the character and language of Al-Ma’arri did not pay any attention to it. Therefore, I made this research a continuation of those efforts, using semantic research as a means of revealing Al-Maarri's ef
... Show MoreAl Huweizah Marsh is considered as the largest marsh at the southern part of Iraq. About one third of the marsh is located within the Iranian territory. Iran began to construct earth dikes along the Iraqi-Iranian international borders to separate the Iranian part of the marsh. The electrical conductivity, EC, value was adopted to be the indicator for the water salinity within the marsh. A steady two-dimensional water quality routing model was implemented by using the RMA2 and RMA4 softwares within the SMS computer package to estimate the distribution of the
EC values within the marsh seasonally during the wet, moderate and dry water years. The EC distribution Patterns were estimated considering the expected two cases of the marsh futu
ABSTRACT
The purpose of this research is to investigate the relationship between organizational ethnography and resonant leadership in the University of Kufa, importance of research includes rare of studies that investigated the relationship between organizational ethnography and resonant leadership. Moreover, it comes as an attempt to reduce or bridge the knowledge gap between the variables of the research. As well as try to put the right foundations and appropriate for industry leaders of various Iraqi organizations aimed at profit or service. Based on the random sampling, 450 questionnaires were distributed to employees at the University of Kufa. Only 392 questionnaires were returned. Out of which 104 questionnaires we
... Show MoreThe study aimed to measure the effect of applying the disclosure and transparency standards criteria adopted by the Saudi Arabian Monetary Authority on improving performance indicators in the Saudi banking sector, by measuring the extent of the impact of the bank's financial indicators represented by liquidity, profitability and return on assets in Saudi banks by applying the criteria of disclosure and transparency, which is one of the Main principles in the list of governance, which was approved by the Saudi Arabian Monetary Authority. The analytical approach was followed to achieve the goal of the study, as the financial statements of Saudi banks were analyzed during a period of 8-year to test four hypotheses related to measuri
... Show MoreThe study seeks to analyze the perceptions of audience in UAE towards the performance of Emirates Satellite TV Channels. It analyzed the exposure motivations of audience to satellite TV channels, its positive and negative aspects and to what extent they abide by media ethics. A survey is conducted with a sample of four hundred. The study shows significant differences between male and female towards the characteristics of TV channels, its positive and negative aspects and its commitments to media ethics.
The study also shows that the expectancy value model and third person effect model are applicable in studying the perceptions of audience and media people in UAE t
... Show MoreThe aim of this study was to propose and evaluate an eco-epidemiological model with Allee effect and nonlinear harvesting in predators. It was assumed that there is an SI-type of disease in prey, and only portion of the prey would be attacked by the predator due to the fleeing of the remainder of the prey to a safe area. It was also assumed that the predator consumed the prey according to modified Holling type-II functional response. All possible equilibrium points were determined, and the local and global stabilities were investigated. The possibility of occurrence of local bifurcation was also studied. Numerical simulation was used to further evaluate the global dynamics and the effects of varying parameters on the asymptotic behavior of
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