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 research deals with the principle of the prohibition of international waterway diversion in the law of international watercourses. The research reviews individual and collective doctrinal efforts that have touched upon the principle as an internationally wrongful act because of its serious damage and consequences for downstream States. The research addresses the nature of the principle of the prohibition of diversion of international watercourses; its various effects; principles of international law establishing the principle of prohibition of diversion; and its application in State practice and international justice. This principle has been enshrined in most international treaties and judicial decisions. The principle of prohibition
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The Umayyad poets tried to invest all artistic tools in order to achieve a measure of creativity in their texts. The phenomenon of visual composition is breaking the familiar writing system, with the aim of increasing the number of possible connotations. The visual in the Umayyad poetry tries to replace it through expression with the visual image, and its manifestations were manifested by the multiplication of punctuation marks in the body of the poetic text and the tearing of the single poetic line by cutting it into several sentences or repetition.
Keywords: visual formation, poetic writing, Umayyad poetry, recipien
The Impact of Intellectual trends on the nature of the Economic Structure of Iraq
Abstract
The current research aims to identify the level of organizational relaxation among the employees of educational directorates in the Sultanate of Oman. The descriptive approach was used, as well as a questionnaire of two parts to collect data. The questionnaire consists of (15) items divided into three themes about the relaxing level. It was applied on a sample of (406) male & female employees of the educational directorates who were randomly selected. The results of the study showed that the level of organizational relaxation was low. The study results also found that there were no statistically significant differences at the level of significance (α ≤ 0.05) of the study sample in the level of org
... Show MoreThis research aims at studying and analyzing the creative research thinking of the educational staffs in private universities and colleges, and also the role of incentives, in its materialistic and moral sides in the development of such thinking and the pack of restraints which cause decline in interest level of the scientificresearch, and its weak role in the development of universities in Iraq, despite the interest of the Ministry of Higher Education and Scientific Research in Iraq in scientific research as part ofits academic and humanitarian targets.Based on study and analysis, and the using if some of statistical methods such as the Alpha Chronobaghcoefficient and the (T. Test)(F. Test),the research reached a number of conclusions.
... Show MoreA simple, fast, inexpensive and sensitive method has been proposed to screen and optimize experimental factors that effecting the determination of phenylephrine hydrochloride (PHE.HCl) in pure and pharmaceutical formulations. The method is based on the development of brown-colored charge transfer (CT) complex with p-Bromanil (p-Br) in an alkaline medium (pH=9) with 1.07 min after heating at 80 °C. ‘Design of Experiments’ (DOE) employing ‘Central Composite Face Centered Design’ (CCF) and ‘Response Surface Methodology’ (RSM) were applied as an improvement to traditional ‘One Variable at Time’ (OVAT) approach to evaluate the effects of variations in selected factors (volume of 5×10-3 M p-Br, heating time, and temperature) on
... Show MoreLinear regression is one of the most important statistical tools through which it is possible to know the relationship between the response variable and one variable (or more) of the independent variable(s), which is often used in various fields of science. Heteroscedastic is one of the linear regression problems, the effect of which leads to inaccurate conclusions. The problem of heteroscedastic may be accompanied by the presence of extreme outliers in the independent variables (High leverage points) (HLPs), the presence of (HLPs) in the data set result unrealistic estimates and misleading inferences. In this paper, we review some of the robust
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This tribe had lived in Iraq since the first century A.D. Persia tried to
keep this tribe away from Iraq, but without result. The tribe managed to get
victory against Persia in the battle of Dhyqar.
When the Muslims had come to conquer Iraq, Rabi’a welcomed them
and takes part in that action.
That tribe helped the caliph Ali in the Jammal and Sifffen wars in order
to remain Iraq the center of the Islamic caliphates this tribe had felt very sad
and sorrowful when the caliphate become to the Umayyad . This tribe did not
give up, so this tribe did what could be done to help those who had revolted
against the Umayyad. Rabi’a did that in order to get ride of the Mudriat alsham,
and favored Rabi
This research aims to examine the relationship between learning organization and behavior of work teams. The variable of the learning organization took four dimensions depending on the study (sudhartna & Li, 2004): Common cultural values , communication, knowledge transfer and the characteristics of workers. The behavior of teams was identified on the basis of realizing of the respondents of their organization to work as a team where the research relied concepts applied in the study (Hakim , 2005) , and chose to research the case of a service organization for the study and relied on four dimensions of coordination , cooperation , sharing of information , the performance of the team, and was a curriculum approach and des
... Show MoreThis study tackles the contribution of the environmental audit in achieving the sustainability of the environmental balance in the Sudanese business environment. The study aimed at clarifying the role of environmental auditing in supporting sustainable development, and to recognize the role of environmental audit in urging businesses to abide by environmental protection. It also shows how effective the environmental audit reports are, in providing the reliable data for the concerned authorities, in order to use it in making the decisions that would help in the sustainability of the environmental balance. To achieve the goals of the study, a questionnaire was developed and distributed to the registered chartered accountants in Sud
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