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 aims to demonstrate the quality of the auditor’s report by analyzing a number of models represented by the auditor’s report based on the Iraqi audit evidence and the auditor’s report in light of the application of international auditing standards for the report and the auditor’s report in light of the application of the International Auditing Standard 701, in addition to explaining the impact of applying the International Auditing Standard 701. International Auditing 701 in enhancing the quality of the auditor's report, and in order to reach these goals, a comparison was made on international experiences before and after the application of the standard in addition to the results of the questionnaire distribut
... Show MoreThe path and its nodal purposes
Abstract
This study has come to indicate the doctrinal purposes of faith in this path، It was composed of an introduction and four demands، as follows:
The first requirement: the intentions of distinguishing between the ranks of people according to divine justice، as those who pass by him are different. Some of them are safe by his work، surviving from the fire، and some of them are not peace، he falls into the fire of Hell، and all of them are different in their ranks in a manner consistent with the justice of God Almighty and the works they have given themselves.
The second requirement: the purposes of divine mercy in honoring the Prophet and his nation on the path and embodying their
Abstract
This study aims to identify the degree to which the first cycle teachers use different feedback patterns in the e-learning system in addition to the differences in the degree of use according to specialization, teaching experience, and in-service training in the field of classroom assessment, as well as the interaction between them. The study sample consisted of (350) female teachers of the first cycle in government schools in Muscat Governorate for the academic year 2020/2021. The study used a questionnaire that contained four different patterns of feedback, which are reinforcement, informative, corrective, and interpretive feedback. The psychometric properties of the que
... Show MoreThe Internet has added another dimension to public relations in institutions and organisations, as it provided tools and communication channels, especially social networking sites, which provided information and data on public relations for the institution through these websites. In addition to its communication with its audience, and the audience's interaction with it, so our research tagged (the effectiveness of public relations of the Sunni Endowment Diwan through social networking sites): An analytical study of the official Facebook page of the Diwan that addresses the knowledge and monitoring of the contents of the official Facebook page that public relations adopt in providing information, data, and activities of the Sunni End
... Show MoreThe main idea of this research stems from the challenges faced by managers working in service organizations, which are responsible for providing services to a large segment of the society. Therefore, they must use strategic intelligence to manage their performance and enhance their performance to serve the society. Organizational Performance The present study aimed to identify the concepts of strategic intelligence and its impact on organizational performance to raise awareness and awareness of the importance of the subject for the university. And to identify the perceptions of leaders about strategic intelligence and determine the nature of the relationship (impact and correlation) between strategic inte
... Show MoreThe research focuses on addressing one of the most important concepts of modern management that deal with the relationship between government institutions and stakeholders, Which now occupies a special significance in trends modern management, a concept of corporate governance it takes the internal mechanisms due to its key role and is important in the process of corporate governance and looking for building Effective mechanisms for this concept in light of laws and regulations that are available in the local environment and on the functioning of government institutions, Search has been applied in the Federal Board of Supreme Audit of Iraq (BSA) due to its particular importance as an institution control and as a awaits the societ
... Show MoreThis study was conducted to prepare protein concentrates from AL-Zahdidate’s pits by using alkaline methods where the chemical composition of the pits were (7.30, 1.04, 5.80, 8.68 and 77.19) % for each of the moisture, ash, protein, fat and carbohydrates respectively and the chemical composition of the concentrate protein was (6.62, 4.10, 26.70, 0.93, and 58.65) % respectively. The content of protein concentrate from the metallic elements (144.07, 25.11, 15.02, 0.49, 0.59, 0.27, 0.22 and 234.6) mg/ 100 g each of potassium, magnesium, calcium, iron, manganese, copper, zinc and phosphorus respectively. The results of SDS-PAGE showed five bands with weights molecular ranged between 11000-70000 Dalton. Give the biscuit which contain protei
... Show MoreThe relationship between the elements of the climate and the productivity of citrus (kg
/ tree) in the province of Karbala has been determined through the use of simple correlation
coefficient of Pearson (rp) and (t) test. The result for each of oranges, limes and tangerine had
all shown moral and relevant statistical indications; except for relative humidity, which were
not linked to a moral relationship with productivity of tangerine, oranges and limes.As for the
relationship between climatic factors and yield of bitter orange were all not significant
statistically and very weak.
In order to determine the strength of the correlation between fluctuations in the
climatic elements and fluctuations in the productivi
The current research deals with the specificity of the interior design of the classes of art education in the schools of excellence for the province of Muthanna and its center of the city of Samawah. In the first chapter، the problem of research was determined by the lack of internal spaces of the classes of art education to designs that meet the requirements and needs of students in these educational stages. The importance of the research in being an addition to knowledge in the field of interior design and engineering department of the general directorates of the Ministry of Education. The objectives of the research were determined through.
- Identifying the reality of the halls of art education for sch
this research seeks to shed light on the study of the impact of Putinism on the Russian-Ukrainian war that broke out in early 2022, by studying the contents of the speeches of Russian President Vladimir Putin before the start of the war. This research uses the structural approach in analyzing the components of the Russian political regime. The research tools used in this research include case studies, content analysis of Russian speeches, and personal meetings of researchers and specialists in Russian affairs. It sums up a number of important conclusions, most notably are : 1) Putinism is a totalitarian regime that includes the political doctrine during the era of Russian President Vladimir Putin. 2) Duginism represents the totality of t
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