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.
Abu Firas al- Hamdani is one of the most prominent abbasid poets Who characterized their poetry with high artistic quality especially in his Romyate which blended the quality of feelings of grief, sorrow, longing and nostalgia that makes sensitive self conflict that stems with time from one hand and with place on the other hand. Because we are dealing with a poet lived west spatial coercive which it has been hurt him within a time conflict swinging between despair and some times patience and hope other times between the present and his painful reality also between the past and the beautiful times.
This researcher is to stand on this Romyat by monitoring effectiveness of the poetic text based on conflict between the presenc
Haruki Murakami (1949-present) is a contemporary Japanese writer whose works have been translated into fifty languages and won him plenty of Japanese and international awards. His short stories are well constructed in a weird realistic manner and are mixed with elements of surrealism. His novels and short stories fall under the genre of magical realism. One of the major revolving themes that Murakami wrote about was the haunting feeling of emptiness and disconnectedness in a world which seems to care much for materialism and self-interests.
The paper explores two of Murakami’s short stories in his book After the Quake (2000) and the relevance of their themes and characters to Iraq after the q
... Show MoreThe use of different materials in the art of ceramics is considered a new form of contemporary philosophical trends. That was introduced to art as a result of societal changes and transformations and rapid technological development. And it plays a very essential part in the beauty of the artistic structure. The contrast of using different materials in ceramics is considered a part of the old historical achievements since the art of ceramics used to follow strict standards. Which does not apply to contemporary ceramic, Because recently, it has become free from any religious and social control and created a whole new intellectual context, heading to new horizons in which it works with the newly added materials as a strategy to ensure the a
... Show MoreReceipt date:12/7/2020 accepted date:24/1/2021 Publication date:31/12/2021
This work is licensed under a Creative Commons Attribution 4.0 International License.
The constant characteristic of international relations is the constant change due to political, economic and military developments in addition to technology, and this in turn has led to many transformations in the concept of power, its uses, and the elements that form power and its distribution, and according to those variables, the concept of power has shifted from hard to soft, up to smart powe
... Show MoreThe research aims to measure the impact of the empowerment strategy on the performance of employees by mediating organizational trust in the organization. The problem of research was the weak ability of most business organizations to employ the strategy of empowerment in maximizing the performance of employees, and the importance of research in practice lies in the results of a guide to managers in how to use the strategy of empowerment in maximizing the performance of employees by mediating organizational trust dimensions of ability, benevolence, integrity. In order to achieve the objectives of the research and to answer the questions of the problem, the researcher has adopted the descriptive analytical approach as the questionnaire was
... Show MoreThe advertisement is important in maximizing the resources of journalistic institutions. It helps them to perform their duties and also establishes their independence. However, this type of communication has often overlapped with other types of communication, including media and publicity.
Those in charge of the press bear responsibility in this regard, as some of them harness the advertisement for personal purposes; or detrimental to media content. Many communication authorities have drawn attention to the danger of confusing the concepts and levels of the three activities and have moved towards establishing rules to reduce overlap between them.
The aim of this research is to try to disengage the concept of advertising,
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The research aims to measure, assess and evaluate the efficiency of the directorates of Anbar Municipalities by using the Data Envelopment Analysis method (DEA). This is because the municipality sector is consider an important sector and has a direct contact with the citizen’s life. Provides essential services to citizens. The researcher used a case study method, and the sources of information collection based on data were monthly reports, the research population is represented by the Directorate of Anbar Municipalities, and the research sample consists of 7 municipalities which are different in terms of category and size of different types. The most important conclusion reached by the research i
... Show MoreIn the beta decay process, a neutron converts into a proton, or vice versa, so the atom in this process changes to a more stable isobar. Bethe-Weizsäcker used a quasi-experimental formula in the present study to find the most stable isobar for isobaric groups of mass nuclides (A=165-175). In a group of isobars, there are two methods of calculating the most stable isobar. The most stable isobar represents the lowest parabola value by calculating the binding energy value (B.E) for each nuclide in this family, and then drawing these binding energy values as a function of the atomic number (Z) in order to obtain the mass parabolas, the second method is by calculating the atomic number value of the most stable isobar (ZA). The results show
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