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.
الوصف In this time, most researchers toward about preparation of compounds according to green chemistry. This research describes the preparation of 2-fluoro-5-(substituted benzylideneamino) benzonitrile under reflux and microwave methods. Six azomethine compounds (B1-6) were synthesized by two methods under reflux and assisted microwave with the comparison between the two methods. Reflux method was prepared of azomethine (B1-6) by reaction of 5-amino-2-fluorobenzonitrile with some aldehyde derivatives with (50–100) mL of absolute ethanol and some quantity of GAA and time is limited between (2–5) hours with a yield between (60–70) percent with recrystallization for appropriate solvents. But the microwave-assisted method was synthe
... Show MoreAutorías: Hadeer Idan Ghanim, Ishraq Mahmood. Localización: Revista iberoamericana de psicología del ejercicio y el deporte. Nº. 3, 2021. Artículo de Revista en Dialnet.
The aimed of the research was recognize the Big five personality factors and Academic procrastination among Baghdad university students, recognized differences between the gender according to Big five personality factors and Academic procrastination , to recognized differences between specialization (scientific, human), and to recognize the relationship Between the variables of the research, and the extend of contribution Big five personality factors in Academic procrastination , to achieve these aims , Adopt scale to measure the Big five personality factors for (John Danahue & Kentle) , As we as the preparation of scale Based on An amber of previous scales to measure Academic procrastination, After processing the data st
... Show MoreThis study aimed to measure the innovative thinking and cognitive cessation among university students. The sample consisted of (400) male and female students at al Mustansiriya University for the academic year (2018/2019). The results of the study showed that there are differences in innovative thinking and cognitive inhibition according to the gender variable in favor of males. There is a positive relationship between innovative thinking and cognitive inhibition. In light of these findings, the researcher presented a set of conclusions and recommendations.
Based on the theoretical ideas of E. Cassirer, a methodology is developed and an analysis of the painting work by J. Saleem, a representative of modern Iraqi painting, is carried out. While comparing the artistic programme of J. Saleem and the views of E. Cassirer, the methodological potential of the theory of the symbol for the art criticism analysis of the regional painting is clarified. Comparative analysis with the inclusion of the works of B. Buffet, B. Turetsky, J. Miro helps to see the multiple meanings of the same cultural symbol in thematically similar works of different years, created in different socio-cultural circumstances. The article shows that for regions with a shorter history (France, Russia, Spain, etc.), which have lo
... Show MoreAnkylosing spondylitis (AS) is a common, highly heritable inflammatory arthritis affecting primarily the spine and pelvis. This study was aimed to investigate the relationship between the rs27044 polymorphism in Endoplasmic reticulum aminopeptidase-1 (ERAP-1) with the susceptibility and severity of AS correlated with some biochemical markers such as hematological parameter (Erythrocytes sedimentation rate (ESR)) and immunological parameters (C-reactive protein (CRP), Human leukocyte antigen-B27 (HLA-B27), Interlukin-6 (IL-6) and Interlukin-23 (IL-23)), and oxidative stress parameters (Glutathione (GSH) and Malondialdehyde (MDA)) in a sample of Iraqi population. A total of 60 blood samples were collected from AS patients requited Rhe
... Show MoreMicrobial fuel cell is a device that uses the microorganism metabolism for the production of electricity under specific operating conditions. Double chamber microbial fuel cell was tested for the use of two cheap electrode materials copper and aluminum for the production of electricity under different operating conditions. The investigated conditions were concentration of microorganism (yeast) (0.5- 2 g/l), solutions temperature (33-45 oC) and concentration of glucose as a substrate (1.5- 6 g/l). The results demonstrated that copper electrode exhibit good performance while the performance of aluminum is poor. The electricity is generated with and without the addition of substrate. Addition of glucose substrate
... Show MoreThis research aims to present a proposed model for disclosure and documentation when performing the audit according to the joint audit method by using the questions and principles of the collective intelligence system, which leads to improving and enhancing the efficiency of the joint audit, and thus enhancing the confidence of the parties concerned in the outputs of the audit process. As the research problem can be formulated through the following question: “Does the proposed model for disclosure of the role of the collective intelligence system contribute to improving joint auditing?”
The proposed model is designed for the disclosure of joint auditing and the role
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Idiomatic expressions in Russian journalism make one important borrowed means for making a dialogue with the receiver's intellect in so far as it has the distinct feature of having clarity and exactness of meaning. The meaning is seen as a shortcut for covering a series of concepts and details so as to arrive at the intended meaning. This is done by stimulating the reader by the use of certain clear idioms. The use of such idioms in a journalistic text is not for a linguistic purpose only, but it is a cultural and social phenomenon reflecting the type of current changes in the society and it aims at discoursing with the reader's mind. This paper is a practi
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