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.
Biological drugs have an active substance that is made by a living organism or derived from a living organism. They are one of the important therapy options used in a wide range of diseases especially life-threatening diseases. Biological therapy opens new opportunities for treating different diseases for which drug therapy is minimal, but they have considerable differences in the safety consequences in comparison with non-biological drugs. The aim of the current study was to assess the post-marketing safety profile of biological drugs used in Iraqi hospitals by the analysis of the reported adverse drug reactions regarding their severity, seriousness, preventability, expectedness, and outcome. It is a retrospective study of the individu
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The article critically analyzes traditional translation models. The most influential models of translation in the second half of the 20th century have been mentioned, among which the theory of formal and dynamic equivalence, the theory of regular correspondences, informative, situational-denotative, functional-pragmatic theory of communication levels have been considered. The selected models have been analyzed from the point of view of the universality of their use for different types and types of translation, as well as the ability to comprehend the deep links established between the original and the translation.
Аннотация
Politeness strategies are of significant importance to maintain the face of the addressee. Senders of formal congratulatory letters seek to create a positive image in the minds of their addresses by performing particular illocutionary acts and face-saving acts (FSAs) in the form of written texts. To the best knowledge of the researcher, this topic received little attention from linguistic researchers, especially on the pragma-stylistic level. The importance of this study arises from the fact that congratulatory formal letters are an effective tool in the successful performance of foreign relations and thus deserve investigation. The current study investigates the pragmastylistic aspects of illocutionary acts and FSA Politeness Stra
... Show MoreThe provided research paper offers a thorough analysis of the semiotic analysis present in tobacco-free initiative advertisements from the year 2021. The study delves into the intricate process of decoding the diverse signs, symbols, and visual components integrated into these anti-smoking campaigns. The core aim of this investigation is to comprehend and explore the semiotic tactics that underlie these advertisements, with a particular emphasis on visual communication as a pivotal tool in shaping the public's attitudes and behaviors towards tobacco usage. The research introduces a significant theoretical framework, the "Taxonomy of Image-Text Relations and Functions" theory, as proposed by Emily E. Marsh and Marilyn Dom
... Show MoreIn this paper, the Active Suspension System (ASS) of road vehicles was investigated. In addition to the conventional stiffness and damper, the proposed ASS includes a fuzzy controller, a hydraulic actuator, and an LVDT position sensor. Furthermore, this paper presents a nonlinear model describing the operation of the hydraulic actuator as a part of the suspension system. Additionally, the detailed steps of the fuzzy controller design for such a system are introduced. A MATLAB/Simulink model was constructed to study the proposed ASS at different profiles of road irregularities. The results have shown that the proposed ASS has superior performance compared to the conventional Passive Suspension System (PSS), where the body displacemen
... Show MoreThe present study was conducted as atrial to treat secondary hydatidosis in white mice of species Mus musculus. (Balb/c strain), experimentally infected with secondary hydatid cysts of sheep origin. The immunization process was carried by using CF/PS antigen which consist hydatid cyst fluid (CF) antigen, and protoscolices (PS) antigen mixed together and by using two concentrations 7.5, 15 mg/ml. Then derivatives of Benzimidazole, Mebendazole (M) and drugs Albendazole (A) were used 10 and 40 μm/gm body weight respectively. The mixture of drugs was used one week after challenged dose. Criteria taken into consideration to define the activity of the immunization and chemotherapy in this study were reduction in the numeral of cysts, the coeffic
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