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.
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This study examined >140 relevant publications from the last few years (2018–2021). In this study, classification was reviewed depending on the operation's progress. Electrocoagulation (EC), electrooxidation (EO), electroflotation (EF), electrodialysis (ED), and electro-Fenton (EFN) processes have received considerable attention. The type of action (individual or hybrid) for each electrochemical procedure was evaluated, and statistical analysis was performed to compare them as a new manner of reviewing cited papers providing a massive amount of information efficiently to the readers. Individual or hybrid operation progress of the electrochemical techniques is critical issues. Their design, operation, and maintenance costs vary depending o
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The research revolves around the collection of pending hadiths on Abdullah bin Amr bin Al-Aas, may God be pleased with them, and it is required in these hadiths that they be among those in which there is no room for diligence, such as telling about unseen matters, signs of the Hour, or a statement of virtue and reward for obedience, or punishment for disobedience.
The research consists of an introduction, two topics, and a conclusion of the first topic in the translation of Abdullah bin Amr bin Al-Aas, and the second topic, the pending hadiths of Abdullah bin Amr bin Al-Aas, may God be pleased with them, in which there is no room for diligence in collecting and studying
At the end of this rese
The research dealt with the design of the cost accounting system for the transport service and its Role in improving the efficiency of pricing decisions through the application of the cost system based on ABC activities. The main activities were defined and cost guides were to measure the cost of each service and to determine the cost of each service for the purpose of providing management with appropriate information and pricing decisions The problem of research in the lack of adoption by some public companies in the service sector on the cost accounting system to calculate the cost of service as well as the lack of identification of productive activities and service activities and therefore cannot make the appropriate decision t
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