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 Strait of Malacca is one of the waterways of strategic, political, and economic importance. This strait has an increasing importance to its neighboring countries. The Strait of Malacca is very important for China, especially in the field of energy supply security, as the vast ma jority of energy supplies come to China through this strait. China seeks to ensure its security, especially in light of the cooperation of countries that look forward to this strait as well as the United States, and what the United States may cause to China, as many of them are at war with China over the China Sea, not to mention the countries that overlook it. The importance of the research emerges from the basic idea that the strategic importance of the Strait
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... Show MoreOyster mushroom (Pleurotus ostreatus (Jacq. ex Fr.) P. Kumm.) is involved in the destruction of dead wood which is the main place of settlement of several living organisms. After humification, dead wood also becomes an important component of forest soils.
The purpose of the research is to study temperature and moisture conditions of extensive cultivation of oyster mushrooms on various wood substrates. To accomplish this goal, the following tasks were set: to determine the amount of effective stress temperatures and moisture content of substrates and their influence on the appearance of fruiting bodies of the oyster mushroom; to study the features of the extensive culti
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