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.
This paper presents two main parts: The first part involves manufacturing the specimens form composite material for mechanical testing (tensile, flexural and fatigue tests), then design a custom foot orthesis (CFO) and manufacturing from composite lamination (3nylglass 2carbon fiber 3nylglass) for patient suffer from flexible flat foot since birth and over-pronation. The second part of this research involves a design a model of custom foot orthesis in (solid work 2018) and then analysis of custom foot orthosis in engineering analysis program (ANSYS V.18.2).The applied pressure in boundary condition adopted from Force Sensor Resistance (FSR 402 ) in various regions in foot after wearing composite CFO. Used a composite materials in engineerin
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Longer follow-up defense , one of basketball skills that require the team collective action involving active part and consistent to acquire bouncing balls even not be a chance for members of the team striker acquisition rebounding from the target area and bring it back again , which reduces the chances of scoring, and it enables team members defender of the performance of fast attack and score points for being the increase your chances of success.In light of the foregoing, reflected the importance of research in achieving the objective basis of skill tests that require a circumstance similar to the circumstances of the game with the standard operating procedures for the registration, and that the validity judged by consistency between tests
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Capillary pressure is a significant parameter in characterizing and modeling petroleum reservoirs. However, costly laboratory measurements may not be sufficiently available in some cases. The problem amplifies for carbonate reservoirs because relatively enormous capillary pressure curves are required for reservoir study due to heterogeneity. In this work, the laboratory measurements of capillary pressure and formation resistivity index were correlated as both parameters are functions of saturation. Forty-one core samples from an Iraqi carbonate reservoir were used to develop the correlation according to the hydraulic flow units concept. Flow zone indicator (FZI) and Pore Geometry and Structure (PGS) approaches were used to identify
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