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.
New nitrone and selenonitrone compounds were synthesized. The condensation method between N-(2-hydroxyethyl) hydroxylamine and substituted carbonyl compounds such as [benzil, 4, 4́-dichlorobenzil and 2,2́ -dinitrobenzil] afforded a variety of new nitrone compounds while the condensation between N-benzylhydroxylamine and substituted selenocarbonyl compounds such as [di(4-fluorobenzoyl) diselenide and (4-chlorobenzoyl selenonitrile] obtained selenonitrone compounds. The condensation of N-4-chlorophenylhydroxylamine with dibenzoyl diselenide obtained another type of selenonitrone compounds. The structures of the synthesized compounds were assigned based on spectroscopic data (FT-IR,
... Show MoreIn this research, the structural behavior of reinforced concrete columns made of normal and hybrid reactive powder concrete (hybrid by steel and polypropylene fibers) subjected to chloride salts with concentration was 8341.6 mg/l. The study consists of two parts, the first one is experimental study and the second one is theoretical analysis. Three main variables were adopted in the experimental program; concrete type, curing type and loading arrangement. Twenty (120x120x1200) mm columns were cast and tested depending on these variables. The samples were reinforced using two different bars; Ø8 for ties and Ø12 with minimum longitudinal reinforcement (0.01Ag). The specimens were divided into two main groups based o
... Show MoreDental caries (tooth decay) is one of the most prevalent infectious disease and although of multifactorial origin, Streptococcus mutans is considered the principal pathogen in its development (i.e. bacterial processes damage hard tooth structure (enamel, dentine and cementum), producing dental cavities (holes in the teeth). The bactericidal properties of the Nd:YAG laser has been researched analyzing its use in caries prevention and bacterial reduction. One hundred twenty five samples were collected from carious teeth and isolated bacteria were diagnosed using microscopic examination, culture, biochemical tests, and Api 20 strep system. The results of this study showed that a noticeable decrease in the viability of Streptococcus mutans w
... Show MoreThis study aimed at evaluating the torsional capacity of reinforced concrete (RC) beams externally wrapped with fiber reinforced polymer (FRP) materials. An analytical model was described and used as a new computational procedure based on the softened truss model (STM) to predict the torsional behavior of RC beams strengthened with FRP. The proposed analytical model was validated with the existing experimental data for rectangular sections strengthened with FRP materials and considering torque-twist relationship and crack pattern at failure. The confined concrete behavior, in the case of FRP wrapping, was considered in the constitutive laws of concrete in the model. Then, an efficient algorithm was developed in MATLAB environment t
... Show MoreBackground: The longevity of any prosthesis depends on the materials from which it was fabricated, that is why, defects in the material properties may reduce the service life of prosthesis and necessitate its replacement. The aim of this study was to evaluate the effect of adding different concentrations of Polyamide-6 (Nylon-6) on the tear and tensile strength of A-2186 RTV silicone elastomer. Materials and Methods: 80 samples were fabricated by the addition of 0%, 1%, 3% and 5% by weight PA-6 micro-particles powder to A-2186 platinum RTV silicone elastomer. The study samples were divided into four (4) groups, each group containing 20 samples. One control group was prepared without PA-6 micro particles and three experimental groups were pr
... Show MoreThis new azo dye 7-(3-hydroxy-phenylazo)-quinoline-8-ol was subsequently used to prepare a series of complexes with the chlorides of Fe, Co, Zn, Ru, Rh and Cd. The compounds identified by 1H and 13C-NMR, FT-IR, UV-Vis, mass spectroscopy, as well as TGA, DSC, and C.H.N., conductivity, magnetic susceptibility, metal and chlorine content. The results showed that the ligand behaves in a trigonal behavior, and that the complexes gave tetrahedral, except for Fe, Ru and Rh octahedral was given, that all of them are non-electrolytes. The effectiveness of both the compounds in inhibiting free radicals was evaluated by the ability to act as an antioxidant was measured using DPPH as a free radical and gallic acid as a standard substance, the
... Show MoreThis paper presents a point multiplication processor over the binary field GF (2233) with internal registers integrated within the point-addition architecture to enhance the Performance Index (PI) of scalar multiplication. The proposed design uses one of two types of finite field multipliers, either the Montgomery multiplier or the interleaved multiplier supported by the additional layer of internal registers. Lopez Dahab coordinates are used for the computation of point multiplication on Koblitz Curve (K-233bit). In contrast, the metric used for comparison of the implementations of the design on different types of FPGA platforms is the Performance Index.
The first approach attains a performance index
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