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.
Support vector machine (SVM) is a popular supervised learning algorithm based on margin maximization. It has a high training cost and does not scale well to a large number of data points. We propose a multiresolution algorithm MRH-SVM that trains SVM on a hierarchical data aggregation structure, which also serves as a common data input to other learning algorithms. The proposed algorithm learns SVM models using high-level data aggregates and only visits data aggregates at more detailed levels where support vectors reside. In addition to performance improvements, the algorithm has advantages such as the ability to handle data streams and datasets with imbalanced classes. Experimental results show significant performance improvements in compa
... Show MoreIn this research, we have added nano anatase TiO2 as a partial replacement of Portland cement by a weight percentage of (0.25 to 1%) for the development of properties for protection against bacteria. The control mix was made by using "the cement to sand" proportion about (1: 2.75) with the "water to cement" proportion of (0.5) to study the structure, porosity, water absorption, density, mechanical properties, as well as anti-bacterial behavior. Inspections have been done such as scanning electron microscopy (SEM), and atomic force microscope (AFM) for mortar. Experimental results showed that after the addition of Nano powders in cement mortar, the structural properties improved significantly with the development of hydration o
... Show MoreThe operating characteristics of optoelectronic devices depend critically on the properties physical of the constituent materials, interesting compound has been focused on this research formed from group III and V of the periodic table. Thin film n-InSb heterjuntion were successfully fabricated on p-Si substrates by thermal evaporation technique at different annealing temperature (as prepared, 400,500,600) °C. The effect of annealing temperature on the structural, surface morphology, optical and optoelectronic properties of InSb films were investigated and studied. The crystal structure of the film was characterized by X-ray diffraction and techniques. AFM techniques inspect the surface morphology of InSb films, the study presented the val
... Show MoreConducted two trials separate plants Defla first two seasons, 1998 and 1999 to test the susceptibility Altgveria three varieties including Azharha colored white and pink Qati and pink Qtmr and second seasons 1999 and 2000, two types color Azhaarhama white and pink Qati treated mind half-timbered two types of Alaoxinat IBA and NAA and three concentrations as well as repeatersAdhrt results low Almaah rooting
Well-dispersed Cu2FeSnSe4 (CFTSe) nanoparticles were first synthesized using the hot-injection method. The structure and phase purity of as-synthesized CFTSe nanoparticles were examined by X-ray diffraction (XRD) and Raman spectroscopy. Their morphological properties were characterized by scanning electron microscopy (SEM) and transmission electron microscopy (TEM). The average particle sizes of the nanoparticles were about 7-10 nm. The band gap of the as-synthesized CFTS nanoparticles was determined to be about 1.15 eV by ultraviolet-visible (UV-Vis) spectrophotometry. Photoelectrochemical characteristics of CFTSe nanoparticles were also studied, which indicated their potential application in solar energy water splitting.
In this work we investigate and calculate theoretically the variation in a number of optoelectronic properties of AlGaAs/GaAs quantum wire laser, with emphasis on the effect of wire radius on the confinement factor, density of states and gain factor have been calculated. It is found that there exist a critical wire radius (rc) under which the confinement of carriers are very weak. Whereas, above rc the confinement factor and hence the gain increase with increasing the wire radius.
The efficiency of internal combustion engines (ICE) is usually about thirty percent of the total energy of the fuel. The residual energy is lost in the exhaust gas, the lubrication, and the cooling water in the radiators. Recently much of the researcher’s efforts have focused on taking advantage of wasted energy of the exhaust gas. Using a thermoelectric generator (TEG) is one of the promising ways. However, TEG depends entirely on the temperature difference, which may be offered by the exhaust muffler. An experimental test has been conducted to study the thermal performance of a different muffler internal design. The researchers resort to the use of lost energy in an ICE using TEG, which is one of the ways to take adv
... Show MoreSCADA is the technology that allows the operator to gather data from one or more various facilities and to send control instructions to those facilities. This paper represents an adaptable and low cost SCADA system for a particular sugar manufacturing process, by using Programmable Logic Controls (Siemens s7-1200, 1214Dc/ Dc/ Rly). The system will control and monitor the laboratory production line chose from sugar industry. The project comprises of two sections the first one is the hardware section that has been designed, and built using components suitable for making it for laboratory purposes, and the second section was the software as the PLC programming, designing the HMI, creating alarms and trending system. The system will ha
... Show MoreThere are many different methods for analysis of two-way reinforced concrete slabs. The most efficient methods depend on using certain factors given in different codes of reinforced concrete design. The other ways of analysis of two-way slabs are the direct design method and the equivalent frame method. But these methods usually need a long time for analysis of the slabs.
In this paper, a new simple method has been developed to analyze the two-way slabs by using simple empirical formulae, and the results of final analysis of some examples have been compared with other different methods given in different codes of practice.
The comparison proof that this simple proposed method gives good results and it can be used in analy
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