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 inflammator

A simple and novel method was developed by combination of dispersive liquid-liquid microextraction with UV spectrophotometry for the preconcentartion and determination of trace amount of malathion. The presented method is based on using a small volume of ethylenechloride as the extraction solvent was dissolved in ethanol as the dispersive solvent, then the binary solution was rapidly injected by a syringe into the water sample containing malathion. The important parameters, such the type and volume of extraction solvent and disperser solvent, the effect of extraction time and rate, the effect of salt addition and reaction conditions were studied. At the optimum conditions, the calibration graph was linear in the range of 2-100 ng mL-1 of ma

The relationship between costs of environment and costs of product life – cycle.    Boubtlessly when the economical unit exercise their productive works, they lead to pollution in water, air and soil as well as all stages of product life- cycle from Rans Dstage, production stage, packaging stage and finally abandonment stage-             Pollution causes environmental costs. Lgnoring or hiding environmental costs and no taking them in consideration with product cost lead to a wrong account of preduot cost.

Therefore, environmental costs should be included and matched for all stages with in product costs to know which activities, processes

Many isolated rural communities are located in regions where there is an abundant and reliable supply of solar energy, but where the distance to the nearest power station is many tens or even hundreds of kilometre. It is therefore mainly in these areas that rural electrification is now being provided by PV generators. since Stand-Alone PV generator can offer the most cost-effective and reliable option for providing power needed in remote places. Accordingly these isolated rural canters are fitted with PV for lighting, a refrigerator, a television and socket to supply kitchen appliances

The present study employed the NAG-4SX3-3D analyzer to precisely measure the energy response of the sensor. The goal was to enhance the understanding of this technology by providing expert information about the device. This technology offers an economical, quick, accurate, and sensitive approach. By utilizing the turbidity method, Cyproheptadine hydrochloride (CPH) was quantified in pharmaceutical samples without the need for additional substances. CPH is expected to undergo a direct reaction with calcium hexacyanoferrate, resulting in the formation of white precipitates. The linear range for CPH measurement falls within the range of (0.008–30) mM. The relative standard deviation (RSD) for six repetitions at concentrations of (6 and

Chilled ceilings systems offer potential for overall capital savings. The main aim of the present research is to investigate the thermal performance of the indirect contact closed circuit cooling tower, ICCCCT used with chilled ceiling, to gain a deeper knowledge in this important field of engineering which has been traditionally used in various industrial & HVAC systems. To achieve this study, experimental work were implemented for the ICCCCT use with chilled ceiling. In this study the thermal performances of closed wet cooling tower use with chilled ceiling is experimentally and theoretically investigated. Different experimental tests were conducted by varying the controlling parameters to investigate their effects

     A new colorimetric-flow injection method has been developed and validated for the detection of Cefotaxime sodium in pharmaceutical formulations. This method stands out for its rapid and sensitive nature. The formation of a brown-colored complex between Cefotaxime sodium and the Biuret reagent in a highly alkaline environment serves as the basis for the detection. The intensity of this colored complex is measured using a custom-built Continuous Flow Injection Analyzer, enabling accurate quantification of Cefotaxime sodium. Optimization studies of the chemical and physical parameters such as dilution of Biuret reagent, effect of the medium basicity, flow rate, sample loop and others have been investigated. The calibration gra

The residual limb within the prosthesis, is often subjected to tensile or fatigue stress with varying temperatures. The fatigue stress and temperatures difference which faced by amputee during his daily activities will produces an environmental media for growth of fungi and bacteria in addition to the damage that occurs in the prosthesis which minimizingthe life of the prosthetic limb and causing disconfirm feeling for the amputee.

In this paper, a mechanical and thermal properties of composite materials prosthetic socket made of different lamination for perlon/fiber glass/perlon, are calculated by using tesile test device under varying temperatures ( from 20oC to 60oC), also in this paper a device for measuring rotational bendin

The sports institutions in general are affected and contact with sport in particular the environmental factor, whether political or economic, which makes them in constant need to consider their administrative applications to increase the confidence of their employees because of their suitability or consistency with the new reality according to the sports activities that relate to it, The stalemate in administrative and technical aspects of the administrative work method in the majority of the Olympic sports federations makes the achievement of most of the goals far from the present reality, and the selection of suitable alternatives to achieve the objectives by those who disagree with the concepts of modern dictatorial standards It leads to