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

In this research, a low cost, portable, disposable, environment friendly and an easy to use lab-on-paper platform sensor was made. The sensor was constructed using a mixture of Rhodamine-6G and gold nanoparticles also Sodium chloride salt. Drop–casting method was utilized as a technique to make a platform which is a commercial office paper. A substrate was characterized using Field Emission Scanning Electron Microscope, Fourier transform infrared spectroscopy, UV-visible spectrophotometer and Raman Spectrometer. Rh-6G Raman signal was enhanced based on Surface Enhanced Raman Spectroscopy technique utilized gold nanoparticles. High Enhancement factor of Plasmonic commercial office paper reaches up to 0.9 x105 because of local surface pl

FUZZY CONTROLLERS F'OR SINGLE POINT CONTROLLER-I (SPC-l) SYSTEMS

This work aims to optimize surface roughness, wall angle deviation, and average wall thickness as output responses of ALuminium-1050 alloy cone formed by the single point incremental sheet metal forming process. The experiments are accomplished based on the use of a mixed level Taguchi experimental design with an L18 orthogonal array. Six levels of step depth, three levels of tool diameter, feed rate, and tool rotational speed have been considered as input process parameters. The analyses of variance (ANOVA) have been used to investigate the significance of parameters and the effect of their levels for minimum surface roughness, minimum wall angle deviation, and maximum average wall thickness. The results indicate that step depth and tool r

Research seeks to clarify the role of insurance companies to make great efforts for the success of the operation promotional products and to check it out for, there should be questions selling the company offers to salesmen producers and the latter projecting on the ground to serve customers and deal with them in style to earn satisfaction with.

        The method was used questionnaire and distributed through selected sample included producers in the company and the other sample Staff Planning and marketing researcher used the style gather the necessary information and subjected to analysis using statistical methods arithmetic, standard deviation and concluded search for a set of results which

Buildings begin to deteriorate gradually over time due to several factors, including environmental influences, improper use of the building, and neglected repairs for damages during the building's life span. Effective maintenance practices can minimize operational costs, extend the life of building systems and components, improve energy efficiency, and maintain property value. This paper aims to review articles related to building maintenance to identify factors affecting maintenance practices. After conducting the review, the result was that there were 33 factors affecting building maintenance categorized into six groups: management-related factors, manpower-related factors, technical-related factors, financial-