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

Circular data (circular sightings) are periodic data and are measured on the unit's circle by radian or grades. They are fundamentally different from those linear data compatible with the mathematical representation of the usual linear regression model due to their cyclical nature. Circular data originate in a wide variety of fields of scientific, medical, economic and social life. One of the most important statistical methods that represents this data, and there are several methods of estimating angular regression, including teachers and non-educationalists, so the letter included the use of three models of angular regression, two of which are teaching models and one of which is a model of educators. ) (DM) (MLE) and circular shrinkage mod

In this research tri metal oxides were fabricated by simple chemical spray pyrolysis technique from (Sn(NO₃)₂.20 H₂O, Zn(NO₃)₂.6 H₂O, Cd(NO₃)₂.4 H₂O) salts at concentration 0.1M with mixing weight ratio 50:50 were fabricated on silicon substrate n-type (111). (with & without the presence of grooves by the following diemensions (20μm width, 7.5μm depth) with thickness was about ( 0.1 ±0.05 µm) using water soluble as precursors at a substrate temperature 550 ºC±5, with spray distance (15 cm) and their gas sensing properties toward H₂S gas at different concentrations (10,50,100,500 ppmv) in air were investigated at room te

Reaxys Chemistry database information SciVal Topics Metrics Abstract A novel CoO–ZnO nanocomposite was synthesized by the photo irradiation method using a solution of cobalt and zinc complexes and used as a coating applied by electrophoretic deposition (EPD) for corrosion protection of stainless steel (SS) in saline solution. The samples were characterized using powder XRD, scanning electron microscopy (SEM) and electrochemical polarization. It was also found that the coating was still stable after conducting the corrosion test: it contained no cracks and CoO–ZnO nanocomposites clearly appeared on the surface. SEM showed that the significant surface cracking disappeared. XRD confirmed that CoO–ZnO nanocomposites comprised CoO and Zn

The digital camera which contain light unit inside it is useful with low illumination but not for high. For different intensity; the quality of the image will not stay good but it will have dark or low intensity so we can not change the contrast and the intensity in order to increase the losses information in the bright and the dark regions. . In this search we study the regular illumination on the images using the tungsten light by changing the intensities. The result appears that the tungsten light gives nearly far intensity for the three color bands(RGB) and the illuminated band(L).the result depend on the statistical properties which represented by the voltage ,power and intensities and the effect of this parameter on the digital

A recently reported Nile red (NR) dye conjugated with benzothiadiazole species paves the way for the development of novel organic-based sensitizers used in solar cells whose structures are susceptible to modifications. Thus, six novel NR structures were derived from two previously developed structures in laboratories. In this study, density functional theory (DFT) calculations and time-dependent DFT (TD-DFT) were used to determine the optoelectronic properties of the NR-derived moieties such as absorption spectra. Various linkers were investigated in an attempt to understand the impact of π-linkers on the optoelectronic properties. According to the findings, the presence of furan species led to the planarity of the molecule and a reduction

In line with the advancement of hardware technology and increasing consumer demands for new functionalities and innovations, software applications grew tremendously in term of size over the last decade. This sudden increase in size has a profound impact as far as testing is concerned. Here, more and more unwanted interactions among software systems components, hardware, and operating system are to be expected, rendering increased possibility of faults. To address this issue, many useful interaction-based testing techniques (termed t-way strategies) have been developed in the literature. As an effort to promote awareness and encourage its usage, this chapter surveys the current state-of-the-art and reviews the state-of-practices in t

In line with the advancement of hardware technology and increasing consumer demands for new functionalities and innovations, software applications grew tremendously in term of size over the last decade. This sudden increase in size has a profound impact as far as testing is concerned. Here, more and more unwanted interactions among software systems components, hardware, and operating system are to be expected, rendering increased possibility of faults. To address this issue, many useful interaction-based testing techniques (termed t-way strategies) have been developed in the literature. As an effort to promote awareness and encourage its usage, this chapter surveys the current state-of-the-art and reviews the state-of-practices in t

This paper presents an experimental study for strengthening existing columns against axial compressive loads. The objective of this work is to study the behavior of concrete square columns strengthening with circulation technique. In Iraq, there are significantly more reinforced rectangular and square columns than reinforced circular columns in reinforced concrete buildings. Moreover, early research studies indicated that strengthening of rectangular or square columns using wraps of CFRP (Carbon Fiber Reinforced Polymer) provided rather little enhancement to their load-carrying capacity. In this paper, shape modification technique was performed to modify the shape (cross section) of the columns from square columns into circular colu

A new spectrophotometric method for the determination of allopurinol drug was investigated. The proposed method was based on the reaction of the intended drug with catechol and Fe(II) to form a blue soluble complex which was measured at λmax 580 nm. A graph of absorbance versus concentration shown that Beer’s law was obeyed over the concentration range of 2–10 μg ml–1 with molar absorptivity of 9.4 x 103 l mol–1 cm–1 and Sandell sensitivity of 1.4 x 10–2 μg cm–2. A recovery percentage of 100% with RSD of 1.0%–1.3% was obtained. The proposed method was applied successfully for the determination of allopurinol drug in tablets with a good accuracy and