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

Videogames are currently one of the most widespread means of digital communication and entertainment; their releases are attracting considerable interest with growing number of audience and revenues each year. Videogames are examined by a variety of disciplines and fields. Nevertheless, scholarly attention concerned with the discourse of videogames from a linguistic perspective is relatively scarce, especially from a pragma-stylistic standpoint. This book addresses this vital issue by providing a pragma-stylistic analysis of the digital discourse of two well-known action videogames (First Person Shooter Games). It explores the role of the digital discourse of action videogames in maintaining real-like interactivity between the game and the

           In this paper, the homotopy perturbation method (HPM) is presented for treating a linear system of second-kind mixed Volterra-Fredholm integral equations. The method is based on constructing the series whose summation is the solution of the considered system. Convergence of constructed series is discussed and its proof is given; also, the error estimation is obtained. Algorithm is suggested and applied on several examples and the results are computed by using MATLAB (R2015a). To show the accuracy of the results and the effectiveness of the method, the approximate solutions of some examples are compared with the exact solution by computing the absolute errors.

Massive multiple-input multiple-output (massive-MIMO) is considered as the key technology to meet the huge demands of data rates in the future wireless communications networks. However, for massive-MIMO systems to realize their maximum potential gain, sufficiently accurate downlink (DL) channel state information (CSI) with low overhead to meet the short coherence time (CT) is required. Therefore, this article aims to overcome the technical challenge of DL CSI estimation in a frequency-division-duplex (FDD) massive-MIMO with short CT considering five different physical correlation models. To this end, the statistical structure of the massive-MIMO channel, which is captured by the physical correlation is exploited to find sufficiently

   The aim of this paper is to present the first record of ctenophore species Pleurobrachia pileus (O. F. Müller, 1776) in the coral reef as was recently found in Iraqi marine waters. The specimens were collected from two sites, the first was in Khor Abdullah during May 2015, and the second site was located in the pelagic water of the coral reef area, near the Al-Basrah deep sea crude oil marine loading terminal. Three samples were collected at this site during May 2015, February and March 2018 which showed that P. pileus were present at a densities of 3.0, 2.2 and 0.55 ind./ m³ respectively. The species can affect on the abundance of other zooplankton community through predation.

The article presents the results of studying the conchological variability of the terrestrial mollusk Chondrulopsina fedtschenkoi (Ancey, 1886), known to occur in three regions of the Zaravshan Range (Central Asia): the Urgutsay Gorge, the vicinity of the Gissarak Reservoir and the Ingichka-Irmak Gorge. Conchological variability was determined based on statistical analysis. The climate of the three regions is different, and environmental factors have led to changes in the mollusk shell. The shells have changed in response to environmental factors, these are their adaptive traits for survival; the variability of conchological features is also reflected in the color of the shell, and the intensive development of the color of the shell in m

The study deals with the issue of multi-choice linear mathematical programming. The right side of the constraints will be multi-choice. However, the issue of multi-purpose mathematical programming can not be solved directly through linear or nonlinear techniques. The idea is to transform this matter into a normal linear problem and solve it In this research, a simple technique is introduced that enables us to deal with this issue as regular linear programming. The idea is to introduce a number of binary variables And its use to create a linear combination gives one parameter was used multiple. As well as the options of linear programming model to maximize profits to the General Company for Plastic Industries product irrigation sy

This paper investigated the fatigue life behavior of two composite materials subjected to different times of shot peening (2, 4 and 6 min).The first material prepared from unsaturated polyester with E-glass reinforcement by 33% volume fraction. While, the second one was prepared from unsaturated polyester with aluminum powder by2.5% volume fraction. The experimental results showed that the improvement in endurance limit was obtained (for the first material) at 2, 4 and 6 min shot peening times where the percentage of maximum improvement was 25% at shot peening time of 6 min. While, the endurance limit of the second material decreased at shot peening times of 2, 4 and 6 min where the percentage of maximum reduction was 29 % at shot peenin

The complexity and partially defined nature of jet grouting make it hard to predict the performance of grouted piles. So the trials of cement injection at a location with similar soil properties as the erecting site are necessary to assess the performance of the grouted piles. Nevertheless, instead of executing trial-injected piles at the pilot site, which wastes money, time, and effort, the laboratory cement injection devices are essential alternatives for evaluating soil injection ability. This study assesses the performance of a low-pressure laboratory grouting device by improving loose sandy soil injected using binders formed of Silica Fume (SF) as a chemical admixture (10% of Ordinary Portland Cement OPC mass) to di