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

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

Groundwater quality investigation has been carried out in the western part of Iraq (west longitude '40°40). The physicochemical analyses of 64 groundwater samples collected from seven aquifers were used in the determination of groundwater characterization and assessment. The concept of spatial hydrochemical bi-model was prepared for quantitative and qualitative interpretation. Hydrogeochemical data referred that the groundwater is of meteoric origin and has processes responsible for observed brackishness. The geochemical facies of the groundwater reveal that none of the anions and cations pairs exceed 50% and there are practically mixtures of multi-water types (such as Ca–Mg–Cl–HCO₃ and Na+K–SO4–Cl water type) as do

The prevalence of using the applications for the internet of things (IoT) in many human life fields such as economy, social life, and healthcare made IoT devices targets for many cyber-attacks. Besides, the resource limitation of IoT devices such as tiny battery power, small storage capacity, and low calculation speed made its security a big challenge for the researchers. Therefore, in this study, a new technique is proposed called intrusion detection system based on spike neural network and decision tree (IDS-SNNDT). In this method, the DT is used to select the optimal samples that will be hired as input to the SNN, while SNN utilized the non-leaky integrate neurons fire (NLIF) model in order to reduce latency and minimize devices

This research represents a practical attempt applied to calibrate and verify a hydraulic model for the Blue Nile River. The calibration procedures are performed using the observed data for a previous period and comparing them with the calibration results while verification requirements are achieved with the application of the observed data for another future period and comparing them with the verification results. The study objective covered a relationship of the river terrain with the distance between the assumed points of the dam failures along the river length. The computed model values and the observed data should conform to the theoretical analysis and the overall verification performance of the model by comparing it with anothe

This paper is concerned with pre-test single and double stage shrunken estimators for the mean (?) of normal distribution when a prior estimate (?0) of the actule value (?) is available, using specifying shrinkage weight factors ?(?) as well as pre-test region (R). Expressions for the Bias [B(?)], mean squared error [MSE(?)], Efficiency [EFF(?)] and Expected sample size [E(n/?)] of proposed estimators are derived. Numerical results and conclusions are drawn about selection different constants included in these expressions. Comparisons between suggested estimators, with respect to classical estimators in the sense of Bias and Relative Efficiency, are given. Furthermore, comparisons with the earlier existing works are drawn.

        The research deals with the analysis of the city's commercial center using geographic information systems to solve the problem of congestion by evaluating the efficiency and adequacy of car parking lots according to local and Arab standards. Undoubtedly, the importance of car parking areas, as they are not within the desired efficiency within the city, will lead to congestion and traffic becomes very difficult. Thus, the transportation service loses its most important characteristic, which is the ease of movement. Therefore, there has become an urgent need to study and analyze it, as well as to verify the adequacy of the service, and the amount of deficit required to be provided to solve the tra

The electric quadrupole moments for some scandium isotopes (41, 43, 44, 45, 46, 47Sc) have been calculated using the shell model in the proton-neutron formalism. Excitations out of major shell model space were taken into account through a microscopic theory which is called core polarization effectives. The set of effective charges adopted in the theoretical calculations emerging about the core polarization effect. NushellX@MSU code was used to calculate one body density matrix (OBDM). The simple harmonic oscillator potential has been used to generate the single particle matrix elements. Our theoretical calculations for the quadrupole moments used the two types of effective interactions to obtain the best interaction compared with the exp

The Arabic calligraphy, in the early days of Islam, was used for the purpose of decorating the walls of mosques from inside and outside. Hence, this decorative art must be actively involved in enriching the designs of Islamic fabrics and costumes because it has a highly qualified and aesthetically pleasing look and expresses the originality of the Arab spirit. The research is divided into two section: the first section included the following topics: First "A historical overview of the art of decoration in Islamic Arab thought". Second "linear formations of decorative designs, and the subject of intellectual aesthetic taste of the art of decorating. This section tackles two subsections: first "the beauty of thought between the function an