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 inflammatory conditions which suggest that cystinosis should be included within the differential diagnosis of disorders associated with increased plasma chitotriosidase activity. This study is aimed to estimate serum chitotriosidase level, as a screening marker and therapeutic monitor for cystinosis disease in Iraqi children with cystinosis.
Subjects and Methods: The present study is a case-control study that included samples of 30 children with nephropathic cystinosis, compared to 25 healthy control children from those attending at The Genetic Rare Diseases Center / AL-Emamain AL-Kadhimain Teaching Hospital, Baghdad-Iraq.
Results: Our results reported that cystinotic children had a marked elevation of serum chitotriosidase activity, compared to age-matched healthy children, besides a significant associated with leukocyte-cystine content for cystinotic patients.
CHT1 as a Novel Biomarker
Conclusion: Estimation of serum chitotriosidase activity might aid in monitoring the therapeutic benefits of cysteamine therapy, as well as the prognosis of the disease when WBC cystine assessment is not available.
Key Words: Cystinosis, Cysteamine, Chitotriosidase.
Computer systems and networks are increasingly used for many types of applications; as a result the security threats to computers and networks have also increased significantly. Traditionally, password user authentication is widely used to authenticate legitimate user, but this method has many loopholes such as password sharing, brute force attack, dictionary attack and more. The aim of this paper is to improve the password authentication method using Probabilistic Neural Networks (PNNs) with three types of distance include Euclidean Distance, Manhattan Distance and Euclidean Squared Distance and four features of keystroke dynamics including Dwell Time (DT), Flight Time (FT), mixture of (DT) and (FT), and finally Up-Up Time (UUT). The resul
... Show MoreIn this paper, first and second order sliding mode controllers are designed for a single link robotic arm actuated by two Pneumatic Artificial Muscles (PAMs). A new mathematical model for the arm has been developed based on the model of large scale pneumatic muscle actuator model. Uncertainty in parameters has been presented and tested for the two controllers. The simulation results of the second-order sliding mode controller proves to have a low tracking error and chattering effect as compared to the first order one. The verification has been done by using MATLAB and Simulink software.
The road network serves as a hub for opportunities in production and consumption, resource extraction, and social cohabitation. In turn, this promotes a higher standard of living and the expansion of cities. This research explores the road network's spatial connectedness and its effects on travel and urban form in the Al-Kadhimiya and Al-Adhamiya municipalities. Satellite images and paper maps have been employed to extract information on the existing road network, including their kinds, conditions, density, and lengths. The spatial structure of the road network was then generated using the ArcGIS software environment. The road pattern connectivity was evaluated using graph theory indices. The study demands the abstractio
... Show MoreThe research paper deals with the role of the place making in eco-tourism through a review of international experiences in the eco-tourism industry and its contribution to advancing the reality of tourism there, and attracting the largest number of tourists. The study is divided into five axes: the first is a study of related concepts, and the second is a study of global experiences, which included three countries: (South Bank (Gabriel's Wharf) - London, Rotterdam in the Netherlands, and dealt with each of Happy Streets and Kendrick Mills, and then the Perak River tourist corridor - Malaysia). As for the third axis, it is concerned with analyzing these experiences to reach th
... Show More
The great scientific progress has led to widespread Information as information accumulates in large databases is important in trying to revise and compile this vast amount of data and, where its purpose to extract hidden information or classified data under their relations with each other in order to take advantage of them for technical purposes.
And work with data mining (DM) is appropriate in this area because of the importance of research in the (K-Means) algorithm for clustering data in fact applied with effect can be observed in variables by changing the sample size (n) and the number of clusters (K)
... Show More<span lang="EN-US">This paper presents the comparison between optimized unscented Kalman filter (UKF) and optimized extended Kalman filter (EKF) for sensorless direct field orientation control induction motor (DFOCIM) drive. The high performance of UKF and EKF depends on the accurate selection of state and noise covariance matrices. For this goal, multi objective function genetic algorithm is used to find the optimal values of state and noise covariance matrices. The main objectives of genetic algorithm to be minimized are the mean square errors (MSE) between actual and estimation of speed, current, and flux. Simulation results show the optimal state and noise covariance matrices can improve the estimation of speed, current, t
... Show MoreMalware represents one of the dangerous threats to computer security. Dynamic analysis has difficulties in detecting unknown malware. This paper developed an integrated multi – layer detection approach to provide more accuracy in detecting malware. User interface integrated with Virus Total was designed as a first layer which represented a warning system for malware infection, Malware data base within malware samples as a second layer, Cuckoo as a third layer, Bull guard as a fourth layer and IDA pro as a fifth layer. The results showed that the use of fifth layers was better than the use of a single detector without merging. For example, the efficiency of the proposed approach is 100% compared with 18% and 63% of Virus Total and Bel
... Show MoreThe process of controlling a Flexible Joint Robot Manipulator (FJRM) requires additional sensors for measuring the state variables of flexible joints. Therefore, taking the elasticity into account adds a lot of complexity as all the additional sensors must be taken into account during the control process. This paper proposes a nonlinear observer that controls FJRM, without requiring equipment sensors for measuring the states. The nonlinear state equations are derived in detail for the FJRM where nonlinearity, of order three, is considered. The Takagi–Sugeno Fuzzy Model (T-SFM) technique is applied to linearize the FJRM system. The Luenberger observer is designed to estimate the unmeasured states using error correction. The develop
... Show MoreThis research aims to test the ability of glass waste powder to adsorb cadmium from aqueous solutions. The glass wastes were collected from the Glass Manufacturing Factory in Ramadi. The effect of concentration and reaction time on sorption was tested through a series of laboratory experiments. Four Cd concentrations (20, 40, 60, and 80) as each concentration was tested ten times for 5, 10, 15, 20, 25, 30, 35, 40, 45, and 50 min. Solid (glass wastes) to liquid was 2g to 30ml was fixed in each experiment where the total volume of the solution was 30ml. The pH, total dissolved salts and electrical conductivity were measured at 30ºC. The equilibrium concentration was determined at 25 minutes, thereafter it was noted that the sorption
... Show More