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.
This paper proposes a novel method for generating True Random Numbers (TRNs) using electromechanical switches. The proposed generator is implemented using an FPGA board. The system utilizes the phenomenon of electromechanical switch bounce to produce a randomly fluctuated signal that is used to trigger a counter to generate a binary random number. Compared to other true random number generation methods, the proposed approach features a high degree of randomness using a simple circuit that can be easily built using off-the-shelf components. The proposed system is implemented using a commercial relay circuit connected to an FPGA board that is used to process and record the generated random sequences. Applying statistical testing on th
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The 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 MoreThe 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 MoreObjectives: To assess general social factors for elderly in geriatric homes in Jordan.
Methodology: The study was conducted for clients residing in elderly homes. A purposive sample of (155)
residents were selected. Social resource scale by (Duck University Center) called (OARA) older adult resources
and services was used as tool for data collection. The questions extract data about family structure, patterns of
friends and visiting, availability of a confident, satisfaction with the degree of social interaction and availability
of a helper in the event of illness or disability and the program of trips in the houses.
Results: The findings revealed that the life expectancy for women is greater than for men. In respect to
Active Magnetic Bearings (AMBs) are progressively being implemented in a wide variety of applications. Their exclusive appealing features make them suitable for solving traditional rotor-bearing problems using novel design approaches for rotating machinery. In this paper, a linearized uncertain model of AMBs is utilized to develop a nonlinear sliding mode controller based on Lyapunov function for the electromechanical system. The controller requires measurements of the rotor displacements and their derivatives. Since the control law is discontinuous, the proposed controller can achieve a finite time regulation but with the drawback of the chattering problem. To reduce the effect of this problem, the gain of the uni
... Show MoreThis paper addresses the use of adaptive sliding mode control for the servo actuator system with friction. The adaptive sliding mode control has several advantages over traditional sliding mode control method. Firstly, the magnitude of control effort is reduced to the minimal admissible level defined by the conditions for the sliding mode to exist. Secondly, the upper bounds of uncertainties are not required to be known in advance. Therefore, adaptive sliding mode control method can be effectively implemented. The numerical simulation via MATLAB 2014a for servo actuator system with friction is investigated to confirm the effectiveness of the proposed robust adaptive sliding mode control scheme. The results clarify, after
... Show MoreThis paper presents a new transform method to solve partial differential equations, for finding suitable accurate solutions in a wider domain. It can be used to solve the problems without resorting to the frequency domain. The new transform is combined with the homotopy perturbation method in order to solve three dimensional second order partial differential equations with initial condition, and the convergence of the solution to the exact form is proved. The implementation of the suggested method demonstrates the usefulness in finding exact solutions. The practical implications show the effectiveness of approach and it is easily implemented in finding exact solutions.
Finally, all algori
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