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.
<span>As a result of numerous applications and low installation costs, wireless sensor networks (WSNs) have expanded excessively. The main concern in the WSN environment is to lower energy consumption amidst nodes while preserving an acceptable level of service quality. Using multi-mobile sinks to reduce the nodes' energy consumption have been considered as an efficient strategy. In such networks, the dynamic network topology created by the sinks mobility makes it a challenging task to deliver the data to the sinks. Thus, in order to provide efficient data dissemination, the sensor nodes will have to readjust the routes to the current position of the mobile sinks. The route re-adjustment process could result in a significant m
... Show MoreSegmentation of urban features is considered a major research challenge in the fields of photogrammetry and remote sensing. However, the dense datasets now readily available through airborne laser scanning (ALS) offer increased potential for 3D object segmentation. Such potential is further augmented by the availability of full-waveform (FWF) ALS data. FWF ALS has demonstrated enhanced performance in segmentation and classification through the additional physical observables which can be provided alongside standard geometric information. However, use of FWF information is not recommended without prior radiometric calibration, taking into account all parameters affecting the backscatter energy. This paper reports the implementation o
... Show MoreAbstract
One of the major components in an automobile engine is the throttle valve part. It is used to keep up with emissions and fuel efficiency low. Design a control system to the throttle valve is newly common requirement trend in automotive technology. The non-smoothness nonlinearity in throttle valve model are due to the friction model and the nonlinear spring, the uncertainty in system parameters and non-satisfying the matching condition are the main obstacles when designing a throttle plate controller.
In this work, the theory of the Integral Sliding Mode Control (ISMC) is utilized to design a robust controller for the Electronic Throttle Valve (ETV) system. From the first in
... Show MoreTopology and its applications occupy the interest of many researching centers in the advanced world. From this point of view and because the near open sets play a very important role in general topology and they are now the research topics of many topologists worldwide and its sets doesn’t enter in fibrewise topology yet. Therefore, we use some of the near open sets to be model for introduce results and new spaces in fibrewise topological spaces. Also, there is a very important role of closure operators in constructing a topological spaces, so we introduce a new closure operators on the power set of vertices on graphs and conclusion theorems and new spaces from it. Furthermore, we discuss the relationships of connectedness between some ty
... Show MoreContours extraction from two dimensional echocardiographic images has been a challenge in digital image processing. This is essentially due to the heavy noise, poor quality of these images and some artifacts like papillary muscles, intra-cavity structures as chordate, and valves that can interfere with the endocardial border tracking. In this paper, we will present a technique to extract the contours of heart boundaries from a sequence of echocardiographic images, where it started with pre-processing to reduce noise and produce better image quality. By pre-processing the images, the unclear edges are avoided, and we can get an accurate detection of both heart boundary and movement of heart valves.
As the bit rate of fiber optic transmission systems is increased to more than , the system will suffer from an important random phenomena, which is called polarization mode dispersion. This phenomenon contributes effectively to: increasing pulse width, power decreasing, time jittering, and shape distortion. The time jittering means that the pulse center will shift to left or right. So that, time jittering leads to interference between neighboring pulses. On the other hand, increasing bit period will prevent the possibility of sending high rates. In this paper, an accurate mathematical analysis to increase the rates of transmission, which contain all physical random variables that contribute to determine the transmission rates, is presen
... Show MoreThis 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
... Show MoreAlgorithms using the second order of B -splines [B (x)] and the third order of B -splines [B,3(x)] are derived to solve 1' , 2nd and 3rd linear Fredholm integro-differential equations (F1DEs). These new procedures have all the useful properties of B -spline function and can be used comparatively greater computational ease and efficiency.The results of these algorithms are compared with the cubic spline function.Two numerical examples are given for conciliated the results of this method.
This paper proposes a new method Object Detection in Skin Cancer Image, the minimum
spanning tree Detection descriptor (MST). This ObjectDetection descriptor builds on the
structure of the minimum spanning tree constructed on the targettraining set of Skin Cancer
Images only. The Skin Cancer Image Detection of test objects relies on their distances to the
closest edge of thattree. Our experimentsshow that the Minimum Spanning Tree (MST) performs
especially well in case of Fogginessimage problems and in highNoisespaces for Skin Cancer
Image.
The proposed method of Object Detection Skin Cancer Image wasimplemented and tested on
different Skin Cancer Images. We obtained very good results . The experiment showed that