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.
In this paper, an efficient image segmentation scheme is proposed of boundary based & geometric region features as an alternative way of utilizing statistical base only. The test results vary according to partitioning control parameters values and image details or characteristics, with preserving the segmented image edges.
Based on a finite element analysis using Matlab coding, eigenvalue problem has been formulated and solved for the buckling analysis of non-prismatic columns. Different numbers of elements per column length have been used to assess the rate of convergence for the model. Then the proposed model has been used to determine the critical buckling load factor () for the idealized supported columns based on the comparison of their buckling loads with the corresponding hinge supported columns . Finally in this study the critical buckling factor () under end force (P) increases by about 3.71% with the tapered ratio increment of 10% for different end supported columns and the relationship between normalized critical load and slenderness ratio was g
... Show MoreIn this paper, an exact stiffness matrix and fixed-end load vector for nonprismatic beams having parabolic varying depth are derived. The principle of strain energy is used in the derivation of the stiffness matrix.
The effect of both shear deformation and the coupling between axial force and the bending moment are considered in the derivation of stiffness matrix. The fixed-end load vector for elements under uniformly distributed or concentrated loads is also derived. The correctness of the derived matrices is verified by numerical examples. It is found that the coupling effect between axial force and bending moment is significant for elements having axial end restraint. It was found that the decrease in bending moment was
in the
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 abstraction and examin
... Show MoreGeneral Background: Deep image matting is a fundamental task in computer vision, enabling precise foreground extraction from complex backgrounds, with applications in augmented reality, computer graphics, and video processing. Specific Background: Despite advancements in deep learning-based methods, preserving fine details such as hair and transparency remains a challenge. Knowledge Gap: Existing approaches struggle with accuracy and efficiency, necessitating novel techniques to enhance matting precision. Aims: This study integrates deep learning with fusion techniques to improve alpha matte estimation, proposing a lightweight U-Net model incorporating color-space fusion and preprocessing. Results: Experiments using the AdobeComposition-1k
... Show MoreThe physical behavior for the energy distribution function (EDF) of the reactant particles depending upon the gases (fuel) temperature are completely described by a physical model covering the global formulas controlling the EDF profile. Results about the energy distribution for the reactant system indicate a standard EDF, in which it’s arrive a steady state form shape and intern lead to fix the optimum selected temperature.
High peak to average power ration (PAPR) in orthogonal frequency division multiplexing (OFDM) is an important problem, which increase the cost and complexity of high power amplifiers. One of the techniques used to reduce the PAPR in OFDM system is the tone reservation method (TR). In our work we propose a modified tone reservation method to decrease the PAPR with low complexity compared with the conventional TR method by process the high and low amplitudes at the same time. An image of size 128×128 is used as a source of data that transmitted using OFDM system. The proposed method decrease the PAPR by 2dB compared with conventional method with keeping the performance unchanged. The performance of the proposed method is tested with
... Show Moreهناك دائما حاجة إلى طريقة فعالة لتوليد حل عددي أكثر دقة للمعادلات التكاملية ذات النواة المفردة أو المفردة الضعيفة لأن الطرق العددية لها محدودة. في هذه الدراسة ، تم حل المعادلات التكاملية ذات النواة المفردة أو المفردة الضعيفة باستخدام طريقة متعددة حدود برنولي. الهدف الرئيسي من هذه الدراسة هو ايجاد حل تقريبي لمثل هذه المشاكل في شكل متعددة الحدود في سلسلة من الخطوات المباشرة. أيضا ، تم افتراض أن مقام النواة
... Show MoreThere were two types of plows used widely in agricultural fields in the country. The first plow was moldboard plow, while the second one was chisel plow. There were large numbers of Iraqi farmers that used chisel plow for such farming practices. Researchers found that moldboard plows gave the highest rate of carbon dioxide emission. They observed that that with chisel plows they got the lowest carbon dioxide emission. Chisel plow saved energy as compared to moldboard plow and the cost of using the chisel plows was less than the moldboard plows. Chisel plow decreased carbon dioxide emissions from the soil and improve soil properties. The benefits of using chisel plows were more than using moldboard plows.