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.
Clinical keratoconus (KCN) detection is a challenging and time-consuming task. In the diagnosis process, ophthalmologists must revise demographic and clinical ophthalmic examinations. The latter include slit-lamb, corneal topographic maps, and Pentacam indices (PI). We propose an Ensemble of Deep Transfer Learning (EDTL) based on corneal topographic maps. We consider four pretrained networks, SqueezeNet (SqN), AlexNet (AN), ShuffleNet (SfN), and MobileNet-v2 (MN), and fine-tune them on a dataset of KCN and normal cases, each including four topographic maps. We also consider a PI classifier. Then, our EDTL method combines the output probabilities of each of the five classifiers to obtain a decision b
Free Space Optical (FSO) technology offers highly directional, high bandwidth communication channels. This technology can provide fiber-like data rate over short distances. In order to improve security associated with data transmission in FSO networks, a secure communication method based on chaotic technique is presented. In this paper, we have turned our focus on a specific class of piece wise linear one-dimensional chaotic maps. Simulation results indicate that this approach has the advantage of possessing excellent correlation property. In this paper we examine the security vulnerabilities of single FSO links and propose a solution to this problem by implementing the chaotic signal generator “reconfigurable tent map”. As synchronizat
... Show MoreThis research presents experimental and theoretical investigation of 15 reinforced concrete spliced and nonspliced girder models. Splices of hooked dowels and cast in place joints, with or without strengthening steel plates were used. Post-tensioning had been used to enhance the splice strength for some spliced girders. The ANSYS computer program was used for analyzing the spliced and non-spliced girders. A nonlinear three dimensional element was used to represent all test girders. The experimental results have shown that for a single span girder using steel plate connectors in the splice zone has given a sufficient continuity to resist flexural stresses in this region. The experimental results have shown that the deflection of hooked do
... Show MoreThis study examined >140 relevant publications from the last few years (2018–2021). In this study, classification was reviewed depending on the operation's progress. Electrocoagulation (EC), electrooxidation (EO), electroflotation (EF), electrodialysis (ED), and electro-Fenton (EFN) processes have received considerable attention. The type of action (individual or hybrid) for each electrochemical procedure was evaluated, and statistical analysis was performed to compare them as a new manner of reviewing cited papers providing a massive amount of information efficiently to the readers. Individual or hybrid operation progress of the electrochemical techniques is critical issues. Their design, operation, and maintenance costs vary depending o
... Show MoreA computerized investigation has been carried out on the design of six electrodes electrostatic lenses used in electron gun application. The Finite-Element Method (FEM) was used in the solution of Laplace equation for determine the axial potential distribution. The electron trajectory under zero magnification condition. The optical properties, spherical and chromatic aberrations, the object and image focal length and object and image position are calculated. A very good futures for the electron gun with these lenses have been computed where are a beam current of 8.7*10-7A can be supplied using cathode tip of radius 10nm.
The present work concerns with simulating unsteady state equilibrium model for production of methyl oleate (biodiesel) from reaction of oleic acid with methanol using sulfuric acid as a catalyst in batch reactive distillation. MESHR equations of equilibrium model were solved using MATLAB (R2010a). The validity of simulation model was tested by comparing the simulation results with a data available in literature. UNIQUAC liquid phase activity coefficient model is the most appropriate model to describe the non-ideality of OLAC-MEOH-MEOL-H2O system. The chemical reactions rates results from EQ model indicating the rates are controlled by chemical kinetics. Several variables was studied such as molar ratio of methanol to oleic acid 4:1, 6:1
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