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.
The estimation of quantity of liquid that is collected from gas/oil separation system is a very complex task because it requires the application of the flash calculations which needs to solve the cubic equation of state and to use some numerical techniques. These difficulties can be overcome by a computer model which requires a lot of experimental data, long time, and experience.
This paper adopts a new technique to simplify this problem. It suggests new correlations for optimum separator pressure for separation station of heavy oils. The correlations have been achieved for two- and three- stage separation systems.
The co
The rotation effect upon Morse potential had been studied and the values of the effective potential in potential curves had been calculated for electronic states (X2?+g , B ?u ) K2 molecule. The calculation had been computed for rotational quantum number (J = 5). Also, drawing potential curves for these systems had been done using Herzberg and Gaydon equations. It was found that the values of the dissociation energy which resulting from using Herzberg equation greater than that of Gaydon equation. Besides, it was found that the rotation effect for (X and B) electronic states in Morse potential is very small and in this case may negligible.
Researchers are increasingly using multimodal biometrics to strengthen the security of biometric applications. In this study, a strong multimodal human identification model was developed to address the growing problem of spoofing attacks in biometric security systems. Through the use of metaheuristic optimization methods, such as the Genetic Algorithm(GA), Ant Colony Optimization(ACO), and Particle Swarm Optimization (PSO) for feature selection, this unique model incorporates three biometric modalities: face, iris, and fingerprint. Image pre-processing, feature extraction, critical image feature selection, and multibiometric recognition are the four main steps in the workflow of the system. To determine its performance, the model wa
... Show MoreThe conception and experimental assessment of a removable friction-based shear connector (FBSC) for precast steel-concrete composite bridges is presented. The FBSC uses pre-tensioned high-strength steel bolts that pass through countersunk holes drilled on the top flange of the steel beam. Pre-tensioning of the bolts provides the FBSC with significant frictional resistance that essentially prevents relative slip displacement of the concrete slab with respect to the steel beam under service loading. The countersunk holes are grouted to prevent sudden slip of the FBSC when friction resistance is exceeded. Moreover, the FBSC promotes accelerated bridge construction by fully exploiting prefabrication, does not raise issues relevant to precast co
... Show MoreKrawtchouk polynomials (KPs) and their moments are promising techniques for applications of information theory, coding theory, and signal processing. This is due to the special capabilities of KPs in feature extraction and classification processes. The main challenge in existing KPs recurrence algorithms is that of numerical errors, which occur during the computation of the coefficients in large polynomial sizes, particularly when the KP parameter (p) values deviate away from 0.5 to 0 and 1. To this end, this paper proposes a new recurrence relation in order to compute the coefficients of KPs in high orders. In particular, this paper discusses the development of a new algorithm and presents a new mathematical model for computing the
... Show MoreIn this paper, we propose a method using continuous wavelets to study the multivariate fractional Brownian motion through the deviations of the transformed random process to find an efficient estimate of Hurst exponent using eigenvalue regression of the covariance matrix. The results of simulations experiments shown that the performance of the proposed estimator was efficient in bias but the variance get increase as signal change from short to long memory the MASE increase relatively. The estimation process was made by calculating the eigenvalues for the variance-covariance matrix of Meyer’s continuous wavelet details coefficients.
A niger, a fungus which doesn't have high ability to production lipid, this fungus has been select to investigate the non oleaginicity. In this search, there are explorations about: i) growth profile ii) enzymes profile iii) isoforms. Growth profile shows that this fungus doesn't have ability to accumulate lipid more than 6% while bio mass are around 10g/l in spite of the presence of glucose in the media till the end of cultivation time and excision of nitrogen within 24 hrs. In enzyme study, we investigate all lipogenic enzymes Malic enzyme (ME), Fatty acid synthase (FAS), ATP: Citrate lays (ACL), NAD+ isocitrate dehydrogenase (NAD+ICDH), Glucose-6-phosphate (G6PD), and 6-phosphogluconate dehydrogenase (6PGD), all these enzymes show, ac
... Show MoreInvestment Bases directly and closely to an environment characterized by political, social and economic stability, and through a range of policies and institutions and economic laws that affect investor confidence and convince him directing investments to country without the other, where inter conditions and circumstances affecting the trends of capital and settle in, and political situation of the country and what is characterized of stability or disorder as well as economic conditions that are affected by what is distinguishes the country from geographic and demographic characteristics are reflected on availability of production elements and country's infrastructure.
... Show MoreSpatial and frequency domain techniques have been adopted in this search. mean
value filter, median filter, gaussian filter. And adaptive technique consists of
duplicated two filters (median and gaussian) to enhance the noisy image. Different
block size of the filter as well as the sholding value have been tried to perform the
enhancement process.