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.
As a result of the development and global openness and the possibility of companies providing their services outside their spatial boundaries that were determined by them, and the transformation of the world due to the development of the means of communication into a large global market that accommodates all products from different regions and of the same type and production field, competition resulted between companies, and the race to obtain the largest market share It ensures the largest amount of profits, and it is natural for the advertising promotion by companies for their product to shift from an advertisement for one product to a competitive advertisement that calls on the recipient to leave the competing product and switch to it
... Show MoreBackground. “Polyetheretherketone (PEEK)” is a biocompatible, high-strength polymer that is well-suited for use in dental applications due to its unique properties. However, achieving good adhesion between PEEK and hydrophilic materials such as dental adhesives or cement can be challenging. Also, this hydrophobicity may affect the use of PEEK as an implant material. Surface treatment or conditioning is often necessary to improve surface properties. The piranha solution is the treatment of choice to be explored for this purpose. Methods. PEEK disks of 10 mm diameter and 2 mm thickness were used in this study. Those samples were divided into five groups (each group has five samples). The first is the control group, in which no
... Show MoreThe direct application of cold atmospheric plasma (CAP) is the main scope of plasma medicine in or on the organism for curative purposes. Cold plasma is both effective in disrupting a wide range of microorganisms including multiple drug resistant ones (MDRs) and to stimulate proliferation of mammalian cells. It has obtained by Floating Electrode Dielectric Barrier Discharge (FE-DBD) system. The present study aimed to show the effected of cold plasma on the fertility hormones LH, Prolactin, Estrogen, and Testosterone hormones for healthy adult female rats (Albino) / bulb c). There are divided into many groups according to time exposure of plasma (15, 30, 60, and 90 second) and a refere
This 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 MoreFlexible joint robot (FJR) manipulators can offer many attractive features over rigid manipulators, including light weight, safe operation, and high power efficiency. However, the tracking control of the FJR is challenging due to its inherent problems, such as underactuation, coupling, nonlinearities, uncertainties, and unknown external disturbances. In this article, a terminal sliding mode control (TSMC) is proposed for the FJR system to guarantee the finite-time convergence of the systems output, and to achieve the total robustness against the lumped disturbance and estimation error. By using two coordinate transformations, the FJR dynamics is turned into a canonical form. A cascaded finite-time sliding mode observer (CFTSMO) is construct
... Show MoreIn this paper, two parameters for the Exponential distribution were estimated using the
Bayesian estimation method under three different loss functions: the Squared error loss function,
the Precautionary loss function, and the Entropy loss function. The Exponential distribution prior
and Gamma distribution have been assumed as the priors of the scale γ and location δ parameters
respectively. In Bayesian estimation, Maximum likelihood estimators have been used as the initial
estimators, and the Tierney-Kadane approximation has been used effectively. Based on the MonteCarlo
simulation method, those estimators were compared depending on the mean squared errors (MSEs).The results showed that the Bayesian esti
Objective: The study aims at assessing the food frequency intake and dietary habits for diabetic pregnant
women.
Methodology: A descriptive study is carried out for the period from November4th 2013 to August
25th 2014. A purposive "non-probability" sample of one hundred diabetic pregnant women is selected from
the Diabetic and Endocrine Center in Al-Amarha City. A questionnaire is developed as a tool of data
collection. Content validity of the study instrument is determined through panel of experts. Split-half
reliability technique is used for reliability determination of the study instrument which depicts a reliability
coefficient of (0.79) for the entire scale. A structured interview with each diabetic pregnant wom
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 MoreThe phenomenon of young people frequenting closed cafes spread in Baghdad to smoke hookahs and cigarettes has increased. This phenomenon is associated with unemployment, an increase in leisure time and the deterioration of economic conditions. This phenomenon has an impact on indoor air quality and exposes workers to the risk of exposure to various pollutants, including particulate matter, therefore, we examined some indicators (PM2.5, PM10) IAQ for a month in summer and another month in winter in six different locations in the Rusafa district. PM2.5 and PM10 concentrations, relative humidity (RH) and temperatures were measured using (Multifunction Air Quality Detector BENETECH -China). The results showed an increase in temperatures inside
... Show More