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.
A non-parametric kernel method with Bootstrap technology was used to estimate the confidence intervals of the system failure function of the log-normal distribution trace data. These are the times of failure of the machines of the spinning department of the weaving company in Wasit Governorate. Estimating the failure function in a parametric way represented by the method of the maximum likelihood estimator (MLE). The comparison between the parametric and non-parametric methods was done by using the average of Squares Error (MES) criterion. It has been noted the efficiency of the nonparametric methods based on Bootstrap compared to the parametric method. It was also noted that the curve estimation is more realistic and appropriate for the re
... Show MoreThis researd exhibit's a method to determine the change in Gibbs function,(enthai py,entropy. and specific heat capacity) tor monovariant heterogeneous equilibria .The thermodynamical quan.tities were obtained jndirectly with m the measurment of temperature dependent on eql,lilibrium system.
Cloud computing provides huge amount of area for storage of the data, but with an increase of number of users and size of their data, cloud storage environment faces earnest problem such as saving storage space, managing this large data, security and privacy of data. To save space in cloud storage one of the important methods is data deduplication, it is one of the compression technique that allows only one copy of the data to be saved and eliminate the extra copies. To offer security and privacy of the sensitive data while supporting the deduplication, In this work attacks that exploit the hybrid cloud deduplication have been identified, allowing an attacker to gain access to the files of other users based on very small hash signatures of
... Show MoreIn this paper, we derived an estimator of reliability function for Laplace distribution with two parameters using Bayes method with square error loss function, Jeffery’s formula and conditional probability random variable of observation. The main objective of this study is to find the efficiency of the derived Bayesian estimator compared to the maximum likelihood of this function and moment method using simulation technique by Monte Carlo method under different Laplace distribution parameters and sample sizes. The consequences have shown that Bayes estimator has been more efficient than the maximum likelihood estimator and moment estimator in all samples sizes
This paper presents on the design of L-Band Multiwavelength laser for Hybrid Time Division Multiplexing/ Wavelength Division Multiplexing (TDM/WDM) Passive Optical Network (PON) application. In this design, an L-band Mulltiwavelength Laser is designed as the downstream signals for TDM/WDM PON. The downstream signals ranging from 1569.865 nm to 1581.973 nm with 100GHz spacing. The multiwavelength laser is designed using OptiSystem software and it is integrated into a TDM/WDM PON that is also designed using OptiSystem simulation software. By adapting multiwavelength fiber laser into a TDM/WDM network, a simple and low-cost downstream signal is proposed. From the simulation design, it is found that the proposed design is suitable to be used
... Show MoreIn this article, the solvability of some proposal types of the multi-fractional integro-partial differential system has been discussed in details by using the concept of abstract Cauchy problem and certain semigroup operators and some necessary and sufficient conditions.
In this work Nano crystalline (Cu2S) thin films pure and doped 3% Al with a thickness of 400±20 nm was precipitated by thermic steaming technicality on glass substrate beneath a vacuum of ~ 2 × 10− 6 mbar at R.T to survey the influence of doping and annealing after doping at 573 K for one hour on its structural, electrical and visual properties. Structural properties of these movies are attainment using X-ray variation (XRD) which showed Cu2S phase with polycrystalline in nature and forming hexagonal temple ,with the distinguish trend along the (220) grade, varying crystallites size from (42.1-62.06) nm after doping and annealing. AFM investigations of these films show that increase average grain size from 105.05 nm to 146.54 nm
... Show MoreThe spectral characteristics and the nonlinear optical properties of the mixed donor (C-480) acceptor (Rh-6G) have been determined. The spectral characteristics are studied by recording their absorption and fluorescence spectra. The nonlinear optical properties were measured by z-scan technique, using Q-switched Nd: YAG laser with 1064 nm wavelength. The results showed that the optimum concentration of acceptor is responsible for increasing the absorption and the emission bandwidth of donor to full range and to 242 nm respectively by the energy transfer process, also the efficiency of the process was increased by increasing the donor and acceptor concentration. The obtained nonlinear properties results of the mixture C-480/ Rh-6G showed
... Show MoreThe main aim of this paper is to apply a new technique suggested by Temimi and Ansari namely (TAM) for solving higher order Integro-Differential Equations. These equations are commonly hard to handle analytically so it is request numerical methods to get an efficient approximate solution. Series solutions of the problem under consideration are presented by means of the Iterative Method (IM). The numerical results show that the method is effective, accurate and easy to implement rapidly convergent series to the exact solution with minimum amount of computation. The MATLAB is used as a software for the calculations.
Increased downscaling of CMOS circuits with respect to feature size and threshold voltage has a result of dramatically increasing in leakage current. So, leakage power reduction is an important design issue for active and standby modes as long as the technology scaling increased. In this paper, a simultaneous active and standby energy optimization methodology is proposed for 22 nm sub-threshold CMOS circuits. In the first phase, we investigate the dual threshold voltage design for active energy per cycle minimization. A slack based genetic algorithm is proposed to find the optimal reverse body bias assignment to set of noncritical paths gates to ensure low active energy per cycle with the maximum allowable frequency at the optimal supply vo
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