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.
High cost of qualifying library standard cells on silicon wafer limits the number of test circuits on the test chip. This paper proposes a technique to share common load circuits among test circuits to reduce the silicon area. By enabling the load sharing, number of transistors for the common load can be reduced significantly. Results show up to 80% reduction in silicon area due to load area reduction.
Roof in the Iraqi houses normally flattening by a concrete panel. This concrete panel has poor thermal properties. The usage of materials with low thermal conductivity and high specific heat gives a good improvements to the thermal properties of the concrete panel, thus, the indoor room temperature improves. A Mathcad program based on a mathematical model employing complex Fourier series built for a single room building. The model input data are the ambient temperature, solar radiation, and sol-air temperature, which have been treated as a periodic function of time. While, the room construction is constant due to their materials made of it, except the roof properties are taken as a variable generated practically from the
... Show MoreOptimum allocation of water for restoration of Iraqi marshes is essential for different related authorities. Abo-Ziriq marsh area about 120 km2 is situated 40 km east of Al-Nassryia city. After comparing the measured annual water qualities with the Iraqi standards for surface water quality evaluation, Abo-Ziriq marsh water quality was in acceptable limit. Hydro balance computation were done for each month by using interface among the HEC-RAS, HEC-GeoRAS and ArcView GIS software and built a number of eco-hydro relationships to simulate the marsh ecosystem by using HEC-EFM program to estimate water allocation adequate for ecosystem requirement and constructs a GIS hydraulic reference map to show inundation area, depth grid and velocity dis
... Show MoreIn this article, the nonlinear problem of Jeffery-Hamel flow has been solved analytically and numerically by using reliable iterative and numerical methods. The approximate solutions obtained by using the Daftardar-Jafari method namely (DJM), Temimi-Ansari method namely (TAM) and Banach contraction method namely (BCM). The obtained solutions are discussed numerically, in comparison with other numerical solutions obtained from the fourth order Runge-Kutta (RK4), Euler and previous analytic methods available in literature. In addition, the convergence of the proposed methods is given based on the Banach fixed point theorem. The results reveal that the presented methods are reliable, effective and applicable to solve other nonlinear problems.
... Show MorePure nano Ferro fluid was synthesized by chemical co-precipitation method. The composite of polyaniline with nano sized Ferro fluid was prepared by In-situ–chemical oxidation polymerization method with ammonium per sulphate as an oxidant in aqueous hydrochloric acid under constant stirring at room temperature. The optical properties, absorption, transmission, optical energy gap (Eg) and optical constant refractive index (n) have been investigated. The value of the Eg decreased with increasing Ferro fluid concentration.
The aim of this research is to design and construct a
semiconductor laser range finder operating in the near infrared range
for ranging and designation. The main part of the range finder is the
transmitter which is a semiconductor laser type GaAs of wavelength
0.904 μm with a beam expander and the receiver; a silicon pin
detector biased to approve the fast response time with it's collecting
optics. The transmitters pulse width was 200ns at a threshold current
of 10 Ampere and maximum operating current of 38 Ampere. The
repetition rate was set at 660Hz and the maximum operating output
power was around 1 watt. The divergence of the beam was 0.268o
the efficiency of the laser was 0.03% at a duty cycle of 1.32x
Extracting, studying and interpreting the morphological database of a basin is a basic building block for building a correct geomorphological understanding of this basin. In this work, Arc GIS 10.8 software and SRTM DEM satellite images were used. The principle of data integration was adopted by extracting the quantitative values of the morphometric characteristics that are affected by the geomorphological condition of the studied basin, then eliciting an optimal conception of the geomorphological condition of the basin from the meanings and connotations of these combined transactions. Hypsometric integration was extracted for each region in the basin separately with the value of integration of the plot curve for the relative heights of
... Show MoreThis paper reports a fiber Bragg grating (FBG) as a biosensor. The FBGs were etched using a chemical agent,namely,hydrofluoric acid (HF). This implies the removal of some part of the cladding layer. Consequently, the evanescent field propagating out of the core will be closer to the environment and become more sensitive to the change in the surrounding. The proposed FBG sensor was utilized to detect toxic heavy metal ions aqueous medium namely, copper ions (Cu2+). Two FBG sensors were etched with 20 and 40 μm diameters and fabricated. The sensors were studied towards Cu2+ with different concentrations using wavelength shift as a result of the interaction between the evanescent field and copper ions. The FBG sensors showed
... Show MoreDegenerate parabolic partial differential equations (PDEs) with vanishing or unbounded leading coefficient make the PDE non-uniformly parabolic, and new theories need to be developed in the context of practical applications of such rather unstudied mathematical models arising in porous media, population dynamics, financial mathematics, etc. With this new challenge in mind, this paper considers investigating newly formulated direct and inverse problems associated with non-uniform parabolic PDEs where the leading space- and time-dependent coefficient is allowed to vanish on a non-empty, but zero measure, kernel set. In the context of inverse analysis, we consider the linear but ill-pose
Darifenacin hydrobromide is a selective ?3 receptor antimuscarinic drug and it is used in the management of urinary frequency, urgency, and incontinence in detrusor instability. It slightly soluble in water, undergoes extensive hepatic first-pass metabolism and has short elimination half-life (3–4 hours). Therefore, It has low bioavailability (15.4 % - 18.6 %). Darifenacin hydrobromide loaded NLCs were formulated by emulsification sonication using different ratios of solid lipid to liquid lipid, different types of surfactants, and different concentration of surfactants. Formula sixteen was considered as an optimized formula based on its particle size, PDI, zeta potential and entrapment efficiency. Formula sixteen subjected t
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