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 new Azo‐Schiff base ligand L was prepared by reaction of m‐hydroxy benzoic acid with (Schiff base B) of 3‐[2‐(1H–indol‐3‐yl)‐ethylimino]‐1.5‐dimethyl‐2‐phenyl‐2,3‐dihydro‐1H‐pyrazol‐4‐ylamine. This synthesized ligand was used for complexation with different metal ions like Ni(II), Co(II), Pd(II) and Pt(IV) by using a molar ratio of ligand: metal as 1:1. Resulted compounds were characterized by NMR (1H and 13C), UV–vis spectroscopy, TGA, FT‐IR, MS, elemental analysis, magnetic moment and molar conductivity studies. The activation thermodynamic parameters, such as ΔE*, ΔH*, ΔS*, ΔG*and
... Show MoreThe paper presents research results of the vibration transmitted from the steering wheel of the
tractor with a 2-wheel drive to the driver’s hands. The vibration measurements were carried out on the
tractor randomly chosen from the collage of agriculture / university of Baghdad. Before testing the
tractor was examined and adjusted following the producer’s recommendations. The vibration levels
were measured during the operation tillage at idling and at full load .The field was 3١٫٧ m above level
sea. Soil was treated at soil constant moisture (1٧-20 %) with depth of plowing (١٧ cm). During
operation the weather temperature was measured (15 C) and humidity was ( 27 % ) The vibration level
on the steering whee
This study examines experimentally the performance of a horizontal triple concentric tube heat exchanger TCTHE made of copper metal using water as cooling fluid and oil-40 as hot fluid. Hot fluid enters the inner annular tube of the TCTHE in a direction at a temperature of 50, 60 and 70 oC and a flow rate of 20 l/hr. On the other hand, the cooling fluid enters the inner tube and the outer annular tube in the reverse direction (counter current flow) at a temperature of 25 oC and flow rates of 10, 15, 20, 25, 30 and 35 l/hr. The TCTHE is composed of three copper tubes with outer diameters of 34.925 mm, 22.25 mm, and 9.525 mm, and thicknesses of 1.27 mm, 1.143 mm, and 0.762 mm, respectively. TCTHE tube's length was 670
... Show MoreThis study introduces a highly sensitive trapezium-shaped PCF based on an SPR refractometric sensor with unique design features. The structure of a sensor was designed and analyzed using COMSOL Multiphysics v5.6 based on Finite Element Method (FEM) with a focus on investigating the influence of various geometric parameters on its performance. The two channels were coated with a metallic gold layer to provide chemical stability, and a thin layer of TiO₂ improved the gold's adhesion to the fiber. The findings indicate that the proposed sensor achieves maximum amplitude and wavelength sensitivities of 1,779 RIU⁻¹ and 30,500 nm/RIU, respectively, with corresponding resolutions of 3.2
A comparison between the resistance capacity of a single pile excited by two opposite rotary machines embedded in dry and saturated sandy soil was considered experimentally. A small-scale physical model was manufactured to accomplish the experimental work in the laboratory. The physical model consists of: two small motors supplied with eccentric mass 0·012 kg and eccentric distance 20 mm representing the two opposite rotary machines, an aluminum shaft with 20 mm in diameter as the pile, and a steel plate with dimensions of (160 × 160 × 20 mm) as a pile cap. The experimental work was achieved taking the following parameters into consideration, pile embedment depth ratio (L/d; length to diameter) and operating freq
... Show MoreThroughout this paper, a generic iteration algorithm for a finite family of total asymptotically quasi-nonexpansive maps in uniformly convex Banach space is suggested. As well as weak / strong convergence theorems of this algorithm to a common fixed point are established. Finally, illustrative numerical example by using Matlab is presented.
The effect of molecules intersystem crossing (Kisc) on characteristics
(energy and duration) of a Passive Q- switched Laser Pulse has been
studied by mathematical description (rate equations model) for
temporal performance of which was used as a saturable absorber
material (passive switch) with laser. The study shows that the energy
and duration pulse are decreasing while the molecules intersystem
crossing into saturable absorber energy levels is increasing.
The research aims at the scholars’attention to the importance and role of Arab Muslim women through books and historical studies that historians and scholars have taken up like Ibn Al-Jawzi (597 AH) and his book Safwat Al-Safwa, where he presented the conduct of worship and their words and deeds, and explained to women worshipers other aspects of their lives, such as the scientific aspect Crafts and works as well as their prominent role in preaching and religious guidance, and the impact of this on their morals and qualities. This study showed the strength of the personality of women and their high interest in worship. The importance of the study lies in knowledge.The research followed the role of Muslim women to carry t
... Show MoreDeep learning has recently received a lot of attention as a feasible solution to a variety of artificial intelligence difficulties. Convolutional neural networks (CNNs) outperform other deep learning architectures in the application of object identification and recognition when compared to other machine learning methods. Speech recognition, pattern analysis, and image identification, all benefit from deep neural networks. When performing image operations on noisy images, such as fog removal or low light enhancement, image processing methods such as filtering or image enhancement are required. The study shows the effect of using Multi-scale deep learning Context Aggregation Network CAN on Bilateral Filtering Approximation (BFA) for d
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