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.
In this work the effect of choosing tri-circular tube section had been addressed to minimize the end effector’s error, a comparison had been made between the tri-tube section and the traditional square cross section for a robot arm, the study shows that for the same weight of square section and tri-tube section the error may be reduced by about 33%.
A program had been built up by the use of MathCAD software to calculate the minimum weight of a square section robot arm that could with stand a given pay load and gives a minimum deflection. The second part of the program makes an optimization process for the dimension of the cross section and gives the dimensions of the tri-circular tube cross section that have the same weight of
... Show MoreThe Electrical power system has become vast and more complex, so it is subjected to sudden changes in load levels. Stability is an important concept which determines the stable operation of the power system. Transient stability analysis has become one of the significant studies in the power system to ensure the system stability to withstand a considerable disturbance. The effect of temporary occurrence can lead to malfunction of electronic control equipment. The application of flexible AC transmission systems (FACTS) devices in the transmission system have introduced several changes in the power system. These changes have a significant impact on the power system protection, due to differences inline impedance, line curre
... Show MoreMale reproductive health is intricately regulated by molecular and physiological processes, with the aryl hydrocarbon receptor (AhR) playing a crucial role. AhR is activated by various ligands and influences the onset and progression of diseases. The aim of this study was to evaluate the role of AhR on spermatogenesis in adult male rats were affected by resveratrol (RES) and CH223191, an AhR antagonist. The study include forty rats were randomly divided into four equal groups: Control group, DMSO group, RES group and AhR‾ group, the rats received respective treatments intraperitoneally twice weekly for 60 days, and various parameters related to male reproductive health were evaluated. The AhR that activation by the RES treatment w
... Show MoreIn this study tungsten oxide and graphene oxide (GO-WO2.89) were successfully combined using the ultra-sonication method and embedded with polyphenylsulfone (PPSU) to prepare novel low-fouling membranes for ultrafiltration applications. The properties of the modified membranes and performance were investigated using Fourier-transform infrared spectroscopy (FT-IR), scanning electron microscopy (SEM), contact angle (CA), water permeation flux, and bovine serum albumin (BSA) rejection. It was found that the modified PPSU membrane fabricated from 0.1 wt.% of GO-WO2.89 possessed the best characteristics, with a 40.82° contact angle and 92.94% porosity. The permeation flux of the best membrane was the highest. The pure water permeation f
... Show MoreParticulate matter (PM) emitted from diesel engine exhaust have been measured in terms of mass, using
99.98 % pure ethanol blended directly, without additives, with conventional diesel fuel (gas – oil),to
get 10 % , 15 %, 20 % ethanol emulsions . The resulting PM collected has been compared with those
from straight diesel. The engine used is a stationary single cylinder, variable compression ratio Ricardo
E6/US. This engine is fully instrumented and could run as a compression or spark ignition.
Observations showed that particulate matter (PM) emissions decrease with increasing oxygenate
content in the fuel, with some increase of fuel consumption, which is due to the lower heating value of
ethanol. The reduction in
Aerial manipulation of objects has a number of advantages as it is not limited by the morphology of the terrain. One of the main problems of the aerial payload process is the lack of real-time prediction of the interaction between the gripper of the aerial robot and the payload. This paper introduces a digital twin (DT) approach based on impedance control of the aerial payload transmission process. The impedance control technique is implemented to develop the target impedance based on emerging the mass of the payload and the model of the gripper fingers. Tracking the position of the interactional point between the fingers of gripper and payload, inside the impedance control, is achieved using model predictive control (MPD) approach.
... Show MoreIn this research, a novel synthesis of CaONPs has been developed via an environmentally friendly, green method. Garlic extract (Allium sativum) was used as a green-reducing and stabilizing agent for CaONPs. The average particle size of CaONPs was approximately 24.42 nm. The synthesized CaONPs were identified by using Fourier transform infrared (FT-IR) spectroscopy, U.V.-vis spectrum, X-ray diffraction (XRD), Field Emission-Scanning Electron Microscopy (FE-SEM), Transmission Electron Microscopy, transmission electron microscopy (TEM), Energy Dispersive X-ray spectroscopy (EDX), Atomic Force Microscopy (AFM), and zeta potential (Zp) analysis. The current study highlights the notable applications for CaONPs. First, an antimicrobial assay revea
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