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.
Microbial water disinfection with UV rays is a universal technology. Disinfection is a method used to treat drinking water. This can be accomplished using physical and/or chemical processes. Physical Methods: Heating and UV rays are two main methods - UV rays to destroy cells and kill bacteria. The physical process generally gives drinking water an instant purification without producing harmful substances. However, there is no pollution in the water to ensure continuous cleaning. This study’s primary goal is to obtain environmentally safe drinking water in situations of water shortages and homes that lack clean water. Therefore, resort to appropriate home treatment. Therefore, an ex
This work is a trial to ensure the absolute security in any quantum cryptography (QC) protocol via building an effective hardware for satisfying the single-photon must requirement by controlling the value of mean photon number. This was approximately achieved by building a driving circuit that provide very short pulses (≈ 10 ns) for laser diode -LD- with output power of (0.7-0.99mW) using the available electronic components in local markets. These short pulses enable getting faint laser pulses that were further attenuated to reach mean photon number equal to 0.08 or less.
Internet of Things (IoT) is a recent technology paradigm that creates a global network of machines and devices that are capable of communicating with each other. Security cameras, sensors, vehicles, buildings, and software are examples of devices that can exchange data between each other. IoT is recognized as one of the most important areas of future technologies and is gaining vast recognition in a wide range of applications and fields related to smart homes and cities, military, education, hospitals, homeland security systems, transportation and autonomous connected cars, agriculture, intelligent shopping systems, and other modern technologies. This book explores the most important IoT automated and smart applications to help the reader u
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Abstract
Friction stir welding is a relatively new joining process, which involves the joining of metals without fusion or filler materials. In this study, the effect of welding parameters on the mechanical properties of aluminum alloys AA2024-T351 joints produced by FSW was investigated.
Different ranges of welding parameters, as input factors, such as welding speed (6 - 34 mm/min) and rotational speed (725 - 1235 rpm) were used to obtain their influences on the main responses, in terms of elongation, tensile strength, and maximum bending force. Experimental measurements of main responses were taken and analyzed using DESIGN EXPERT 8 experimental design software which was used to develop t
... Show MoreThis paper aims to evaluate the reliability analysis for steel beam which represented by the probability of Failure and reliability index. Monte Carlo Simulation Method (MCSM) and First Order Reliability Method (FORM) will be used to achieve this issue. These methods need two samples for each behavior that want to study; the first sample for resistance (carrying capacity R), and second for load effect (Q) which are parameters for a limit state function. Monte Carlo method has been adopted to generate these samples dependent on the randomness and uncertainties in variables. The variables that consider are beam cross-section dimensions, material property, beam length, yield stress, and applied loads. Matlab software has be
... Show MoreThe Multiple Signal Classification (MUSIC) algorithm is the most popular algorithm to estimate the Angle of Arrival (AOA) of the received signals. The analysis of this algorithm (MUSIC) with typical array antenna element ( ) shows that there are two false direction indication in the plan
aligned with the axis of the array. In this paper a suggested modification on array system is proposed by using two perpendiculars crossed dipole array antenna in spite of one array antenna. The suggested modification does not affect the AOA estimation algorithm. The simulation and results shows that the proposed solution overcomes the MUSIC problem without any effect on the performance of the system.
Objective: The aim of the study is to assess the personal hygiene of adult patients with
diabetic foot.
Methodology: ٨ descriptive study was carried out in Baghdad teaching hospital, Al-Karama teaching hospital
and Al-Kindey teaching hospital for the period of 10/1/2006 to 1/9/2006. A purposive "non probability" sample
of (100) patient.
Questionnaire was constructed for achieving the purpose of the study. Data were collected through the
application of the questionnaire and interview technique. Data were analyzed through descriptive statistical
approach (frequency & percentage) and inferential statistical approach (chi-square ع correlation) by using of
SPSS.
Results: The study results indicated that the ra
The present work evaluated the differences in mechanical properties of two athletic prosthetic feet samples when subjected to impact while running. Two feet samples designated as design A and B were manufactured using layers of different orientations of woven glass fiber reinforced with unsaturated polyester resin as bonding epoxy. The samples’ layers were fabricated with hand lay-up method. A theoretical study was carried out to calculate the mechanical properties of the composite material used in feet manufacturing, then experimental load-deflection test was applied at 0 degree position and 25 degree dorsiflexion feet position and impact test were applied for both feet designs to observe the behavior
... Show MoreThis study aims to develop a recommendation engine methodology to enhance the model’s effectiveness and efficiency. The proposed model is commonly used to assign or propose a limited number of developers with the required skills and expertise to address and resolve a bug report. Managing collections within bug repositories is the responsibility of software engineers in addressing specific defects. Identifying the optimal allocation of personnel to activities is challenging when dealing with software defects, which necessitates a substantial workforce of developers. Analyzing new scientific methodologies to enhance comprehension of the results is the purpose of this analysis. Additionally, developer priorities were discussed, especially th
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