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.
This research adopts the estimation of mass transfer coefficient in batch packed bed distillation column as function of physical properties, liquid to vapour molar rates ratio (L / V), relative volatility (α), ratio of vapour and liquid diffusivities (DV / DL), ratio of vapour and liquid densities (ρV / ρL), ratio of vapour and liquid viscosities (μV/ μL).
The experiments are done using binary systems, (Ethanol Water), (Methanol Water), (Methanol Ethanol), (Benzene Hexane), (Benzene Toluene). Statistical program (multiple regression analysis) is used for estimating the overall mass transfer coefficient of vapour and liquid phases (KOV and KOL) in a correlation which represented the data fairly well.
KOV = 3.3 * 10-10
... Show MoreThe need for quick airborne transportation is critical, especially in emergencies. Drones with suspended payloads might be used to accomplish quick airborne transportation. Due to the environment or the drone's motion, the slung load may oscillate and lead the drone to fall. The altitude and attitude controls are the backbones of the drone's stability, and they must be adequately designed. Because of their symmetrical and simple structure, quadrotor helicopters are one of the most popular drone classes. In this work, a genetic algorithm with two weighted terms fitness function is used to adjust a Proportional-Integral-Derivative (PID) controller to compensate for the altitude and attitude controllers in a quadrotor drone with a slun
... Show Moreالخلفية: العقدية المقيحة المعروفة أيضًا باسم ""(GAS) هي احدى مسببات الأمراض ذات الأهمية الصحية العامة، حيث تصيب 18.1 مليون شخص في جميع أنحاء العالم وتقتل 500000 شخص كل عام. الهدف: حددت هذه المراجعة المقالات المنشورة حول عوامل الخطر واستراتيجيات الوقاية والسيطرة لأمراض المكورات العقدية. المواد والأساليب: تم إجراء بحث منهجي لتحديد الأوراق المنشورة على قواعد البيانات الإلكترونية Web of Science و PubMed و Scopus و Google Scholar في مح
... Show MoreLet R be a 2-torision free prime ring and ?, ?? Aut(R). Furthermore, G: R×R?R is a symmetric generalized (?, ?)-Biderivation associated with a nonzero (?, ?)-Biderivation D. In this paper some certain identities are presented satisfying by the traces of G and D on an ideal of R which forces R to be commutative
As one type of heating furnaces, the electric heating furnace (EHF) typically suffers from time delay, non-linearity, time-varying parameters, system uncertainties, and harsh en-vironment of the furnace, which significantly deteriorate the temperature control process of the EHF system. In order to achieve accurate and robust temperature tracking performance, an integration of robust state feedback control (RSFC) and a novel sliding mode-based disturbance observer (SMDO) is proposed in this paper, where modeling errors and external disturbances are lumped as a lumped disturbance. To describe the characteristics of the EHF, by using convection laws, an integrated dynamic model is established and identified as an uncertain nonlinear second ord
... Show MoreIn this paper a new structure for the AVR of the power system exciter is proposed and designed using digital-based LQR. With two weighting matrices R and Q, this method produces an optimal regulator that is used to generate the feedback control law. These matrices are called state and control weighting matrices and are used to balance between the relative importance of the input and the states in the cost function that is being optimized. A sample power system composed of single machine connected to an infinite- bus bar (SMIB) with both a conventional and a proposed Digital AVR (DAVR) is simulated. Evaluation results show that the DAVR damps well the oscillations of the terminal voltage and presents a faster respo
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This research dealt with highlighting the images of marriage through the modern means of communication and the legal ruling for it. Dowry even be knotted on them; because they are then one of the parties to the contract and contracted in one contract, and this is prohibited by law and law and common sense.
It turns out that it is permissible to advertise marriage through the Internet in accordance with the rules and standards that preserve values and morals and safeguard dignity and chastity. The acts in Islamic law are vested with intentions. If intentions are true, then the act is true, and Internet marriage is what falls under this order.<
... Show MoreDistributed Denial of Service (DDoS) attacks on Web-based services have grown in both number and sophistication with the rise of advanced wireless technology and modern computing paradigms. Detecting these attacks in the sea of communication packets is very important. There were a lot of DDoS attacks that were directed at the network and transport layers at first. During the past few years, attackers have changed their strategies to try to get into the application layer. The application layer attacks could be more harmful and stealthier because the attack traffic and the normal traffic flows cannot be told apart. Distributed attacks are hard to fight because they can affect real computing resources as well as network bandwidth. DDoS attacks
... Show MorePoetic objects may go towards a specific purpose and dissolve and interact with them to produce
A purpose characterized by the title but belongs to the U from afar.
The yarn goes towards praise with a purely horizontal relationship that mentions virtues but crowns them towards women.
In lamentation remember the virtues but crown them towards the dead.
In wisdom remember the virtues but crown them towards time.
In the description remember the virtues but crown them towards nature. All objects are inline
Horizontal We will prove this in the axes of research has required the plan to be a blind, five
The preamble, a brief overview of the life of Dabal All Khuzaie and the works and poets of the first axis
The direction