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.
Abstract
This research attempt to explain the essential aspects of one important model in management of Bank risks , that is (stress testing) , which increase the concentrate on it resulting the negative affects of Global financial crisis that it accuar in 2008 to study the application possibilities in iraqian banks to enhancing the safety and financial soundness Becuase the classical tools in Risk management don’t give clear image on Banks ability in facing risks, hence the Basel committee on Banking supervision focusing in agreement of Basel 2,3 on stress testing when it doing the internal capital adequacy assessment process (ICAAP) .
To achieving the reseach obje
... Show MoreIn The Name of Allah Most Gracious Most Merciful
It is no secret to everyone that the endowment is an important nucleus for the prosperity of Islamic civilization, especially in the fields of education, health, economy, and defensive military actions that fall within the door of jihad, and so on. Al-Ashraf, Qom Al-Quds, Cairo, and other parts of the Islamic world. What we will see in the research.
In this paper, a new equivalent lumped parameter model is proposed for describing the vibration of beams under the moving load effect. Also, an analytical formula for calculating such vibration for low-speed loads is presented. Furthermore, a MATLAB/Simulink model is introduced to give a simple and accurate solution that can be used to design beams subjected to any moving loads, i.e., loads of any magnitude and speed. In general, the proposed Simulink model can be used much easier than the alternative FEM software, which is usually used in designing such beams. The obtained results from the analytical formula and the proposed Simulink model were compared with those obtained from Ansys R19.0, and very good agreement has been shown. I
... Show MoreThis investigation proposed an identification system of offline signature by utilizing rotation compensation depending on the features that were saved in the database. The proposed system contains five principle stages, they are: (1) data acquisition, (2) signature data file loading, (3) signature preprocessing, (4) feature extraction, and (5) feature matching. The feature extraction includes determination of the center point coordinates, and the angle for rotation compensation (θ), implementation of rotation compensation, determination of discriminating features and statistical condition. During this work seven essential collections of features are utilized to acquire the characteristics: (i) density (D), (ii) average (A), (iii) s
... Show MoreAgricultural companies could push investment forward and help in production base variation and developing production techniques. They also play a significant role in organizing and providing requirements of market economy. This work aimed at studying economic efficiency of the six agricultural companies that are registered at Iraqi stock market for the period 2005-2016. Capital of these companies was between (300 million-7.5 billion IQD). Several financial analysis indicators were applied such as activity percentages. Average of capital circulation was 0.91 for private company of agricultural production، and lowest share was for Iraqi company for producing and marketing field crops. Assets average circulation was highest for the pr
... Show MoreLowering the emission, fuel economy and torque management are the essential
requirements in the recent development in the automobile industry. The main engine control
input that satisfies the above requirements is the throttling angle which adjusts the air mass
flow rate to the engine port. Due to the uncertainty and the presence of the nonlinear
components in its dynamical model, the sliding mode control theory is utilized in this work
for the throttle valve angle control system to design a robust controller for this system in the
presence of a nonlinear spring and Coulomb friction. A continuous sliding mode control law
which consists of a saturation function, instead of a signum function, and the integral of
ano
Online service is used to be as Pay-Per-Use in Cloud computing. Service user need not be in a long time contract with cloud service providers. Service level agreements (SLAs) are understandings marked between a cloud service providers and others, for example, a service user, intermediary operator, or observing operators. Since cloud computing is an ongoing technology giving numerous services to basic business applications and adaptable systems to manage online agreements are significant. SLA maintains the quality-of-service to the cloud user. If service provider fails to maintain the required service SLA is considered to be SLA violated. The main aim is to minimize the SLA violations for maintain the QoS of their cloud users. In this res
... Show MoreMII Al-Itabi, WH Abdulameer, OSS Al-Mashhadani, Review of International Geographical Education Online, 2021 - Cited by 1
The semantic analysis is characterized by its ability to describe the communicative pattern and its high efficiency in questioning its semantic manifestations and its deliberative purposes, which helped to understand the symbolic, contextual and cultural manifestations of the structure of the pattern, whether verbal or nonverbal. In order to highlight the role of the semiautomatic approach and its application to a sample of slogans collected from "media, social networking sites, in-kind views, media institutions, etc." to study the components and analyze the contents and highlight the signs. His boat logo is not just uttered as a linguistic transmission of a message, but as a political Tghieria actually . For example, the slogan "People Wan
... Show More