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.
Objectives: Recently, there have been important advances in the clinical application of targeted hybrid near-infrared (NIR) fluorescent-radioactive tracers. ICG-99mTc-nanocolloid, for example, is already being used by some centres for sentinel lymph node biopsy in head and neck cancer. The radioactive component allows imaging at depths which would not be possible with NIR alone and, once exposed, the NIR fluorescence reporter can be imaged at very high resolution. Gamma detection is currently carried out with a separate hand-held gamma camera or with a non-imaging probe. Visualisation of NIR fluorescence during surgery requires a dedicated NIR camera, several of which are available commercially. We describe a novel hand-held hybrid NIR-gamm
... Show MoreAddressed the problem of the research is marked (experimentation in caves fee) concept and its role in experimentation deviate Display Num formal charges caves. The search came in four sections: general framework for research and identified the research problem and the need for him. With an indication of the importance of his presence. Then specify the search for the goals of (revealed the nature and role of experimentation in determining the nature of Manifesting fee documented on the walls of caves), followed by the establishment of the three search limits (objectivity, the temporal and spatial) were then determine the terms related to the title. Then provide the theoretical framework and indicators that resulted from academic theorizi
... Show MoreThe current research aims through its chapters to verify the relationship and impact of strategic leadership as an independent variable in the marketing performance as a respondent variable, in a leap cement plant, and try to come up with a set of recommendations that contribute to enhancing the practice and adoption of the two variables in the organization under discussion. And based on the importance of the research topic to the community, and to the researched organization and its members, the analytical and analytical approach was adopted in the completion of this research, and the research community included a leap cement plant in Anbar Governorate, while the research sample was represented by (department heads, and people o
... Show MoreOne of the troublesome duties in chemical industrial units is determining the instantaneous drop size distribution, which is created between two immiscible liquids within such units. In this work a complete system for measuring instantaneous droplet size is constructed. It consists of laser detection system (1mW He-Ne laser), drop generation system (turbine mixer unit), and microphotography system. Two immiscible liquids, water and kerosene were mixed together with different low volume fractions (0.0025, 0.02) of kerosene (as a dispersed phase) in water (as a continuous phase). The experiments were carried out at different rotational speed (1180- 2090 r.p.m) of the turbine mixer. The Sauter mean diameter of the drops was determined by la
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It is easy to talk about democracy, but it is difficult to practice. We talk about postmodernism, but difficult to be embodied in the ground. Yet, the age of democracy and modernity at the same time prompted the researcher to try to find a media concept for it. It does not mean that this concept has not yet formed. But the rooting of democracy and the approaching of states and groups towards it, made it necessary to by studied again.
The relationship between democracy and the media has made them look like one concept. The existence of one is linked to the existence of the other. The reality is only a linguistic formulation, but the social and cultural aspect is related to democracy origin
... Show MoreThis paper aims at exploring the impact of the Iraq-Iran war in the poetry of Adnan Al-Sayegh. Al-Sayegh participation in this war makes him a first hand witness to the atrocities of the trenches and fight in the first lines. This war did not only change his life and world view for good, it changes the nature of his poetry as well. As aresult, war becomes a central issue not only in the poetry Al-Sayegh wrote in the 1980s and 1990s Iraq, but also in the exile.
Key Words: War, Al-Sayegh, Poetry.
Soils that cause effective damages to engineer structures (such as pavement and foundation) are called problematic or difficult soils (include collapsible soil, expansive soil, etc.). These damages occur due to poor or unfavorited engineering properties, such as low shear strength, high compressibility, high volume changes, etc. In the case of expansive soil, the problem of the shrink-swell phenomenon, when the soil reacts with water, is more pronounced. To overcome such problems, soils can be treated or stabilized with many stabilization ways (mechanical, chemical, etc.). Such ways can amend the unfavorited soil properties. In this review, the pozzolanic materials have been selected to be presented and discussed as chem
... Show MoreMedium Access Control (MAC) spoofing attacks relate to an attacker altering the manufacturer assigned MAC address to any other value. MAC spoofing attacks in Wireless Fidelity (WiFi) network are simple because of the ease of access to the tools of the MAC fraud on the Internet like MAC Makeup, and in addition to that the MAC address can be changed manually without software. MAC spoofing attacks are considered one of the most intensive attacks in the WiFi network; as result for that, many MAC spoofing detection systems were built, each of which comes with its strength and weak points. This paper logically identifies and recognizes the weak points
and masquerading paths that penetrate the up-to-date existing detection systems. Then the
The current research aims to identify the types and rates and the reasons for the crimes that are with the origin and sexual scattered deviation between the fabric of Iraqi society, which reticent about because of the culture of the community, where he offered a researcher investigator justice and tribal leaders and lawyers about proportion and the types and causes of crime is the origin of sexual deviance and finally a question asked a researcher in 1000 young lived their adolescence days of the former regime (1979-2003) from going to brothels researcher found that 920 people and 92% had gone to the house to practice adultery and that 70 of them, and by 7% had engaged in masturbation only and that 10 of them have not committed anything
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