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.
Ultrasonic pulse echo measurements on porous alumina as ceramic
material with porosities ranging from (20-40)% showed effect of volume
fraction of porosity on both thermal and elastic properties. A quadratic relationships, by using a least squares method, is deduced for the dependence of the shear velocity, longitudinal velocity, shear modulus, Young's modulus, bulk modulus, Poisson 's ratio, Debye temperature, specific heat, and thermal conductivity on the total porosity. By these relationships, the thermal and elastic properties results of pore-free alumina were calculated. The elastic properties results of
... Show MoreThe reconciliation of tax reconciliation is one of the legal methods used by the financial authority in Iraq, which is done with the taxpayer
The research dealt with the weakness of tax revenues for many reasons, including tax evasion, which led to the search for ways to reduce evasion to increase the tax revenue, and settlement reconciliation one of these means .
The research proceeded from the premise that the use of a more broadly settled settlement would govern the tax evasion of taxpayers.
The researchers used a series of studies and previous research, books and other sources related to the subject of research, and this was done through the theoretical framework, and the practical aspect that included the fin
... Show MoreGas adsorption phenomenon on solid surface has been used as a mean in separation and purification of gas mixture depending on the difference in tendencies of each component in the gas mixture to be adsorbed on the solid surface according to its behaviour. This work concerns to study the possibilities to separate the gas mixture using adsorption-desorption phenomenon on activated carbon. The experimental results exhibit good separation factor at temperature of -40 .
Recently emerging pandemic SARS CoV-2 conquered our world since December 2019. Continuous efforts have been done to find out effective immunization and precise treatment stetratigies A way from therapeutic options that were tried in SARS CoV-2, an increased attention is directed to predict natural products and mainly phytochemicals as collaborative measures for this crisis. In this review, most of the mentioned compounds specially flavonoids (biacalin, hesperidin, quercetin, luteolin,, and phenolic (resveratrol, curcumin, and theaflavin) exert their effect through interfering with the action of one or more of this proteins (spike protein, papain like protease, 3 chymotrypsin like cysteine protease, and RNA dependent RNA
... Show MoreThe discourse surrounding lingual sovereignty within the African postcolonial context is profoundly intertwined with the fabric of cultural identity and self-determination. Language serves not merely as a conduit for communication but as a repository for a people's collective consciousness, encapsulating their traditions, thoughts, and perspectives. In the realm of postcolonial literature, this dialogue often grapples with the paradox of expressing indigenous narratives through the linguistic tools of former colonizers. Chinua Achebe's seminal work, "Things Fall Apart," exemplifies this conundrum, artfully weaving the orature and culture of Umuofia within the English language. Achebe's choice to write in English—a language imposed upon hi
... Show MoreIn recent days, the escalating need to seamlessly transfer data traffic without discontinuities across the Internet network has exerted immense pressure on the capacity of these networks. Consequently, this surge in demand has resulted in the disruption of traffic flow continuity. Despite the emergence of intelligent networking technologies such as software-defined networking, network cloudification, and network function virtualization, they still need to improve their performance. Our proposal provides a novel solution to tackle traffic flow continuity by controlling the selected packet header bits (Differentiated Services Code Point (DSCP)) that govern the traffic flow priority. By setting the DSCP bits, we can determine the appropriate p
... Show MoreThe novel Vierordt’s approach, or simultaneous equation method, was created and validated for the concurrent determination of vincristine sulfate (VCS) and bovine serum albumin (BSA) in pure solutions utilizing UV spectrophotometry. It is simple, precise, economical, rapid, reliable, and accurate. This method depends on measuring absorbance at two wavelengths, 296 nm and 278 nm, which correspond to the λmax of VCS and BSA in deionized water, respectively. The calibration curves of VCS and BSA are linear at concentration ranges of 10–60 μg/mL and 200–1600 μg/mL, with correlation coefficient values (R2) of 1 and 0.999, respectively. The limits of detection (LOD) and quantification (LO
... Show MoreThis research is based on the idea of showing the extent to which the public relies on satellite channels as sources for news of the demonstrations in Iraq .This was the essence of the problem for which the researcher set several goals, including knowing the public’s confidence in the news of these satellite channels and comparing them with others. The researcher chose an available intended sample of (117) respondents in Baghdad - Karkh and Rusafa by adopting the survey method and applying a questionnaire form and the theory of media dependence for the period from 15/11/2019 to 1/1/2021 . By using statistical methods, the researcher reached many results, the most important of which are: Satellite channels are a source for 79% of the pu
... Show MoreBackground: Hair loss is a common distressing disease and challenging problem for many dermatologist. Telogen effluvium is the most common hair loss disease in which nutritional deficiencies may precipitate the disease through their effect on hair structure and growth.
Study Aim : Validating role of serum ferritin level and body mass index in Chronic Telogen Effluvium and analyzing association between these factors with socioeconomic, demographic, gynecological factors and weight loss effect. Establishing a nutritional preventive advice to improve treatment successfulness and decrease the disease occurrence.
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