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.
The public budget in Iraq depends on a number of legislations across its fourth stages, starting from preparation to implementation and control; one of these legislations is the amended law of financial management and public Debt. No. (95) In 2004. Accordingly, the public budget cycle faces various failures, some of them resulted from the shortcomings in the legislation depended that effect on the public budget in a way or another; whereas the other failure resulted from no applying the legislation that adversely effect on the public budgeting stages that call for studying them and paying the attention toward them to present the suggestions that contribute in handling and developing public budgetin
... Show MoreIn this study the adsorption of cefixime on to selected Iraqi clay bentonite. The aim of this study is to search for selective active surface in adsorption of the drug and to act as physical antidotes in treatment of poisoning if the drug is taken in quantities higher than the recommended dosages. Quantitative estimation of the drug adsorption has been done by utilizing the technique of UV spectrophotometry in λmax (273) nm at different conditions of temperature (25, 37, 45) ˚C found the adsorption decrease with increase the temperature. Study of clay weight of bentonite (0.1-1.5) gm found the adsorption increase with increase of clay weight, study effect of pH (1.2, 3, 5, 7) on adsorption of bentonite found the optimum adsorption
... Show MoreThe current research was conducted to report the synthesis of alumina powder from Iraqi kaolin. The kaolin was transformed to metakaolin by calcinations at temperature 800 °C for three hours. Then the calcined kaolin was treated with (1.5 M) from H2SO4 for 6 hours to form Al2(SO4)3.12H2O solution. The precipitate was dried at 80oC for 10 hours and calcinations at different temperatures for two hours. The samples which result was characterized by X–Ray diffraction (XRD) and X–Ray fluorescence (XRF). The results indicate to the crystalline hydrate aluminum sulfate for the sample that be as – synthesis and when calcinations at 600 oC transformed into aluminum sulfate phase. The phases of alumina which we obtain consisted of a gamma a
... Show MoreA colorectal polyp (CRP) is a growth on the lining of the colon or rectum. The incidence of CRPs is rapidly increasing with age. They are most often benign. The rectum is the most commonly affected site.
To determine the incidence, clinical presentation, and histopathologic types of CRP in Iraqi patients.
It is a cross-sectional screening study conducted in the medical c
In this work, production of silicon metal at high purity of 99% by using Iraqi–starting materials (Iraqi sand and plant coal)was reported, electric arc–furnaces assembly was manufactured inside, the graphite electrodes were made from graphite scrap, this system is operate to produce about 800 gm /6hr of silicon metal to meet the need for manufacturing silicon oils, resins, solar cells, and electronic parts. The procedure, equipments and analysis data were described as well.
Predicting peterophysical parameters and doing accurate geological modeling which are an active research area in petroleum industry cannot be done accurately unless the reservoir formations are classified into sub-groups. Also, getting core samples from all wells and characterize them by geologists are very expensive way; therefore, we used the Electro-Facies characterization which is a simple and cost-effective approach to classify one of Iraqi heterogeneous carbonate reservoirs using commonly available well logs.
The main goal of this work is to identify the optimum E-Facies units based on principal components analysis (PCA) and model based cluster analysis(MC
... Show MoreIn developing countries, individual students and researchers are not able to afford the high price of the subscription to the international publishers, like JSTOR, ELSEVIER,…; therefore the governments and/or universities of those countries aim to purchase one global subscription to the international publishers to provide their educational resources at a cheaper price, or even freely, to all students and researchers of those institutions. For realizing this concept, we must build a system that sits between the publishers and the users (students or researchers) and act as a gatekeeper and a director of information: this system must register its users and must have an adequate security to e
... Show MoreMany oil and gas processes, including oil recovery, oil transportation, and petroleum processing, are negatively impacted by the precipitation and deposition of asphaltene. Screening methods for determining the stability of asphaltenes in crude oil have been developed due to the high cost of remediating asphaltene deposition in crude oil production and processing. The colloidal instability index, the Asphaltene-resin ratio, the De Boer plot, and the modified colloidal instability index were used to predict the stability of asphaltene in crude oil in this study. The screening approaches were investigated in detail, as done for the experimental results obtained from them. The factors regulating the asphaltene precipitation are different fr
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