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.
Microwave heating is caused by the ability of the materials to absorb microwave energy and convert it to heat. The aim of this study is to know the difference that will occur when heat treating the high strength aluminum alloys AA7075-T73 in a microwave furnace within different mediums (dry and acidic solution) at different times (30 and 60) minutes, on mechanical properties and fatigue life. The experimental results of microwave furnace heat energy showed that there were variations in the mechanical properties (ultimate stress, yielding stress, fatigue strength, fatigue life and hardness) with the variation in mediums and duration times when compared with samples without treatment. The ultimate stress, yielding stress and fatigue streng
... Show MoreIn this research, the kinetic studies of four isoenzymes of Asprtate aminotransferase, which partially purified from the urine of chronic renal failure patients were carried out .The four isoenzymes were obeyed Michaelis-Menton's equation and the optimum concentration of their substrate (Aspartic acid) was (166.5x10-3) mole/liter,and their Km values were determined. Four isoenzymesI,II,III,IV have shown an optimum pH at 7.4.The four isoenzymes obeyed Arrhenius equation up to 37º C and their Ea and Q10 constants were determined .
A simple physical technique was used in this study to create stable and cost-effective copper oxide (CuO) nanoparticles from pure copper metal using the pulsed laser ablation technique. The synthesis of crystalline CuO nanoparticles was confirmed by various analytical techniques such as particle concentration measurement using atomic absorption spectrometry (AAS), field emission scanning electron microscopy (FE-SEM), the energy dispersive X-ray (EDX), and X-ray diffraction (XRD) to determine the crystal size and identify of the crystal structure of the prepared particles. The main characteristic diffraction peaks of the three samples were consistent. The corresponding 2θ is also consistent, and the cytotoxicity of the nanoparticles was
... Show MoreBackground: Pulpotomy is an accepted treatment for the management of cariously exposed pulps in symptom free primary molars to achieve one of the most important goals for Pedodontists, which is the retention of the pulpally involved deciduous teeth healthy until the time of normal exfoliation. The purpose of this study was to evaluate the relative success of pulpotec, formocresol and Mineral Trioxide Aggregate (MTA) in cariously exposed primary molar teeth, using clinical and radiographical examinations. Materials and methods: Thirty nine children with 45 primary molars requiring pulpotomy were selected in this study, 15 teeth treated by each type of pulpotomy medicament. Clinical and radiographical follow up for the patients was performed
... Show MoreThe present study aims to investigate the effect of wheat natural phytase, fermentation and baking processes in the destruction of phytic acid during the process of making wheat bread for some Iraqi mills. The concentration of phytic acid was (850.32, 802.14, 531.84 mg / 100g) for the flour of AL-Brairie, AL-Nesr and AL-Al-Doura mills respectively. At the end of the fermentation processes, the decrease in the concentration of phytic acid in the samples produced from the flour obtained from the three mills was (47.06, 26.98, 40.00%) respectively, while inorganic phosphorus concentration in all treatments increased by 32.4, 42.37 and 36.21 %, respectively. It was found that the activity of wheat natural phytase enz
... Show MoreThis study was designed to investigate the effect of sesame oil on lipid profile and liver enzyme in male albino rats treated with Carbone tetrachloride (CCl4).
Forty adult male rats were divided into four equal groups, first group was daily administrated with tap water, the second group was injected with CCl4 (80mg/kg.BW/day), the third group was administrated with sesame oil (150mg/kg.BW/day) and the fourth group was injected with CCl4 (80mg/kg.BW/day) and was administrated with sesame oil (150 mg /kg.BW /day) for 30 days. The statistical results of the present study showed a significant (p<0.05) increase in the level of cholesterol , triglycerides and Low density
... Show Moren the present work, a study is carried out to remove chromium (III) from
aqueous solution by: activated charcoal , attapulgite and date palm leaflet powder
(pinnae). The effect of various parameters such as contact time, and temperature has
been studied. The isotherm equilibrium data were well fitted by Freundlich and
Langmuir isotherm models. The adsorption capacity of chromium (III) that was
observed by activated charcoal , attapulgite and date palm leaflet powder (pinnae)
increased with the rise of temperature when the concentrations of Cr (III) were 600,
700 and 100mg/L respectively. The greatest adsorption capacity ofactivated
charcoal , attapulgite and date palm leaflet powder (pinnae) at 10°C was 7.51, 5.3
Background: Oncogenesis in the oral cavity is widely believed to result from cumulative genetic alterations that cause a transformation of the mucosa from normal to dysplastic to invasive carcinoma. The p16 gene produces p16 protein, which in turn inhibits phosphorylation of retinoblastoma (Rb), p16 play a significant role in early carcinogenesis. A number of epidermal growth factor receptor (EGFR) family, HER2/neu, has received much attention because of its therapeutic implications. The aims of the study were to evaluate and compare the immunohistochemical expression of the cell cycle protein P16 INK4a and c-erbB2 (HER2/neu) in NOM, OED, and OSCC. Correlate both marker expression with each other as well as with various clinicopathological
... Show MoreThe liability limit liability limit and reinsurance re factors insurance are important in influencing the achievement of company's competitive goals and achieve success and excellence in the market and because the limit of liability and reinsurance aimed to increase output with the stability of inputs leading to increased insurance business and increase profits. The study was based on two variables presidents interact with each other to form the intellectual and philosophical framework has two ( the limit of liability and reinsurance ) . The study was launched from a problem expressed by a number of targeted questions answered from the elucidation of theoretical philosophy and intellectual goals of these variables and highlight the impor
... Show MoreThis work involves synthesis and characterization of some new 1, 3, 4-thiadiazole or pyrazoline derivatives heterocyclic containing indole ring. The new 2-amino-1, 3, 4thiadiazole derivatives[IV] and [V]a, b were synthesized by cyclization reaction of 2-methyl1H-indole-carbothiosemicarbazide[III] in H2SO4 acid or by reaction of indole-3-acetic acid or indole-3-butanoic acid with thiosemicarbazide in the presence of phosphorous oxychloride, respectively. Amide derivatives [VI]-[VIII] were synthesized by the reaction equimolar of 2amino-1, 3, 4-thiadiazoles and (acetyl chloride, benzoyl chloride, anisoyl chloride and heptanoyl chloride) in DMF and pyridine as accepter. The new pyrazolone derivatives [XI]a, b were s
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